John Murtagh's General Practice 8e

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Page ix

Contents The authors Foreword

v viii

Acknowledgments Preface

xi

xii

Making the most of your book Reviewers

xiii

xvii

Laboratory reference values

xx

Normal values: worth knowing by heart Abbreviations

xxii

xxiii

Part 1

The basis of general practice

1

Chapter 1

The nature, scope and content of general practice

Chapter 2

Consulting skills

Chapter 3

Communication skills

Chapter 4

Counselling skills

Chapter 5

Health promotion and patient education

Chapter 6

Prevention in general practice

Chapter 7

Research and evidence-based medicine

Chapter 8

Inspection as a clinical skill

Part 2

Diagnostic perspective in general practice

Chapter 9

A safe diagnostic model

Chapter 10

Depression

Chapter 11

Diabetes mellitus

Chapter 12

Drug and alcohol problems

Chapter 13

Anaemia

Chapter 14

Endocrine and metabolic disorders

Chapter 15

Spinal dysfunction

Chapter 16

Urinary tract infection

Chapter 17

Malignant disease

Chapter 18

Baffling viral and protozoal infections

Chapter 19

Baffling bacterial infections

2

9 15 23 36

45 56

65 71

72

81 91 109

126 134

147 150 159 184

168

Chapter 20

Infections of the central nervous system

Chapter 21

Connective tissue disease and the systemic vasculitides

Chapter 22

Neurological dilemmas

Chapter 23

Genetic conditions

Part 3

Presenting symptoms and problem solving in general practice

Chapter 24

Abdominal pain

Chapter 25

Arthritis

Chapter 26

Anorectal disorders

299

Chapter 27

Thoracic back pain

308

Chapter 28

Low back pain

Chapter 29

Bruising and bleeding

Chapter 30

Chest pain

Chapter 31

Constipation

Chapter 32

Cough

Chapter 33

Deafness and hearing loss

Chapter 34

Diarrhoea

Chapter 35

Dizziness/vertigo

Chapter 36

Dyspepsia (indigestion)

Chapter 37

Dysphagia

450

Chapter 38

Dyspnoea

455

Chapter 39

The painful ear

Chapter 40

The red and tender eye

Chapter 41

Pain in the face

498

Chapter 42

Fever and chills

509

Chapter 43

Faints, fits and funny turns

Chapter 44

Haematemesis and melaena

Chapter 45

Headache

Chapter 46

Hoarseness

Chapter 47

Jaundice

Chapter 48

Nasal disorders

Chapter 49

Nausea and vomiting

Chapter 50

Neck lumps

198 204

214 232

254 276

321 341

351 373 385 400

410 429 439

468 482

519 534

537 555 559 577 594

588

253

Chapter 51

Neck pain

Chapter 52

Shoulder pain

Chapter 53

Pain in the arm and hand

Chapter 54

Hip, buttock and groin pain

Chapter 55

Pain in the leg

Chapter 56

The painful knee

Chapter 57

Pain in the foot and ankle

Chapter 58

Walking difficulty and leg swelling

Chapter 59

Palpitations

Chapter 60

Sleep disorders

Chapter 61

Sore mouth and tongue

Chapter 62

Sore throat

Chapter 63

Tiredness/fatigue

Chapter 64

The unconscious patient

Chapter 65

Urinary disorders

Chapter 66

Visual failure

Chapter 67

Weight change

Part 4

Mental health

Chapter 68

Depression and other mood disorders

Chapter 69

The disturbed patient

Chapter 70

Anxiety disorders

Chapter 71

Difficult behaviours

Part 5

Chronic disease management

Chapter 72

Allergic disorders including hay fever

Chapter 73

Asthma

Chapter 74

Chronic obstructive pulmonary disease

Chapter 75

Cardiovascular disease

Chapter 76

Chronic heart failure

Chapter 77

Hypertension

Chapter 78

Dyslipidaemia

Chapter 79

Chronic kidney disease

Chapter 80

Obesity

599 613 627 644

657 675 696 714

721 735 746

757 766 774 783 795 808 Page x

819 820

825 841 850 859 860

868 889 893 901 918 931

923

881

Chapter 81

Osteoporosis

937

Chapter 82

Chronic pain

942

Part 6

Child and adolescent health

Chapter 83

An approach to the child

Chapter 84

Specific problems of children

962

Chapter 85

Surgical problems in children

977

Chapter 86

Common childhood infectious diseases (including skin eruptions)

Chapter 87

Behavioural and developmental issues and disorders in children

Chapter 88

Child abuse

Chapter 89

Emergencies in children

Chapter 90

Adolescent health

Part 7

Women’s health

Chapter 91

Cervical cancer screening

Chapter 92

Family planning

1054

Chapter 93

Breast disorders

1065

Chapter 94

Abnormal uterine bleeding

Chapter 95

Lower abdominal and pelvic pain in women

Chapter 96

Premenstrual syndrome

Chapter 97

The menopause

Chapter 98

Vaginal discharge

Chapter 99

Vulvar disorders

Chapter 100

Basic antenatal care

Chapter 101

Postnatal care

1139

Part 8

Men’s health

1149

Chapter 102

Men’s health: an overview

Chapter 103

Scrotal pain

Chapter 104

Inguinoscrotal lumps

Chapter 105

Disorders of the penis

Chapter 106

Disorders of the prostate

Part 9

Sexual health

Chapter 107

The subfertile couple

951 952

1014 1022 1037 1045 1046

1082 1101

1105 1113 1122 1130

1150

1154 1159 1169 1177

1187 1188

1089

988 1001

Chapter 108

Sexual health

Chapter 109

Sexually transmitted infections

Chapter 110

Intimate partner violence and sexual assault

Part 10

Problems of the skin

Chapter 111

A diagnostic and management approach to skin problems

Chapter 112

Pruritus

Chapter 113

Common skin problems

Chapter 114

Acute skin eruptions

Chapter 115

Skin ulcers

Chapter 116

Common lumps and bumps

Chapter 117

Pigmented skin lesions

Chapter 118

Hair disorders

1317

Chapter 119

Nail disorders

1328

Part 11

Accident and emergency medicine

Chapter 120

Emergency care

Chapter 121

Stroke and transient ischaemic attacks

Chapter 122

Thrombosis and thromboembolism

Chapter 123

Common skin wounds and foreign bodies

Chapter 124

Common fractures and dislocations

Part 12

Health of specific groups

Chapter 125

The elderly patient

Chapter 126

End of life/palliative care

Chapter 127

The health of Aboriginal and Torres Strait Islander peoples

Chapter 128

Refugee health

Chapter 129

Travellers’ health and tropical medicine

Appendix Index

1478 1483

1196 1206 1218

1225 1226

1236 1246 1268

1280 1290 1308

1339

1340 1361 1368 1374 1388

1411 1412 1428

1447 1455

1438

Page xi

Acknowledgments Professor Murtagh would like to thank the Publication Division of the Royal Australian College of General Practitioners for supporting his past role as Medical Editor of Australian Family Physician, which provided an excellent opportunity to gather material for this book. Acknowledgment is also due to those medical organisations that have given permission to use selected information from their publications. They include the Preventive and Community Medicine committee of the RACGP (Guidelines for Preventive Activities in General Practice), Therapeutic Guidelines Limited, the Hypertension Guideline Committee: Research Unit RACGP (South Australian Faculty) and the Medical Observer, publishers of A Manual for Primary Health Care, for permitting reproduction of Appendices I–IV. For decades, Therapeutic Guidelines (TG) has set the gold standard for practice guidelines, beginning with the benchmark antibiotic guidelines. The panels for the various disciplines include experts from many fields whose collective wisdom and evidence base in their deliberations inspires confidence and authority for treatment decisions. General practitioners also have input in the panels. The authors of Murtagh’s General Practice wish to thank Therapeutic Guidelines Limited for the outstanding information which provides an authoritative framework for our publication. Therapeutic Guidelines is the ultimate therapeutic reference across all categories, from analgesics and antibiotics to ulcers and wound management. Special thanks to the late Chris Sorrell for his art illustration, and to Nicki Cooper, Jenny Green and Caroline Menara for their skill and patience in typing the manuscript. Many of the quotations at the beginning of chapters appear in either Robert Wilkins (ed), The Doctor’s Quotation Book, Robert Hale Ltd, London, 1991, or Maurice B. Strauss (ed), Familiar Medical Quotations, Little, Brown & Co., New York, 1958. Thanks also to Professor Roger Pepperell, Dr Bruce Mugford, Dr Lucie Stanford, Dr Mohammad Shafeeq Lone, Dr Brian Bedkobar for content advice and Professor Chris White for technical support, Dr Ebrahim Pishan, Dr Joseph Turner and Lesley Rowe for reviewing the manuscript, and to the publishing and production team at McGraw-Hill Education (Australia) for their patience and assistance in so many ways. Finally, thanks to Dr Ndidi Victor Ikealumba for his expert review of General Practice sixth edition, tropical medicine and his subsequent contribution.

Photo credits Figure 31.4, p. 383 is used with permission from Harrison’s Principles of Internal Medicine 20th edn (2018) by JL Jameson, AS Fauci, DL Kasper, SL Hauser, DL Longo and J Loscalzo,

McGraw-Hill Education US. Figure 124.11, p. 1398 is used with permission from Clinical Sports Medicine 4th edn (2009) by P Brukner and K Khan, McGraw-Hill Educations (Australia) Figure 28.6, p. 328 is used with permission from Practical Office Orthopedics (2017) by E Parks, McGraw-Hill Education US. Photographs appearing on the pages below are from The Colour Atlas of Family Medicine (2008) and 2nd edn (2013) and The Color Atlas and Synopsis of Family Medicine 3rd edn (2019) by RP Usatine, MA Smith, EJ Mayeaux Jr and H Chumley, McGraw-Hill Education US, with the kind permission of the following people: Dr Richard P Usatine: Figure 119.7 (photo), p. 1330. Dr Richard P Usatine, 2e: Figure 61.1, p. 747; Figure 61.2, p. 749; Figure 61.8, p. 755; Figure 116.21, p. 1302. Dr Richard P Usatine, 3e: Figure 8.3, p. 68; Figure 25.5, p. 284; Figure 30.13, p. 362; Figure 39.12, p. 478; Figure 61.6, p. 752; Figures 84.6 and 84.7, p. 971; Figure 84.8, p. 972; Figure 93.5, p. 1071; Figure 98.5, p. 1119; Figure 99.1, p. 1124; Figure 109.5, p. 1214; Figure 113.11, p. 1258; Figure 117.2, p. 1310; Figures 118.5 and 118.6, p. 1324; Figure 119.7 (photo), p. 1330. Dr William Clark: Figure 39.3, p. 471; Figure 39.6, p. 472; Figure 39.7, p. 473; Figure 48.1, p. 580. Frontline Medical Communications: Figure 88.4, p. 1019; Figure 115.6, p. 1288.

Page xii

Paul D. Comeau: Figure 40.6, p. 489. DEA: Figure 12.6, p. 117. Dr Nicolette Deveneau: Figure 93.2, p. 1069. Javier La Fontaine DPM: Figure 115.5, p. 1287. Dr Michelle Rowe: Figure 12.5, p. 117. Dr C. Blake Simpson: Figure 46.1, p. 557. Dr Marc Solioz: Figure 8.1, p. 66. Dr Eric Kraus: Figure 112.5, p. 1240. Dr Hugh Newton-John: Figure 9.1, p. 73; Figure 20.4, p. 200; Figure 50.3, p. 59.7; Figure 86.3, p. 991. Professor Barry Firkin and Professor Hatem Salem: Figure 9.4, p. 77.

Dr Peter Ryan: Figure 21.4, p. 209. Dr Marissa Lassere: Figure 21.5, p. 209. Professor John Masterton: Figure 25.12, p. 292; Figure 26.2, p. 300; Figure 26.3, p. 302. Bruce Black: Figure 39.8, p. 474; Figure 39.10, p. 476. John Colvin and Joseph Reith: Figure 40.2, p. 487; Figure 40.3, p. 487; Figure 40.4, p. 488; Figure 40.5, p. 489; Figure 40.8, p. 491; Figure 40.12, p. 493; Figure 40.14, p. 493. Robin Marks: Figure 57.13, p. 710; Figure 112.10, p. 1243; Figure 116.17, p. 1300. Dr Peter Couran: Figure 113.15, p. 1260. Dr John Troller: Figure 116.9, p. 1297.

Page xiii

Preface The discipline of general practice has become complex, expansive and challenging, but nevertheless remains manageable, fascinating and rewarding. John Murtagh’s General Practice attempts to address the issue of the base of knowledge and skills required in modern general practice. Some of the basics of primary healthcare remain the same. In fact, there is an everlasting identity about many of the medical problems that affect human beings, be it a splinter under a nail, a stye of the eyelid, a terminal illness or simply stress-related anxiety. Many of the treatments and approaches to caring management are universal and timeless. This text covers a mix of traditional and modern practice with an emphasis on the importance of clinical reasoning, early diagnosis, strategies for solving common presenting problems, continuing care, holistic management and ‘tricks of the trade’. One feature of our discipline is the patient who presents with undifferentiated problems featuring an overlap of organic and psychosocial components. There is the constant challenge to make an early diagnosis and identify the ever-lurking, life-threatening illness. Hence the ‘must not be missed’ catch cry throughout the text. To reinforce this awareness, ‘red flag pointers’ to serious disease are included where appropriate. The general practice diagnostic model, which pervades all the chapters on problem solving, is based on the authors’ experience, but readers can draw on their own experience to make the model work effectively for themselves. This eighth edition expands on the challenging initiative of diagnostic triads (or tetrads), which act as a brief aide-memoire to assist in identifying a disorder from three (or four) key symptoms or signs. A particular challenge in the preparation of the text was to identify as much appropriate and credible evidence-based information as relevant. This material, which still has its limitations, has been combined with considerable collective wisdom from experts, especially from the Therapeutic Guidelines series. A key objective of this publication is to achieve a balance between science and the art of general practice. To provide updated accuracy and credibility, the authors have had the relevant chapters peer reviewed by independent experts in the respective disciplines. These consultants are acknowledged in the reviewers section. The revised editions also have the advantage of co-authorship from experienced general practitioner Dr Jill Rosenblatt. Additional authors include Dr Clare Murtagh, a general practitioner with experience in medical education, and Dr Justin Coleman, past president of the Australasian Medical Writers Association with special interests in ‘Choosing wisely’ programs and evidence-based medicine. A comprehensive book such as this one, which presents a basic overview of primary medicine, cannot possibly cover all the medical problems likely to be encountered. An attempt has been made, however, to focus on problems that are common, significant, preventable and treatable. Recent content includes expanded material on genetic disorders and infectious diseases, particularly coronaviruses and acute respiratory distress syndrome. John Murtagh’s General Practice is written as a user-friendly text with the recent graduate, the

international medical graduate and the medical student in mind. However, all primary-care practitioners will gain useful information from the book’s content.

Page xiv

Making the most of your book Diagnostic strategy models Diagnostic strategy models for common presenting problems form the backbone of this book. General Practice is renowned for this unique and powerful learning feature, which was introduced in the first edition.

Key facts and checkpoints Key facts and checkpoints provide accurate statistics and local and global contexts.

The staff of Asclepius The staff of Asclepius icon highlights diseases for when you are specifically searching for information on a particular disease.

Page xv

Red and yellow flags Red and yellow flags alert you to potential dangers. Red is the most urgent, but yellow also requires careful consideration.

Clinical framework Clinical framework based on major steps of clinical features, investigations, diagnosis, management and treatment reflects the key activities in the daily tasks of general practitioners.

Seven masquerades checklist This unique feature of the book reminds you of potential and hidden dangers underlying patient presentations.

Diagnostic triads Key features that may discriminate between one disease and another are clearly presented.

Page xvi

Evidence-based research Evidence-based research is recognised with a full chapter on research in general practice and evidence base, including more on qualitative models. In addition, substantial references are provided for every chapter.

Extensive coverage of paediatric and geriatric care, pregnancy and complementary therapies Extensive coverage of paediatric and geriatric care, pregnancy and complementary therapies is integrated throughout, as well as devoted chapter content providing more comprehensive information in these areas.

Practice tips Practice tips consist of key points that are of use in the clinical setting.

Page xvii

Clinical photos Clinical photos provide authentic, visual examples of many conditions and serve as either a valuable introduction or confirmation of diagnosis.

Full colour illustrations Full colour illustrations are provided, with more than 600 diagrams in the clean, simple style that has proved so popular.

Significantly enhanced index The index has more sub-categories with bold page numbers indicating the main treatment of a topic, enabling you to quickly pinpoint the most relevant information. Page numbers in italics refer to figures and tables. Entries with ‘see also’ have crossreferences to related, but more specific information on the topic.

Patient education resources Indicates where you can find relevant information from Murtagh’s Patient Education, eighth edition, to photocopy and hand out to patients.

Page xviii

Reviewers Content consultants The authors are indebted to the many consultants who reviewed parts of the manuscript relevant to their areas of expertise and provided help and advice. Dr Marion Bailes, Dr Joanne Gardiner and Dr Kate Walker

refugee health

Associate Professor Deborah Bateson

family planning

Dr Roy Beran

epilepsy; neurological dilemmas

Dr James Best

depression, anxiety, male health, child and adolescent health, communication skills

Dr Clare Boema

family planning

Dr John Boxall

palpitations

Dr Penny Burns

disaster medicine, pandemics

Dr Jill Cargnello

hair disorders

Dr Belinda Chan

breast disorders

Dr Paul Coughlin and Professor Hatem Salem

bruising and bleeding; thrombosis and thromboembolism

Mr Rod Dalziel

shoulder pain

Dr David Dilley

pain in the arm and hand

Dr David Dunn and Dr Hung The Nguyen

the health of Indigenous peoples

Dr Robert Dunne

common skin wounds and foreign bodies

Associate Professor John Eden

the menopause

Professor Jon Emery

genetic disorders, malignant disease

Dr Fiona Fargie

sexually transmitted infections

Genetic Health Services, Victoria

genetic disorders

Dr Lindsay Grayson and Associate Professor Joseph Torresi

travel medicine, the returned traveller and tropical medicine

Mr John Griffiths

pain in the hip and buttock

Professor Michael Grigg

pain in the leg

Dr Gary Grossbard

the painful knee

Dr Eliza Hannam

postnatal care

Dr Peter Hardy-Smith

the red and tender eye; visual failure

Associate Professor Peter Holmes

cough; dyspnoea; asthma; COPD

Professor Michael Kidd, Dr Ron McCoy and Dr Alex Welborn

human immunodeficiency virus infection

Professor Gab Kovacs

abnormal uterine bleeding; the subfertile couple

Professor Even Laerum

research in general practice

Mr Peter Lawson (deceased), Dr Sanjiva Wijesinha and Dr Andrew Pattison

men’s health, disorders of the penis, prostatic disorders, scrotal pain, inguinoscrotal lumps

Dr Jessica Lowe

cervical cancer screening

Dr Peter Lowthian

arthritis

Mr Frank Lyons

common fractures and dislocations

Dr John Mackellar

child abuse and domestic violence

Dr Linda Mann

basic antenatal care

Professor Barry McGrath

hypertension

Dr Joe McKendrick

malignant disease

Dr Kim Matthews

the subfertile couple

Dr Luke Murtagh

pain and its management

Professor Robyn O’Hehir

allergic disorders, including hayfever

Dr Michael Oldmeadow

tiredness

Dr Frank Panetta

chest pain

Dr Geoff Quail

pain in the face, sore mouth and tongue

Mr Ronald Quirk

pain in the foot and ankle

Dr Ian Rogers

emergency care

Professor Avni Sali

abdominal pain, lumps in the breast, jaundice, constipation, dyspepsia, nutrition

Dr Stanley Santiagu and Dr Jemma Dalrymple

abnormal uterine bleeding

Dr Ronald Schweitzer

intimate partner violence and sexual assault

Dr Deshan Sebaratnam and Dr Margit Polcz

problems of the skin

Dr Heidi Spillane

sexual health

Dr Hugo Standish

urinary tract infection, chronic kidney failure

Dr Richard Stark

neurological diagnostic triads

Dr Liz Sturgiss

obesity

Professor Geoff Sussman

skin ulcers

Dr Paul Tallman

stroke and transient ischaemic attacks

Dr Alison Walsh

breastfeeding, postnatal breast disorders

Professor Greg Whelan

alcohol problems, drug problems

Dr Lynne Wray

vaginal discharge, vulvar disorders

Dr Alan Yung

fever and chills, sore throat

Dr Ronnie Yuen

diabetes mellitus, thyroid and other endocrine disorders Page xix

Page xx

Laboratory reference values These reference values and ranges are given in the system of international units (SI) and may vary from laboratory to laboratory. An asterisk (*) indicates that paediatric reference ranges differ from the adult range given. Electrolytes/renal Sodium

135–145 mmol/L

Potassium*

3.5–5.0 mmol/L

Chloride

95–110 mmol/L

Bicarbonate

23–32 mmol/L

Urea

3–8.0 mmol/L

Creatinine

♀ 50–110; ♂ 60–120 μmol/L

eGFR

>60 mL/min/1.72 m2

Calcium*

2.10–2.60 mmol/L (total)

Phosphate

0.90–1.35 mmol/L

Magnesium*

0.65–1.00 mmol/L

Uric acid*

♀ 0.12–0.40; ♂ 0.15–0.45 mmol/L

Liver function/pancreas Bilirubin*

11.1 mmol/L. The OGTT should be reserved for true borderline cases and for diagnosing gestational diabetes, where a 75 mg OGTT is recommended at 24–28 weeks’ gestation.7

Prediabetes This is the condition where the VPG is elevated above the normal range (i.e. 6.1–6.9) but does not satisfy the type 2 diagnostic criteria. It includes two states: impaired fasting glucose (IFG)

impaired glucose tolerance (IGT) A diagnosis of prediabetes is not a call to start medication, but it increases the urgency of promoting lifestyle changes such as weight reduction and increased physical activity. Urinalysis is unreliable in diagnosis since glycosuria occurs at different plasma glucose values in patients with different kidney thresholds. For a summary of diagnosis of diabetic states, refer to FIGURE 11.2

.

FIGURE 11.2 Blood glucose levels: venous plasma (mmol/L) Source: Reproduced with permission from RACGP. General Practice Management of Type 2 Diabetes: 2016–18. East Melbourne, 2016 (book available from: http://www.diabetesaustralia.com.au or http://www.racgp.org.au).

Table 11.3

Test

Interpreting diagnostic tests for diabetes8 Normal

Intermediate hyperglycaemia

Diabetes

Venous blood glucose concentration

Oral glucose tolerance test

fasting

up to 6 mmol/L

random

up to 6 mmol/L

2-hour venous blood glucose concentration

up to 7.7 mmol/L

HbA1c

6.1–6.9 mmol/L

≥7 mmol/L

≥11.1 mmol/L 7.8–11 mmol/L

≥11.1 mmol/L

≥48 mmol/mol (6.5%)

Diabetes in children Page 95 A study by Sinah and colleagues detected impaired glucose tolerance in 25% of 55 obese children (4 to 10 years of age) and 21% of 112 obese adolescents (11 to 18 years of age).9 Type 2 diabetes was identified in 4% of obese adolescents. However, over 30% of newly diagnosed diabetes in children and adolescents is upon presentation with diabetic ketoacidosis. Children with type 1 diabetes usually exhibit the classic features of polyuria, polydipsia, weight loss and lethargy. Be aware of unusual presentations such as urinary disorders including enuresis or daytime wetting accidents when a misdiagnosis of urinary infection or some other condition is sometimes forthcoming. The diagnosis can be made by an elevated random or fasting blood sugar. Oral glucose tolerance tests are inappropriate in the very young. Upon diagnosis it is appropriate to refer the child or adolescent to a multidisciplinary diabetes team. The sick child with a high blood glucose is an emergency presentation of type 1 diabetes until proven otherwise.

Gestational diabetes Gestational diabetes is the new onset of abnormal glucose tolerance during pregnancy. Pregnancy is diabetogenic for those with a genetic predisposition. All pregnant women should be screened at 24–28 weeks with a 75 g oral glucose tolerance test (OGTT). The definition of gestational diabetes mellitus (GDM) by the Australasian Diabetes in Pregnancy Society has widened considerably in the past two decades, and far more women are captured by the lower thresholds.10 The 2014 consensus definition of gestational diabetes is a fasting plasma glucose of ≥5.1 mmol/L, or a post-75 g oral glucose load at 1 hour ≥10.0, or at 2 hours 8.5–11.0. Refer to CHAPTER 100 .

Diabetes in the elderly

The incidence of diabetes rises with age. The elderly have increased mortality and morbidity from the disease, but also are at increased risk from aggressive treatment regimens. Careful monitoring is required, especially with adverse drug effects aggravated by polypharmacy and comorbidities. Special issues include diet, foot care and postural hypotension.

Complications of diabetes Complications may occur in patients with both type 1 and type 2 diabetes, even despite early diagnosis and treatment (see FIG. 11.3 ).

FIGURE 11.3 The complications of diabetes

People with type 1 diabetes still have a significantly reduced life expectancy. The main causes of death are diabetic nephropathy and vascular disease (myocardial infarction and stroke). Diabetes causes both macrovascular and microvascular complications but microvascular disease is specific to diabetes. Special attention should be paid to the association of type 2 diabetes with obesity, hypertension and dyslipidaemia—the ‘deadly quartet’.6,11 Macrovascular complications include: ischaemic/coronary heart disease cerebrovascular disease peripheral vascular disease An analysis of type 2 diabetes in the HOPE study12,13 showed a benefit of ramipril to reduce the risk of: death (24%) myocardial infarction (22%) stroke (33%) cardiovascular death (37%) overt nephropathy (24%) Consider organs/problems affected by diabetes under the mnemonic ‘KNIVES’: Kidney Nerves Infection Vessels Eyes Skin

Microvascular disease The small vessels most affected from a clinical viewpoint are the retina, nerve sheath and kidney glomerulus. In younger people it takes about 10 to 20 years after diagnosis for the problems of diabetic retinopathy, neuropathy and nephropathy to manifest.

Nephropathy Prevention of diabetic nephropathy is an essential goal of treatment. Early detection of the yardstick, which is microalbuminuria, is important as the process can be reversed with optimal control, particularly of blood pressure. The dipstick method is unreliable and the preferred hospital method of 24-hour urine collection is considered impractical in general practice. Screening is done simply by a first morning urine sample to determine the albumin–creatinine ratio (see CHAPTER 79 ). ACE inhibitors (or angiotensin II receptor blockers if a cough develops) should be used for evidence of hypertension. Page 96

Retinopathy and maculopathy Retinopathy develops as a consequence of microvascular disease of the retina. Its prevalence is related to the duration of illness but up to 20% of people with type 2 diabetes have diabetic retinopathy at the time of diagnosis. The European multicentre study14,15 showed that diabetes is the single most common cause of blindness in European adults in the 16–64 years age groups. Assessment of the fundus by an expert is recommended every 1–2 years, via direct ophthalmoscopy (with dilated pupils), retinal photography or, if necessary, fluorescein angiography. Early diagnosis of serious retinopathy is vital since the early use of laser photocoagulation may delay and prevent visual loss.

Neuropathy The following types of neuropathy may occur: radiculopathy (diabetic lumbosacral radiculoplexopathy) sensory polyneuropathy isolated or multiple mononeuropathy isolated peripheral nerve lesions (e.g. median nerve) cranial nerve palsies (e.g. III, VI) amyotrophy autonomic neuropathy, which may lead to: erectile dysfunction postural hypotension and syncope impaired gastric emptying (gastroparesis)

diarrhoea delayed or incomplete bladder emptying loss of cardiac pain → ‘silent’ ischaemia hypoglycaemic ‘unawareness’ sudden arrest, especially under anaesthetic Page 97

Infections People with poorly controlled diabetes are prone to infections, especially: skin: mucocutaneous candidiasis (e.g. balanitis, vulvovaginitis), staphylococcal infections (e.g. folliculitis) urinary tract: cystitis (women), pyelonephritis and perinephric abscess lungs: pneumonia (staphylococcal, streptococcal pneumonia), tuberculosis

Diabetic metabolic complications Hypoglycaemia Diabetic ketoacidosis Hyperosmolar hyperglycaemia Lactic acidosis

Other complications Cataracts Refractive errors of eye Sleep apnoea Depression Musculoskeletal: neuropathic joint damage (Charcot-type arthropathy), tendon rupture Foot ulcers (related to neuropathy)

Prevention of diabetes

Several large studies have demonstrated it is possible to prevent or delay the onset of type 2 diabetes in those at risk.14,15 This involves intensive lifestyle intervention in individuals who are overweight with impaired glucose tolerance or raised fasting blood glucose. The ongoing DiRECT trial demonstrates that even once type 2 diabetes has been present for a few years, remission of diabetes is a realistic aim in general practice, using intense dietary measures for 3 months followed by structured support for weight loss management.16 The primary strategy is to follow the SNAP guidelines (Smoking, Nutrition, Alcohol, Physical activity), particularly with a view to weight loss.17 The essentials are healthy eating, weight loss and physical activity. This represents an important approach that GPs can recommend to their patients at risk. The enormous health gains that can be made in this prediabetic population by concentrating on SNAP outweigh any later health gains from diabetes medication (see CHAPTER 80 ).

Management of diabetes The main objectives for the GP are to prevent the development of cardiovascular disease and other complications. Aim to achieve:4 1. reduction of ‘lifestyle’ risks—weight, smoking, low physical activity 2. strict glycaemic control as measured by HbA1c (target varies with circumstance, but usually ≤7%) 3. blood pressure control (≤140/90 mmHg, lower if tolerated)18 4. control of blood lipid levels Note: Refer to the estimations of cardiovascular risk (see FIGS. 75.1 CHAPTER 75 ).

and 75.2

in

Management principles Provide detailed and comprehensive patient education, support and reassurance. Achieve control of presenting symptoms. Achieve blood pressure control (huge impact on mortality risk). Develop a diabetes care plan. Emphasise the importance of the diet: good nutrition, adequate protein, complex carbohydrates (low carb diets are an option), restrict fats and sugars. Promptly diagnose and treat urinary tract infection.

Page 98

Treat and prevent life-threatening complications of ketoacidosis or hyperosmolar coma. Treat and prevent hypoglycaemia in those taking insulin and oral hypoglycaemic agents. Organise self-testing of blood glucose for those on insulin. Detect and treat complications of diabetes—neuropathy, nephropathy, retinopathy, vascular disease. Ensure immunisation schedule, including influenza and pneumococcus, is updated.

Monitoring techniques Blood glucose estimation (mainly useful if on insulin; fasting and postprandial) Urine glucose (of limited usefulness) Urine or blood ketones (for type 1 diabetes) Glycated haemoglobin (HbA1c) (3-monthly) Microalbuminuria (usually urine ACR, regarded as an early and reversible indicator of nephropathy) Blood pressure Serum lipid levels Kidney function (serum urea/creatinine eGFR) ECG Control guidelines are summarised in FIGURE 11.4

and TABLE 11.4

.

FIGURE 11.4 Blood glucose control guidelines for diabetes management

Table 11.4

Suggested guidelines for glycaemic control (blood glucose mmol/L)4,5,19

Before meals (fasting)

Ideal 7.7

After meals (2 hours postprandial)

11

HbA1c %*

≤7

>8

*HbA1c is an index reflecting the mean plasma glucose levels over the preceding 2–3 months (assume a reference range of 4.5–8%).

Blood glucose monitoring at home This is done using a glucose meter (glucometer). A wide variety of meters and smart phone apps are available: patients will require advice about what suits them.

How often and when? Type 1 diabetes: four times a day (before meals and before bedtime) at first and for problems twice a day (at least once) may settle for 1–2 times a week (if good control) Type 2 diabetes: important for those on insulin, not routinely recommended for oral medication (monitor with HbA1c instead, in most circumstances) more useful for pregnant women, frail elderly, heavy machinery operators or symptomatic hypoglycaemia

Goals of management4,5 All people with diabetes should be encouraged to maintain the following goals for optimum management: Blood glucose (fasting)

ideal 4–6 mmol/L NHMRC 6–8 mmol/L

Blood glucose (postprandial)

8–10 mmol/L

HbA1c

≤7% (53 mmol/mol)

Total cholesterol

2 per year confirmed sterile pyuria other features of kidney disease, e.g. haematuria pregnancy Others: with failed antibiotic treatment with recent international travel

Basic investigations include: MCU—microscopy and culture Kidney function tests: plasma urea and creatinine, eGFR Intravenous urogram (IVU) and/or ultrasound Special considerations: In children: micturating cystourethrogram (MCUG), nuclear scans (DMSA, MAG3) In adult males: consider prostatic infection studies if IVU normal In severe pyelonephritis: ultrasound or IVU (urgent) to exclude obstruction In pregnant women: ultrasound to exclude obstruction

Treatment (non-pregnant women)3,7 Use for 10 days in women with known urinary tract abnormality: trimethoprim 300 mg (o) daily for 3 days (first choice) or cephalexin 500 mg (o) 12 hourly for 5 days or amoxicillin + clavulanate 500/125 mg (o) 12 hourly for 5 days or nitrofurantoin 100 mg (o) 6 hourly for 5 days or norfloxacin 400 mg (o) 12 hourly for 3 days (if resistance to above agents proven and if susceptible, Caution about tendinopathy and tendon rupture.) No follow-up is required if women remain asymptomatic after treatment. Note: Avoid using important quinolones—norfloxacin or ciprofloxacin—as first-line agents. Cotrimoxazole is not first line because it has no advantage over trimethoprim and has more side effects. Treatment failures are usually due to a resistant organism or an underlying

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abnormality of the urinary tract.

Pregnant women8,9 UTI in pregnant women requires careful surveillance. Asymptomatic bacteriuria should always be excluded during early pregnancy because it tends to be blown into a full infection. Treat asymptomatic bacteriuria in pregnancy as for acute cystitis. Treatment of acute cystitis (empirical): cephalexin 500 mg (o) 12 hourly for 5 days or nitrofurantoin 100 mg (o) 6 hourly for 5 days or amoxicillin + clavulanate 500/125 mg (o) 12 hourly for 5 days Repeat MCU 1--2 weeks after completion.

Adult males7 Consider the cause (see risk factors above). Investigations: MCU, U&E, ultrasound. Treatment (empirical, while awaiting investigation): trimethoprim 300 mg (o) daily for 7 days or cephalexin 500 mg (o) 12 hourly for 7 days or amoxicillin + clavulanate 500/125 mg (o) 12 hourly for 7 days or nitrofurantoin 100 mg (o) 6 hourly for 7 days or norfloxacin 400mg (o) 12 hourly for 7 days Note: All males with a UTI should be investigated to exclude an underlying abnormality, e.g.

prostatitis, obstruction.

Acute pyelonephritis10 Urine microscopy and culture is mandatory. Mild cases can be treated with oral therapy alone for a longer duration than the recommended course for uncomplicated cystitis. For empirical therapy, use amoxicillin/clavulanate (875/125 mg (o) 12 hourly) for 10–14 days or ciprofloxacin (500 mg (o) 12 hourly) for 7 days. Modify empirical therapy based on culture and susceptibility results. For severe infection with suspected septicaemia, admit to hospital and treat initially with parenteral antibiotics after taking urine for microscopy and culture, and blood for culture. It is a particular problem if acquired in pregnancy. amoxicillin/ampicillin 2g IV 6 hourly plus gentamicin 4–6 mg/kg/day, single daily IV dose Follow with oral therapy for a total of 14 days. Drug levels of gentamicin require monitoring. Gentamicin can be replaced with IV cefotaxime or ceftriaxone. Consider investigation for an underlying urinary tract abnormality, especially in men and in patients that remain unwell after 72 hours of treatment.

Recurrent or chronic urinary tract infections11,12 Page 156 Recurrent infections occur as a relapse of a previously treated infection or because of re-infection, often with differing organisms. Persistent (chronic) UTIs indicate that the organism is resistant to the antimicrobial agents employed or that there is an underlying abnormality such as a kidney stone or a chronically infected prostate in the male patient. MCU is mandatory. Such infections may be treated with prolonged courses of an appropriate antibiotic or removal of the focus of infection.13

In men and children, an anatomical abnormality is usual, while recurrent cystitis in women often occurs despite a normal tract.

Treatment14 Treat an acute episode of recurrent UTI as for cystitis or pyelonephritis

Prevention including antibiotic prophylaxis14 In some female patients with recurrent UTI a single dose of a suitable agent within 2 hours after

intercourse is adequate but, in more severe cases, courses may be taken for 3–6 months or on occasions longer. Adult doses given: trimethoprim 150 mg (o) nocte or cephalexin 250 mg (o) nocte Non-antibiotic strategies Drinking more water is recommended. Women are generally encouraged to gently wash or wipe their bottom from front to back after opening their bowels. Continuous prophylaxis with the bacteriostatic agent methenamine hippurate 1g (o) bd may also be helpful, although evidence is inconsistent. In postmenopausal women with atrophic vaginitis, topical oestrogen therapy may reduce the risk of recurrent UTIs. Cranberry products are no longer recommended.

Urinary tract infection in children By the age of 10 years, about 3% of boys15 and 10% of girls will have had at least one episode of a urinary tract infection. UTI in infants and very young children is often kidney in nature and may be associated with generalised symptoms such as fever, vomiting, diarrhoea and failure to thrive. Offensive urine may be noted. Causes of ‘smelly’ urine in children are urinary infection and/or dehydration, especially with gastroenteritis. Symptoms of dysuria and frequency appear only after the age of 2 years when the child is able to indicate the source of the discomfort. In a girl or boy (rare presentation) with symptoms of dysuria and frequency, an underlying abnormality may be present with a reported incidence of vesicoureteric reflux (VUR) as high as 40% and scarred kidneys (reflux nephropathy) in 27%.3 Thus the early detection of children with VUR and control of recurrent kidney infection could prevent the development of scars, hypertension and chronic kidney failure. Radiological investigation of children with UTIs shows normal kidneys in approximately 66% and reflux in approximately 33%. Children require further investigation with an ultrasound if they are seriously unwell with a UTI, have complicated or recurrent UTIs. Children with recurrent UTIs may require additional imaging and specialist input is recommended. A low threshold for further investigation is appropriate in children 50 mm/hr C-reactive protein—elevated Mild anaemia (normochromic, normocytic)

Treatment for uncomplicated disease8 Refer any patient with suspected GCA urgently. Prednisolone

Starting dose: temporal (giant cell) arteritis: 1 mg/kg (usually 60 mg) (o) daily initially for 4 weeks (+ aspirin 100 mg/day) then gradual reduction according to ESR/CRP polymyalgia rheumatica: 15 mg (o) daily for 4 weeks, then reduce daily dose by 2–5 mg every 4 weeks to 10 mg/day, then 1 mg every 4–8 weeks to zero Taper down gradually to the minimum effective dose (often 1 part of CNS is involved, although not necessarily at time of presentation. Episodes are separated in time and space. Practically MS can only be diagnosed after a second relapse or when the MRI shows new lesions.11 An early diagnosis requires evidence of contrast-enhancing lesions or new T2 lesions on the MRI indicating dissemination in time. The diagnostic criteria is based on the internationally accepted 2010 McDonald criteria (refer to: www.nice.org.uk).12,13 Other neurological disorders such as infections (e.g. encephalitis), malignancies, spinal cord compression, spinocerebellar degeneration and others must be excluded.

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Investigation Lumbar puncture: oligoclonal IgG detected in CSF in 90% of cases14 (only if necessary) Visual evoked potentials: abnormal in about 90% of cases MRI scan: usually abnormal, demonstrating MS lesions in about 90% of cases14

Course and prognosis The course is variable and difficult to predict. An early onset (50% (F); >60% (M)

Increased serum ferritin level: >200 mcg/L (F); >300 mcg/L (M) (see CHAPTER 13

)

CT, MRI or FerriScan—increased iron deposition in liver Liver biopsy (if liver function test enzymes are abnormal or ferritin >1000 mcg/L or hepatomegaly)—FerriScan now preferred Genetic studies: HFE gene—a C282Y and/or H63D mutation Screen first-degree relatives (serum ferritin levels and serum transferrin saturation in older relatives and genetic testing in younger ones). No need to screen before adulthood. HbEPG gene for pregnant patient and partner. Routine screening not recommended

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Note: Full blood count (FBE) and erythrocyte sedimentation rate are normal.

Management Refer for specialist care Weekly venesection 500 mL (250 mg iron) until serum iron stores are normal (may take at least 2 years), then every 3–4 months to keep serum ferritin level 1 hour Arthritis in three or more regions Swelling in five or more joints Bilateral compression tenderness of the metatarsophalangeal joints Symmetry of the areas affected Presence of rheumatoid nodules Rheumatoid factor positivity Raised inflammatory markers (ESR/CRP) in absence of infection Anticyclic citrullinated peptide antibody positivity Bony erosions evident on radiographs of the hands or feet, although these are uncommon in early disease

Key points If the RA factor is positive, it is non-specific—order the anti-CCP antibody to confirm the diagnosis. RA has a strong cardiovascular risk factor.

Principles of management12,22 Give patient education support and appropriate reassurance. The diagnosis generally has distressful implications, and so the patient and family require careful explanation and support. Some have little or no long-term problems but even in mild cases, continuing care and medical supervision is important. There has been a radical shift from palliation to early induction of disease remission, to prevent joint damage and reduce morbidity from malignancy (especially lymphoma) and cardiovascular disease. Page 290 Since many studies show disease progression in the first 2 years, relative aggressive treatment with disease-modifying antirheumatic drugs (DMARDs) from the outset is advisable, rather than to start stepwise with analgesics and NSAIDs only.23 Use a team approach where appropriate, including an early specialist referral for obvious or suspected RA or positive anti-CCP for diagnosis and collaborative support. Fully assess the person’s functional impairment and impact on home life, work and social activity. Involve the family in decision making. Make judicious use of pharmaceutical agents. For serious cases, consultant collaboration is essential. Review regularly, continually assessing progress and drug tolerance. The disease activity can be monitored with plain X-rays, ultrasound of hands (especially if hands are thick), CRP ± ESR. Specific advice Rest and splinting. This is necessary where practical for any acute flare-up of arthritis. Exercise. It is important to have regular exercise, especially walking and swimming. Have hydrotherapy in heated pools. Smoking cessation. This is strongly recommended. Referral. Referring to physiotherapists and occupational therapists for expertise in exercise supervision, physical therapy and advice regarding coping in the home and work is important. Joint movement. Each affected joint should be put daily through a full range of motion to keep it mobile and reduce stiffness. Diet. Although there is no special diet that seems to cause or cure RA, a nourishing, wellbalanced diet is common sense and obesity must be avoided. Some evidence supports both a

Mediterranean diet and vegetarianism.24 There is also some evidence that avoiding animal fats (dairy products and some meats) and using fish oils is beneficial.25 Therapies used in the management of rheumatoid arthritis are presented in TABLE 25.4 Table 25.4

.

Therapies used in the management of rheumatoid arthritis22,26

Education (rest, literature, weight loss, joint protection advice) NSAIDs Simple analgesics DMARDs: Conventional synthetic DMARDs Immunosuppressants: azathioprine cyclosporin leflunomide methotrexate Biological DMARDs Cytokine inhibitors anti-TNF α agents: abatacept, adalimumab, certolizumab, etanercept, infliximab, golimumab, rituximab anti-interleukin-1 agents; tocilizumab Gold salts Quinolones: hydroxychloroquine chloroquine Others: D-penicillamine sulfasalazine Glucocorticoids: oral prednisolone intra-articular intravenous (steroid ‘pulses’) Fish body oil Physical therapy (hydrotherapy, isometric exercises) Occupational therapy (splints, aids and appliances) Orthopaedic surgery (synovectomy, joint replacement, arthrodesis, plastic hand surgery)

Chiropody, footwear, insoles Source: Reilly and Littlejohn22,26

Management (pharmacological) Best under consultant direction. NSAIDs are effective and still have a place, but the adverse effects are a problem. The use of DMARDs and biological DMARDs improves long-term outcomes. Methotrexate is the ‘backbone’ of treatment, and should be continued when starting other DMARDs. Supplementation with folic acid can improve gastrointestinal symptoms and reduce the risk of liver dysfunction. Beware of the increased risk of infection in patients on combination DMARD regimes. When indicated, vaccination for pneumococcus, influenza, hepatitis A and B and HPV is recommended for all DMARDs. Any pain should be managed with paracetamol or NSAIDs. Avoid opioid analgesics if possible. Glucocorticoids are appropriate for flares of RA. Page 291

Fish oil Fish oil in doses to deliver 4 g of omega-3 long-chain polyunsaturated fatty acids daily (typically 0.2 g/kg) over several months has been shown to reduce symptoms and the need for NSAIDs through its anti-inflammatory activity.12,25 Glucocorticoids Oral use should be considered in those with severe disease as a temporary adjunct to DMARD therapy and where other treatments have failed or are contraindicated. The dose is prednisolone 10 mg (o) daily. Avoid doses higher than 15 mg daily if possible. Intra-articular injections of depot preparations are effective in larger joints. Disease-modifying antirheumatic drugs (DMARDs)

These agents target synovial inflammation and prevent joint damage. The choice depends on several factors, but is best left to the specialist coordinating care. In most patients with recently diagnosed RA, methotrexate is the cornerstone of management and should be commenced as early as possible. Initial dose: methotrexate 5–10 mg (o) once weekly on a specified day, increasing to maximum of 25 mg weekly or SC depending on clinical response and toxicity. Add folic acid 5–10 mg twice weekly (not on the day methotrexate is given).12 Biological DMARDs (bDMARDs) are the newer agents, which should be considered if remission is not achieved with appropriate methotrexate monotherapy, ‘triple therapy’ or other combinations. All bDMARDs are more effective when combined with methotrexate. As a rule, don’t use two biologicals together.

Warning: All practitioners should be aware of the increased risk of infectious diseases such as the atypical pneumonias, tuberculosis and listeriosis while taking bDMARDs. All patients should report unusual or unexpected fever or symptoms. Injection site reactions are common.

Standard initial drug therapy Monotherapy with methotrexate (or occasionally another DMARD) is standard. Less than 20% will reach disease remission; if not achieved, increase the dose or consider combination therapy. Many people are managed on conventional DMARDs. Combination therapy Consider standard triple therapy: methotrexate + sulfasalazine + hydroxychloroquine. Triple therapy can be used if methotrexate monotherapy has failed or initially on diagnosis, depending on the severity of the disease. Monitoring for FBE, LFTs and annual eye checks is necessary. Several other double combinations may be used (e.g. methotrexate with cyclosporin, leflunomide or a bDMARD).

Connective tissue diseases The connective tissue disorders have the common feature of arthritis or arthralgia. Refer to CHAPTER 21 . Arthritis is the commonest clinical feature of SLE (over 90%).13 It is a symmetrical polyarthritis involving mainly small and medium joints, especially the proximal interphalangeal and carpal

joints of the hand. It is usually non-erosive and non-deforming, although deformities of fingers and thumbs can occur due to laxity of ligaments, tendons and capsules, causing joint instability. The initial presentation is similar to RA. Scleroderma can present as a polyarthritis affecting the fingers of the hand in 25% of patients, especially in the early stages. Soft tissue swelling produces a ‘sausage finger’ pattern. Arthralgia and arthritis occur in about 50% of those with polymyositis/dermatomyositis and may be the presenting feature before the major feature of muscle weakness and wasting of the proximal muscles of the shoulder and pelvic girdles appear. The small joints of the hand are usually affected and it may resemble RA.

Crystal arthritis Arthritis, which can be acute, chronic or asymptomatic, is caused by a variety of crystal deposits in joints. The three main types of crystal arthritis are monosodium urate (gout), calcium pyrophosphate dihydrate (CPPD) and calcium phosphate (usually hydroxyapatite).27 Refer to TABLE 25.5 . Page 292

Table 25.5

Crystal-induced disorders

Crystals Monosodium urate

Associated disease/syndrome Acute gout Tophaceous gout Asymptomatic Chronic gouty arthritis

Typical joints or region affected Metatarsophalangeal joint of big toe Also: other foot joints, ankle, knee and patellar bursa, wrist, fingers

Calcium pyrophosphate dihydrate (CPPD)

Acute pseudogout Destructive arthropathy (like RA) Asymptomatic (most common)

Knee, wrist In older people >60 years (average age 72) F > M (2.7:1)

Basic calcium phosphate

Acute calcific periarthritis Destructive

Shoulder (supraspinatus)

arthropathy Acute arthritis

Gout (monosodium urate crystal disorder) Gout is an abnormality of uric acid metabolism resulting in hyperuricaemia and urate crystal deposition. Urate crystals deposit in: joints—acute gouty arthritis soft tissue—tophi and tenosynovitis urinary tract—urate stones Four typical stages of gout are recognised: Stage 1—asymptomatic hyperuricaemia Stage 2—acute gouty arthritis Stage 3—intercritical gout (intervals between attacks) Stage 4—chronic tophaceous gout and chronic gouty arthritis Asymptomatic hyperuricaemia: 10 times more common than gout13 Elevated serum uric acid (>0.42 mmol/L in men, > 0.36 mmol/L in women) Absence of clinical manifestations Usually does not warrant treatment

Clinical features Typical clinical features of gout include:12 mainly a disorder of men (5–8% prevalence) onset earlier in men (40–50) than women (60+) acute attack: excruciating pain in great toe (see FIG. 25.12 skin over joint—red, shiny, swollen and hot exquisitely tender to touch

), early hours of morning

relief with colchicine, NSAIDs, corticosteroids can subside spontaneously (3–10 days) without treatment

FIGURE 25.12 Gout showing typical red, shiny, swollen arthritis of the first MTP joint with desquamation of the skin

Causes/precipitating factors Foods: seafood, meat, liver, kidney Alcohol excess (e.g. binge drinking) Surgical operation Starvation, dehydration, acute illness Drugs (FACT: frusemide, aspirin, alcohol, cytotoxic drugs, thiazide diuretics) Chronic kidney disease Myeloproliferative disorders Lymphoproliferative disorders (e.g. leukaemia) Sugary soft drinks,28 fruit juices containing fructose Cytotoxic agents (tumour lysis) Hypothyroidism

Low-dose aspirin Others The arthritis Monoarthritis in 90% of attacks: MTP joint great toe—75% other joints—usually lower limbs: other toes, mid foot, ankles, knees Polyarticular onset is more common in old men and may occur in DIP and PIP joints of fingers. No synovial joint is immune. Page 293 Refer to FIGURE 25.13

.

FIGURE 25.13 Gout: possible joint distribution Other features Prone to recurrence

Tophi in ears, elbows (olecranon bursa), big toes, fingers, Achilles tendon (can take many years) Can cause patellar bursitis Can get cellulitis (does not respond to antibiotics) Nodular gout More common in postmenopausal women with kidney impairment taking diuretic therapy. Causes pain and tophaceous deposits around osteoarthritic interphalangeal (especially DIP) joints of fingers.

Diagnosis Synovial fluid aspirate of affected joint, bursa or tophus → typical uric acid crystals using compensated polarised microscopy; this should be tried first (if possible) as it is the only real diagnostic feature Elevated serum uric acid (up to 30% can be within normal limits with a true acute attack)27 X-ray: punched out erosions at joint margins

Management Management of gout includes these principles: good advice and patient education information provision of rapid pain relief preventing further attacks prevention of destructive arthritis and tophi dealing with precipitating factors and comorbid conditions (e.g. alcohol dependence, obesity, CKD, polycythaemia vera, diabetes, hypertension) The acute attack12,28,29 NSAIDs (except aspirin), in full dosage, are first-line and effective. Give orally until symptoms abate (up to 4–5 days) then continue for one week or Corticosteroids:

prednisolone 15–30 mg (o) daily until symptoms abate,30,31 then decrease gradually or local corticosteroid injection (but very painful) up to a maximum of two affected sites31 or intramuscular (in difficult cases) e.g. tetracosactrin 1 mg or Colchicine: colchicine 1 mg (o) statim, then 0.5 mg 1 hour later as a single dose 1-day course (total dose is 1.5 mg)12,28 Note: Must be given early Avoid if kidney impairment Avoid use with macrolide antibiotics, e.g. clarithromycin, especially in CKD Avoid long-term use Note: Avoid changes to urate-lowering therapy during an acute attack of gout Avoid aspirin and urate pool lowering drugs (probenecid, allopurinol, sulfinpyrazone)30 Monitor kidney function and electrolytes Long-term therapy When acute attack subsides, preventive measures with the aim of treating through diet include: weight reduction a healthy, well-balanced diet avoidance of purine-rich food, such as organ meats (liver, brain, kidneys, sweetbread), tinned fish (sardines, anchovies, herrings), shellfish and game reducing red and processed meats, fried chips and sweet treats reduced intake of alcohol

reduced intake of sugary soft drinks (fructose)32

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good fluid intake (e.g. water—2 litres a day) avoidance of drugs such as diuretics (thiazides, frusemide) and salicylates/low-dose aspirin wearing comfortable shoes avoidance of prolonged fasting

Prevention (drug prophylaxis) Allopurinol (a xanthine oxidase inhibitor) is the first-line drug of choice: dose 100–300 mg daily. Indications: frequent acute attacks (or even >1 attack in 12 months) tophi or chronic gouty arthritis kidney stones or uric acid nephropathy hyperuricaemia Adverse effects: rash (2%) severe allergic reaction (rare) Precautions: beware of kidney insufficiency and elderly patients—use lower doses beware of drug interactions: azathioprine and 6 mercaptopurine—potentially lethal amoxicillin—prone to rashes avoid initiating or changing allopurinol during an acute attack Method: treatment of intercritical and chronic gout Commence allopurinol 6–8 weeks after last acute attack. Start with 50 mg daily for 4 weeks and then increase by 50 mg every 2 to 4 weeks to maximum 900 mg daily.

Check uric acid level after 4 weeks: aim for level 30 minutes Awoken with pain during second half of night Improvement with exercise and not relieved by rest Limitation of lumbar spine motion in sagittal and frontal planes Chest expansion ↓ relative to normal values Unilateral sacroiliitis (grade 3 to 4) Bilateral sacroiliitis (grade 2 to 4)

Reactive arthritis Reactive arthritis is a form of arthropathy in which non-septic arthritis and often sacroiliitis develop after an acute urogenital infection (usually Chlamydia trachomatis) or an enteric infection (e.g. Salmonella, Shigella). DxT urethritis + conjunctivitis ± iritis + arthritis → reactive arthritis

The arthritis (Reiter syndrome), which commences 1–3 weeks post infection, tends to affect the larger peripheral joints, especially the ankle (talocrural) and knees, but the fingers and toes can be affected in a patchy polyarthritic fashion. Mucocutaneous lesions, including keratoderma blennorrhagica and circinate balanitis, may occur, although the majority develop peripheral arthritis only (see FIG. 25.14 ).

FIGURE 25.14 Possible clinical features of reactive arthritis Page 296

Enteropathic spondyloarthritidy Inflammatory bowel disease (ulcerative colitis, Crohn disease and Whipple disease) may rarely be associated with peripheral arthritis and sacroiliitis.

Psoriatic arthritis Like reactive arthritis, this can develop a condition indistinguishable from ankylosing spondylitis. It is therefore important to look beyond the skin condition of psoriasis, for about 5% will develop psoriatic arthropathy. It can have several manifestations: 1. mainly DIP joints 2. identical RA pattern but RA factor negative 3. identical ankylosing spondylosis pattern with sacroiliitis and spondylitis 4. monoarthritis, especially knees 5. severe deformity or ‘mutilans’ arthritis

Unclassified spondyloarthritides This category seems to be frequently encountered in family practice. The person clearly has a spondyloarthropathy but fails to meet the criteria for any one of the individual entities within the group. A typical patient is a young male in his third decade with a painful knee or other joint, unilateral (or bilateral) back pain with one of the entheseal problems (e.g. plantar fasciitis).

Investigations for spondyloarthritides X-rays: radiological sacroiliitis is central to the diagnosis changes include narrowing of SIJs, margin irregularity, sclerosis of peri-articular bone and eventually bony fusion. Spondylitis usually follows ESR and CRP: most patients have an elevated ESR and CRP at some stage of their disease HLA-B27: this test has low specificity and has limited value except that it predicts risk to offspring if positive Microbiology: in patients with a history of reactive arthritis, cultures should be obtained from

the urethra, faeces, urine and blood32

Principles of management Identify the most active elements of the disease and treat accordingly. Provide patient and family education with appropriate reassurance: this is vital. Stress that, although the disease is non-curable, treatment is effective and long-term prognosis generally good. Provide regular assessment and support. Give genetic counselling—in cases of ankylosing spondylitis with positive HLA-B27, the risk to offspring is significant. Give advice regarding work, especially with posture. Acute anterior uveitis requires prompt treatment and monitoring by an ophthalmologist. Refer for physiotherapy for exercises, stretching program, postural exercises and hydrotherapy. Appropriate physiotherapy slows deterioration in spinal function.33 Consider referral for occupational therapy. Pharmacological agents:12 NSAIDs (e.g. indomethacin 75–200 mg (o) daily or 100 mg rectally nocte daily or ketoprofen 100 mg rectally nocte to control pain, stiffness and synovitis) sulfasalazine (if NSAIDs ineffective) intra-articular corticosteroids for severe monoarthritis and intralesional corticosteroids for enthesopathy Refer for advice on above and especially for DMARD and bDMARD therapy.

Cautions Careful monitoring is required with NSAIDs and sulfasalazine. Systemic corticosteroids are not indicated. Immunosuppressants (low-dose weekly methotrexate) and bDMARDs may be needed for severe intractable problems with psoriasis and reactive arthritis. These conditions should be managed in collaboration with a specialist.

When to refer Consider referring most severe true inflammatory disorders for diagnosis and initiation of treatment (e.g. RA, spondyloarthropathy, connective tissue disorders and suspicion of a vasculitide) Osteoarthritis: generalised joint pain associated systemic symptoms deteriorating joint function intractable pain (especially at rest) if surgical procedure is contemplated12 Rheumatoid arthritis:

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all patients initially persistent inflammation of a joint or joints patient ill and corticosteroids contemplated if a surgical procedure is contemplated Spondyloarthropathies: initial referral for confirmation of diagnosis and initiation of treatment disease unresponsive to conventional treatment sudden deterioration in symptoms, especially pain onset of uveitis or other ocular complications adverse drug reactions Undiagnosed arthritis in presence of constitutional symptoms Suspicion of a suppurative or serious infective condition (e.g. septic arthritis, endocarditis, brucellosis) Children with evidence of juvenile idiopathic arthritis (e.g. Still syndrome)

Practice tips Morning stiffness and pain, improving with exercise = RA. Flitting polyarthritis and fever = rheumatic fever; ?endocarditis; ?SLE. Polyarthritis (usually PIPs) and rash = viral arthritis or drug reaction. If rheumatoid arthritis involves the neck, beware of atlantoaxial subluxation and spinal cord compression. If the patient is young—think of SLE. If a patient returns from overseas with arthralgia, think of drug reactions, hepatitis, Lyme disease, but if the pain is intense consider dengue fever. Consider the possibility of Lyme disease in people with a fever, rash and arthritis who have been exposed to tick bites overseas. If a patient presents with Raynaud phenomenon and arthritis, especially of the hands, consider foremost RA, SLE and systemic sclerosis. Avoid the temptation to apply on doubtful grounds a broad label such as arthritis or rheumatoid, or a precise diagnosis such as RA, and introduce drugs.34

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Gout Osteoarthritis Rheumatoid arthritis Page 298

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Cleland LG, James MJ, Proudman SM. Fish oil: what the prescriber needs to know. Arthritis Research and Therapy, 2006; 8(1): 202–11.

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Reilly P, Littlejohn G. Current treatment concepts in arthritis. Aust Fam Physician, 1989; 18: 1499–1509.

27

Hall S. Crystal arthritis: a clinician’s view. Aust Fam Physician, 1991; 20: 1717–24.

28

Graf SW et al. Australian and New Zealand recommendations for the diagnosis and management of gout: integrating systematic literature review and expert opinion in the 3e Initiative. Int J Rheum Dis, 2015; 18(3): 341–51.

29

Buckley N (Chair). Australian Medicines Handbook. Adelaide: Australian Medicines Handbook Pty Ltd, 2018: 704–6.

30

Wechalekar MD et al. The efficacy and safety of treatments for acute gout: results from a series of systematic literature reviews including Cochrane reviews on intraarticular glucocorticoids, colchicine, nonsteroidal antiinflammatory drugs, and interleukin-1 inhibitors. J Rheumatol Suppl, Sept 2014; 92: 15–25.

31

Janssens H et al. Use of oral prednisolone or naproxen for treatment of gout arthritis: a double blind randomised equivalence trial. Lancet, 2008; 371(9627): 1854–60.

32

Choi HK, Curham G. Soft drinks, fructose consumption and the risk of gout in men: prospective cohort study. BMJ online, 31 January 2008: 39449.819271 BE.

33

Edmonds JP. Spondyloarthropathies. Med J Aust, 1997; 166: 214–18.

34

Hart FD. Early clinical diagnosis of 12 forms of arthritis. Modern Medicine Australia, 1989; March: 34–40.

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26 Anorectal disorders

Duncan ill with very bad piles—operated on last night, or, since that sounds alarming, lanced. Can’t really sympathise with that particular disease, though the pain is terrible. Must laugh. VIRGINIA WOOLF 1934, DIARY ENTRY Anorectal problems are common in family practice and tend to cause anxiety that is often related to the fear of cancer. Although the majority of rectal bleeding and lumps have non-cancerous causes, the fear of cancer may be well founded, so it is important to consider it in any presentation of rectal bleeding. Anorectal problems include: pain lumps discharge bleeding pruritus Common anorectal conditions are illustrated in FIGURE 26.1

.

FIGURE 26.1 Common anorectal conditions

Anorectal pain (Proctalgia) The complaint may be that defecation is painful or almost impossible because of anorectal pain.

Causes Pain without swelling: anal fissure anal herpes ulcerative proctitis proctalgia fugax solitary rectal ulcer

Page 300

tenesmus Painful swelling: perianal haematoma strangulated internal haemorrhoids abscess: perianal, ischiorectal pilonidal sinus fistula-in-ano (intermittent) anal carcinoma

Anal fissure Anal fissures cause pain on defecation and usually develop after a period of constipation (may be a brief period) and tenesmus. Other associations are childbirth and opioid analgesics.1 Sometimes the pain can be excruciating, persisting for hours and radiating down the back of both legs. Anal fissures, especially if chronic, can cause minor anorectal bleeding (bright blood) noted as spotting on the toilet paper.

Examination On inspection the anal fissure is usually seen in the anal margin—90% are situated in the midline posteriorly (6 o’clock). The fissure appears as an elliptical ulcer involving the lower third of the anus from the dentate line to the anal verge (see FIG. 26.2 ).1

FIGURE 26.2 Anal fissure with prominent skin tag situated in the mid posterior position of the anal verge: the 6 o’clock position Digital examination and sigmoidoscopy are difficult because of painful anal sphincter spasm. If there are multiple fissures, Crohn disease should be suspected. Crohn fissures look different, being indurated, oedematous and bluish in colour. In chronic anal fissures a sentinel pile is common and in longstanding cases, a subcutaneous fistula is seen at the anal margin, with fibrosis and anal stenosis.1

Red flag pointers for anorectal pain Weight loss Change in bowel habits

Fever >38°C Recurrent (consider Crohn disease) Exquisitely painful PR (consider abscess)

Treatment The aim is to disrupt the cycle of anal sphincter spasm, allowing improved blood flow to assist healing. Management is conservative: patients should avoid hard stools, and use warm salt (sitz) baths after bowel movements to relax the internal anal sphincter. A high-residue diet and avoidance of constipation (aim for soft bulky stools) may lead to resolution and long-term prevention. A combined local anaesthetic and corticosteroid ointment applied to the fissure, particularly before passing a stool, can provide relief but may not promote healing. A conservative treatment is the application of diluted glyceryl trinitrate ointment (e.g. Rectogesic 2% three times daily for 6 weeks to the lower anal canal) with a gloved finger gently inserted into the anal canal. It achieves healing rates of 50–70%, significantly more than placebo ointment.2,3 Transient headache is the main adverse effect. An alternative is 2% diltiazem cream applied twice daily for 6–8 weeks. An acute anal fissure will usually heal spontaneously or within a few weeks of treatment involving a high-fibre diet, sitz baths or laxatives.4 Lateral internal sphincterotomy is indicated in patients with a recurrent fissure and a chronic fissure with a degree of fibrosis and anal stenosis.5 This surgical procedure is the gold-standard surgical procedure. An alternative ‘chemical’ sphincterotomy, which is as effective as surgical treatment, is injection of botulinum toxin into the sphincter.

Proctalgia fugax (levator ani spasm) Clinical features Episodic fleeting rectal pain Varies from mild discomfort to severe spasm Last 3–30 minutes Often wakes the person from sound sleep Can occur any time of day A functional bowel disorder of unknown aetiology Affects adults, being more common in women

Management6

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Explanation and reassurance re self-healing An immediate drink (preferably hot) and local warmth with firm flannel pressure to the perineum Salbutamol inhaler (2 puffs statim) worth a trial but anecdotal evidence only Alternatives include glyceryl trinitrate spray for the symptoms or possibly antispasmodics, calcium-channel blockers and clonidine.

Solitary rectal ulcer syndrome These ulcers occur in young adults; they can present with pain but usually present as the sensation of a rectal lump causing obstructed defecation and bleeding with mucus. The ulcer, which is usually seen on sigmoidoscopy about 10 cm from the anal margin on the anterior rectal wall, can resemble cancer. Management is difficult and a chronic course is common. Treatment includes a high-residue diet and the avoidance of constipation.

Tenesmus Tenesmus is an unpleasant sensation of incomplete evacuation of the rectum. It causes attempts to defecate at frequent intervals. The most common cause is irritable bowel syndrome. Another common cause is an abnormal mass in the rectum or anal canal, such as cancer (e.g. prostate, anorectal), haemorrhoids or a hard faecal mass. In some cases, despite intensive investigation, no cause is found and it appears to be a functional problem.

Perianal haematoma A perianal haematoma (thrombosed external haemorrhoid) is a purple tender swelling at the anal margin caused by rupture of an external haemorrhoidal vein following straining at toilet or some other effort involving a Valsalva manoeuvre. The degree of pain varies from a minor discomfort to severe pain. It has been described as the ‘five-day, painful, self-curing pile’, which may lead to a skin tag. Spontaneous rupture with relief of symptoms can occur.

Management Surgical intervention is recommended, especially early in the presence of severe discomfort. The treatment depends on the time of presentation after the appearance of the haematoma. 1. Within 24 hours of onset. Perform simple aspiration without local anaesthetic using a 19 gauge needle while the haematoma is still fluid. 2. From 1 to 3 days of onset. The blood has clotted and a simple incision under local anaesthetic over the haematoma with deroofing with scissors (like taking the top off a boiled egg) to remove the thrombosis by squeezing is recommended. Removal of the haematoma reduces the chances of recurrence and the development of a skin tag, which can be a source of anal

irritation. 3. Day 4 onwards. The haematoma is best left alone unless it is very painful or (rarely) infected. Resolution is evidenced by the appearance of wrinkles in the previously stretched skin. Follow-up Review in 4 weeks for rectal examination to examine for any underlying internal haemorrhoid that may predispose to further recurrence. Prevention includes an increased intake of dietary fibre and avoidance of straining at stool.

Strangulated haemorrhoids A marked oedematous circumferential swelling will appear if all the haemorrhoids are involved. If only one haemorrhoid is strangulated, proctoscopy will help to distinguish it from a perianal haematoma. Initial treatment is with rest and ice packs and then haemorrhoidectomy at the earliest possible time. It is best to refer for urgent surgery.

Perianal cellulitis6 This occurs mainly in preschool and school-aged children. It is usually caused by Streptococcus pyogenes. Symptoms are perianal redness and pain on defecation. Check for a fissure. After swabbing, treat with oral cephalexin for 10 days.

Perianal anorectal abscess This is caused by infection by polymicrobial organisms in one of the anal glands that drain the anal canal.

Clinical features Severe, constant, throbbing pain Fever and toxicity Hot, red, tender swelling adjacent to anal margin Non-fluctuant swelling

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Careful examination is essential to make the diagnosis. Look for evidence of a fistula, Crohn disease and anorectal cancer.

Treatment Drain via a cruciate incision, which may need to be deep (with trimming of the corners) over the point of maximal induration. A drain tube can be inserted for 7–10 days. Packing is not

necessary. Antibiotics If a perianal or perirectal abscess is recalcitrant or spreading with cellulitis, use: metronidazole 400 mg (o) 12 hourly for 5–7 days plus cephalexin 500 mg (o) 6 hourly for 5–7 days7

Ischiorectal abscess An ischiorectal abscess presents as a larger, more diffuse, tender, dusky red swelling in the buttock. The presence of an abscess is usually very obvious but the precise focus is not always obvious on inspection. Antibiotics are of little help and surgical incision and drainage under deep anaesthesia is necessary as soon as possible.

Pilonidal sinus and abscess Recurrent abscesses and discharge in the sacral region (at the upper end of the natal cleft about 6 cm from the anus) can be caused by a midline pilonidal sinus, which often presents as a painful abscess. Once the infection has settled it is important to excise the pits, allow free drainage of the midline cavity and lateral tracks and remove all ingrown hair. Antibiotics, which should be guided by culture (e.g. cephalexin and metronidazole), are given to complement surgical drainage only if there is severe surrounding cellulitis.6 Pilonidal means ‘a nest of hairs’ and the problem is particularly common in hirsute young men (see FIG. 26.3 ). Refer for excision of the sinus network if necessary, possibly marsupialisation.

FIGURE 26.3 Shaving reveals a pilonidal sinus and a lateral sinus opening. It shows the characteristic tuft of hairs protruding from the midline sinus.

Fistula-in-ano5

An anal fistula is a tract that communicates between the perianal skin (visible opening) and the anal canal, usually at the level of the dentate line. It usually arises from chronic perianal infection, especially following discharge of an abscess. It is common in Crohn disease. Symptoms include: recurrent abscesses; discharge of blood, pus or serous fluid; swelling and anal pain. A surgical opinion is necessary to determine the appropriate surgical procedures, which may be complex if it traverses sphincter musculature. One method is the Seton management, whereby thin silicone, silk or latex slings are inserted under general anaesthetic. This allows drainage and then guides surgical removal of the tracts.8 Anorectal lumps Anorectal lumps are relatively common and patients are often concerned because of the fear of cancer. A lump arising from the anal canal or rectum, such as an internal haemorrhoid, tends to appear intermittently upon defecation, and reduce afterwards.1 Common prolapsing lesions include second- and third-degree haemorrhoids, hypertrophied anal papilla, polyps and rectal prolapse. Common presenting lumps include skin tags, fourth-degree piles and perianal warts (see TABLE 26.1 ). Table 26.1

Common anal lumps

Prolapsing lumps Second- and third-degree haemorrhoids Rectal prolapse Rectal polyp Hypertrophied anal papilla Persistent lumps Skin tag Perianal warts (condylomata accuminata) Anal cancer Fourth-degree haemorrhoids Perianal haematoma Perianal abscess Page 303

Skin tags The skin tag is usually the legacy of an untreated perianal haematoma. It may require excision for aesthetic reasons, for hygiene or because it is a source of pruritus ani or irritation. A tag may be associated with a chronic fissure.

Treatment (method of excision) A simple elliptical excision at the base of the skin tag is made under local anaesthetic. Suturing of the defect is usually not necessary. Perianal incisions/excisions rarely become infected.

Perianal warts It is important to distinguish the common viral warts from the condylomata lata of secondary syphilis. Local therapy includes the application of podophyllin every 2 or 3 days by the practitioner or imiquimod. Cryotherapy or diathermy are alternatives.

Rectal prolapse This is protrusion from the anus to a variable degree of the rectal mucosa (partial) or the full thickness of the rectal wall. It appears to be associated with constipation and chronic straining, leading to a lax sphincter. Features can include mucus discharge, bleeding, tenesmus, a solitary rectal ulcer and faecal incontinence (75%). Visualisation of the prolapse is an important part of the diagnosis. Surgery such as rectopexy (fixing the rectum to the sacrum) is the only effective treatment for a complete prolapse.5 Temporary shrinking of a visible prolapse in an emergency situation can be achieved by a liberal sprinkling of fine crystalline sugar.

Internal haemorrhoids Haemorrhoids or piles are common and tend to develop between the ages of 20 and 50 years. In developed nations, roughly one in two adults has had a haemorrhoid by the age of 50.2 Internal haemorrhoids are a complex of dilated arteries, branches of the superior haemorrhoidal artery and veins of the internal haemorrhoidal venous plexus (see FIG. 26.4 ). The commonest cause is chronic constipation related to a lack of dietary fibre and inappropriate bowel habit.

FIGURE 26.4 Classification of haemorrhoids Anatomically there are three classical sites, namely 3, 7 and 11 o’clock (see FIG. 26.5

).

FIGURE 26.5 Three sites of primary haemorrhoids, looking into the anus from

below

Clinical stages and pathology2 Stage 1: First-degree internal haemorrhoids: three bulges form above the dentate line. Bright bleeding is common. Stage 2: Second-degree internal haemorrhoids: the bulges increase in size and slide downwards so that the person is aware of lumps when straining at stool, but they disappear upon relaxing. Bleeding is a feature. Stage 3: Third-degree internal haemorrhoids: the pile continues to enlarge and slide downwards, requiring manual replacement to alleviate discomfort. Bleeding is also a feature. Stage 4: Fourth-degree internal haemorrhoids: prolapse has occurred and replacement of the prolapsed pile into the anal canal is impossible.

Symptoms Bleeding is the main and, in many people, the only symptom. The word ‘haemorrhoid’ means flow of blood. Other symptoms include prolapse, mucoid discharge, irritation/itching, tenesmus, incomplete bowel evacuation and pain (see FIG. 26.6 ). Page 304

FIGURE 26.6 Severely prolapsed haemorrhoids requiring surgery

Treatment Invasive treatment of haemorrhoids is based on three main procedures: rubber band ligation,

cryotherapy and sphincterotomy. Injection is now not so favoured, while a meta-analysis concluded that rubber band ligation was the most effective non-surgical therapy.9 Surgery is generally reserved for large strangulated piles. The best treatment, however, is prevention; softish bulky faeces that pass easily prevent haemorrhoids. People should be advised to have an adequate intake of non-caffeinated fluids and a diet with enough fibre by eating plenty of fresh fruit, vegetables, wholegrain cereals or bran. They should respond to the urge to defecate and avoid straining at stool, complete their bowel action within a few minutes and avoid using laxatives.

Anal discharge Anal discharge refers to the involuntary escape of fluid from or near the anus. The causes may be considered as follows.5 1. Continent Anal fistula Pilonidal sinus STIs: anal warts, gonococcal ulcers, genital herpes Solitary rectal ulcer syndrome Cancer of anal margin 2. Incontinent Minor incontinence—weakness of internal sphincter Severe incontinence—weakness of levator ani and puborectalis 3. Partially continent Faecal impaction Rectal prolapse

Anal (faecal) incontinence An Australian survey suggested 1 in 9 adults suffer some degree of faecal incontinence, which is a common reason for institutionalisation of the elderly.10 Patients may be reluctant to seek medical advice and doctors often do not ask specifically about the condition. The problem is as common in men as in women. Apart from ageing, risk factors include perianal injury, such as childbirth injury, anal surgery,

irritable bowel syndrome and neurological disorders. If there are symptoms of anal incontinence postnatally, early referral to a physiotherapist, continence nurse adviser or colorectal surgeon is advisable.11 Among the various treatments there are surgical possibilities, which vary from direct sphincter repair, directed injections such as collagen and silicone into the anal sphincter, and an artificial anal sphincter (e.g. Acticon Neosphincter). A colostomy may be the last resort. It is worth keeping in mind asking patients about the possibility of this problem and knowing ‘to whom to refer’.

Rectal bleeding Patients present with any degree of bleeding from a smear on the toilet tissue to severe haemorrhage. Various causes are presented in FIGURE 26.7 . Common causes are polyps, colon and rectal cancer, ischaemic colitis, diverticular disease and haemorrhoids.

FIGURE 26.7 Various causes of rectal bleeding Local causes of bleeding include excoriated skin, anal fissure, a burst perianal haematoma and anal cancer. A characteristic pattern of bright bleeding is found with haemorrhoids. It is usually small non-prolapsing (therefore not visible) haemorrhoids that bleed. The nature of the blood (e.g. bright red, dark red or black) and the nature of the bleeding (e.g. smear, streaked on stool, mixed with stool, massive) gives an indication of the source of the bleeding (see TABLE 26.2 ). Black tarry (melaena) stool indicates bleeding from the upper gastrointestinal tract and is rare distal to the lower ileum. Those with melaena should be admitted to hospital. Page 305

Table 26.2

Presentation and causes of rectal bleeding

Bright red blood in toilet separate from faeces

Internal haemorrhoids

Bright red blood on toilet paper

Internal haemorrhoids Fissure Anal cancer Pruritus Anal warts and condylomata

Blood and mucus on underwear

Third-degree haemorrhoids Fourth-degree haemorrhoids Prolapsed rectum Mucosal prolapse Prolapsed mucosal polyp

Blood on underwear (no mucus)

Ulcerated perianal haematoma Anal cancer

Blood and mucus mixed with faeces

Colorectal cancer Proctitis Colitis, ulcerative colitis Large mucosal polyp Ischaemic colitis

Blood mixed with faeces (no mucus)

Small colorectal polyps Small colorectal cancer

Melaena (black tarry stools)

Gastrointestinal bleeding (usually upper) with long transit time to the anus

Torrential haemorrhage (rare)

Diverticular disorder Angiodysplasia

Large volumes of mucus in faeces (little blood)

Villous papilloma of rectum Villous papilloma of colon

Blood in faeces with menstruation (rare)

Rectal endometriosis

Source: Reproduced with permission from Orlay G. Office Proctology. Sydney: Australasian Medical Publishing Company, 1987: 11–52.

Frequent passage of blood and mucus indicates a rectal tumour or proctitis, whereas more proximal tumours or extensive colitis present different patterns.

Substantial haemorrhage, which is rare, can be caused by diverticular disorder, angiodysplasia or more proximal lesions such as Meckel diverticulum and even duodenal ulcers. Angiodysplasias are 5 mm collections of dilated mucosal capillaries and thick-walled submucosal veins, found usually in the ascending colon of elderly people who have no other bowel symptoms. The bleeding is persistent and recurrent. The site is identified by technetium-labelled red cell scan or colonoscopy. The history should also include an analysis of any associated symptoms such as pain, diarrhoea or constipation, presence of lumps and a sensation of urgency or unsatisfied defecation. The latter symptoms point to a rectal cause. Associated change of bowel habit suggests a diagnosis of cancer of the rectum or left colon. Bleeding from right colon cancer is often occult, presenting as anaemia. The examination includes a general assessment, anal inspection, digital rectal examination and proctosigmoidoscopy. Eighty per cent of rectal tumours are within fingertip range. Even if there is an anal lesion, proximal bleeding must be excluded in all cases by sigmoidoscopy2 and by colonoscopy if there are any bowel symptoms or no obvious anal cause or a doubt about a lesion causing the symptoms.

Red flag pointers for rectal bleeding Age >50 years, especially new bleeding Change of bowel habit Weight loss Weakness, fatigue Brisk bleeding Constipation Haemorrhoids (may be sinister) Family history of cancer

Pruritus ani Pruritus ani, which is itching of the anus, can be a distressing symptom that is worse at night, during hot weather and during exercise. It is seen typically in adult males with considerable inner drive, often at times of stress and in hot weather when sweating is excessive. In children, threadworm infestation should be suspected. It may be part of general itching, such as with a skin disorder, or localised whereby various anorectal disorders have to be excluded. Seborrhoeic

dermatitis is a particularly common underlying factor. Consider also the more uncomfortable lichen sclerosus with its ivory white sclerotic plaques, which may also be present in the genital region.

Signs The skin changes can vary from minimal signs to marked pathology that can show linear ulceration, maceration or lichenification (see FIG. 26.8 ). Superficial skin changes can be moist and macerated or dry and scaly. Full anorectal examination is necessary. Page 306

FIGURE 26.8 Lichen chronicus simplex. Lichenification from scratching with longstanding pruritus (CHAPTER 114 )

Causes and aggravating factors Psychological factors: stress and anxiety fear of cancer Generalised systemic or skin disorders: seborrhoeic dermatitis eczema

lichen sclerosus diabetes mellitus candidiasis psoriasis (look for fissures in natal cleft) antibiotic treatment worms: pinworm (threadworm) diarrhoea causing excoriation Crohn disease Local anorectal conditions: haemorrhoids, skin tags fissures/fistula faecal incontinence warts Zealous hygiene or lack of hygiene Contact dermatitis: dyed or perfumed toilet tissue, soap, powder clothing Excessive sweating (e.g. tight pantyhose in summer)

Diagnosis Urinalysis (?diabetes) Anorectal examination Scrapings and microscopy to detect organisms Stool examination for intestinal parasites

Treatment Treat the cause (if known) and break the scratch cycle.

Page 307

Avoid local anaesthetics, antiseptics. Advise aqueous cream or a soap substitute to wash anus (instead of soap). Most effective preparations (for short13 courses): methylprednisolone aceponate 0.1% in a fatty ointment; once daily until symptoms settle (up to 4 weeks) or hydrocortisone 1% cream/ointment or hydrocortisone 1% cream with clioquinol 3% or clotrimazole 1% (especially if dermatosis and Candida suspected) If an isolated area and resistant, infiltrate 0.5 mL of triamcinolone intradermally. Fractionated Xray therapy can be used if very severe. For a lichenified perianal area, use a potent corticosteroid ointment, e.g. betamethasone dipropionate 0.05% daily until clear. Patient education about anal hygiene is essential.

Practice tips for pruritus ani Most cases of uncomplicated pruritus ani resolve with simple measures, including explanation and reassurance. Avoid perfumed soaps and powders. Use bland aqueous cream or a mild soap substitute. Otherwise prescribe a corticosteroid, especially methylprednisolone aceponate 0.1%. Once symptoms are controlled, use hydrocortisone 1%.13 Lifestyle stress and anxiety underlie most cases. In obese people with intertrigo and excessive sweating, strap the buttocks apart with adhesive tape. Consider perianal lichen simplex and lichen sclerosus in those presenting with ‘a sore bottom’.

Patient education resources

Hand-out sheets from Murtagh’s Patient Education 8th edition: Anal fissure Haemorrhoids Pruritus ani

References 1

Gold D. Benign anal conditions: how to treat. Australian Doctor, 20 January 2012: 19–25.

2

Lund JN, Scholefield JH. A randomised, prospective, double-blind, placebo-controlled trial of glyceryl trinitrate ointment in treatment of anal fissure. Lancet, 1997; Jan 4: 11–13.

3

Nelson R. Nonsurgical therapy for anal fissure. Cochrane Database Syst Rev. 2006, Issue 4: Art No. CD003431.

4

Utzig MJ, Kroesen AJ, Buhr HJ. Conservative treatment of anal fissure. Am J Gastroenterol, 2003; 98: 968–74.

5

Schnitzler M. Benign perianal conditions. Update. Medical Observer, 23 March 2007: 31– 4.

6

Perianal disorders [published 2016]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2016. www.tg.org.au, accessed September 2019.

7

Antibiotics [published 2019]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2019. www.tg.org.au, accessed September 2019.

8

Subhas G et al. Setons in the treatment of anal fistula: review of variations in materials and techniques. Dig Surg, 2012; 29(4): 292–300.

9

MacRae HM, McLeod RS. Comparison of haemorrhoidal treatments: a meta-analysis. Can J Surg, 1997; 40(1): 14–7.

10

Kalantar JS, Howell S, Talley NJ. Prevalence of faecal incontinence and associated risk factors: an underdiagnosed problem in the Australian community? Med J Aust, 2002; 176: 54–7.

11

Rieger N. Faecal incontinence: how to treat. Australian Doctor, 15 February 2008: 21–6.

12

Orlay G. Office Proctology. Sydney: Australasian Medical Publishing Company, 1987: 11–52.

13

Pruritus ani [published 2015]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2015. www.tg.org.au, accessed September 2017.

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27 Thoracic back pain

The maladies that afflict the clerks aforesaid arise from three causes; first constant sitting, secondly the incessant movement of the hand and always in the same direction, and thirdly the strain on the midline from the effort not to disfigure the books by error or cause loss to their employers. THE PHYSICIAN RAMAZZINI 1713 Thoracic (dorsal) or upper back pain, which is defined as pain localised between the neck and above the costal margin, is common in people of all ages. It accounts for 10–15% of all spinal pain and has a 1-year prevalence in 20% of adults. Dysfunction of the joints of the thoracic spine, with its unique costovertebral joints (which are an important source of back pain), is commonly encountered in medical practice, especially in people whose lifestyle creates stresses and strains through poor posture and heavy lifting. It is also referred to as non-specific thoracic spinal pain. Muscular and ligamentous strains may be common, but they rarely come to light in practice because they are self-limiting and not severe. This dysfunction can cause referred pain to various parts of the chest wall and can mimic the symptoms of various visceral diseases, such as angina, biliary colic and oesophageal spasm. In similar fashion, heart and gall bladder pain can mimic spinal pain.

Key facts and checkpoints The commonest site of pain in the spine is the costovertebral articulations, especially the costotransverse articulation (see FIG. 27.1 ). Pain of thoracic spinal origin may be referred anywhere to the chest wall, but the commonest sites are the scapular region, the paravertebral region 2–5 cm from midline and, anteriorly, over the costochondral region. Thoracic (also known as dorsal) pain is more common in patients with abnormalities such as kyphosis and Scheuermann disease. Trauma to the chest wall (including falls on the chest such as those experienced in body contact sport) commonly lead to disorders of the thoracic spine.

Unlike the lumbar spine, the joints are quite superficial and it is relatively easy to find the affected (painful) segment. Intervertebral disc prolapse is very uncommon in the thoracic spine. The older person presenting with chest pain should be regarded as having a cardiac cause until proved otherwise. If the chest pain is non-cardiac, then the possibility of referral from the thoracic spine should be considered. The thoracic spine is the commonest site in the vertebral column for metastatic disease. Scheuermann disease, which affects the lower thoracic spine in adolescents, is often associated with kyphosis and recurrent thoracic back pain. Always inspect the thoracic spine of the younger patient for kyphosis and scoliosis, ideally at 9 years of age. Palpation is the most important component of the physical examination.

FIGURE 27.1 The functional unit of the thoracic spine

A diagnostic approach A summary of the diagnostic strategy model is presented in TABLE 27.1

. Page 309

Table 27.1

Thoracic back pain: diagnostic strategy model

Probability diagnosis Musculoligamentous strains (mainly postural) Vertebral dysfunction (non-specific back pain) Serious disorders not to be missed

Cardiovascular: acute coronary syndromes, esp. myocardial infarction dissecting aneurysm pulmonary infarction epidural haematoma (blood-thinning agents) Neoplasia: myeloma lung (with infiltration) metastatic disease Severe infections: epidural abscess pleurisy infectious endocarditis osteomyelitis Pneumothorax Osteoporosis Pitfalls (often missed) Angina Gastrointestinal disorders oesophageal dysfunction peptic ulcer (penetrating) hepatobiliary pancreatic Herpes zoster Spondyloarthropathies Costochondritis: Tietze syndrome Fibromyalgia syndrome Polymyalgia rheumatica Notalgia paraesthetica Chronic infection: tuberculosis brucellosis Seven masquerades checklist Depression Diabetic radiculopathy Spinal dysfunction

Is the patient trying to tell me something? Yes, quite possible with many cases of back pain.

Probability diagnosis The commonest cause of thoracic back pain is musculoskeletal, due usually to musculoligamentous strains caused by poor posture. However, these pains are usually transitory and present rarely to the practitioner. The problems that commonly present are those caused by dysfunction of the lower cervical and thoracic spinal joints, especially those of the mid-thoracic (interscapular) area. Arthritic conditions of the thoracic spine are not overly common although degenerative osteoarthritis is encountered at times; the inflammatory spondyloarthropathies are uncommon. The various systemic infectious diseases such as influenza and Epstein–Barr mononucleosis can certainly cause diffuse backache but should be assessed in context.

Serious disorders not to be missed A special problem with the thoracic spine is its relationship with the many thoracic and upper abdominal structures that can refer pain to the back. These structures are listed in TABLE 27.2 but, in particular, myocardial infarction and dissecting aneurysm must be considered. A complex problem described by neurosurgeons is the presentation of severe sudden thoracic back pain caused by an epidural haematoma related to aspirin or warfarin therapy. Visceral disease causing a rupture or leakage should be kept in mind. Table 27.2

Non-musculoskeletal causes of thoracic back pain

Heart

Myocardial infarction Angina Pericarditis

Great vessels, lungs

Dissecting aneurysm Pulmonary embolism (rare) Pulmonary infarction Pneumothorax Pneumonia/pleurisy

Oesophagus

Oesophageal rupture Oesophageal spasm Oesophagitis

Oesophageal cancer Subdiaphragmatic disorders of:

Gall bladder Stomach Duodenum Pancreas Subphrenic collection

Miscellaneous infections

Herpes zoster Bornholm disease Infective endocarditis

Psychogenic

Cardiopulmonary problems The acute onset of pain can have sinister implications in the thoracic spine where various lifethreatening cardiopulmonary and vascular events have to be kept in mind. The pulmonary causes of acute pain include spontaneous pneumothorax, pleurisy and pulmonary infarction. Thoracic back pain may be associated with infective endocarditis due to embolic phenomena. The ubiquitous myocardial infarction or acute coronary occlusion may, uncommonly, cause interscapular back pain, while the very painful dissecting or ruptured aortic aneurysm may cause back pain with hypotension. Page 310

Osteoporosis Osteoporotic pathological fracture, especially in people over 60 years, including both men and women, must always be considered in acute thoracic pain. The association with pain following inappropriate physical therapy such as spinal manipulation should also be considered.

Acute infections Infective conditions that can involve the spine include osteomyelitis, tuberculosis, brucellosis, syphilis and Salmonella infections. Such conditions should be suspected in young people (osteomyelitis), farm workers (brucellosis) and migrants from South-East Asia and developing nations (tuberculosis). The presence of poor general health and fever necessitates investigations for these infections.

Neoplasia Fortunately, tumours of the spine are uncommon. Nevertheless, they occur frequently enough for the full-time practitioner to very occasionally encounter metastatic disease. The three common primary malignancies that metastasise to the spine are those originating in the lung, breast and the prostate (all paired structures). The less common primaries are the thyroid,

kidney, adrenals and malignant melanoma. Reticuloses such as Hodgkin lymphoma can involve the spine. Primary malignancies that develop in the vertebrae include multiple myeloma and sarcoma. Benign tumours to consider are often neurological in origin. The osteoid osteoma is aggravated by consuming alcohol and relieved by aspirin. The tumours of the spine are summarised in TABLE 27.3 Table 27.3

Of bone

Spinal

.

Significant tumours affecting the thoracic and lumbar spine Benign Osteoid osteoma Haemangioma Osteoblastoma Aneurysmal bone cyst Eosinophilic granuloma

Malignant Primary: multiple myeloma lymphomas (e.g. Hodgkin) sarcoma

Extradural: lipoma neuroma fibroma Intradural: neuroma ependymoma chordoma meningioma

Secondary: breast lung prostate adrenals/kidney thyroid melanoma Direct spread: stomach large bowel pancreas uterus/cervix/ovary

Source: Reproduced with permission from Kenna C, Murtagh J. Back Pain and Spinal Manipulation (2nd edn). Oxford: Butterworth-Heinemann, 1997: 165–74.

The symptoms and signs that should alert the clinician to malignant disease are: back pain occurring in an older person unrelenting back pain, unrelieved by rest (this includes night pain) rapidly increasing back pain

constitutional symptoms (e.g. unexplained weight loss, fever, malaise) a history of treatment for cancer (e.g. excision of skin melanoma)

Red flag pointers for thoracic back pain2 The red flag pointers are similar to those for low back pain (see CHAPTER 28 especially with regards to trauma, malignancy and suppurative infection.

),

FBE, ESR, CRP and a plain X-ray of the thoracic spine should be the initial screening test in the presence of these pointers. Page 311 A trap for the thoracic spine is lung cancer, such as mesothelioma, which can invade parietal pleura or structures adjacent to the vertebral column.

Pitfalls Pitfalls include ischaemic heart disease presenting with interscapular pain, herpes zoster at the pre-eruption stage and the various gastrointestinal disorders. Two commonly misdiagnosed problems are a penetrating duodenal ulcer presenting with lower thoracic pain and oesophageal spasm, which can cause thoracic back pain. Inflammatory rheumatological problems are not common in the thoracic spine but occasionally a spondyloarthropathy such as ankylosing spondylitis manifests here, although it follows some time after the onset of sacroiliitis.

Seven masquerades checklist Spinal dysfunction is the outstanding cause in this checklist. Depression always warrants consideration in any pain syndrome, especially back pain. It can certainly cause exaggeration of pre-existing pain from vertebral dysfunction or some other chronic problem.

Psychogenic considerations Psychogenic or non-organic causes of back pain can present a complex dilemma in diagnosis and management. The causes may be apparent from the incongruous behaviour and personality of the patient, but often the diagnosis is reached by a process of exclusion. There is obviously some functional overlay in everyone with acute or chronic pain, hence the importance of appropriate reassurance to these patients that their problem invariably subsides with time and that they do not have cancer.

Anatomical and clinical features

The functional unit of the thoracic spine is illustrated in FIGURE 27.1 . Although there is scant literature and evidence about the origins of pain in the thoracic spine,3 the strongest evidence indicates that pain from the thoracic spine originates mainly from the apophyseal joints and rib articulations. Any one thoracic vertebra has 10 separate articulations, so the potential for dysfunction and the difficulty in clinically pinpointing the precise joint at a particular level are apparent. The costovertebral joints are synovial joints unique to the thoracic spine and have two articulations—costotransverse and costocentral. Together with the apophyseal joints, they are capable of presenting with well-localised pain close to the midline or as referred pain, often quite distal to the spine, with the major symptoms not appearing to have any relationship to the thoracic spine. Generalised referral patterns are presented in FIGURE 15.2 dermatome pattern is outlined in FIGURE 27.2 .

(see CHAPTER 15

), while the

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FIGURE 27.2 Dermatomes for the thoracic nerve roots, indicating possible referral areas Source: Reproduced with permission from C Kenna and J Murtagh. Back Pain and Spinal Manipulation (2nd edn), Oxford: Butterworth-Heinemann, 1997

The pain pattern acts as a guide only because there is considerable dermatomal overlap within the individual and variation from one person to another. It has been demonstrated that up to five

nerve roots may contribute to the innervation of any one point in the anterior segments of the trunk dermatomes, a fact emphasised by the clinical distribution of herpes zoster.

Upper thoracic pain2 Dysfunction of the joints of the upper thoracic spine usually gives rise to localised pain and stiffness posteriorly but also can cause distal symptoms, probably via the autonomic nervous system. A specific syndrome called the T4 syndrome4 has been shown to cause vague pain and paraesthesia in the upper limbs and diffuse, vague head and posterior neck pain. Examination may reveal hypomobility of the upper thoracic segments. It can respond to spinal manipulation, which restores mobility. However, most of the pain, stiffness and discomfort arise from dysfunction of the upper and middle thoracic segments with a presentation of ‘pain between my shoulder blades’.

Costovertebral joint dysfunction2 The unique feature of the thoracic spine is the costovertebral joint. Dysfunction of this joint commonly causes localised pain approximately 3–4 cm from the midline where the rib articulates with the transverse process and the vertebral body. In addition, it is frequently responsible for referred pain ranging from the midline, posterior to the lateral chest wall, and even anterior chest pain. When the symptoms radiate laterally, the diagnosis is confirmed only when movement of the rib provokes pain at the costovertebral joint. This examination will simultaneously reproduce the referred pain. Confusion arises for the clinician when the patient’s history focuses on the anterior chest pain and fails to mention the presence of posterior pain, should it be present.

The clinical approach History The history of a person presenting with thoracic back pain should include a routine pain analysis, which usually provides important clues for the diagnosis. The age, sex and occupation are relevant. Pain in the thoracic area is very common in people who sit bent over for long periods, especially working at desks. Students and office workers are therefore at risk, as are breastfeeding mothers, who have to lift their babies. Spines that are kyphotic or scoliotic, or have a ‘hunchback’ secondary to disease such as tuberculosis and poliomyelitis, are prone to recurrent thoracic pain.

Older people are more likely to present with a neoplasm or osteoporosis. Osteoporosis is usually a trap because it is symptomless until the occurrence of a compression fracture. Symptoms following such a fracture can persist for 3 months. Pain that is present day and night indicates a sinister cause. Features of the history that give an indication that the pain is arising from dysfunction of the thoracic spine include: Aggravation and relief of pain on trunk rotation. The pain may be increased by rotating (twisting) towards the side of the pain but eased by rotating in the opposite direction. Aggravation of pain by coughing, sneezing or deep inspiration. This can produce a sharp catching pain which, if severe, tends to implicate the costovertebral joint. Care must be taken to rule out pneumonia and pleurisy. Relief of pain by firm pressure. Patients may state that their back pain is eased by firm pressure such as leaning against the corner of a wall. It is very important to consider myocardial ischaemia during history-taking.

Key questions Can you recall injuring your back? (Lifting something heavy or a fall onto your chest or back?) Is the pain present during the night? Do you have low back pain or neck pain? Does the pain come on after walking or any strenuous effort? Does the pain come on after eating or soon after going to bed at night? Have you noticed a fever or sweating at any time, especially at night? Have you noticed a rash near where you have the pain? What drugs are you taking? Do you take drugs for arthritis or pain? Cortisone? What happens when you take a deep breath, cough or sneeze?

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Examination The examination of the thoracic spine is straightforward with the emphasis on palpation of the spine—central and laterally. This achieves the basic objective of reproducing the patient’s symptoms and finding the level of pain. The ‘LOOK, FEEL, MOVE, (consider) X-RAY’ clinical approach is most appropriate for the thoracic spine.

Inspection Careful inspection, especially of posture, is important since it may be possible to observe at a glance why the person has thoracic pain. Note the symmetry, any scars, skin creases and deformities, ‘flat spots’ in the spine, the nature of the scapulae or evidence of muscle spasm. Look for kyphosis and scoliosis. Kyphosis may be generalised, with the back having a smooth uniform contour, or localised where it is due to a collapsed vertebra, such as occurs in an older person with osteoporosis. Generalised kyphosis is common in the elderly, especially those with degenerative spinal disease. In the young it may reflect the important Scheuermann disease. The younger person in particular should be screened for scoliosis (see FIG. 27.3 ), which becomes more prominent on forward flexion (see FIG. 27.5 ). Look for any asymmetry of the chest wall, inequality of the scapulae and differences in the levels of the shoulders. A useful sign of scoliosis is unequal shoulder levels and apparent ‘winging’ of scapula. When viewed anteriorly a difference in the levels of the nipples indicates the presence of scoliosis, or other problems causing one shoulder to drop. Inspection should therefore take place with posterior, lateral (side) and anterior views. For acute pain, check the skin for evidence of herpes zoster (rash or scars).

FIGURE 27.3 Adolescent idiopathic scoliosis: typical configuration of the trunk and thoracic spine

Palpation1 The best position is to have the patient prone on the examination table with the thoracic spine preferably in slight flexion, if the table head can be lowered. Test passive extension of each joint with firm pressure from the pad of the thumbs or the bony hand (either the pisiform prominence or the lateral border of the fifth metacarpal). Spring up and down with a few firm oscillations, keeping the elbows straight, but being well above the patient. Ask if the pressure reproduces the pain. Apart from asking ‘Is that the pain?’, note: the distribution of pain and its change with movement the range of movement

the type of resistance in the joint any muscle spasm Palpation must follow a set plan in order to reproduce the patient’s pain. The sequence is as follows: 1. central—over spinous processes 2. unilateral—over apophyseal joints (2–3 cm from midline) 3. transverse—on side of spinous processes 4. unilateral—costotransverse junctions (4–5 cm from midline) 5. unilateral—over ribs (spring over posterior rib curve with ulnar border of hand, along axis of rib)

Movements There are four main movements of the thoracic spine to assess, the most important of which is rotation, as this is the movement that so frequently reproduces the pain where it is facet joint or costovertebral in origin. The movements of the thoracic spine and their normal ranges are: 1. 2. 3. 4.

Extension Lateral flexion L and R Flexion Rotation L and R

30° 30° 90° 60°

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Ask the patient to sit on the table with hands placed behind the neck and then perform the movements. Check these four active movements, noting any hypomobility, the range of movement, reproduction of symptoms and function and muscle spasm.

Neurological examination This includes sensory testing for altered sensation in a dermatomal distribution.

Investigations The plain X-ray and MRI scan is of no utility in patients with non-specific back pain but has a place in the presence of red flags, where the MRI is most appropriate to investigate suspected serious pathology. A plain X-ray may exclude basic bony abnormalities and diseases, such as

osteoporosis and malignancy. However, bear in mind that the majority of spinal X-rays and MRIs have abnormalities if you look hard enough, increasing steadily with age. Studies of lumbar and cervical MRIs in healthy volunteers find disc bulges in 70–90%.5 CT scanning has a minimal role in the evaluation of thoracic spinal pain. Other investigations to consider are: FBE and ESR/CRP serum alkaline phosphatase serum electrophoresis for multiple myeloma Bence–Jones protein analysis Brucella agglutination test blood culture for pyogenic infection and bacterial endocarditis tuberculosis studies HLA-B27 antigen for spondyloarthropathies ECG or ECG stress tests (suspected angina) gastroscopy or barium studies (peptic ulcer) MRI scanning if myelopathy is suspected radionuclide bone scan if neoplastic or metabolic disease is suspected

Thoracic back pain in children The most common cause of thoracic back pain in children is ‘postural backache’, also known as ‘TV backache’, which is usually found in adolescent schoolgirls and is a diagnosis of exclusion. Important, although rare, problems in children include infections (tuberculosis, discitis and osteomyelitis) and tumours such as osteoid osteoma and malignant osteogenic sarcoma. Dysfunction of the joints of the thoracic spine in children and particularly in adolescents is very common and often related to trauma such as a heavy fall in sporting activities or falling from a height (e.g. off a horse). Fractures, of course, have to be excluded. Inflammatory disorders to consider are juvenile ankylosing spondylitis and spinal osteochondrosis (Scheuermann disease), which may affect adolescent males in the lower thoracic spine (around T9) and thoracolumbar spine. The latter condition may be asymptomatic, but can be associated with back pain, especially as the person grows older. It is the commonest cause of

kyphosis.

Kyphosis6 Kyphosis is the normal curve of the thoracic spine when viewed from the side. The normal range is 20–45° (see FIG. 27.4 ). An excessive angle (>45–50°) occurs with a kyphotic deformity. In children, a congenital cause is likely (present from infancy); in adolescents it is usually due to Scheuermann disease or is postural; in adults consider ankylosing spondylitis—and osteoporosis in the elderly. Tuberculosis of the spine can cause a gross deformity. Children with significant kyphosis should be referred for consideration of an intervention: exercises, bracing or surgery.

FIGURE 27.4 Illustration of kyphosis, which is measured by the angle between the uppermost and lowermost inclined vertebrae on the lateral X-ray

Scheuermann disease

This is a structural saggital plane deformity with a dominant autosomal inheritance pattern affecting the T7, 8, 9 or T11, 12 areas. Page 315

Clinical features Age 11–17 years Males > females Lower thoracic spine Thoracic pain or asymptomatic Increasing thoracic kyphosis over 1–2 months Wedging of the vertebrae Pain in the wedge, especially on bending (only 20% present with pain) Tight hamstrings, cannot touch toes Diagnosis confirmed by X-ray (lateral standing)—shows Schmorl node and anterior vertebral body wedging

Treatment Explanation and support Extension exercises, postural correction and avoidance of sports involving lifting and bending have minimal evidence but are often suggested7 Consider bracing or surgery if serious deformity

Adolescent idiopathic scoliosis A degree of scoliosis is detectable in 5% of the adolescent population.8 The vast majority of curves, occurring equally in boys and girls, are mild and of no consequence. Eighty-five per cent of significant curves in adolescent scoliosis occur in girls.8 Inheritance is a factor. The highest incidence is in first-degree female relatives (12%). The scoliotic deformity develops at around 10 years of age. Such curves appear during the peripubertal period, usually coinciding with the growth spurt. A screening test is to note the contour of the back on forward flexion (see FIG. 27.5 ).

FIGURE 27.5 Screening for adolescent idiopathic scoliosis: testing asymmetry by forward flexion. Viewed from behind the subject.

The test The subject stands with the feet parallel and together, and bends forward as far as possible with outstretched hands, palms facing each other, pointed between the great toes.

Investigation A single erect PA spinal X-ray is sufficient;9 the Cobb angle (see FIG. 27.6 measurement yardstick.

) is the usual

FIGURE 27.6 Scoliosis: the Cobb angle method of curve measurement

Management Aims To preserve good appearance—level shoulders and no trunk shift Prevent increasing curve in adult life: less than 45° Not to produce a straight spine on X-ray Methods Braces:

Milwaukee brace (rarely used) high-density polyethylene underarm orthosis to be worn for 20–22 hours each day until skeletal maturity is reached. Surgical correction: depends on curve and skeletal maturity

Guidelines for treatment Still growing: 20°

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Thoracic back pain in adults Elderly patients: thoracic back pain due to mechanical causes is not such a feature in the elderly, although vertebral dysfunction still occurs quite regularly. However, when the older person presents with thoracic pain, a very careful search for organic disease is necessary. Special problems to consider are: malignant disease (e.g. multiple myeloma, lung, prostate) osteoporosis vertebral pathological fractures polymyalgia rheumatica Paget disease (may be asymptomatic) herpes zoster

visceral disorders: ischaemic heart disease, penetrating peptic ulcer, oesophageal disorders, biliary disorders

Dysfunction of the thoracic spine Also referred to as non-specific thoracic back pain, this is the outstanding cause of pain in adulthood presenting to the practitioner and is relatively easy to diagnose. It is often referred to as the thoracic hypomobility syndrome with the disorder arising in the facet joints, costovertebral joints and thoracic musculoligamentous structure, singularly or in combination. The most efficacious treatment for painful dysfunctional problems varies according to practitioners with a special interest in this area. There is a paucity of studies and evidence supporting the multiplicity of therapies, especially focal injections and physical therapy. Many claim and bear testimony that appropriate skilled mobilisation and manipulation therapy provides effective short-term, sometimes immediate, relief.10

Typical profile:1 Age

Any age, especially between 20 and 40 years

History of injury

Sometimes slow or sudden onset

Site and radiation

Spinal and paraspinal—interscapular, arms, lateral chest, anterior chest, substernal, iliac crest

Type of pain

Dull, aching, occasionally sharp; severity related to activity, site and posture

Aggravation

Deep inspiration, postural movement of thorax, slumping or bending, walking upstairs, activities (e.g. lifting children, making beds), beds too hard or soft, sleeping or sitting for long periods

Association

Chronic poor posture

Diagnosis confirmation

Examination of spine, therapeutic response to manipulation

Management First-line management Explanation with printed information Reassurance, including spontaneous recovery likely Continued activity according to pain level Back education program

Analgesics, if required (e.g. paracetamol 1 g (o) qid or 1.33 g (o) 8 hourly), is first line Posture education and specific mobilising exercise program, esp. extension and rotation exercises to overcome stiffness Physical therapy: a short course of spinal mobilisation and manipulation (if appropriate) to help with pain and mobility Spinal mobilisation and manipulation The evidence for manipulative therapies relieving back pain is generally disappointing; two Cochrane systematic reviews found that it is probably no better than placebo for acute pain, and very little better for chronic pain.11,12 However, individual practitioners and patients seem to find immediate pain relief from spinal mobilisation or the more forceful manipulative therapy (used with extreme care, especially with osteoporosis). Many techniques can be employed, the choice depending on which part of the back is affected.9

Thoracic disc protrusion Fortunately, a disc protrusion in the thoracic spine is uncommon. This reduced incidence is related to the firm splintage action of the ribcage. Most disc protrusions occur below T9, with the commonest site, as expected, being T11–12. The common presentation is back pain and radicular pain that follows the appropriate dermatome so disc protrusion should be considered in those with neurological signs at thoracic levels. This may include a flaccid area of the lower abdominal musculature. However, disc lesions in the thoracic spine are prone to produce spinal cord compression, manifesting as sensory loss, bladder incontinence and signs of upper motor neurone lesion. The disc is relatively inaccessible to surgical intervention, but may be reached via the transthoracic lateral approach. Page 317

Syrinx A syrinx usually comes to notice as a radiological finding in the presence of thoracic back pain when it may in fact be asymptomatic. It is a rare, fluid-filled neurological cavity within the spinal cord. It is usually a congenital anomaly, but a neoplasm needs to be excluded. An MRI of the spinal cord defines the problem. Any symptoms usually appear between adolescence and age 50, due to a central cord syndrome (CHAPTER 51 ). A syrinx usually begins at the cervical level and extends down. Treat conservatively, but refer to a specialist, who may consider surgical intervention if it is symptomatic.

Muscle injury

Muscular injuries such as tearing are uncommon in the chest wall. The strong paravertebral muscles do not appear to be a cause of chest pain, but strains of intercostal muscles, the serratus anterior and the musculotendinous origins of the abdominal muscles can cause pain. Injuries to these muscles can be provoked by attacks of violent sneezing or coughing, or overstrain, e.g. lifting a heavy suitcase down from an overhead luggage rack.

Scapulothoracic joint disorders13 The gliding plane between the scapula and thoracic wall permits a considerable range of scapular movement, which contributes significantly to movement of the shoulder. Several muscles, including the rhomboids, serratus anterior and levator scapulae, help stabilise scapular movement and may be a source of pain in the scapular region.

Snapping scapula The person complains of a loud cracking or snapping sound upon abduction of the scapula. There is often associated crepitus. Pain is felt along the medial scapular border. Some people develop a habit (‘tic’) of neurotically clicking the shoulder back and forth. On examination, there is usually generalised hypermobility of the scapula, abnormal movement and tenderness to palpation along the medial edge on full abduction. The cause (uncommon) may be an underlying bony abnormality such as a bony spur on the superior border of the scapula or an osteoma. X-rays should include a lateral view of the scapula to search for this possibility.

Treatment Explanation and reassurance (if X-rays normal)—otherwise resect any bone abnormality. Avoid repeated scapular movement and ‘trick’ movements. Appropriate exercises under physiotherapy supervision. Infiltrate any very tender area in the muscle (with care) with local anaesthetic and steroid. Deep massage to the tender focus.

Scapulocostal syndrome This condition causes localised pain and tenderness, often severe, along the upper part of the medial scapular border, with radiation around the chest wall and shoulder girdle to the neck. Pain is usually worse with prolonged shoulder use towards the end of the day. It is commonly seen in typists, gymnasts and other sportspeople. It is related to poor posture. The cause may include friction between the scapula and the thoracic wall, scoliosis, trauma and myofascial strain due to poor posture.

Treatment Avoid the movements producing the pain. Posture and re-education exercises and scapula stretching. Deep friction massage. Local injections of local anaesthetic and corticosteroid into the tender area.

Winging of the scapula The asymmetry may not be apparent until the patient tries to contract the serratus anterior against resistance by pushing the outstretched arm against a wall. There may be parascapular discomfort. The common cause is neurogenic paralysis of the serratus anterior muscle. Paralysis may result from injury to the long thoracic nerve (from C5, 6, 7 nerve roots) such as a neck injury or a direct blow to the suprascapular area and from injury to the brachial plexus such as excessive carrying of heavy packs, severe traction on the arm or forceful cervical manipulation. Most cases settle spontaneously, although it may take 1–2 years.

Fibromyalgia, fibrositis and myofascial trigger points Fibromyalgia is relatively uncommon, but when encountered it presents an enormous management problem. It is not to be confused with so-called fibrositis or tender trigger points. Referral to a specialist with expertise in this condition or to a multidisciplinary pain clinic for the definitive diagnosis is recommended. Page 318 Fibrositis is not a diagnosis but a symptom, indicating a localised area of tenderness or pain in the soft tissues, especially of the upper thoracic spine. It is probably almost always secondary to upper thoracic or lower cervical spinal dysfunction.

Myofascial trigger points As described by Travell and Rinzler in 1952,14 a trigger point is characterised by local tenderness in a muscle that twitches upon stimulation and causes referred pain when subjected to pressure. However, under blinded conditions there is little consistency in the science behind reliably identifying those points.15 Regardless, local injection is relatively easy and safe, and individuals may experience temporary pain relief.

Treatment16 Identify the maximal point of pain and inject 5–8 mL of local anaesthetic (e.g. lignocaine/lidocaine 1%. Do not use corticosteroids) into the painful point (see FIG. 27.7 Post-injection massage or exercises should be performed.

).

FIGURE 27.7 Injection for myofascial trigger points

Fibromyalgia syndrome17 AKA chronic diffuse non-inflammatory pain. Its pathophysiology is poorly understood.

Clinical features The main diagnostic features are:18 1. a history of widespread pain (neck to low back) affecting all four body quadrants

2. fatigue, sleep problems, cognitive disturbance 3. pain in 11 of 18 tender points on digital palpation (the original definition in 1990) 4. pain for at least 3 months These points must be painful, not tender. Smythe and Moldofsky have recommended 14 of these points on a map as a guide for management17 (see FIG. 27.8 ). No consistent measurable investigations have been identified. If ESR/CRP are elevated, look for alternative diagnoses.

FIGURE 27.8 Fibromyalgia syndrome: typical tender points (the tender point map represents the 14 points recommended for use as a standard for diagnostic or therapeutic studies)

Other features Female to male ratio = 4:1 Usual age onset 29–37 years: diagnosis 44–53 years Positive family history Psychological disorders (e.g. anxiety, depression, tension headache, irritable digestive system) Page 319

This disorder is difficult to treat, and the GP needs to coordinate care and be responsive to individual needs as they evolve over time.18 Management requires considerable explanation, support (counselling) and reassurance. Best evidence to date supports the value of educational programs and regular aerobic exercise.18,19,20 Treat pain (simple analgesics—paracetamol) and depression (psychologist referral, antidepressants) on their merits. Consider referral to a specialist or fibromyalgia clinic. Referral to allied health practitioners can be very useful. Note: NSAIDs and narcotics are of no proven benefit. Medication (often disappointing but worth a trial) Antidepressants (of short-term value, where relevant);21 start low then monthly increments as tolerated: amitriptyline 10–50 mg (o) nocte or dothiepin 25–75 mg (o) nocte or duloxetine 30 mg (o) mane, increasing to 60 mg over 2 weeks22 Note: NSAIDs and narcotics are of no proven benefit.

Diffuse idiopathic skeletal hyperostosis (DISH) DISH is ligamentous ossification of the spine leading to progressive stiffness and possible pain in some parts. Possible associated metabolic disorder, e.g. diabetes. Ankylosing spondylitis is a differential diagnosis. There is no specific treatment. Manage as for spinal dysfunction.

Serious pitfalls The following points regarding serious vertebral organic disease are worth repeating in more

detail.

Metastatic disease23 Secondary deposits in the thoracolumbar spine may be the first presenting symptoms of malignant disease. Any patient of any age presenting with progressive severe nocturnal back pain should be regarded as having a tumour and investigated with a technetium bone scan as part of the primary investigations. Secondary deposits in the spine can lead to rapid onset paralysis due to spinal cord infarction. Many such metastases can be controlled in the early stages with radiotherapy.

Multiple myeloma Osteoporotic vertebral body collapse should be diagnosed only when multiple myeloma has been excluded. Investigations should include an ESR, Bence–Jones protein analysis and immunoglobulin electrophoresis. Early treatment of multiple myeloma can hold this disease in remission for many years and prevent crippling vertebral fractures (see CHAPTER 17 ).

Infective discitis, vertebral osteomyelitis and epidural/subdural abscess Severe back pain in an unwell patient with fluctuating temperature (fever) should be considered as infective until proved otherwise. Investigations should include blood cultures, serial X-rays and nuclear bone scanning. Biphasic bone scans using technetium with either indium or gallium scanning for white cell collections usually clinch this diagnosis. Strict bed rest with high-dose antibiotic therapy is usually curative. If left untreated, vertebral end plate and disc space collapse is common and extremely disabling. Consider tuberculosis osteomyelitis in people at risk. Suspect an epidural abscess in the presence of persistent and increased back pain. Percuss the spine for localised tenderness (see CHAPTER 20 ).

When to refer Persistent pain or dysfunction—refer to a physiotherapist or exercise specialist. Evidence or suspicion of a sinister cause (e.g. neoplasia, infective discitis/osteomyelitis in a child). Suspicion of cardiac or gastrointestinal referred (persistent) pain. Significant adolescent scoliosis or kyphosis (e.g. Scheuermann disease).

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Exercises for your thoracic spine Fibromyalgia Scoliosis

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Practice tips Feelings of anaesthesia or paraesthesia associated with thoracic spinal dysfunction are rare. Thoracic back pain is frequently associated with cervical lesions. Upper thoracic pain and stiffness is common after ‘whiplash’. The T4 syndrome of upper to mid-thoracic pain with radiation (and associated paraesthesia) to the arms is well documented. Symptoms due to a fractured vertebra usually last 3 months and to a fractured rib 6 weeks. The pain of myocardial ischaemia, from either angina or myocardial infarction, can cause referred pain to the interscapular region of the thoracic spine. Beware of the old trap of herpes zoster in the thoracic spine, especially in the older person. Consider multiple myeloma as a cause of an osteoporotic collapsed vertebra. Examine movements with the patient sitting on the couch and hands clasped behind the neck. Spinal disease of special significance in the thoracic spine includes osteoporosis and neoplasia, while disc lesions, inflammatory diseases and degenerative diseases (spondylosis) are encountered less frequently than with the cervical and lumbar spines. It is imperative to differentiate between spinal and cardiac causes of chest pain: either cause is likely to mimic the other. A working rule is to consider the cause as cardiac until the examination and investigations establish the true cause. Always X-ray the thoracic spine following substantial trauma, especially after

motor vehicle accidents, as wedge compression fractures (typically between T4 and T8) are often overlooked. Plain X-ray, CT scan or MRI are of no utility in patients with non-specific thoracic back pain. MRI is the most appropriate imaging to investigate suspected serious pathology.

References 1

Kenna C, Murtagh J. Back Pain and Spinal Manipulation (2nd edn). Oxford: ButterworthHeinemann, 1997: 165–74.

2

Australian Acute Musculoskeletal Pain Guidelines Group. Evidence-Based Management of Acute Musculoskeletal Pain—A Guide for Clinicians. Bowen Hills, QLD: Australian Academic Press, 2004.

3

Johansson MS et al. Incidence and prognosis of mid-back pain in the general population: a systemic review. Eur J Pain, 2017; 21(1): 20–8.

4

McGuckin N. The T4 syndrome. In: Grieve GD, ed. Modern Manual Therapy of the Vertebral Column. London: Churchill Livingstone, 1986: 370–6.

5

Nakashima H et al. Abnormal findings on magnetic resonance images of the cervical spines in 1211 asymptomatic subjects. Spine, 2015 (March); 40(6): 392–8.

6

Sponseller P. The 5-minute Orthopaedic Consult. Philadelphia: Lippincott, Williams and Wilkins, 2001: 184–5.

7

Bezalel T et al. Scheuermann’s disease: current diagnosis and treatment approach. J Back Musculoskelet Rehabil, 2014; 27(4): 383–90.

8

Stephens J. Idiopathic adolescent scoliosis. Aust Fam Physician, 1984; 13: 180–4.

9

Anonymous. The Easter Seal Guide to Children’s Orthopaedics. Toronto: The Easter Seal Society, 1982: 64–7.

10

Schiller L. Effectiveness of spinal manipulation therapy in the treatment of mechanical thoracic back pain. J Manip Physiol Ther, 2001; 24: 394–401.

11

Rubinstein SM et al. Spinal manipulative therapy for chronic low‐back pain. Cochrane Database of Syst Rev, 2011; Issue 2.

12

Rubinstein SM et al. Spinal manipulative therapy for acute low‐back pain. Cochrane Database of Syst Rev, 2012; Issue 9.

13

Corrigan B, Maitland G. Practical Orthopaedic Medicine. Sydney: Butterworths, 1986: 384–5.

14

Travell J, Rinzler SH. The myofascial genesis of pain. Postgrad Med, 1952; 11: 425–34.

15

Quintner J et al. A critical evaluation of the trigger point phenomenon. Rheumatology, March 2015; 54(3): 392–9.

16

Simons D. Understanding effective treatments of myofascial trigger points. J Bodyw Mov Ther, 2002; 6: 81–5.

17

Smythe HA, Moldofsky H. Two contributions to understanding of the ‘fibrositis’ syndrome. Bull Rheum Dis, 1977; 28: 928–31.

18

Guymer E, Littlejohn G. Fibromyalgia. Australian Family Physician, Oct 2013; 42(10): 690–4.

19

Clauw DJ. Fibromyalgia: a clinical review. JAMA, 2014; 311 (15): 1547–55.

20

Theadom A et al. Mind and body therapy for fibromyalgia. Cochrane Database Syst Rev, 2015; Issue 4: Art No. CD001980.

21

Fibromyalgia [published 2017]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2017. www.tg.org.au, accessed January 2020.

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Chappell AS et al. A 1-year safety and efficacy study of duloxetine in patients with fibromyalgia. Clin J Pain, 2009; 25(5): 365–75.

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Young D, Murtagh J. Pitfalls in orthopaedics. Aust Fam Physician, 1989; 18: 653–4.

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28 Low back pain

Last Wednesday night while carrying a bucket of water from the well, Hannah Williams slipped upon the icy path and fell heavily upon her back. We fear her spine was injured for though she suffers acute pain in her legs she cannot move them. The poor wild beautiful girl is stopped in her wildness at last. FRANCIS KILVERT 1874 Low back pain accounts for at least 5% of general practice presentations. It is a massive problem worldwide. The most common cause is minor soft tissue injury, but patients with this do not usually seek medical help because the problem settles within a few days. Most back pain in patients presenting to GPs is postulated to be due to dysfunction of elements of the mobile segment, namely the facet joint, the intervertebral joint (with its disc) and the ligamentous and muscular attachments. This problem, often referred to as mechanical back pain, will be described as vertebral dysfunction—a general term that, while covering radicular and non-radicular pain, includes dysfunction of the joints of the spine, although the specific origin in most instances cannot be determined. It is therefore appropriate to refer to this as ‘non-specific back pain’.1

Key facts and checkpoints Back pain accounts for 2.6% of all presenting problems in Australian general practice.2 In the US it is the commonest cause of limitation of activity in those under the age of 45.3 Approximately 85–90% of the population will experience back pain at some stage of their lives, while 70% of the world’s population will have at least one disabling episode of low back pain in their lives.3 At least 50% of these people will recover within 2 weeks and 90% within 6 weeks, but recurrences are frequent and have been reported in 40–70% of

patients; 2–7% develop chronic pain.4 It most commonly occurs in those aged 30–60 years, the average age being 45 years.5 It is difficult to assign a specific pathoanatomical cause in acute back pain (perhaps 8–15%),6 but the most common cause is probably a minor muscle/ligament strain (often don’t present to a doctor), followed by dysfunction of the intervertebral joints of the spine (‘mechanical back pain’) and spondylosis (synonymous with osteoarthritis and degenerative back disease). L5 and S1 nerve root lesions represent most of the cases of sciatica presenting in general practice. They tend to present separately but can occur together with a massive disc protrusion. An intervertebral disc prolapse is causative in only 6–8% of cases of back pain,3 and only a small fraction of those require urgent diagnosis and surgical treatment.

Causes of low back pain To develop a comprehensive diagnostic approach, the practitioner should have a clear understanding of the possible causes of low back and leg pain (see FIG. 28.1 ) and of the relative frequency of their clinical presentations. Page 322

FIGURE 28.1 Relevant causes of back pain with associated buttock and leg pain

Anatomical and pathophysiological concepts Studies have focused on the importance of disruption of the intervertebral disc in the cause of back pain. A very plausible theory has been advanced by Maigne5 who proposes the existence, in the involved mobile segment, of a minor intervertebral derangement (MID). He defines it as ‘isolated pain in one intervertebral segment, of a mild character, and due to minor mechanical cause’. The MID always involves one of the two apophyseal joints in the mobile segment, thus initiating

nociceptive activity in the posterior primary dermatome and myotome. Maigne points out that the functional ability of the mobile segment depends intimately upon the condition of the intervertebral disc. Thus, if the disc is injured, other elements of the segment will be affected.

FIGURE 28.2 Reflex activity from a MID in the intervertebral motion segment. Apart from the local effect caused by the disruption of the disc (A), interference can occur in the facet joint (B) and interspinous ligament (C) leading possibly to muscle spasm (D) and skin changes (E) via the posterior rami. Source: Reproduced with permission from C Kenna and J Murtagh. Back Pain and Spinal Manipulation. Sydney: Butterworths, 1989

In theory, any structure with a nociceptive nerve supply may be a source of pain. Such structures include the ligaments, fascia and muscles of the lumbosacral spine, intervertebral joints, facet joints, dura mater and sacroiliac joints.7 Actually, pain can theoretically arise from any innervated structure in the region of the spine. It can be neurogenic, spondylogenic, viscerogenic, vasculogenic or rarely psychogenic. Page 323

A diagnostic approach

A summary of the diagnostic strategy model is presented in TABLE 28.1 Table 28.1

Low back pain: diagnostic strategy model

Probability diagnosis Vertebral dysfunction (non-specific low back pain) Musculoligamentous strain/sprain Spondylosis (degenerative OA) Serious disorders not to be missed Cardiovascular: ruptured aortic aneurysm retroperitoneal haemorrhage (anticoagulants) Neoplasia: myeloma carcinoma of pancreas metastases Severe infections: vertebral osteomyelitis epidural abscess septic discitis tuberculosis pelvic abscess/PID Osteoporotic compression fracture/other fracture Cauda equina compression Pitfalls (often missed) Spondyloarthropathies: ankylosing spondylitis reactive arthritis psoriasis bowel inflammation Sacroiliac dysfunction Spondylolisthesis Claudication: vascular neurogenic/spinal canal stenosis Paget disease

.

Prostatitis Endometriosis Seven masquerades checklist Depression Spinal dysfunction UTI Is the patient trying to tell me something? Quite likely. Consider lifestyle, stress, work problems, malingering, conversion reaction. Note: Associated buttock and leg pain included.

Probability diagnosis7 The commonest cause of low back pain is vertebral dysfunction or mechanical pain, which then has to be further analysed. The term can embrace musculoskeletal strain, discogenic and posterior ligament pain, and facetogenic dysfunction/pain. Degenerative changes in the lumbar spine (lumbar spondylosis) are commonly found in the older age group. This problem, and one of its complications, spinal canal stenosis, is steadily increasing along with the ageing population.

Serious disorders not to be missed It is important to consider malignant disease, especially in an older person. It is also essential to consider infection such as acute osteomyelitis and tuberculosis, which is often encountered in recent immigrants, especially those from Asia and central Africa. The uncommon epidural or subdural abscess should also be kept in mind, especially if any fever (see CHAPTER 20 ). These conditions are considered in more detail under infections of the central nervous system. For pain or anaesthesia of sudden onset, especially when accompanied by neurological changes in the legs, consider cauda equina compression due to a massive disc prolapse and also retroperitoneal haemorrhage. It is important to ask patients if they are taking anticoagulants. See TABLE 28.2 . Table 28.2

‘Red flag’ pointers to serious low back pain conditions8

Age >50 years or 37.8°C Constant pain—day and night esp. severe night pain Unexplained weight loss

Symptoms in other systems, e.g. cough, breast mass Significant trauma; sometimes mild trauma Features of spondyloarthropathy, e.g. peripheral arthritis (e.g. age 12 weeks): beneficial—back exercises, multidisciplinary treatment program possible benefit—weight loss, analgesics, NSAIDs, trigger point injections, spinal mobilisation/manipulation Page 332

Radiculopathy Radicular pain, caused by nerve root compression from a disc protrusion (most common cause), tumour or narrowed intervertebral foramina typically produces pain in the leg related to the dermatome and myotome innervated by that nerve root. Leg pain may occur alone without back pain and vary considerably in intensity. The two nerve roots that account for most of these problems are L5 and S1, and the commonest disc lesion is L4–5, closely followed by L5–S1. A disc can be confined, extruded or sequestrated. Most settle with time (6–12 weeks). The management is outlined at the end of this chapter and under ‘Sciatica’ (see CHAPTER 55 ).

Spondylolisthesis About 5% of the population have spondylolisthesis but not all are symptomatic. The pain is caused by extreme stretching of the interspinous ligaments or of the nerve roots. The onset of back pain in many of these people is due to concurrent disc degeneration rather than a mechanical problem. The pain is typically aggravated by prolonged standing, walking and exercise. The physical examination is quite diagnostic. Physical examination (significant): stiff waddling gait, increased lumbar lordosis, flexed knee stance, tender prominent spinous process of ‘slipped’ vertebrae, limited flexion, hamstring tightness or spasm Diagnosis confirmation: lateral X-ray (standing) (see FIG. 28.8

)

FIGURE 28.8 Spondylolisthesis: illustrating a forward shift of one vertebra on another

Management This instability problem can be alleviated with relief of symptoms by getting patients to follow a strict flexion exercise program for at least 3 months. The objective is for patients to ‘splint’ their own spine by strengthening the abdominal and spinal muscles. Extension of the spine should be avoided, especially hyperextension. Gravity traction might help. Recourse to lumbar corsets or surgery (for spinal fusion) should be resisted, although it is appropriate in a few severe intractable cases.

Lumbar spondylosis Lumbar spondylosis, also known as degenerative osteoarthritis or osteoarthrosis, is a common problem of wear and tear that may follow vertebral dysfunction, especially after severe disc disruption and degeneration. Stiffness of the low back is the main feature of lumbar spondylosis. Although most people live with and cope with the problem, progressive deterioration can occur, leading to subluxation of the facet joints. Subsequent narrowing of the spinal and intervertebral foramen leads to spinal canal stenosis (see FIG. 28.9 ).

FIGURE 28.9 Lumbar spondylosis with degeneration of the disc and facet joint, leading to narrowing of the spinal canal and intervertebral foramen (spinal canal stenosis) Page 333

Management

Basic analgesics (depending on patient response and tolerance) NSAIDs (judicious use) Appropriate balance between light activity and rest Exercise program and hydrotherapy (if available)—physiotherapy supervision Regular mobilisation therapy may help Consider trials of electrotherapy such as TENS Consider decompressive surgery for spinal canal stenosis (see CHAPTER 55

)

The spondyloarthritides The seronegative spondyloarthropathies are a group of disorders characterised by involvement of the sacroiliac joints with an ascending spondylitis and extraspinal manifestations, such as oligoarthritis and enthesopathies (see FIG. 28.10 ; refer to CHAPTER 25 ). The pain and stiffness that are the characteristic findings of spinal involvement are typical of inflammatory disease: namely, worse in the morning, may occur at night and improves rather than worsens with exercise.

FIGURE 28.10 (a) Ankylosing spondylitis and psoriasis: main target areas on vertebral column and girdle joints and ribs, (b) Crohn disease and ulcerative colitis: main target areas of enteropathies. Reactive arthritis targets the lumbar spine and sacroiliac joints only. The main disorders in this group are ankylosing spondylitis, psoriatic arthritis, reactive arthritis and the inflammatory bowel diseases. Hence the importance of searching for a history of psoriasis, diarrhoea, urethral discharge, eye disorders and episodes of arthritis in other joints.

Treatment The earlier the treatment, the better the outlook; the prognosis is usually good (see earlier in chapter). Refer to consultant for shared care. The basic objectives of treatment are:

prevention of spinal fusion in a poor position relief of pain and stiffness maintenance of optimum spinal mobility

Malignant disease It is important to identify malignant disease and other space-occupying lesions as early as possible because of the prognosis and the effect of a delayed diagnosis on treatment. With respect to the neurological features, more than one nerve root may be involved and major neurological signs may be present without severe root pain. The neurological signs will be progressive. Page 334 If malignant disease is proved and myeloma is excluded, a search should be made for the six main primary malignancies that metastasise to the spine (see FIG. 28.11 ). If the bone is sclerotic, consider prostatic secondaries, some breast secondaries or Paget disease.

FIGURE 28.11 Important primary malignancies metastasising to the spine. Note the difference between sclerotic and osteoporotic metastases; multiple myeloma also causes osteoporotic lesions.

Non-organic back pain Like headache, back pain is a symptom of an underlying functional, organic or psychological

disorder. Preoccupation with organic causation of symptoms may lead to serious errors in the assessment of back pain. Any vulnerable aching area of the body is subject to aggravation by emotional factors. Depressed people are generally less demonstrative than those with extreme anxiety and conversion disorders and malingerers, so it is easier to mistakenly overlook the non-organic basis for their problem. Where relevant, a trial of antidepressants for a minimum of 3 weeks can be reasonable, and quite often a positive response with relief of backache eventuates. Failure to consider psychological factors in the assessment of low back pain may lead to serious errors in diagnosis and management. Each instance of back pain poses a stimulating exercise in differential diagnosis. A comparison of organic and non-organic features is presented in TABLE 28.5 .

Table 28.5

Comparison of general clinical features of organic and nonorganic based low back pain9

Presentation

Organic disorders Appropriate

Pain

Localised

Bilateral/diffuse Sacrococcygeal

Pain radiation

Appropriate Buttock, specific sites

Inappropriate Front of leg/whole leg

Time pattern

Pain-free times

Constant, acute or chronic

Paraesthesia/anaesthesia

Dermatomal Points with finger

May be whole leg Shows with hands

Response to treatment

Variable Delayed benefit

Patient often refuses treatment Initial improvement (often dramatic) then deterioration (usually within 24 hours)

Symptoms

Signs

Non-organic disorders Often dramatic

Observation

Appropriate Guarded

Overreactive under scrutiny Inconsistent

Tenderness

Localised to appropriate level

Often inappropriate level Withdraws from probing finger

Spatial tenderness (Magnuson)

Consistent

Inconsistent

Active movements

Specific movements affected

Often all movements affected

Axial loading test

No back pain (usually)

Back pain

SLR ‘distraction’ test

Consistent

Inconsistent

Sensation

Dermatomal

Non-anatomical ‘sock’ or ‘stocking’

Motor

Appropriate myotome

Muscle groups (e.g. leg ‘collapses’)

Reflexes

Appropriate May be depressed

Brisk hyperactive

Page 335

Assessment of the pain demands a full understanding of the patient. One must be aware of his or her type of work, recreation, successes and failures; and one must relate this information to the degree of incapacity attributed to the back pain. Patients with psychogenic back pain, especially the very anxious, tend to overemphasise their problem. They are usually demonstrative, the hands being used to point out various painful areas almost without prompting. There is diffuse tenderness even to the slightest touch and the physical disability is out of proportion to the alleged symptoms. The pain distribution is often atypical of any dermatome and the reflexes are almost always hyperactive. It must be remembered that patients with psychogenic back pain—for example, depression and conversion disorders—do certainly experience back pain and they do not fall for the traps set for the malingerer. A validated risk stratification tool such as the STarT Back screening tool (SBST) can be used to quantify psychosocial risk for levels of pain or disability as low, medium or high in order to guide prognosis and treatment.10

Treatment options for back pain

General aspects of management1,8 The aim of treatment is to reduce pain, maintain function, and minimise disability and work absenteeism and importantly the risk of chronicity. Advice to stay active. Evidence from randomised controlled trials confirms that, in people with acute low back pain, advice to stay active speeds symptomatic recovery, reduces chronic disability and results in less time off work compared with bed rest or usual care.8 Encourage the person to stay at work or return early if possible. Keep moving despite discomfort.9 The caring knowledgeable therapist. Evidence supports the positive value of education and reassurance from a confident, supportive and knowledgeable therapist. Patient education. Appropriate educational material leads to a clear insight into the causes and aggravation of the back disorder plus coping strategies. This can be a component of cognitive behaviour therapy (if considered appropriate). Page 336 Heat. Thermography is beneficial. Heat in the form of heat bags, hot flannels and similar methods can be of benefit, especially in the first 2–4 weeks of acute low back pain. Small trials using cold applications were equivocal.12 Evidence shows that heat is more effective than placebo for pain relief. 8 Exercises. An early graduated exercise program as soon as the acute phase settles has reasonable evidence supporting it in primary care.13 All forms of exercise (extension, flexion and isometric) appear to be equally effective (see FIG. 28.12 ). Supervised swimming is an excellent activity for back disorders. Physiotherapist supervision is optimal.

FIGURE 28.12 Examples of exercises for low back pain: (a) rotation exercise, (b) flexion exercise Studies support the use of exercises for chronic back pain rather than acute pain.4

Pharmacological agents Basic analgesics14

Evidence indicates that paracetamol is ineffective for non-specific low back pain.15 However, individual patients may and do experience a benefit and, because of that and its safety profile, a trial of paracetamol may be considered if NSAIDs are contraindicated or not effective.8

NSAIDs14 NSAIDs fare somewhat better than paracetamol in systematic reviews, showing some improvements in pain and disability compared to placebo.16 They may be particularly useful where there is clinical evidence of inflammation, especially with the spondyloarthropathies, severe spondylosis and in acute radicular pain, to counter irritation on the nerve root. The various different NSAIDs (including COX-2 inhibitors) appear to have roughly equal efficacy.

Muscle relaxants Muscle relaxants (benzodiazepines, e.g. diazepam, or baclofen) are effective in the management of non‐specific low back pain.17 However, the common adverse effects (sedation, neurological, addiction) require that they be used with caution and for short periods only.

Opioids8,14 The role of opioids is limited because any pain-control benefits are outweighed by the potential risks. They are not recommended but may be considered when paracetamol and NSAIDs are not recommended or are unable to provide pain control.

Injection techniques Trigger point injection There is limited evidence (e.g. a trial injecting the most painful part of the medial iliac crest) that local anaesthetic injections may be effective for relatively isolated points using 5–8 mL of local anaesthetic.18

Chymopapain This enzyme has been advocated for the treatment of acute nuclear herniation that is still intact. The indications are similar for surgical discectomy. However, studies show that although it is more effective than placebo, it is less effective than surgical discectomy.19

Facet joint injection Corticosteroid injection under radio-image intensification is widely used in some clinics. The procedure is delicate and expertise is required. Best evidence to date does not support the use of these injections.18

Epidural injections

Injections of local anaesthetic with or without corticosteroids are sometimes used for chronic pain, especially for nerve root pain. The most recent Cochrane review indicates that corticosteroids don’t differ from placebo injections, and local anaesthetic agents have not been studied alongside placebo.18 If chosen, a reasonable option is the caudal (trans-sacral) epidural injection for persistent sciatica using 15 mL of half-strength local anaesthetic only (e.g. 0.25% bupivacaine) (see FIG. 28.13 ). Page 337

FIGURE 28.13 Caudal epidural injection: the needle should lie free in the space and be well clear of the dural sac

Physical therapy Active exercises are the best form of physical therapy (see FIG.28.12a, b

).

Passive spinal stretching at the end range is a safe, effective method (see FIG. 28.14 ). Spinal mobilisation is a gentle, repetitive, rhythmic movement within the range of movement of the joint. It is safe and modestly effective, and a variation of stretching.

FIGURE 28.14 Lumbosacral spinal stretching technique (for right-sided pain): a traditional technique. Illustration shows direction of line of force. Source: Reproduced with permission from C Kenna and J Murtagh. Back Pain and Spinal Manipulation. Sydney: Butterworths, 1989

Spinal manipulation is a high-velocity thrust at the end range of the joint. It seems to produce a faster response, but requires greater skill. Evidence is conflicting, but it may possibly be effective for uncomplicated dysfunctional low back pain (without radicular pain), especially acute pain (see FIG. 28.15 ).4,8 In chronic low back pain, regular spinal manipulation results in a slight improvement at one month that disappears by six months.20 Adverse effects are uncommon, but can be serious.

FIGURE 28.15 Lumbar spinal stretching manipulation: illustration of the specific technique for the L4–5 level with arrows indicating the direction of applied force Source: Reproduced with permission from C Kenna and J Murtagh. Back Pain and Spinal Manipulation. Sydney: Butterworths, 1989

Other treatments Because back pain is so common, so frustrating and seemingly so resistant to most treatments except the passage of time, there is a vast array of suggested therapies that are not supported by independent evidence. Few have been ‘proven’ not to work (that proof takes enormous effort) but the GP who advocates them should be aware they are relying on anecdote more than science. Where benefits are marginal or non-existent, it is important to preference safer, cheaper interventions over those that require significant time, money or risk. Hydrotherapy Traction (little or no impact)21 TENS Therapeutic ultrasound Facet joint injection Posterior nerve root (medial branch) blocks with or without denervation (by cryotherapy or radiofrequency) Percutaneous vertebroplasty (injection of bone cement into fractured vertebra of osteoporosis)

Deep friction massage (in conjunction with mobilisation and manipulation) Acupuncture (acute: no evidence, chronic: evidence of short-term relief)22 Biofeedback Gravitational methods (home therapy)

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Lumbar supports (don’t prevent pain; conflicting studies on relieving pain)23

Management guidelines for lumbosacral disorders (summary) The management of ‘mechanical’ back pain depends on the cause. Since most of the problems are mechanical and there is a tendency to natural resolution, conservative management is quite appropriate. The rule is: ‘if patients with uncomplicated back pain receive no treatment, one-third will get better within 1 week and by 3 weeks almost all the rest of the other two-thirds are better’.24,25 Practitioners should have a clear-cut management plan with a firm, precise, reassuring and conservative clinical approach. The problems can be categorised into general conditions for which the summarised treatment protocols are outlined. Acute pain = pain less than 6 weeks Subacute pain = pain 6–12 weeks Chronic pain = pain greater than 12 weeks

Acute low back pain8 The common problem of low back pain caused by facet joint dysfunction and/or limited disc disruption usually responds well to the following supportive therapy (see box). The typical patient is aged 20–55 years, is well and has no radiation of pain below the knee.25,26 Most of these patients can expect to be relatively pain-free in 14 days and can return to work earlier than that (some may not miss work and this should be encouraged).

Management of non-specific acute low back pain (summary)27 Explanation and reassurance about no evidence of serious damage or disease; cognitive behaviour therapy

Back education program Encouragement of normal daily activities, including work, and taking responsibility for own management Optional non-opioid analgesics (NSAIDs) Prescribe exercises (provided non-aggravating) Physical therapy: stretching of affected segment, consider spinal mobilisation or manipulation (if no contraindication)8,14,25 Review in about 5 days (probably best time for consideration of physical therapy) No investigation needed initially

Sciatica with or without low back pain Sciatica is a more complex and protracted problem to treat, but most cases will gradually settle within 12 weeks (refer to CHAPTER 55 ). Conservative management is usually recommended in the first 6–8 weeks.

Acute8 Explanation and reassurance Back education program Resume normal activities as soon as possible Regular non-opioid analgesics with review as the patient mobilises NSAIDs for 10–14 days, then cease and review (low-quality evidence for mild improvement, but with side effects)28 If severe pain unrelieved, add an opioid that works well for the patient, such as tapentadol SR 50 mg (o) bd as necessary, for short-term use27 Walking and swimming Weekly or 2-weekly follow-up Consider: a course of corticosteroids for very severe pain,8 e.g. prednisolone 50 mg for 5 days, then 25 mg for 5 days, gradually tapering to 3 weeks in total. or

30 mg daily mane for 3 weeks, tapering to 0 over next 2 weeks (efficacy not clearly established)

Chronic Reassurance that problem will subside (assuming no severe neurological defects) Consider epidural anaesthesia (if slow response) Explore depressive symptoms: consider amitriptyline 10–25 mg (o) nocte increasing to maximum 75–100 mg or duloxetine Note: An important controlled prospective study comparing surgical and conservative treatment in patients with sciatica over 10 years showed that there was significant relief of sciatica in the surgical group for 1–2 years but not beyond that time. At 10 years, both groups had the same outcome, including neurological deficits.29 Surgery has a limited role.

Sacroiliac dysfunction See CHAPTER 55

.

Chronic back pain The basic management of the patient with uncomplicated chronic back pain should consider the following options: Page 339 back education program and ongoing support encouragement of normal activity exercise program mindfulness-based stress reduction (evidence based) paracetamol (e.g. 500 or 665 mg (o) 8 hourly) although not tested in any RCT NSAIDs for 14 days (especially if inflammation, i.e. pain at rest—relieved by activity and poor response to non-pharmacological treatment) and review antidepressants (but only if depressed)30 trial of mobilisation or manipulation (at least three treatments)—if no contraindications19,31 (low-quality evidence) consider a multidisciplinary rehabilitation team approach or a ‘back school’ (but evidence again suggests just trivial improvement)32

General guidelines for surgical intervention for radiculopathy Absolute Bladder/bowel control disturbance; perineal sensory change Progressive motor disturbance (e.g. significant foot drop, weakness in quadriceps) Relative Severe prolonged pain or disabling pain Failure of conservative treatment with persistent pain (problem of permanent nerve damage) If all four of the following criteria are met:8 leg pain equal to or worse than back pain positive straight leg raise test no response to conservative therapy after 4–6 weeks imaging shows a lesion corresponding to symptoms

Prevention of further back pain Patients should be informed that an ongoing back care program should give them an excellent outlook. Prevention includes: education about back care, including a good layperson’s reference golden rules to live by: how to lift, sit, bend, play sport and so on an exercise program: a tailor-made program for the patient

When to refer Urgent referral Myelopathy, especially acute cauda equina compression syndrome Severe radiculopathy with progressive neurologic deficit

Spinal fractures

Other referrals Recalcitrant spinal canal stenosis Neoplasia or infection Undiagnosed back pain Paget disease Continuing pain of 3 months’ duration without a clearly definable cause

Practice tips Back pain that is related to posture, aggravated by movement and sitting, and relieved by lying down is due to vertebral dysfunction, especially a disc disruption. The pain from most disc lesions is generally relieved by rest. Plain X-rays are of limited use, especially in younger patients, and may appear normal in disc prolapse. Remember the possibility of depression as a cause of back pain; if suspected, consider a trial of antidepressants. If back pain persists, possibly worse during bed rest at night, consider malignant disease, depressive illness or other systemic diseases. Pain that is worse on standing and walking, but relieved by sitting, is probably caused by spondylolisthesis. If pain and stiffness is present on waking and lasts longer than 30 minutes upon activity, consider inflammation. Avoid using strong analgesics (especially opioids) in any chronic non-malignant pain state. Bilateral back pain is more typical of systemic diseases, while unilateral pain typifies mechanical causes. Back pain at rest and morning stiffness in a young person demand careful investigation: consider inflammation such as ankylosing spondylitis and reactive arthritis. A disc lesion of L5–S1 can involve both L5 and S1 roots. However, combined L5

and S1 root lesions should still be regarded with suspicion (e.g. consider malignancy). A large central disc protrusion can cause bladder symptoms, either incontinence or retention. Low back pain of very sudden onset with localised spasm and protective lateral deviation may indicate a facet joint syndrome. The T12–L1 and L1–2 discs are the groin pain discs. The L4–5 disc is the back pain disc. The L5–S1 disc is the leg pain disc. Severe limitation of SLR (especially to less than 30°) indicates lumbar disc prolapse. A preventive program for dysfunctional back pain based on back care awareness and exercises is helpful. Remember that most back problems resolve within a few weeks, so avoid overtreatment.

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Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Backache Exercises for your lower back Sciatica Spondylosis

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29 Bruising and bleeding

My pa is one mask of brooses both blue and green. CHARLES DICKENS (1812–1870), NICHOLAS NICKLEBY Many people present with the complaint that they bruise easily but only a minority turn out to have an underlying blood disorder. Purpura is bleeding into the skin or mucous membranes, appearing as multiple small haemorrhages that do not blanch on pressure. Smaller purpuric lesions that are 2 mm or less in diameter (pinhead size) are termed petechiae, while larger purpuric lesions are called ecchymoses (see FIG. 29.1 ).

FIGURE 29.1 Purpuric rash (petechiae and ecchymoses) Bruises are large areas of bleeding that result from subcutaneous bleeding. If bruising is abnormal and out of proportion to the offending trauma, then a disturbance of haemostasis is suggested (see FIG. 29.2 ). The three spontaneous, intrinsically linked pathways that arrest bleeding following injury are vasoconstriction, formation of a platelet plug and activation of coagulation factors.

FIGURE 29.2 Severe bleeding in a woman with diabetes and systemic fibrinolysis. Note the bleeding following insulin injections into the abdominal wall and an injection into the shoulder joint. Photo courtesy Hatem Salem

Differential diagnosis ‘Palpable purpura’ due to an underlying systemic vasculitis is an important differential problem. The petechiae are raised so finger palpation is important. The cause is an underlying vasculitis affecting small vessels (e.g. polyarteritis nodosa). The decision as to which individuals require investigation is difficult and depends on whether the haemostatic defect is due to local or systemic pathology.1 The ability to identify a bleeding disorder is important because of implications for surgery, pregnancy, medication and genetic counselling. Page 342

Key facts and checkpoints Purpura = petechiae + ecchymoses. Abnormal bleeding is basically the result of disorders of (1) the platelet, (2) the coagulation mechanism or (3) the blood vessel. There is no substitute for a good history in the assessment of bleeding disorders. The first step is an assessment of personal and family histories. When someone describes ‘bruising easily’ it is important to exclude thrombocytopenia due to bone marrow disease and clotting factor deficiencies such as haemophilia. The commonest cause of an acquired bleeding disorder is drug therapy (e.g. aspirin, NSAIDs, cytotoxics and oral anticoagulants). Bleeding secondary to platelet defects is usually spontaneous, associated with a petechial rash and occurs immediately after trauma or a cut wound.1 The bleeding is usually mucosal (e.g. bleeding from gingiva, menorrhagia, epistaxis and petechiae). Bleeding caused by coagulation factor deficiency is usually traumatic and delayed (e.g. haemorrhage occurring 24 hours after a dental extraction in haemophilia). Laboratory assessment should be guided by the clinical impression. The routine screening tests for the investigation of a true bleeding disorder can occasionally be normal, even despite a severe haemorrhagic state. Second-line investigations will need to be undertaken.

Causes of clinical disorders Bleeding disorders can result from: coagulation deficiencies (reduction or inhibition of circulatory coagulation factors) platelet abnormalities: of platelet number or function vascular defects: of vascular structure or endothelium Bleeding disorders can also be divided into impaired primary or secondary haemostasis. Primary haemostatic disorders which are the most common include von Willebrand disease (vWD), thrombocytopenia and platelet function disorders. Secondary causes include disorders of fibrin formation and the haemophilias.2 A list of differential diagnoses of systemic bleeding disorders is presented in TABLE 29.1 Table 29.1

Classification of bleeding disorders1

Vascular disorders Inherited Hereditary haemorrhagic telangiectasia Connective tissue disease, e.g. Marfan syndrome Easy bruising syndrome Acquired Senile purpura Infection, e.g. dengue, meningococcal Henoch-Schonlein purpura Corticosteroid purpura Vitamin C deficiency (scurvy) Painful bruising syndrome Platelet disorders Inherited Fanconi syndrome Glanzmann disease Acquired (immune) Idiopathic thrombocytopenic purpura Aplastic anaemia Drug induced thrombocytopenia, e.g. heparin Thrombotic thrombocytopenia purpura

.1

Post-transfusion purpura Non-immune Disseminated intravascular coagulation Myeloproliferative disorders Kidney failure/uraemia Bone marrow replacement (e.g. leukaemia) or failure Coagulation disorders Inherited Haemophilia A Haemophilia B von Willebrand disease (types 1, 2 and 3) Acquired Disseminated intravascular coagulation (DIC) Vitamin K deficiency Oral anticoagulation therapy or overdose Acquired haemophilia Liver disease Page 343

The clinical approach Differentiation of coagulation factor deficiencies and platelet disorders as the cause of a bleeding problem can usually be determined by a careful evaluation of the history and physical examination.

History Factors that suggest the presence of a systemic bleeding defect include: spontaneous haemorrhage severe or recurrent haemorrhagic episodes, e.g. epistaxis bleeding from multiple sites, e.g. mouth, bladder, bowel bleeding out of proportion to the degree of trauma cutaneous bleeding gastrointestinal bleeding

postpartum haemorrhage bleeding from tooth extraction/oral cavity menstrual history, e.g. menorrhagia muscle haematomas or haemarthrosis If a bleeding diathesis is suspected it is essential to determine whether local pathology is contributing to the blood loss (e.g. postoperative bleeding, postpartum bleeding, gastrointestinal haemorrhage).

Diagnostic tips Platelet abnormalities present as early bleeding following trauma. Coagulation factor deficiencies present with delayed bleeding after initial haemostasis is achieved by normal platelets. A normal response to previous coagulation stresses (e.g. dental extraction, circumcision or pregnancy) indicates an acquired problem. If acquired, look for evidence of MILD: Malignancy, Infection, Liver disease, Drugs. A diagnostic strategy is outlined in TABLE 29.2 Table 29.2

.

Purpura: diagnostic strategy model

Probability diagnosis Simple purpura (easy bruising syndrome) Senile purpura (common on limbs of older people after minimal trauma) Corticosteroid-induced purpura Immune thrombocytopenic purpura Henoch–Schönlein purpura Liver disease, especially alcoholic cirrhosis Increased intravascular pressure, e.g. coughing, vomiting Serious disorders not to be missed Malignant disease: leukaemia myeloma Myelodysplasia Aplastic anaemia

Myelofibrosis Severe infections: septicaemia meningococcal infection measles typhoid dengue/chikungunya HIV and other blood-borne viruses (e.g. Hepatitis C) Disseminated intravascular coagulation Thrombotic thrombocytopenic purpura Fat embolism Pitfalls (often missed) Haemophilia A, B, vWD Post-transfusion purpura Trauma (e.g. domestic violence, child abuse) Rarities: hereditary telangiectasia (Osler–Weber–Rendu syndrome) Ehlers–Danlos syndrome scurvy Fanconi syndrome Seven masquerades checklist Drugs: many examples (see Medication record Anaemia: aplastic anaemia

)

Psychogenic factors Factitial purpura

Family history A positive family history can be a positive pointer to the diagnosis: sex-linked recessive pattern: haemophilia A or B autosomal dominant pattern: vWD, dysfibrinogenaemias autosomal recessive pattern: deficiency of coagulation factors V, VII and X Enquire whether the person has noticed blood in the urine or stools and whether menorrhagia is

present in women. A checklist for a bleeding history is presented in TABLE 29.3 . The actual size and frequency of the bruises should be recorded where possible and if none are present at the time of the consultation the patient should return if any bruises reappear. Page 344 Table 29.3

Checklist for a bleeding history

Skin bruising

Tonsillectomy

Epistaxis

Other operations

Injury

Childbirth

Domestic violence

Haematuria

Menorrhagia

Rectal bleeding

Haemarthrosis

Drugs

Tooth extraction

Family history

Unusual haematomas

Comorbidities (e.g. liver disease, kidney disease)

Key questions How long has the problem been apparent to you? Do you remember any bumps or falls that might have caused the bruising? What sort of injuries cause you to bruise easily? Have you noticed bleeding from other areas such as your nose or gums? Have you noticed any rashes or blood blisters in your mouth? Has anyone in your family had a history of bruising or bleeding? What is your general health like? Do you have any tiredness, weight loss, fever or night sweats? Did you notice a viral illness or sore throat beforehand? How much alcohol do you drink? What happened in the past when you had a tooth extracted? Have you ever had painful swelling in your joints? Note: A validated bleeding assessment tool is a useful guide to the diagnosis of vWD.

Medication record It is mandatory to obtain a complete drug history. Examples of drugs and their responses are: vascular purpura: prednisolone/other steroids thrombocytopenia: cytotoxic drugs carbamazepine gold sodium valproate heparin ranitidine sulfonamides quinine, quinidine thiazide diuretics penicillins, vancomycin chloramphenicol functional platelet abnormalities: aspirin and other antiplatelet drugs NSAIDs coagulation factor deficiency: warfarin direct oral anticoagulants (e.g. dabigatran, rivaroxaban)

Examination Careful examination of the skin is important. Note the nature of the bleeding and the distribution of any rash, which is characteristic in Henoch–Schönlein purpura. Senile purpura in the elderly is usually seen over the dorsum of the hands, extensor surface of the forearms and the shins.

Purpura on the legs indicates platelet disorders, meningococcal septicaemia and paraproteinaemias; on the fingers and toes indicates vasculitis. Note the lips and oral mucosa for evidence of hereditary telangiectasia. Blood-filled vesicles of the oral mucosa (wet purpura) is a strong risk factor for intracranial haemorrhage in immune thrombocytopenic purpura and requires urgent intervention. Gum hypertrophy occurs in monocytic leukaemia. Search for evidence of malignancy, such as sternal tenderness, lymphadenopathy and hepatosplenomegaly. Examine the ocular fundi for evidence of retinal haemorrhages. Urinalysis, searching for blood (microscopic or macroscopic), is important.

Investigations The initial choice of investigations includes a full blood count, blood film and basic coagulation studies. Basic coagulation studies include prothrombin time (PT), international normalised ratio (INR) and activated partial thromboplastin time (aPTT). Further tests if coagulation defect suspected: fibrinogen level thrombin time (TT) If platelet pathology suspected: platelet count and blood film platelet function analyser (PFA-100) If inherited disorders suspected: factor VIII vW factor activity vW factor antigen The full blood examination and blood film is useful in pinpointing the aetiology. Platelet morphology gives a diagnostic guide to inherited platelet disorders. Other sophisticated tests, such as von Willebrand screening and platelet aggregation (e.g. PFA-100), can be advised by the consulting haematologist. One of considerable value is the bone marrow examination, which is useful to exclude the secondary causes of thrombocytopenia, such as leukaemia, other marrow infiltrations and aplastic anaemia. Other tests to consider: ESR/CRP, blood group, autoimmune screening, kidney Page 345 function tests, LFTs, serology for blood-borne infections, ferritin, plasma electrophoresis, skin biopsy. Be cautious of pseudo-thrombocytopenia due to laboratory error or platelet clumping—exclude on a blood film and consider a repeat collection.

A summary of appropriate tests is presented in TABLE 29.4 coagulation factor deficiencies in TABLE 29.5 . Table 29.4

and of blood changes for some

Laboratory investigation checklist for the easy bruiser

Full blood count Platelet count Prothrombin time (PT) and international normalised ratio (INR) Thrombin time (TT) Activated partial thromboplastin time (aPTT)

Table 29.5

Blood changes for specific coagulation factor disorders

Haemophilia A vWD

Vitamin K deficiency

PT

Normal

Normal

↑

aPTT

↑

↑

↑

TT

Normal

Normal

Normal

Abnormal bleeding in children Abnormal bleeding in children is not uncommon and once again the clinical history, particularly the past and family history, provides the most valuable information. It is important to keep nonaccidental injury in mind in the child presenting with ‘easy bruising’. However, it is appropriate to exclude a bleeding disorder, especially a platelet disorder. Vigorous coughing or vomiting in a child can cause petechiae on or around the eyelids. Coagulation disorders, including haemophilia and vWD, are usually suspected on clinical grounds because of widespread bruising or because of prolonged bleeding following procedures such as circumcision and tonsillectomy. A common condition is haemorrhagic disease of the newborn, which is a self-limiting disease usually presenting on the second or third day of life because of a deficiency of coagulation factors dependent on vitamin K. The routine use of prophylactic vitamin K in the newborn infant has virtually eliminated this problem. Idiopathic (immune) thrombocytopenic purpura (ITP) is the commonest of the primary platelet

disorders in children. Both acute and chronic forms have an immunological basis. The diagnosis is based on the peripheral blood film and platelet count. The platelet count is commonly below 50 000/mm3 (50 × 109/L). Spontaneous remission within 4 to 6 weeks occurs with acute ITP in childhood.3 The commonest vascular defects in childhood are: anaphylactoid (Henoch–Schönlein) purpura infective states nutritional deficiency (usually inadequate dietary vitamin C)

Henoch–Schönlein purpura4 HSP, which is a type of IgA vasculitis, is the commonest vasculitis of children. It affects the small vessels, producing a leucocytoclastic vasculitis with a classic triad of nonthrombocytopenic purpura, large joint arthritis and abdominal pain. It is diagnosed clinically by the characteristic distribution of the rash (palpable purpura) over the lower limbs, extending onto the buttocks (see FIG. 29.3 ), but it can also involve the upper limbs, trunk and even the face.

FIGURE 29.3 Henoch−Schönlein purpura in a 5-year-old boy showing the typical distribution of the rash on the lower limbs Page 346 The onset of HSP typically follows an upper respiratory tract infection including a group A streptococcal tonsillopharyngitis.

The bleeding time, coagulation time and platelet counts are normal. The prognosis is good; most recover fully in a few months.

Clinical features All ages, mainly in children 2–8 years Rash, mainly on buttocks and legs (see FIG. 29.4

)5

Rash can occur on hands, arms and trunk Arthritis (in two-thirds): mainly ankles and knees Abdominal pain—colicky (vasculitis of GIT) Haematuria (in 90%): reflects nephritis

FIGURE 29.4 Henoch–Schönlein purpura: typical distribution

Associations Kidney involvement—deposition of IgA immune complex (a serious complication) Melaena Intussusception Scrotal involvement

Investigations FBE (if abnormal platelets or white cells, consider alternative diagnosis) Urine: protein and blood; spun specimen, micro for casts

Management Largely symptomatic—analgesics No specific therapy Short course of steroids for abdominal pain (if intussusception excluded) If haematuria: follow-up urine microscopy and kidney function especially if no resolution (in approximately 5%) DxT arthralgia + purpuric rash ± abdominal pain → Henoch–Schönlein purpura

Practice tip Beware of CKD in HSP.

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Infective states The purpura associated with severe infections, such as meningococcaemia and other septicaemias, is due primarily to a severe angiitis. Disseminated intravascular coagulation usually follows.3

Vascular, platelet and coagulative disorders

The features of vascular disorders are: easy bruising and bleeding into skin ± mucous membrane bleeding investigations normal

Abnormal bleeding in older people In older adults, the outstanding causes are senile purpura and purpura due to steroids.6 The cause in both instances is atrophy of the vascular supporting tissue.

Simple purpura (easy bruising syndrome) This is a benign disorder occurring in otherwise healthy women usually in their 20s or 30s. The feature is bruising on the arms, leg and trunk with minor trauma. The woman may complain of heavy periods. However, major challenges to the haemostatic mechanism, such as dental extraction, childbirth and surgery, have not been complicated by excessive blood loss.

Factitial purpura Unexplained bruising or bleeding may represent self-inflicted abuse or abuse by others. In selfinflicted abuse, the bruising is commonly on the legs or areas within easy reach of the patient.

Platelet disorders The features are: petechiae ± ecchymoses bleeding from mucous membranes platelet counts 40 years of age Rectal bleeding/haematochezia (fresh blood) Family history of cancer Positive FOBT

Pitfalls The pitfalls can be summarised as follows: impacted faeces depressive illness purgative abuse local anal lesions drugs Those with impacted faeces often present with spurious (paradoxical) diarrhoea. This is a form of idiopathic constipation and is very commonly encountered in general practice, especially in bedridden elderly people. Anal pain or stenosis, such as fissure-in-ano, thrombosed haemorrhoids, perianal haematoma or ischiorectal abscess, lead to constipation because the person is hesitant to defecate.

General pitfalls and tips Ensure the person is truly constipated, and not having unrealistic expectations of regularity. Ensure that the anthraquinone group of laxatives, including ‘Ford pills’, is never used long term because they cause melanosis coli and associated megacolon. Page 375 Be very wary of alternating constipation and diarrhoea (e.g. colon cancer).

In a busy practice be careful not to let ‘familiarity breed contempt’ (e.g. onset of hyperparathyroidism, cancer). A normal rectal examination does not exclude cancer.

Seven masquerades checklist Three of the primary masquerades (see TABLE 31.1 ) are important causes of constipation, namely drugs, depression and hypothyroidism. Many drugs (see TABLE 31.2 ) may be associated with constipation, especially codeine and its derivatives, antidepressants, aluminium and calcium antacids. Cations that constipate include: barium, calcium, aluminium, iron, bismuth. A careful drug history is thus mandatory, because fortunately the constipation usually resolves once the drug is withdrawn. Constipation can be a significant symptom in all types of depressive illness and may be aggravated by treatment with antidepressants. Table 31.2

Drugs associated with constipation

Analgesics (inhibitors of prostaglandin synthesis) Antacids (containing calcium carbonate or aluminium hydroxide) Anticholinergic agents, antispasmodics Antidiarrhoeal agents Anti-epileptics Antihistamines (H1-receptor blockers)* Antiparkinson drugs* Antipsychotic drugs,* e.g. clozapine, risperidone Barbiturates Barium sulphate Benzodiazepines Calcium-channel blockers (verapamil) Calcium supplements Cholestyramine Clonidine Cough mixtures Cytotoxic drugs Diuretics that cause hypokalaemia Gabapentin Ganglionic blocking agents Heavy metal (especially lead) 5-HT3-receptor antagonists, e.g. ondansetron Iron supplements

Laxatives (chronic use) Monoamine oxidase inhibitors Muscle relaxants Opioid analgesics (e.g. codeine) SSRIs Tricyclic antidepressants* *Denotes anticholinergic effect.

The metabolic causes of constipation include hypothyroidism, and the rarer hypercalcaemia and porphyria. Diabetes rarely can be associated with constipation when an autonomic neuropathy can lead to alternating bouts of constipation and diarrhoea. Page 376

Psychogenic considerations Constipation may be a manifestation of an underlying functional problem and psychiatric disorder, such as depression, anorexia nervosa, schizophrenia or drug misuse. Narcotic misuse must always be considered, and laxatives may cause rebound constipation. More commonly, it may reflect an inactive lifestyle and provide a good opportunity for appropriate counselling.

The clinical approach History It is important to ask patients to define exactly what they mean by constipation. Some people believe that just as the earth rotates on its axis once a day, so should their bowels open daily to ensure good health. As always, a careful history is appropriate, including stool consistency, frequency, ease of evacuation, pain on defecation and the presence of blood or mucus. A dietary history is very relevant.

Key questions How often do you go to the toilet? What are your bowel motions like? Are they bulky and hard, like rabbit pellets, or soft? Is there pain on opening your bowels?

Have you noticed any blood? Have you noticed any lumps? Do you have any soiling on your underwear? How do you feel in yourself? What medications are you taking?

Diary Ask the patient to keep a 10-day diary recording frequency and nature of stools, and whether any difficulty was experienced when passing stool.

Examination The important aspects are abdominal palpation and rectal examination. Palpation may reveal the craggy mass of a neoplasm, faecal retention (especially in the thin patient) or a tender spastic colon. The perianal region should be examined for localised disease. The patient should be asked to bear down to demonstrate perianal descent, haemorrhoids or mucosal prolapse. Perianal sensation and the anal reflex should be tested. Digital rectal examination is mandatory, and may reveal a rectal tumour and faecal impaction, as well as testing for rectal size and tone. If there is a history from infancy, a normal or narrow rectum suggests congenital megacolon (Hirschsprung disorder) but, if dilated, acquired megacolon. General signs that may be significant in the diagnosis of constipation are summarised in FIGURE 31.2 .

FIGURE 31.2 Possible significant abdominal signs in the patient with constipation

Rectal examination The most important first step is to do the examination. Method Explain to the patient what will happen. After inspection with the patient in the left lateral position and with knees drawn up, a lubricated gloved index finger is placed over the anus. Part the buttocks. Ask the patient to concentrate on slow deep breathing. With gentle pressure the finger is then inserted slowly into the anal canal and then into the rectum (it helps patient comfort if they push down or squeeze to accommodate the finger). Page 377

Rotate the finger anteriorly to feel the prostate in males and the cervix in females.

The finger will reach to about 7–8 cm with gentle thrusting into the perineum. Examine the whole circumference of the rectum by sweeping the finger from posterior on both sides. Points to note Any pain: fissure, proctitis, excoriation from diarrhoea (a rectal examination will not be possible in the presence of a fissure) Induration from a chronic fissure or fistula in the anal canal The sphincter tone The nature of the faeces (?impaction) The rectal wall: cancer is usually indurated, elevated and ulcerated; a villous adenoma has a soft velvety feel Posteriorly: the sacrum and coccyx Laterally: the side walls of the pelvis Anteriorly: cervix and pouch of Douglas in the female; prostate and rectovesical pouch in the male Prostate examination It feels larger if the patient has a full bladder. The normal prostate is a firm smooth rubbery bilobed structure (with a central sulcus) about 3 cm in diameter. A craggy hard mass suggests cancer. An enlarged smooth mass suggests benign hypertrophy. A tender, nodular or boggy mass suggests prostatitis.

Practice tip on treatment Before resorting to a good old-fashioned ‘3H’ enema (hot water, high and a hell of a lot), use a sorbitol compound (e.g. Microlax 5 mL enema). It can be carried in the doctor’s bag, is very easy to insert and is most effective.

A common pitfall In the female, the cervix or a vaginal tampon can be mistaken for a mobile extrarectal tumour.

Endoscopy Sigmoidoscopy—in particular, flexible sigmoidoscopy with examination of the rectosigmoid—is important in excluding local disease; search for abnormalities such as blood, mucus or neoplasia. The insufflation of air sometimes reproduces the pain of the irritable bowel syndrome. It is worth noting that 60% of polyps and cancers will occur in the first 60 cm of the bowel4 and diverticular disorder should be evident with the flexible sigmoidoscope. The presence of melanosis coli is an important sign—it may give a pointer to the duration of the constipation and the consequent chronic intake (perhaps denied) of anthraquinone laxatives.

Investigations These can be summarised as follows: Haematological: haemoglobin ESR Stools for occult blood Biochemistry (where suspected): thyroid function tests serum calcium serum potassium carcinoembryonic antigen (a targeted tumour marker rather than a screen) Radiological: CT colonography (virtual colonography) double contrast barium enema (especially for primary colonic disease, e.g. megacolon) bowel transit studies, using radio-opaque shapes taken orally and checking progress by abdominal X-ray or stool collection Physiological tests:

anal manometry—test anal tone rectal sensation and compliance, using an inflatable rectal balloon dynamic proctography, to determine disorders of defecation rectal biopsy, to determine aganglionia

Idiopathic constipation It is best to classify idiopathic constipation into three subgroups: 1 simple constipation 2 slow transit constipation 3 normal transit constipation (irritable bowel syndrome) Of these, the commonest is simple constipation, which is essentially related to a faulty diet and bad habit. Avery Jones,5 who defined the disorder, originally described it as being due to one or more of the following causes: faulty diet—inadequate dietary fibre neglect of the call to stool unfavourable living and working conditions lack of exercise travel Dyschezia, or lazy bowel, is the term used to describe a rectum that has become unresponsive to faecal content, and this usually follows repeated ignoring of calls to defecate.

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Slow transit constipation occurs primarily in women with an apparently normal colon, despite a high-fibre intake and lack of the other causes described by Avery Jones. Many are young, with a history dating from early childhood or, more commonly, adolescence. Constipation may follow childbirth, uncomplicated abdominal surgery or a period of severe dieting. However, in the majority no precipitating cause is evident. A defecatory disorder is where there is a paradoxical contraction rather than normal relaxation of the anal sphincter and associated muscles responsible for evacuation. Also known as dyssynergic dysfunction.

Management

Most patients have simple constipation and require reassurance and education once an organic cause has been excluded. Encourage modification of lifestyle. Provide psychological counselling and biofeedback for dyssynergic problems. Advice to patients Adequate exercise, especially walking, is important. Develop good habits: answer the call to defecate as soon as possible. Develop the ‘after breakfast habit’. Allow time for a good relaxed breakfast and then sit on the toilet. Don’t miss meals—food stimulates motility. Avoid codeine compounds (tablets or mixture). Take plenty of fluids, especially water and fruit juices (e.g. prune juice). Eat an optimal bulk diet. Eat foods that provide bulk and roughage, such as vegetables and salads, cereals (especially wheat fibre), fresh and dried fruits, and wholemeal bread. Enough fibre should be taken to convert stools that sink into stools that float. Examples of food with good bulk properties are presented in TABLE 31.3 .6 Fruit has good fibre, especially in the skin, and some have natural laxatives (e.g. prunes, figs, rhubarb, apricots). Table 31.3

Foods with bulk-forming properties (from least to most)

Potato Banana Cauliflower Peas Cabbage Lettuce Apple Carrot Bran

Treatment (pharmaceutical preparations) Some patients may not tolerate unprocessed bran but tolerate pharmaceutical preparations better (see TABLE 31.4 ). An appropriate choice would be one of the hydrophilic bulk-forming agents such as ispaghula or psyllium. Avoid stimulant laxatives except for short sharp treatments.

Table 31.4

Therapeutic agents (laxatives) to treat constipation (with examples)

Hydrophilic bulk-forming agents Psyllium mucilloid (Agiofibe, Metamucil) Sterculia (Granocol, Normacol) Ispaghula (Agiolax, Fybogel) Methylcellulose Wheat bran/dextrin (Benefiber) Crude fibre (Fibyrax Extra) Stimulant (irritant) laxatives Sodium picosulfate Anthraquinones: senna (Senokot/Sennetabs), senna with dried fruits (Nu-Lax), sennosides A and B; cascara Frangula bark (in Normacol Plus) Castor oil Triphenylmethanes: bisacodyl (e.g. Dulcolax); picosulfate Osmotic laxatives Macrogol 3350 with electrolytes (e.g. Movicol) Magnesium sulphate (Epsom salts/Colocap Balance) Magnesium hydroxide (milk of magnesia) Lactulose (several agents) Mannitol Sodium phosphate mixture Sorbitol (Sorbilax) Saline laxatives Stool-softening/lubricating agents Liquid paraffin (Agarol) Docusate—poor evidence of efficacy Poloxamer Glycerin suppositories Sorbital/sodium compounds (Microlax) Laxatives in suppository form Glycerin/glycerol suppository

Glycerin/glycerol suppository Sorbitol sodium compounds (e.g. Fleet Enema) Sodium phosphate enema (e.g. Fleet) Stimulant microenema or suppository (e.g. Bisa-lax) Stool-softener microenema (e.g. Enamax) Prokinetic agent Prucalopride Note: ColonLYTELY used for colonoscopy preparation contains macrogol, sodium sulphate and other mineral salts.

First-line therapy7 Use a general bulking agent, e.g. psyllium or ispaghula granules 1–2 teaspoons (o) once or twice daily, or commercial products as per suggested dose. Second-line therapy Use an osmotic laxative or a fibre-based stimulant preparation, e.g. macrogol 3350 + 1–2 sachets, each dissolved in 125 mL water once daily or lactulose syrup 15–30 mL (o) daily until response, then 10–20 mL daily or dried fruits with senna leaf (Nu-Lax) 10 g nocte or docusate + senna (50–80 mg), 1–2 tabs nocte Third-line therapy (Recheck cause.) Magnesium sulphate 1–2 teaspoons (15 g) in water once or twice daily (if normal kidney function) or as capsules (Colocap Balance) 15 caps over 15 minutes

or combined bulking/stimulating agent (e.g. frangula/sterculia [Normacol plus]) or glycerin suppository (retain for 15–20 minutes) or sodium citrate or phosphate enema (e.g. Fleet Enema) or Microlax enema Page 379

Constipation in children Constipation is quite common in children and is idiopathic in 95%. The most common factor is diet. Constipation often begins after weaning or with the introduction of cow’s milk. It is rare with breastfeeding. Low fibre intake and a family history of constipation may be associated factors.8 Most children develop normal bowel control by 4 years of age (excluding any physical abnormality). It is normal to have a bowel movement every 2–3 days, providing it is of not unusual consistency and is not painful. Constipation usually appears between 2 and 4 years of age, and up to a third of primary schoolaged children will report constipation over a 12-month period. In toddlers, the gender distribution is equal, but by age 5, boys are more likely to get constipation than girls, with the frequency of faecal incontinence three times higher in boys. Consider constipation in a child who has recently recommenced bedwetting. Constipation in children is defined as having two or more of the following over the previous 2 months: 1 episode of faecal incontinence per week (previously referred to as encopresis) large stools in rectum or palpable on abdominal examination retentive posturing (e.g. ‘stiff as a board’ standing/lying, tip toes, crossed legs, braces against furniture) and withholding behaviour (e.g. refuses, hides, requests nappy, denies need to go) painful defecation Faecal incontinence, which is a consequence of chronic constipation, is the passage of stool in an inappropriate place in children who have been toilet trained. It can present as soiling (encopresis)

due to faecal retention with overflow of liquid faeces (spurious diarrhoea). Constipation is nearly always functional (>95%),7 though the GP should check for any red flags for a pathological cause (see below). The key feature in functional constipation is chronic faecal retention leading to rectal dilatation and insensitivity to the normal defecation reflex.

Red flag pointers for organic causes in children Blood in stools Perianal disease Fever Weight loss/delayed growth Delayed meconium/thin strip-like stools Vomiting Urinary symptoms (although bedwetting fairly common) Abnormal neurological findings in legs Medications used for children with behavioural/developmental issues

Normally the rectum is empty just prior to defecation; with faecal retention, the rectum is stretched, weak and numb and it can leak.

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Other important conditions Hirschsprung disorder: consider if delay in passing first meconium stool and subsequent constipation Anal fissure in infants: consider if stool hard and associated with pain or bleeding the mainstay of treatment is dietary manipulation

Principles of treatment of functional constipation7,8 Encourage relaxed child–parent interaction with toilet training, such as appropriate encouragement, ‘after breakfast habit’ training, regular toileting (where possible), three

times/day for 3–5 minutes, reinforce desired behaviour with stickers on an age-appropriate chart. Introduce psychotherapy or behaviour modification program, especially where ‘fear of the toilet’ exists. Establish an empty bowel: remove any severely impacted faeces with microenemas (e.g. Microlax), and even disimpaction under anaesthesia if necessary, particularly if faecal ‘rocks’ are visible on X-ray. Advice for parents of children over 18 months: Drink ample non-milk fluids each day—several glasses of water, unsweetened fruit juice (be cautious of cow’s milk). Use prune juice, which contains sorbitol. Get regular exercise—walking, running, outside games or sport. Provide high-fibre foods—high-fibre cereals, wholegrain bread, brown rice, wholemeal pasta, fresh fruit with skins left on where possible, dried fruits such as sultanas, apricots or prunes, fresh vegetables. Advice on correct posture and position—‘how to do a poo’:8 Feet supported (e.g. with a foot stool) Knees higher than bottom and apart Leaning forward with elbows on knees Encourage child to push out stomach Ensure privacy (including at preschool/school) Laxatives—if constipation has been brief in duration, treat for 3 months, but for chronic constipation, treat for 6 months minimum. Can use macrogol 3350 (Movicol), paraffin oil or lactulose. For acute faecal impaction, high-dose laxatives can be used until liquid stools are achieved, and then revert back to maintenance treatment. Enemas are suitable only for children with acute severe rectal pain or distress and are rarely required. Use a pharmaceutical preparation as a last resort to achieve regularity. First line6

Paraffin oil (e.g. Parachoc): RCT evidence indicates suitable and better than stimulant laxative

or osmotic laxative (e.g. lactulose): 1–3 mg/kg 1–5 years: 10 mL per day >5 years: 15 mL per day or macrogol 3350 with electrolytes: 2–12 years: 1 sachet Movicol-Half in 60 mL water once daily >12 years: 1 sachet Movicol (or 2 Movicol-Half) daily Severe constipation/faecal impaction: consider admission to hospital abdominal X-ray macrogol 3350 with electrolytes (double above doses and water) Microlax enema If unsuccessful, add ColonLYTELY via nasogastric tube or sodium phosphate enema (Fleet Enema) (not 50 years

Lifetime risk This is determined by the family history (see TABLE 31.5 Table 31.5

).

Family history and lifetime risk of colorectal cancer10

Family history

Lifetime risk

None: population risk

1:50

One first-degree relative >45 years

1:17

One first-degree relative and one second-degree relative

1:12

One first-degree relative 50 years Smoking history Asbestos exposure history Persistent cough Overseas travel

TB exposure Haemoptysis Unexplained weight loss Dyspnoea Fever

It is also important not to overlook asthma in which a nocturnal cough, without wheezing, is a feature in children.

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Pitfalls Causes that tend to be overlooked, especially in the presence of a normal X-ray, are gastrooesophageal reflux, postnasal drip and asthma. Gastro-oesophageal reflux is more common as a cause of reflex coughing, especially at night, than appreciated. However, do not use PPIs in the absence of suggestive GORD symptoms.5 Whooping cough, especially immunisation-modified, can be difficult to diagnose, particularly if the characteristic whoop is absent.

General pitfalls Attributing cough due to bronchial carcinoma in a smoker to ‘smoker’s cough’ Overlooking TB, especially in the elderly, by attributing symptoms to old age, bronchitis or smoking Overlooking the fact that bronchial carcinoma can develop in a person with other pulmonary conditions, such as chronic bronchitis Being slow to order a chest X-ray Failing to recognise that pertussis presents in adults

Seven masquerades checklist The applicable masquerade is drugs, many of which can produce a wide variety of disorders of the respiratory tract that cause a cough. Pulmonary infiltration with fibrosis may result from some cytotoxic drugs, especially bleomycin. Over 20 different drugs are known to produce an SLE-like syndrome, sometimes complicated by pulmonary infiltrates and fibrosis. Cough can be a feature of some of the ACE inhibitors and beta blockers, inhaled steroids and sulfasalazine.

Psychogenic considerations

A cough can occur for psychosocial reasons. Coughing is under cerebral control and a slight cough before commencing a speech is normal and presumably assists in clearing mucus from around the vocal cords.6 This can readily become a nervous habit or mannerism. A typical ‘psychogenic’ cough is barking in quality—the ‘Cape Barren goose’ cough. It does not occur during sleep.

The clinical approach History The nature of the cough may provide important diagnostic clues, but it is the associated symptoms, such as the nature of the sputum, breathlessness, wheezing and constitutional symptoms, that provide the most helpful diagnostic value. A diagnosis of asthma should not be made if the predominant symptom is cough without airflow limitation such as wheeze. A history of smoking habits, past and present, is essential and an occupational and hobby history requires investigation. Significant occupations (past or present) include mining (pneumoconiosis), aircraft manufacturing (asbestosis and mesothelioma), farming (‘farmer’s lung’—allergic pneumonitis from mouldy hay) and bird handling (‘bird fancier’s lung’—allergic alveolitis or psittacosis from pigeons or budgerigars). A past history of recurrent lung infections from childhood is suggestive of cystic fibrosis and bronchiectasis, a history of hay fever and eczema suggests asthma, while a family history involves asthma, cystic fibrosis, emphysema (α1-antitrypsin deficiency) and tuberculosis.

Key questions7 How would you describe the cough? How long has the cough been present? Do you cough up sputum? Describe the sputum, especially its colour. Is there any blood in the sputum? How much sputum do you produce—a teaspoon, an eggcup or more? Is there a burning sensation in your throat or chest when you cough? Have you noticed any other symptoms? What about chest pain, or fever, shivers or sweats? Do you have a wheeze? Have you had previous attacks of wheezing or hay fever?

Is there a history of asthma in your family? Have you lost weight? Has anyone in the family had TB or a persistent cough? How much do you smoke? Are you exposed to any smoke or fumes? What kind of work do you do, now and in the past? Is there a chance you have been exposed to asbestos? Do you keep birds or pets at home? Do you have any birds (e.g. pigeons) nesting outside your bedroom? Is there a possibility of a foreign body such as a peanut ‘having gone down the wrong way’? Have you had an operation recently or been confined to bed? Have you noticed any swelling of your legs? Page 388

Examination Physical examination includes a general examination with a search for features such as enlarged cervical or axillary glands, which may indicate bronchial carcinoma, as would Horner syndrome (constricted pupil, ptosis). A careful examination of the lungs and cardiovascular system is also appropriate. Fine crackles on auscultation indicate pulmonary oedema or heart failure, interstitial pulmonary fibrosis and early lobar pneumonia, while coarse crackles indicate resolving pneumonia, bronchiectasis and TB. Careful inspection of the sputum forms an important part of the physical examination of the lungs. This should include its colour and consistency, presence of particulate matter and a 24-hour sputum watch.

Investigations This applies particularly to those with haemoptysis. Possible investigations include:8 haemoglobin, blood film and white cell count sputum cytology and culture ESR (elevated with bacterial infection, bronchiectasis, TB, lung abscess and bronchial carcinoma), CRP pulmonary function tests/spirometry

radiology: plain chest X-ray (shows many problems) helical CT scan: helps more precise localisation of lesion, may show cavitation CT pulmonary angiogram bronchography: shows bronchiectasis (a very unpleasant procedure) ventilation/perfusion isotope scan: for pulmonary infarction echocardiogram (pulmonary hypertension) skin tests lung biopsy bronchoscopy (best at time of haemoptysis) However, all that is needed initially is a plain chest X-ray.

Diagnostic characteristics There are important characteristics of cough that may point to the causation. TABLE 32.1 compares typical causes of dry and productive cough. Character of the cough Brassy → tracheitis and bronchitis (major bronchi); extrinsic pressure on trachea (e.g. tumour) Barking → laryngeal disorders (e.g. laryngitis) Croupy (with stridor) → laryngeal disorders (e.g. laryngitis, croup) Hollow ‘bovine’ (no power) → vocal cord paralysis (left-recurrent laryngeal nerve) Weak cough → indicates bronchial carcinoma Paroxysmal with whoops → whooping cough Painful → tracheitis; left ventricular failure Dry chronic → GORD, drugs (e.g. ACEI) Timing Nocturnal cough →

asthma left ventricular failure postnasal drip chronic bronchitis whooping cough Waking cough → bronchiectasis, asthma chronic bronchitis GORD habitual Associations Changing posture → bronchiectasis lung abscess Meals → hiatus hernia (possible) oesophageal diverticulum tracheo-oesophageal fistula Wheezing → asthma Breathlessness → asthma left ventricular failure COPD

Sputum

A healthy, non-smoking individual produces approximately 100–150 mL of mucus a day. This normal bronchial secretion is swept up the airways towards the trachea by the mucociliary clearance mechanism and is usually swallowed. The removal from the trachea is assisted also by occasional coughing, although this is carried out almost subconsciously.6 Excess mucus is expectorated as sputum. The commonest cause of excess mucus production is cigarette smoking. Mucoid sputum is clear and white. Character of sputum Clear white (mucoid) → normal or uninfected bronchitis Yellow or green (purulent) → due to cellular material (neutrophils or eosinophil granulocytes) ± infection (not necessarily bacterial infection)

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asthma due to eosinophils bronchiectasis (copious quantities) Rusty → lobar pneumonia (S. pneumoniae): due to blood Thick and sticky → asthma Profuse, watery → alveolar cell carcinoma Thin, clear mucoid → viral infection Redcurrant jelly → bronchial carcinoma Profuse and offensive → bronchiectasis; lung abscess Thick plugs (cast-like) → allergic bronchopulmonary Aspergillus; bronchial carcinoma Pink frothy sputum → pulmonary oedema

Haemoptysis Bloodstained sputum (haemoptysis), which varies from small flecks of blood to massive bleeding, requires thorough investigation. Always consider malignancy or TB. Often the diagnosis can be made by chest X-ray. Causes are presented in TABLE 32.4 . Haemoptysis must be distinguished from blood-stained saliva caused by nasopharyngeal bleeding or sinusitis and also from haematemesis.6 Acute bronchitis produces streaky haemoptysis. Table 32.4

Haemoptysis (adults): diagnostic strategy model

Probability diagnosis Acute chest infection: URTI (24%) bronchitis Chronic bronchitis Trauma: chest contusion, prolonged coughing Cause often unknown (22%) Serious disorders not to be missed Vascular: pulmonary infarction/embolus LHF → pulmonary oedema mitral stenosis pulmonary hypertension AV malformation Infection: lobar pneumonia (rusty sputum) tuberculosis lung abscess Cancer/tumour (4%): bronchogenic carcinoma tumour of the larynx or trachea Other: blood disorders including anticoagulants cystic fibrosis Pitfalls (often missed) Foreign body Bronchiectasis (13%) Iatrogenic (e.g. endotracheal intubation) Goodpasture syndrome or other vasculitides Spurious haemoptysis (blood from nose or throat) Factitious (e.g. Munchausen syndrome) Note: Haemoptysis must be distinguished from bloodstained saliva caused by nasopharyngeal bleeding or sinusitis, also haematemesis.6 Copious haemoptysis is due to bronchiectasis or TB.

Impaired cough

Weak or ineffective cough affects ability to clear lower respiratory tract secretions, predisposing to more serious infection, particularly lower lobe pneumonia. Causes include chest wall or abdominal pain/injury, chest wall deformity, decreased cough strength and central respiratory depression.

Productive cough Chronic bronchitis: mucoid or purulent; rarely exceeds 250 mL per day6 Bronchiectasis: purulent sputum; up to 500 mL/day Asthma: mucoid or purulent; tenacious sputum Lung abscess: purulent and foul-smelling Foreign body: can follow impaction

Cough in children3 Cough in children is a very common symptom, but troublesome persistent cough is a great cause of anxiety among parents and probably the commonest symptom for which the family doctor is consulted. Age-related causes of chronic cough (present at least 4 weeks) are presented in TABLE 32.5 . Most children with chronic cough do NOT have asthma. A chest X-ray is advisable for a persistent cough. Page 390 Table 32.5

Age related causes of chronic cough in children (to consider)9

Infants Congenital/structural abnormalities, e.g. tracheao-esophageal fistula Milk inhalation/reflux Household smoke exposure Toddler/preschool Foreign body inhalation Asthma Viral induced wheeze Bronchiolitis/bronchitis Whooping cough Cystic fibrosis Croup

Older children Asthma Acute or chronic bronchitis Chronic rhinitis Smoke exposure Atypical pneumonia Adolescents Asthma Smoking/other inhalants Psychogenic

Common causes of cough generally are: asthma recurrent viral bronchitis acute URTIs allergic rhinitis croup Disorders not to be missed are: asthma cystic fibrosis inhaled foreign body tracheo-oesophageal fistula pneumonia whooping cough Several clinicians describe the catarrhal child syndrome as the commonest cause of chronic cough.3 This refers to children who develop a postnasal drip following acute respiratory infection and allergic rhinitis. Recurrent cough in children can usually be explained by recurrent viral respiratory infections which commonly occur when first exposed to other children. Their airways tend to be overactive. There is a slight predisposition to asthma. If asthma is suspected, a therapeutic trial of salbutamol 200 mcg 4 hourly via a spacer may be

worthwhile.

Psychogenic causes Habit cough can occur in children, especially those with a history of school phobia. The cough does not occur during sleep and remains unchanged with exertion or infection.

Croup (laryngotracheobronchitis) Clinical features Characteristic harsh barking inspiratory cough with stridor Prodrome of URTI for 2 days Sounds like a dog barking or a seal Children 6 months to 6 years Fever variable (rarely >39°) Usually 11 pm to 2 am Auscultation confirms inspiratory stridor Occurs in small local epidemics Management—refer to CHAPTER 89

.

Pneumonia in children Clinical features Tachypnoea, expiratory grunt Possible focal chest signs Diagnosis often only made by chest X-ray

Pathogens Viruses are the most common cause in infants. Mycoplasma is common in children over 5 years. S. pneumoniae is a cause in all age groups.

Pathogens are difficult to isolate—may need blood culture.

Treatment Depends on age and tropical vs non-tropical. Almost all those under 48 months should be admitted to hospital. Indicators for hospital admission are shown in the box. Minimal handling Careful observations including pulse oximetry Attend to hydration Antibiotics indicated in all cases, even though many are viral. Refer to Therapeutic Guidelines for various age groups, tropical regions and specific confirmed bacterial species. A simplified overview follows: Mild to moderate: amoxicillin 25 mg/kg up to 1 g orally, 8 hourly for 3 days (mild) or 5–7 days (moderate) plus (if atypical bacteria suspected) azithromycin or clarithromycin or doxycycline Severe:10 cefotaxime IV or ceftriaxone IV (if staphylococcus aureus suspected, add clindamycin or lincomycin)

Pneumonia in children: guidelines for hospitalisation11 Infants: RR > 70 Intermittent apnoea Not feeding Older children: RR > 50 Grunting Signs of dehydration

Both groups: SaO2 ≤92% Cyanosis Difficulty breathing Family/social issues

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Cough in the elderly Important causes of cough to consider in the elderly include chronic bronchitis, lung cancer, pulmonary infarct (check calves), bronchiectasis and left ventricular failure, in addition to the acute upper and lower respiratory infections to which they are prone. It is important to be surveillant for bronchial carcinoma in an older person presenting with cough, bearing in mind that the incidence rises with age. One study found the causes of chronic cough in the elderly to be postnasal drip syndrome 48%, gastro-oesophageal reflux 20% and asthma 17%.12

Common respiratory infections Respiratory infections, especially those of the upper respiratory tract, are usually regarded as trivial, but they account for an estimated one-fifth of all time lost from work and three-fifths of time lost from school, and are thus of great importance to the community.13 The majority of respiratory infections are viral in origin and antibiotics are therefore not indicated. URTIs are those involving the nasal airways to the larynx, while lower respiratory tract infections (LRTIs) affect the trachea downwards. Combined URTIs and LRTIs include influenza, measles, whooping cough and laryngotracheobronchitis.

The common cold (acute coryza) This highly infectious URTI, which is often mistakenly referred to as ‘the flu’, produces a mild systemic upset and prominent nasal symptoms (see FIG. 32.1 ).

FIGURE 32.1 The main symptoms and complications of the common cold

Clinical features 24–48 hours of weakness Malaise and tiredness Sore, runny nose Sneezing Sore throat Slight fever Other possible symptoms: headache hoarseness cough The watery nasal discharge becomes thick and purulent in about 24 hours and persists for up to a week. Secondary bacterial infection is uncommon.

Management Advice to the patient includes: rest—adequate sleep and rest, especially if weak

drink adequate fluids stop smoking (if applicable) analgesics—paracetamol (acetaminophen) or aspirin (max. 8 tablets a day in adults) steam inhalations for a blocked nose cough drops or syrup for a dry cough gargle aspirin in water or lemon juice for a sore throat (avoid aspirin in children 38°C plus at least one respiratory symptom and one systemic symptom cough (dry) sore throat coryza prostration or weakness myalgia headache rigors or chills

Complications

Tracheitis, bronchitis, bronchiolitis Secondary bacterial infection Pneumonia due to Staphylococcus aureus (mortality up to 20%)1 Toxic cardiomyopathy with sudden death (rare) Encephalomyelitis (rare) Depression (a common sequela)

Diagnosis Nasopharyingeal swabs for PCR or other rapid specific tests

Management Advice to the patient includes: rest in bed until the fever subsides and patient feels better analgesics: paracetamol and aspirin or ibuprofen are effective, especially for fever fluids: maintain high fluid intake (water and fruit juice) freshly squeezed lemon juice and honey preparation Antiviral agents5 Neuraminidase inhibitors (cover influenza A and B): zanamivir (Relenza) 10 mg by inhalation bd for 5 days oseltamivir (Tamiflu) 75 mg (o) bd (child 2 mg/kg) for 5 days Both should be commenced within 36 hours of onset and given for 5 days. Note: These antiviral agents have questionable benefit in a low-risk population, but treatment for vulnerable patients during an epidemic may be appropriate.

Prevention Influenza vaccination for influenza A and B (recommended annually) offers some protection for Page 393 up to 70% of the population for about 12 months.1 (See CHAPTER 6 .)

Coronavirus respiratory infections

Known for the past severe influenza syndromes MERS-CoV and SARS, it has emerged as COVID-19. This respiratory illness has had more worldwide impact than any other in the 21st century, in terms of both individual morbidity and mortality, and the subsequent socioeconomic effects. Prevention of the spread of this coronavirus is via public health measures (handwashing, social distancing, personal protective equipment) and a number of novel vaccines. At the time of writing there is no effective treatment specific to SARS-CoV-2, although various supportive interventions including medications can reduce the severity for hospitalised patients. Prevention through population vaccination is being addressed.

Bronchitis Acute bronchitis This is acute inflammation of the tracheobronchial tree that usually follows an upper respiratory infection. Although generally mild and self-limiting, it may be serious in debilitated patients.

Clinical features Features of acute infectious bronchitis are: cough and sputum (main symptoms) wheeze and dyspnoea usually viral infection can complicate chronic bronchitis—often due to Haemophilus influenzae and Streptococcus pneumoniae scattered wheeze on auscultation fever or haemoptysis (uncommon) Outcome It improves spontaneously in 4–8 days in healthy patients.

Treatment5 Symptomatic treatment Inhaled bronchodilators for airflow limitation Distinguish acute bronchitis from bacterial pneumonia and bacterial infective exacerbations of COPD, where antibiotics are useful

Antibiotics are not indicated for acute bronchitis

Chronic bronchitis This is a chronic productive cough for at least 3 successive months in 2 successive years: wheeze, progressive dyspnoea recurrent exacerbations with acute bronchitis occurs mainly in smokers Refer to COPD (in CHAPTER 74

).

Pneumonia17 This is inflammation of lung tissue. It usually presents as an acute illness with cough, fever and purulent sputum plus physical signs and X-ray changes of consolidation. It can be broadly classified as typical or atypical, which are caused by different bacteria, viruses or other organisms. The initial presentation of pneumonia can be misleading, especially when the patient presents with constitutional symptoms (fever, malaise and headache) rather than respiratory symptoms. A cough, although usually present, can be relatively insignificant in the total clinical picture. This diagnostic problem applies particularly to atypical pneumonia but can occur with bacterial pneumonia, especially lobar pneumonia.

Community-acquired pneumonia5,11 CAP occurs in people who are not or have not been in hospital recently, and who are not institutionalised or immunocompromised, i.e. the majority of people in general practice. The choice of antibiotic is initially empirical. CAP is usually caused by a single organism, especially Streptococcus pneumoniae, which is demonstrating increasing antibiotic resistance.10 Treatment is usually for 5–10 days for most bacterial causes, 2 weeks for Mycoplasma or Chlamydia infection and 2–3 weeks for Legionella. Viruses are often present in CAP (25–44%), whether as a sole cause or as a predecessor to bacteria.18

Typical pneumonia The commonest community-acquired infections are Streptococcus pneumoniae (majority), Haemophilus influenzae10 (mainly in COPD), M. pneumoniae (young adults) and Klebsiella pneumoniae. Clinical features

Rapidly ill with high temperature, dry cough, pleuritic pain, rigors or night sweats 1–2 days later may be rusty-coloured sputum Rapid and shallow breathing follows Examination: focal chest signs, consolidation Investigations: CXR, sputum M&C, oxygen saturation, specific tests/serology, PCR Complications: pleural effusion, empyema, lung abscess, respiratory failure A particular complication of influenza is a streptococcus pneumoniae infection 2–4 weeks Page 394 later18

The atypical pneumonias Refer to CHAPTER 19

.

Clinical features Fever without chills, malaise Headache Minimal respiratory symptoms, non-productive cough Signs of consolidation absent Chest X-ray (diffuse infiltration) worse than chest signs Causes Virus, e.g. influenza Mycoplasma pneumoniae—the commonest: adolescents and young adults treat with: roxithromycin 300 mg (o) daily or doxycycline 100 mg bd for 14 days Legionella pneumophila (legionnaire disease):

related to cooling systems in large buildings incubation 2–10 days Diagnostic criteria include: prodromal influenza-like illness a dry cough, confusion or diarrhoea very high fever (may be relative bradycardia) lymphopaenia with moderate leucocytosis hyponatraemia Patients can become prostrate with complications. Treat with: azithromycin IV (first line) or erythromycin (IV or oral) plus (if very severe) ciprofloxacin or rifampicin Chlamydia pneumoniae: similar to Mycoplasma Chlamydia psittaci (psittacosis): treat with doxycycline, roxithromycin or erythromycin Coxiella burnetti (Q fever): treat with doxycycline 200 mg (o) statim, then 100 mg daily for 14 days

Antibiotic treatment according to severity5,13 This is usually empirical ± ‘tools’ such as CORB.

Mild pneumonia This does not require hospitalisation. Amoxicillin 1 g 8 hourly for 5–7 days plus (if atypical pneumonia suspected, or suboptimal improvement in 2 days) doxycycline 100 mg bd for 5–7 days

Moderately severe pneumonia This requires hospitalisation (see Guidelines box for severe pneumonia and hospital admission). Monitor with CXR; oximeter (keep O2 saturation ≥94%). Neonates Age over 65 years Coexisting illness High temperature: >38°C Clinical features of severe pneumonia Involvement of more than one lobe Inability to tolerate oral therapy benzylpenicillin 1.2 g IV 4–6 hourly for 7 days (switch to oral amoxicillin when improved) or procaine penicillin 1.5 g IM daily (drugs of choice for S. pneumoniae) plus doxycycline or ceftriaxone 1 g IV daily for 7 days (in penicillin-allergic patient) If not so severe and oral medication tolerated, can use amoxicillin/clavulanate or cefaclor or doxycycline In tropical regions use a different regimen (refer to Therapeutic Guidelines) If atypical pneumonia, use doxycycline, erythromycin, roxithromycin or clarithromycin

Severe pneumonia The criteria for severity (with increased risk of death) are presented in the box on Guidelines for severe pneumonia and hospital admission.11,17 The CORB score indicates severity (Confusion, Hypoxia pO2100 per minute BP BC

Equal in both ears

Conduction deafness

Negative: BC > AC

Louder in the deaf ear

Very severe conduction deafness

Negative: BC > AC May hear BC only

Louder in the deaf ear

Sensorineural deafness

Positive: AC > BC

Louder in the better ear

Very severe sensorineural deafness

‘False’ negative (without masking)

Louder in the better ear

AC = air conduction; BC = bone conduction

Audiometric assessment Audiometric assessment includes the following: pure tone audiometry impedance tympanometry electric response audiometry oto-acoustic emission testing

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Pure tone audiometry4,5 Pure tone audiometry is a graph of frequency expressed in hertz versus loudness expressed in decibels. The tone is presented either through the ear canal (a test of the conduction and the cochlear function of the ear) or through the bone (a test of cochlear function). FIGURES 33.6

and 33.7

are typical examples of pure tone audiograms.

FIGURE 33.6 Pure tone audiogram for severe conductive deafness in left ear Source: Black4

FIGURE 33.7 Pure tone audiogram for unilateral (left) sensorineural deafness. Suspect a viral or congenital origin in children; check adults for acoustic neuroma. The difference between the two is a measure of conductance. If the two ears have different thresholds, a white noise masking sound is applied to the better ear to prevent it hearing sound presented to the test ear. The normal speech range occurs between 0 and 20 dB in soundproof conditions across the frequency spectrum. Tympanometry Tympanometry measures the mobility of the tympanic membrane, the dynamics of the ossicular chain and the middle-ear air cushion. The test consists of a sound applied at the external auditory meatus, otherwise sealed by the soft probe tip.

Imaging CT and gadolinium-enhanced MRI can identify retrocochlear pathology such as acoustic neuroma and cochlear nerve agenesis.

Deafness in children Deafness in childhood is relatively common and often goes unrecognised. One to two of every 1000 newborn infants suffer from sensorineural deafness.1 Congenital deafness may be due to inherited defects, to prenatal factors such as maternal intra-uterine infection or drug ingestion during pregnancy, or to perinatal factors such as birth trauma, and haemolytic disease of the newborn. Deafness may be associated with Down syndrome and Waardenburg syndrome. Waardenburg syndrome, which is dominantly inherited, is diagnosed in a person with a white forelock of hair and different coloured eyes. Acquired deafness accounts for approximately half of all childhood cases. Purulent otitis media and secretory otitis media are common causes of temporary conductive deafness. However, one in 10 children will have persistent middle-ear effusions and mild to moderate hearing loss in the 15–40 dB range.6 Permanent deafness in the first few years of life may be due to virus infections, such as mumps or meningitis, ototoxic antibiotics and several other causes.

Screening1 Screening should begin at birth so that language input can allow optimal language development. The aim of screening should be to recognise every deaf child by the age of 8 months to 1 year— before the vital time for learning speech is wasted. High-risk groups should be identified and screened; for example, a family history of deafness, maternal problems of pregnancy, perinatal problems, survivors of intensive care, very low birthweight and gestation 21 g/3 days]).

Coeliac disease2 Synonyms: coeliac sprue, gluten-sensitive enteropathy. Note: It can appear at any age; refer to coeliac disease in children (see later in chapter).

It is widely underdiagnosed because most patients present with non-GIT symptoms, such as tiredness. There is a genetic factor in this autoimmune disorder with a 1 in 10 chance if a first-degree relative is affected. Consider screening under 2 years if there is such an association. Page 418

Clinical features Classic tetrad: diarrhoea, weight loss, iron/folate deficiency, abdominal bloating Malaise, lethargy Flatulence Mouth ulceration Diarrhoea with constipation (alternating) Pale and thin patient No subcutaneous fat

Diagnosis Elevated faecal fat Characteristic duodenal biopsy: villous atrophy (key test) Total IgA level IgA transglutaminase antibodies (>90% sensitivity and specificity) Deamidated gliadin peptide (DGP-IgG) also highly sensitive and specific

Associations Iron-deficiency anaemia Malignancy, especially lymphoma, GIT Type 1 diabetes Pernicious anaemia Primary biliary cirrhosis Subfertility Dermatitis herpetiformis

IgA deficiency Autoimmune thyroid disease Osteoporosis Neurological (e.g. seizures, ataxia, peripheral neuropathy) Down syndrome

Management Diet control: high complex carbohydrate and protein, low fat, lifelong gluten-free (no wheat, barley, rye and oats) Treat specific vitamin and mineral deficiencies Give pneumococcal vaccination (increased risk of pneumococcus sepsis) Coeliac support group and Coeliac Australia Gluten-free diet Avoid foods containing gluten either as an obvious component (e.g. flour, bread, oatmeal) or as a hidden ingredient (e.g. dessert mix, stock cube). Forbidden foods include: standard bread, pasta, crispbreads, flour standard biscuits and cakes breakfast cereals made with wheat or oats oatmeal, wheat bran, barley/barley water ‘battered’ or breadcrumbed fish, etc. meat and fruit pies most stock cubes and gravy mixes

Whipple disease This is a rare malabsorption disorder usually affecting white males. It is caused by the bacillus Tropheryma whipplei. It may involve the heart, lungs and CNS. It is fatal if missed.

Clinical features

Males >40 years Chronic diarrhoea (steatorrhoea) Arthralgia (migratory seronegative arthropathy mainly of peripheral joints) Weight loss Lymphadenopathy ± Fever

Diagnoses PCR for T. whipplei Jejunal biopsy—stunted villi

Treatment IV ceftriaxone for 2 weeks then cotrimoxazole or tetracycline for up to 12 months. This produces a dramatic improvement.

Diarrhoea in the elderly The older the person, the more likely a late onset of symptoms that reflect serious underlying organic disease, especially malignancy. Colorectal cancer needs special consideration. Frail or bedridden people have an increasing likelihood with age of faecal impaction with spurious diarrhoea. The possibility of drug interactions (e.g. digoxin) and ischaemic colitis should also be considered.

Ischaemic colitis This is due to atheromatous occlusion of mesenteric vessels (low blood flow) (see CHAPTER 24 ).

Clinical features Clinical features include: sharp abdominal pain in an elderly person with bloody diarrhoea (low blood flow) or periumbilical pain and diarrhoea about 15–30 minutes after eating may be bruits over central abdomen other evidence of generalised atherosclerosis barium enema shows ‘thumb printing’ sign due to submucosal oedema

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the definitive test is aortography and selective angiography of mesenteric vessels most episodes resolve—may be followed by a stricture

Diarrhoea in children The commonest cause of diarrhoea in children is acute infective gastroenteritis, followed by antibiotic-induced diarrhoea. However, certain conditions that develop in infancy and childhood require special attention. The presentation of small amounts of redcurrant jelly-like stool with intussusception should be kept in mind. Of the many causes, only a few could be considered common. Important causes of diarrhoea in children are: infective gastroenteritis antibiotics overfeeding (loose stools in newborn) dietary indiscretions toddler’s diarrhoea sugar (carbohydrate) intolerance food allergies (e.g. milk, soy bean, wheat, eggs) maternal deprivation malabsorption states: cystic fibrosis, coeliac disease Note: Exclude surgical emergencies (e.g. acute appendicitis), infections (e.g. pneumonia), septicaemia, otitis media 2 years old showed that dilute apple juice was better tolerated and resulted in less treatment failure than ORS.7 If acute invasive or persistent Salmonella are present, give antibiotics (ciprofloxacin or azithromycin). Avoid Drugs: antidiarrhoeals, anti-emetics and antibiotics Full-strength lemonade or similar sugary soft drink: osmotic load too high, can use if diluted 1 part to 4 parts water but sugar may be poorly tolerated To treat or not to treat at home Treat at home—if family can cope, vomiting is not a problem and no dehydration. Admit to hospital—if dehydration or persisting vomiting or family cannot cope; also infants 12 mo: 80 mL

Allow for continuing loss. Example: 8 month 10 kg child with 5% dehydration: Fluid loss = 5 × 10 × 10 = 500 mL Maintenance = 100 × 10 = 1000 mL Total 24-hour requirement (min.) = 1500 mL Approximate average hourly requirement = 60 mL Aim to give more (replace fluid loss) in the first 6 hours. Rule of thumb: give 100 mL/kg (infants) and 50 mL/kg (older children) in first 6 hours. Days 2 and 3 Reintroduce your baby’s milk or formula diluted to half strength (i.e. mix equal quantities of milk or formula and water). Their normal food can be continued but do not worry that your child is not eating food. Solids can be commenced after 24 hours. Best to start with bread, plain biscuits, jelly, stewed apple, rice, porridge or non-fat potato chips. Avoid fatty foods, fried foods, raw vegetables and fruit, and wholegrain bread. Day 4 Increase milk to normal strength and gradually continue reintroduction to usual diet. Breastfeeding. If your baby is not vomiting, continue breastfeeding but offer extra fluids (preferably ORS) between feeds. If vomiting is a problem, express breast milk for the time being while you follow the oral fluid program. Note: Watch for lactose intolerance as a sequela—explosive diarrhoea after introducing formula. Replace with a lactose-free formula.

Chronic diarrhoea in children6 Sugar intolerance Synonyms: carbohydrate intolerance, lactose intolerance. The commonest offending sugar is lactose. Diarrhoea often follows acute gastroenteritis when milk is reintroduced into the diet (some recommend waiting for 2 weeks). Stools may be watery, frothy, smell like vinegar and tend to excoriate the buttocks. They contain sugar. Exclude giardiasis.

A simple test follows. Line the napkin with thin plastic and collect fluid stool. Mix 5 drops of liquid stool with 10 drops of water and add a Clinitest tablet (detects lactose and glucose but not sucrose). A positive result suggests sugar intolerance. Diagnosis: lactose breath hydrogen test.

Treatment Remove the offending sugar from the diet. Use milk preparations in which the lactose has been split to glucose and galactose by enzymes, or use soy protein. Note: Most milk allergies improve with age.

Toddler’s diarrhoea (‘cradle crap’) A clinical syndrome of loose, bulky, non-offensive stools with fragments of undigested food in a well, thriving child. The onset is usually between 8 and 20 months. Associated with high fructose intake (fruit juice diarrhoea). Diagnosis by exclusion; treatment by dietary adjustment.

Cow’s milk protein intolerance8 This is not as common as lactose intolerance. Diarrhoea is related to taking a cow’s milk formula and relieved when it is withdrawn. Page 422 Allergic responses to cow’s milk protein may result in a rapid or delayed onset of symptoms. Delayed onset may be more difficult to diagnose, presenting with diarrhoea, malabsorption or failure to thrive. It is diagnosed by unequivocal reproducible reactions to elimination and challenge. If diagnosed, remove cow’s milk from the diet and replace with either soy milk or a hydrolysed or an elemental formula (see CHAPTER 72 ).

Inflammatory bowel disorders These disorders, which include Crohn disease and ulcerative colitis, can occur in childhood. A high index of suspicion is necessary to make an early diagnosis. Approximately 5% of cases of chronic ulcerative colitis have their onset in childhood.5

Chronic enteric infection Responsible organisms include Salmonella sp., Campylobacter, Yersinia, G. lamblia and E. histolytica. With persistent diarrhoea, it is important to obtain microscopy of faeces and aerobic and anaerobic stool cultures. G. lamblia infestation is not an uncommon finding and may be associated with malabsorption, especially of carbohydrate and fat. Giardiasis can mimic coeliac disease.

Coeliac disease (See earlier in chapter.) Clinical features in childhood: usually presents at 9–18 months, but any age previously thriving infant anorexia, lethargy, irritability failure to thrive malabsorption—abdominal distension offensive frequent stools Diagnosis: duodenal biopsy (definitive). Treatment: remove gluten from diet.

Cystic fibrosis Cystic fibrosis, which presents in infancy, is the commonest of all inherited disorders (1 per 2500 live births). Refer to CHAPTER 23 .

Acute gastroenteritis in adults Features Invariably a self-limiting problem (1–3 days) Abdominal cramps Possible constitutional symptoms (e.g. fever, malaise, nausea, vomiting) Other meal-sharers affected → food poisoning

Consider dehydration, especially in the elderly Consider possibility of enteric fever

Traveller’s diarrhoea The symptoms are usually as above, but very severe diarrhoea, especially if associated with blood or mucus, may be a feature of a more serious bowel infection such as amoebiasis. Possible causes of diarrhoeal illness are presented in CHAPTER 129 . Most traveller’s diarrhoea is caused by E. coli, which produces a watery diarrhoea within 14 days of arrival in a foreign country. Another organism is Cryptosporidium parvum. If moderate to severe, azithromycin is recommended for 2–3 days. (For specific treatment refer to the section on Traveller’s diarrhoea in CHAPTER 129 .)

Persistent traveller’s diarrhoea Any traveller with persistent diarrhoea after visiting less developed countries, especially India and China, may have a protozoal infection such as amoebiasis or giardiasis. If there is a fever and blood or mucus in the stools, suspect amoebiasis. Giardiasis is characterised by abdominal cramps, flatulence and bubbly, foul-smelling diarrhoea.

Principles of treatment of diarrhoea Acute diarrhoea Maintenance of hydration: anti-emetic injection (for severe vomiting) prochlorperazine IM, statim or metoclopramide IV, statim Antidiarrhoeal preparations: (avoid if possible, but loperamide preferred) loperamide (Imodium) 2 caps statim then 1 after each unformed stool (max. 8 caps/day) or diphenoxylate with atropine (Lomotil) 2 tabs statim then 1–2 (o) 8 hourly

General advice to patient

Rest Your bowel needs a rest and so do you. It is best to reduce your normal activities until the diarrhoea has stopped. Page 423 Diet Eat as normally as possible but drink small amounts of clear fluids such as water, tea, lemonade and yeast extract (e.g. Vegemite). Then eat low-fat foods such as stewed apples, rice (boiled in water), soups, poultry, boiled potatoes, mashed vegetables, dry toast or bread, biscuits, most canned fruits, jam, honey, jelly, dried skim milk or condensed milk (reconstituted with water). At first, avoid alcohol, coffee, strong tea, fatty foods, fried foods, spicy foods, raw vegetables, raw fruit (especially with hard skins), wholegrain cereals and cigarette smoking. On the third day introduce dairy produce, such as a small amount of milk in tea or coffee and a little butter or margarine on toast. Add also lean meat and fish (either grilled or steamed).

Treatment (antimicrobial drugs)2,9 Bacterial diarrhoea in adults and older children is usually self-limiting and does not require antibiotic treatment (they may be used to shorten the course of a persistent infection). Campylobacter, Salmonella, Shigella and E. coli are the most common causes. As a rule, use oral rehydration solution 2–3 L orally over 24 hours if mild to moderate dehydration. If severe, intravenous rehydration with N saline is recommended. It is advisable not to use antimicrobials except where the following specific organisms are identified. The drugs should be selected initially from the list below or modified according to the results of culture and sensitivity tests.3 Only treat if symptoms have persisted for more than 48 hours. Adult doses are shown for the following specific enteric infections based on faecal culture. Recommended empirical therapy is ciprofloxacin or norfloxacin. Shigella dysentery (moderate to severe) cotrimoxazole (double strength) 1 tab (o) 12 hourly for 5 days: use in children (children’s doses) or norfloxacin 400 mg (o) 12 hourly for 5 days (preferred for adults) or ciprofloxacin 500 mg (o) bd for 5 days Giardiasis

This protozoal infestation is often misdiagnosed. It should be considered for a persistent profuse, watery, bubbly, offensive diarrhoea (see CHAPTER 129 ). tinidazole 2 g (o), single dose (may need repeat) or metronidazole 400 mg (o) tds for 7 days (in children: 30 mg/kg/day [to max. 1.2 g/day] as single daily dose for 3 days) Salmonella enteritis Antibiotics are not generally advisable, but if severe or prolonged, use: ciprofloxacin 500 mg (o) bd for 5–7 days or azithromycin 1 g (o) day, then 500 mg for 6 days or ceftriaxone IV or ciprofloxacin IV if oral therapy not tolerated Note: Salmonella is a notifiable disease; infants under 15 months are at risk of invasive Salmonella infection. Campylobacter A zoonosis that is usually self-limiting. Antibiotic therapy indicated in severe or prolonged cases: azithromycin 500 mg (o) 12 hourly for 3 days or ciprofloxacin 500 mg (o) 12 hourly for 3 days or norfloxacin 400 mg (o) 12 hourly for 5 days Cryptosporidium species Usually self-limiting, may need fluid and electrolytes and antimotility agents.

If severe, nitazoxanide (shared care). Amoebiasis (intestinal) See CHAPTER 129

.

metronidazole 600–800 mg (o) tds for 6–10 days plus diloxanide furoate 500 mg (o) tds for 10 days Blastocystis hominis (a parasitic infection) Pathogenicity is disputed: give therapy only if severe. Associated with poor hygiene (travel, pets, dam/tank water, oysters). metronidazole for 7 days Specialist advice should be sought.

Treatment for special enteric infections Typhoid/paratyphoid fever See CHAPTER 129

.

azithromycin 1 g (o) daily for 7 days or (if not acquired in the Indian subcontinent or South-East Asia) ciprofloxacin 500 mg (o) 12 hourly for 7–10 days (use IV if oral therapy not tolerated) Page 424

If ciprofloxacin is contraindicated (e.g. in children) or not tolerated, then use: ceftriaxone 3 g IV daily until culture and sensitivities available, then choose oral regimens If severe: administer same drug and dosage IV for first 4–5 days. Cholera Antibiotic therapy reduces the volume and duration of diarrhoea. Rehydration is the key. azithromycin 1 g (child 20 mg/kg up to 1 g) (o) as a single dose or

ciprofloxacin 1 g (o) as a single dose For pregnant women and children: amoxicillin (child: 10 mg/kg up to) 250 mg (o) 6 hourly for 4 days

Inflammatory bowel disease3 Two important disorders are ulcerative colitis (UC) and Crohn disease, which have equal sex incidence and can occur at any age, but onset peaks between 20 and 40 years. Inflammatory bowel disease (IBD) should be considered when a young person presents with: bloody diarrhoea and mucus colonic pain and fever urgency to visit toilet and feeling of incomplete defecation constitutional symptoms including weight loss and malaise extra-abdominal manifestations such as arthralgia, low back pain (spondyloarthropathy), eye problems (iridocyclitis), liver disease and skin lesions (pyoderma gangrenosum, erythema nodosum) Investigations include FBE, vitamin B12 and folate, LFTs (abnormal enzymes), HLA-B27, faecal calprotectin (if normal, no intestinal inflammation; if abnormal, needs colonoscopy) and lactoferrin.

Ulcerative colitis Clinical features Mainly a disease of Western societies Mainly in young adults (15–40 years) High-risk factors—family history, previous attacks, low-fibre diet Recurrent attacks of loose stools Blood, or blood and pus, or mucus in stools Abdominal pain slight or absent Fever, malaise and weight loss uncommon

Begins in rectum (continues proximally)—affects only the colon: it usually does not spread beyond the ileocaecal valve An increased risk of carcinoma after 7–10 years

Main symptom Bloody diarrhoea

Diagnosis Faecal calprotectin: a sensitive test Proctosigmoidoscopy: a granular red proctitis with contact bleeding Barium enema: characteristic changes

Prognosis Mortality rates are comparable to the general population without UC10 Recurrent attacks common

Crohn disease Synonyms: regional enteritis, granulomatous colitis. The cause is unknown but there is a genetic link.

Clinical features Recurrent diarrhoea in a young person (15–40 years) Blood and mucus in stools (less than UC) Colicky abdominal pain (small bowel colic) Right iliac fossa pain (confused with appendicitis) Constitutional symptoms (e.g. fever, weight loss, malaise, anorexia, nausea) Signs include perianal disorders (e.g. anal fissure, fistula, ischiorectal abscess), mouth ulcers Skip areas in bowel: ½ ileocolic, ¼ confined to small bowel, ¼ confined to colon, 4% in upper GIT

Main symptom

Colicky abdominal pain

Diagnosis Sigmoidoscopy: ‘cobblestone’ appearance (patchy mucosal oedema) Colonoscopy: useful to differentiate from UC Biopsy with endoscopy

Prognosis Less favourable than UC with both medical and surgical treatment. A 20-year Norway study showed a 1.3 times mortality risk compared to a matched population Page 425 without Crohn disease.11

Management principles of both Education and support, including support groups Treat under consultant supervision Treatment of acute attacks depends on severity of the attack and the extent of the disorder: mild attacks: manage out of hospital severe attacks: hospital, to attend to fluid and electrolyte balance Role of diet controversial: consider a high-fibre diet but maintain adequate nutrition Pharmaceutical agents (the following can be considered): 5-aminosalicylic acid derivatives (mainly UC): sulfasalazine (mainstay), olsalazine, mesalazine. Usually start with these agents corticosteroids (mainly for acute flares): oral, parenteral, topical (rectal foam, suppositories or enemas) for severe disease, immunomodifying drugs (e.g. azathioprine, cyclosporin, methotrexate) and anti-TNF and biological agents (e.g. adalimumab, vedolizumab, infliximab) Surgical treatment: reserve for complications; avoid surgery if possible

Alternating diarrhoea and constipation Alternating diarrhoea and constipation are well-known symptoms of incomplete bowel obstruction (cancer of colon and diverticular disease) and irritable bowel syndrome.

Irritable bowel syndrome (IBS)3,9,12 Clinical features Typically in younger women (21–40 years) Any age or sex can be affected May follow attack of gastroenteritis/traveller’s diarrhoea Cramping abdominal pain (central or iliac fossa)—see FIGURE 34.4 Pain usually relieved by passing flatus or by defecation Variable bowel habit (constipation more common) Diarrhoea usually worse in morning—several loose, explosive bowel actions with urgency The Bristol stool chart was devised to assist with the subclassification of stool types and bowel habits (see: www.continence.org.au/pages/bristol-stool-chart.html) Often precipitated by eating Faeces sometimes like small hard pellets or ribbon-like Anorexia and nausea (sometimes) Bloating, abdominal distension, borborygmi Tiredness common

FIGURE 34.4 Classic symptoms of irritable bowel syndrome The Rome III diagnostic criteria for IBS are presented in TABLE 34.11 Table 34.11

.

Rome III diagnostic criteria for irritable bowel syndrome*13

In the preceding 3 months, the patient has had abdominal discomfort for at least 3 days per month with two of the following three features: relieved by defecation onset associated with a change in stool frequency onset associated with a change in form (appearance) of stool (loose, watery or pellet-like) Symptoms that cumulatively support the diagnosis of irritable bowel syndrome: abnormal stool frequency (for research purposes may be defined as more than three bowel movements per day or fewer than three bowel movements per week) abnormal stool form (lumpy/hard or watery/mushy) abnormal stool passage (straining, urgency or feeling of incomplete evacuation) passage of mucus bloating or feeling of abdominal distension Note: Red flags must be excluded *in absence of structural or metabolic abnormalities to explain symptoms

IBS is a diagnosis of exclusion. A thorough physical examination, investigations (FBE, ESR and stool microscopy or culture) and colonoscopy are necessary. Insufflation of air at colonoscopy may reproduce the abdominal pain of IBS. Page 426

Possible related causes These include bowel infection, food irritation (e.g. spicy foods), lactose (milk) intolerance, excess-fibre wheat products, high fatty foods, carbonated drinks, laxative overuse, use of antibiotics and codeine-containing analgesics, psychological factors.

Management The patient must be reassured and educated with advice that the problem will not cause malignancy or inflammatory bowel disease and will not shorten life expectancy. The basis of initial treatment is simple dietary modification (FODMAPs),14 exercise, fluids (2–3 L water daily) and non-fermentable fibre.

Red flag pointers for non-IBS disease15 Age of onset >50 years Fever Unexplained weight loss Rectal bleeding Pain waking at night Persistent daily diarrhoea/steatorrhoea Recurrent vomiting Major change in symptoms Mouth ulcers ↑ CRP, ESR Anaemia Family history of bowel cancer or IBD

Self-help advice to the patient Anyone with IBS should try to work on the things that make the symptoms worse. If you recognise stresses and strains in your life, try to develop a more relaxed lifestyle. You may have to be less of a perfectionist in your approach to life. Focus on establishing a regular eating pattern. Try to avoid any foods that you can identify as causing the problem. You may have to cut out smoking and alcohol and avoid laxatives and codeine (in painkillers). A high-fibre (non-fermentable) and low-carbohydrate diet and 2–3 L of water a day may be the answer to your problem. A low-FODMAP diet can produce good benefits.11,13,16 FODMAP refers to fermentable oligosaccharides, disaccharides, monosaccharides and polyols, which are poorly absorbed. All of these carbohydrates need to be eliminated (under a dietitian’s guidance), then reintroduced one at a time. See: www.med.monash.edu/cecs/gastro/fodmap, available as an app—the Monash University Low-FODMAP Diet.

Diverticular disorder Diverticular disorder is a problem of the colon (90% in descending colon) and is related to lack of fibre in the diet. It is usually symptomless.

Clinical features Typical in middle-aged or elderly—over 40 years Increases with age Present in one in three people over 60 years (Western world) Diverticulosis—symptomless Diverticulitis—infected diverticula and symptomatic (refer CHAPTER 24 Constipation or alternating constipation/diarrhoea Intermittent cramping lower abdominal pain in LIF Tenderness in LIF Rectal bleeding—may be profuse (± faeces) May present as acute abdomen or subacute obstruction Usually settles in 2–3 days

Complications (of diverticulitis) Bleeding—may cause massive lower GIT bleeding Abscess Perforation Peritonitis Obstruction (refer CHAPTER 24

)

Fistula—bladder, vagina

Investigations WBC and ESR—to determine inflammation Sigmoidoscopy

)

Barium enema

Management It usually responds to a high-fibre diet. Avoidance of constipation.

Advice to the patient The gradual introduction of fibre with plenty of fluids (especially water) will improve any symptoms you may have and reduce the risk of complications. Your diet should include: Page 427

1. cereals, such as bran, shredded wheat, muesli or porridge 2. wholemeal and multigrain breads 3. fresh or stewed fruits and vegetables Bran can be added to your cereal or stewed fruit, starting with 1 tablespoon and gradually increasing to 3 tablespoons a day. Fibre can make you feel uncomfortable for the first few weeks, but the bowel soon settles with your improved diet.

When to refer Children with diarrhoea Infant under 3 months Moderate to severe dehydration Diagnosis of diarrhoea and vomiting in doubt (e.g. blood in vomitus or stool, bile-stained vomiting, high fever or toxaemia, abdominal signs suggestive of appendicitis or obstruction) Failure to improve or deterioration A pre-existing chronic illness

Adults with diarrhoea Patient with chronic or bloody diarrhoea Any problem requiring colonoscopic investigation Patients with anaemia

Patients with weight loss, abdominal mass or suspicion of neoplasia Patients with anal fistulae Patients not responding to treatment for giardiasis Infection with E. histolytica Long-term asymptomatic carrier of typhoid or paratyphoid fever Patient with persistent undiagnosed nocturnal diarrhoea Patients with IBS with a significant change in symptoms Patients with inflammatory bowel diseases with severe exacerbations, possibly requiring immunosuppressive therapy and with complications Patients with ulcerative colitis of more than 7 years’ duration (screening by colonoscopy for carcinoma)

Practice tips Oral antidiarrhoeal drugs are contraindicated in children; besides being ineffective they may prolong intestinal recovery. Anti-emetics can readily provoke dystonic reactions in children, especially if young and dehydrated. Acute diarrhoea is invariably self-limiting (lasts 2–5 days). If it lasts longer than 7 days, investigate with culture and microscopy of the stools. If diarrhoea is associated with episodes of facial flushing or wheezing, consider carcinoid syndrome. Recurrent pain in the right hypochondrium is usually a feature of IBS (not gall bladder disease). Recurrent pain in the right iliac fossa is more likely to be IBS than appendicitis. Beware of false correlations or premature conclusions (e.g. attributing the finding of diverticular disorder on barium meal to the cause of the symptoms). Undercooked chicken is a common source of enteropathic bacterial infection. Consider alcohol abuse if a patient’s diarrhoea resolves spontaneously on hospital admission.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Coeliac disease in adults Coeliac disease in children Cystic fibrosis Diarrhoea—acute diarrhoea in adults Diverticular disease Gastroenteritis in children Inflammatory bowel disease Page 428

References 1

Bolin T, Riordan SM. Acute and persistent diarrhoea. Current Therapeutics, 2001; May: 47–57.

2

Cheng AC et al. Australasian Society for Infectious Diseases guidelines for the diagnosis and treatment of Clostridium difficile infection. Med J Aust, 2011; 194(7): 353–8.

3

Supportive measures of gastroenteritis [published 2016]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2016. www.tg.org.au, accessed October 2019.

4

Dalton C. Foodborne illness: how to treat. Australian Doctor, 15 April; 2005: 39–46.

5

Robinson MJ, Roberton DM. Practical Paediatrics (5th edn). Edinburgh: Churchill Livingstone, 2003: 675–90.

6

Oberklaid F. Management of gastroenteritis in children. In: The Australian Paediatric Review. Melbourne: Royal Children’s Hospital, 1990: 1–2.

7

Freedman S et al. Effect of dilute apple juice and preferred fluids versus electrolyte maintenance solution on treatment failure among children with mild gastroenteritis: a randomized clinical trial. JAMA, 2016; 315(18): 1966–74.

8

Gwee A, Rimer R, Marks M. Paediatric Handbook (9th edn). Oxford: Wiley-Blackwell, 2015: 90–5.

9

Onwuezobe IA et al. Antimicrobials for treating symptomatic non-typhoidal Salmonella

infection (Cochrane Review). Cochrane Database Syst Rev, 2012; Issue 11: Art No. CD001167. 10

Manninen P et al. Mortality in ulcerative colitis and Crohn’s disease: a population-based study in Finland. J Crohns Colitis, June 2012; 6(5): 524–8.

11

Hovde Ø et al. Mortality and causes of death in Crohn’s disease: results from 20 years of follow-up in the IBSEN study. Gut, 2014; 63: 771–5.

12

NICE. Irritable bowel syndrome in adults: diagnosis and management of irritable bowel syndrome in primary care, 2008. Available from: www.nice.org.uk, accessed 25 May 2018.

13

Rome Foundation. Rome III diagnostic criteria for functional gastrointestinal disorders. J Gastrointestin Liver Dis, 2006; 15(3): 307–12.

14

Gibson PR. Irritable bowel syndrome. Australian Doctor, 13 April 2012: 17–34.

15

Ellard K, Malcolm A. Irritable bowel syndrome. Medical Observer, 30 March 2007: 29– 32.

16

Gibson PR, Shepherd SJ. Evidence-based dietary management of functional gastrointestinal symptoms: the FODMAP approach. J Gastroenterol Hepatology, 2010; Feb 25(2): 2528.

Page 429

35 Dizziness/vertigo

I got my giddiness in 1690 (at the age of 23) by eating 100 golden pippins at a time at Richmond. Four years later at a place 20 miles further on in Surrey I got my deafness; and these two ‘friends’ have visited me one or other year since, and being old acquaintances have often sought fit to come together. JONATHAN SWIFT (1667–1745), DESCRIBING HIS MÉNIÈRE SYNDROME When patients complain of ‘dizziness’, they can be using this term to describe many different phenomena, and hence a careful history is required to unravel the problem. Others may use different terms, such as ‘giddiness’, ‘swimming in the head’, ‘my brain spinning’, ‘whirling’ and ‘swinging’. ‘Dizzy’ comes from an old English word, dysig, meaning foolish or stupid. Strictly speaking, it means unsteadiness or lightheadedness—without movement, motion or spatial disorientation. ‘Vertigo’, on the other hand, comes from the Latin vertere (to turn) and -igo for a condition. It should describe a hallucination of rotation of self or the surroundings in a horizontal or vertical direction.1 The term ‘dizziness’, however, is generally used collectively to describe all types of equilibrium disorders and, for convenience, can be classified as shown in FIGURE 35.1 .

FIGURE 35.1 Classification of dizziness

Key facts and checkpoints Approximately one-third of the population will have suffered from significant dizziness by age 65 and about a half by age 80.2 The commonest causes in family practice are postural hypotension and hyperventilation. The ability to examine and interpret the sign of nystagmus accurately is important in the diagnostic process. A drug history is very important, including prescribed drugs and others such as alcohol, cocaine, marijuana and illicit drugs. Ménière syndrome is overdiagnosed. It has the classic triad: vertigo–tinnitus– deafness (sensorineural). Vertebrobasilar insufficiency is also overdiagnosed as a cause of vertigo. It is a rare cause but may result in dizziness and sometimes vertigo but rarely in isolation.

Defined terminology Vertigo2

Vertigo is defined as an episodic sudden sensation of circular motion of the body or of its surroundings or an illusion of motion, usually a rotatory sensation. Other terms used to describe this symptom include ‘everything spins’, ‘my head spins’, ‘the room spins’, ‘whirling’, ‘reeling’, ‘swaying’, ‘pitching’ and ‘rocking’. It is frequently accompanied by autonomic symptoms such as nausea, retching, vomiting, pallor and sweating. Vertigo is characteristically precipitated by standing, by turning the head or by movement. Patients have to walk carefully and may become nervous about descending stairs or crossing the road, and usually seek support. Therefore, the vertiginous person is usually very frightened and tends to remain immobile during an attack and may feel their feet being lifted under them. Patients may feel as though they are being impelled by some outside force that tends to pull them to one side, especially while walking. True vertigo is a symptom of disturbed function involving the vestibular system or its Page 430 central connections. It invariably has an organic cause. Important causes are presented in TABLE 35.1 , while FIGURE 35.2 illustrates central neurological centres that can cause vertigo. Some features of central vertigo include gait ataxia out of proportion to vertigo, diplopia, hemisensory loss, slurred speech, difficulty swallowing and abnormal eye movements. With peripheral vertigo, hearing loss, tinnitus, ear fullness and a positive head impulse test may be present.2 Table 35.1

Causes of vertigo

Peripheral disorders Labyrinth: labyrinthitis: viral or suppurative Ménière syndrome benign paroxysmal positional vertigo (BPPV) drugs trauma chronic suppurative otitis media Eight nerve: vestibular neuronitis acoustic neuroma drugs Cervical vertigo Central disorders Brain stem (TIA or stroke): vertebrobasilar insufficiency infarction

Cerebellum: degeneration tumours Migraine Multiple sclerosis

FIGURE 35.2 Diagrammatic illustration of central centres that can cause vertigo Nystagmus is often seen with vertigo and, since 80–85% of causes are due to an ear problem, tinnitus and hearing disorders are also occasionally associated. In acute cases there is usually a reflex autonomic discharge producing sweating, pallor, nausea and vomiting.

Giddiness Giddiness is a sensation of uncertainty or ill-defined lightheadedness. Other terms used include ‘a swimming sensation’, ‘walking on air’ and ‘ground going beneath me’. It usually contains no elements of rotation, impulsion, tinnitus, deafness, nausea or vomiting. Page 431 The patient with giddiness, although fearful of falling or swooning, can nonetheless walk without difficulty if forced to do so.

Giddiness is also a typical psychoneurotic symptom.

Syncopal episodes Syncope may present as a variety of dizziness or lightheadedness in which there is a sensation of impending fainting or loss of consciousness. Presyncope is a sensation of feeling faint. Common causes are cardiogenic disorders and postural hypotension, which are usually drug-induced.

Disequilibrium Disequilibrium implies a condition in which there is a loss of balance or instability while walking, without any associated sensations of spinning. Other terms used to describe this include ‘unsteadiness on feet’, ‘the staggers’, ‘swaying feeling’ and ‘dizzy in the feet’. Disequilibrium is usually of neurogenic origin.

A diagnostic approach A summary of the diagnostic strategy model is presented in TABLE 35.2 Table 35.2

Dizziness/vertigo: diagnostic strategy model

Probability diagnosis Anxiety-hyperventilation (G) Postural hypotension (G/S) Simple faint—vasovagal (S) Acute vestibulopathy (V) Benign paroxysmal positional vertigo (V) Motion sickness (V) Vestibular migraine (V) Cervical dysfunction/spondylosis Serious disorders not to be missed Neoplasia: acoustic neuroma posterior fossa tumour other brain tumours, primary or secondary Intracerebral infection (e.g. abscess) Cardiovascular:

.

arrhythmias myocardial infarction aortic stenosis Cerebrovascular: vertebrobasilar insufficiency brain-stem infarct (e.g. PICA thrombosis) Multiple sclerosis Carbon monoxide poisoning Pitfalls (often missed) Ear wax (G) Otosclerosis Arrhythmias Hyperventilation Alcohol and other drugs Cough or micturition syncope Vestibular migraine/migrainous vertigo Parkinson disease Ménière syndrome (overdiagnosed) Rarities: Addison disease (CHAPTER 14 ) neurosyphilis autonomic neuropathy hypertension subclavian steal perilymphatic fistula Shy–Drager syndrome Seven masquerades checklist Depression Diabetes (possible: hypo/hyper) Drugs Anaemia Thyroid disorder (possible) Spinal dysfunction UTI (possible) Is the patient trying to tell me something? Very likely. Consider anxiety and/or depression.

G = giddiness; S = syncope; V = vertigo

Probability diagnosis In medical school we gain the wrong impression that the common causes of dizziness or vertigo are the relatively uncommon causes, such as Ménière syndrome, aortic stenosis, Stokes–Adams attacks, cerebellar disorders, vertebrobasilar disease and hypertension. In the real world of medicine, one is impressed by how often dizziness is caused by relatively common benign conditions, such as hyperventilation associated with anxiety, simple syncope, postural hypotension due to drugs and old age, inner ear infections, wax in the ears, post head injury, motion sickness and alcohol intoxication. In most instances making the correct diagnosis (which, as ever, is based on a careful history) is straightforward, but finding the underlying cause of true vertigo can be very difficult. The common causes of vertigo seen in general practice are benign paroxysmal positional vertigo (BPPV), accounting for about 25% of cases, acute vestibulopathy (vestibular neuronitis) and vestibular migraine. Viral labyrinthitis is basically the same as vestibular neuronitis, except that the whole of the inner ear is involved so that deafness and tinnitus arise simultaneously with severe vertigo. The most common causes of recurrent spontaneous vertigo are vestibular migraine and Ménière syndrome. Page 432

Serious disorders not to be missed Neoplasia The important serious disorders to keep in mind are space-occupying tumours, such as acoustic neuroma, medulloblastoma and other tumours (especially posterior fossa tumours) capable of causing vertigo, intracerebral infections and cardiovascular abnormalities. It is important to bear in mind that the commonest brain tumour is a metastatic deposit from lung cancer.3

Red flags for dizziness/vertigo Neurological signs Ataxia out of proportion to vertigo Nystagmus out of proportion to vertigo Central nystagmus

Central eye movement abnormalities

Acoustic neuroma This uncommon tumour should be suspected in the patient presenting with the symptoms shown in the diagnostic triad below. Headache may occasionally be present. DxT (unilateral) tinnitus + hearing loss + unsteady gait → acoustic neuroma

Diagnosis is best clinched by high-resolution MRI. Audiometry and auditory evoked responses are also relevant investigations.

Cardiac disorders Cardiac disorders that must be excluded for giddiness or syncope are the various arrhythmias, such as Stokes–Adams attacks caused by complete heart block, aortic stenosis and myocardial infarction.

Cerebrovascular causes The outstanding cerebrovascular causes of severe vertigo are vertebrobasilar insufficiency and brain-stem infarction. Vertigo is the commonest symptom of transient cerebral ischaemic attacks in the vertebrobasilar distribution.1 Severe vertigo, often in association with hiccoughs and dysphagia, is a feature of the variety of brain-stem infarctions known as the lateral medullary syndrome due to posterior inferior cerebellar artery (PICA) thrombosis. There is a dramatic onset of vertigo with cerebellar signs, including ataxia and vomiting. There are ipsilateral cranial nerve (brain stem) signs with contralateral spinothalamic sensory loss of the face and body. Diagnosis is by CT or MRI scanning.

Neurological causes Important neurological causes of dizziness are multiple sclerosis and complex partial seizures. The lesions of multiple sclerosis may occur in the brain stem or cerebellum. Young patients who present with a sudden onset of vertigo with ‘jiggly’ vision but without auditory symptoms should be considered as having multiple sclerosis. Five per cent of cases of multiple sclerosis present with vertigo.

Pitfalls A list of conditions causing dizziness that may be misdiagnosed is presented in TABLE 35.2

.

Wax in the ear certainly causes dizziness, though its mechanism of action is controversial. Cough and micturition syncope do occur, although they are uncommon. Ménière syndrome is a pitfall in the sense that it tends to be overdiagnosed.

Seven masquerades checklist Of these conditions, drugs and vertebral dysfunction (of the cervical spine) stand out as important causes. Depression demands attention because of the possible association of anxiety and hyperventilation. Diabetes mellitus has an association through the possible mechanisms of hypoglycaemia from therapy or from an autonomic neuropathy.

Drugs Drugs usually affect the vestibular nerve rather than the labyrinth. Drugs commonly associated with dizziness are presented in TABLE 35.3 . Table 35.3

Drugs that can cause dizziness

Alcohol Antibiotics: streptomycin, gentamicin, kanamycin, tetracyclines Antidepressants Anti-epileptics: phenytoin Antihistamines Antihypertensives Aspirin and salicylates Cocaine, cannabis Diuretics in large doses: intravenous frusemide, ethacrynic acid Glyceryl trinitrate Quinine: quinidine Tranquillisers: phenothiazines, phenobarbitone, benzodiazepines Page 433

Cervical spine dysfunction It is not uncommon to observe vertigo in patients with cervical spondylosis or post cervical spinal injury. It has been postulated4 that this may be caused by the generation of abnormal impulses from proprioceptors in the upper cervical spine, or by osteophytes compressing the vertebral arteries in the vertebral canal. Some instances of BPPV are associated with disorders of the cervical spine.

Psychogenic considerations This may be an important aspect to consider in the patient presenting with dizziness, especially if the complaint is giddiness or lightheadedness. An underlying anxiety, particularly agoraphobia and panic disorder, may be the commonest cause of this symptom in family practice and clinical investigation of hyperventilation may confirm the diagnosis. The possibility of depression must also be kept in mind.5 Many of these patients harbour the fear that they may be suffering from a serious disorder, such as a brain tumour or multiple sclerosis, or face an impending stroke or insanity. Appropriate reassurance to the contrary is often positively therapeutic for that patient.

The clinical approach The essentials of the diagnostic approach include careful attention to the history and physical examination, and judicious selection of specific office tests and special investigations.

History It is important to get patients to explain the precise nature of the symptoms, even asking their opinion as to the cause of their dizziness.

Key questions The following questions should be addressed: Is it vertigo or pseudovertigo? Symptom pattern: paroxysmal or continuous? effect of position and change of posture? Any aural symptoms? Tinnitus? Deafness? Any visual symptoms? Any neurological symptoms? Any nausea or vomiting? Any symptoms of psychoneurosis? Any recent colds? Any recent head injury (even trivial)?

Any drugs being taken? alcohol? marijuana? hypotensives? psychotropics? other drugs?

Examination A full general examination is appropriate with particular attention being paid to the cardiovascular and central nervous systems and the auditory and vestibular mechanisms.

Guidelines Examination guidelines are: 1. ear disease: auroscopic examination: ?wax, ?drum hearing tests Weber and Rinne tests 2. the eyes: visual acuity test movements for nystagmus 3. cardiovascular system: evidence of atherosclerosis blood pressure: supine, standing, sitting cardiac arrhythmias 4. cranial nerves: 2nd, 3rd, 4th, 6th and 7th corneal response for 5th

8th—auditory nerve 5. the cerebellum or its connections: gait coordination reflexes Romberg test finger–nose test: ?past pointing 6. the neck, including cervical spine 7. general search for evidence of: anaemia polycythaemia alcohol dependence

Office tests for dizziness Ask the patient to perform any manoeuvre that may provoke the symptom. Carry out head positional testing to induce vertigo and/or nystagmus (e.g. Hallpike manoeuvre in FIG. 35.3 or head impulse test/head thrust). Avoid if prominent spontaneous vertigo and nystagmus. The 3-part HINTS test (head impulse, nystagmus, test of skew) is useful for distinguishing the cause of vertigo as either central (urgent) or peripheral. Page 434 Take blood pressure measurements in three positions. Perform forced hyperventilation (20–25 breaths per minute) for 2 minutes. Carry out palpation of carotid arteries and carotid sinus (with care).

Investigations Appropriate laboratory tests should be selected from TABLE 35.4

Investigations (select only if indicated) Table 35.4

.

Haemoglobin Blood glucose ECG: ?Holter monitor Audiometry Brain-stem evoked audiometry Caloric test Visual evoked potentials (MS) Electrocochleography Electro-oculography (electronystagmography) Rotational tests Radiology: chest X-ray (?bronchial carcinoma) cervical spine X-ray CT scan MRI (the choice to locate acoustic neuroma or other tumour—may detect MS and vascular infarction)

Diagnostic guidelines A sudden attack of vertigo in a young person following a recent URTI is suggestive of vestibular neuritis. Dizziness is a common symptom in menopausal women and is often associated with other features of vasomotor instability. Phenytoin therapy can cause cerebellar dysfunction. Postural and exercise hypotension are relatively common in the older atherosclerotic patient. Acute otitis media does not cause vertigo but chronic otitis media can, particularly if the patient develops a cholesteatoma, which then erodes into the internal ear causing a perilymphatic fistula.

Dizziness in children Dizziness is not a common symptom in children. Vertigo can have sinister causes and requires referral because of the possibility of tumours, such as a medulloblastoma. A study by Eviatar6 of vertigo in children found that the commonest cause was a seizure focus particularly affecting the temporal lobe. Other causes included psychosomatic vertigo, vestibular migraine and vestibular neuritis.

Apart from the above causes it is important to consider: infection (e.g. meningitis, meningoencephalitis, cerebral abscess) trauma, especially to the temporal area middle-ear infection labyrinthitis (e.g. mumps, measles, influenza) BPPV (short-lived attacks of vertigo in young children between 1 and 4 years of age: tends to precede adulthood migraine)7 hyperventilation drugs—prescribed illicit drugs (e.g. cocaine, marijuana) cardiac arrhythmias alcohol toxicity A common trap is the acute effect of alcohol in curious children who can present with the sudden onset of dizziness.

‘Dizzy turns’ in girls in late teens These are commonly due to blood pressure fluctuations. Give advice related to reducing stress, lack of sleep and excessive exercise. Reassure that it settles with age (rare after 25 years).

Dizziness in the elderly Dizziness is a relatively common complaint of the elderly. Common causes include postural hypotension related mainly to drugs prescribed for hypertension or other cardiovascular problems. Cerebrovascular disease, especially in the areas of the brain stem, is also relevant in this age group. True vertigo can be produced simply by an accumulation of wax in the external auditory meatus, being more frequent than generally appreciated. Middle-ear disorder is also sometimes the cause of vertigo in an older person but disorder of the auditory nerve, inner ear, cerebellum, brain stem and cervical spine are common underlying factors. Malignancy, primary and secondary, is a possibility in the elderly. The possibility of cardiac

arrhythmias as a cause of syncopal symptoms increases with age.

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‘Dizzy turns’ in elderly women If no cause such as hypertension is found, advise them to get up slowly from sitting or lying, and to wear firm elastic stockings.

Common general conditions Acute vestibulopathy (vestibular failure) Causes: vestibular neuritis stroke—AICA or PICA Vestibular neuritis covers both vestibular neuronitis and labyrinthitis, which are considered to be a viral infection of the vestibular nerve and labyrinth respectively, causing a prolonged attack of vertigo that can last for several days and be severe enough to require admission to hospital.8 This is more likely with labyrinthitis. It is analogous to a viral infection of the 7th nerve causing Bell palsy. The attack is similar to Ménière syndrome except that there is no hearing disturbance. DxT acute vertigo + nausea + vomiting → vestibular neuronitis

DxT same symptoms + hearing loss ± tinnitus → acute labyrinthitis

Characteristic features Single attack of vertigo without tinnitus or deafness Usually preceding ‘flu-like’ illness Mainly in young adults and middle age Abrupt onset with vertigo, ataxia, nausea and vomiting Generally lasts days to weeks Examination shows lateral or unidirectional nystagmus—rapid component away from side of

lesion (no hearing loss) Caloric stimulation confirms impaired vestibular function It is basically a diagnosis of exclusion.

Treatment Rest in bed, lying very still Gaze in the direction that eases symptoms The following drugs can be used for the first 2 days (see TABLE 35.5

):

prochlorperazine (Stemetil) 5–10 mg (o) 6–8 hrly or 12.5 mg IM (if severe vomiting), but may slow recovery or promethazine 10–25 mg IM or slow IV, then 10–25 mg (o) for 48 hrs or ondansetron 4–8 mg (o) 2–3 hrly or (recommended as best) diazepam (which decreases brain-stem response to vestibular stimuli)2 5–10 mg IM for the acute attack (care with respiratory depression), then 5 mg (o) tds for 2–3 days Table 35.5

Symptomatic relief of acute vertigo: pharmaceutical options10

Anti-emetics: prochlorperazine metoclopramide ondansetron Antihistamines: promethazine betahistine Benzodiazepines (short period use for vertigo): diazepam lorazepam

A short course of corticosteroids often promotes recovery (e.g. prednisolone 1 mg/kg (up to 100 mg) (o) daily in morning for 5 days, then taper over 15 days and cease).1,9

Outcome Both are self-limiting disorders and usually settle over 5–7 days or several weeks. Labyrinthitis usually lasts longer and during recovery rapid head movements may bring on transient vertigo.

Benign paroxysmal positional vertigo BPPV is a common type of acute vertigo that is induced by changing head position—particularly tilting the head backwards, changing from a recumbent to a sitting position or turning to the affected side.

Features Affects all ages, especially the elderly The female to male ratio is 2:1 Recurs periodically for several days Each attack is brief, usually lasts 10–60 seconds and subsides rapidly Severe vertigo on getting out of bed

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Can occur on head extension and turning head in bed Attacks are not accompanied by vomiting, tinnitus or deafness (nausea may occur) In one large series 17% were associated with trauma, 15% with viral labyrinthitis, while about 50% had no clear predisposing factor other than age. One accepted theory of causation is that fine pieces of floating crystalline calcium carbonate deposits (otoconia) that are loose in the labyrinth settle in the posterior semicircular canal and generate endolymphatic movement.11 It may also be a variation of cervical dysfunction. Diagnosis is confirmed by head position testing: head impulse (head thrust) test or Hallpike manoeuvre (refer to YouTube for these manoeuvres).12 In the latter, from a sitting position, the patient’s head is rapidly taken to a head-hanging position 30° below the level of the couch —do three times, with the head (1) straight, (2) rotated to the right, (3) rotated to the left. Hold on for 30 seconds and observe the patient carefully for vertigo and nystagmus. There is a latent period of a few seconds before the onset of the symptoms—see FIGURE 35.3 . Tests of hearing and vestibular function are normal

There is usually spontaneous recovery in weeks (most return to regular activity after 1 week) Recurrences are common: attacks occur in clusters

FIGURE 35.3 Hallpike manoeuvre: positional testing for benign paroxysmal positional vertigo (head rotated to 45° then taken rapidly from a sitting position to a hanging position). Repeat with head turned to the opposite side. A positive response is the onset of symptoms ± nystagmus with the affected ear lowermost.

Management Give appropriate explanation and reassurance Avoidance measures: encourage the patient to move in ways that avoid the attack Drugs are not recommended—usually ineffective Special exercises Cervical traction may help Particle repositioning manoeuvres Patient-performed exercises. Most patients appear to benefit from exercise, such as the Brandt– Daroff procedure13 or the Cawthorne–Cooksey exercises10 that consist essentially of repeatedly inducing the symptoms of vertigo. Rather than resorting to avoidance measures, the patient is

instructed to perform positional exercises to induce vertigo, hold this position until it subsides, and repeat this many times until the manoeuvre does not precipitate vertigo. The attacks then usually subside in a few days. Therapist-performed exercises. Physical manoeuvres performed as an office procedure include the Epley and Semont manoeuvres, which aim to dislodge otoliths from a semicircular canal. The Epley manoeuvre has a high (77%) success rate on the initial attempt and up to 100% on further attempts.2 Surgical treatment Rarely surgical treatment is required; it involves occlusion of the posterior semicircular canal rather than selective neurectomy.

Ménière syndrome This is caused by a build-up of endolymph. It is an uncommon condition which is commonest in the 30–50 years age group. It is characterised by paroxysmal attacks of vertigo, tinnitus, nausea and vomiting, sweating and pallor, deafness (progressive). Onset is abrupt—patient may fall and then be bedridden for 1–2 hours. Patient doesn’t like moving head. Attacks last 30 minutes to several hours. There is a variable interval between attacks (twice a month to twice a year). Nystagmus is observed only during an attack (often to side opposite affected inner ear). Examination: sensorineural deafness (low tones) caloric test: impaired vestibular function audiometry: sensorineural deafness, loudness recruitment special tests There are characteristic changes in electrocochleography.

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DxT vertigo + vomiting + tinnitus + sensorineural deafness → Ménière syndrome

Treatment The aim is to reduce endolymphatic pressure by reducing the sodium and water content of the endolymph. Prophylaxis hydrochlorothiazide 25 mg (o) daily or with triamterene or amiloride combination (o) once daily. Acute attack1,14 Anticipation of attack (fullness, tinnitus): prochlorperazine 25 mg suppository Treatment: For severe attack, diazepam 5 mg IV ± prochlorperazine 12.5 mg IM, or if episodic, a diuretic, e.g. hydrochlorothiazide 25 mg (o) daily Long term Reassurance with a careful explanation of this condition to the patient, who often associates it with malignant disease Excess intake of salt, tobacco and coffee to be avoided A low-salt diet is the mainstay of treatment ( 10% Vomiting Older age >50 years Chronic NSAID use Severe frequent symptoms Family history of upper GIT or colorectal cancer Short history of symptoms

Unresponsive H. pylori treatment Barrett oesophagus screening in high-risk patients

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Complications Oesophagitis ± oesophageal ulcer Iron-deficiency anaemia Oesophageal stricture Respiratory: chronic cough, asthma, hoarseness Barrett oesophagus (from prolonged reflux)

Investigations8 Endoscopy (see Red flag pointers)—perform prior to empirical therapy. Limited role—about one-third negative Barium swallow and meal 24-hour ambulatory oesophageal pH monitoring

Management of GORD8,9,10,11 Stage 1 Patient education/appropriate reassurance Consider acid suppression or neutralisation Attend to lifestyle: reduce weight if overweight (this alone may abolish symptoms) reduce or cease smoking reduce or cease alcohol (especially with dinner) avoid fatty foods (e.g. pastries, french fries) reduce or cease coffee, tea and chocolate avoid coffee and alcohol late at night

avoid gaseous drinks leave at least 3 hours between the evening meal and retiring increase fibre intake (e.g. high-fibre cereals, fruit and vegetables) small regular meals and snacks eat slowly and chew food well sleep on the left side main meal at midday; light evening meal avoid spicy foods and tomato products Drugs to avoid: anticholinergics, theophylline, nitrates, calcium-channel blockers, doxycycline. Pill-induced (i.e. before absorption) oesophagitis occurs, especially with tetracyclines, slow-release potassium, iron sulphate, corticosteroids, NSAIDs—avoid taking dry; use ample fluids Elevation of head of bed or wedge pillow: if GORD occurs in bed, sleep with head of bed elevated 10–20 cm on wooden blocks or use a wedge pillow (preferable) Antacids (see TABLES 36.4 and 36.5 ): best is liquid alginate/antacid mixture, e.g. Gaviscon/Mylanta plus 20 mL on demand or 1–2 hours after meals and at bedtime Table 36.4

Antacids in common use

Antacids Water soluble:

Calcium carbonate Sodium: bicarbonate citrotartrate Note: Excess is prone to cause alkalosis—apathy, mental changes, stupor, kidney dysfunction, tetany

Water insoluble:

Aluminium: hydroxide glycinate phosphate Magnesium: alginate

carbonate hydroxide trisilicate Combination antacids Antacid + alginic acid Antacid + oxethazaine Antacid + simethicone

Table 36.5

Side effects of common antacids

Aluminium hydroxide

Constipation

Magnesium trisilicate

Diarrhoea

Sodium bicarbonate

Alkalosis Milk-alkali syndrome Aggravation of hypertension

Calcium carbonate

Alkalosis Constipation Milk-alkali syndrome Hypercalcaemia

Antacids are appropriate for rapid relief of mild intermittent or occasional breakthrough symptoms but are ineffective for long-term management. Stage 28 If no relief after several weeks, the following approaches are recommended by the Gastroenterological Society of Australia (GESA).8 Reduce acid secretion. Select from: Proton-pump inhibitor (PPI) for 4 weeks (preferred agent) 30–60 minutes before food lansoprazole 30 mg mane or omeprazole 20 mg mane

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or pantoprazole 40 mg mane or esomeprazole 20 mg mane or rabeprazole 20 mg mane H2-receptor antagonists (oral use for 8 weeks) famotidine 20 mg bd or nizatidine 150 mg bd or 300 mg nocte or ranitidine 150 mg bd pc or 300 mg nocte Antacids are useful for daytime symptoms Although the more traditional step-up approach of 1. Antacids → 2. H2-receptor antagonists → 3. PPI can be used, there has been a change to favour a high-level (more potent) initial therapy with PPIs at standard dose (a step-down approach; see FIG. 36.1 ). This is based on the grounds of outcomes, speed of response and total cost. May need to eradicate H. pylori if present, although there is no consistent evidence of an association with GORD.10

FIGURE 36.1 The stepwise approach to the management of dyspeptic symptoms7 Surgery is usually for young people with severe reflux. The gold standard is a short, loose 360degree laparoscopic fundoplication.

Hiatus hernia See FIGURE 36.2

.

Common, especially in obese and >50 years. Most asymptomatic but GORD common. Diagnosis by barium swallow.

FIGURE 36.2 Hiatus hernia: rolling and sliding Source: Longmore M, Wilkinson IB et al. Oxford Handbook of Clinical Medicine (9th edn). Oxford, 2014: 245. Reproduced with permission of the Licensor through PLSclear.

Types Sliding: GO junction slides into chest. Acid reflux common. Rolling (paraoesophageal): bulge of stomach herniates into chest. GORD uncommon but prone to strangulation.

Treatment Weight loss, esp. for GORD (treat reflux symptoms). Consider PPIs. Surgery for intractable symptoms and for repair of rolling hernia.

Functional (non-ulcer) dyspepsia9,12 This term applies to the 60% of patients presenting with dyspepsia in which there is discomfort on eating in the absence of demonstrable organic disease. This can be considered in two categories (although there is overlap): ulcer-like dyspepsia—localised pain or dysmotility-like dyspepsia—diffuse discomfort

Ulcer-like dyspepsia Treat as for GORD. A practical approach is to commence with a 4-week trial of a PPI or an H2receptor antagonist and cease if symptoms resolve.9

Dysmotility-like dyspepsia Clinical features Discomfort with early sense of fullness on eating Nausea Overweight Emotional stress Poor diet (e.g. fatty foods) Similar lifestyle guidelines to GORD

Management Treat as for GORD (stage 1). Include antacids. If not responsive: Step 1: H2-receptor antagonists Step 2: prokinetic agents domperidone 10 mg tds or metoclopramide 10 mg tds Consider possibility of Barrett oesophagus or gastroparesis (see CHAPTER 49

). Page 446

Barrett oesophagus Usually a metaplastic response to prolonged reflux A premalignant condition (adenocarcinoma)

Lower oesophagus lined with gastric mucosa (at least 3 cm) of metaplastic columnar epithelium Prone to ulceration Needs careful management, which includes PPIs for symptoms of oesophagitis + reflux Consider 2-yearly endoscopies with biopsies Diagnosed by endoscopy and biopsy

Peptic ulcer disease12,13 Features (general) Common: 10% incidence over a lifetime, but decreasing Peptic ulcers accounted for 1 in every 500 deaths in Australia14 in 2018 DU:GU = 4:1 DUs common in men 3:1 Risk factors: male sex family history smoking (cause and delayed healing) stress more common in blood group O NSAIDs 2–4 times increase in GU and ulcer complications H. pylori Unproven risk factors: corticosteroids alcohol (except for gastric erosion) diet (does reduce recurrence of PU) Types of ulcers:

lower oesophageal gastric stomal (postgastric surgery) duodenal Note: If NSAIDs and H. pylori are not implicated, it is referred to as idiopathic (affects a small population group).

Clinical features Episodic burning epigastric pain related to meals (1–2 hours after) Relieved by food or antacids (generally) Dyspepsia common May be ‘silent’ in elderly on NSAIDs Physical examination often unhelpful

Investigations Endoscopy (investigation of choice):15 92% predictive value Barium studies: 54% predictive value Serum gastrin (consider if multiple ulcers) H. pylori test: serology or urea breath test; diagnosis usually based on urease test performed at endoscopy

Complications Perforation Bleeding → haematemesis and melaena Obstruction—pyloric stenosis Anaemia (blood loss) Cancer (in GU) Oesophageal stenosis

Bleeding peptic ulcer This can be treated with endoscopic haemostasis with electrocautery heater probe or injection of adrenaline or both. Also IV omeprazole 80 mg bolus, then 8 mg/hr IV infusion for 3 days. Surgery is an option. IV esomeprazole, omeprazole or pantoprazole can also be used.

Management of peptic ulcer disease Aims of treatment: relieve symptoms accelerate ulcer healing prevent complications minimise risk of relapse The treatment of a GU is similar to that for a DU except that GUs take about 2 weeks longer to heal and the increased risk of malignancy has to be considered. Stage 19 General measures: (lifestyle and symptom relief) same principles as for GORD stop smoking avoid irritant drugs: NSAIDs, aspirin normal diet but avoid foods that upset antacids If H. pylori positive—eradicate with combined therapy. Confirm eradication with a urea breath test (DU) or repeat gastroscopy (GU) and repeat if still present.10 If H. pylori negative—treat with full-dose PPI. Proton-pump inhibitors Proton-pump inhibitors (PPIs) provide more potent acid suppression and heal GUs and DUs more rapidly than H2-receptor antagonists. 4–8-week oral course PPIs are frequently used for longer than needed, with many people on long-term high

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doses which are unnecessary and potentially harmful (risk of C. difficile, osteoporotic fractures, pneumonia, nutritional deficiencies). Consider deprescribing for those without Barrett oesophagus, high-grade oesophagitis or GI bleeding. Long-term users may experience rebound symptoms upon cessation; reduce gradually and offer prn occasional use.16 Use with caution in: the elderly those on drugs, especially warfarin, anticonvulsants, beta blockers liver disease

Therapy to eradicate Helicobacter pylori17,18 This organism has a proven link with PU disease (both DU and benign non-drug induced GU), gastric cancer and MALToma (a gastric lymphoma) because of mucosal infection. This hypothesis is supported by a very low relapse of DU in subjects eradicated of H. pylori. Most infected people are asymptomatic but infection leads to a lifetime risk of peptic ulcer disease in 15–20% and of gastric cancer in up to 2%.12 Twenty per cent of people have a variety of symptoms including those from gastritis and duodenitis. Treatment is based on combination triple or quadruple therapy, which can achieve a successful eradication rate of 85–90%.

Drug treatment regimens (examples)9 First-line therapy: PPI (e.g. omeprazole or esomeprazole 20 mg) plus clarithromycin 500 mg plus amoxicillin 1 g All orally twice daily for 7 days; this is the preferred regimen (available as a combination pack) Note: A 10–14-day course improves eradication rate by approx. 5%.1 or PPI + clarithromycin + metronidazole 400 mg (twice daily for 7 days)—if hypersensitive to penicillin or

PPI + amoxicillin + levofloxacin (for salvage therapy) or other combinations: quadruple therapy, e.g. bismuth + PPI + tetracycline + metronidazole (for failed triple combination) Note: Resistance to metronidazole is common (>50%) and to clarithromycin is increasing (about 5% plus), but uncommon with tetracycline and amoxicillin.6 Antacids are good for daytime relief. Maintenance anti-secretory therapy is usually unnecessary for H. pylori ulcers after successful eradication.9 For children with confirmed H. pylori: PPI + amoxicillin + clarithromycin Surgical treatment Indications (now uncommon) include: failed medical treatment after 1 year complications: uncontrollable bleeding perforation pyloric stenosis suspicion of malignancy in GU

NSAIDs and peptic ulcers9,19 1. Ulcer identified in NSAID user: stop NSAID (if possible) check smoking and alcohol use try alternative anti-inflammatory analgesic: paracetamol enteric-coated, slow-release aspirin

corticosteroids intra-articular or oral PPI for 4 weeks (gives best results) Note: Healing time is doubled if NSAID continued.3 About 90% heal within 12 weeks. Check healing by endoscopy at 12 weeks. Do H. pylori test. 2. Prevention of ulcers in NSAID user:19 Primary prophylaxis is usually reserved for those at significantly increased risk, e.g. older persons (>75 years) and past history PU. Use one of the following PPIs:9 esomeprazole 20 mg bd for 7 days or omeprazole 20 mg daily or pantoprazole 40 mg daily Increased dietary fibre assists DU healing and prevention. Note: Do H. pylori test and, if present, it should be eradicated with combination therapy after the ulcer has healed, especially in people who continue to take NSAIDs.1

Autoimmune gastritis9 This is an inflammatory condition with antibodies to parietal cells and intrinsic factor. It is asymptomatic and may lead to pernicious anaemia. Diagnosis is confirmed by histology or endoscopy. H. pylori is absent. Treat with iron and vitamin B12 if they are low. Page 448

Stomach cancer This is the fourth most common cancer worldwide.

Clinical features Male to female ratio = 3:1 Usually asymptomatic early

Consider if upper GIT symptoms in patients over 40 years, especially weight loss Recent-onset dyspepsia in middle age Dyspepsia unresponsive to treatment Vague fullness or epigastric distension Anorexia, nausea ± vomiting Dysphagia—a late sign Onset of anaemia Changing dyspepsia in GU Changing symptoms in pernicious anaemia H. pylori is implicated as a cause. Its treatment reduces the risk1 and is recommended in highrisk groups Also implicated in gastric mucosa-associated lymphoid tissue (MALT) lymphoma Risk factors: ↑ age, blood group A, smoking, sugar, atrophic gastritis, diet—high in salted and smoked foods

Limited physical findings Palpable abdominal mass (20%) Signs (see FIG. 36.3

) in advanced cases

FIGURE 36.3 Late signs of stomach cancer DxT malaise + anorexia + dyspepsia + weight loss → stomach cancer

DxT triple loss of appetite + weight + colour → stomach cancer

Investigations Endoscopy and biopsy is optimal test Barium meal—false negatives

Treatment Surgical excision: may be curative if diagnosed early but overall survival is poor (22% at 5 years)

When to refer Infants with persistent gastro-oesophageal reflux not responding to simple measures Failure to respond to stage 1 therapy for heartburn, when endoscopy is required Patients with persistent or recurrent ulcers Any patient with a PU complication, such as haemorrhage, obstruction or perforation

Practice tips Scleroderma is a rare but important cause of oesophagitis. Advise patients never to ‘dry swallow’ medications. Persistent dysphagia always warrants investigation, not observation. Beware of attributing anaemia to oesophagitis. Epigastric pain aggravated by any food, relieved by antacids = chronic GU. Epigastric pain before meals, relieved by food = chronic DU. Keep in mind the malignant potential of a GU. A change in the nature of symptoms with a GU suggests the possibility of malignant change. Avoid the long-term use of water-soluble antacids. Investigate the alarm symptoms—dysphagia, bleeding, anaemia, weight loss, waking at night, pain radiating to the back.

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Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Barrett oesophagus Heartburn Hiatus hernia Peptic ulcer Reflux disease Reflux in infants

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RACGP Choosing Wisely panel. The Royal Australian College of General Practitioners Recommendations: 1. Don’t use proton pump inhibitors (PPIs) long term in patients with uncomplicated disease without regular attempts at reducing dose or ceasing. April 2005. Available from: https://www.choosingwisely.org.au/recommendations/racgp, accessed March 2021.

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Ford A et al. Eradication therapy for peptic ulcer disease in Helicobacter positive patients (Cochrane Review). Cochrane Database. Syst Rev, 2004; Issue 4: Art No. CD003840.

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Sugano K et al. Kyoto global consensus report on Helicobacter pylori gastritis. Gut, 2015; 64(9): 1353–67.

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Chan FK, To KF, Wu JC. Eradication of Helicobacter pylori and risk of peptic ulcers in patients starting long term treatment with NSAIDs: a randomised trial. Lancet, 2002; 359(9300): 9–13.

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37 Dysphagia

We swallow approximately 1200 times daily, largely subconsciously. While we take the fundamental function for granted, disordered swallowing can be a devastating condition, with substantial morbidity for those affected. IAN COOK 1996 Dysphagia is difficulty in swallowing. It is a common problem affecting up to 22% of patients at some point in the general practice setting.1 It is usually associated with a sensation of hold-up of the swallowed bolus and is sometimes accompanied by pain. Its origin is considered as either oropharyngeal or oesophageal. Oropharyngeal dysphagia is usually related to neuromuscular dysfunction and is commonly caused by stroke. Oesophageal dysphagia is usually due to motor disorders, such as achalasia or diffuse oesophageal spasm, and to peptic oesophageal strictures often secondary to reflux. In this type of dysphagia there is a sensation of a hold-up, which may be experienced in either the cervical or retrosternal region.1 Causes are usually classified as functional, mechanical and neurological (see TABLE 37.1 ). Table 37.1

Causes of dysphagia

Functional

Examples: muscle tension, ‘express swallowing’

Neurological

Examples: stroke, myasthenia, MND

Mechanical luminal mural extramural

Example: foreign body Example: stricture, tumour Example: extrinsic compression (i.e. goitre)

Dysphagia must not be confused with globus sensation, which is the sensation of the constant ‘lump in the throat’ although there is no actual difficulty swallowing food. If dysphagia is progressive or prolonged then urgent attention is necessary.

There are only a few common causes of dysphagia and these are usually readily diagnosed on the history and two or three investigations. A careful history is very important, including a drug history and psychosocial factors.

Diagnostic guidelines Any disease or abnormality affecting the tongue, pharynx or oesophagus can cause dysphagia. Patients experience a sensation of obstruction at a definite level with swallowing food or water; hence, it is convenient to subdivide dysphagia into oropharyngeal and oesophageal. Pain from the oropharynx is localised to the neck. Pain from the oesophagus is usually felt over the T2–6 area of the chest. Oropharyngeal causes: difficulty initiating swallowing; food sticks at the suprasternal notch level; regurgitation; aspiration. Oesophageal causes: food sticks to mid to lower sternal level; pain on swallowing solid foods, especially meat, potatoes and bread, and then eventually liquids. A pharyngeal pouch usually causes regurgitation of undigested food and gurgling may be audible over the side of the neck. Neurological disorders typically result in difficulty swallowing or coughing or choking due to food spillover, especially with liquids. Dysphagia for solids only indicates a structural lesion, such as a stricture or tumour. Dysphagia for liquids and solids is typical of an oesophageal motility disorder, namely achalasia.2 GORD tends to exclude achalasia. Scleroderma may lead to a peptic stricture. Gastroenterologists suggest the ‘big three’ common causes referred for specialist investigation are benign peptic stricture, cancer and achalasia.3 Intermittent dysphagia for both liquids and solids is characteristic of a motility disorder such as oesophageal achalasia. Malignant oesophageal obstruction is usually evident when there is a short history of rapidly Page 451 progressive dysphagia and significant weight loss.4

Red flag pointers for dysphagia

Age >50 years Recent or sudden onset Unexplained weight loss Painful swallowing Progressive dysphagia Dysphagia for solids Hiccoughs Hoarseness Neurological symptoms/signs

A summary of the diagnostic strategy model is presented in TABLE 37.2 Table 37.2

.

Dysphagia: diagnostic strategy model (excluding oropharyngeal infections and strokes)

Probability diagnosis Functional (e.g. ‘express’ swallowing, psychogenic) Tablet-induced irritation Pharyngotonsillitis GORD/reflux oesophagitis Serious disorders not to be missed Neoplasia: cancer of the pharynx, oesophagus, stomach extrinsic tumour AIDS (opportunistic oesophageal infection in immunocompromised, also candidiasis, herpes and viral oesophagitis) Stricture, usually benign peptic stricture Oesophageal food bolus obstruction Scleroderma Neurological causes: pseudobulbar palsy multiple sclerosis/myasthenia gravis motor neurone disease (amyotrophic sclerosis)

Parkinson disease Pitfalls (often missed) Foreign body Drugs (e.g. phenothiazines, bisphosphonates) Subacute thyroiditis Extrinsic lesions (e.g. lymph nodes, goitre) Upper oesophageal web (e.g. Plummer–Vinson syndrome) Diffuse oesophageal spasm Eosinophilic oesophagitis Radiotherapy Achalasia Upper oesophageal spasm (mimics angina) Rarities (some): Sjögren syndrome aortic aneurysm aberrant right subclavian artery lead poisoning cervical osteoarthritis (large osteophytes) other neurological causes other mechanical causes Seven masquerades checklist Depression Drugs Thyroid disorder Is the patient trying to tell me something? Yes. Could be functional. ?Globus sensation.

Examination It is worthwhile focusing on the following features: general examination including hands and skin ?scleroderma assess nutritional status including BMI mouth, pharynx, larynx (look for paralysis) ?dentition neck, especially for lymph nodes and thyroid

neurological, especially cranial nerve function and muscle weakness disorders or evidence of stroke special oesophageal obstruction test: hand the patient a glass of water and place a stethoscope over the left upper quadrant of abdomen measure time between swallowing and murmur produced by bolus passing the cardia (normal: 7–10 seconds) assess aspiration risk, e.g. through having a sip of water1

Investigations Full blood examination: ?anaemia Neurological cause: oesophageal motility study (manometry) Mechanical: extrinsic compression (e.g. barium swallow, CT scan, chest X-ray) intrinsic (e.g. endoscopy ± barium swallow) PET scan: good for identifying oesophageal cancer and gastro-oesophageal function The primary investigation in suspected pharyngeal dysphagia is a video barium swallow,5 while endoscopy is generally the first investigation in cases of suspected oesophageal dysphagia. Barium swallow should precede endoscopy in the latter when there is a suspected oesophageal ‘ring’ and suspected oesophageal dysmotility. If endoscopy and radiology are negative, consider oesophageal motility studies to look specifically for achalasia or other less common motility disorders. Page 452

Specific conditions Benign peptic stricture Fibrous stricture of lower third oesophagus (can be higher) Follows years of reflux oesophagitis Usually older people Dysphagia with solid food

Diagnosis confirmed by endoscopy and barium swallow

Treatment Dilate the stricture Treat reflux vigorously

Oesophageal cancer Dysphagia at beginning of meal Progressive dysphagia for solid food steadily progressive over weeks Can remain silent and tends to be invasive when diagnosed Hiccoughs may be an early sign Hoarseness and cough (upper third) Discomfort or pain—throat, retrosternal, interscapular Weight loss can be striking Associations: GORD, tobacco, Barrett oesophagus Diagnosis confirmed by barium swallow and endoscopy Both SCC upper third (commonest) and adenocarcinoma, distal third Adenocarcinoma associated with Barrett mucosa Plummer–Vinson syndrome Treatment is usually palliative surgery DxT fatigue + progressive dysphagia + weight loss → oesophageal cancer

Barrett oesophagus See CHAPTER 36

.

Achalasia6 A disorder of oesophageal motility

Widely dilated oesophagus Empties poorly through a smoothly tapered lower end Gradual onset of dysphagia for both liquids and solids Fluctuating symptoms—dysphagia, regurgitation Chest discomfort Diagnosis confirmed by barium swallow or manometry Manometry is the only way to diagnose with certainty1

Treatment Conservative in the elderly (e.g. nifedipine/or endoscopic botulinum toxin injection into the sphincter) Pneumatic dilatation of lower oesophageal sphincter or surgical myotomy Note: Prokinetic drugs have no place in treatment.

Drug-induced oesophageal injury3 Tetracycline, especially doxycycline, can cause painful ulceration in all age groups. Delayed passage of some drugs (due to pre-existing disorders) can cause local ulceration, even perforation (especially in the elderly) (e.g. iron tablets, slow-release potassium, aspirin, NSAIDs, bisphosphonates, zidovudine, antibiotics). The elderly are prone to the problem if they ingest drugs upon retiring to bed with insufficient liquid washdown.

Management Stop drugs or swallow them upright with a glass of water Take antacids

Globus sensation (cricopharyngeal spasm) Also referred to as ‘globus hystericus’ or ‘lump in the throat’, it is the subjective sensation of a lump in the throat. It appears to be associated with psychological stress (e.g. unresolved hurt, grief, non-achievement). Suppression of sadness is most often implicated.7 No specific aetiology or physiological mechanism has been established. The symptom can be associated with GORD, from frequent swallowing or an emotionally based dry throat.

Clinical features Sensation of being ‘choked up’ or ‘something stuck’ or lump—a very real sensation Not affected by swallowing Eating and drinking may provide relief Normal investigations Page 453

Approach to patient Careful history and examination Exclude organic cause (refer TABLE 37.2

)

May require investigations if doubtful diagnosis

Management Usually settles with education, reassuring support and time (up to several months) Avoid swallowing very hot drinks No drug of proven value Treat any underlying psychological disorder

Odynophagia6 Pain on swallowing is basically caused by irritation of an inflamed or ulcerated (in particular) mucosa by the swallowed food bolus, usually meat, which may impact. Food bolus impaction may be very serious. If drinking water or, better still, 25–50 mL of a carbonated drink is ineffective, urgent upper GIT endoscopy may be required. Important causes include: GORD (the commonest cause) with associated oesophagitis oesophageal spasm, especially distal oesophagus oesophageal candidiasis, especially in the immunosuppressed herpes simplex oesophagitis, in the immunosuppressed cytomegalovirus oesophagitis, in the immunosuppressed

pill-induced oesophagitis/ulceration oesophageal cancer achalasia

Infective oesophagitis Common infective causes—candida species, herpes simplex virus (HSV), cytomegalovirus. These are more prone to occur in the immunocompromised. Present with odynophagia and/or dysphagia. Diagnosis is by upper GI endoscopy and biopsy.

Oesophageal candidiasis Treat with nystatin 100 000 units/mL suspension, 1 mL (o) 6 hrly for 10–14 weeks. If poor response, fluconazole (o), or IV if not tolerated orally. If no response to fluconazole, itraconazole (o). Refer to therapeutic guidelines.

Herpes simplex Treat with aciclovir IV until oral therapy possible, then famciclovir or valaciclovir.

Eosinophilic oesophagitis6,8,9 Eosinophilic oesophagitis is increasingly being recognised as a cause of dysphagia, gastrooesophageal reflux and acute food bolus obstruction in both children (particularly) and adults. It may present as infant colic.9 It should be considered in those who regularly experience food getting stuck in their throat. It is associated with allergic disorders such as hay fever, cow’s milk allergy and asthma. The IgE is elevated. Refer to gastroscopy, which may show eosinophilic infiltrates in the oesophagus on mucosal biopsies. However, symptoms usually resolve within 72 hours of eliminating the offending food. A six-food elimination diet (cow’s milk protein, wheat, soy, eggs, seafood and peanuts) has been shown to reduce symptoms in up to 90%.1 Treatment of the acute attack includes IM buscopan and a swallowed topical corticosteroid aerosol, e.g. fluticasone twice daily for 8 weeks.6

Practice tips Although dysphagia is a common psychogenic symptom, it must always be taken seriously and investigated.

Mechanical dysphagia represents cancer until proved otherwise. Progressive dysphagia and weight loss in an elderly patient is oesophageal cancer until proved otherwise. Oesophageal cancer usually causes pain, wasting and regurgitation. Globus sensation or hystericus, an anxiety disorder, should not be confused with dysphagia. It is the subjective sensation of a lump or mass in the throat. Particularly seen in young women. Cancer-induced achalasia occurs with tumours at the gastro-oesophageal junction usually due to adenocarcinoma of the stomach. Severe oesophageal reflux predisposes to adenocarcinoma. Oesophageal strictures can be benign, usually secondary to chronic reflux oesophagitis, or due to malignancy. Be careful of a change of symptoms in the presence of longstanding reflux. Consider stricture or cancer. A prominent hard lymph node in the left supraclavicular fossa (Troisier sign) is suggestive of cancer of the stomach. Dysphagia can be caused by a tight fundoplication and can be diagnosed by manometry or barium swallow.1

Page 454

Dietary adjustments (dysphagia diets) 1. Thin liquids, e.g. fruit juice, coffee, tea 2. Nectar-thick liquids, e.g. creamy soup, tomato juice 3. Honey-consistency diet (honey-thick liquids) 4. Pudding-thick foods, e.g. mashed bananas, cooked cereals, purees 5. Mechanical soft foods, e.g. meat loaf, baked beans, casseroles 6. Chewy foods, e.g. pizza, cheese, bagels 7. Foods that fall apart, e.g. bread, rice, muffins Consider nutritional methods such as nasogastric feeding and gastrostomy.

References 1

Kuo P, Holloway R. Dysphagia: how to treat. Australian Doctor, 8 February 2013: 21–8.

2

Trate DM, Parkman HP, Fisher RS. Dysphagia: evaluation, diagnosis and treatment. Primary Care, 1996; 23: 417–32.

3

Breen K. A practical approach to patients with dysphagia or pain on swallowing. Modern Medicine Australia, 1992; 3: 50–6.

4

Abeygunasekera S. Difficult and painful swallowing: a guide for GPs. Medicine Today, 2003; 4(10): 33–40.

5

Roman S, Kahrilas PJ. Management of spastic disorders of the esophagus. Gastroenterol Clin North Am, 2013; 42(1): 27–43.

6

Disorders of the oesophagus [published 2016]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2016. www.tg.org.au, accessed October 2019.

7

Porter RS, Kaplan JL, eds. The Merck Manual (19th edn). NJ: Merck, Sharp & Dohme Corp, 2011: 78.

8

Kakakios A, Heine R. Eosinophilic oesophagitis. Med J Aust, 2006; 185(7): 401.

9

Thomson K, Tey D, Marks M. Paediatric Handbook (8th edn). Oxford: Wiley-Blackwell, 2009: 232.

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38 Dyspnoea

When man grows old … there is much gas within his thorax, resulting in panting and troubled breathing. HUANG TI (2697–2597 BCE), THE YELLOW EMPEROR’S CLASSIC OF INTERNAL MEDICINE Dyspnoea is the subjective sensation of breathlessness that is excessive for any given level of physical activity. It is a cardinal symptom affecting the cardiopulmonary system and can be very difficult to evaluate. Appropriate breathlessness following activities such as running to catch a bus or climbing several flights of stairs is not abnormal but may be excessive due to obesity or lack of fitness.

Key facts and checkpoints Determination of the underlying cause of dyspnoea in a given patient is absolutely essential for effective management. The main causes of dyspnoea are lung disease, heart disease, obesity and functional hyperventilation.1 The most common cause of dyspnoea encountered in family practice is airflow obstruction, which is the basic abnormality seen in chronic asthma and chronic obstructive pulmonary disease (COPD).2 Wheezing, which is a continuous musical or whistling noise, is an indication of airflow obstruction. Some patients with asthma do not wheeze and some patients who wheeze do not have asthma. Other important pulmonary causes include restrictive disease, such as fibrosis, collapse and pleural effusion. Dyspnoea is not inevitable in lung cancer but occurs in about 60% of cases.3

Normal respiratory rate is 12–16 breaths/minute.

Terminology It is important to emphasise that dyspnoea or breathlessness is a subjective sensation of the desire for increased respiratory effort and must be considered in relation to the patient’s lifestyle and individual tolerance of discomfort. It also depends on the age, physical fitness and physical expectations of the person. Patients may complain of tightness in the chest and this must be differentiated from angina. The American Thoracic Society guide for grades of dyspnoea is: Grade 0 No breathlessness except with strenuous exercise Grade 1 Breathlessness when hurrying on the level or walking up a slight hill Grade 2 Walks slower than people of the same age on the level because of breathlessness or has to stop for breath when walking at own pace on level Grade 3 Stops for breath after walking about 100 metres or a few minutes on the level Grade 4 Too breathless to leave the house or breathless when dressing or undressing

Glossary of terms Hyperpnoea An increased level of ventilation (e.g. during exertion). Hyperventilation Overbreathing. Orthopnoea Breathlessness lying down flat. Paroxysmal nocturnal dyspnoea Inappropriate breathlessness causing waking from sleep. Tachypnoea An increased rate of breathing.

Difference between heart and lung causes The distinguishing features between dyspnoea due to heart disease and to lung disease are presented in TABLE 38.1 . Page 456 Table 38.1

Comparison of distinguishing features between dyspnoea due to heart disease and lung disease

Lung disease

Heart disease

History of respiratory disease

History of hypertension, cardiac ischaemia or valvular heart disease

Slow development

Rapid development

Present at rest

Mainly on exertion

Productive cough common

Cough uncommon and then ‘dry’

Aggravated by respiratory infection

Usually unaffected by respiratory infection

Source: Adapted with permission from Stenton C. The MRC breathlessness scale. Occup Med (Lond). 2008; 58(3): 226–227. doi:10.1093/occmed/kqm162

The history is a good indication and a useful guideline is that dyspnoea at rest is typical of lung disease, especially asthma, while it tends to be present on effort with heart disease as well as with COPD.

Wheezing Wheezing is any continuous musical expiratory noise heard with the stethoscope or otherwise. Wheeze includes stridor, which is an inspiratory wheeze.

Common causes of wheezing Localised: partial bronchial obstruction: impacted foreign body impacted mucus plugs extrinsic compression tracheomalacia Generalised: asthma obstructive bronchitis bronchiolitis

‘Cardiac asthma’ and bronchial asthma The term ‘cardiac asthma’ is (somewhat confusingly) used to describe a wheezing sensation such as that experienced with paroxysmal nocturnal dyspnoea. Differentiating features are presented in TABLE 38.2 . Table 38.2

Comparison of distinguishing features between ‘cardiac asthma’ and bronchial asthma

Dyspnoea

Cardiac Mainly inspiratory

Bronchial Mainly expiratory

Cough

Follows dyspnoea

Precedes dyspnoea

Sputum

Pink and frothy

Thick and gelatinous

Relief

Standing up (by an open window) Intravenous diuretic/CPAP, morphine

Coughing up sputum Bronchodilator

Lung signs

Mainly crackles

Mainly wheezes

Is it asthma or COPD? This question is often asked, especially in the middle-aged or elderly person with dyspnoea. Differentiating features are presented in TABLE 38.3 . A mixed picture can occur, particularly as people with decades of asthma get older (esp. smokers). Table 38.3

Comparison of asthma and COPD

Symptoms 500 mL)

Towards opposite side (if massive)

Decreased (unilateral)

Stony dull

Absent or decreased

Absent or decreased

Pneumothorax (large)

Towards opposite side (if tension)

Decreased (unilateral)

Hyperresonant

Absent or decreased

Absent or decreased

Fibrosis (generalised)

Midline

Decreased (bilateral)

Normal

Vesicular

Increased

Bronchiectasis

Midline

Slight decrease

Resonant to dull

Bronchial

Normal or decreased

Careful inspection is mandatory. The patient should be stripped to the waist and observed for factors such as cyanosis, clubbing, pallor, mental alertness, dyspnoea at rest, use of accessory muscles, rib retraction and any other abnormalities of the chest wall. A coarse tremor or flap of the outstretched hands indicates carbon dioxide intoxication.6 To obtain maximum value from auscultation, request the patient to open their mouth and take deep breaths. Adventitious sounds that are not audible during tidal breathing may then be heard. Wheezes are high-pitched continuous sounds heard either in expiration or inspiration, being more pronounced in expiration. Crackles are short interrupted sounds heard mainly at the end of inspiration, resembling the crackling sound of hair being rubbed between the fingers near the ear. Fine crackles, previously referred to as crepitations, occur typically in lobar pneumonia and diffuse interstitial fibrosis, and are not cleared by coughing. Medium crackles are typical of congestive cardiac failure, while coarse crackles indicate airway mucus and usually clear on coughing. Page 460

Causes of pulmonary crackles Left ventricular failure Idiopathic pulmonary fibrosis Extrinsic allergic alveolitis Pneumonia Bronchiectasis Chronic bronchitis

Asbestosis Pulmonary fibrosis

Investigations The two most important initial investigations for respiratory disease are chest X-ray and pulmonary function tests.

Pulmonary function tests (PFTs) These relatively simple tests provide considerable information. Peak expiratory flow rate The most practical instrument for office use to detect chronic airway obstruction due to asthma or chronic bronchitis is the mini peak flow meter, which measures peak expiratory flow rate (PEFR). However, it gives considerably less information than spirometry. The interpretation of the tests, which vary according to sex, age and height, requires charts of predicted normal values. A chart for PEFR in normal adult subjects is presented in Appendix V. The value for a particular patient should be the best of three results. Spirometry Spirometry is the gold standard test, and increasingly available in general practice. The measurement of the forced vital capacity (FVC) and the forced expiratory volume in one second (FEV1) provide a very useful guide to the type of ventilatory deficit. Both the FVC and the FEV1 are related to sex, age and height. The FEV1 expressed as a percentage of the FVC is an excellent measure of airflow limitation. In normal subjects it is approximately 70%. A normal spirometry pattern is shown in FIGURE 38.1 and abnormal patterns in FIGURE 38.2 . FIGURE 38.3 summarises the relative values for these conditions.

FIGURE 38.1 Spirometry patterns showing normal flow volume loop

FIGURE 38.2 Maximum expiratory and inspiratory flow volume curves with examples of relative changes of patterns

FIGURE 38.3 Spirograms Lung volume Tidal volume (TV) and vital capacity (VC) can be measured by a simple spirometer but the total lung capacity and the residual volume are measured by the helium dilution method in a respiratory laboratory (rarely required). Page 461 Diffusing capacity (gas transfer factor) This test measures the carbon monoxide uptake by a single breath analysis for whole lungs. In normal lungs the transfer factor is a true measure of the diffusing capacity of the lungs for oxygen and depends on the thickness of the alveolar-capillary membrane.5 Gas transfer is usually reduced in patients with severe degrees of emphysema and fibrosis, anaemia and congestive cardiac failure. Useful for COPD and interstitial lung disease. Normal in asthma. Pulse oximetry An outstanding monitoring aid is transcutaneous pulse oximetry, which estimates oxygen saturation (SpO2) of capillary blood. The estimates are generally very accurate and correlate to within 5% of measured arterial O2 saturation (SaO2).7 The ideal level is 97–100%; median levels —neonates 97%, children 98%, adults 98%. 300 mL fluid in pleural space Can be detected clinically if >500 mL fluid Can be subpulmonary—simulates a raised diaphragm May be asymptomatic Dyspnoea common with large effusion Chest pain in setting of pleuritis, infection or trauma Signs: refer TABLE 38.6 The fluid may be transudate or exudate (diagnosed by aspirate) If bloodstained—malignancy, pulmonary infarction, TB

Transudate Protein content 200 IU/L Causes Infection—bacterial pneumonia, pleurisy, empyema, TB, viral Malignancy—bronchial carcinoma, mesothelioma, metastatic Pulmonary infarction

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Connective tissue diseases (e.g. SLE, RA) Acute pancreatitis Lymphoma Sarcoidosis HIV with parasitic pneumonia

Management Aspiration if symptomatic: may require repeats and pleurodesis. Treat the underlying cause.

Dyspnoea in children There are numerous causes of dyspnoea in children but the common causes are asthma, bronchiolitis and pulmonary infections. The important infections that can be fatal—croup, epiglottitis and myocarditis—must be kept in mind and intensively managed. Bronchiolitis is an important cause of respiratory distress in infants under 6–12 months. It should not be confused with asthma (refer to CHAPTER 89 ) and does not respond to salbutamol or corticosteroids. Sudden breathlessness or stridor may be due to an inhaled foreign body. Signs of lobar collapse may be present but physical examination may be of little help and a chest X-ray is essential. Cardiovascular disorders, including congenital heart disease, can cause dyspnoea. Extra respiratory causes include anaemia, acidosis, aspiration, poisoning and hyperventilation.

Dyspnoea in the elderly Dyspnoea in the elderly is common and is caused usually by heart failure and COPD. Other associations with ageing include lung cancer, pulmonary fibrosis and drugs. The classic problem of the aged is acute heart failure that develops typically in the early morning hours. The acute brain syndrome is a common presentation of all these disorders.

Respiratory disease in the elderly The respiratory system, like most other bodily systems, matures until about the age of 25 years and subsequently slowly loses efficiency due to a variety of factors such as disease, smoking, pollution and ageing. There is a decline in lung function and gas exchange, and decreased ventilatory responses to hypoxia and hypercapnia.

Heart failure

Heart failure occurs when the heart is unable to maintain sufficient cardiac output to meet the demands of the body. Dyspnoea is a common early symptom as pulmonary congestion causes hypoxia (increased ventilation) and decreased compliance (increased work). The incidence of congestive cardiac failure (CCF) has been increasing steeply, partly due to the ageing population.

Symptoms Increasing dyspnoea progressing to (in order): fatigue, especially exertional fatigue paroxysmal nocturnal dyspnoea weight change: gain or loss It is convenient to divide heart failure into left and right heart failure but they rarely occur in isolation and often occur simultaneously. Right failure is invariably secondary to left failure. The distinction between systolic and diastolic dysfunction is increasingly being replaced by the related concept of heart failure with reduced or preserved ejection fraction (HFrEF and HFpEF). Both present in the same way clinically; diagnosis requires echocardiography and sometimes other tests such as BNP. This permits an accurate diagnosis and prognosis, and helps guide treatment. Refer to Chronic heart failure (CHAPTER 76

).

Chronic obstructive pulmonary disease Chronic bronchitis and emphysema should be considered together as both these conditions usually coexist to some degree in each patient. An alternative, and preferable, term—chronic obstructive pulmonary disease (COPD)—is used to cover chronic bronchitis and emphysema with chronic airflow limitation.6 For more detail on the management of COPD refer to CHAPTER 74

.

Interstitial lung diseases (ILD) Interstitial lung diseases comprise a group of disorders that have the common features of inflammation (pneumonitis) and fibrosis of the interalveolar septum, representing a non-specific reaction of the lung to injury of various causes.8,9 In many there is a hypersensitivity reaction to various unusual antigens. The fibrosis may be localised as following unresolved pneumonia, bilateral as with tuberculosis or widespread. Page 463

Consider the possibility of fibrosis of the lungs in chronic dyspnoea and a dry cough

with normal resonance. If ILD is suspected, referral to a specialist physician for diagnosis is advisable. Causes of widespread interstitial pulmonary fibrosis include: idiopathic pulmonary fibrosis hypersensitivity pneumonitis (extrinsic allergic alveolitis) drug-induced lymphangitis carcinomatosis various occupational lung disorders (pneumoconiosis) sarcoidosis acute pulmonary oedema immunological/multisystemic disease (e.g. connective tissue disorders, rheumatoid arthritis, vasculitis, inflammatory bowel disease) Common clinical features: dyspnoea and dry cough (insidious onset) fine inspiratory crackles at lung base with faint breath sounds cyanosis and finger clubbing may be present PFTs: restrictive ventilatory deficit decrease in gas transfer factor characteristic X-ray changes High-resolution CT scanning has been a major advance in diagnosis. May show ‘honeycomb lung’.

Idiopathic pulmonary fibrosis Idiopathic pulmonary fibrosis, also known as idiopathic fibrosing interstitial pneumonia and cryptogenic fibrosing alveolitis, is the most common diagnosis among those presenting with interstitial lung disease. People usually present in the fifth to seventh decade with the clinical features as outlined under interstitial lung diseases, such as slowly progressive dyspnoea over months to years. Chest X-ray

abnormalities are variable but include bilateral diffuse nodular or reticulonodular shadowing favouring the lung bases. High-resolution CT scans are effective for diagnosis. Open lung biopsy may be needed for diagnosis and staging. The usual prognosis is poor, with death occurring about 3.5–5 years after diagnosis. The usual treatment is high doses of oral corticosteroids with azathioprine and no smoking.9,10 If refractory, refer to a palliative care service.11

Pulmonary sarcoidosis Sarcoidosis is a multisystemic disorder of unknown aetiology, which is characterised by noncaseating granulomatous inflammation that involves the lung in about 90% of affected patients. A characteristic feature is bilateral hilar lymphadenopathy, which is often symptomless and detected on routine chest X-ray (CXR). Radiological lung involvement can be associated with or occur independently of hilar lymphadenopathy.

Clinical features8,9 May be asymptomatic (one-third) Onset usually third or fourth decade (but any age) Bilateral hilar lymphadenopathy (on CXR) Cough Fever, malaise, arthralgia Skin lesions: erythema nodosum, lupus pernio Ocular lesions (e.g. anterior uveitis) Other multiple organ lesions (uncommon) Overall mortality 2–5% Erythema nodosum with an acute swinging fever, malaise and arthralgia in a young adult female is diagnostic of sarcoidosis.

Diagnosis Histological evidence from biopsy specimen, usually transbronchial biopsy (essential if an alternative diagnosis, e.g. lymphoma, cannot be excluded) or skin biopsy in cases of erythema nodosum. A better modern diagnostic method is biopsy via video-assisted thoracoscopy. Supporting evidence:

elevated serum ACE (non-specific) PFTs: restrictive pattern; impaired gas transfer in advanced cases +tive Kveim test (not recommended these days) serum calcium

Treatment Sarcoidosis may resolve spontaneously (hilar lymphadenopathy without lung involvement does not require treatment). Indications for treatment with corticosteroids: no spontaneous improvement or worsening after 3–6 months symptomatic pulmonary lesions eye, CNS and other systems involvement hypercalcaemia, hypercalciuria erythema nodosum with arthralgia persistent cough

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Glossary of terms Chronic airflow limitation A physiological process measured as impairment of forced expiratory flow, which is the major cause of dyspnoea in these patients. Chronic bronchitis A clinical condition characterised by a productive cough on most days for at least 3 months of the year for at least 2 consecutive years in the absence of any other respiratory disease that could be responsible for such excessive sputum production (such as tuberculosis or bronchiectasis). COPD A chronic, slowly progressive disorder characterised by the presence of airway obstruction, which may (or may not) be partially reversible by bronchodilator therapy.8 Emphysema This is defined in pathological rather than clinical terms as permanent dilatation and destruction of lung tissue distal to the terminal bronchioles.

Corticosteroid treatment9

Prednisolone 0.5 mg/kg (up to 50 mg) (o) daily for 4–6 weeks, then reduce to lowest dose that maintains improvement.9 If there is a response, taper the dose to 10–15 mg (o) daily as a maintenance dose for 6–12 months.9 Prednisolone 20–30 mg for 2 weeks for erythema nodosum of sarcoidosis.

Hypersensitivity pneumonitis Hypersensitivity pneumonitis (extrinsic allergic alveolitis) is characterised by a widespread diffuse inflammatory reaction in both the small airways of the lung and the alveoli, due to the inhalation of allergens, which are usually spores of micro-organisms such as thermophilic actinomycetes in ‘farmer’s lung’ or (more commonly) avian protein from droppings or feathers in ‘bird fancier’s lung’. Occupational causes of extrinsic alveolitis have been described by Molina12 (see TABLE 38.7 ). Table 38.7

Various causes of hypersensitivity pneumonitis (extrinsic allergic alveolitis)

Occupation/disease Farmer’s lung

Source of antigen Mouldy hay, grain and straw

Bagassosis

Mouldy sugar cane fibre (bagasse)

Bird fancier’s lung

Avian proteins: dropping dust (e.g. from pigeons); ‘bloom’ on budgerigar feathers

Mushroom worker’s lung

Mushroom compost

Cheese washer’s lung

Moulds or mites on cheese

Wheat weevil lung

Infested wheat flour (insect)

Ventilator pneumonitis

Humidified hot air system Air-conditioning system

Wood pulp worker’s disorder

Contaminated wood dust

Detergent worker’s disorder

Proteolytic enzymes

Suberosis

Mouldy cork bark

Rat handler’s lung

Rat urine and serum

Malt worker’s lung

Mouldy barley

Coffee worker’s lung

Coffee dust

Sisal worker’s lung

Sisal dust

Sericultural workers

Silkworms

Furrier’s lung

Fur dust

Sausage workers

Dust

Prawn workers

Prawn fumes

Illness may present as acute or subacute episodes of pyrexia, chills and malaise with dyspnoea and a peripheral neutrophil several hours after exposure.12 Management is based on prevention, namely avoiding exposure to allergens or wearing protective, fine-mesh masks. Prednisolone can be used (with caution) to control acute symptoms. Note that this allergic disorder is different from the infection psittacosis.

Drug-induced interstitial lung disease9 Drugs are an important cause of this disorder and have three main effects: 1. Alveolitis with or without pulmonary fibrosis. This is mainly due to cytotoxic drugs, nitrofurantoin and amiodarone. The drug should be removed and consideration given to prescribing prednisolone 50 mg (o) daily for several weeks, depending on response. 2. Eosinophilic reactions. This is presumably an immunological reaction, which may present as wheezing, dyspnoea, a maculopapular rash and pyrexia. The many implicated drugs include various antibiotics, NSAIDs, cytotoxic agents, major tranquillisers and antidepressants, and anti-epileptics. Treatment is drug removal and a short course of prednisolone 20–40 mg (o) daily for 2 weeks. Page 465 3. Non-cardiogenic acute pulmonary oedema. This is rare and has been reported to occur with opioids, aspirin, hydrochlorothiazide, β2-adrenoceptor agonists (given IV to suppress premature labour), cytotoxics, interleukin-2, heroin.

Occupational pulmonary disease Various types of acute and chronic pulmonary diseases are related to exposure to noxious substances such as dusts, gases and vapours in the workplace. Common chemical causes include formaldehyde used in processed woods, e.g. chipboard and medium-density fibre. GPs have a crucial role in the identification of the possible work-relatedness of lung disease. Disorders due to chemical agents include: obstructive airways disorders, such as occupational asthma, acute bronchitis, (chronic) industrial bronchitis, byssinosis (asthma-like condition due to cotton dust) hypersensitivity pneumonitis

pulmonary fibrosis (pneumoconiosis) due to mineral dust lung cancer due to industrial agents such as asbestos, various hydrocarbons pleural disorders, usually associated with asbestosis

Pneumoconiosis The term ‘pneumoconiosis’ refers to the accumulation of dust in the lungs and the reaction of tissue to its presence, namely chronic fibrosis. The main cause worldwide is inhalation of coal dust, a specific severe variety being progressive massive fibrosis (complicated coal worker’s pneumoconiosis) in which the patient suffers severe dyspnoea of effort and a cough often productive of black sputum. TABLE 38.8 summarises the important causes. Table 38.8

Selected pneumoconioses

Fibrotic lung disease Coal dust

Agent

Typical occupations

Coal worker’s pneumoconiosis

Coal dust

Coal mining

Metallic iron or iron oxide

Mining

Metal dust Siderosis

Welding Foundry work Inorganic dusts Silicosis

Silica (silicon dioxide)

Quarrying Demolition Rock mining Stone masons Sandblasting

Silicate dusts Asbestosis

Asbestos

Mining Shipbuilding Insulation Power stations Wharf labouring

Of particular concern are diseases caused by inhalation of fibres of asbestos, which is a mixture of silicates of iron, magnesium, cadmium, nickel and aluminium. These diseases include asbestosis, diffuse pleural thickening, pleural plaques, mesothelioma and increased bronchial carcinoma in smokers. Pulmonary asbestosis has classic X-ray changes but high-resolution CT scans may be required to confirm the presence of calcified pleural plaques. It usually takes 10–20 years from exposure for asbestosis to develop and 20–40 years for mesothelioma to develop,8 while bronchial carcinoma is caused by the synergistic effects of asbestosis and cigarette smoking. Silicosis Silicosis, caused by the inhalation of very fibrogenic silica particles, is a constant concern for workers. Mild cases cause no or minimal symptoms, but those affected experience progressive dyspnoea, intense dry cough and weakness. Investigations include FBE, chest X-ray, CT scan, pulse oximetry and spirometry (restrictive ventilatory defect). Management: avoid further exposure, wear special protective masks and check for associated tuberculosis.

Acute respiratory distress syndrome (ARDS) ARDS, also known as acute lung injury and formerly called ‘adult respiratory distress syndrome’, refers to acute hypoxaemic respiratory failure following a pulmonary or systemic insult with no apparent cardiogenic cause of pulmonary oedema. This occurs about 12–48 hours after the event.13 The most common cause is sepsis, which accounts for about one-third of ARDS patients. The mortality rate is 30–40%, increasing if accompanied by sepsis. Management is based on early diagnosis, early referral, identification and treatment of the underlying condition Page 466 and then optimal intensive care.14 Clinical features Sudden onset of respiratory distress Stiff lungs—reduced lung compliance Refractory hypoxaemia Bilateral pulmonary infiltrates on X-ray No apparent evidence of congestive heart failure Absence of elevated left atrial pressure Specific gas exchange abnormalities

Signs: tachypnoea, laboured breathing, rib retraction, central cyanosis, fine crackles on auscultation The differential diagnoses are pneumonia and acute heart failure. Common risk factors/associations for ARDS include (indirectly—systemic)—sepsis, shock, trauma, burns, drug overdose (e.g. heroin), multiple transfusions, obstetric complications (e.g. eclampsia, amniotic fluid embolism), and many direct causes such as pulmonary aspiration, toxic gas inhalation, blast injury and pneumonia (e.g. COVID-19, SARS). Admit to an intensive care unit.

Coronavirus infection and COVID-1915 The disease caused by the SARS-CoV-2 coronavirus was declared by WHO to be a pandemic in March 2020 (see CHAPTER 18 ). It has since proven to be the most deadly respiratory pandemic in a century.16 Widespread vaccination commenced around a year after the virus was first identified. The mortality rate is around 1–2% (around 10 times the seasonal influenza rate), with most fatalities in those aged over 50 years; deaths in children are very rare. Effective primary preventive measures at an individual level include: regular handwashing, social/physical distancing, avoiding large gatherings (particularly indoors), wearing face masks and coughing/sneezing etiquette. The majority of transmission is via asymptomatic (usually presymptomatic) individuals—hence the need for universal precautions (see CHAPTER 18 ).

Key features Most get mild symptoms similar to URTIs: mild fever, dry cough, sore throat, rhinorrhoea, malaise, headache, muscle pain. Diarrhoea and vomiting are common, and loss of taste/smell is notable. Dyspnoea (respiratory distress caused by pneumonia) increases with the severity of the illness, and is a cardinal feature in those requiring ICU admission. Extrapulmonary complications include septic shock, acute kidney injury (proteinuria is common), altered mental state and multiorgan failure. There is no effective, specific treatment for the virus; treatment is supportive. Long COVID: of those hospitalised, 70% report fatigue and half remain breathless 1–2 months post-discharge.17 Dyspnoea is usually due to resolving infection and deconditioning—but bear in mind the increased risk of lung fibrosis, pulmonary embolus, myocarditis, heart failure and rhythm disturbance.18 GPs should critically review ongoing symptoms, offer supportive treatment and investigate where necessary.

Practice tips Remember to order a chest X-ray and pulmonary function tests in all doubtful cases of dyspnoea. All heart diseases have dyspnoea as a common early symptom. Increasing dyspnoea on exertion may be the earliest symptom of incipient heart failure. Several drugs can produce a wide variety of respiratory disorders, particularly pulmonary fibrosis and pulmonary eosinophilia. Amiodarone and cytotoxic drugs, especially bleomycin, are the main causes. Dyspnoea in the presence of lung cancer may be caused by many factors, such as pleural effusion, lobar collapse, upper airway obstruction and lymphangitis carcinomatosis. The abrupt onset of severe dyspnoea suggests pneumothorax or pulmonary embolism. If a patient develops a relapse of dyspnoea while on digoxin therapy, consider the real possibility of digoxin toxicity and/or electrolyte abnormalities leading to left heart failure. Recurrent attacks of sudden dyspnoea, especially waking the patient at night, are suggestive of asthma or left heart failure. Causes of hyperventilation include drugs, asthma, thyrotoxicosis and panic attacks/anxiety.

Bronchial carcinoma Dyspnoea is associated with about 60% of cases of lung cancer3 (see CHAPTER 32 ). It is not a common early symptom unless bronchial occlusion causes extrinsic collapse. In advanced cancer, whether primary or secondary, direct spread or metastases may cause dyspnoea. Other factors include pleural effusion, lobar collapse, metastatic infiltration, upper airway obstruction due to superior vena cava (SVC) obstruction and lymphangitis carcinomatosis. A special problem arises with coexisting chronic bronchitis and emphysema. Page 467

When to refer

Patients with acute onset of severe dyspnoea All patients with heart failure resistant to initial therapy or where the diagnosis is in doubt Patients with pulmonary disease of uncertain aetiology, especially those requiring respiratory function tests Those in whom lung cancer is suspected

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Acute respiratory distress syndrome Asthma Asthma in children Chronic obstructive pulmonary disease Asthma: dangerous asthma Heart failure Silicosis

References 1

Stenton C. The MRC breathlessness scale. Occup Med (Lond), 2008; 58(3): 226–7.

2

Cormack J, Marinker M, Morrell D. Practice: A Handbook of Primary Health Care. London: Kluwer-Harrap Handbooks, 1980; 3(29): 3.

3

Walsh TD. Symptom Control. Boston: Blackwell Scientific Publications, 1989: 157–64.

4

Beck ER, Francis JL, Souhami RL. Tutorials in Differential Diagnosis (3rd edn). Edinburgh: Churchill Livingstone, 1993: 37.

5

Kelly DT. Cardiac failure. In: MIMS Disease Index (2nd edn). Sydney: IMS Publishing, 1996: 97–9.

6

Kumar PJ, Clark ML. Clinical Medicine (7th edn). London: Elsevier, 2009: 819–28.

7

Porter RS, Kaplan JL. The Merck Manual of Diagnosis and Therapy (19th edn). NJ: Merck Sharp & Dohme Corp., 2011: 1856.

8 9

Papadakis MA, McPhee SP. Current Medical Diagnosis and Treatment (52nd edn). New York: The McGraw-Hill Companies, 2013: 1602. Interstitial lung disease [updated 2020]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines; 2020. www.tg.org.au, accessed October 2019.

10

National Institute for Health and Care Excellence (NICE). Idiopathic pulmonary fibrosis [CG163]. London: NICE, 2013. Available from: www.nice.org.uk, accessed 29 May 2018.

11

Raghu G et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med, 2011; 183(6): 788–824.

12

Molina C. Occupational extrinsic allergic alveolitis. In: Pepys J, ed. Clinics in Immunology and Allergy. London: WB Saunders, 1984: 173–90.

13

Rameri VM et al. Acute respiratory distress syndrome. The ARDS Definition Task Force: the Berlin definition. JAMA, 2012, Jun 20; 307: 2526–33.

14

Ware LB et al. The acute respiratory distress syndrome. N Engl J Med, 2000; 342: 1334.

15

Cascella M et al. Features, evaluation, and treatment of coronavirus. [Updated 4 Oct 2020]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; January 2020. Available from: https://www.ncbi.nlm.nih.gov/books/NBK554776/, accessed March 2021.

16

Piper K. Here’s how Covid-19 ranks among the worst plagues in history. Vox, 11 January 2021. Available from: https://www.vox.com/future-perfect/21539483/covid-19-blackdeathplagues-in-history, accessed March 2021.

17

Williams F. Long COVID: who is at risk? The Conversation, 5 January 2021. Available from: https://theconversation.com/long-covid-who-is-at-risk-151797, accessed March 2021.

18

Greenhalgh T et al. Management of post-acute covid-19 in primary care. BMJ, 2020; 370: m3026.

Page 468

39 The painful ear

The ears should be kept perfectly clean; but it must never be done in company. It should never be done with a pin, and still less with the fingers, but always with an ear picker. ST JEAN BAPTISTE DE LA SALLE (1651–1719) Pain in the ear (otalgia) is a common symptom in general practice. It affects all ages, but is most prevalent in children, where otitis media is the commonest cause. Ear pain may be caused by disorders of the ear or may arise from other structures, and in many instances the precise diagnosis is difficult to make. Important causes of ear pain are summarised in TABLE 39.1 .1 Table 39.1

Causes of ear pain

1 Ear External ear: Perichondritis Otitis externa: – Candida albicans – Aspergillus nigra – Pseudomonas spp. – Staphylococcus aureus Furunculosis Trauma Neoplasia Herpes zoster (Ramsay–Hunt syndrome) Viral myringitis Wax-impacted Middle ear: Eustachian insufficiency Eustachian tube dysfunction

Barotrauma Acute otitis media Chronic otitis media and cholesteatoma Acute mastoiditis 2 Periotic cause Dental disorders, e.g. dental abscess; malocclusion Upper cervical spinal dysfunction TMJ arthralgia Parotitis Temporal arteritis Lymph node inflammation Other referred causes Pharyngeal disorders Tonsillitis Glossopharyngeal neuralgia

A patient with a painful ear often requests urgent attention, and calls in the middle of the night from anxious parents of a screaming child are commonplace. Infants may present with nothing except malaise, vomiting or screaming attacks.

Key facts and checkpoints Of patients presenting with earache, 77% can be expected to have acute otitis media and 12% otitis externa. Approximately 1 in every 25 patients in general practice will present with an earache. Two-thirds of children will sustain at least one episode of otitis media by their second birthday; 1 in 7 children will have had more than 6 episodes by this age. Peak prevalence is 9–15 months.2 Otitis media is unlikely to be present if the tympanic membrane (TM) is mobile. Pneumatic otoscopy greatly assists diagnosis since the most valuable sign of otitis media is absent or diminished motility of the TM. Bullous myringitis, which causes haemorrhagic blistering of the eardrum or external ear canal, is an uncommon cause of severe pain. It is caused by a virus, probably influenza.3 Consider herpes zoster.

The role of antibiotics (usually amoxicillin) is limited. Otitis externa can be distinguished from otitis media by pain on movement of the pinna.

A diagnostic approach The five self-posed questions can be answered using the diagnostic strategy model (see TABLE 39.2 ). Table 39.2

The painful ear: diagnostic strategy model

Probability diagnosis Otitis media (viral or bacterial) Otitis externa (fungal, viral or bacterial) TMJ arthralgia Eustachian tube dysfunction Serious disorders not to be missed Neoplasia of external ear Cancer of other sites (e.g. tongue, nasopharynx) Herpes zoster (Ramsay–Hunt syndrome) Acute mastoiditis Cholesteatoma Necrotising otitis externa Pitfalls (often missed) Foreign bodies in ear Hard ear wax Barotrauma Dental causes (e.g. abscess) Referred pain: neck, throat Unerupted wisdom tooth and other dental causes TMJ arthralgia Facial neuralgias, esp. glossopharyngeal Chondrodermatitis nodularis helicis Furuncles of canal or pinna Post tonsillectomy:

Page 469

from the wound from TMJ due to mouth gag Seven masquerades checklist Depression Spinal dysfunction (cervical) Is the patient trying to tell me something? Unlikely, but always possible with pain. More likely in children. Consider factitious pain.

Probability diagnosis The commonest cause of ear pain is acute otitis media. Chronic otitis media (often painless) and otitis externa are also common. In the tropics, ‘tropical ear’ due to acute bacterial otitis is a particular problem. Temporomandibular joint (TMJ) arthralgia, which may be acute or chronic, is also common and must be considered, especially when otitis media and otitis externa are excluded.

Serious disorders not to be missed As always, it is important not to overlook malignant diseases, especially the obscure ones, such as cancer of the tongue, palate or tonsils, which cause referred pain. Locally destructive cholesteatoma associated with chronic otitis media must be searched for. It signifies the ‘unsafe’ ear (see FIG. 39.1 ) that must be distinguished from the so-called ‘safe’ ear (see FIG. 39.2 ).

FIGURE 39.1 Infected ear: unsafe perforation

FIGURE 39.2 Infected ear: safe perforation Herpes zoster should be considered; it is easily missed if it does not erupt on the pinna and is confined to the ear canal (usually the posterior wall), and especially in the older person.

Pitfalls The medical aphorism ‘more things are missed by not looking than by not knowing’ applies particularly to the painful ear—good illumination and focusing of the auroscope are mandatory. Particular attention should be paid to the external canal—look for hard wax, otitis externa, furuncles and foreign objects such as insects. It may not be possible to visualise the TMs so consider cleaning the canal to permit this (if possible, on the first visit), particularly if there are any atypical presenting features. Otitis media may coexist with otitis externa. Barotrauma should be considered, especially if pain follows air travel or diving. Page 470

General pitfalls Failing to visualise the TM before diagnosis and treatment Not checking out possible referral sites such as the oropharynx and teeth Overlooking musculoskeletal causes such as TMJ arthralgia and cervical spondylosis Failing to recognise the unsafe ear

Red flag pointers for painful ear Offensive discharge >9 days

Downward displacement of pinna Swelling behind ear Neurological symptoms (e.g. headaches, drowsiness) Older person: unexplained, intractable ear pain Persistent fever

Seven masquerades checklist Of the conditions in the checklist, depression and dysfunction of the upper cervical spine have to be considered. Depressive illnesses should be considered in any patient complaining of chronic pain. Disorders of the upper cervical spine are an occasional overlooked cause of periotic pain. Pain from the C2 and C3 levels is referred to the posterior region of the ear.

Psychogenic considerations Such factors are unlikely, although pain in the periotic region can be magnified by a depressive state.

The clinical approach History In assessing the painful ear the relevant features are: site of pain and radiation details of the onset of pain nature of the pain aggravating or relieving factors, especially swimming associated features such as deafness, discharge, vertigo, tinnitus and irritation of the external ear, sore throat Agonising pain may be caused by perichondritis or furunculosis of the external ear and by the rare problem of herpes zoster (Ramsay–Hunt syndrome).3 Movement of the pinna markedly increases the pain of acute otitis externa and perichondritis, and movement of the jaw usually causes an exacerbation of TMJ arthralgia or severe otitis externa.

Key questions (especially children) Where is the pain? Is it in the ear, behind or below it? Is it in one ear or both ears? Have you noticed any other symptoms such as sore throat, fever or vomiting? Has anyone hit you over the ear? Has there been a discharge from the ear? Have you noticed any deafness? Are you allergic to penicillin? Have you been swimming in a spa, and where? Have you been in an aeroplane?

Examination The person’s general state and behaviour is observed during the history taking. Sudden, jabbing pain may indicate neuralgia, particularly glossopharyngeal neuralgia or a severe infection. The external ear is carefully inspected and the pinna manipulated to determine any tenderness. Palpate the face and neck and include the parotid glands, the regional lymph nodes, the mastoid process and the skin. Inspect the TMJs—tenderness from dysfunction typically lies immediately in front of the external auditory meatus. Palpate the TMJ over the lateral aspect at the joint disc. Ask the patient to open the mouth fully; tenderness is maximal. The TMJ can be palpated posteriorly by inserting the little finger into the external canal. Inspect both ear canals and TMs with the auroscope, using the largest earpiece that comfortably fits into the canal. Better visualisation of the TM can be achieved by pulling the pinna back and downwards in young children and up and back in older children—see FIGURE 39.3 for normal appearance. The diagnostic examination features of acute otitis media are illustrated in FIGS. 39.8 and 39.9 . Impacted wax does not often explain the otalgia. If herpes zoster involves the facial nerve, vesicles may be noted in and around the external auditory meatus (notably the posterior wall). Page 471

FIGURE 39.3 Normal right tympanic membrane If the diagnosis is still doubtful, look for causes of referred pain; inspect the cervical spine, the nose and postnasal space and the mouth, including the teeth (percuss molars with a tongue depressor), pharynx and larynx. Pharyngeal and mandibular causes of periotic pain are summarised in FIGURES 39.4 39.5 .

and

FIGURE 39.4 Pharyngeal causes of otalgia Source: Courtesy of Bruce Black

FIGURE 39.5 Mandibular causes of otalgia Source: Courtesy of Bruce Black

Inspect sites supplied by the nerves V2, IX, X, XI, C1, C2 and C3 to exclude other causes of referred pain.

Investigations Investigations are seldom necessary. Office-based hearing tests are useful, especially for children; use speech discrimination, hair rubbing and/or tuning fork tests. For potentially ongoing conditions such as chronic otitis media, refer for audiometry. Audiometry combined with tympanometry and physical measurement of the volume of the ear canal can be performed in children, irrespective of age. Swabs from discharge, especially to determine bacterial causes, such as Staphylococcus aureus or Pseudomonas spp. infection, may be necessary. However, swabs are of no value if the TM is intact. Radiology and CT/MRI scanning may be indicated for special conditions such as a suspected extraotic malignancy.

Ear pain in children Important causes of primary otalgia in children include otitis media (particularly acute), otitis

externa, external canal furuncle or abscess, chronic eczema with fissuring of the auricle, impacted wax, foreign body, barotrauma, perichondritis, mastoiditis and bullous myringitis. Secondary otalgia includes pharyngeal lesions, dental problems, gingivostomatitis, mumps and postauricular lymphadenopathy. Peritonsillar abscess (quinsy) may cause ear pain.

Foreign bodies Foreign bodies (FBs) are frequently inserted into the ear canal (see FIG. 39.6 ). They can usually be syringed out or lifted with thin forceps. Various improvised methods can be used to remove FBs in cooperative children. These include a probe to roll out the FB, a hooked needle or a rubber catheter used as a form of suction, or otherwise a fine sucker.4 Page 472

FIGURE 39.6 Foreign body (bead) in ear canal of a 3-year-old child, showing reactive tissue in ear canal

Probe method This requires good vision using a head mirror or head light and a thin probe. The probe is inserted under and just beyond the FB. Lever it in such a way that the tip of the probe ‘rolls’ the foreign body out of the obstructed passage. With practice, this can be done with the probe inserted through the middle of an auriscope whose lens is slid half-way open.

Rubber catheter suction method The only equipment required for this relatively simple and painless method is a straight rubber catheter (large type) and perhaps a suction pump. The end of the catheter is cut at right angles, a thin smear of petroleum jelly is applied to the rim and this end is applied to the FB. Suction is applied either orally or by a pump. Gentle pump suction is preferred but it is advisable to pinch closed the suction catheter until close to the FB as the hissing noise may frighten the child.

Insects in the ear Live insects should be immobilised by first instilling drops of vinegar, methylated spirits or olive oil, and then syringing the ear with warm water. Dead flies that have originally been attracted to pus are best removed by suction or gentle syringing. Note: If simple methods such as syringing fail to dislodge the FB, it is important to refer for examination and removal under microscopic vision. Syringing should not be performed if there is a possibility of the FB perforating the TM.

Otitis media in children Otitis media is very common in children and is the most common reason a child is brought in for medical attention. Persistent middle-ear effusions may follow and affect the language and cognitive development of young children. An abrupt onset is a feature.

Clinical features It is a clinical diagnosis Two peaks of incidence: 9–15 months of age, and school entry Seasonal incidence coincides with URTIs Bacteria cause two-thirds of cases5 The commonest organisms are Streptococcus pneumoniae, Haemophilus influenzae and Moraxella catarrhalis Fever, irritability, otalgia and otorrhoea may be present The main symptoms in older children are increasing earache and hearing loss Pulling at the ears is a common sign in infants Removal of wax may be necessary to visualise the TM (about 30% have occluded views), although with the decreasing role of antibiotics this visualisation becomes less crucial Visualisation of the tympanic membrane Use the largest ear speculum that will comfortably fit in the child’s ear. A good technique to enable the examination of the ears (also nose and throat) in a reluctant child is where the child is held against the parent’s chest while the parent’s arm embraces the child’s arm and trunk. Use of the pneumatic otoscope may reveal absent or limited movement.

Note the following features of the TM: translucency, colour, position and motility.

Treatment Provide adequate pain relief with paracetamol or a NSAID. Short-term use of topical 2% lignocaine drops is effective for severe cases. Many children with viral URTIs have mild reddening or dullness of the eardrum and antibiotics are not warranted, particularly in the absence of systemic features (fever and vomiting).5 Best practice in healthy, non-Indigenous children over 6 months of age is shared decision making with parents about symptomatic relief with analgesics and watchful waiting.6 The role of antibiotics in acute otitis media has diminished in response to emerging evidence from large double-blinded RCTs. The number needed to treat (NNT) with an antibiotic to prevent pain in one child at day 2–3 is 20, and no reduction in pain is found compared to placebo Page 473 at day 1 or day 7.6,7 The antibiotic of choice for children who fit into those categories is a 5-day course of:5 amoxicillin 15 mg/kg 8 or 12 hourly up to 500 mg (o) or amoxicillin 30 mg/kg 12 hourly (o) If β-lactamase-producing bacteria are suspected or documented, or initial treatment fails, use: amoxicillin/clavulanate 22.5+3.2 mg/kg 12 hourly

Possible clinical indications for antibiotics in children with painful otitis media5,6 Consider immediate and aggressive treatment: infants 3 months, arrange for a hearing assessment and consider referral to an ENT surgeon for possible tympanostomy tubes (grommets). However, bear in mind that the evidence of benefit for grommets is also modest—a minor improvement in hearing (around 10 dB) at 3–6 months that subsequently disappears as natural resolution catches up. Grommets have not been demonstrated to benefit speech, language or behaviour.13 A positive outcome from arranging hearing assessment may be altering classroom seating position and the use of sound amplifiers; these are frequently used in remote area Aboriginal and Torres Strait Islander schools, where rates of glue ear are very high.

Recurrent acute bacterial otitis media Antibiotic prevention of acute otitis media is indicated (arguably) if it occurs more often than every other month or for three or more episodes in 6 months or >4 in 12 months.14 Treat as for acute otitis media. Consider: chemoprophylaxis (for about 4 months) amoxicillin twice daily (first choice) or cefaclor twice daily Consider Pneumococcus vaccine in children over 18 months of age (if not already given) in combination with the antibiotic. Avoid smoke exposure (cigarettes and wood fires) and group child care. Consider review by ENT consultant.

Viral infections Most children with viral URTIs have mild–moderate reddening or dullness of the eardrum and antibiotics are not warranted. If painful bullous otitis media is present, either prick the bulla with a sterile needle for pain relief or instil dehydrating eardrops such as anhydrous glycerol.

Ear pain in adults and the elderly Causes of otalgia that mainly afflict the elderly include herpes zoster (Ramsay–Hunt syndrome), TMJ arthralgia, temporal arteritis and neoplasia. It is especially important to search for evidence of malignancy.

Acute otitis media Acute otitis media causes deep-seated ear pain, deafness and often systemic illness (see FIG. 39.8 ). The sequence of symptoms is a blocked ear feeling, pain and fever. Discharge may follow if the TM perforates, with relief of pain and fever.

FIGURE 39.8 Acute otitis media causing true otalgia. The ear drum bulges laterally due to pus in the middle ear. Perforation and otorrhoea imminent. The commonest organisms are viruses (adenovirus and enterovirus), and the bacteria H. influenzae, S. pneumoniae, Moraxella catarrhalis and β-haemolytic streptococci. The two cardinal features of diagnosis are inflammation and middle-ear effusion.

Appearance of the tympanic membrane (all ages) Translucency. If the middle-ear structures are clearly visible through the drum, otitis media is unlikely. Colour. The normal TM is a shiny pale-grey to brown: a yellow colour is suggestive of an effusion.

Diagnosis The main diagnostic feature is the redness of the TM. The inflammatory process usually begins in the upper posterior quadrant and spreads peripherally and down the handle of the malleus (see FIG. 39.9 ). The TM will be seen to be reddened and inflamed with engorgement of the vessels, particularly along the handle of the malleus. The loss of light reflex follows and anatomical features then become difficult to recognise as the TM becomes oedematous. Bulging of the drum is a late sign. Blisters are occasionally seen on the TM and this is thought to be due to a viral

infection in the epidermal layers of the drum.

Page 475

FIGURE 39.9 The appearances of the left tympanic membrane in the progressive development of acute otitis media

Treatment of acute otitis media (adults) Analgesics to relieve pain Adequate rest in a warm room Antibiotics for 5 days, repeated if necessary Treat associated conditions (e.g. adenoid hypertrophy) Follow-up: review and test hearing audiometrically Antibiotic treatment5 First choice: amoxicillin 750 mg (o) bd for 5 days5 or 500 mg (o) tds for 5 days A longer course (up to 10 days) may be required depending on severity and response to 5-day course. Alternatives: doxycycline 100 mg (o) bd for 5–7 days (daily for milder infections) or cefaclor 250 mg (o) tds for 5–7 days or (if resistance to amoxicillin is suspected or proven) amoxicillin/potassium clavulanate 500/125 mg (o) tds for 5 days (the most effective antibiotic) Consider surgical intervention for failed therapy. Page 476

Chronic otitis media There are two types of chronic suppurative otitis media and they both present with deafness and

discharge without pain. The discharge occurs through a perforation in the TM: one is safe (see FIG. 39.10A ), the other unsafe (see FIG. 39.10B ).

FIGURE 39.10 (a) Chronic otitis media with loss of the tympanic membrane; this is ‘safe ear’, (b) unsafe ear: chronic otitis media with attic cholesteatoma

Chronic discharging otitis media (safe)5,15 If aural discharge persists for >6 weeks after a course of antibiotics, treatment can be with topical

steroid and antibiotic combination drops, following ear toilet. The toileting can be done at home by dry mopping with a rolled tissue spear. If persistent, referral to exclude cholesteatoma or chronic osteitis is advisable.

Recognising the unsafe ear Examination of an infected ear should include inspection of the attic region, the small area of drum between the lateral process of the malleus, and the roof of the external auditory canal immediately above it. A perforation here renders the ear ‘unsafe’ (see FIG. 39.1 ); other perforations, not involving the drum margin (see FIG. 39.2 ), are regarded as ‘safe’.15

Cholesteatoma16 Refer CHAPTER 33

.

The status of a perforation depends on the presence of accumulated squamous epithelium (termed cholesteatoma) in the middle ear because this erodes bone. An attic perforation contains such material; safe perforations do not. Red flags for cholesteatoma include meningitis-type features, cranial nerve deficits, sensorineural hearing loss and persistent deep ear pain. Cholesteatoma is visible through the hole as white flakes, unless it is obscured by discharge or a persistent overlying scab (or wax). Either type of perforation can lead to chronic infective discharge, the nature of which varies with its origin. Mucus admixture is recognised by its stretch and recoil when this discharge is being cleaned from the external auditory canal. The types of discharge are compared in TABLE 39.3 . Table 39.3

Comparison of types of discharge

Source

Unsafe Cholesteatoma

Safe Mucosa

Odour

Foul

Inoffensive

Amount

Usually scant, never profuse

Can be profuse

Nature

Purulent

Mucopurulent

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Table 39.4

Ear discharge: diagnostic strategy model

Probability diagnosis Acute otitis media with perforation

Chronic suppurative otitis media Furuncle (boil) of ear canal Infected otitis externa Reactive skin conditions (e.g. eczema) Liquefied wax Serious disorders not to be missed Infections: Pseudomonas pyocyanea cholesteatoma herpes zoster oticus mastoiditis Cancer: malignancy with discharge (e.g. SCC) Other: cerebrospinal fluid otorrhoea (fractured temporal bone) necrotising otitis media Pitfalls (often missed) Foreign body with infection/liquidisation (e.g. insects) Trauma ± blood Rarities: keratitis obliterans branchial or salivary fistula Wegener granulomatosis Is the patient trying to tell me something? Factitious ?Excessive manipulation of ear canal Diagnostic tip Acute ear discharge is most likely due to otitis externa or perforated ear drum with otitis media

Management If an attic perforation is recognised or suspected, specialist referral is essential. Cholesteatoma cannot be eradicated by medical means: surgical removal is necessary to prevent a serious infratemporal or intracranial complication. Adjunct suction with care may be necessary to decompress the mass.

Diagnostic strategy for ear discharge (otorrhoea) mass Otitis externa15 Otitis externa (see FIG. 39.11 ), also known as ‘swimmer’s ear’, ‘surfer’s ear’ and ‘tropical ear’, is common in a country whose climate and coastal living leads to extensive water sports. It is more prevalent in hot humid conditions and therefore in the tropics.

FIGURE 39.11 Otitis externa Predisposing factors are allergic skin conditions, ear canal trauma, water penetration (swimming, humidity, showering), water and debris retention (wax, dermatitis, exostoses), foreign bodies, contamination from swimming water including spas, and use of Q tips and hearing aids.

Common responsible organisms Bacteria: Pseudomonas sp. Escherichia coli S. aureus Proteus sp. Klebsiella sp. Fungi: Candida albicans

Aspergillus sp.

Clinical features Itching at first Otalgia/pain (mild to intense) in 70% Fullness in ear canal Scant discharge Hearing loss

Signs Oedema (mild to extensive) Tenderness on moving auricle or jaw Erythema Discharge (offensive if coliform) Pale cream ‘wet blotting paper’ debris—C. albicans (see FIG. 39.12 Black spores of Aspergillus nigra TM granular or dull red

)

FIGURE 39.12 Acute otitis externa showing purulent discharge and narrowing of the ear canal Obtain culture, especially if resistant Pseudomonas sp. suspected, by using small ear swab. Note: ‘Malignant’ otitis externa occurs in diabetics due to Pseudomonas infection at base of skull.

Management Aural toilet Meticulous aural toilet by gentle suction and dry mopping with a wisp of cotton wool on a fine brooch under good lighting is the keystone of management. This enables topical medication to be applied directly to the skin. Page 478 Syringing This is appropriate in some cases but the canal must be dried meticulously afterwards. For most cases it is not recommended. Topical antimicrobials for acute diffuse otitis externa5,17 Most effective, especially when the canal is open, is an antibacterial, antifungal and corticosteroid preparation, e.g.: Kenacomb, Otodex or Sofradex drops (2–3 drops tds) or Locacorten-Vioform drops (2–3 drops bd) or Ciproxin HC (3 drops bd) Use all for 7 days. Be cautious of ear drops with neomycin (hypersensitivity). The tragus should be pumped for 30 seconds after instillation by pressing on it repeatedly, within the limitation of any pain. Other measures15 Strong analgesics are essential Antibiotics have a minimal place in treatment unless a spreading cellulitis has developed (refer if in doubt)

Prevent scratching and entry of water Use a wick soaked in combination steroid and antibiotic ointment for more severe cases Follow up ENT opinion for ‘red flags’ Dressings Dressings are recommended in moderate and severe otitis media. After cleaning and drying, insert a cotton ear wick (an alternative is 10–20 cm of 4 mm Nufold gauze—see FIG. 39.13 ) impregnated with a steroid and antibiotic cream. For severe oedematous otitis externa, a wick (e.g. Pope ear wick) is important and will reduce the oedema and pain in 12–24 hours (see FIG. 39.13 ). The wick can be soaked in an astringent (e.g. aluminium acetate 4% solution or glycerin and 10% ichthammol). The wick needs replacement daily until the swelling has subsided.

FIGURE 39.13 Insertion of a wick; it is packed gradually by short back-andforth movements of the forceps Source: Courtesy of Bruce Black

Practice tip for severe ‘tropical ear’ Prednisolone (o) 15 mg statim then 10 mg 8-hourly for six doses followed by: Merocel ear wick topical Kenacomb or Sofradex drops

Prevention Keep the ear dry, especially those involved in water sports Protect the ear with various waterproofing methods:

cotton wool coated with petroleum jelly an antiseptic drying agent (e.g. ethanol) after swimming and showering tailor-made ear plugs (e.g. EAR foam plugs) silicone putty or Blu-Tack a bathing cap pulled well forward allows these plugs to stay in situ Avoid poking objects such as hairpins and cotton buds in the ear to clean the canal If water enters, shake it out or use Aquaear drops (acetic acid/isopropyl alcohol), 4–5 drops to help dry the canal Page 479

Necrotising otitis externa This severe complication, usually due to Pseudomonas aeruginosa, can occur in the person who is elderly, immunocompromised or has diabetes. It involves cartilage and bone, and should be considered where there is treatment failure, severe persistent pain or fever and visible granulation tissue. Urgent referral is advisable.

Ear exostoses (‘surfer’s ear’) These periosteal bony overgrowths are usually caused by water retention in the ear. They are often multiple. They tend to trap keratin, wax and water, leading to infection.

Prevention Use plugs or Blu-Tack to waterproof ear. Dry thoroughly with hair dryer after swimming. They may require surgical removal.

Acute localised otitis externa (furunculosis) Furunculosis is a staphylococcal infection of the hair follicle in the outer cartilaginous part of the ear canal. It is usually intensely painful. Fever occurs only when the infection spreads in front of the ear as cellulitis. The pinna is tender on movement—a sign that is not a feature of acute otitis media. The furuncle (boil) may be seen in the external auditory meatus (see FIG. 39.14 ).

FIGURE 39.14 Furuncle (boil) in hair-bearing area at opening of the ear canal

Management If pointing, it can be incised after a local anaesthetic or freezing spray Warmth (e.g. use hot washcloth, hot-water bottle) If fever with cellulitis—flu/dicloxacillin or cephalexin

Perichondritis Perichondritis is infection of the cartilage of the ear characterised by severe pain of the pinna, which is red, swollen and exquisitely tender. It is rare and follows trauma or surgery to the ear. As the organism is frequently P. pyocyaneus, the appropriate antibiotics must be carefully chosen (e.g. ciprofloxacin).

Infected ear lobe In a pierced ear, the cause is most likely a contact allergy to nickel in an earring, complicated by a S. aureus infection.

Management Discard the earrings Clean the site to eliminate residual traces of nickel

Swab the site and then commence antibiotics (e.g. flucloxacillin or erythromycin) Instruct the person to clean the site daily, and then apply the appropriate ointment Use a ‘noble metal’ stud to keep the tract patent Advise the use of only gold, silver or platinum studs in future

Eustachian tube dysfunction This is a common cause of discomfort.17 Symptoms include fullness in the ear, pain of various levels and impairment of hearing. The most common causes of dysfunction are disorders causing oedema of the tubal lining, such as viral URTI and allergy when the tube is only partially blocked; swallowing and yawning may elicit a crackling or popping sound. Examination reveals retraction of the TM and decreased mobility on pneumatic otoscopy. The problem is usually transient after a viral URTI.

Treatment Systemic and intranasal decongestants (e.g. pseudoephedrine or corticosteroids in allergic patients) Autoinflation by forced exhalation against closed nostrils (avoid in active intranasal infection) Avoid air travel, rapid altitude change and underwater diving

Otic barotrauma Barotrauma is damage caused by undergoing rapid changes in atmospheric pressure in the presence of an occluded Eustachian tube (see FIG. 39.15 ). It affects scuba divers and aircraft travellers. Page 480

FIGURE 39.15 Mechanism of barotrauma, with blocking of the Eustachian

tube due to increased pressure at the sites indicated Source: Courtesy of Bruce Black

The symptoms include temporary or persistent pain or pressure in both ears, deafness, vertigo, tinnitus and perhaps discharge. Inspection of the TM may reveal (in order of seriousness): retraction; erythema; haemorrhage (due to extravasation of blood into the layers of the TM); fluid or blood in the middle ear; perforation. Perform conductive hearing loss tests with tuning fork.

Treatment Most cases are mild and resolve spontaneously in a few days, so treat with analgesics and reassurance. Menthol inhalations are soothing and effective. Refer if any persistent problems for consideration of the Politzer bag inflation or myringotomy.

Prevention Flying. Perform repeated Valsalva manoeuvres during descent. Use decongestant drops or sprays before boarding the aircraft, and then 2 hours before descent. Diving. Those with nasal problems, otitis media or chronic tubal dysfunction should not dive.

Penetrating injury to tympanic membrane A penetrating injury to the TM can occur in children and adults from various causes such as pencils and slivers of wood or glass. Bleeding invariably follows and infection is the danger.

Management Remove blood clot by suction toilet or gentle dry mopping Ensure no FB is present Check hearing Prescribe a course of broad-spectrum antibiotics (e.g. cotrimoxazole) Prescribe analgesics Instruct the person not to let water enter ear Review in 2 days and then regularly At review in 1 month, the drum should be virtually healed Check hearing 2 months after injury

Complete healing can be expected within 8 weeks in 90–95% of such cases.18

Temporomandibular joint arthralgia If rheumatoid arthritis is excluded, a set of special exercises, which may include ‘chewing’ a piece of soft wood over the molars, invariably solves this problem (see CHAPTER 41 ). If an obvious dental malocclusion is present, referral is necessary.

When to refer Otitis media Incomplete resolution of acute otitis media Persistent middle-ear effusion for 3 months after an attack of acute otitis media Persistent apparent or proved deafness Evidence or suspicion of acute mastoiditis or other severe complications Frequent recurrences (e.g. four attacks a year) Presence of craniofacial abnormalities

Other ear problems Attic perforation/cholesteatoma FBs in ear not removed by simple measures such as syringing No response to treatment after 2 weeks for otitis externa Suspicion of carcinoma of the ear canal Acute TM perforation that has not healed in 6 weeks Chronic TM perforation (involving lower two-thirds of TM)

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Practice tips The pain of acute otitis media may be masked by fever in babies and young children. A red TM is not always caused by otitis media. The blood vessels of the drum

head may be engorged from crying, sneezing or nose blowing. In crying babies, the TM as well as the face may be red. In otitis externa, most cases will resolve rapidly if the ear canal is expanded and then cleaned meticulously. If an adult presents with ear pain but normal auroscopy, examine possible referral sites, namely TMJ, mouth, throat, teeth and cervical spine. Consider mastoiditis if foul-smelling discharge is present over 7+ days. Antibiotics have no place in the treatment of otic barotrauma. It is good practice to make relief of distressing ear pain a priority. Adequate analgesics must be given. There is a tendency to give too low a dose of paracetamol in children. The installation of nasal drops in infants with a snuffy nose and acute otitis media can indirectly provide amazing pain relief. Spirit ear drops APF are a cheap and simple agent to use for recurrent otitis externa where wetness of the ear canal is a persistent problem.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Earache in children Ear infection (otitis media) Ear: otitis externa Ear: wax in your ear

Resources National Health and Medical Research Council (NHMRC). Australian Clinical Practice Guidelines: Available from: https://www.clinicalguidelines.gov.au. National Institute for Health and Care Excellence (NICE). Respiratory tract infection: Antibiotic prescribing. London: NICE, 2008. Available from: https://www.nice.org.uk/guidance/cg69/evidence/full-guideline-196853293. The Royal Children’s Hospital Melbourne. Australian clinical practice guidelines for managing acute otitis media. Melbourne: RCH, last updated April 2018. Available from: https://www.rch.org.au/clinicalguide/guideline_index/Acute_otitis_media/.

References 1

Black B. Otalgia. Aust Fam Physician, 1987; 16: 292–6.

2

Lieberthal AS et al. The diagnosis and management of acute otitis media. Pediatrics, 2013; 131(3): e964–99.

3

Ludman H. ABC of Otolaryngology (3rd edn). London: BMJ, 1993.

4

Murtagh J, Coleman J. Practice Tips (8th edn). Sydney: McGraw-Hill, 2019: 126–30.

5

Ear, nose and throat infections [published 2019]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2019. www.tg.org.au, accessed October 2020.

6

Morris PS, Leach AJ. Managing otitis media: an evidence-based approach. Australian Prescriber, 2009; 32(6): 155–9.

7

Venekamp RP et al. Antibiotics for acute otitis media in children. Cochrane Database Syst Rev, 2015 Jun 23; (6): CD000219.

8

Gunasekera H. Otitis media in children: how to treat. Australian Doctor, 18 July 2008: 33– 40.

9

Van Dongen TM et al. Treatment of otitis media in children under 2 years of age. N Eng J Med, 2014; 370: 723–33.

10

Perera R et al. Autoinflation for hearing loss associated with otitis media with effusion. Cochrane Database of Syst Rev, 2013; Issue 5: CD006285.

11

Simpson SA et al. Oral or topical nasal steroids for hearing loss associated with otitis media with effusion in children. Cochrane Database of Syst Rev, 2011; Issue 5: CD001935.

12

Griffin G, Flynn CA. Antihistamines and/or decongestants for otitis media with effusion (OME) in children. Cochrane Database of Syst Rev, 2011; Issue 9: CD003423.

13

Browning GG et al. Grommets (ventilation tubes) for hearing loss associated with otitis media with effusion in children. Cochrane Database of Syst Rev, 2010; Issue 10: Art. No. CD001801.

14

Buckley N (Chair). Australian Medicines Handbook. Adelaide: Australian Medicines Handbook Pty Ltd, 2013: 386–7.

15

Kaushik V, Malik T, Saeed SR. Intervention for acute otitis externa (Cochrane Review). Cochrane Database. Syst Rev, 2010; Issue 1: Art No. CD004740.

16

Black B. Otitis media: how to treat. Australian Doctor, 29 November 2002: I–VIII.

17

Wall GM et al. Ciprofloxacin 0.3% dexamethasone 0.1% sterile otic suspension for topical treatment ear infections: a review of the literature. Pediatr Infect Dis J, 2009; 28(2): 141–4.

18

Kruger R, Black B. Penetrating injury eardrum. Aust Fam Physician, 1986; 15: 735.

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40 The red and tender eye

Those with sore eyes … find the light painful, while the darkness, which permits them to see nothing, is restful and agreeable. DIO CHRYSOSTOM (40–115 CE) A red eye accounts for at least 80% of eye problems encountered in general practice.1 An accurate history combined with a thorough examination will permit the diagnosis to be made in most cases without recourse to specialist ophthalmic equipment. A summary of the diagnostic strategy model is presented in TABLE 40.1 . Table 40.1

The red and tender eye: diagnostic strategy model

Probability diagnosis Conjunctivitis: bacterial adenovirus allergic Irritants: stye pterygium/pinguecula Serious disorders not to be missed Acute glaucoma Uveitis: acute iritis choroiditis Corneal ulcer Herpes simplex keratitis Microbial keratitis (e.g. fungal, amoebic, bacterial)

Herpes zoster ophthalmicus Penetrating injury Endophthalmitis Orbital cellulitis Trachoma Pitfalls (often missed) Scleritis/episcleritis Foreign body Trauma—contusion, penetrating injury Ultraviolet light ‘keratitis’ Blepharitis Cavernous sinus arteriovenous fistula Seven masquerades checklist Drugs (hypersensitivity) Thyroid disorder (hyperthyroidism) Is the patient trying to tell me something? Unlikely.

Key facts and checkpoints Acute conjunctivitis accounts for over 25% of all eye complaints seen in general practice.2 Viral conjunctivitis (compared to bacterial) is more common in adults, is usually bilateral and discharge is more watery than purulent.3 Viral conjunctivitis can be slow to resolve and may last for weeks. Pain and visual loss suggest a serious condition such as glaucoma, uveitis (including acute iritis) or corneal ulceration. Beware of the unilateral red eye—think beyond bacterial or allergic conjunctivitis. It is rarely conjunctivitis and may be a corneal ulcer, keratitis, foreign body, trauma, uveitis or acute glaucoma.4 Keratitis (inflammation of the cornea) is one of the most common causes of an uncomfortable red eye. Apart from the well-known viral causes (herpes simplex, herpes zoster, adenovirus and measles), it can be caused by fungal infection (usually on a damaged cornea), bacterial infection, protozoal infection or inflammatory disorder such as ankylosing spondylitis.5 Herpes simplex keratitis (dendritic ulcer) often presents painlessly, as the

neurotrophic effect grossly diminishes sensation.

The clinical approach The five essentials of the history are: history of trauma (including wood/metalwork—foreign body) vision the degree and type of discomfort presence of discharge presence of photophobia

Page 483

The social and occupational history is also very important. This includes a history of exposure to a ‘red eye’ at school, work or home; incidents at work such as injury, welding, foreign bodies or chemicals; and genitourinary symptoms. When examining the unilateral red eye, keep the following diagnoses in mind: trauma foreign body, including intra-ocular (IOFB) corneal ulcer iritis (uveitis) viral conjunctivitis (commonest type) acute glaucoma The manner of onset of the irritation often gives an indication of the cause. Conjunctivitis or uveitis generally has a gradual onset of redness, while a small foreign body will produce a very rapid hyperaemia. Photophobia occurs usually with uveitis and keratitis. It is vital to elicit careful information about visual acuity. The wearing of contact lenses is important as these are prone to cause infection or the ‘overwear syndrome’, which resembles an acute ultraviolet (UV) burn.

The key eye symptoms The key eye symptoms are: itch

irritation pain (with pus or watering) loss of vision (red or white eye) red = front of eye white = back of eye

Key questions Have you noticed blurring of your vision? Have you been in close contact with others with the same condition? Have you had a cold or running nose recently? Do you wear contact lenses? Can you recall scratching or injuring your eye? What were you doing at the time you noticed trouble? Have you been putting any drops, ointments or cosmetics in or around your eye? Do you suffer from hay fever? Do you have any problems with your eyelids? Had your eyes been watering for some time beforehand? Have you had any other problems? Have you been exposed to arc welding?

Loss of vision in the red eye Consider: iritis (uveitis) scleritis acute glaucoma (pain; nausea and vomiting) chemical burns

Red eye red flags (urgent ophthalmic referral)6,7 Severe ocular pain Severe orbital pain Reduction ophthalmic or loss of vision Diplopia Dilated pupil Abnormal corneal signs Globe displacement Endophthalmitis Microbial keratitis ± contact lens use

The painful red eye Causes to consider: foreign body keratitis uveitis (iritis) episcleritis scleritis acute glaucoma hypopyon (pus in the anterior chamber) endophthalmitis (inflammation of internal structures—may follow surgery) corneal abrasion/ulceration Pain with discharge: keratitis

Pain with photophobia: uveitis episcleritis

Examination The basic equipment: eye testing charts at 45 cm (18 in) and 300 cm (10 ft) multiple pinholes torch (and Cobalt blue or ultraviolet light) magnifying aid (e.g. binocular loupe) glass rod or cotton bud to aid eyelid eversion fluorescein sterile paper strips anaesthetic drops ophthalmoscope Ishihara colour vision test tonometer (if available, e.g. Schiotz) The four essentials of the examination are: testing and recording vision meticulous inspection under magnification testing the pupils testing ocular tension4 Also: local anaesthetic test fluorescein staining subtarsal examination

Inspection

Page 484

A thorough inspection is essential, noting the nature of the inflammatory injection, whether it is localised (episcleritis) or diffuse, viewing the iris for any irregularity, observing the cornea, and searching for foreign bodies, especially under the eyelids, and for any evidence of penetrating injury. No ocular examination is complete until the upper eyelid is everted and closely inspected. Both eyes must be examined since many patients presenting with conjunctivitis in one eye will have early signs of conjunctivitis in the other. Use fluorescein to help identify corneal ulceration. Local anaesthetic drops instilled prior to the examination of a painful lesion are recommended. The local anaesthetic test is a sensitive measure of a surface problem—if the pain is unrelieved a deeper problem must be suspected. Palpate for enlarged pre-auricular lymph nodes, which are characteristic of viral conjunctivitis. The nature of the injection is important. In conjunctivitis the vessels are clearly delineated and branch from the corners of the eye towards the cornea, since it involves mainly the tarsal plate. Episcleral and scleral vessels are larger than conjunctival vessels and are concentrated towards the cornea (see FIG. 40.1 ).

FIGURE 40.1 Physical signs to search for in a patient with a red eye (eyelids everted) Ciliary injection appears as a red ring around the limbus of the cornea (the ciliary flush), and the individual vessels, which form a parallel arrangement, are not clearly visible. Ciliary injection may indicate a more serious deep-seated inflammatory condition such as anterior uveitis or a deep corneal infection. The presence of fine follicles on the tarsal conjunctivae indicates viral infection while a cobblestone appearance indicates allergic conjunctivitis. Note: Slit lamp examination is ideal for the examination of the eye.

Investigations These include:

swab of discharge for micro and culture or for viral studies ESR/CRP imaging

Red eye in children Children can suffer from the various types of conjunctivitis (commonly), uveitis and trauma. Of particular concern is orbital cellulitis, which may present as a unilateral swollen lid and can rapidly lead to blindness if untreated. Bacterial, viral and allergic conjunctivitis are common in all children. Conjunctivitis in infants is a serious disorder because of the immaturity of tissues and defence mechanisms. Serious corneal damage and blindness can result.

Neonatal conjunctivitis (ophthalmia neonatorum) This is conjunctivitis in an infant less than 1 month old and is a notifiable disease. Chlamydial and gonococcal infections are uncommon but must be considered if a purulent discharge is found in the first few days of life.3 In both conditions the parents must be investigated for associated venereal infection and treated accordingly (this includes contact tracing) (see CHAPTER 109 ). Chlamydia trachomatis accounts for 50% or more of cases. Its presentation in neonates is acute, usually 1–2 weeks after delivery, with moderate mucopurulent discharge. It is a systemic disease and may be associated with pneumonia. The diagnosis is confirmed by PCR tests on the conjunctival secretions. Treatment is with azithromycin 20 mg/kg orally, daily for 3 days. Regular face washing and the treatment of all household contacts is recommended.3 Neisseria gonorrhoeae conjunctivitis, which usually occurs within 1–2 days of delivery, requires vigorous treatment with intravenous cephalosporins or penicillin and local sulfacetamide drops. The discharge is highly infectious and the organism has the potential for severe corneal infection Page 485 with perforation and blindness7 or septicaemia.3 Other common bacterial organisms can cause neonatal conjunctivitis, and herpes simplex virus type II can cause conjunctivitis and/or eyelid vesicles or keratitis.2

Trachoma More than 6 million people worldwide have trachoma-caused blindness. Trachoma is a chronic chlamydial conjunctivitis that is prevalent in the Aboriginal and Torres Strait Islander population, particularly in dry, remote regions. C. trachomatis is usually transmitted by human contact between children and parents and also by flies, especially where hygiene is inadequate. It is the most common cause of blindness in the world. Recurrent and untreated disease leads to lid scarring and inturned lashes (entropion) with corneal ulceration and

visual loss. It is important to commence control of the infection in childhood.

Treatment Prevention/community education Antibiotics—azithromycin Surgical correction (where relevant)

Blocked nasolacrimal duct Delayed development of the nasolacrimal duct occurs in about 6% of infants,3 resulting in blocked lacrimal drainage; the lacrimal sac becomes infected, causing a persistent discharge from one or both eyes. In the majority of infants, spontaneous resolution of the problem occurs by the age of 6 months.

Management Bathing with normal saline Frequent massage over the lacrimal sac Referral for probing of the lacrimal passage before 6 months if the discharge is profuse and irritating or between 6 and 12 months if the problem has not self-corrected (refer CHAPTER 84 ) Reserve topical antibiotics for true secondary infection (uncommon)

Red eye in the elderly Elderly people have an increased risk of acute glaucoma, uveitis and herpes zoster. Acute angle closure glaucoma should be considered in anyone over the age of 50 presenting with an acutely painful red eye. Eyelid conditions such as blepharitis, trichiasis, entropion and ectropion are more common in the elderly.

Acute conjunctivitis Acute conjunctivitis is defined as an episode of conjunctival inflammation lasting less than 3 weeks.2 The two major causes are infection (either bacterial or viral) and acute allergic or toxic reactions of the conjunctiva (see TABLE 40.2 ).

Major causes of a red eye

Table 40.2

Major causes of a red eye Site of inflammation Conjunctiva, including lining of lids (usually bilateral)

Pain

Discharge

Vision

Irritation —gritty

Purulent, lids stuck in the morning

Normal

Photophobia No

Viral conjunctivitis

Conjunctiva, lining of lids often follicular (uni or bilateral)

Gritty

Watery

Normal

No

Allergic (vernal) conjunctivitis

Conjunctiva, papillary swellings on lid linings (bilateral)

Gritty— itching

Watery

Normal

No

Contact hypersensitivity (dermatoconjunctivitis)

Conjunctiva and eyelids Oedema

Itching

Watery

Normal —may be blurred

No

Subconjunctival haemorrhage

Beefy red area fading at edge (unilateral)

No

No

Normal

No

Herpes simplex keratitis

Unilateral— circumcorneal Dendritic ulcer

Yes— gritty

No, reflex lacrimation

Blurred, but variable, depends on site

Yes

Corneal ulcer

Unilateral— circumcorneal (exclude foreign body)

Yes

No, reflex lacrimation

Blurred, but variable, depends on site

Yes

Scleritis/episcleritis

Localised deep redness Tender area

Yes

No

Normal

Yes

Acute uveitis/iritis

Maximum around cornea

Yes— radiates to brow,

No, reflex lacrimation

Blurred

Yes

Bacterial conjunctivitis

temple, nose Acute glaucoma

Diffuse but maximum circumcorneal

Yes, severe with nausea and vomiting

No, reflex lacrimation

Haloes around lights

Yes

Practice tip Be cautious of loss of vision, pain or photophobia—refer if appropriate.

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Clinical features Diffuse hyperaemia of tarsal or bulbar conjunctivae Absence of ocular pain, good vision, clear cornea Infectious conjunctivitis is usually bilateral (especially after the first day) with a discharge, and a gritty or sandy sensation

Bacterial conjunctivitis Bacterial infection may be primary, secondary to a viral infection or secondary to blepharitis.

History Purulent discharge with sticking together of eyelashes in the morning is typical. It usually starts in one eye and spreads to the other. There may be a history of contact with a person with similar symptoms. The organisms are usually picked up from contaminated fingers, face cloths or towels.

Clinical features Gritty red eye Purulent discharge, usually without lymphadenopathy Clear cornea

Examination There is usually a bilateral mucopurulent discharge with uniform engorgement of all the conjunctival blood vessels and a non-specific papillary response (see FIG. 40.2 ). Fluorescein staining is negative.

FIGURE 40.2 Acute bacterial conjunctivitis with mucopurulent discharge, no corneal stain

Causative organisms These include: Streptococcus pneumoniae Haemophilus influenzae Staphylococcus aureus Streptococcus pyogenes N. gonorrhoeae (a hyperacute onset) Pseudomonas aeruginosa Diagnosis is usually clinical, but a swab should be taken for smear and culture with:2 hyperacute or severe purulent conjunctivitis prolonged infection neonates

Management3

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Limit the spread by avoiding close contact with others, use of separate towels and good ocular hygiene. Clear away debris and mucus with saline solution before topical treatment. Exclude serious causes and a foreign body. Mild cases Mild cases may resolve with saline irrigation of the eyelids and conjunctiva but may last up to 14 days if untreated.8 An antiseptic eye drop such as propamidine isethionate 0.1% (Brolene) 1–2 drops, 6–8 hourly for 5–7 days can be used. Cooled black tea is reportedly widely used in Middle Eastern countries with good effect. More severe cases Chloramphenicol 0.5% eye drops, 1–2 drops 2 hourly for 24 hours,1 decrease to 4 times a day for another 7 days (max. 10 days—cases of aplastic anaemia have been reported with long-term use). Also use chloramphenicol 1% eye ointment each night or, alternatively, framycetin 0.5% eye drops, 1–2 drops every 1–2 hours for the first 24 hours, decreasing to 8 hourly until discharge resolves for up to 7 days. Note: Never pad a discharging eye.

Practice tip Brick-red eye—think of chlamydia.

Specific organisms Pseudomonas and other coliforms: use topical gentamicin and tobramycin. Chloromycetin ineffective. N. gonorrhoeae: use appropriate systemic antibiotics depending on sensitivity (use Gram stain culture and PCR). Use ceftriaxone or cefotaxime 1 g IM or IV as a single dose (adults).3 Chlamydia trachomatis—may be sexually transmitted (a full STI screen is advisable). Differs from trachoma-causing strains. Shows a brick-red follicular conjunctivitis with a stringy mucus discharge. Treatment is with azithromycin.

Viral conjunctivitis The most common cause of this very contagious condition is adenovirus.

History

It is commonly associated with URTIs and is the type of conjunctivitis that occurs in epidemics (pink eye).1 The conjunctivitis usually has a 2–3 week course; it is initially one-sided but with cross-infection occurring days later in the other eye. It can be a severe problem with a very irritable, watering eye.

Examination The examination should be conducted with gloves. It is usually bilateral with diffuse conjunctival infection and productive of a scant watery discharge. Viral infections typically but not always produce a follicular response in the conjunctivae (tiny, pale lymphoid follicles) and an associated pre-auricular lymph node (see FIG. 40.3 ). Subconjunctival haemorrhages may occur with adenovirus infection. High magnification, ideally a slit lamp, may be necessary to visualise some of the changes, such as small corneal opacities, follicles and keratitis.

FIGURE 40.3 Viral conjunctivitis: watery eye, lid swelling, typical eyelid follicles, associated local lymphadenopathy Diagnosis is based on clinical grounds and a history of infected contacts. Viral culture and serology can be performed to identify epidemics.

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Treatment Limit cross-infection by appropriate rules of hygiene and patient education. Treatment is symptomatic—cool compress or saline bathing, possibly with topical lubricants (artificial tear preparations) or vasoconstrictors (e.g. phenylephrine). Do not pad; avoid bright light. Watch for secondary bacterial infection. Avoid corticosteroids, which reduce viral shedding and prolong the problem.

Primary herpes simplex infection

This viral infection produces follicular conjunctivitis. About 50% of patients have associated lid or corneal ulcers/vesicles, which are diagnostic.2 Dendritic ulceration highlighted by fluorescein staining is diagnostic (see FIG. 40.4 detection or culture may allow confirmation.

). Antigen

FIGURE 40.4 Herpes simplex keratitis—gritty, watery eye with typical dendritic ulcer, stained with fluorescein

Treatment (herpes simplex keratitis) Attend to eye hygiene Aciclovir 3% ointment, five times a day for 14 days or for at least 3 days after healing3 Atropine 1% 1 drop, 12 hourly, for the duration of treatment will prevent reflex spasm of the pupil (specialist supervision) Debridement by an ophthalmologist Never use corticosteroids, and refer all new cases early to an ophthalmologist.

Chlamydial conjunctivitis Chlamydial conjunctivitis is encountered in three common situations: neonatal infection (first 1–2 weeks) young patient with associated venereal infection isolated Aboriginal and Torres Strait Islander people with trachoma Take swabs for culture and PCR testing.

Systemic antibiotic treatment:8 neonates: azithromycin 20 mg/kg orally, daily for 3 days3 children over 6 kg and adults: azithromycin 1 g (o) as single dose Note: Treat contacts in cases of STI.

Allergic conjunctivitis Allergic conjunctivitis results from a local response to an allergen. It includes: vernal (hay fever) conjunctivitis contact hypersensitivity reactions, e.g. reaction to preservatives in drops

Vernal (hay fever) conjunctivitis This is usually seasonal and related to pollen exposure, particularly in younger people. There is usually associated rhinitis (see CHAPTER 72 ).

Treatment3 Tailor treatment to the degree of symptoms. Artificial tear preparations may give adequate symptomatic relief. Oral antihistamines may be required but topical measures usually suffice. Eye drop options: 1. Medications with both antihistamine and mast cell stabilising properties ketotifen or olopatadine twice daily, or azelastine 2–4 times daily 2. Mast cell stabilisers cromoglycate or lodoxamide 4 times daily 3. Antihistamines levocabastine 2–3 times daily 4. Topical corticosteroids (severe cases, should refer) Avoid vasoconstrictors (e.g. naphazoline, tetryzoline).

Contact hypersensitivity Common topical allergens and toxins include topical ophthalmic medications, especially

antibiotics, contact lens solutions (often the contained preservative) and a wide range of cosmetics, soaps, detergents and chemicals. Clinical features include burning, itching and watering with hyperaemia and oedema of the conjunctiva and eyelids. A skin reaction of the lids usually occurs. Page 489

Treatment Withdraw the causative agent. Apply normal saline compresses. Treat with naphazoline or phenylephrine. If not responding, refer for possible corticosteroid therapy.

Subconjunctival haemorrhage Subconjunctival haemorrhage, which appears spontaneously, is a beefy red localised haemorrhage with a definite posterior margin (see FIG. 40.5 ). If it follows trauma and extends backwards, it may indicate an orbital fracture. It is usually caused by a sudden increase in intrathoracic pressure such as coughing and sneezing. It is not related to hypertension but it is worthwhile measuring the blood pressure to help reassure the patient.

FIGURE 40.5 Subconjunctival haemorrhage: usually a localised haemorrhage that appears spontaneously. It is pain free. If traumatic and extends posteriorly, it may indicate an orbital fracture.

Management No local therapy is necessary. The haemorrhage absorbs over 2 weeks. Patient explanation and reassurance is necessary (‘a highly visible minor bruise’). If haemorrhages are recurrent, a bleeding tendency should be excluded.

Episcleritis and scleritis Episcleritis and scleritis present as a localised area of inflammation (see FIGS 40.1 and 40.6 ). The episclera is a vascular layer that lies just beneath the conjunctiva and adjacent to the sclera. Both may become inflamed, but episcleritis (which is more localised) is essentially self-limiting, while scleritis (which is rare) is more serious as the eye may perforate.6 Both conditions may be confused with inflammation associated with a foreign body, pterygium or pinguecula. There are no significant associations with episcleritis, which is usually idiopathic, but scleritis may be associated with connective tissue disease, especially rheumatoid arthritis and herpes zoster and rarely sarcoidosis and tuberculosis.

FIGURE 40.6 Episcleritis showing inflammation of the conjunctival and episclerical tissue only. Note the absence of violaceous colour that is seen in scleritis.

Clinical features Episcleritis: no discharge no watering vision normal (usually) often sectorial usually self-limiting Treat with topical or oral steroids.

Scleritis: painful loss of vision urgent referral

History A red and sore eye is the presenting complaint. There is usually no discharge but there may be reflex lacrimation. Scleritis is much more painful than episcleritis6 and the eye becomes intensely red.

Examination With scleritis, there is a localised area of inflammation that is tender to touch (FIG. 40.6 ), and which is more extensive than with episcleritis, being uniform across the eye. The inflamed vessels are larger than the conjunctival vessels. Application of topical phenylephrine 2.5% produces blanching of superficial episcleral vessels but no blanching of deep scleral vessels. Page 490

Management An underlying cause such as an autoimmune condition should be identified. Refer the patient, especially for scleritis. Corticosteroids or NSAIDs may be prescribed.

Uveitis (iritis) The iris, ciliary body and the choroid form the uveal tract, which is the vascular coat of the eyeball.3 Anterior uveitis (acute iritis or iridocyclitis) is inflammation of the iris and ciliary body and this is usually referred to as acute iritis (see FIG. 40.7 ). The iris is sticky and sticks to the lens. The pupil may become small because of adhesions, and the vision is blurred.

FIGURE 40.7 Diagrammatic representation of eye structures involved in inflammatory disorders Causes include autoimmune-related diseases such as the seronegative arthropathies (e.g. ankylosing spondylitis), SLE, IBD, sarcoidosis and some infections (e.g. toxoplasmosis and syphilis).

Clinical features Eye redness, esp. around the edge of the iris Eye discomfort or pain Increased tearing Blurred vision Sensitivity to light Floaters in the field of vision Small pupil The examination findings are summarised in TABLE 40.2 . The affected eye is red, with the infection being particularly pronounced over the area covering the inflamed ciliary body (ciliary flush). However, the whole bulbar conjunctivae can be infected. The patient should be referred to a consultant. Slit lamp examination aids diagnosis. Management involves finding the underlying cause. Treatment includes pupil dilatation with atropine drops and topical steroids to suppress inflammation. Systemic corticosteroids may be

necessary. The prognosis of anterior uveitis is good if treatment and follow-up are maintained, but recurrence is likely. Posterior uveitis (choroiditis) may involve the retina and vitreous membrane. Blurred vision and floating opacities in the visual field may be the only symptoms. Pain is not a feature. Referral to detect the causation and for treatment is essential.

Acute glaucoma Acute glaucoma should always be considered in a patient over 50 years presenting with an acutely painful red eye. Permanent damage will result from misdiagnosis. The attack characteristically strikes in the evening or early morning when the pupil becomes semidilated.6

Clinical features Patient >50 years Pain in one eye ± Nausea and vomiting Impaired vision Haloes around lights Hazy cornea Fixed semidilated pupil Eye feels hard

Management Urgent ophthalmic referral is essential since emergency treatment is necessary to preserve the eyesight. If immediate specialist attention is unavailable, treatment can be initiated with acetazolamide (Diamox) 500 mg IV and pilocarpine 4% drops to constrict the pupil or pressurelowering drops.

Keratoconjunctivitis sicca Dry eyes are a common problem, especially in elderly women. Lack of lacrimal secretion can be functional (e.g. ageing), or due to systemic disease (e.g. rheumatoid arthritis, SLE, Sjögren syndrome), drugs (e.g. β-blockers) or other factors, including menopause. Up to 50% of patients with severe dry eye have Sjögren syndrome. Page 491

Clinical features

A variety of symptoms Dryness, grittiness, stinging and redness Sensation of foreign body (e.g. sand) Photophobia if severe Slit light examination diagnostic with special stains

Treatment Treat the cause. Bathe eyes with clean water. Use artificial tears: hypromellose (e.g. Tears Naturale), polyvinyl alcohol (e.g. Tears Plus). Be cautious of adverse topical reactions. Refer severe cases.

Eyelid and lacrimal disorders There are several inflammatory disorders of the eyelid and lacrimal system that present as a ‘red and tender’ eye without involving the conjunctiva. Any suspicious lesion should be referred.

Stye (external hordeolum) A stye is an acute abscess of a lash follicle or associated glands of the anterior lid margin, caused usually by S. aureus. The patient complains of a red tender swelling of the lid margin, usually on the medial side (see FIG. 40.8 ). A stye may be confused with a chalazion, orbital cellulitis or dacryocystitis.

FIGURE 40.8 Hordeolum: a stye. This is a focal staphylococcal infection of the root of an eyelash.

Management Use heat to help it ‘point’ and discharge by using direct steam from a thermos (see FIG. 40.9 ) onto the enclosed eye or by hot-water compresses. If fluctuant and pointing, perform lash epilation to allow drainage of pus (incise with a size 11 blade if epilation does not work). Squeezing is discouraged. Do not use antibiotics (topical or oral) unless secondary spread (cellulitis).6

FIGURE 40.9 Steaming the painful eye: allow steam to rise from a thermos onto the closed eye for 10–15 minutes

Chalazion (meibomian cyst) Also known as an internal hordeolum, this granuloma of the meibomian gland in the eyelid may become inflamed and present as a tender irritating lump in the lid. Look for evidence of blepharitis. Differential diagnoses include sebaceous gland carcinoma and basal cell carcinoma.

Management Conservative treatment may result in resolution. This involves heat either as steam from a thermos or by applying a hot compress (a hand towel soaked in hot water) followed by light massage. If the chalazion is very large, persistent or uncomfortable, or is affecting vision, it can be incised and curetted under local anaesthesia. This is best performed through the inner conjunctival surface using a chalazion clamp (blepharostat) (see FIG. 40.10 ). Page 492

FIGURE 40.10 Excision of a meibomian cyst, using a chalazion clamp and curette Meibomianitis is usually a staphylococcal micro-abscess of the gland, and oral antistaphylococcal antibiotics (not topical) are recommended (e.g. di/flucloxacillin 500 mg (o) 6 hourly for adults). Surgical incision and curettage may also be necessary.

Blepharitis This common chronic condition of the eyelids may involve inflammation of the lid margins (anterior blepharitis) which is commonly associated with secondary ocular effects such as styes, chalazia and conjunctival or corneal ulceration (see FIG. 40.11 ). Posterior blepharitis, which involves abnormalities of the submucus meibomian glands at the rim of the eyelids, is frequently associated with seborrhoeic dermatitis (especially) and atopic dermatitis, and less so with rosacea.8 There is a tendency to colonisation of the lid margin with S. aureus, which causes an ulcerative infection. May have lash loss and trichiasis if chronic.

FIGURE 40.11 Blepharitis: common complicating features The two types are: anterior blepharitis—staphylococcal posterior blepharitis—seborrhoeic (mainly) and rosacea

Clinical features9 Persistent sore eyes or eyelids Irritation, grittiness, burning, dryness and ‘something in the eye’ sensation, worse in mornings Lid or conjunctival swelling and redness Crusts or scales around the base of the eyelids Discharge or stickiness, especially in morning Inflammation and crusting of the lid margins

Management Anterior blepharitis Eyelid hygiene is the mainstay of therapy. The crusts and other debris should be gently cleaned with a cotton wool bud dipped in a 1:10 dilution of baby shampoo or a solution of sodium bicarbonate, once or twice daily. Application of a warm water compress or saline soak with gauze for 5 minutes is also effective. Proprietary lid solutions or wipes can also be used. If not controlled, apply chloromycetin 1% ointment once or twice daily for up to 4 weeks and review. Refer resistant cases. Posterior blepharitis Follow the same hygiene methods as above but with firm eye massage in a circular motion towards the lid margins to closed eyes, for 5–10 minutes twice a day. Ocular lubricants such as artificial tear preparations may greatly relieve symptoms of keratoconjunctivitis sicca (dry eyes). Control scalp seborrhoea with regular medicated shampoos. If persistent, short-term use of a mild topical corticosteroid ointment (e.g. hydrocortisone 0.5%) can be effective. Treat infection with an antibiotic ointment smeared on the lid margin (this may be necessary

for several months) (e.g. tetracycline hydrochloride 1% or framycetin 0.5% or chloramphenicol 1% ointment to lid margins 3–6 hourly).8 If not controlled by topical measures, use systemic antibiotics such as doxycycline 50 mg daily for at least 8 weeks (erythromycin for children 3 mm corneal ulcer severe conjunctivitis

uveitis/acute iritis Behçet syndrome acute glaucoma giant cell arteritis orbital cellulitis (pre- and post-) acute dacryocystitis keratitis episcleritis/scleritis endophthalmitis herpes zoster ophthalmicus Note: As a general rule never use corticosteroids or atropine in the eye before referral to an ophthalmologist.

Practice tips Avoid long-term use of any medication, especially antibiotics (e.g. chloramphenicol: course for a maximum of 10 days).2 Note: Beware of allergy or toxicity to topical medications, especially antibiotics, as a cause of persistent symptoms. As a general rule, avoid using topical corticosteroids or combined corticosteroid/antibiotic preparations. Never use corticosteroids in the presence of a dendritic ulcer. To achieve effective results from eye ointment or drops, remove debris such as mucopurulent exudate with bacterial conjunctivitis or blepharitis by using a warm solution of saline (dissolve a teaspoon of kitchen salt in 500 mL of boiled water) to bathe away any discharge from conjunctiva, eyelashes and lids. A gritty sensation is common in conjunctivitis but the presence of a foreign body must be excluded.6 Beware of the contact lens ‘overwear syndrome’, which is treated in a similar way to flash burns.

Red eye golden rules Always test and record vision Beware of the unilateral red eye Conjunctivitis is almost always bilateral Irritated eyes are often dry Never use steroids if herpes simplex is suspected A penetrating eye injury is an emergency Consider an intra-ocular foreign body Beware of herpes zoster ophthalmicus if the nose is involved Irregular pupils: think iritis, injury and surgery Never pad a discharging eye Refer patients with eyelid ulcers If there is a corneal abrasion look for a foreign body Source: Based on J Colvin and J Reich4

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Blepharitis Bloodshot eye Chalazion (meibomian cyst) Conjunctivitis Flash burns to the eyes Foreign body in the eye Stye

Watering eyes

References 1

McDonnell P. Red eye: an illustrated guide to eight common causes. Modern Medicine Australia, 1989; October: 37–9.

2

Della NG. Acute conjunctivitis. In: MIMS Disease Index. Sydney: IMS Publishing, 1991– 92: 113–15.

3

Eye infections [published 2019]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2019. www.tg.org.au, accessed October 2019.

4

Colvin J, Reich J. Systematic examination of the red eye. Aust Fam Physician, 1976; 5: 153–65.

5

Butler TK et al. Infective keratitis in older patients: a 4 year review. Br J Ophthalmol, 2005: 89(5): 591–6.

6

Kuzniarz M. Red eye. Medical Observer, 11 May 2012: 27–30.

7

Buckley N (Chair). Australian Medicines Handbook. Adelaide: Australian Medicines Handbook Pty Ltd, 2018: 485–6.

8

Lindsley K et al. Interventions for chronic blepharitis (Cochrane Review). Cochrane Database. Syst Rev, 2012; Issue 5: Art No. CD005556.

9

Colvin J. Painful eye: an emergency call. Aust Fam Physician, 1985; 14: 1258.

10

Watson SL. Common corneal conditions. MedicineToday, 2005; 6(5): 22–30.

11

Schein OD, Poggio EC. Ulcerative keratitis in contact lens wearers. Cornea, 1990; 9(1): 55–8.

12

Lazarus MG. Complications of contact lenses. In: MIMS Disease Index (2nd edn). Sydney: IMS Publishing, 1996: 121–3.

13

Sehu W, Zagora S. The red eye. Australian Doctor. 12 October 2012: 23–30.

14

Barry P, Cordovés L, Gardner S. European Society of Cataract Refractive Surgeons (ESCRS). Guidelines for prevention and treatment of endophthalmitis following cataract surgery. Dublin, Ireland, 2013.

41 Pain in the face Page 498

It’s as though the devil suddenly thrust red hot electric needles through my right cheek towards my ear. PATIENT (ANONYMOUS), DESCRIBING ‘TIC DOULOUREUX’ When someone complains of pain in the face rather than the head, the physician has to consider foremost the possibilities of dental disorders, sinus disease, especially of the maxillary sinuses, temporomandibular joint (TMJ) dysfunction, eye disorders, lesions of the oropharynx or posterior third of the tongue, trigeminal neuralgia and chronic paroxysmal hemicrania. The key to the diagnosis is the clinical examination because even the most sophisticated investigation may provide no additional information. A basic list of causes of facial pain is presented in TABLE 41.1 .1 The causes can vary from the simple, such as aphthous ulcers, herpes simplex and dental caries, to serious causes, such as carcinoma of the tongue, sinuses and nasopharynx or osteomyelitis of the mandible or maxilla. Table 41.1

Diagnoses to consider in orofacial pain

Positive physical signs Cervical spinal dysfunction Dental pathology Erysipelas Eye disorders Herpes zoster Nasopharyngeal cancer Oropharyngeal disorders: ulceration (aphthous, infective, traumatic, others) cancer gingivitis/stomatitis

tonsillitis erosive lichen planus Paranasal sinus disorders Parotid gland: mumps sialectasis cancer pleomorphic adenoma TMJ dysfunction Temporal arteritis Absent physical signs Atypical facial pain Chronic paroxysmal hemicrania Depression-associated facial pain Facial migraine (lower half headache) Glossopharyngeal neuralgia Migrainous neuralgia (cluster headache) Trigeminal neuralgia (tic douloureux)

Key facts and checkpoints Dental disorders are the commonest cause of facial pain, accounting for up to 90% of pain in and about the face.2 The most common dental disorders are dental caries and periodontal diseases. Dental pain is invariably localised to the dental region of the face. The mean age of onset of trigeminal neuralgia is 50 years. There is a similarity in the ‘occult’ causes of pain in the ear and in the face (refer to FIGS 39.4 and 39.5 ). Sinusitis occurs mainly as part of a generalised upper respiratory infection. Swimming is another common predisposing factor. Dental root infection must be sought in all cases of maxillary sinusitis.

A diagnostic approach A summary of the diagnostic strategy model is presented in TABLE 41.2 Table 41.2

.

Pain in the face: diagnostic strategy model

Probability diagnosis Dental pain: caries periapical abscess fractured tooth Maxillary/frontal sinusitis TMJ dysfunction Serious disorders not to be missed Cardiovascular: myocardial ischaemia aneurysm of cavernous sinus internal carotid aneurysm ischaemia of posterior inferior cerebellar artery Neoplasia: cancer: mouth, sinuses, nasopharynx, tonsils, tongue, larynx, salivary gland metastases: orbital, base of brain, bone Severe infections: herpes zoster erysipelas periapical abscess → osteomyelitis acute sinusitis → spreading infection Temporal arteritis Pitfalls (often missed) TMJ dysfunction Migraine variants: facial migraine chronic paroxysmal hemicrania Atypical facial pain Eye disorders: glaucoma

iritis optic neuritis Chronic dental neuralgia (odontalgia) Salivary gland: infection, mumps, suppuration, calculus, obstruction, cancer Acute glaucoma (upper face) Cranial nerve neuralgias: trigeminal neuralgia glossopharyngeal neuralgia Seven masquerades checklist Depression Spinal dysfunction (cervical spondylosis) Is the patient trying to tell me something? Quite probably. Atypical facial pain has underlying psychogenic elements.

Probability diagnosis The commonest cause of facial pain is dental disorders, especially dental caries. Another common cause is sinusitis, particularly maxillary sinusitis. TMJ dysfunction causing TMJ arthralgia is a very common problem encountered in general practice and it is important to have some simple basic strategies to give the patient.

Red flag pointers for pain in the face Persistent pain: no obvious cause Unexplained weight loss Trigeminal neuralgia: possible serious cause Herpes zoster involving nose Person >60 years: consider temporal arteritis, malignancy

Serious disorders not to be missed

Page 499

It is important not to overlook cancer of various structures, such as the mouth, sinuses, nasopharynx, tonsils, tongue, larynx and parotid gland, which can present with atypical chronic facial pain. It is important therefore to inspect these areas, especially in the elderly, but lesions in the relatively inaccessible nasopharynx can be easily missed. Nasopharynx cancer spreads upwards to the base of the skull early and patients can present with multiple cranial nerve palsies before either pain or bloody nasal discharge.1 Tumours may arise in the bones of the orbit, for example, lymphoma or secondary cancer, and may cause facial pain and proptosis. Similarly, any space-occupying lesion or malignancy arising from the region of the orbit or base of the brain can cause facial pain by involvement (often destruction) of trigeminal sensory fibres. This will lead to a depressed ipsilateral corneal reflex. Also, aneurysms developing in the cavernous sinus1 can cause pain via pressure on any of the divisions of the trigeminal nerve, while aneurysms from the internal carotid arising from the origin of the posterior communicating artery can cause pressure on the oculomotor nerve. Temporal arteritis typically causes pain over the temporal area but can cause ischaemic pain in the jaws when chewing.

Pitfalls Commonly overlooked causes of facial pain include TMJ arthralgia and dental disorders, especially of the teeth, which are tender to percussion, and oral ulceration. Diagnosing the uncommon migraine variants, particularly facial migraine and chronic paroxysmal hemicrania, often presents difficulties, including differentiating between the neuralgias. Glossopharyngeal neuralgia, which is rare, causes pain in the back of the throat, around the tonsils and adjacent fauces. The lightning quality of the pain of neuralgia gives the clue to diagnosis. Page 500

Common pitfalls Failing to refer unusual or undiagnosed causes of facial pain Overlooking infective dental causes, which can cause complications Failing to consider the possibility of malignant disease of ‘hidden’ structures in the older patient Unaware that facial pain never crosses the midline

Seven masquerades checklist Of these, depression and cervical spinal dysfunction must be considered. The upper cervical spine can cause facial pain from lesions of C2 or C3 via the lesser occipital or greater auricular

(see FIG. 41.1 ) nerves, which may give pain around the ear. It is important to remember that the C2 and C3 nerves share a common pathway with the trigeminal nerve (see CHAPTER 51 ).

FIGURE 41.1 Dermatomes of C2 and C3, with the overlap area indicated Depressive illness can present with a variety of painful syndromes and facial pain is no exception. The features of depression may be apparent and thus antidepressants should be prescribed. Usually the facial pain and the depression subside concomitantly.

Psychogenic considerations Psychogenic factors have to be considered in every painful condition. They are considered to be high in patients with atypical facial pain.

The clinical approach History Diagnosis of nearly all types of facial pain must be based almost entirely on the history. It is often difficult to delineate the exact nature and distribution of the pain. The history should include the typical analysis of pain, especially noting the site and radiation of the pain.

Examination

Examination can often confirm the clinical impression given by the history—particularly palpation/percussion/pressure around the teeth, TMJs and sinuses. The patient’s general state and behaviour should be noted. Any sudden jabbing pain in the face causing the characteristic ‘tic’ may indicate neuralgia. Palpate the face and neck to include the parotid glands, eyes, regional lymph nodes and the skin. Inspect and palpate the TMJs and cervical spine. Carefully inspect the nose, mouth, pharynx and postnasal space. In particular inspect the teeth, percussing each tooth if dental disorder is suspected. Bimanual palpation of the floor of the mouth is performed to detect induration or submandibular and submental lymph node enlargement. The sinuses, especially the maxillary sinuses, should be inspected and a (hygienically protected) torch light can be placed inside the mouth to test transillumination of the maxillary sinuses. This works best when one symptomatic side can be compared with an asymptomatic side. Perform a neurological examination on the cranial nerves with special emphasis on the trigeminal, oculomotor and glossopharyngeal nerves.

Investigations If investigations are being contemplated referral may be appropriate, particularly as fibre-optic nasopharyngoscopy is so commonly available as an ENT or maxillofacial ‘office test’. The association of multiple sclerosis and tumours with neuralgias may have to be investigated. Radiological investigations include plain X-rays of the paranasal sinuses, CT scans, MRI and orthopantomograms. Page 501

Facial pain in children Apart from trauma, facial pain in children is almost invariably due to dental problems, rarely migraine variants and occasionally childhood infections such as mumps and gingivostomatitis. A serious problem sometimes seen in children is orbital cellulitis secondary to ethmoiditis. Sinusitis occurs in children, especially older children, and it should be suspected with persistent pain and bilateral mucopurulent rhinorrhoea (beyond 10 days).

Facial pain in the elderly Many of the causes of facial pain have an increased incidence with age, in particular trigeminal neuralgia, herpes zoster, cancer, glaucoma, TMJ dysfunction, sialolithiasis and cervical spondylosis. Glossopharyngeal neuralgia does not seem to have a particular predilection for the elderly. Xerostomia due to decreased secretions of salivary glands may cause abrasion with minor trauma. It may aggravate the pain of glossitis, which is common in the elderly.

Dental disorders Dental caries Dental caries, impacted teeth, infected tooth sockets and dental roots can cause pain in the maxillary and mandibular regions. Caries with periapical and apical abscess formation produces pain from infection extending around the apex of the tooth into the alveolar bone. Retention of a fractured root may cause unilateral paroxysmal pain. Impacted third molars (wisdom teeth) may be associated with surrounding soft tissue inflammation (pericoronitis), causing pain that may be localised to the mandible or radiate via the auriculotemporal nerve to the ear. Candida albicans, which is an oral commensal, may colonise dentures causing hyperaemia and painful superficial ulceration of the denture-bearing mucosa. Antibiotics are of no proven use.

Features of dental caries Pain is usually confined to the affected tooth but it may be diffuse. Pain is almost always aggravated by thermal changes in the mouth: cold—if dental pulp vital hot—if dental pulp is necrotic Pain may be felt in more than one tooth. Dental pain will not cross the midline. Antibiotics are not indicated.

Treatment of dental pain Arrange urgent dental consultation Pain relief:3 aspirin 600 mg (o) 4–6 hourly or ibuprofen 400 mg (o) 4–6 hourly or paracetamol 0.5–1 g (o) 4–6 hourly

Tooth abscess, inflamed wisdom tooth, spreading dental

infection or root canal infection Dental treatment will usually alleviate the problem; however, if severe: metronidazole 400 mg (o) 12 hourly for 5 days plus either amoxicillin 500 mg (o) tds for 5 days or phenoxymethylpenicillin 500 mg (o) 6 hourly for 5 days If unresponsive, or if single preparation preferred: amoxicillin/clavulanate 875/125 mg (o) bd (adjust all preparations for children’s dosages) For patients hypersensitive to penicillin: clindamycin 300–450 mg (o) 8 hourly for 5 days

Gingivitis and periodontitis Refer to CHAPTER 61.

Alveolar osteitis (dry socket) This complicates about 5% of tooth extractions and is due to failure of healing. Refer for toileting (socket irrigation with warm saline) and insertion of dressing to reduce pain. This usually heals naturally in 14–21 days. Antibiotics are of no proven use (see FIG. 41.2 ).3,4

FIGURE 41.2 Dry tooth socket: this is a very painful condition mainly in the lower molars, unrelieved by analgesics following a tooth extraction 1–3 days earlier. The socket has few or no blood clots and sensitive bone surfaces are covered by a greyish layer of necrotic tissue.

Knocked-out or broken teeth If a permanent (second) tooth is knocked out, it can be saved by immediate proper care. Likewise, a broken tooth should be saved and urgent dental attention sought.

The knocked-out tooth Using a glove, hold the tooth by its crown and place it in its original position, preferably immediately (see FIG. 41.3 ); if dirty, put it in milk or saline before replacement or, better still, place it under the tongue and ‘wash it’ in saliva. Do not use water, and do not wipe or touch the root. Fix the tooth by moulding strong aluminium foil over it and the adjacent teeth.

Page 502

Refer to a dentist or dental hospital as soon as possible.

FIGURE 41.3 Replacement of a knocked-out tooth Note: Teeth replaced within half an hour have a 90% chance of successful re-implantation.

Ludwig angina4 This is a rapidly swelling cellulitis occurring in both the sublingual and submaxillary spaces without abscess formation, often arising from a root canal infection. It resembles an abscess and should be treated as one. It is potentially life-threatening as it can compromise the airway.

Management Culture and sensitivity testing

Specialist consultation Empirical treatment: amoxicillin 2 g IV, 6 hourly plus metronidazole 500 mg IV, 12 hourly

Pain from paranasal sinuses Infection of the paranasal sinuses may cause localised pain. Localised tenderness and pain may be apparent with frontal or maxillary sinusitis. Sphenoidal or ethmoidal sinusitis causes a constant pain behind the eye or behind the nose, often accompanied by nasal blockage. Chronic infection of the sinuses may be extremely difficult to detect. The commonest organisms are Streptococcus pneumoniae, Haemophilus influenzae and Moraxella catarrhalis. Expanding lesions of the sinuses, such as mucocoeles and tumours, cause local swelling and displace the contents of the orbit—upwards for maxillary, laterally for the ethmoids and downwards for the frontal.

Maxillary sinusitis The maxillary sinus is the one most commonly infected.5 It is important to determine whether the sinusitis is caused by stasis following a URTI or acute rhinitis, or due to dental root infection. Most episodes are of viral origin, and in the first few days this is indistinguishable from bacterial infections.6

Clinical features (acute sinusitis) Facial pain and tenderness (over sinuses) Toothache Headache Purulent postnasal drip Nasal discharge Nasal obstruction Rhinorrhoea Cough (worse at night)

Prolonged fever Epistaxis Suspect bacterial cause if high fever and purulent nasal discharge.

Clinical features (chronic sinusitis) Vague facial pain Offensive postnasal drip Nasal obstruction Toothache Malaise Halitosis

Some simple office tests Diagnosing sinus tenderness7 Page 503 To differentiate sinus tenderness from non-sinus bone tenderness palpation is useful. This is best done by palpating a non-sinus area first and last (see FIG. 41.4 ), systematically exerting pressure over the temporal bones (T), then the frontal (F), ethmoid (E) and maxillary (M) sinuses, and finally zygomas (Z), or vice versa.

FIGURE 41.4 Diagnosing sinus tenderness: T (temporal) and Z (zygoma) represent no sinus bony tenderness, for purposes of comparison Differential tenderness both identifies and localises the main sites of infection (see FIG. 41.4

).

Diagnosing unilateral sinusitis A simple way to assess the presence or absence of fluid in the frontal sinus, and in the maxillary sinus (in particular), is the use of transillumination. It works best when one symptomatic side can be compared with an asymptomatic side. It is necessary to have the patient in a darkened room and to use a small, narrow-beam torch. For the maxillary sinuses remove dentures (if any). Shine the light inside the mouth (with lips sealed, and torch hygienically covered e.g. plastic bag), on either side of the hard palate, pointed at the base of the orbit. A dull glow seen below the orbit indicates that the antrum is air-filled. Diminished illumination on the symptomatic side indicates sinusitis. In the first few days, viral and bacterial sinusitis are indistinguishable. A CT scan may show mucosal thickening without fluid levels. Plain films are not indicated.

Management (acute bacterial sinusitis) Principles Exclude dental root infection.

Control predisposing factors. Consider antibiotic therapy, but remember it is a self-limiting condition that has equal outcomes at day 10 with or without antibiotics.6 The ‘number needed to harm’ with antibiotic side effects is unfavourable compared to the ‘number needed to treat’ with symptom resolution. Establish drainage by stimulation of mucociliary flow and relief of obstruction. Guidelines for antibiotic therapy Consider therapy for severe cases that fail to improve over the first 5–7 days and display at least three of the following: persistent mucopurulent nasal discharge (>7–10 days) facial pain poor response to decongestants tenderness over the sinuses, especially maxillary tenderness on percussion of maxillary molar and premolar teeth that cannot be attributed to by a single tooth Measures Analgesics Antibiotics (if indicated):3 amoxicillin 500 mg (o) tds or 1 g (o) bd for 5 days or (if sensitive to penicillin) doxycycline 100 mg (o) bd for 5 days or cefuroxime 500 mg (o) tds for 5 days or amoxicillin + clavulanate 875/125 mg (o) tds for 5–10 days if poor response to above (indicates resistant H. influenzae) In complicated or severe disease, use intravenous cephalosporins or flucloxacillin

Nasal decongestants (oxymetazoline-containing nasal drops or sprays)5 for 5 days (only if congestion) Inhalations (an important adjunct) Nasal saline irrigation Antihistamines and mucolytics are of no proven value. Cefuroxime is preferred to cephalexin or cefaclor because of superior anti-pneumococcal activity.3 Page 504

Invasive methods Surgical drainage may be necessary by atrial lavage or frontal sinus trephine.

Inhalations for sinusitis The old method of towel over the head and inhalation bowl can be used, but it is better to direct the vapour at the nose. Equipment needed is a container, which can be an old disposable bowl, a wide-mouthed bottle or tin, or a plastic container. Guard against accidental burns. For the inhalant, several household over-the-counter preparations are suitable such as Friar’s Balsam (5 mL), Vicks VapoRub (1 teaspoon) or menthol (5 mL). The cover can be made from a paper bag (with its base cut out), a cone of paper or a small cardboard carton (with the corner cut away). Method 1. Add 5 mL or 1 teaspoon of the inhalant to 0.5 L (or 1 pint) of boiled water in the container. 2. Place the paper or carton over the container. 3. Get the patient to apply nose and mouth to the opening and breathe in the vapour deeply and slowly through the nose, and then out slowly through the mouth. 4. This should be performed for 5–10 minutes, three times a day, especially before retiring. After inhalation, upper airway congestion can be relieved by autoinsufflation.

Chronic sinusitis Chronic sinusitis (>12 weeks) or recurrent sinusitis may arise from chronic infection or allergy. It may be associated with nasal polyps and vasomotor rhinitis, but is frequently associated with a structural abnormality of the upper airways. Refer to CHAPTER 48 . It does not usually cause pain unless an acute infection intervenes. Initial measures are the same

as for allergic rhinitis;6 use oral or intranasal antihistamine and add in an intranasal corticosteroid (see CHAPTER 72 ). Nasal saline irrigation is a useful addition or alternative. After one month, resistant cases (particularly those with nasal polyps) should be referred to a specialist. While waiting, a temporary trial of oral prednisolone 25 mg may be reasonable. Surgical intervention will benefit chronic recurrence with mechanical blockage.

TMJ dysfunction This condition is due to abnormal movement of the mandible, especially during chewing. The basic causes are dental malocclusion and masticatory muscle dysfunction. Check for bruxism. The pain is felt over the joint and tends to be localised to the region of the ear and mandibular condyle, but it may radiate forwards to the cheek and even the neck.

Examination Check for pain and limitation of mandibular movements, especially on opening the mouth. Palpate about the joint bilaterally for tenderness, which typically lies immediately in front of the external auditory meatus; palpate the temporalis and masseter muscles. Palpate the TMJ over the lateral aspect of the joint disc. Ask the patient to open the mouth fully when tenderness is maximal. The TMJ can be palpated posteriorly by inserting the little finger into the external canal. Check for crepitus in mandibular movement.

Management If organic disease such as rheumatoid arthritis and obvious dental malocclusion is excluded, a special set of instructions or exercises can alleviate the annoying problem of TMJ arthralgia in about 3 weeks. Warm packs may help. Provide patient education advice and self-care. Method 1: ‘Chewing’ the piece of soft wood Obtain a rod of soft wood approximately 15 cm long and 1.5 cm wide. An ideal object is a large carpenter’s pencil. Instruct the patient to position this at the back of the mouth so that the molars grasp the object with the mandible thrust forward. The patient then rhythmically bites on the object with a grinding movement for 2–3 minutes at least 3 times a day. Method 2: The ‘six by six’ program This is a specific program recommended by some dental surgeons. The six exercises should be

carried out six times on each occasion, six times a day, taking about 1–2 minutes. Instruct the patient as follows: 1. Hold the front one-third of your tongue to the roof of your mouth and take six deep breaths.

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2. Hold the tongue to the roof of your mouth and open your mouth six times. Your jaw should not click. 3. Hold your chin with both hands keeping the chin still. Without letting your chin move, push up, down and to each side. Remember, do not let your chin move. 4. Hold both hands behind your neck and pull chin in. 5. Push on upper lip so as to push head straight back. 6. Pull shoulders back as if to touch shoulder blades together. These exercises should be pain-free. If they hurt, do not push them to the limit until pain eases.

Treatments Injection into the TMJ8 Indications: painful rheumatoid arthritis, osteoarthritis or TMJ dysfunction not responding to conservative measures. Inject a 1 mL solution of local anaesthetic and corticosteroid in equal parts, around 1 cm anterior to where the top of the tragus meets the face. Dental management that may be required for malfunction of the bite includes dental occlusal splinting. NSAIDs: a trial of NSAIDs, e.g. ibuprofen 400 mg (o) tds for 10 days, for TMJ inflammation may need consideration. Cease if no response after 10 days.

Inflammatory or ulcerative oropharyngeal lesions A variety of ulcerative conditions and infections of structures such as gingivae, tongue, tonsils, larynx and pharynx can cause facial pain (refer to CHAPTER 61 ). Gingivostomatitis, herpes labialis (cold sores) and aphthous ulceration are common examples. Lesions of the posterior third of the tongue, the oropharynx, tonsils and larynx may radiate to the region of the ear via the tympanic branch of the ninth nerve or the auricular branch of the tenth nerve.

Trigeminal neuralgia Trigeminal neuralgia (tic douloureux) is a condition of often unknown cause that typically occurs

in patients over the age of 50, affecting the second and third divisions of the trigeminal nerve and on the same side of the face. Brief paroxysms of pain, often with associated trigger points, are a feature.

Clinical features Site: sensory branches of the trigeminal nerve (see FIG. 41.5 right side)

) almost always unilateral (often

Radiation: tends to commence in the mandibular division and spreads to the maxillary division and (rarely) to the ophthalmic division Quality: excruciating, searing jabs of pain like a burning knife or electric shock Frequency: variable and no regular pattern Duration: seconds to 1–2 minutes (up to 15 minutes) Onset: spontaneous or trigger point stimulus Offset: spontaneous Precipitating factors: talking, chewing, touching trigger areas on face (e.g. washing, shaving, eating), cold weather or wind, turning onto pillow Aggravating factors: trigger points usually in the upper and lower lip, nasolabial fold or lower eyelid (see FIG. 41.6 ) Relieving factors: nil Associated features: rarely occurs at night; spontaneous remissions for months or years Signs: there are no signs, normal corneal reflex

FIGURE 41.5 Typical cutaneous sensory distribution of the trigeminal nerve and its branches

FIGURE 41.6 Trigeminal neuralgia: typical trigger points

Causes Unknown

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Local pressure on the nerve root entry zone by tortuous pulsatile dilated small vessels (probably up to 75%) Multiple sclerosis Neurosyphilis Tumours of the posterior fossa Note: Precise diagnosis of a condition that can become a burdensome ‘label’ is important. MRI may be helpful.

Treatment Patient education, reassurance and empathic support is very important in these patients. Medical therapy carbamazepine (from onset of the attack to resolution)9 50 mg (elderly patient) or 100 mg (o) bd initially; gradually increase the dose to avoid drowsiness every 7 days to 400 mg bd Alternative drugs if carbamazepine not tolerated or ineffective (but question the diagnosis if lack of response): oxcarbazepine 300 mg bd gabapentin 300 mg at night initially, increasing gradually to 600–1200 mg tds lamotrigine 25 mg (o) alternate daily, slowly increasing every 14 days if necessary to 100 mg bd phenytoin 300–500 mg daily phenytoin 300 mg daily baclofen 5 mg bd initially, increasing every 4 days up to 10–20 mg tds Surgery Refer to a neurosurgeon if medication ineffective Possible procedures include: decompression of the trigeminal nerve root (e.g. gel foam packing between the nerve and

blood vessels) neuroablative treatment, e.g. thermocoagulation/radiofrequency neurolysis surgical division of peripheral branches

Glossopharyngeal neuralgia9,10,11 This is an uncommon condition of the ninth cranial nerve and branches of the vagus nerve with similar clinical features of severe, lancinating pains, particularly felt in one ear, the base of the tongue or beneath the angle of the jaw. The pain usually lasts 30–60 seconds. Sites: back of throat around tonsillar fossa and adjacent fauces deep in ear, back of tongue Radiation: ear canal, neck Triggers: swallowing (esp. cold liquids), coughing, talking, yawning, laughing Treatment: as for trigeminal neuralgia

Migrainous neuralgia (cluster headache) As described in CHAPTER 45 , the pain is unilateral and centred around the eye with associated lacrimation and stuffiness of the nose.

Facial migraine (lower half headache)9 Migraine may rarely affect the face below the level of the eyes, causing pain in the area of the cheek and upper jaw. It may spread over the nostril and lower jaw. The pain is dull and throbbing, and nausea and vomiting are commonly present. The treatment is as for other varieties of migraine with simple analgesics or ergotamine for infrequent attacks.

Paroxysmal hemicrania/hemicrania continua In the rare condition of chronic or episodic paroxysmal hemicrania, there is a unilateral facial pain that can resemble chronic cluster headache but the duration is briefer, about 15 minutes, and it may recur many times a day even for years. It responds dramatically to indomethacin, e.g. indomethacin 25 mg (o) tds initially, then adjusting.12 The chronic form is termed hemicrania continua, which persists for more than 3 months. Page 507

Herpes zoster and postherpetic neuralgia Refer to CHAPTER 114

. Herpes zoster may present as hyperaesthesia or a burning sensation in

any division of the fifth nerve, especially the ophthalmic division.

Atypical facial pain Also known as persistent idiopathic facial pain, it is mainly a diagnosis of exclusion whereby patients, usually middle-aged to elderly women, complain of diffuse pain in the cheek (unilateral or bilateral) without demonstrable organic disease. The pain does not usually conform to a specific nerve distribution (although in the maxillary area), varies in intensity and duration and is not lancinating as in trigeminal neuralgia. It is usually described as deep-seated and ‘boring’, severe, continuous and throbbing in nature. It is a very confusing and difficult problem to treat. These patients tend to show psychoneurotic tendencies and it can be exacerbated by stress, but caution is needed in labelling them as functional.

Treatment Trial of an antidepressant,9 e.g.: amitriptyline 10–75 mg nocte (first line) or dothiepin 25–150 mg nocte

Temporal arteritis This may produce mild or severe unilateral or bilateral headache. There may be ischaemic pain in the jaws when chewing. There may be marked scalp tenderness over the affected arteries. See CHAPTER 21 for management.

Erysipelas Classical erysipelas is a superficial form of cellulitis involving the face. It usually presents with the sudden onset of butterfly erythema with a well-defined edge (see FIG. 41.7 ). It often starts around the nose and there may be underlying sinus or dental infection which should be investigated. There is an associated ‘flu-like’ illness and fever. It is invariably caused by Streptococcus pyogenes. Treatment is by phenoxymethylpenicillin or di/flucloxacillin for 7–10 days.

FIGURE 41.7 Erysipelas: typical spreading distribution of the infection

When to refer Severe trigeminal or glossopharyngeal neuralgia Unusual facial pain, especially with a suspicion of malignancy Continuing pain of uncertain cause Positive neurological signs, such as impaired corneal reflex, impaired sensation in a trigeminal dermatome, slight facial weakness, hearing loss on the side of the neuralgia Possible need for surgical drainage of sinusitis—indications for surgery include failure of appropriate medical treatment, anatomical deformity, polyps, uncontrolled sinus pain5 Dental root infection causing maxillary sinusitis Other dental disorders

Practice tips Malignancy must be excluded in the elderly with facial pain. Problems from the molar teeth, especially the third (wisdom), commonly present with peri-auricular pain without aural disease and pain in the posterior cheek. Facial pain never crosses the midline; bilateral pain means bilateral lesions.13 If no obvious cause of persistent pain, refer to exclude sinister cause: don’t overdiagnose sinusitis.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Sinusitis Temporomandibular joint dysfunction Page 508

References 1

Beck ER, Francis JL, Souhami RL. Tutorials in Differential Diagnosis. Edinburgh: Longman Cheshire, 1987: 161–4.

2

Gerschman JA, Reade PC. Orofacial pain. Aust Fam Physician, 1984; 13: 14–24.

3

Oral and dental [published 2019]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2019. www.tg.org.au, accessed February 2021.

4

Kumar R, Sambrook P, Goss AW. Mismanagement of dental infection. Aust Prescr, 2011; 34: 40–1.

5

Chow AW et al. IPSA clinical practice guidelines for acute bacterial rhinosinusitis in children and adults. Clin Infect Dis, 2012; 54(8): e72–e112.

6

Acute rhinosinusitis [published 2019]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2019. www.tg.org.au, accessed February 2021.

7

Bridges-Webb C. Diagnosing sinus tenderness: practice tip. Aust Fam Physician, 1981; 10: 742.

8

Murtagh J, Coleman J. Practice Tips (8th edn). Sydney: McGraw-Hill, 2019: 48–9.

9

Headache and facial pain [published 2017]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2017. www.tg.org.au, accessed September 2017.

10

Mendelsohn M, Lance J, Wheatley D. Facial pain: how to treat. Australian Doctor, 7 November; 2003: 31–6.

11

Wiffen PJ et al. Carbamazepine for acute and chronic pain in adults (Cochrane Review). Cochrane Database. Syst Rev, 2011; Issue 19: Art No. CD005451.

12

Burns R. Pitfalls in headache management. Aust Fam Physician, 1990; 19: 1825.

13

Quail G. Diagnosing facial pain: non oro-dental causes. New Zeal Dent J, 2017; 113: 17– 21.

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42 Fever and chills

Why! The fever itself is nature’s instrument. THOMAS SYDENHAM (1624–1689), MEDICAL OBSERVATIONS Although fever is a sign of disease and usually occurs in response to infection (mainly viral), its presence is recognised as playing an important role in the individual’s defence against infection. The infecting pathogen triggers hypothalamic receptors, causing the thermostatic mechanisms to be reset to maintain core temperature at a higher level. The elevation in temperature results from increased heat production (e.g. shivering) or decreased loss (e.g. peripheral vasoconstriction). The elevation in body temperature activates T-cell production, increases the effectiveness of interferons and limits the replication of some common viruses.1

Key facts and checkpoints Fever plays an important physiological role in the defence against infection. Normal body temperature (measured orally mid-morning) is 36–37.2°C (average 36.8°C). Fever can be defined as an early-morning (6 am) maximal oral temperature >37.2°C or a temperature >37.8°C at other times of the day, typically 4 pm.2 Oral temperature is about 0.4°C lower than core body temperature. Axillary temperature is 0.5°C lower than oral temperature. Rectal, vaginal and ear drum temperatures are 0.5°C higher than oral and reflect core body temperature. There can be a normal diurnal variation of 0.5–1°C. Fevers due to infections have an upper limit of 40.5–41.1°C. Hyperthermia (temperature above 41.1°C) and hyperpyrexia appear to have no upper limit.

Infection remains the most important cause of acute fever.3 Symptoms associated with fever include sweats, chills, rigors and headache. Causes of fever besides infections include malignant disease, mechanical trauma (e.g. crush injury), vascular accidents (e.g. infarction, cerebral haemorrhage), immunogenic disorders (e.g. drug reactions, SLE), acute metabolic disorders (e.g. gout), and haemopoietic disorders (e.g. acute haemolytic anaemia).3 Drugs can cause fever, presumably because of hypersensitivity.3 Important examples are allopurinol, antihistamines, barbiturates, cephalosporins, cimetidine, methyldopa, penicillins, isoniazid, quinidine, phenolphthalein (including laxatives), phenytoin, procainamide, salicylates and sulfonamides. Drug fever should abate by 48 hours after discontinuation of the drug.4 Infectious diseases at the extremes of age (very young and aged)3 often present with atypical symptoms and signs. Their condition may deteriorate rapidly. Overseas travellers or visitors may have special, even exotic infections and require special evaluation (refer to CHAPTER 129 ). Immunologically compromised patients (e.g. those with AIDS) pose a special risk for infections, including opportunistic infections. A febrile illness is characteristic of the acute infection of HIV: at least 50% have an illness that presents like glandular fever.

Chills/rigors2 The abrupt onset of fever with a chill or rigor is a feature of some diseases. Examples include: bacteraemia/septicaemia pneumococcal pneumonia pyogenic infection with bacteraemia lymphoma pyelonephritis visceral abscesses (e.g. perinephric, lung) malaria

biliary sepsis (Charcot triad—jaundice, right hypochondrial pain, fever/rigors) Features of a true chill are teeth chattering and bed shaking, which is quite different from the chilly sensations that occur in almost all fevers, particularly those in viral infections. The event lasts 10–20 minutes.

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Other features: shaking cannot be stopped voluntarily absence of sweating cold extremities and pallor (peripheral vascular shutdown) dry mouth and pilo-erection: lasts 10–20 minutes

Hyperthermia Hyperthermia or hyperpyrexia is a temperature greater than 41.1°C. A more accurate definition is a state when the body’s metabolic heat production or environmental heat load exceeds normal heat loss capacity. Hyperthermia may be observed particularly in the tropics, in malaria and heatstroke. It can occur with CNS tumours, infections or haemorrhages because of their effect on the hypothalamus.

Heatstroke (sunstroke, thermic fever)5 This is the sudden onset of hot, dry, flushed skin with a rapid pulse, temperature above 40°C, and confusion or altered conscious state in a person exposed to a very hot environment. The BP is usually not affected initially but circulatory collapse may precede death. It is a life-threatening emergency. The diagnosis is clinical. Differential diagnoses include severe acute infection, toxic shock, food, chemical and drug poisoning. The elderly and debilitated are susceptible, as are children left in cars.

Treatment Immediate effective cooling water applied to skin—cool sprays, fanning Icepacks at critical points (e.g. axillae, neck, head) Full body immersion works, but caution in sick people Aim to bring down temperature by 1°C every 10 minutes

Malignant hyperthermia This is a rare hereditary disorder characterised by rapidly developing hyperpyrexia, muscular

rigidity and acidosis in patients undergoing major surgery.

Sweats Sweating is a heat loss mechanism, and diffuse sweating that may soak clothing and bedclothing permits rapid release of heat by evaporation. In febrile patients the skin is usually hot and dry— sweating occurs in most when the temperature falls. It is characteristic of only some fevers (e.g. septic infections and rheumatic fever).

Febrile neutropenia This is fever usually with a temperature ≥38°C in a patient with neutrophils 38.5°C). The usual dose of 10–15 mg/kg every 4–6 hours may represent undertreatment. Use 20 mg/kg as a loading dose and then 15 mg/kg maintenance ibuprofen 5–10 mg/kg every 6 hours is a suitable antipyretic Summary: Tepid sponging for the first 30 minutes combined with paracetamol is preferred management.

Advice to parents

Dress the child in light clothing (stripping off is unnecessary). Do not overheat with too many clothes, rugs or blankets. Give frequent small drinks of light fluids, especially water. Sponging the forehead with water is optional, but do not immerse in a cold bath.

Febrile convulsions Refer to CHAPTER 89

.

Fever in the elderly The elderly tend to have a problem with impaired thermoregulation and so they may not develop a fever in response to suppurative infection compared with younger people. This can be misleading in the diagnostic process.

Important facts Any fever in the elderly is significant. Viral infection is a less common cause of fever in the elderly. Fever in the elderly is sepsis until proven otherwise (common sites are the lungs and urinary tract). The elderly are more vulnerable to hyperthermia and hypothermia. Heatstroke classically occurs in epidemic form during a heatwave. The syndrome consists of hyperpyrexia, decreased sweating, delirium and coma. The core temperature is usually over 41°C.

‘Alarm bell’ signs In many patients the existence of a life-threatening infective illness is obvious and prompt action is essential. In others the diagnosis is not clear-cut but there are certain warning signs (see Red flags box ). These ‘red flag’ symptoms and signs are obviously super ‘sensitive’. Patients with some of these features may have potentially life-threatening diseases, but this list would include many with viral infections. A more targeted subset of this list using specific numerical thresholds is used to create Early Warning Scores (EWS), variations of which include REWS (Remote—Australia), MEWS (Modified), NEWS (National—UK) and PEWS (Paediatric).

Fever of undetermined origin

Fever of undetermined origin (FUO), also referred to as pyrexia of unknown origin (PUO), has the following (Petersdorf–Beeson modified) criteria:11 illness for at least 3 weeks fevers >38.3°C (100.9°F) on several occasions undiagnosed after 1 week of intensive study

Red flag pointers for fever High fever Repeated rigors Drenching night sweats Severe myalgia (?sepsis) Severe pain anywhere (?sepsis) Severe sore throat or dysphagia (?Haemophilus influenzae epiglottitis) Altered mental state Incessant vomiting Unexplained rash Jaundice Marked pallor Tachycardia Tachypnoea

Most cases represent unusual manifestations of common diseases and not rare or exotic diseases. Examples are tuberculosis, bacterial endocarditis, hepatobiliary disease and lung cancer.12 Keep in mind that the longer the duration of fever, the less likely the diagnosis is to be infectious —fevers that last greater than 6 months are rarely infectious (only 6%). One study showed that 9% are factitious.13 Patients with FUO in definite need of further investigation are:

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babies 40°C adults >50 years people with diabetes the immunocompromised travellers

A diagnostic approach A knowledge of the more common causes of FUO is helpful in planning a diagnostic approach (refer to TABLE 42.1 ). Table 42.1

Diagnostic strategy for fever that is prolonged (FUO)

Probability diagnosis Pyogenic abscess (anywhere, e.g. liver, pelvis) Pneumonia (viral, bacterial, atypical) Epstein–Barr mononucleosis Viral upper respiratory tract infection Urinary infection (incl. chronic pyelonephritis) Serious disorders not to be missed Vascular: vasculitides (polyarteritis nodosa, giant cell arteritis/polymyalgia) Infection: HIV/AIDS malaria and other tropical diseases zoonoses (e.g. leptospirosis, Q fever, listeriosis) typhoid/paratyphoid fever tuberculosis osteomyelitis chronic septicaemia/bacteraemia infective endocarditis Lyme disease syphilis (secondary) Cancer (up to 30%):

lymphoma and leukaemia solid cancers (e.g. lung, kidney) disseminated Other: febrile neutropenia inflammatory bowel disease (e.g. Crohn) Pitfalls (often missed) Connective tissue disorder (e.g. rheumatoid arthritis, systemic lupus erythematosus) Sarcoidosis Drug idiosyncrasies Rarities: factitious fever Note: Up to 20% remain unknown.

History The history should include consideration of past history, occupation, travel history, sexual history, IV drug use (leads to endocarditis and abscesses), animal contact, medication and other relevant factors. Symptoms such as pruritus, a skin rash and fever patterns may provide clues for the diagnosis. The average patient with a difficult FUO needs to have a careful history taken on at least three separate occasions.14

Examination A common mistake is the tendency to examine the patient only once and not re-examine. The patient should be examined regularly (as for history taking) as physical signs can develop eventually. HIV infection must be excluded. Special attention should be paid to the following (see FIG. 42.3 ): skin—look for rashes, vesicles and nodules the eyes and ocular fundi temporal arteries sinuses and ears (canal and TM) teeth and oral cavity—?dental abscess, other signs heart—murmurs, pericardial rubs

lungs—abnormalities including consolidation, pleuritic rub abdomen—enlarged/tender liver, spleen or kidney rectal and pelvic examination (note genitalia) lymph nodes, especially cervical (supraclavicular) blood vessels, especially of the legs—?thrombosis urine (analysis)

FIGURE 42.3 Sites to consider in FUO

Investigations15 FUOs in primary care (not hospitalised) should be investigated as a ‘series’, rather than ordering everything at once in ‘parallel’. Start with a broad brush, then focus in on likely possibilities. Basic investigations include: haemoglobin, red cell indices and blood film white cell count ESR/C-reactive protein chest X-ray and sinus films urine examination (analysis and culture) routine blood chemistry blood cultures Further possible investigations (depending on clinical features): stool microscopy and culture

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culture of sputum (if any) specific tests for malaria, typhoid, EBM, Q fever, brucellosis, psittacosis, cytomegalovirus, toxoplasmosis, syphilis, various tropical diseases and others NAAT (e.g. PCR) tests HIV screening tests for rheumatic fever tuberculin test tests for connective tissue disorders (e.g. DNA antibodies, C-reactive protein) upper GIT series with small bowel follow-through CT and ultrasound scanning for primary and secondary neoplasia gall bladder functioning occult abscesses MRI—best for detecting lesions of the nervous system

echocardiography—for suspected endocarditis isotope scanning for specific causes aspiration or needle biopsy laparoscopy for suspected pelvic infection tissue biopsies (e.g. lymph nodes, skin, liver, bone marrow) as indicated

FUO in children Page 517 Fever in children is usually a transient phenomenon and subsides within 4–5 days. At least 70% of all infections are viral. Occasionally a child will present with FUO whose clinical symptoms and signs may be masked from antibiotic administration. Common causes of prolonged fever in children differ from those in adults. Most cases are not due to unusual or esoteric disorders,16 the majority representing atypical manifestation of common diseases.

A summary of the common causes (with the most common ranked first) is as follows.16

Infectious causes (40%) Viral syndrome Urinary tract infection Pneumonia Pharyngitis Sinusitis Meningitis

Collagen–vascular disorders (15%) Rheumatic arthritis Systemic lupus erythematosus Rheumatic fever Henoch–Schönlein syndrome

Neoplastic disorders (7%) Leukaemia

Reticulum cell sarcoma Lymphoma

Inflammatory diseases of the bowel (4%)

Septicaemia The diagnosis of septicaemia can be easily missed, especially in small children, the elderly and the immunocompromised, and in the absence of classic signs, which are: fever (± shivering) muscle pain rash (suggestive of meningococcus) tachycardia tachypnoea cool extremities Patients with septicaemia require urgent referral as it has a very high mortality rate.17 Investigations should include two sets of blood cultures and other appropriate cultures (e.g. urine, wound, sputum). Empirical initial treatment in adults (after blood cultures) is vancomycin IV and gentamicin IV.18

Glossary of terms Bacteraemia The transient presence of bacteria in the blood (usually implies asymptomatic) caused by local infection or trauma. Septicaemia (sepsis) The multiplication of bacteria or fungi in the blood, usually causing a systemic inflammatory response (SIRS). SIRS is defined as two or more of (in adults): temperature >38°C or 20/min heart rate >90/min WCC >12 × 109/L or 8–10 days a month

Practice tips Anyone >55 years presenting with unaccustomed headache has an organic disorder such as temporal arteritis (TA), intracerebral tumour or subdural haematoma until proved otherwise. The ESR is an excellent screening test to diagnose TA but occasionally can be normal in the presence of active TA. If a patient presents twice within 24 hours to the same practice or hospital with headache and vomiting, consider other causes apart from migraine before discharging the patient.9 If migraine attacks are severe and unusual (e.g. always on the same side) consider the possibility of cerebral vascular malformation. CT scans and MRI have superseded other investigations in the diagnosis of cerebral tumours and intracranial haemorrhage but should be ordered sparingly and judiciously. If a headache is occipital in origin or accompanied by neck pain, consider the likely possibility of cervical dysfunction and refer to the appropriate therapist once the diagnosis is established. For recurrent migraine sufferers emphasise the importance of trigger factor avoidance and of taking aspirin and metoclopramide medication at the earliest warning of an attack.

A severe headache of sudden onset is SAH until proved otherwise. It is overlooked sometimes. SAH is sometimes overlooked because it is not considered in the differential diagnosis. Suspect with very severe and protracted headache, drowsiness and neck stiffness. Medical evidence indicates that most headaches are related to fatigue, stress or migraine triggers and respond to application of heat or cold, exercise and common analgesics, including aspirin and ibuprofen.21 A history of migraine with aura over the past five years is a contraindication to prescribing the combined contraceptive pill (venous thromboembolism risk).14 The use of narcotics for migraine treatment (such as pethidine and codeine) is to be avoided whenever possible—the frequent use of ergotamine, analgesics or narcotics can transform episodic migraine into chronic daily headache.4 Migraine, especially chronic and recurrent, is associated with an increased risk of anxiety and depressive disorders.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition:

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Migraine Tension headache

References 1

World Health Organization. Headache disorders fact sheet. 8 April 2016. Available from: https://www.who.int/news-room/fact-sheets/detail/headache-disorders, accessed March 2021.

2

Wright M. Recurrent headaches in children. Australian Paediatric Review, 1991; 1(6): 1– 2.

3

Anthony M. Migraine and tension headache. In: MIMS Disease Index (2nd edn). Sydney: IMS Publishing, 1996: 313–16.

4

Joubert J. Diagnosing headache. Aust Fam Physician, 2005; 34(8): 621–5.

5

Beran R. Headache in Neurology for General Practitioners. Sydney: Elsevier, 2012: 46–

55. 6

Cormack J, Marinker M, Morrell D. The patient complaining of headache. In: Practice. London: Kluwer Medical, 1982: 3–12.

7

Lance JW. Headache and facial pain. Med J Aust, 2000; 172: 450–5.

8

Cheng S, Stark R. Thunderclap headache: when the risk of doing nothing is too high. Medicine Today, 2017; 18(7): 14–19.

9

Smith L. Childhood headache. In: Australian Doctor Education, GP Paediatrics, 2005.

10

Headache [published 2017]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2017. www.tg.org.au, accessed October 2017.

11

Burns R. Pitfalls in headache management. Aust Fam Physician, 1990; 19: 1821–6.

12

IHS Classification ICHD-3: 2013. Available from: www.ihs-classification.org/, accessed March 2021.

13

Day TJ. Migraine and other vascular headaches. Aust Fam Physician, 1990; 19: 1797–804.

14

Sexual and Reproductive Health [published 2020]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2020. www.tg.org.au, accessed March 2021.

15

Heywood J, Zagami A. Treating acute migraine attack. Current Therapeutics, 1997; 37(12): 33–7.

16

Buckley N (Chair). Australian Medicines Handbook. Adelaide: Australian Medicines Handbook Pty Ltd, 2018: 757–9.

17

Mannix LK et al. Combination treatment for menstrual migraine and dysmenorrhea using sumatriptan-naproxen: two randomized controlled trials. Obstet Gynecol, 2009; 114(1): 106–13.

18

Linde K et al. Acupuncture for the prevention of episodic migraine. Cochrane Database of Syst Rev, 2016; Issue 6: Art. No. CD001218.

19

Anthony M. The treatment of migraine—old methods, new ideas. Aust Fam Physician, 1993; 22: 1401–5.

20

Hutton R, Stark R. Unusual primary headaches: keys to an accurate diagnosis. Medicine Today, 2013; 14(10): 38–44.

21

Rosser W, Shafir MS. Evidence-Based Family Medicine. Hamilton: BC Decker Inc., 1998: 164–6.

Page 555

46 Hoarseness

Hoarseness results from imperfect phonation due to impairment of normal vocal cord mobility or vibration. It is an important symptom as it may signal a serious cause such as malignancy or a disease with potential for airway obstruction.1 RAYMOND L CARROLL 1996 Hoarseness (dysphonia) is defined as an altered voice due to a laryngeal disorder.2 It is an important symptom of laryngeal disease presenting in general practice, and ranges from the very common, trivial, self-limiting condition of viral upper respiratory tract infection to a lifethreatening disorder (see TABLE 46.1 ). It may be of sudden presentation lasting only a few days or develop gradually and persist for weeks or months. The cut-off point between acute and chronic hoarseness is three weeks’ duration, by which time most self-limiting conditions have resolved. Hoarseness pertains to harsh, raspy, gravelly or rough tones of voice rather than pitch or volume. Rarely, hoarseness can be a functional or deliberate symptom referred to as ‘hysterical aphonia’.3 In this condition, people purposely hold the cords apart while speaking. Table 46.1

Hoarseness: diagnostic strategy model

Probability diagnosis Viral URTI: acute laryngitis Non-specific irritative laryngitis (Reinke oedema) Vocal abuse (shouting, screaming, etc.) Nodules and polyps of cords Presbyphonia in elderly: ‘tired’ voice Acute tonsillitis Serious disorders not to be missed Cancer: larynx, lung, including recurrent laryngeal nerve palsy, oesophagus, thyroid Imminent airway obstruction (e.g. acute epiglottitis, croup) Other rare severe infections (e.g. TB, diphtheria) Foreign body

Motor neurone disease Aortic arch aneurysm Myasthenia gravis Pitfalls (often missed) Toxic fumes Vocal abuse Benign tumours of vocal cords (e.g. polyps, ‘singer’s nodules’, papillomas) Gastro-oesophageal reflux → pharyngolaryngitis Goitre Vocal cord palsy Dystonia Physical trauma (e.g. post-intubation), haematoma Fungal oropharyngeal infections (e.g. Candida with steroid inhalation, immunocompromised) Allergy (e.g. angioedema) Leukoplakia Vocal cord dysfunction ‘Floppy trachea’ syndrome Systemic autoimmune disorders (e.g. SLE, Wegener granulomatosis, myaesthenia gravis) Seven masquerades checklist Drugs: antipsychotics anabolic steroids opium users Smoking → non-specific laryngitis Steroids → steroid inhaler laryngitis Hypothyroidism, acromegaly Is the patient trying to tell me something? Functional aphonia Functional stridor Page 556

Key facts and checkpoints In acute hoarseness, the diagnosis is usually obvious from the history alone. Examples include acute upper respiratory tract infection (URTI), vocal overuse or

regular steroid inhalation. Think ‘hypothyroidism’ if unusual hoarseness develops. Laryngeal cancer must be excluded if hoarseness persists for longer than 3 weeks in an adult. It can arise intrinsic or extrinsic to the vocal cords. Intermittent hoarseness is invariably secondary to a benign disorder. Constant or progressive hoarseness suggests malignancy. Non-malignant vocal cord lesions, which include polyps, vocal nodules, contact ulcers, granulomas, other benign tumours and leukoplakia, account for about half of all chronic voice disorders. In cases of chronic hoarseness the larynx must be visualised for diagnosis but the following are common: children—‘screamer’s nodules’ adults—non-specific irritant laryngitis Acute laryngeal oedema may develop as a component of the life-threatening acute angioedemic allergic response. Elderly or debilitated patients may exhibit a shaky or soft ‘pseudohoarse’ voice due to a weakened respiratory effort. This is termed phonaesthenia or presbyphonia. Contact ulcers of the larynx occur on the posterior third of the vocal cords where the mucosa is thin. The resultant weak hoarse voice may be accompanied by painful phonation. The ulcers may develop into granulomas. Apart from intubation, the condition is usually found in forceful orators who misuse their larynx when attempting to lower the pitch of their voice.3

The clinical approach History Note the nature and duration of the voice change. Inquire about corticosteroid inhalations, excessive or unaccustomed voice straining (especially singing), recent surgery, possible reflux, smoking or exposure to environmental pollutants. Elicit associated respiratory or general symptoms such as cough and weight loss. Consider symptoms of hypothyroidism or Parkinson disease.

Examination

Palpate the neck for enlargement of the thyroid gland or cervical nodes. Perform a simple oropharyngeal examination except if epiglottitis is suspected. Check for signs of hypothyroidism, such as coarse dry hair and skin, slow pulse and mental slowing. With chest examination listen for stridor. Perform indirect laryngoscopy if skilled in the procedure. Note voice characteristics e.g.:4 raspy—laryngopharyngeal reflux deep—hypothyroidism, Reinke oedema gravelly—vocal cord mass/nodule soft—Parkinson disease, vocal cord paralysis intermittent—functional dysphonia, vocal cord dysfunction strained—muscle tension dysphonia

Investigations The following need to be considered: Thyroid function tests. Chest X-ray if it is possibly due to lung cancer with recurrent laryngeal nerve palsy. Indirect laryngoscopy (the gag reflex may preclude this). Direct laryngoscopy with a flexible fibre-optic endoscope with possible biopsy (the most sensitive investigation). The choice of imaging to detect suspected neoplasia or laryngeal trauma is a CT scan.

Red flags History of significant smoking Dysphagia/odynophagia/otalgia Neck mass Haemoptysis Stridor Constitutional, e.g. weight loss, fever

Management principles Acute hoarseness Treat according to cause. Advise vocal rest or minimal usage at normal conversational level. Avoid irritants (e.g. dust, tobacco, alcohol). Consider inhalations and cough suppressants in cases of acute URTI and coughing paroxysms.

Chronic hoarseness Establish the diagnosis. Consider referral to ENT specialist.

Hoarseness in children Page 557 It is worth bearing in mind that stridor in infants can be caused by a congenital abnormality of the larynx, including laryngomalacia (congenital laryngeal stridor), which is particularly noticeable when the child is asleep; laryngeal stenosis (congenital laryngeal narrowing); and laryngeal paralysis due to birth trauma of the vagus nerve. Vocal cord paralysis/palsy is the most common laryngeal abnormality in children (20% of cases) after laryngomalacia.3

In children exclude the acute infections—laryngotracheobronchitis (croup), tonsillitis and epiglottitis. Persistent hoarseness in kinder/primary school-aged children is due commonly to vocal cord nodules related to vocal abuse, such as screaming and yelling, often due to noisy children’s games. It is important to exclude a juvenile papilloma in a hoarse child.5

Specific conditions Acute laryngitis Most cases are caused by the respiratory viruses—rhinovirus, influenza, para-influenza, Coxsackie, adenovirus and respiratory syncytial virus—resulting in vocal cord oedema. Hoarseness is a useful feature to distinguish viral from bacterial upper respiratory infections,

although don’t discount group A Streptococcus. Short-term vocal abuse is also a factor. The main symptom is hoarseness, which usually persists for 3–14 days and leads to loss of voice. It is often self-limiting. Even speaking can be painful. Aggravating factors include smoking, excessive alcohol drinking and exposure to irritants and pollutants, air-conditioning systems and very cold weather.

Management Rest at home, including voice rest (the best treatment). Use the voice sparingly, avoid whispering. Use a warm sialagogue (e.g. hot lemon drinks). Drink ample fluids, especially water. Avoid smoking, passive smoke and alcohol. Have hot, steamy showers as humidity helps. Use steam inhalations (e.g. 5 minutes, 3 times a day). Use cough suppressants, especially mucolytic agents. Use simple analgesics, such as paracetamol or aspirin, for discomfort. Antibiotics are of no use unless there is evidence of bacterial infection (unusual). Corticosteroids are rarely indicated.

Chronic laryngitis: ‘barmaid syndrome’ This typically occurs in a heavy smoker who works in a heavy smoking environment, who is a heavy drinker and continually talks or sings. It is a combination of vocal abuse and chemical irritation. Hoarseness often comes and goes. Treatment involves modification of these factors and screening for vocal cord tumours.

Benign tumours of the vocal cords These include nodules (most common) (see FIG. 46.1 ), polyps (second most common), cysts and papules. Vocal cord nodules, including ‘singer’s nodules’, may respond well to conservative measures such as voice rest and vocal therapy. If not, they can be removed by microlaryngeal surgery or laser therapy. Dependent polyps and papillomas are removed by microsurgery.

FIGURE 46.1 Vocal cord nodules

Pharyngolaryngitis Also termed laryngopharingitis, it presents with a raspy voice, chronic throat clearing and GORD, e.g. heartburn. If due to laryngopharyngeal reflux, treat with an 8–12 week empirical course of proton-pump inhibitors as well as dietary and lifestyle modification.2 It tends to be overdiagnosed, but should be referred to an otorhinolaryngologist if symptoms are persistent.4

Steroid inhaler induced laryngitis4 This common problem should be evident and readily treatable. Examine for oral thrush and, if present, treat with antifungal medication. Check inhaler/spacer technique and advise rinsing and gargling after use. Page 558

Laryngeal cancer Squamous cell carcinoma usually occurs in people with a history of chronic laryngitis, smoking and alcohol use. Symptoms include hoarseness, stridor, haemoptysis and dysphagia. It may be preceded by leukoplakia, which is treated by vocal cord stripping under microsurgery. The diagnosis based on persistent hoarseness is made after fibre-optic laryngoscopy and biopsy by a specialist. The patient may present with an unexplained cervical lymph node. The condition is curable if detected early. Small local tumours can be treated by radiotherapy or laser therapy. Larger tumours usually require laryngectomy and perhaps dissection of the cervical lymph nodes (commando operation). Such radical surgery demands considerable patient support, including education about speech, eating and tracheostomy care.

Vocal cord dysfunction6

This condition is paradoxical vocal (or fold) adduction on inspiration and abduction on expiration, causing inspiratory airway obstruction and stridor. It tends to be misdiagnosed as asthma. Apart from dyspnoea, wheezing and stridor (usually inspiratory) symptoms may include intermittent hoarseness, chest and/or throat tightness, a noisy rasping sound and a choking or suffocating sensation. Patients may complain about a feeling ‘like breathing through a straw’. Diagnosis is by observing inspiratory closure of the vocal cords with direct laryngoscopy. The mainstay of treatment is speech therapy.

Excessive dynamic airways collapse (‘floppy trachea’)7 Also known as adult tracheobronchomalacia, this is defined as pathological collapse and narrowing of the airway lumen by ≥50%. It is due to laxity of the posterior membrane into the airway lumen (in the presence of structurally intact cartilage) during forced expiration. Symptoms include breathing difficulty, coughing, difficulty clearing secretions, dyspnoea and stridor. Respiratory failure and death can occur. Diagnosis is with CT scanning and fibre-optic bronchoscopy. Treatment varies from conservative to surgery (minimal to radical). Refer to a respiratory physician.

When to refer1 Acute cases that are unexplained, fail to respond by 3–4 weeks or recur; people >45 years. All chronic cases. Any case with stridor or non-tender cervical lymphadenopathy. Chronic hoarseness secondary to vocal abuse—refer for voice therapy.

Practice tips Consider intubation as a possible cause of transient hoarseness. Consider gastro-oesophageal reflux disease in the elderly but avoid such a diagnosis without specialist investigation for other causes. If stridor is present with acute hoarseness, the airway is compromised. Be on stand-by for possible emergency intervention. Prevention is the best treatment for laryngeal cancer (i.e. quit smoking). Recurrent laryngeal nerve palsy may be associated with cancer of the lung and mediastinum, or diabetes, or may be idiopathic.

References 1

Carroll RL. Hoarseness. In: MIMS Disease Index (2nd edn). Sydney: IMS Publishing, 1996: 239–40.

2

Bova R, McGuinness J. Hoarseness: a guide to voice disorders. Medicine Today, 2007; 8(2): 38–44.

3

Priestly J, Havas TE. Benign vocal fold lesions. Medical Observer, 19 November 2012: 1– 3.

4

Cooper L, Quested RA. Hoarseness: an approach for the general practitioner. Aust Fam Physician, 2016; 45(6): 378–81.

5

Birman C, Fitzsimons, Quayle S. Little voices: therapy update. Australian Doctor, 7 May 2004: 49–50.

6

Idrees M et al. Vocal cord dysfunction in bronchial asthma. A review article. J Asthma, 2015; 52(4): 327–35.

7

Murgu S, Colt H. Tracheobronchomalacia and excessive dynamic airway collapse. Clin Chest Med, 2013; 34(3): 527–55.

Page 559

47 Jaundice

The disease is produced by black bile when it flows into the liver. The symptoms are these: ‘an acute pain in the liver, also below the breast, a feeling of suffocation is strong during these days and becomes less strong later’. The liver is tender to palpation and the complexion of the patient is somewhat livid. These are symptoms that occur in the beginning but as the disease progresses, the fever diminishes in strength and the patient feels sated after ingesting a little amount of food. He must drink melikration [a mixture of water and honey]. HIPPOCRATES ON HEPATITIS Jaundice is a yellow discolouration of the skin and mucosal surfaces caused by the accumulation of excessive bilirubin.1 It is a cardinal symptom of hepatobiliary disease and haemolysis. Important common causes include gallstones, hepatitis A, hepatitis B, hepatitis C, drugs, alcohol and Gilbert syndrome. The commonest clinical encounter with jaundice is physiological jaundice in the newborn. As always, the history and examination are paramount, but investigations are essential to clinch the diagnosis of jaundice. The three major categories of jaundice are (see FIG. 47.1 obstructive: – extrahepatic – intrahepatic hepatocellular haemolytic

):

FIGURE 47.1 The jaundice pathway

Table 47.1

Abbreviations used in this chapter

Hepatitis A virus

HAV

Hepatitis A antibody

anti-HAV

Immunoglobulin M

IgM

Immunoglobulin G

IgG

Hepatitis B virus

HBV

Hepatitis B surface antigen

HBsAg

Hepatitis B surface antibody

anti-HBs

Hepatitis B core antibody

anti-HBc

Hepatitis B-e antigen

HBeAg

Hepatitis C virus

HCV

Hepatitis C virus antibody

anti-HCV

Hepatitis D (Delta) virus

HDV

Hepatitis E virus

HEV

Hepatitis F virus

HFV

Hepatitis G virus

HGV

Key facts and checkpoints Jaundice is defined as a serum bilirubin level exceeding 19 µmol/L.2 Clinical jaundice manifests only when the bilirubin level exceeds 50 µmol/L.1 However, jaundice is difficult to detect visually below 85 µmol/L if lighting is poor. Jaundice can be distinguished from yellow skin due to hypercarotenaemia (dietary excess of carrots, pumpkin, mangoes or pawpaw) and hypothyroidism because it involves the sclera. The most common causes of jaundice recorded in a general practice population are (in order) viral hepatitis, gallstones, pancreatic cancer, cirrhosis, pancreatitis and drugs.3 Always take a full travel, drug and hepatitis contact history in any patient presenting with jaundice. Acute hepatitis is usually self-limiting in patients with hepatitis A and in adults with hepatitis B but progresses to chronic infections with hepatitis C (curable) and children with hepatitis B (containable).4 A fatty liver (steatosis) can occur not only with alcohol excess but also with obesity, diabetes and starvation. There is usually no liver damage and thus no jaundice. Almost all patients with chronic hepatitis C will be cured with a course of oral directacting antiviral agents, but only if they are diagnosed, assessed, treated and monitored appropriately.

A diagnostic approach A summary of the diagnostic strategy model is presented in TABLE 47.2

.

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Table 47.2

Jaundice (adults): diagnostic strategy model

Probability diagnosis Hepatitis A, B, C (mainly B, C) Gallstones in common bile duct Alcoholic hepatitis/cirrhosis Serious disorders not to be missed Malignancy: pancreas biliary tract hepatocellular (hepatoma) metastases Severe infections: septicaemia ascending cholangitis fulminant hepatitis HIV/AIDS Rarities: Wilson disease Reye syndrome acute fatty liver of pregnancy Pitfalls (often missed) Gallstones Gilbert syndrome (↓ hepatic uptake) Cardiac failure Primary biliary cirrhosis Autoimmune chronic active hepatitis Primary sclerosing cholangitis Chronic viral hepatitis Haemochromatosis Viral infections (e.g. CMV, EBV) Leptospirosis Seven masquerades checklist Drugs (e.g. flucloxacillin) Anaemia Is the patient trying to tell me something?

Is the patient trying to tell me something? Not usually applicable.

Probability diagnosis The answer depends on the age and social grouping of the patient, especially if the patient indulges in risk-taking behaviour or has travelled overseas. Viral hepatitis A, B or C account for the majority of cases of jaundice. In the middle-aged and elderly group, a common cause is obstruction from gallstones or cancer. It is common for older people to have painless obstructive jaundice; bear in mind that the chances of malignancy increase with age. Alcoholic liver disease is common and may present as chronic alcoholic cirrhosis with liver failure or as acute alcoholic hepatitis. It is worth emphasising that such patients can make a dramatic recovery when they cease drinking alcohol. In family practice we encounter many cases of drug-induced jaundice, especially in the elderly. These drugs are outlined later in the chapter, under ‘Seven masquerades checklist’.

Serious disorders not to be missed Malignancy must always be suspected, especially in the elderly patient and those with a history of chronic active hepatitis (e.g. post-hepatitis B or C infection). The former is more likely to have carcinoma of the head of the pancreas, and the latter, hepatocellular carcinoma (hepatoma). Metastatic cancer must be kept in mind, especially in those with a history of surgery, such as large bowel cancer, melanoma and stomach cancer. An enlarged, knobbly, hard liver is a feature. Hepatic failure can be associated with severe systemic infection (e.g. septicaemia and Page 561 pneumonia), and after surgery in critically ill patients. The classic Charcot triad of upper abdominal pain, fever (and chills) and jaundice indicates ascending cholangitis until proved otherwise. Wilson syndrome, although rare, must be considered in all young people with acute hepatitis. A history of neurological symptoms, such as a tremor or a clumsy gait, and a family history is important. If Wilson disease is suspected, an ocular slit lamp examination, serum ceruloplasmin levels (low in 95% of patients) and a liver biopsy should be performed. Early diagnosis and treatment mean a better prognosis. Reye syndrome is a rare and severe complication of influenza and some other viral diseases, especially in children when given aspirin. There is rapid development of hepatic failure and encephalopathy.

Pitfalls

Gallstones, especially in the absence of upper abdominal pain, can be overlooked, so this possibility should be kept in mind in the elderly. Gilbert syndrome is worth considering, especially as it is the commonest form of unconjugated hyperbilirubinaemia. It affects at least 3% of the population and does not require treatment. Cardiac failure can present as jaundice with widespread tenderness under the right costal margin. It can be insidious in onset or manifest with gross acute failure. It can be confused with acute cholecystitis. The biochemical abnormalities seen are very variable. Usually there is a moderate rise in bilirubin and alkaline phosphatase and sometimes, in acute failure, a marked elevation of transaminase may occur, suggesting some hepatocellular necrosis. There are many other pitfalls for a family doctor, who may encounter the conditions very rarely, if at all. Such disorders include: inherited conjugated hyperbilirubinaemias (Dubin–Johnson and Rotor syndromes) caused by faulty excretion by liver cells haemochromatosis (associated pigmentation and diabetes) chronic active hepatitis primary biliary cirrhosis primary sclerosing cholangitis (associated with ulcerative colitis)

General pitfalls Excluding jaundice by examining the sclera in artificial light Not realising that the sclera in elderly patients often have an icteric appearance (without jaundice) Omitting to take a careful history, including illicit drugs Not referring for liver biopsy for all those with chronic hepatitis

Seven masquerades checklist Of this group the haemolytic anaemias and drugs have to be considered.

Drug-related jaundice Drug-induced jaundice is common and many drugs are implicated. The patterns of drug-related liver damage include cholestasis, necrosis (‘hepatitis’), granulomas, chronic active hepatitis, cirrhosis, hepatocellular tumours and veno-occlusive disease.4,5 Some drugs, such as methyldopa, can initiate haemolysis.

The important drugs to consider are presented in TABLE 47.3 . Antibiotics, especially flucloxacillin, amoxicillin + clavulanate and erythromycin, are commonly implicated. Table 47.3

Drugs that can cause jaundice

Haemolysis Methyldopa Hepatocellular damage Dose-dependent: paracetamol (can cause acute hepatic necrosis) salicylates tetracycline Dose-independent: anaesthetics (e.g. halothane) antidepressants (e.g. MAOIs, duloxetine) anti-epileptics (e.g. phenytoin, sodium valproate, carbamazepine) antibiotics (a long list, e.g. penicillins, sulfonamides) antimalarials (e.g. Fansidar) antiretrovirals (e.g. efavirenz, nevirapine) antituberculosis (e.g. isoniazid) anti-inflammatories (e.g. NSAIDs, various) carbon tetrachloride cardiovascular (e.g. amiodarone, methyldopa, hydralazine, perhexiline) statins (e.g. simvastatin) Cholestasis Anti-thyroid drugs Chlorpromazine Erythromycin estolate Penicillins, esp. flucloxacillin Gold salts Oral contraceptives/oestrogens Synthetic anabolic steroids (e.g. methyltestosterone) Hypoglycaemic drugs (e.g. chlorpropamide) Amitriptyline Others Allopurinol Cimetidine (aggravated by alcohol)

Page 562

DMARDs (e.g. methotrexate, azathioprine, ipilimumab) Immunomodulators (e.g. interferon, TNF-alpha I) Etretinate Nitrofurantoin Illicit drugs, e.g. MDMA/ecstasy, cocaine Vitamin A (mega dosage) Various complementary medicines (e.g. herbal agents)

Haemolysis The person may present with the symptoms of underlying anaemia and jaundice with no noticeable change in the appearance of the urine and stool. The degree of haemolysis may vary from the lemon-yellow tinge of pernicious anaemia in an elderly patient to a severe haemolytic crisis precipitated by drugs or broad beans (favism) in a person with an inherited red cell deficiency of glucose-6-phosphate dehydrogenase (G6PD). More common causes include the hereditary haemolytic anaemias, such as congenital spherocytosis and thalassaemia major. Acquired causes include incompatible blood transfusions, malignancies (such as lymphoma), severe sepsis and some drugs. Splenomegaly occurs in most people with haemolytic anaemia, and decreased red cell survival can be measured.

Psychogenic considerations This is not really applicable for an organic problem such as jaundice. Nevertheless, the cause may be related to lifestyle factors that the patient may be reluctant to reveal, such as sexual orientation or intravenous drug abuse. The GP’s skills at empathetic questioning become paramount.

Red flag pointers for jaundice Unexplained weight loss Progressive jaundice including painless jaundice Oedema Cerebral dysfunction (e.g. confusion, somnolence)

The clinical approach

History The history should include questioning about the following: any episodes of jaundice change in colour of faeces and urine anorexia, sore throat, weight loss, pruritus abdominal pain residence and members of household contact with others with hepatitis or jaundice recent overseas travel exposure to blood or blood products needle-stick injuries or exposure to needles, such as acupuncture, tattooing and intravenous drugs dietary history—shellfish, drinking water sexual history and orientation drug history, including alcohol, paracetamol recent medical history, including surgery

Page 563

family history—family contacts who have had jaundice, haemolytic disease and other genetic liver diseases ethnic history—liable to haemolytic disease, contact with hepatitis B occupational history—exposure to hazards

Significance of various symptoms Pain in the right hypochondrium: gallstones acute hepatitis (a constant ache) cholecystitis Anorexia, dark urine, fever:

viral hepatitis probable alcoholic liver disease possible drug-induced hepatitis possible Pruritus: cholestasis probable possible with all liver diseases Arthralgia, rash: viral hepatitis autoimmune hepatitis

Examination The abdominal examination is very important. The liver should be palpated carefully for enlargement, consistency and tenderness under the right costal margin. Search for enlargement of the gall bladder and the spleen. The gall bladder lies in the transpyloric line. A palpable gall bladder indicates extrahepatic biliary obstruction, and splenomegaly may indicate haemolytic anaemia, portal hypertension or viral hepatitis. Test for ascites. Skin excoriation may indicate pruritus, which is associated with cholestatic jaundice. Look for evidence of chronic liver disease, such as palmar erythema, easy bruising, spider naevi and muscle wasting, and testicular atrophy and gynaecomastia. Test for hepatic flap (asterixis) and fetor, which indicate liver failure. Search for lymphadenopathy, which may be indicative of malignancy. The examination should include dipstick urine testing for bilirubin and urobilinogen. A summary of the possible findings is presented in FIGURE 47.2

.

FIGURE 47.2 Possible findings on examining the jaundiced patient

Investigations The main investigations are FBE, LFTs and the standard viral serology for the infective causes, particularly hepatitis A, B and C virus (also EBV and CMV). A summary of the general findings for liver function tests is shown in TABLE 47.4 . Consideration should be given to ordering fractionalisation of bilirubin to determine whether it is conjugated or unconjugated (important in diagnosis of Gilbert syndrome). Table 47.4

Characteristic liver function tests for selected types of liver disease

Liver function tests (serological)

Hepatocellular (viral) hepatitis

Bilirubin

↑ to ↑ ↑ ↑

↑ unconjugated

Alkaline phosphatase

↑ 2N

N

↑ ↑ to ↑ ↑

↑ ↑ ↑ >5N

N

N or ↑

N

↑

Gammaglutamyl transferase

N or ↑

N

↑↑

N

↑

Albumin

N or ↓

N

N

N

N to ↓

Globulin

N or ↑

N

N

N

N

Obstruction

N: is within normal limits

Diagnostic markers for hepatitis Hepatitis A: IgM antibody (anti-HAV) Hepatitis B: surface antigen (HBsAg) Hepatitis C: HCV antibody (anti-HCV)

Hepatobiliary imaging Tests to identify causes such as malignancy or gallstones are now sophisticated and should be chosen with care. X-ray: a plain abdominal X-ray shows gallstones that are opaque (15–20%) Transabdominal ultrasound (US): the most useful investigation for detecting gallstones and dilatation of the common bile duct; also detects liver metastases and other diffuse liver diseases. The rapid increase in the diagnostic label of ‘fatty liver’ parallels the increased use of high resolution ultrasound. HIDA scintiscan: useful in diagnosis of acute cholecystitis CT scan: for diagnosis of enlargement of the head of the pancreas and other pathology; indicated if US unsatisfactory

Page 564

PTC: percutaneous transhepatic cholangiography: shows imaging of biliary tree ERCP: endoscopic retrograde cholangiopancreatography; PTC and ERCP (best) determine the cause of the obstruction and relieves it by sphincterotomy and removal of CBD stones MRCP: magnetic resonance cholangiopancreatography provides non-invasive planning for obstructive jaundice Liver isotopic scan: useful for liver cirrhosis, especially of the left lobe Fibroscan (transient elastography) to measure liver fibrosis

Specific tests Some specific tests include: autoantibodies for autoimmune chronic active hepatitis and primary biliary cirrhosis carcinoembryonic antigen to detect liver secondaries, especially colorectal serum iron studies, especially transferrin saturation—elevated in haemochromatosis alpha-fetoprotein—elevated in hepatocellular carcinoma; mild elevation with acute or chronic liver disease (e.g. cirrhosis) serum ceruloplasmin level—low in Wilson disease liver biopsy EBV/cytomegalovirus serology (consider if hepatitis serology negative) Page 565

Jaundice in children Jaundice in the infant Jaundice in the newborn is clinically apparent in 50% of term babies and more than 80% of preterm.6 Icterus is therefore common and almost invariably physiologically benign. However, it is a cause for concern as there are many other causes and investigation is needed to determine whether the bilirubin is conjugated (always pathological) or unconjugated. If conjugated, consider the serious biliary atresia (stools are white); also a cyst obstructing the bile duct or neonatal hepatitis. Prompt referral is essential. Jaundice occurring in the first 24 hours after birth is not due to immature liver function but is pathological and usually due to haemolysis consequent on blood group incompatibility. In primigravidas it is usually due to ABO incompatibility. Rhesus factor is the most severe form of alloimmune haemolytic disease of the newborn. At birth, it presents with hydrops, anaemia,

jaundice and hepatosplenomegaly. Prevention is with injections of anti-D immunoglobulin for Rh negative women at 28 and 34–36 weeks. Causes of pathological jaundice are presented in TABLE 47.5 Table 47.5

Such causes demand referral.

Pathological neonatal jaundice: diagnostic strategy model (brief)

Probability diagnosis Physiological: day 2–5 ABO incompatibility: first 24 hours Breast milk jaundice: late first week Serious disorders not to be missed ABOI: first 24 hours Biliary atresia (conjugated) Haemolysis: ABOI, G6PD, Rhesus incompatibility Sepsis Seven masquerades checklist Drugs Hypothyroidism Hereditary spherocytosis, other inherited disorders Polycythaemia

Bilirubin encephalopathy Unconjugated bilirubin can be regarded as a neurological poison. With increasing serum levels an encephalopathy (which may be transient) can develop, but if persistent can lead to the irreversible brain damage known as kernicterus. The level of bilirubin causing kernicterus is totally unpredictable, but a guideline as a cause for concern in babies with Rh disease is a serum unconjugated bilirubin of 340 µmol/L (20 mg/dL). Guidelines for treatment for hyperbilirubinaemia (at 24–36 hours)—an example: >285 µmol/L—phototherapy >360 µmol/L—consider exchange transfusion An example of a normogram is presented in FIGURE 47.3

.

FIGURE 47.3 Typical normogram for decision making in healthy infants with jaundice

Physiological jaundice This mild form of jaundice, which is very common in infants, is really a diagnosis of exclusion. In a term infant the serum bilirubin rises quickly after birth to reach a maximum by day 3–5, then declines rapidly over the next 2–3 days before fading more slowly for the next 1–2 weeks. Management includes phototherapy.

ABO blood group incompatibility This is antibody-mediated haemolysis (Coomb test positive): Mother is O Child is A or B Jaundice develops within first 24 hours. Page 566

Treatment Perform a direct Coomb test on infant. Phototherapy is required immediately.

These children require follow-up developmental assessment including audiometry.

Breast milk jaundice If the secondary causes of prolonged jaundice are excluded, the baby is well and breastfeeding, the likely cause of unconjugated elevated bilirubin is breast milk jaundice. It occurs in 2–4% of breastfed infants. It usually begins late in the first week and peaks at 2–3 weeks. Diagnosis can be confirmed by suspending (not stopping) breastfeeding for 24–48 hours, although some doctors recommend against this. The serum bilirubin falls and then breastfeeding can continue. The mother, who can express milk for this short period, must be reassured that there is nothing wrong with the milk and advised to resume.

Jaundice in older children Viral infection is the commonest cause of jaundice in the older child, especially hepatitis A and hepatitis B. It is uncommon for viral hepatitis to become chronic in childhood.

Jaundice in the elderly If an elderly person presents with jaundice the usual causes and investigations have to be considered. Obstructive jaundice is the commonest form of jaundice in the elderly and may be caused by gallstones blocking the common bile duct (may be painless) and carcinoma of the head of the pancreas, the biliary tract itself, the stomach or multiple secondaries for other sites. While it is not uncommon for a gallstone to produce marked obstructive jaundice and yet be painless, it is appropriate to adhere to the old adage that painless obstructive jaundice is due to neoplasm—particularly if the gall bladder is palpable (Courvoisier’s law). Alcoholic liver disease, although most frequently affecting those aged 40 to 60 years, can present for the first time over age 60 years. The commonest cause of hepatocellular jaundice in the elderly is probably alcoholic cirrhosis; hepatitis A is still relatively uncommon in old persons. Drugs do not cause jaundice in the elderly as frequently as they once did, particularly as phenothiazines, especially chlorpromazine, are not prescribed as often as previously. However, drugs should be considered as a potential cause and a careful check of the drug history is important.

Infective causes of jaundice A generation ago hepatitis A (infectious hepatitis or yellow jaundice) was the commonest recognised form of viral hepatitis, presenting usually with an abrupt onset of fever, anorexia, nausea and vomiting. It usually occurred in epidemics and hence was common in overcrowded institutions and camps. Now hepatitis B and C are the most commonly reported types of viral hepatitis with an onset that is more insidious and with a longer incubation period.4,7 Symptoms include malaise, anorexia, nausea and polyarthritis. Acute hepatitis C is often subclinical.

The various forms of hepatitis are summarised in TABLE 47.6 . All forms of hepatitis are common in developing countries and travellers are at risk of contracting these infections: hepatitis A and E from faeco-oral transmission; and hepatitis B, C, D and G from intravenous drugs and bodily fluids (from sexual transmission, in particular, for hepatitis B). Table 47.6

Characteristic profiles of viral hepatitis A–E

Characteristic

Hepatitis A

Hepatitis B

Hepatitis C

Hepatitis D

Hepatitis E

Pseudonyms

Infectious hepatitis

Serum hepatitis

Parenterally transmitted non-A, nonB

Delta hepatitis

Enterically transmitted non-A, non-

Agent (virus)

27 nm RNA

42 nm DNA

50 nm RNA

35 nm RNA

30 nm RNA

Transmission

Faecal–oral Contaminated water/food

Blood and other body fluids Mother to child

Infected blood ?Other body fluids

Blood and other body fluids

Faecal–ora Contamina water/food

Incubation period

15–45 days

40–180 days

14–180 days

30–50 days

15–45 days

Severity of acute illness

Mild to moderate; often subclinical—no jaundice

Mild to severe; jaundice common; arthralgia and rash common

Mild to moderate; often subclinical

Moderate to severe; high mortality; usually jaundice

Mild to moderate; often subclinical

Chronic liver disease

No

Yes 10– 15%

Yes 65– 80%

Yes Potentially worst

No

Carrier state

No

Yes

Yes

Yes

No

Risk in travellers

Yes, applies to all A–E: East and South-East Asia, Asian subcontinent (e.g. India), South Pacific Islands (e.g. Fiji), sub-Saharan Africa, Mexico, Russia, other developing countries. A and E with poor sanitation; B, C, D also with I drug use; B, D sexual contact.

Antigens

HAV Ag

HBsAg, HBcAg, HBeAg

HCV Ag

HDV Ag

HEV Ag

Diagnosis— antibodies

anti-HAV

HBsAg anti-HBc anti-HBs

anti-HCV

anti-HDV

anti-HEV

Vaccine

Hepatitis A vaccine

Hepatitis B vaccine

None

Hepatitis B vaccine

None

Curable

No progression

No

Yes

No

Variable

Evidence points to more viruses causing non-ABC hepatitis.8 Hepatitis F virus has been claimed to be transmitted enterically while the newly designated hepatitis G virus (HGV) is transmitted parenterally. It does not appear to cause a severe illness in recipients. It can be predicted that the hepatitis alphabet will continue to expand. In hepatitis A, liver damage is directly due to the virus, but in hepatitis B and C it is due to an immunologic reaction to the virus. Other infections that can present with jaundice as part of a systemic disease are malaria, Epstein– Barr mononucleosis, cytomegalovirus, Q fever, toxoplasmosis, syphilis, leptospirosis and, rarely, measles, varicella, yellow fever, rubella, herpes simplex, dengue fever, Lassa fever and Marburg and Ebola virus.

Hepatitis A Hepatitis A is becoming relatively less prevalent in developed countries. It is enterically transmitted and arises from the ingestion of contaminated food, such as shellfish, or water. There is no carrier state and it does not cause chronic liver disease. Hepatitis A most often causes a subclinical or self-limited clinical illness.

Clinical features Pre-icteric (prodromal) phase: anorexia, nausea ± vomiting malaise headache distaste for cigarettes in smokers

mild fever ± diarrhoea

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± upper abdominal discomfort Icteric phase (many patients do not develop jaundice): dark urine pale stools hepatomegaly splenomegaly (palpable in 10%) Recovery usually in 1–10 weeks (average 6 weeks). Fulminant hepatitis with liver coma and death may occur but is rare.

Investigations LFTs and viral markers confirm the diagnosis. The antibodies to HAV are IgM, which indicates active infection, and IgG antibodies, which means past infection and lifelong immunity and which is common in the general population. Ultrasound is useful to exclude bile duct obstruction, especially in an older patient.

Outcome and treatment Hepatitis A has an excellent prognosis with most patients making a complete recovery, and patients should be reassured. The mortality is less than 0.5%. Admission to hospital is not usually necessary. There is no specific treatment, so management is as follows. Provide appropriate reassurance and patient education. Rest as appropriate. Follow a fat-free diet. Avoid alcohol, smoking and hepatotoxic drugs (until recovery). Advise on hygiene at home to prevent spread to close contacts and family members. Hep A can also be spread sexually and by IV drug use. Wash hands carefully after using the toilet and disinfect them with antiseptic. Do not handle food for others with your fingers. Do not share cutlery and crockery during meals.

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Do not use tea-towels to dry dishes.

Prevention Simple health measures such as good sanitation, effective garbage disposal and handwashing are probably responsible for the major decrease in the disease. Immune serum globulin (0.03–0.06 mL/kg IM) confers satisfactory passive immunity for close contacts (within 2 weeks of contact) and for travellers to endemic areas for up to 3 months. An active vaccine consisting of a two-dose primary course is the best means of prevention.

Hepatitis B Hepatitis B has protean clinical manifestations. Transmission is by blood spread, percutaneous, sexual transmission, perinatal spread or by close prolonged family contact. Infection may be subclinical or self-limited acute hepatitis. Fulminant hepatitis is rare. Five per cent of subjects go on to become chronic carriers of the virus. Most are ‘healthy carriers’ but some may develop chronic active hepatitis, cirrhosis and hepatoma. The serology of hepatitis B involves antibody responses to the four main antigens of the virus (core, DNA polymerase, protein X and surface antigens). Passive and active vaccines are available, and should be used freely in groups at risk, including babies of infected mothers. High-risk groups are presented in TABLE 47.7 . The clinical features are the same as those found in hepatitis A infection but may be less abrupt in onset but more severe in the long term.7 A serum sickness-like immunological syndrome may be seen with transient rashes (e.g. urticaria or a maculopapular rash), and polyarthritis affecting small joints in up to 25% of cases in the prodromal period. Table 47.7

Higher-risk groups for contracting hepatitis B (vaccination advisable)7

Babies born to hepatitis B positive (carrier) mothers Migrants from high prevalence regions, e.g. Africa, Asia Garbage collectors Health care workers Household contacts of hepatitis B carriers Institutionalised people with intellectual disability Users of intravenous drugs People on kidney dialysis Men who have sex with men Prisoners Recipients of blood or blood products (prior to testing)

Sex industry workers Sexual partners of hepatitis B carriers (especially acute HBV) Travellers to endemic areas

Investigations5,9,10 The main viral investigation for HBV is HBsAg (surface antigen), which is searched for routinely. If detected, indicating hepatitis B positive or carrier, a full viral profile is then formed. HBeAg is a soluble protein from the pre-core and core. Antibodies develop to both HBsAg and HBeAg. HBsAg may disappear or persist. Its presence indicates a current or chronic infection as well as a carrier state (see FIG. 47.4 ).

FIGURE 47.4 Time course of acute hepatitis B infection Chronic hepatitis B (carriage) is defined as the presence of HBsAg for at least 6 months. The typical natural history model of chronic hepatitis B infection is outlined in FIGURE 47.5 . The revised four phases of the chronic infection are: 1. Immune tolerant—HBeAg +ve; normal LFTs 2. Immune clearance—HBeAg +ve or −ve chronic hepatitis; abnormal LFTs 3. Immune control—inactive carrier (residual) state; HBeAg −ve; normal LFTs 4. Immune escape (reactivation)—HBeAg −ve; anti-HBe +ve; abnormal LFTs

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Practice tip Phase 2: Spontaneous seroconversion 1 Page 572

Hepatitis E Hepatitis E is an enterically transmitted virus that occurs in outbreaks in certain countries with a poor water supply, such as some Asian subcontinent countries. Epidemiologically, HEV behaves like HAV, with well-documented water-borne epidemics in areas of poor sanitation. There is a high case fatality rate in endemic areas (10–20%) and in pregnant females.

Hepatitis F Researchers claim to have identified HGF virus, which is spread enterically.18 Treatment can be comfortably managed within general practice.

Hepatitis G HGV has been identified as a transfusion-spread virus. It has subsequently been found to be prevalent among Queensland blood donors.10,19

Cholestatic jaundice Cholestasis refers to the syndrome of biliary obstructive jaundice whereby there is obstruction to the flow of bile from the hepatocyte to the duodenum, thus causing bilirubin to accumulate in the blood. It is classified into two main groups: intrahepatic cholestasis—at the hepatocyte or intrahepatic biliary tree level extrahepatic cholestasis—obstruction in the large bile ducts by stones or bile sludge The significant causes are listed in TABLE 47.8 Table 47.8

.

Significant causes of cholestasis in adults

Intrahepatic Alcoholic hepatitis/cirrhosis Drugs Primary biliary cirrhosis Viral hepatitis

Extrahepatic Cancer of bile ducts Cancer of pancreas Other cancer: primary or secondary spread Cholangitis Primary sclerosing cholangitis (?autoimmune) IgG4-related disease Common bile duct gallstones Pancreatitis Postsurgical biliary stricture or oedema

Symptoms Jaundice (greenish tinge) Dark urine and pale stools Pruritus—worse on palms and soles Pain varies from nil to severe

Gallstones and jaundice Gallstones can be found in the following (see FIG. 47.8

):

gall bladder (asymptomatic up to 75%)—the majority remain here neck of gall bladder (biliary ‘colic’ or acute cholecystitis) cystic duct (biliary ‘colic’ or acute cholecystitis) common bile duct—may cause severe biliary ‘colic’, cholestatic jaundice or cholangitis

FIGURE 47.8 Clinical presentation of gallstones Acute cholecystitis is accompanied by mild jaundice in 25% of cases, due to accompanying common duct stones.17 Common bile duct stones may be asymptomatic or may present with any one or all of the triad of abdominal pain, jaundice and fever. The jaundice varies, depending on the amount of obstruction. The liver is moderately enlarged if the obstruction lasts for more than a few hours. The investigations of choice for cholestatic jaundice are ultrasound and ERCP. Page 573

Acalculous cholecystitis Acute—in very ill patients often with diabetes. Chronic—associated with polyps, sludge or inflammation.

Acute cholangitis This is due to bacterial infection of the bile ducts secondary to abnormalities of the bile duct, especially gallstones in the common duct. Other causes are neoplasms and biliary strictures. Charcot triad (present in 70%) is shown in the diagnosis box.

DxT fever (often with rigor) + upper abdominal pain + jaundice → acute cholangitis

Older patients can present with circulatory collapse and Gram-negative septicaemia. Urgent referral is necessary.

Carcinoma of head of the pancreas Pancreatic cancer is the fourth commonest cause of cancer death in the UK and US.17

Clinical features M>F Mainly >60 years of age Obstructive jaundice Pain (over 75%)—epigastric and back Enlarged gall bladder (50–75%) Possible features Weight loss, malaise, diarrhoea Migratory thrombophlebitis Palpable hard, fixed mass Metastases (e.g. left supraclavicular gland of Virchow—Troisier sign) Occult blood in stool Glycosuria

Diagnosis Scanning by ultrasound or CT scan may show mass ERCP DxT jaundice + constitutional symptoms (malaise, anorexia, weight loss) + epigastric pain (radiating to back) → pancreatic cancer

Prognosis Prognosis is poor: 5-year survival rates vary from 5–11%.

Cirrhosis of the liver Cirrhosis is accompanied by jaundice as a late and serious manifestation with the exception of primary biliary cirrhosis, where jaundice appears before advanced liver failure. The development of jaundice usually indicates that there is minimal hepatic reserve and is therefore found in conjunction with other signs of liver failure (see FIG. 47.9 ).

FIGURE 47.9 Possible features of chronic alcoholic liver disease

Causes Common: alcohol excess chronic viral hepatitis (esp. HBV, HCV) Others: autoimmune chronic active hepatitis primary biliary cirrhosis (autoimmune) haemochromatosis Wilson disease drugs (e.g. methotrexate) cryptogenic (no cause found)

Clinical features Anorexia, nausea ± vomiting Swelling of legs Abdominal distension Bleeding tendency Drowsiness, confusion or coma (if liver failure)

Signs Spider naevi (distribution of superior vena cava) Palmar erythema of hands Peripheral oedema and ascites Jaundice (obstructive or hepatocellular) Enlarged tender liver (small liver in long-term cirrhosis) Ascites Gynaecomastia

± Splenomegaly (portal hypertension)

Investigations FBE Fibroscan ± Biopsy

Complications Ascites Portal hypertension and GIT haemorrhage Portosystemic encephalopathy Hepatoma Kidney failure Page 574

Autoimmune chronic active hepatitis (ACAH)5 Also termed idiopathic ACAH, this usually affects young females (10–40 years) who present insidiously with progressive fatigue, anorexia and jaundice. Diagnosis is made by abnormal LFTs (which should raise suspicion), positive smooth muscle antibodies, a variety of other autoantibodies and a typical liver biopsy. If untreated, most patients die within 3–5 years. Treatment is with prednisolone orally, monitored according to serum alanine aminotransferase levels, and supplemented with azathioprine or mercaptopurine. About 80% respond, while 20% develop chronic liver disease. Specialist referral is advisable.

Primary sclerosing cholangitis5 This uncommon inflammatory disorder of the biliary tract presents with progressive jaundice and other features of cholestasis such as pruritus. It is often associated with ulcerative colitis. Diagnosis is based on characteristic cholangiopancreatography findings. There is no specific therapy, but refer for possible ERCP. Ursodeoxycholic acid may benefit some patients. Patients have an increased risk of colorectal cancer.

Primary biliary cirrhosis5 Page 575 This is an uncommon cause of chronic liver disease that often presents with pruritis, malaise and an obstructive pattern of liver biochemistry. Found mainly in women. Treatment is with ursodeoxycholic acid orally.

Alcoholic liver disease The main effects of alcohol excess on the liver are: acute alcoholic liver disease fatty liver alcoholic hepatitis (progresses to cirrhosis if alcohol consumption continues) alcoholic cirrhosis If diagnosed, patients are advised to stop drinking alcohol for life except for fatty liver when small amounts can be drunk later.

Fatty liver Alcohol can cause hepatic steatosis (fatty liver), which is almost universal in obese alcoholics. Non-alcoholic causes include obesity, diabetes mellitus, hypertriglyceridaemia and corticosteroids. A significant number with this very common condition (one in five Australians) will develop cirrhosis. Fatty liver is usually asymptomatic but some complain of malaise and tiredness. Serology is unhelpful. Diagnosis is by liver biopsy and perhaps CT scan. The treatment is weight loss through diet, which improves liver function and reduces fatty deposits.

Haemochromatosis See CHAPTER 23

.

Special patient groups The returned overseas traveller The overseas traveller presenting with jaundice may have been infected by any one of the viruses —hepatitis A, B, C, D or E. All are prevalent in developing countries, especially in south-eastern and eastern Asia, some Pacific islands and Africa. Other causes to consider are malaria, ascending cholangitis and drug-induced hepatic damage due to, for example, the antimalarials, including mefloquine (Lariam) and Fansidar. Refer to CHAPTER 129 .

Jaundice during pregnancy Important hepatic disorders in pregnancy leading to jaundice are intrahepatic cholestasis of pregnancy, acute fatty liver of pregnancy and severe pre-eclampsia. Delivery is advisable at 37– 38 weeks.

Postoperative jaundice There are many possible causes of postoperative jaundice either in the immediate or the longterm postoperative phase. Hypoxia associated with shock in a severely ill patient or in a patient with cardiopulmonary disease may lead to transient abnormalities in liver function. Other causes include: post-transfusion hepatitis coincident viral hepatitis drugs, including anaesthetics transfusion overload (haemolysis) sepsis unmasked chronic liver disease and biliary tract disease cholestasis: post major abdominal surgery

Neonates of HBeAg positive mothers These neonates should have the following (see CHAPTER 101

):

hepatitis B immunoglobulin IM within 24 hours of birth hepatitis B vaccine at birth, 1 month and 6 months The vaccine may fail because some infants can be infected in utero.

When to refer All patients with fulminant hepatitis All patients with chronic liver disease or cirrhosis Painless obstructive jaundice Evidence of malignancy Symptomatic gallstones Acute fatty liver of pregnancy (very urgent) Suspected rare conditions (e.g. Wilson syndrome)

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Practice tips All drugs should be suspected as potential hepatotoxins. With hepatitis A, the presence of IgM antibodies reflects recent infection, and IgG antibodies indicate past infection and lifelong immunity. There is no chronic carrier state of hepatitis A and E. All patients with jaundice should be tested for hepatitis B surface antigen (HBsAg). Hepatitis B infection is usually benign and short-lived, but it can be fatal if chronic hepatitis develops, which may lead later to cirrhosis and hepatocellular carcinoma. Up to 5% of patients with hepatitis B will become chronic carriers (especially users of IV drugs). Such carriers are identified by persistent titres of HBsAg and possibly HBeAg, the latter indicating the presence of the whole virus, and active replication and high infectivity. A raised gamma-glutamyl transferase accompanied by a raised MCV is a good screening test for alcohol abuse. A systolic murmur may be heard over the liver in alcoholic hepatitis and hepatoma. A distaste for smoking (with jaundice) suggests acute viral hepatitis. Life expectancy is short with ascites.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Fatty liver Gallstones Hepatitis A Hepatitis B Hepatitis C

References 1

Kincaid-Smith P, Larkins R, Whelan G. Problems in Clinical Medicine. Sydney: McLennan & Petty, 1989: 251.

2

Coffman D, Chalstrey J, Smith-Laing G. Gastrointestinal Disorders. Edinburgh: Churchill Livingstone, 1986: 106.

3

Sandler G, Fry J. Early Clinical Diagnosis. Lancaster: MTP Press, 1986: 468–90.

4

Croagh C, Desmond D. Viral hepatitis: an A, B, C guide. Medicine Today, 2007; 8(7): 47– 56.

5

Viral hepatitis. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited. www.tg.org.au, accessed October 2019.

6

Gwee A, Rimer R, Marks M. Paediatric Handbook (9th edn). Melbourne: Blackwell Science, 2015: 335–6.

7

Ruff TA, Gust I. Hepatitis, viral (acute and chronic). In: MIMS Disease Index (2nd edn). Sydney: IMS Publishing, 1996: 226–30.

8

Bowden DS, Moaven LD, Locarnini SA. New hepatitis viruses: are there enough letters in the alphabet? Med J Aust, 1996; 164: 87–9.

9

Cossart Y. Recent advances in diagnosis and management of viral hepatitis. Common sense pathology. RCPA + Australian Doctor, 2006: 2–8.

10

World Health Organization. Guidelines for the prevention, care and treatment of persons with chronic hepatitis B infections. Geneva: WHO, 2015.

11

Liaw YF, Chi CM. Hepatitis B viral infection. Lancet, 2009; 373: 582–92.

12

Singal DK, George J. Chronic hepatitis C. Australian Doctor, 15 February 2001: i–viii.

13

Webster DP, Klerneman P, Dusheiko GM. Hepatitis C. Lancet, 2015; 385(9973): 1124– 35.

14

Khoo A, Tse E. A practical overview of the treatment of chronic hepatitis C virus infection. AJGP, 2016; 45(10): 718–20.

15

Baker D. Curing hepatitis C in general practice. A 12-step guide. Medicine Today, 2016; 17(10): 14–22.

16

Strasser S. Managing hepatitis C in general practice. Aust Prescr, 2017; 40: 64–9.

17

McPhee SJ et al. Current Medical Diagnosis and Treatment (49th edn). New York: The McGraw-Hill Companies, 2010: 636.

18

Deka N, Sharma MD, Mukerjee R. Isolation of the novel agent from human stool that is associated with sporadic human hepatitis. J Virol, 1994; 68: 7810–15.

19

Moaven LD et al. Prevalence of hepatitis G virus in Queensland blood donors. Med J Aust, 1996; 165: 369–71.

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48 Nasal disorders

The face of Mrs Gamp—the nose in particular—was somewhat red and swollen, and it was difficult to enjoy her society without becoming conscious of the smell of spirits. CHARLES DICKENS (1812–1870), MARTIN CHUZZLEWIT Disorders of the nose, which include the everyday problems of rhinitis, postnasal drip, epistaxis, folliculitis and disorders of smell, are very common in everyday general practice. The main functions of the nose are: airflow filtration—of dust, organisms and other air-borne particles olfaction (smell) self-cleansing and moisturising of the mucous membrane humidification and warming of air in its passage to the lungs vocal resonance The main symptoms of nasal disorders are discharge, blockage, sneezing, anosmia, itching, postnasal drip, bleeding and snoring (see TABLE 48.1 ).1 Table 48.1

Typical symptoms for nasal disorders2

Foreign body

Unilateral discharge, unilateral blockage

Acute sinusitis

Facial pain, toothache, nasal discharge, postnasal drip

Allergic rhinitis

Sneezing, rhinorrhoea, itch, eye irritation

Infective rhinitis

Blockage, purulent discharge, postnasal drip

Deviated septum

Blockage, postnasal drip

Nasal polyps

Blockage, reduced sense of smell

Nasal tumour

Blockage, unilateral discharge, epistaxis

Adenoidal hypertrophy

Bilateral blockage, snoring, halitosis

Nasal vestibulitis

Local pain, crusting, malodour

Nasal discharge is a common and important symptom to evaluate. The characteristics of nasal discharge are summarised in TABLE 48.2 . Table 48.2

Characteristics of nasal discharge

Nature of discharge Think of Blood Neoplasia, trauma, bleeding disorder, rhinitis, infection, hypertension Mucopurulent

Bacterial rhinitis, foreign body

Serosanguineous

Neoplasia, foreign body

Watery/mucoid

Viral rhinitis, allergic rhinitis, vasomotor rhinitis, CSF

A major presenting problem is nasal obstruction with the complaint of a blocked or ‘stuffy’ nose. In those without a current URTI, common causes are physiological (the nasal cycle), rhinosinusitis (allergic or non-allergic), polyps, adenoid hypertrophy and mechanical such as septal deformity.

Red flag pointers for nasal disorders Unilateral nasal ‘polyp’ Unilateral bloodstained discharge Toddler with offensive nasal discharge, esp. unilateral Post-traumatic periseptal swelling Rhinitis medicamentosa Chronic sinusitis + LRTI = ?Wegener granulomatosis

Disorders of smell

The basic sense of smell is detected in the olfactory region by the olfactory nerve (cranial nerve I) while irritant sensors in the nose, mediated by the maxillary branch of the trigeminal nerve (cranial nerve V), detect some noxious odours. The disorders can be classified as:3 anosmia—no smell hyposmia—reduced smell hyperosmia—increased sensitivity to odours dysosmia—distortion of smell perception

Page 578

cacosmia—normal odours seem foul or unpleasant parosmia—a perverse sense of smell Disorders of smell can be caused by conductive or sensorineural disturbances, part of normal ageing or considered as idiopathic (see TABLE 48.3 ). Conductive disorders present as anosmia or hyposmia, while sensorineural disorders can present with all of the above disorders.3 Most cases of idiopathic anosmia are considered to be viral neuropathies and may last from a few days to several months. Head trauma, which can cause conductive or sensorineural disturbances, is considered to be caused either from a fracture of the skull involving the cribriform plate or, more commonly, by posterior head trauma. Some patients will never recover their sense of smell. There is no effective treatment. Those with anosmia lack flavour discrimination and often have accompanying loss of sense of taste. They are also vulnerable to an unawareness of smoke, gas, dangerous chemicals and unhealthy food. Table 48.3

Causes of reduced sense of smell

Conductive defects Head trauma Nasal polyps Septal deviation Rhinitis and sinusitis Rare (not to be missed) Nasal tumour Wegener granulomatosis Central/sensorineural defects Ageing

Chemicals (e.g. benzene, chlorine, formaldehyde, cement dust) Cigarette and other smoking/inhalation Drugs Endocrine disorders (e.g. diabetes, hypothyroidism) Frontal lobe tumour Parkinson disease Head trauma Kallmann syndrome (anosmia + hypogonadism) Nutritional deficiencies Viral infections

The clinical approach History: head injury or surgery, recent URTI, drugs, occupation including chemical exposure Physical examination, including inspection via a Thudicum nasal speculum Sniff test—qualitative and quantitative odours (e.g. coffee, cloves, lemon, peppermint, water placebo). Ammonia (for irritant sensation) Investigations (e.g. CT scan for sinus disease, nasal polyps)

Treatment of anosmia2,4 Explanation and reassurance Education about smoke detectors, caution about chemicals including gas, excessive perfume, food safety including milk and meat contamination Consider dietary supplement (weak supportive evidence) with daily zinc sulphate, vitamin A and thiamine For chronic anosmia following an URTI: prescribe a nasal decongestant such as Spray-Tish Menthol for 5–7 days

Rhinitis Rhinitis is inflammation of the nose causing sneezing, nasal discharge or blockage for more than an hour during the day. Rhinitis is subdivided into various types:

According to time span: seasonal rhinitis: occurs only during a limited period, usually springtime perennial rhinitis: present throughout the year According to pathophysiology: allergic rhinitis: an IgE-mediated atopic disorder vasomotor rhinitis: due to parasympathetic overactivity Both allergic and vasomotor rhinitis have a strong association with asthma. The classification can be summarised as: seasonal allergic rhinoconjunctivitis = hay fever perennial rhinitis allergic (usually due to house dust mites) non-allergic = vasomotor: eosinophilic, non-eosinophilic Note: Allergic rhinitis (hay fever) is presented in detail in CHAPTER 72

.

Clinical features Nasal symptoms: sneezing nasal obstruction and congestion hypersecretion—watery rhinorrhoea, postnasal drip reduced sense of smell

Page 579

itching nose (usually allergic) Throat symptoms: dry and sore throat itching throat Irritated eyes (allergic) Abnormal nasal mucous membrane—pale, boggy, mucoid discharge. A transverse nasal

crease indicates nasal allergy, especially in a child. Allergens Pollens from trees (spring) and grass (in summer) Moulds House dust mites (perennial rhinitis) Hair, fur, feathers (from cats, dogs, horses, birds) Some foods (e.g. cow’s milk, eggs, peanuts, peanut butter)

Diagnosis Allergic rhinitis—nasal allergy: detection of allergen-specific IgE antibodies (not specific) RAST test or skin testing for specific allergens (can get false negatives) Vasomotor rhinitis—a diagnosis of exclusion.

Other causes of rhinitis Chronic infection (viral, bacterial, fungal) Rhinitis of pregnancy Rhinitis medicamentosa—following overuse of OTC decongestant nasal drops or oxymetazoline sprays Drug-induced rhinitis: various antihypertensives aspirin phenothiazines oral contraceptives cocaine, marijuana Chemical or environmental irritants (vasomotor rhinitis): smoke and other noxious fumes

paints and sprays cosmetics

Factors aggravating rhinitis (vasomotor) Emotional upsets Fatigue Alcohol Chilly, damp weather Air-conditioning Sudden changes in temperature and humidity

Rhinosinusitis Acute sinusitis Acute sinusitis is acute inflammation in the mucous membranes of the paranasal sinuses. About 5% of URTIs are complicated by an acute sinusitis,4 which is mainly viral initially, while secondary bacterial infection can follow. Any factor that narrows the sinus openings into the nasal cavity (the ostia) will predispose to acute sinusitis. The two prime clinical presentations are: 1. an URTI persisting for longer than 10 days 2. an URTI that is unusually severe with pyrexia and a purulent nasal discharge Refer to CHAPTER 41 not helpful.

for features of acute maxillary sinusitis. Note that antibiotics are usually

Chronic sinusitis Chronic sinusitis is the most common complication of acute sinusitis. In chronic sinusitis, the symptoms and signs of inflammation persist for more than 8–12 weeks and are more likely to be associated with factors that impair drainage via the osteomeatal complex, including nasal polyps.

Treatment5 Intranasal irrigation with saline

Trial antihistamine nasal spray Add in (or switch to, first or second line) intranasal corticosteroid spray While awaiting surgical options, pain may be relieved by oral prednisolone If the above therapies are ineffective, a mechanical saline sinus irrigation procedure to remove stagnant mucus is beneficial.6 Refer: for surgical drainage if there is no response to the above regimen those with orbital or facial cellulitis (refer urgently)

Nasal polyps Nasal polyps are round, soft, pale, pedunculated outgrowths arising from the nasal or sinus mucosa. They are basically prolapsed, congested, oedematous mucosa, described by some as ‘bags of water’ (see FIG 48.1 ). They occur in patients with all types of rhinitis, but especially in allergic rhinitis (see FIG. 48.2 ). Polyps usually arise from the middle meatus and turbinates. Page 580

FIGURE 48.1 Nasal polyp in right nasal cavity in a patient with inflamed mucosa from allergic rhinitis

FIGURE 48.2 Transverse cross-section of nose, demonstrating origin of nasal polyps Symptoms include nasal obstruction, watery discharge, postnasal drip and loss of smell. Note: Nasal polyps may be associated with asthma and aspirin sensitivity. Cystic fibrosis should be considered in any child with nasal polyps. A polyp that does not have the typical smooth, pale appearance may be malignant. A unilateral ‘polyp’ may be a neoplasm. If there is a purulent discharge, swab and give antibiotics.

Treatment The initial treatment should be medical.7 A medical ‘polypectomy’ can be achieved with oral steroids, for example, prednisolone 50 mg daily for 7 days. Supplement this with a corticosteroid spray (betamethasone, fluticasone, budesonide, mometasone) starting simultaneously and continuing for at least 3 months.8 Give antibiotics for any purulent nasal discharge. Simple polyps can be readily snared and removed, but referral to a specialist surgeon is advisable for surgical intervention since the aim is to remove the polyp with the mucosa of the sinuses (often ethmoidal cells) from which it arises. This complex procedure reduces the incidence of recurrence.

Epistaxis9 This common emergency should, in some instances, be treated as a life-threatening problem. The

common situation is intermittent anterior bleeding from Kiesselbach plexus located in Little area, seen in children and the young adult (90% of episodes), while posterior epistaxis (10%) is more common in the older hypertensive patient. It has a strong association with higher URTI (rhinitis, sinusitis), hot dry climates and trauma. Neoplasms should be kept in mind. Bleeding often occurs at night due to vascular vasodilation. Causes of epistaxis are presented in the diagnostic strategy model in TABLE 48.4 . The secret of good management is to have the right equipment, good lighting and effective local anaesthesia. Table 48.4

Epistaxis: diagnostic strategy model

Probability diagnosis Idiopathic: spontaneous from Little area URTI: common cold, influenza, sinusitis Rhinitis Vestibulitis Trauma (incl. nose picking, nose injury) Drugs (e.g. anticoagulants, aspirin) Serious disorders not to be missed Vascular: hypertension and arteriosclerosis Infection: systemic febrile illness (e.g. malaria) HIV/AIDS Cancer/neoplasia: tumours of nose/sinuses/nasopharynx intracranial tumours leukaemia Other: thrombocytopenia coagulopathy (e.g. haemophilia, liver disease) Pitfalls (often missed) Exposure to toxic agents Vitamin deficiencies: C and K Septal granulomas and perforations

Foreign bodies (esp. in children) Cocaine abuse Rarities: hereditary haemorrhagic telangiectasia

Diagnostic tips Recent onset of persistent bleeding in elderly points to carcinoma. Severe epistaxis is often caused by liver disease coagulopathy. Difficult-to-control posterior bleeding is a feature of the hypertensive elderly.

Ideal equipment Head light, Thudicum nasal speculum, Tilley nasal packing forceps, suction cannula and tubing, Co-Phenylcaine forte spray ± 5% cocaine solution. Tamponade options (for difficult bleeding) Merocel expandable or dental pack, Kaltostat, BIPP (bismuth iodoform paraffin paste) with ribbon gauze, dental pack, Foley catheter (no. 12, 14 or 16) with a 30 mL balloon and selfsealing rubber stopper, anterior/posterior balloon, Epistat catheter with or without Kaltostat. Or instead, many retrieval services stock a (rather expensive) Rapid Rhino nasal device for posterior or uncontrolled anterior bleeds. Page 581

Treatment First line is to clear clots—blow the nose and then apply 5–6 sprays of a decongestant nasal spray, e.g. Drixine. Simple tamponade: Pinch ‘soft’ part of nose below the nasal septum between thumb and finger for 5 or up to 20 minutes Apply ice packs to bridge of nose Another simple method is to insert a cotton wool ball soaked in lignocaine with adrenaline or other decongestant Simple cautery of Little area (see FIG. 48.3 nasal spray ± 5% cocaine solution):

) (under local anaesthetic, e.g. Co-Phenylcaine forte

Use one of three methods: electrocautery, trichloracetic acid or silver nitrate stick (preferred). Beware of silver nitrate stains. Apply petroleum jelly to cauterised area.

FIGURE 48.3 Little area on the nasal septum where several blood vessels anastomose. Bleeding is common here, especially in young people. Persistent anterior bleed Merocel (surgical sponge) nasal tampon or Kaltostat pack. Tranexamic acid can be effective, given orally or intravenously. Two RCTs have also demonstrated the effectiveness of topical application, which is of even lower risk and gives more immediate results.10 Pour an ampoule (if none avail, dissolve capsule contents) of tranexamic acid onto ribbon gauze and insert nasally. ‘Trick of the trade’ for intermittent minor anterior epistaxis: topical antibiotic (e.g. Aureomycin ointment) bd or tds for 10 days or (better option) Nasalate nasal cream tds for 7–10 days or Rectinol ointment or Vaseline Avoid digital trauma and nose blowing.

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Severe posterior epistaxis Use a Foley catheter or an Epistat catheter plus anterior pack or the Rapid Rhino nasal pack.

Nasal vestibulitis11 Infection of the nasal vestibule can cause a tender, irritating, crusty problem. First-line simple treatment is the daily application of sesame seed oil (e.g. Fess, Flo, Nozoil) or a thin smear of petroleum gel (Vaseline). Low-grade infections and folliculitis, which are evident on inspection, cause localised pain, crusts and bleeding, especially if picked from habit. Treatment is with bacitracin or preferably mupirocin (intranasal) ointment topically for 5–7 days; apply with cotton bud. Furunculosis of the nasal vestibule is usually due to Staphylococcus aureus. It starts as a small superficial abscess in the skin or the mucous membrane and may develop into a spreading cellulitis of the tip of the nose. The affected area becomes tender, red and swollen. It is best treated by avoiding touching it, hot soaks and systemic antibiotics such as dicloxacillin or as determined by culture from swabs of the vestibule. Tip: Staphylococcus aureus colonises the nose of 20–30% of the population.8 Carriers are prone to transmit nosocomial infection and have an increased risk of serious infections in the presence of serious medical disorders. Treatment includes strict hygiene and eradication with an agent such as mupirocin ointment (match-head size) 2–3 times daily for 5–7 days (max. 10 days).6 Fissure: Painful fissures often develop at the mucocutaneous junction. They may become crusted and chronic. Fissures can be treated by keeping the area moist with petroleum jelly (Vaseline) or saline gel, using hot compresses and the use of an antibiotic or antiseptic ointment if necessary.

Offensive smell from nose This may be caused by vestibulitis but ensure no foreign body is present.

Treatment Take nasal swab for culture. consider mupirocin 2% nasal ointment, instilled 2–3 times a day for 10 days or Kenacomb ointment, instil 2–3 times a day

Rhinophyma This disfiguring swelling of the nose is due to hypertrophy of the nasal sebaceous glands. There is no causal association with alcohol; the visible facial vasodilatory effects of acute intoxication

may perhaps have supported this belief. Rhinophyma is almost exclusive to men over the age of 40 years, often associated with rosacea.

Treatment Good control of rosacea may reduce the risk (see CHAPTER 113

).

If surgical correction is warranted, refer to a specialist. Carbon dioxide laser therapy is the treatment of choice. Shave excision is another effective therapy.

Nasal septal deviation This causes blockage as a solitary symptom. Mild septal deviation tends to cause alternating blockage while severe deviation causes persistent blockage on one side. The septum can be divided into anterior and posterior segments. The anterior portion is necessary to support the cartilaginous pyramid of the nose whereas the posterior portion has no supporting role and can be removed without disturbing the support of the nose. The classic submucous resection operation is therefore suitable for posterior septal deviations. Repair of anterior septal deviations is more complex.

Nasal cosmetic surgery Rhinoplasty is undertaken to improve the function of an obstructed nasal airway or for cosmetic reasons. In counselling for rhinoplasty it is important to undertake careful planning with realistic anticipated outcomes. The GP should provide non-judgmental support for the patient’s decision on cosmetic surgery before referral to an expert in rhinoplasty. Each case has to be assessed individually and the surgery tailored to the deformity. Surgical attention to the airway is important, otherwise the nose may become partially obstructed and stuffy after cosmetic surgery alone. Page 583

Septal perforation A hole in the nasal septum is commonly caused by chronic infection, including tuberculosis, repeated trauma such as vigorous nose ‘picking’ or following nasal surgery. Rarely, it is encountered in syphilis, Wegener granulomatosis (CHAPTER 21) or overzealous medical cautery. It is a known occupational hazard, particularly among chrome workers, and is seen in drug users who sniff cocaine. In about 5–10% of cases, perforation is a result of malignant disease.4 The condition may be asymptomatic depending on the cause, but there is often an irritating nasal crust and a whistling sound on nasal inspiration. It can be demonstrated by looking in one nares while a light is shone in the opposite one. The cartilaginous part is usually involved. The perforation can be closed using a silicone septal button.

If not due to a serious cause, treat with Vaseline or saline gel and topical antibiotics for any infection. Refer if malignancy is suspected, otherwise treat symptomatically.

Nasal fractures2,9 Fractures of the nose can occur in isolation or combined with fractures of the maxilla or zygomatic arch. They may result in nasal bridge bruising, swelling, non-alignment and epistaxis. Always check for a compound fracture or head injury and, if present, leave alone and refer. If the patient is seen immediately (such as on a sports field) with a straightforward lateral displacement, reduction may be attempted ‘on the spot’ with digital manipulation before distortion from soft tissue swelling. This involves simply using the fingers to push laterally on the outside of the nose towards the injured side.2 Tips: X-rays are generally unhelpful unless excluding other facial skeletal injuries and for legal reasons. If a deformity is present, refer the patient within 7 days, ideally from days 3–5. Skin lacerations, i.e. compound fracture, usually require early specialist assessment. The optimal time to reduce a fractured nose is about 10 days after injury. There is a window period of 2–3 weeks before the fracture unites. Closed reduction under local or general anaesthetic is the preferred treatment. Open reduction is more suitable for bilateral fractures with significant septal deviation, bilateral fractures with major dislocations or fractures of the cartilaginous pyramid. Refer: septal haematoma uncontrolled epistaxis recurrent epistaxis concern about cosmetic alignment cribriform plate fracture CSF rhinorrhoea

Haematoma of nasal septum Septal haematoma following injury to the nose can cause total nasal obstruction. It is easily diagnosed as a marked swelling on both sides of the septum when inspected through the nose

(see FIG. 48.4

).

FIGURE 48.4 Inferior view of nasal cavity showing bilateral swelling of septal haematoma It results from haemorrhage between the two sheets of mucoperiosteum covering the septum. It may be associated with a fracture of the nasal septum. Note: This is a most serious problem as it can develop into a septal abscess. The infection can pass readily to the orbit or the cavernous sinus through thrombosing veins and may prove fatal, especially in children. Otherwise it may lead to necrosis of the nasal septal cartilage followed by collapse and nasal deformity.

Treatment Remove blood clot through an incision, under local anaesthetic. Prescribe systemic (oral) antibiotics (e.g. penicillin or erythromycin). Treat as a compound fracture if X-ray reveals a fracture. ENT specialist advice as necessary. Page 584

Stuffy, running nose in adults For simple post-URTI rhinitis, blow the nose hard into disposable paper tissue or a handkerchief until clear, instil a nasal decongestant for 2–3 days (if desired) and also have steam inhalations with Friar’s Balsam or menthol preparations.

Rhinorrhoea This can be normal or abnormal. There is a ‘nasal cycle’ in which there is nasal congestion and decongestion that alternates from side to side and leads to rhinorrhoea. Other causes of normal discharge include vasomotor reactions to external environmental stimuli, such as cold wind and irritants, and postnasal drip (2 L of mucus pass down the back of the nose each day). The diagnostic model for rhinorrhoea is presented in TABLE 48.5 . Table 48.5

Nasal drip (rhinorrhoea) diagnostic strategy model

Probability diagnosis URTI (esp. common cold) Rhinitis (acute infective, allergic, vasomotor) Vasomotor stimulation (e.g. cold wind, smoke, irritants) Sinusitis → postnasal drip Senile rhinorrhoea Serious disorders not to be missed Vascular: cluster headache Infection: chronic infective granulomas, e.g. TB Cancer/tumours: malignancy (nasal fossa, sinus, nasopharynx) Other: CSF rhinorrhoea (post-head injury) Wegener granulomatosis Pitfalls (often missed) Nasal foreign body, e.g. in toddlers Trauma ± blood Adenoid hypertrophy Illicit drugs (e.g. cocaine, opioids, esp. heroin) Inhaled irritant gases or vapour Rarities:

choanal atresia barotrauma Seven masquerades checklist Drugs: topical OTC → rhinitis medicamentosa; narcotics Hyperthyroidism

Senile rhinorrhoea This is a common, distressing problem in the elderly, caused by failure of the vasomotor control of the mucosa. It may be associated with a deviated septum and dryness of the mucosa. There are few physical signs apart from the nasal drip. The treatment is to keep the nasal passages lubricated with an oil-based preparation, e.g. insufflation with an oily mixture (a sesame oilbased preparation, e.g. Nozoil, is suitable) or petroleum jelly. Topical decongestants cause serious side effects in the elderly.

CSF rhinorrhoea Following head injury, clear dripping fluid (+ve for glucose or beta-2 transferrin, a more specific test) may indicate a fracture of the roof of the ethmoid. A useful test is the ‘halo’ or double-ring test, where a bloodstained drop is placed on tissue paper and shows separation of blood and straw-coloured CSF. Refer for assessment, although spontaneous healing can occur.

Neoplasia Malignant nasal disease, which is uncommon, may cause nasal discharge that may be clear at first, becoming thick and offensive. Malignancy should be suspected in the presence of blood. The growth may be in the nasal fossa, sinus or nasopharynx. Benign tumours include papilloma, fibroma, osteoma, juvenile fibroangioma of puberty and nasal polyps. Fibroangiomas occur exclusively in males between the ages of 9 and 24. Patients present with unilateral nasal obstruction and recurrent epistaxis. Malignant tumours include nasopharyngeal carcinoma, with the maxillary sinus being the most common site. Squamous cell carcinoma is the most common, followed by adenocarcinoma melanoma and lymphoma. Malignant or non-healing granuloma, sometimes called ‘midline granuloma’, is a slowly progressing ulceration of the face starting in the region of the nose.4 It may represent a form of T cell malignant lymphoma, which responds to radiotherapy. The differential diagnosis is Wegener granulomatosis (see CHAPTER 21 ). Diagnosis is by CT scan and biopsy. Treatment of nasopharyngeal and sinonasal carcinoma depends on the site, size and histology, but usually involves a combination of surgery and postoperative radiotherapy.

Nasal disorders in children

Nasal problems, especially nasal discharge (rhinorrhoea), are very common in children Page 585 but the pattern of presentation is usually different from that of adults. Sinusitis is uncommon in children under the age of 10, and allergic nasal polyps are relatively rare. If a child presents with polyps, consider the possibility of cystic fibrosis or neoplasia. Rhinitis, epistaxis and nasal foreign bodies are common.

Abnormal causes Adenoid hypertrophy causing postnasal space obstruction Foreign body in nose—usually unilateral discharge Allergic rhinitis Unilateral choanal atresia Sinusitis (possible but rare) Tumour (also rare—consider fibroangioma) Diagnosis may be enhanced by spraying with a vasoconstricting agent and getting the child to blow the nose. A tumour, foreign body or polyp may become visible.

Choanal atresia Acute bilateral nasal obstruction may occur in newborns with congenital bilateral choanal atresia. This leads to anterior nasal discharge and immediate acute respiratory distress, temporarily relieved by crying. Immediate recognition and relief are essential. A finger in the corner of the mouth while finding an oral (Guedel) airway can be life-saving, as can passing a nasal probe down one nostril and perforating the membrane.

Sinusitis Although rare, sinusitis can represent a serious emergency. Red flags requiring consideration include a sick child, pyrexia, rapid onset, unilateral and deteriorating airway obstruction.

Blocked nose and snoring The above causes of nasal blockage may lead to snoring, mouth breathing, reduced sense of smell, dribbling and possibly obstructive sleep apnoea.

Nasal trauma and fractures11 Areas of concern associated with nasal fractures, which are uncommon, are possible child abuse, open fracture, septal haematoma or abscess and eye or facial changes. If a fracture is

undisplaced, the treatment is pain relief, ice compresses and rest. If displaced, refer for closed reduction under general anaesthetic within 1–2 weeks (ideally at 10 days).11 If associated epistaxis does not settle with pressure, temporary packing may be required.

Epistaxis Epistaxis is usually intermittent anterior bleeding from Little area and may follow trauma including nose picking. Bleeding often occurs at night due to vascular vasodilatation. At first, try correction with simple measures (see earlier in chapter) such as pinching below the nasal septum for 5 minutes, supplemented by cold packs. Vaseline applied in the nose at night tends to prevent bleeding, while an antibiotic ointment twice daily for 7–10 days may help. If problematic, refer for an ENT appointment. Tip: Think of a bleeding disorder or a tumour, e.g. juvenile angiofibroma.

Snoring and obstructive sleep apnoea Generally, these problems in children are almost always due to adenotonsillar hypertrophy and most cases are relieved by surgery; CPAP is rarely necessary. Sleep studies are performed to confirm clinical features and allay parental concerns. See CHAPTER 60 .

The snuffling infant Snuffling in infants is usually caused by rhinitis due to an intercurrent viral infection. The presence of yellow or green mucus should not usually be a cause for concern.

Treatment Reassure the parents. Paracetamol mixture or drops for significant discomfort. Get the parents to perform nasal toilet with a salt solution (1 teaspoon of salt dissolved in some boiled water); using a cotton bud, gently clear out nasal secretions every 2 waking hours. Once the nose is clean, saline nose drops or spray (e.g. Narium nasal mist) can be instilled. Stronger decongestant preparations are not advised unless the obstruction is causing a significant feeding problem, when they can be used for up to 4–5 days.

Foreign bodies in the nose The golden rule is ‘a child with unilateral nasal discharge has a foreign body (FB) until Page 586 proved otherwise’. Such foreign bodies usually consist of beads, pebbles, peas, pieces of rubber, plastic and paper or other small objects handled by the child. A rhinolith may develop in time on

the foreign body. In adults, foreign bodies are often rhinoliths, which are sometimes calcium deposits on pieces of gauze or other material that has been used to pack the nose.

Removal of foreign bodies Removal of FBs from the nose in children is a relatively urgent procedure because of the risks of aspiration. A disc/button battery such as a hearing aid battery in the nose is a medical emergency requiring urgent removal under anaesthetic.11 The nose should be examined using a nasal speculum under good illumination. The tip of the nose should be raised and pressed with the tip of a thumb. At first, spray a topical decongestant into the nose and see if the child can blow it out after waiting 10 minutes. Do not attempt to remove FBs from the nose by grasping with ‘ordinary forceps’. Methods of removal 1. Spray with decongestant, wait 10 minutes, then ask the child to blow out the FB. 2. It is best to pass an instrument behind the FB and pull or lever it forward. Examples of instruments are: a Eustachian catheter a probe to roll out the FB a bent hairpin a bent paperclip 3. Snaring the FB This is the appropriate method for soft, irregular FBs such as paper, foam rubber and cotton wool that are clearly visible. Examples of instruments are: a foreign-body remover crocodile forceps fine nasal forceps 4. Glue on a stick Apply SuperGlue to the plastic end of a swab stick. Within seconds, apply it to the FB (avoid the mucosa), wait a minute and then gently extract the FB. 5. Rubber catheter suction technique

The only equipment required is a straight rubber catheter (large type) and perhaps a suction pump. This method involves cutting the end of the catheter at right angles, smearing the rim of the cut end with petroleum jelly and applying this end to the FB, then providing suction. Oral suction may be applied for a recently placed or ‘clean’ object, but gentle pump suction, if available, is preferred. 6. Irritation of the nose Some practitioners sprinkle white pepper into the nose to induce sneezing. It risks inhalation. 7. The ‘kiss and blow’ technique This mouth-to-mouth method is used for a cooperative child with a firm, round foreign body such as a bead impacted in the anterior nares. It is best to supervise the child’s mother to perform the technique, but the practitioner or practice nurse can perform it. Method Use a nasal decongestant spray. After 20 minutes lay the child on an examination couch with a pillow under the head. Obstruct the normal nostril with a finger from the side. Place the mouth over the child’s mouth, blowing into it until a slight resistance is felt (this indicates that the glottis is closed). Then blow hard with a high-velocity puff to cause the FB to ‘pop out’. To encourage cooperation with the technique, the child can be asked to give mother (or other) a ‘kiss’. More than one attempt may be needed, but it is usually very successful and avoids the necessity for a general anaesthetic.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Hay fever Nasal polyps Nosebleed Nose: stuffy, running nose Sinusitis

References 1

Kalish L, Da Cruz M. Nasal obstruction. Medicine Today, March 2009; 10(3): 41–52.

2

Mendelsohn M, Ruhno J. The nose—form and function. Australian Doctor, 2 October 2004: 31.

3

Porter RS, Kaplan JL. The Merck Manual of Diagnoses and Therapy (19th edn). New Jersey: Merck, Sharp & Dohme Corp., 2011: 466–8.

4

Burton M, ed. Hall and Coleman’s Diseases of the Ear, Nose and Throat (15th edn). Edinburgh: Churchill Livingstone, 2000: 107–17.

5

Rhinitis and rhinosinusitis [published 2020]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2020. www.tg.org.au, accessed February 2021.

6

Harvey RJ. Differentiating chronic sino-nasal complaints. Australian Doctor, 6 February 2009: 27–32.

7

Lund JL. Diagnosis and treatment of nasal polyps. BMJ, 1995; 311: 1411–4.

8

Martinez-Devesa P, Patiar S. Oral steroids for nasal polyps (Cochrane Review). Cochrane Database Syst Rev, 2011; Issue 6: Art No. CD005232.

9

Barnes ML at al. Epistaxis: a contemporary evidence based approach. Otolaryngol Clin North Am, 2012; 45(5): 1005–17.

10

Reuben A et al. Novel use of tranexamic acid to reduce the need for Nasal Packing in Epistaxis (NoPac) randomised controlled trial: research protocol. BMJ Open, 2019; 9: e026882.

11

Buckley N (Chair). Australian Medicines Handbook. Adelaide: Australian Medicines Handbook Pty. Ltd, 2018: 426–7.

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49 Nausea and vomiting

Nausea, retching and hypersalivation frequently precede the act of vomiting, which is a highly integrated sequence of involuntary visceral and somatic motor events. HARRISON’S PRINCIPLES OF INTERNAL MEDICINE, 1994 Vomiting or emesis is a rather dramatic event with a diverse number of causes. It is usually preceded by nausea.

Glossary of terms Haematemesis Vomiting of blood. It is presented in CHAPTER 44

.

Nausea The unpleasant sickly sensation that may or may not herald the onset of vomiting. Regurgitation The effortless passage of gastric contents into the mouth in the absence of nausea and without diaphragmatic muscular contractions. Retching An involuntary act with all the movements of vomiting without the expulsion of gastric contents because the cardiac orifice remains closed. Rumination The effortless regurgitation of recently ingested food into the mouth, followed by rechewing and reswallowing or spitting out.1 Vomiting The forceful expulsion of gastric contents through a relaxed upper oesophageal sphincter and out of the mouth.

The clinical approach History A careful history is essential with an emphasis on drug intake, possible psychogenic factors,

including self-induced emesis, weight loss, other GIT symptoms or symptoms suggestive of systemic disease.

Examination If fever is present possible sources of infection (e.g. middle ear, meninges and urinary tract) should be checked. A careful abdominal examination is appropriate in most instances and this includes urinalysis. Look particularly for scars indicating previous surgery. Look for a succussion splash—this indicates pyloric obstruction.

Key facts and checkpoints Nausea and vomiting have a wide range of potential causes emanating from every body system. The common cause of acute nausea and vomiting in most age groups is gastroenteritis. The most common causes of vomiting in children are infections—viral (especially) and bacterial—including otitis media and urinary infection. Drug and alcohol ingestion is a common cause of nausea and vomiting; thus, a drug history is vital in assessment. Vomiting is commonly associated with migraine and may be the only symptom of a variety of migraines. Children with cyclical vomiting syndrome may have a genetic association with migraine. The nature of the vomitus provides a clue: feculent = intestinal obstruction blood = bleeding from oesophagus, stomach or duodenum (mostly) coffee grounds = bleeding from stomach or duodenum

A neurological examination needs to be considered, including ophthalmoscopy. Consider raised intracranial pressure. No examination is complete without assessment of the patient’s physical fitness, including the level of hydration, especially in infants and the very old. In these age groups the history may be difficult to obtain and the consequences of fluid loss are more complicated. Always be mindful of the possibility of pregnancy in the female patient. Look for acid dental erosion as a marker of

bulimia. Page 589

Investigations If necessary, these should consider the underlying cause and also biochemical abnormalities resulting from fluid and electrolyte loss. The following need to be considered: pregnancy test (all females of child-bearing age) microscopy and culture of stools radiology of GIT endoscopy oesophageal motility studies neurological investigation for suspected intracranial pressure (e.g. CT scan, MRI) drug toxicity studies biochemistry cortisol/short synacthen test

Red flags for vomiting Marked pallor Signs of hypovolaemia Peritoneal signs Headache, stiff neck, confusion Distended tympanic abdomen

Diagnostic guidelines Surgical GIT causes are unlikely in the absence of abdominal pain.

Vomiting without bile-stained vomitus = pyloric obstruction. Vomiting of bile = obstruction below duodenal ampulla. Vomiting of ingested food = oesophageal obstruction. Vomiting without nausea and possibly projectile = ↑ intracranial pressure. A summary of the diagnostic strategy model is presented in TABLE 49.1 Table 49.1

Vomiting in adults: diagnostic strategy model

Probability diagnosis Acute gastroenteritis/gastritis Drugs and toxins/alcohol intoxication Motion sickness Pregnancy Migraine/cyclical vomiting GORD Serious disorders not to be missed Bowel obstruction: severe constipation malignancy (e.g. oesophagus, stomach) Severe infection: botulinum poisoning septicaemia meningitis/encephalitis infective endocarditis others (e.g. acute viral hepatitis) Malignancy Intracranial disorders: malignancy, cerebellar haemorrhage Acute appendicitis Acute pancreatitis/biliary colic Acute myocardial infarction (e.g. painless) Pitfalls (mainly adults) Pregnancy (early) Organ failure: liver, kidney (uraemia), heart, respiratory Labyrinthine disorders: Ménière syndrome, labyrinthitis

.

Poisoning: food, chemicals, alcohol Gut motility disorders: achalasia Paralytic ileus Acute glaucoma Substance abuse, e.g. opioids, marijuana Radiation therapy Hypercalcaemia Chronic idiopathic nausea and vomiting Functional obstruction: diabetic gastroparesis, idiopathic gastroparesis Seven masquerades checklist Depression (possible) Diabetes (ketoacidosis) Drugs (various, e.g. cytotoxics, digoxin) Anaemia Thyroid and other endocrine disorders (Addison disease) UTI Is the patient trying to tell me something? Possibly: extreme stress (e.g. panic attacks, anxiety). Consider bulimia (self-induced vomiting) and functional (psychogenic).

Vomiting in infancy Common causes in infants Feeding problems Food intolerance Physiological, e.g. posseting, simple reflux Gastro-oesophageal reflux Gastroenteritis Viral respiratory infections Page 590

Is the vomiting bile-stained?

Green vomiting = urgent surgical referral for possible intestinal malrotation with volvulus (6 hours’ leeway before gangrene of bowel).2 Other causes: meconium ileus, small bowel/duodenal atresia. Non bile-stained vomitus (curdled milk): consider pyloric stenosis, GORD, feeding problems, concealed infection (e.g. UTI, meningitis). Both pyloric stenosis and GORD cause projectile vomiting.

Important warning signs in neonates Excessive drooling of frothy secretions from mouth Bile-stained vomitus—always abnormal Delayed passage of meconium (beyond 24 hours) Inguinal hernias Refer to surgical emergencies in children (CHAPTER 89

).

Specific conditions Posseting The effortless regurgitation of small quantities of undigested milk (breast or formula) after feeding is common in the first 1–4 months, and can continue up to the age of 18 months. In an otherwise healthy and thriving child with normal growth parameters, reassurance is appropriate.

Oesophageal atresia Vomiting occurs with the first feeding. There is excessive drooling of frothy secretions from the mouth. Pass a French gauge 10 catheter through the mouth to aid diagnosis.

Duodenal atresia Regurgitated feedings Bile-stained vomitus Abdominal distension Jaundice Often associated with Down syndrome and cystic fibrosis.

Diagnosis: abdominal X-ray/upper GIT series (double bubble sign). Treated with surgical repair.

Congenital hypertrophic pyloric stenosis Usually sudden onset 3rd–6th week Projectile vomitus Failure to thrive Male:female = 5:1 Gastric peristalsis during test feeding (L → R): feel for pyloric tumour either during test feeding or immediately after vomiting (deep in right epigastrium)—see FIGURE 49.1 ; once felt, further investigation is not necessary Biochemistry: metabolic alkalosis: sodium usually 40 years, esp. >70 years Nodes >2.5 cm Nodes >3–4 cm ?malignancy

Tender mass Purple discolouration (collar-stud abscess) Single, gradually enlarging node Fixed to skin without punctum Associated dysphagia Hard midline thyroid lump Patient at risk of malignancy and HIV Exposure to tuberculosis

The 20:40 and 80:20 rules3,4 The age of the patient is a helpful guide, as causes of neck lumps can be roughly categorised by the ‘20:40 rule’: 0–20 years: congenital, inflammatory, lymphoma, tuberculosis 20–40 years: inflammatory, salivary, thyroid, papillary thyroid cancer, lymphoma >40 years: lymphoma, metastases, i.e. neck lumps are malignant until proven otherwise Most persisting neck lumps (80%) are benign in children while the reverse applies to adults. Imaging techniques that may assist diagnosis include axial CT scan (especially in fat necks), MRI scan (distinguishes a malignant swelling from scar tissue or oedema), tomogram of larynx (laryngocele or malignancy), barium swallow (pharyngeal pouch), sialogram and carotid angiogram.5 A basic suggested approach for the patient presenting with a neck lump is summarised in FIGURE 50.2 .

FIGURE 50.2 A basic approach for patients presenting with a neck lump4

Cervical lymphadenopathy There are many causes, varying from local infections to lymphoproliferative disorders. Most malignant nodes in the supraclavicular area have their primary tumour below the clavicle. Eighty-five per cent of malignant nodes in the anterior triangle have their primary tumour in the head and neck.2 Always search for: other nodes at distant sites possible primary source of infections or neoplasia hepatosplenomegaly Hodgkin lymphoma usually presents with rubbery, painless nodes in the neck.

Consistency of enlarged nodes Rules of thumb are:5,6 hard: secondary carcinoma rubbery: lymphoma

Page 596

soft: sarcoidosis or infection tender and multiple: infection

Causes of cervical lymph node enlargement (lateral cervical swelling) Acute cervical lymphadenitis Acute viral lymphadenitis Acute bacterial lymphadenitis—coccal infection Page 597

Chronic lymph node infection MAIS lymphadenitis (atypical tuberculosis) Tuberculosis Viral infection, e.g. EBM (see FIG. 50.3

), rubella, cytomegalovirus, HIV

Toxoplasma gondii infection Cat-scratch disease—Bartonella henselae infection

FIGURE 50.3 Cervical lymphadenopathy associated with Epstein–Barr mononucleosis

Neoplastic lymphadenopathy Lymphomas, esp. Hodgkin lymphoma Leukaemia

Secondary to unknown primary

Metastatic Check mouth, pharynx, sinuses, larynx, scalp, oesophagus, stomach, breast, lungs, thyroid and skin. A working rule is upper neck—from skin to upper aerodigestive tract; lower neck—from below clavicles (e.g. lung, stomach, breast, colon). Examples: occipital or pre-auricular—check scalp submental—check mouth, tongue, teeth submandibular—check floor of mouth left supraclavicular (under sternomastoid)—consider stomach (Troisier sign) deep anterior cervical—consider larynx, thyroid, oesophagus, lungs

Neck lumps not due to lymph node swelling7 Types and causes Widespread Sebaceous cysts Lipomas

Midline Thyroid nodule (moves upon swallowing) Thyroglossal cysts (moves upwards on tongue protrusion) Dermoid cyst (beneath chin)

Anterior triangle Branchial cyst (in upper part): usually adulthood (20–25 years) Carotid body tumour: opposite thyroid cartilage

smooth and pulsatile can be moved laterally but not vertically usually 40–60 years requires excision (with care) Carotid artery-tortuous or aneurysm Parotid tumour Lateral thyroid tumours

Posterior triangle Developmental remnants: cystic hygroma branchial sinuses and cysts Pancoast tumour (from apex lung) Cervical rib

Submandibular swellings Submandibular salivary gland Cervicofacial actinomycosis (lumpy jaw syndrome): chronic granulomatosis infection due to Gram-positive Actinomyces israelii forms a multilocular abscess (pus has ‘sulphur granules’), difficult to culture infection follows dental extraction or poor dental hygiene, esp. severe caries treat with high-dose penicillin G, 4 months

Sternomastoid tumour Refer CHAPTER 85

.

Pharyngeal pouch A soft, squelchy, indefinite mass

Base of left neck History of difficulty in swallowing

Thyroid nodule The most likely cause of a solitary thyroid nodule is the dominant nodule in a multinodular goitre. Other causes include a true solitary nodule—adenoma, follicular carcinoma or solitary carcinoma —and a colloid cyst. Malignancy must be excluded. Page 598

Investigations Ultrasound FNAB (may resolve a cystic lesion) TFTs

Neck lumps in children Eighty per cent of persisting neck lumps are benign while 20% are malignant. Benign lumps usually occur in the anterior triangle, while malignant lumps are more likely in the posterior triangle. The common midline lump in children is the thyroglossal cyst.3 Consider sternomastoid tumour (fibrosis) in infants (see CHAPTER 85 ).

Lymphadenopathy Most enlarged lymph nodes are either ‘normal’ or local infections (mainly viral), especially if 50 years with new symptoms Constant pain (day and night) Fever >38°C Anterior neck (throat) pain Pain at multiple sites History of cancer Unexplained weight loss Neurological deficit

Spinal cord pathology Radicular pain in arm Rheumatoid arthritis and ankylosing spondylitis Down syndrome

Pitfalls There are many pitfalls in the clinical assessment of causes of neck pain, many of them related to inflammation. Rheumatoid arthritis is the prime severe inflammatory arthropathy that involves the neck but the neck can be affected by the seronegative spondyloarthropathies, particularly ankylosing spondylitis, psoriasis and the inflammatory bowel disorders. While polymyalgia rheumatica affects mainly the shoulder girdle, pain in the lower neck, which is part of the symptom complex, is often overlooked. Diffuse neck pain in myofascial soft tissue with tender trigger areas is part of the uncommon but refractory fibromyalgia syndrome. ‘Yellow flags’ are predictive of chronicity and disability. These include:6 a belief that spinal pain is potentially severely disabling social or financial problems history of alcohol or substance addiction reduced activity levels the presence of a compensation claim Page 602

General pitfalls Failing to appreciate how often the benign problem of facet joint dysfunction occurs in the neck, causing pain and limited movement. This involves failure to appreciate the value of physical therapy, especially exercise programs, in alleviating the problem. Failing to adhere to the idiom: one disc—one nerve root. Involvement of more than one nerve root in the upper limb may mean a neoplastic disorder such as metastatic disease, lymphoma in the thoracic outlet and similar serious diseases. Missing the insidious onset of myelopathy, especially the spasticity component, caused by rheumatoid arthritis, osteophytic overgrowth or, rarely, a soft disc prolapse.

Failing to actively address psychosocial barriers to recovery.

Seven masquerades checklist Cervical spinal dysfunction is the obvious outstanding cause. Thyroiditis may cause neck pain, as in the extremely rare cases of acute specific infection in the thyroid (e.g. syphilis, pyogenic infections), which cause severe pain; non-specific thyroiditis (de Quervain thyroiditis) produces painful swelling with dysphagia. The association between depression and neck pain is well documented.

Psychogenic considerations The neck is one of the commonest areas for psychological fixation following injury. This may involve perpetuation or exaggeration of pain because of factors such as anxiety and depression, conversion reaction and secondary gain. The psychological sequelae that can follow a whiplash injury and chronic neck problems such as spondylosis serve as a reminder that the state of the patient’s cervical spine can profoundly affect his or her life and that we should always be aware of the whole person. A feeling of depression is a very common sequel to such an injury and these patients demand our dutiful care and understanding.

The clinical approach History It is important to analyse the pain into its various components, especially the nature of its onset, its site and radiation, and associated features. The diurnal pattern of the pain will provide a lead to the diagnosis (refer to FIG. 28.3 : the patterns are similar to low back pain).

Key questions Can you point to exactly where in your neck you get the pain? Do you wake up with pain in the morning? Does the pain come on when you have to look up for a while? Do you have trouble reversing your car? Can you recall an injury to your head or neck such as hitting your head on an overhead bar? Does your neck grate or get stiff? Do you get headaches or feel dizzy?

Is the pain present day and night? Do you get pain or pins and needles or numbness in your arms? Does the pain come on with activity? Does the pain wake you at night? Do you feel pain on both sides of your neck and over your shoulders? Do your hands or arms feel weak or clumsy?

Examination It is appropriate to follow the traditional rule for examination of any joint or complex of joints: look, feel, move, measure, test function, look elsewhere and X-ray. Careful examination of the cervical spine is essential for the correct diagnosis and for specific treatment at the painful level. Three objectives of the examination are to: reproduce the patient’s symptoms identify the level of lesion or lesions determine the cause (if possible) A neurological examination is essential if radicular pain is present, or weakness or other upper limb symptoms, including any pain or paraesthesia that extends below the elbow.

Inspection The patient should be examined sitting on a couch, rather than on a chair. The body should be fully supported with the hands resting on the thighs. The following should be noted: willingness to move the head and neck level of the shoulders any lateral flexion contour of the neck from the side In the patient with torticollis the head is held laterally flexed with, perhaps, slight rotation to one side—usually away from the painful side. Patients suffering from whiplash injury and severe spondylosis tend to hold the neck stiff and the head forward, and tend to turn the trunk rather than rotate the neck. Page 603

Palpation For this vital component of the examination it is essential to know the surface anatomy of the neck so that the affected level can be determined. Method The patient lies prone on the examination couch with the forehead resting on the hands (palms up). The neck should be flexed forward and the shoulders relaxed. 1. Central digital palpation Systematically palpate the first spinous processes of the cervical vertebrae. C2 (axis) is the first spinous process palpable beneath the occiput. C7 is the largest ‘fixed’ and most prominent process—situated at the base of the neck. C6 is also prominent but usually ‘disappears’ under the palpating finger with extension of the neck. The spinous processes of C3, C4 and C5 are difficult to palpate because of cervical lordosis but their level can be estimated (see FIG. 51.2 ). Standing at the patient’s head, place opposed pulps of the thumbs on the spinous processes (starting at C2) and then move down the middle line to C7. Press firmly over each and with arms straight oscillate with moderate firmness three or four times to assess pain, stiffness or muscle spasm. 2. Lateral digital palpation The facet joints lie in sequence (called the articular pillar) about 2 to 3 cm from the midline. Press with opposed thumbs against this pillar in a systematic manner on either side of the midline (top to base) to determine any painful area. Palpation should be extended to include the anterior neck, searching for evidence of lymphadenitis, muscle spasm, thyroid disorder and other problems.

FIGURE 51.2 Relative sizes of spinous processes of the cervical spine

Movement Active movements are observed with the patient sitting on the couch. The movements are as follows with normal range indicated: flexion—45° extension—50° lateral flexion (R and L)—45° rotation (R and L)—75° Lhermitte sign is an electric shock-like pain throughout the body (especially in the legs) upon flexion of the neck. It indicates cervical pathology. If there is a full range of pain-free movement, apply overpressure slowly at the end range and note any pain. The range of movements can be plotted on a special grid called a direction of movement (DOM) diagram (see FIG. 51.3 ). This provides a ready reference for serial assessments.

FIGURE 51.3 Direction of movement diagram to record movements of the neck. This record shows restricted and painful movements (indicated by II) in right lateral flexion and right rotation; the other movements are free.

Neurological examination A neurological examination for nerve root lesions (C5 to T1) is indicated if the clinical assessment identifies the presence of neurological symptoms and signs such as pain, paraesthesia or anaesthesia in the arm. Nerve root pressure is indicated by: pain and paraesthesia along the distribution of the dermatome localised sensory loss reduced muscular power (weakness or fatigue or both) hyporeflexia (reduced amplitude or fatigue or both) It is necessary to know the sensory distribution for each nerve root and the motor changes. This is summarised in TABLE 51.3 . The dermatomes are illustrated in FIGURE 51.4 . Table 51.3

Cervical nerve root syndromes

Nerve Sensory change root C5 Outer arm/medial scapula C6

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Outer forearm/thumb/index finger/dorsal forearm

Muscle power

Power loss

Reflex

Deltoid

Abduction arm

Biceps jerk (C5, 6)

Biceps

Elbow flexion Extension wrist

Biceps + brachioradialis (C5, 6)

C7

Hand/middle and ring fingers

Triceps

Elbow extension

Triceps (C7– 8)

C8

Inner forearm/little finger, e.g. pad of finger

Long flexors finger, long extensors thumb

Grip

Fingers (C8)/flexors

T1

Inner arm

Interossei

Finger spread

FIGURE 51.4 Dermatomes of the upper limb, head and neck

Investigations Most non-traumatic, acute neck pain presenting in general practice does not require any imaging or blood test. Where red flags are present, investigations are directed to diagnosing the painful condition and determining if suspected or true organic disease is present in the spine. It is inappropriate to perform sophisticated investigations such as CT scans on most patients. Scanning should be reserved where surgery is contemplated and serious disease is suspected but not confirmed by plain X-ray. Investigations to consider include:

haemoglobin, film and WCC ESR/CRP rheumatoid factor radiology: plain X-ray (not indicated in absence of red flags and major trauma) CT scan (good for bone definition) CT scan and myelogram (if cervical disc surgery contemplated) radionucleide bone scan (for suspected metastatic disease) MRI: the investigation of choice for cervical radiculopathy, myelopathy, suspected spinal infection and tumour These should be selected conservatively. CT imaging has high radiation levels.

Neck pain in children In children and adolescents, neck pain, often with stiffness, may be a manifestation of infection or inflammation of cervical lymph nodes, usually secondary to an infected throat—for example, tonsillitis or pharyngitis. However, it is vital to consider the possibility of meningitis. Sometimes a high fever associated with a systemic infection or pneumonia can cause meningism. In the presence of fever the rare possibility of poliomyelitis should be kept in mind. In both children and adults the presence of cerebral pathology, such as haemorrhage, abscess or tumour are uncommon possibilities.7 Acute torticollis is quite common in this age group and the neck may be involved in chronic juvenile arthritis. Page 605

Neck pain in the elderly In adults the outstanding causes are dysfunction of the joints and spondylosis, with the acute febrile causes encountered in children being rare. However, cerebral and meningeal disorders may cause pain and stiffness in the neck.7 Neck pain is common in rheumatoid arthritis and to a lesser extent the spondyloarthropathies. The painful, acute wry neck can affect all ages and is considered to be caused mainly by acute disorders of the apophyseal joints rather than disc prolapse. However, disc lesions do occur and can cause referred pain or radicular pain. In the elderly, radicular pain can also be caused by impingement of the nerve root in the intervertebral foramen that has become narrowed from the degenerative changes of longstanding spondylosis.

Problems with a higher probability with increasing age include: cervical spondylosis with radiculopathy or myelopathy atlantoaxial subluxation complicating rheumatoid arthritis polymyalgia rheumatica metastatic cancer Pancoast tumour of the lung angina and myocardial infarction pharyngeal and retropharyngeal infection and tumour

Clinical problems of cervical spinal origin Pain originating from disorders of the cervical spine is usually, although not always, experienced in the neck. The patient may experience headache, or pain around the ear, face, arm, shoulder, upper anterior or posterior chest.7 Possible symptoms include: neck pain, stiffness headache, including migraine-like facial pain arm pain (referred or radicular) myelopathy (sensory and motor changes in arms and legs) ipsilateral sensory changes of scalp ear pain (peri-auricular) scapular pain anterior chest pain torticollis dizziness/vertigo in CHAPTER 15 indicates typical directions of referred pain from the cervical spine. Pain in the arm (brachialgia) is common and tends to cover the shoulder and upper arm as FIGURE 15.1

indicated.

Cervical dysfunction Dysfunction of the 35 intervertebral joints that comprise the cervical spine complex is responsible for most cases of neck pain. The problem can occur at all ages and appears to be caused by disorder (including malalignment) of the many facet joints, which are pain-sensitive. Dysfunction of these joints, which may also be secondary to intervertebral disc disruption, initiates a reflex response of adjacent muscle spasm and myofascial tenderness.

Acute non-specific neck pain Acute non-specific neck pain (ANP) is most commonly idiopathic or due to a whiplash accident. Serious causes are rare.8 Dysfunction can follow obvious trauma such as a blow to the head or a sharp jerk to the neck, but can be caused by repeated trivial trauma or activity such as painting a ceiling or gentle wrestling. People often wake up with severe neck pain and blame it on a ‘chill’ from a draught on the neck during the night. This is incorrect because it is usually caused by an unusual twist on the flexed neck for a long period during sleep.

Clinical features9 Typical age range 12–50 years Dull ache (may be sharp) in neck May radiate to occiput, ear, face and temporal area (upper cervical) May radiate to shoulder region, especially suprascapular area (lower cervical) Rarely refers pain below the level of the shoulder Pain aggravated by activity, improved with rest Various degrees of stiffness Neck tends to lock with specific movements, usually rotation Localised unilateral tenderness over affected joints Variable restriction of movement but may be normal X-rays usually normal (or, more accurately: have the same rate of abnormalities as those without neck pain). Imaging is not indicated for the investigation of ANP in the absence of ‘red flags’ and a history of trauma8

Management

The aim of treatment is to reduce pain, maintain function and minimise the risk of chronicity. Provide appropriate reassurance, information and support. Give advice to the patient about rules of living, including the following:

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Do: Stay active and resume normal activities. Keep your neck upright in a vertical position for reading, typing and so on. Keep a good posture—keep the chin tucked in. Sleep on a low, firm pillow or a special conforming pillow. Sleep with your painful side on the pillow. Use heat and massage: massage your neck firmly three times a day using an analgesic ointment. Don’t: Look up in a strained position for long periods. Twist your head often towards the painful side (e.g. when reversing a car). Lift or tug with your neck bent forwards. Work, read or study with your neck bent for long periods. Become too dependent on ‘collars’. Sleep on too many pillows. Monitor the patient’s progress without overtreatment. Analgesia:9 first-line: paracetamol 1 g (o) qid or 1.33 g (o) 8 hourly, or an NSAID consider tricyclic antidepressant for night pain or resistant pain, e.g. amitriptyline or nortriptyline Prescribe an exercise program as early as possible; start with gentle exercises and maintain them at home. Suitable exercises are shown in FIGURE 51.5 . Refer to an appropriate therapist for cervical mobilisation for persisting pain. Mobilisation combined with exercises can be an effective treatment. Occasionally, manipulation may help

with a stubborn ‘locked’ neck but should be left to an expert. If manipulation, which carries the rare but real risk of vertebral artery dissection and stroke, is to be performed, informed consent and an experienced therapist are required.10

FIGURE 51.5 Examples of exercises for the neck: (a) resisted side bending, (b) rotation, (c) chin retraction Approximately 40% of patients recover fully from acute idiopathic ANP, about 30% continue to have mild symptoms, while 30% continue to have moderate or severe symptoms.8

Evidence of benefit (in summary)8

Staying active: resuming normal activities Exercises Combined cervical passive mobilisation/exercises Pulsed electromagnetic therapy (up to 12 weeks)

Chronic non-specific pain (lasting more than 3 months)9 Continue normal activities, exercises and effective analgesics as for ANP. None of the additional treatment modalities have consistent supportive evidence. However, consider the following: a course of antidepressants TENS, especially when drugs are not tolerated hydrotherapy/thermography acupuncture (may provide short-term relief) corticosteroid facet injections (ideally under image intensification) facet joint denervation with percutaneous radiofrequency (if nerve block provides relief) multidisciplinary rehabilitation program Page 607

Cervical spondylosis9 Cervical spondylosis following disc degeneration and apophyseal joint degeneration is far more common than lumbar spondylosis and mainly involves the C5–6 and C6–7 segments. The consequence is narrowing of the intervertebral foramen with the nerve roots of C6 and C7 being at risk of compression. Cervical spondylosis is generally a chronic problem but it may be asymptomatic. In some patients the pain may lessen with age, while stiffness increases.

Clinical features Dull, aching suboccipital neck pain (see FIG. 51.6 Stiffness Worse in morning on arising and lifting head

)

Improves with gentle activity and warmth (e.g. warm showers) Deteriorates with heavy activity (e.g. working under car, painting ceiling) Usually unilateral pain—may be bilateral Pain may be referred to head, arms and scapulae May wake patient at night with paraesthesia in arms C6 nerve root most commonly involved Acute attacks on chronic background Aggravated by flexion (reading) and extension Associated vertigo or unsteadiness Restricted tender movements, especially rotation/lateral flexion Joints tender to palpation X-ray changes invariable

FIGURE 51.6 Cervical spondylosis: typical pain distribution with direction of movement diagram indicating painful and restricted movements

Treatment Provide appropriate reassurance, information and support. Refer for physiotherapy, including warm hydrotherapy. Use regular mild analgesics (e.g. paracetamol). Use NSAIDs: a trial for 2 weeks and then review. Prescribe gentle mobilising exercises as early as possible. Give passive mobilising techniques. Outline general rules to live by, including advice regarding sleeping and pillows, and day-today activities.

Complications Radiculopathy (unilateral or bilateral) Myelopathy—pressure on spinal cord Spinal canal stenosis

Acute torticollis Torticollis (acute wry neck) means a lateral deformity of the neck. This is usually a transient selflimiting acutely painful disorder with associated muscle spasm of variable intensity.

Clinical features Age of patient between 12 and 30 years Patient usually awakes with the problem Pain usually confined to neck but may radiate Deformity of lateral flexion and slight flexion/rotation Deformity usually away from the painful side Loss of extension Mid-cervical spine (C2–3, C3–4, C4–5) Any segment between C2 and C7 can cause torticollis Usually no neurological symptoms or signs The exact cause of this condition is uncertain, but both an acute disc lesion and Page 608 apophyseal joint lesion are implicated, with the latter the more likely cause. Acute torticollis is usually a transient and self-limiting condition that can recover within 48 hours. Sometimes it can last for about a week. Encourage heat massage and early mobility. Avoid cervical collars. Management by mobilisation and muscle energy therapy is very effective.

Muscle energy therapy This therapy relies on the basic physiological principle that the contracting and stretching of muscles leads to automatic relaxation of agonist and antagonist muscles.11,12 Lateral flexion or rotation or a combination of movements can be used, but treatment in rotation is preferred. The direction of contraction can be away from the painful side (preferred) or towards the painful side, whichever is most comfortable for the patient.

Method 1. Explain the method to the patient, with reassurance that it is not painful. 2. Rotate the patient’s head passively and gently towards the painful side to the limit of pain (the motion barrier). 3. Place your hand against the head on the side opposite the painful one. The other (free) hand can be used to steady the painful level—usually C3–4. 4. Request the patient to push the head (in rotation) as firmly as possible against the resistance of your hand. The patient should therefore be producing a strong isometric contraction of the neck in rotation away from the painful side (see FIG. 51.7A ). Your counterforce (towards the painful side) should be firm and moderate (never forceful) and should not ‘break’ through the patient’s resistance. 5. After 5–10 seconds (average 7 seconds) ask the patient to relax; then passively stretch the neck gently towards the patient’s painful side (see FIG. 51.7B ). 6. The patient will now be able to turn the head a little further towards the painful side. 7. This sequence is repeated at the new improved motion barrier. Repeat three to five times until the full range of movement returns. 8. Ask the patient to return the following day for treatment, although the neck may be almost normal.

FIGURE 51.7 Muscle energy therapy for acute torticollis: (a) isometric contraction phase for problem on the left side, (b) relaxation phase towards the affected (left) side The patient can be taught self-treatment at home using this method.

Acceleration hyperextension (whiplash) injury Patients with the whiplash syndrome, preferably referred to as an acceleration hyperextension injury, typically present with varying degrees of pain-related loss of mobility of the cervical spine, headache and emotional disturbance in the form of anxiety and depression. The problem can vary from mild temporary disability to a severe and protracted course. The injury occurs as a consequence of hyperextension of the neck followed by recoil hyperflexion, typically following a rear-end collision between motor vehicles. There is a reversal of the sequence of these movements in a head-on collision. In addition to hyperextension, there is prolongation or anterior stretching plus longitudinal extension of the neck.10 It can also occur with other vehicle accidents and in contact sports such as football. Whiplash causes injury to soft tissue structures, including muscle, nerve roots, the cervical sympathetic chain, ligaments, apophyseal joints and their synovial capsules and intervertebral

discs. Damage to the apophyseal joints appears to be severe, with possible microfractures (not detectable on plain X-ray) and long-term dysfunction. Pain and stiffness of the neck are the most common symptoms. The pain is usually Page 609 experienced in the neck and upper shoulders but may radiate to the suboccipital region, the interscapular region and down the arms. The stiffness felt initially in the anterior neck muscles shifts to the posterior neck. Headache is a common and disabling symptom that may persist for many months. It is typically occipital but can be referred to the temporal region and the eyes. Nerve root pain can be caused by a traction injury of the cervical nerve roots or by inflammatory changes or direct pressure subsequent to herniation of a disc. Paraesthesia of the ulnar border of the hand, nausea and dizziness are all relatively common symptoms. Delayed symptoms are common. A patient may feel no pain until 24 (sometimes up to 96) hours later; most experience symptoms within 6 hours. Complications of whiplash are summarised in TABLE 51.4 . Table 51.4

Complications of whiplash

Referred pain (headache, arm pain) Visual problems Vertigo Dysphagia Depression Compensation neurosis Disc rupture increasing to nerve root pain Osteoarthritis becomes symptomatic

The Canadian guidelines (1995) for whiplash are: Grade I—neck pain, stiffness or tenderness Grade II—neck symptoms + musculoskeletal signs (e.g. decreased range of motion, point tenderness) Grade III—neck symptoms + neurological signs Grade IV—neck symptoms + fracture or dislocation

Management principles The objective of treatment is to obtain a full range of free movement of the neck without pain by attending to both the physical and the psychological components of the problem. Other objectives include an early return to work and discouragement of unnecessary and excessive reliance on cervical collars and legal action. A 2018 systematic review found that poor expectations of recovery, post-traumatic stress symptoms and passive coping are the most consistent prognostic factors of chronic neck pain and/or disability after any whiplash injury.13

Treatment Establish an appropriate empathy and instil patient confidence with a positive, professional approach. Discourage multiple therapists. Provide appropriate reassurance and patient education. Encourage normalisation of activities as soon as possible. Compare the problem with a sprained ankle, which is a similar injury. Inform that an emotional reaction of anger, frustration and temporary depression is common (lasts about 2 weeks). Offer psychotherapy, e.g. CBT for evidence of post-traumatic stress. X-ray is required for ‘red flags’. Prescribe rest only for grades II and III (max. 4 days). Use a cervical collar (limit to 2 days) for grades II and III. Provide collar and refer for grade IV. Use analgesics (e.g. paracetamol)—avoid narcotics. Use a trial of NSAIDs for 14 days (poor evidence). Use tranquillisers, mild—up to 2 weeks. Refer for physiotherapy. Provide neck exercises (as early as possible). Use heat and massage—‘spray and stretch’—or ice. Give passive mobilisation (not manipulation).11 Recovery can take any time from 1–2 weeks up to about 3 months. A valuable reference is the Quebec Task Force Classification of Grades of Whiplash Associated Disorders. Available from:

www.sira.nsw.gov/.

Cervical disc disruption Disruption of a cervical disc can result in several different syndromes. 1. Referred pain over a widespread area due to pressure on adjacent dura mater. Note: A disc disruption is capable of referring pain over such a diffuse area (see FIG. 51.8 that the patient is sometimes diagnosed as functional (e.g. hysterical).

)

2. Nerve root or radicular pain (radiculopathy). The pain follows the dermatomal distribution of the nerve root in the arm. 3. Spinal cord compression (myelopathy).

FIGURE 51.8 Zone of possible referred pain distribution caused by a cervical disc lesion on the right side

Radiculopathy Apart from protrusion from an intervertebral disc, nerve root pressure or irritation causing arm pain can be caused by osteophytes associated with cervical spondylosis. Uncommon causes

include various tumours involving the vertebral segment, the meninges and nerves or their sheaths. The pain follows neurological patterns down the arm, being easier to localise with lower cervical roots, especially C6, C7 and C8. 1. The cervical roots exit above their respective vertebral bodies. For example, the C6 root exits between C5 and C6 so that a prolapse of C5–6 intervertebral disc or spondylosis of the C5–6 junction affects primarily the C6 root (see FIG. 51.4 ).

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2. One disc—one nerve root is the rule. 3. Spondylosis and tumours tend to cause bilateral pain (i.e. more than one nerve root).

Clinical features A sharp aching pain in the neck, radiating down one or both arms Onset of pain may be abrupt, often precipitated by a sudden neck movement on awakening Paraesthesia in the forearm and hand (in particular)—in 90% with proven disc prolapse11 Stiffness of neck with limitation of movement Nocturnal pain, waking patient during night Pain localised to upper trapezius and possible muscle spasm

Investigations Plain X-ray (AP, lateral extension and flexion, oblique views to visualise foramina); not required before 6–8 weeks unless red flags present; not useful for diagnosis or for surgery Plain CT scan CT scan and myelogram—excellent visualisation of structures but invasive MRI—excellent but expensive, sometimes difficult to distinguish soft disc from osteophytes Electromyography—may help delineate lesions requiring surgery

Treatment Many people respond to conservative treatment, especially from a disc prolapse. It is basically a self-limiting disorder—about 10% remain severely disabled:12 neck exercises consider semi-hard cervical collar, especially during the day

analgesics (according to severity—see CHAPTER 28

)

consider a course of corticosteroids for severe neck radicular pain, e.g. prednisone 30 mg (o) daily for 5–10 days then taper off to 3 weeks (limited evidence)10 tranquillisers, especially at night traction (with care) careful mobilisation including exercises (manipulation is contraindicated)

Cervical spondylitic myelopathy Sometimes the presence of large or multiple osteophytes or in the presence of a narrowed spinal canal symptoms of spinal cord involvement may develop.14,15 The common cause is a hard mass of material projecting from the posterior aspect of the vertebral body to indent the spinal cord and possibly the nerve roots at the exit foramina. This resultant spinal cord compression may result in several different clinical presentations, notably myelopathy in particular, but also central cord and anterior cord syndrome. A full neurological assessment is necessary. Urgently refer patients for neurosurgical assessment.

Clinical features Older people, typically men >50 years Insidious onset—symptoms over 1–2 years Numbness and tingling in fingers Leg stiffness Gait disturbance Numb, clumsy hands, especially with a high cervical lesion Signs of UMN: spastic weakness, increased tone and hyper-reflexia (arms > legs) ± clonus

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Neurological deficit, which predicts the level with reasonable accuracy Bowel and bladder function usually spared Note: LMN signs occur at the level of the lesion, and UMN signs and sensory changes occur below this level.

Causes

Cervical spondylosis Atlantoaxial subluxation: rheumatoid arthritis, Down syndrome Primary spinal cord tumours (e.g. meningiomas) Metastasis to cervical spine → epidural spinal cord compression

Investigations MRI scan CT scan with myelogram (most accurate)

Central cord syndrome15,16,17 This rather bizarre condition occurs classically in a person with a degenerative cervical spine following a hyperextension injury that causes osteophytes to compress the cord anteriorly and posteriorly simultaneously. The maximum damage occurs in the central part of the cord, leading to sensory and motor changes in the upper limbs with relative sparing of the lower limbs due to the arrangements of the long tracts in the cord. Fortunately, the prognosis is good, with most patients achieving a good neurological recovery.

Anterior cord syndrome17 Anterior cord syndrome occurs with hyperflexion injuries that produce ‘teardrop’ fractures of the vertebral bodies or extrusion of disc material. The syndrome can also be produced by comminuted vertebral body fractures. It is characterised by complete motor loss and the loss of pain and temperature discrimination below the level of the injury, but deep touch, position, two-point discrimination and vibration sensation remain intact. Because it is probably associated with obstruction of the anterior spinal artery, early surgical intervention to relieve pressure on the front of the cord may enhance recovery. Otherwise the prognosis for recovery is poor.

Down syndrome One of the more sinister problems with trisomy 21 syndrome is hypoplasia of the odontoid process, leading to C1–2 subluxation and dislocation. If unrecognised in the early stages, sudden death can occur in these children. If suspected, flexion–extension lateral views of the cervical spine will highlight the developing instability and the need for early specialist opinion.

Rheumatoid arthritis9,18 Involvement of the cervical spine is usually a late manifestation of rheumatoid arthritis (RA). It is important to be aware of the potentially lethal problem of C1–2 instability due to erosion of the major odontoid ligaments in the rheumatoid spine. These patients are especially vulnerable to disasters when under general anaesthesia and when involved in motor vehicle accidents. Early cervical fusion can prevent tragedies, especially with inappropriate procedures such as cervical manipulation. It is imperative to perform imaging of the cervical spine of all those with severe RA before major surgery to search for C1–2 instability. Lateral plain X-rays in flexion and extension may reveal increased distance in the atlanto–dens interval. This can be assessed further with MRI or CT scanning in a specialist clinic.

Treatment of spondylitic myelopathy Conservative (may help up to 50%):2 soft or semi-hard cervical collar physiotherapy for muscle weakness analgesics and/or NSAIDs Surgery is indicated when the myelopathy interferes with daily activities. One procedure is the Cloward method, which is anterior decompression with discectomy and fusion. The aim of surgery is to halt deterioration.

When to refer Persisting radicular pain in an arm despite conservative treatment Evidence of involvement of more than one nerve root lesion in the arm Severe symptoms with motor weakness Evidence of myelopathy, such as weakness, numbness or clumsiness of the upper limbs Evidence, clinical or radiological, of cervical instability in post-accident victims, or people with Down syndrome or rheumatoid arthritis Page 612

Practice tips ‘One disc—one nerve root’ is a working rule for the cervical spine. The patient should sit on the couch with the thighs fully supported for inspection

and movements of the neck. Be alert for patients with RA and Down syndrome who have cervical instability. Physical treatments such as cervical manipulation may easily cause quadriplegia. All acutely painful conditions of the cervical spine following trauma should be investigated with a careful neurological examination of the limbs, sphincter tone and reflexes. Plain film radiology is mandatory. In conscious patients, flexion and extension lateral cervical spinal plain films are useful for diagnosing instability of spinal segments with or without associated spinal fractures. The so-called whiplash syndrome is a diagnosis of exclusion of spinal fractures or severe ligamentous disruption causing instability, and even then, for medicolegal and psychological reasons, would best be termed a ‘soft tissue injury of the cervical spine’. Most ‘soft tissue cervical spine injuries’ heal within 3 months with conservative treatment. If severe pain persists, follow-up investigations may be required. Dysfunction of the cervical spine is an underestimated cause of headache. Always consider dysfunction of the cervical spine as a possible cause of shoulder pain. Strains and fractures of the apophyseal joints, especially after a whiplash injury, are difficult to detect, and are often overlooked causes of neck and referred pain.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Exercises for your neck Neck: painful neck Whiplash Wry neck (torticollis)

References 1

Gordon SJ, Trott P, Grimmer KA. Waking cervical pain and stiffness, headache, scapula or arm pain: gender and age effects. Australian Journal of Physiotherapy, 2002; 48(1): 9–

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Cohen ML. Neck pain. Modern Medicine Australia, 1989; November: 44–53.

3

Payne R. Neck pain in the elderly: a management review. Modern Medicine Australia, 1988; July: 56–67.

4

Borghouts JA et al. Cost-of-illness of neck pain in The Netherlands in 1996. Pain, 1999; 80: 629–36.

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Bogduk N. Neck pain. Aust Fam Physician, 1984; 13: 26–9.

6

Teichtahl A, McColl G. An approach to neck pain for the family physician. Aust Fam Physician, 2013; 42(11): 774–7.

7

Hart FD. Practical Problems in Rheumatology. London: Dunitz, 1985: 10–14.

8

Australian Acute Musculoskeletal Pain Guidelines Group, National Health and Medical Research Council. Evidence-Based Management of Acute Musculoskeletal Pain: A Guide for Clinicians. Canberra: Australian Government, 2003: 36–43.

9

Global Burden of Disease Study 2013 Collaborators. Global, regional, and national incidence, prevalence, and years lived with disability for 301 acute and chronic diseases and injuries in 188 countries, 1990–2013: a systematic analysis for the Global Burden of Disease Study 2013. The Lancet, 2015; 22: 743–800.

10

Rheumatology [published 2017]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2017. www.tg.org.au, accessed November 2019.

11

Vincent K et al. Systematic review of manual therapies for nonspecific neck pain. Joint Bone Spine, 2013; 80(5): 508–15.

12

Beran RG et al. Serious complications with neck manipulation and informed consent. Med J Aust, 2000; 173: 213–14.

13

Campbell L et al. Psychological factors and the development of chronic whiplashassociated disorder(s). Clinical Journal of Pain, 2018; 34(8): 755–68.

14

Kenna C, Murtagh J. Back Pain and Spinal Manipulation (2nd edn). Oxford: ButterworthHeinemann, 1997: 83–99.

15

Bogduk N. Medical Management of Acute Cervical Radicular Pain: An Evidence Based Approach. Newcastle: Newcastle Bone and Joint Institute, 1999: 5–59.

16

Porter RS, Kaplan JL. The Merck Manual (19th edn). Whitehorse Station: Merck, Sharpe & Dohme Corp., 2011: 1808–9.

17

Young D, Murtagh J. Pitfalls in orthopaedics. Aust Fam Physician, 1989; 18: 645–6.

18

Zhang T, Pope J. Cervical spine involvement in rheumatoid arthritis over time: results from a meta-analysis. Arthritis Res Ther, 2015; 17: 148.

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52 Shoulder pain

Search for clues—difficulty reaching into the hip pocket to remove a wallet may indicate loss of function due to total rupture of the supraspinatus tendon, while a complete rotator-cuff tear may lead the patient to lift the affected limb to the clothes line and leave it suspended there by the hand while hanging out the laundry. MICHAEL HAYES 1996 The painful shoulder is a relatively common and sometimes complex problem encountered in general practice. The diagnostic approach involves determining whether the disorder causing the pain arises from within the shoulder structures or from other sources such as the cervical spine (see FIG. 52.1 ), the acromioclavicular (AC) joint or diseased viscera, especially the heart, lungs and sub-diaphragmatic structures.

FIGURE 52.1 Typical pain zone arising from disorders of the shoulder joint and the lower cervical spine (C5 level) Note: The term tendinopathy or tendinosis is preferred to tendinitis since it has been shown that overuse tendon conditions generally have a non-inflammatory pathology.

Key facts and checkpoints

Virtually all shoulder structures are innervated by the fifth cervical vertebra (C5) nerve root. Pain present in the distribution of the C5 nerve can arise from the: cervical spine upper roots of brachial plexus acromioclavicular joint glenohumeral joint rotator cuff tendons, especially supraspinatus biceps tendon soft tissue (e.g. polymyalgia rheumatica) viscera, especially those innervated by the phrenic nerve (C3, C4, C5) The visceral diseases causing a painful shoulder include cardiac disorders, such as angina and pericarditis; lung diseases, especially Pancoast tumour; mediastinal disorders; and diaphragmatic irritation, as from intra-abdominal bleeding or a subphrenic abscess. A careful history should generally indicate whether the neck or the shoulder is responsible for the pain. By the age of 50, about 25% of people have some wear and tear of the rotator cuff, making it more injury-prone.1 Disorders of the rotator cuff are common, especially supraspinatus tendinopathy. The most effective tests to diagnose these problems are the resisted movement tests.1 Injections of local anaesthetic and long-acting corticosteroid produce excellent results for inflammatory disorders around the shoulder joint, especially for supraspinatus tendinopathy. They are simple to perform and do not require ultrasound guidance. The diagnosis is usually made on the history and examination. Blood tests are usually not necessary and imaging has a limited place and value.2

Functional anatomy of the shoulder Page 614 A working knowledge of the anatomical features of the shoulder is essential for understanding the various disorders causing pain or dysfunction of the shoulder. Apart from the

AC joint (ACJ) there are two most significant functional joints—the glenohumeral (the primary joint) and the subacromial complex (the secondary joint) (see FIG. 52.2 ). The glenohumeral joint is a ball and socket joint enveloped by a loose capsule. It is prone to injury from traumatic forces and develops osteoarthritis more often than appreciated. Two other relevant functional joints are the scapulothoracic and sternoclavicular joints.

FIGURE 52.2 The basic anatomical structures of the shoulder joint The clinically important perihumeral space lies above the glenohumeral joint (GHJ) between the head of the humerus and an arch formed by the bony acromion, the thick coracoacromial ligament and the coracoid process. This relatively tight compartment houses the subacromial bursa and the rotator cuff, particularly the vulnerable supraspinatus tendon.3 Excessive friction and pinching in this space render these structures prone to injury. There is a critical zone of relative ischaemia that appears to affect the rotator cuff about 1 cm medial to the attachment of the supraspinatus tendon,4 and this area is compromised during adduction and abduction of the arm due to pressure on the rotator cuff tendons from the head of the humerus. The so-called ‘impingement interval’ is the space between the undersurface of the acromion and the superior aspect of the humeral head. This space is normally narrow (6–14 mm), especially when the arm is abducted. Such factors are largely responsible for the many rotator cuff syndromes, including subacromial bursitis and lesions of the supraspinatus tendon and also bicipital tendinopathy.

A diagnostic approach A summary of the diagnostic strategy model is presented in TABLE 52.1 Table 52.1

Shoulder pain: diagnostic strategy model

Probability diagnosis Cervical spine dysfunction (referred pain) Rotator cuff tendinopathy ± a tear Adhesive capsulitis (‘frozen shoulder’) AC joint disorders Glenoid labral tears Serious disorders not to be missed Cardiovascular: angina myocardial infarction Neoplasia: Pancoast tumour primary or secondary in humerus Severe infections: septic arthritis (especially children) osteomyelitis Axillary vein thrombosis Rheumatoid arthritis Intra-abdominal pathology, e.g. bleeding Pitfalls (often missed) Polymyalgia rheumatica Cervical dysfunction Gout/pseudogout (uncommon) Osteoarthritis of acromioclavicular joint Bicipital tendon lesions Winged scapula—muscular fatigue pain Seven masquerades checklist Depression Diabetes Drugs

.

Thyroid disorder (rarely) Spinal dysfunction Is the patient trying to tell me something? Shoulder is prone to (uncommonly) psychological fixation for secondary gains, depression and conversion reaction.

Probability diagnosis The commonest causes of pain in the shoulder zone (see FIG. 52.1 ) are cervical disorders and periarthritis (i.e. soft tissue lesions involving the tendons around the glenohumeral joint). The outstanding periarthritic disorders are the rotator cuff disorders (most common) and adhesive capsulitis. The supraspinatus tendon is subjected to considerable friction and wear and tear, and prone to calcific tendinitis and an acute tear.

Serious disorders not to be missed As usual it is important to exclude any malignancy or septic infection, be it septic arthritis or osteomyelitis. Lung cancer (Pancoast syndrome), myeloma and bony metastases should be kept in mind. For pain in the region of the left shoulder the possibility of myocardial ischaemia has to be considered. Referred pain to the right shoulder from myocardial ischaemia is rare, occurring about once for every 20 episodes of left shoulder referral. Referred pain from the diaphragm and intra-abdominal disorders (e.g. biliary, perforated ulcer, splenic rupture) should be kept in mind.

Page 615

With an acute onset of painful capsulitis the possibility of rheumatoid arthritis (or even gout) is worth considering.

Pitfalls The shoulder is notorious for diagnostic traps, especially for referred pain from visceral structures, but polymyalgia rheumatica is the real pitfall. A good rule is to consider it foremost in any older person (over 60) presenting with bilateral shoulder girdle pain that is worse in the morning. Specific pitfalls include: misdiagnosing posterior dislocation of the shoulder joint misdiagnosing recurrent subluxation of the shoulder joint overlooking an avascular humeral head (post fracture) misdiagnosing rotator cuff tear or degeneration

Seven masquerades checklist Of the seven primary masquerades, spinal dysfunction and depression are those most likely to be associated with shoulder pain. The degree to which cervical spondylosis is associated with shoulder pain is not always appreciated. Remember that a patient’s description of ‘shoulder’ pain may include anywhere from the lower border of the scapula to the lateral neck. Ask them to point. Diabetes incurs a higher risk of adhesive capsulitis. Drugs are relevant as corticosteroids can cause avascular necrosis of the humeral head and anabolic steroids (weight-lifters) can cause osteolysis of the AC joint. A summary of common shoulder conditions is presented in TABLE 52.2 Table 52.2

.

Common shoulder conditions5

Problem

Typical age Structure affected group Symptoms

Instability

Labrum/capsule

15– 35

Dislocations

History of dislocation, apprehension sign

Stiffness

Capsule

40– 60

Pain, night pain, loss of movement

Loss of external rotation

Impingement

Rotator cuff (fatigue)

30– 60

Night pain, pain with overhead activities

Impingement signs

Rotator cuff tear

Rotator cuff, esp. supraspinatus

50 +

As above

Impingement signs, weakness external rotation, weakness supraspinatus

Capsulitis

GHJ capsule

50– 60

Constant severe pain, stiffness

Loss of all movements

AC joint pain

ACJ cartilage

25– 45

Localised AC joint pain

Paxinos sign

Arthritis

GHJ cartilage

70 +

Pain, loss of movement

Crepitus

Diagnostic pointers

The clinical approach History In analysing the pain pattern it is appropriate to keep the various causes of shoulder pain in mind (see TABLE 52.3 ). Many of these conditions, such as rheumatoid arthritis, osteoarthritis and gout, are relatively uncommon. Table 52.3

Causes of shoulder pain (excluding trauma, fractures and dislocations)

Cervical: dysfunction spondylosis Cervical radiculopathy Polymyalgia rheumatica (bilateral) Acromioclavicular joint: dysfunction osteoarthritis Shoulder complex Extracapsular: subacromial bursitis rotator cuff disorders: supraspinatus tendinopathy infraspinatus tendinopathy subscapularis tendinopathy bicipital tendinopathy Intracapsular (glenohumeral joint): adhesive capsulitis: idiopathic blunt trauma diabetes rheumatoid inflammation: rheumatoid arthritis ankylosing spondylitis

psoriatic arthropathy osteoarthritis avascular necrosis septic arthritis Winged scapula—muscular fatigue pain Malignant disease: primary or secondary in humerus Pancoast (referred from lung) Referred pain Cardiac: ischaemic heart disease pericarditis Gall bladder Lung mediastinum, including oesophagus diaphragmatic irritation Herpes zoster

A careful history should generally indicate whether the neck or the shoulder (or both) is responsible for the pain. Enquire about features of movement: stiffness and restriction excessive movement/instability weakness rough versus smooth

Red flag pointers for shoulder pain6 Fever (septic arthritis, osteomyelitis) Skin redness or swelling History of trauma (dislocation, fracture, rotary cuff tear) History of inflammatory arthritis Past history cancer

Motor or sensory loss in arm

Page 616

Key questions Did you have any injury, even very minor, before your pain started? Does the pain keep you awake at night? Do you have pain or stiffness in your neck? Do you have pain or restriction when clipping or handling your bra or touching your shoulder blades? (indicates painful internal rotation and a problem of capsular restriction or a disorder of the acromioclavicular joint) Do you have trouble combing or attending to your hair? (indicates problematic external rotation—infraspinatus—and also a disorder of the capsule, e.g. adhesive capsulitis) Is the pain worse when you wake in the morning? (indicates inflammation) Do you have aching in both your shoulders or around your hips?

Page 617

Do you get pain associated with sporting activity, including weight training, or with housework, dressing or other activities? Could you throw a ball underhand for 10–20 m and/or overhead for 20–25 m with your affected arm? Could you lift a full 2 L container (e.g. milk) to the level of your shoulder without bending your elbow (or to the top of your head)? Could you carry a 20–30 kg weight (e.g. full suitcase) by your side? Do NSAIDs give relief?

Examination The diagnosis is based on systematic examination of the cervical spine followed by examination of the shoulder joint. For details of examination of the cervical spine, refer to CHAPTER 51 .

Examination of the shoulder3,6 For the examination of the shoulder it is important to understand the functional anatomy of all important tendons. Follow rule: look, feel, move. The tendon disorders are diagnosed by pain on resisted movement (see TABLE 52.4

). A

knowledge of the anatomical attachments of the rotator cuff tendons to the head of the humerus (see FIG. 52.3 ) provides an understanding of the shoulder movements powered by these muscles. Table 52.4

Tendon disorders: determining resisted movements

Painful resisted movement at shoulder Affected tendon 1 Abduction Supraspinatus 2 Internal rotation

Subscapularis

3 External rotation

Infraspinatus Teres minor* Biceps*

4 Adduction

Pectoralis major Latissimus dorsi*

*Lesser role

FIGURE 52.3 The attachments of the rotator cuff tendons to the head of the humerus Source: Reproduced with permission from C Kenna and J Murtagh. Back Pain and Spinal Manipulation. Sydney: Butterworths, 1989

With tendon disorders (rotator cuff tendons or biceps) there is usually painful restriction of movement in one direction, but with capsulitis and subacromial bursitis there is usually restriction in most directions.

Inspection

Observe the shape and contour of the shoulder joints and compare both sides. Note the posture and the position of the neck and scapula. The position of the scapula provides considerable clinical information. Note any deformity, swelling or muscle wasting.

Palpation Stand behind the patient and palpate significant structures such as the AC joint, the subacromial space, the supraspinatus tendon and the long head of biceps. The subacromial bursa is one area where it is possible to localise tenderness with inflammation. Feel also over the supraspinatus and infraspinatus muscles for muscle spasm and trigger points. The axilla should be palpated for lymphadenopathy.

Movements The movements of the shoulder joint are complex and involve the scapulothoracic joint as well as the glenohumeral joint, with each joint accounting for about half the total range. Significant signs of a painful capsular pattern can be gained by determining the movements of flexion, abduction, external rotation and internal rotation. For each movement, note: the range of movement any pain reproduction any trick movement by the patient scapulothoracic rotation Movements should be tested bilaterally and simultaneously wherever possible. Look for impingement, which is the sign of fleeting interruption of free movement by ‘catching’ of a tendon upon bone.

Page 618

1 Active movements Flexion (anterior elevation) 180° Extension (posterior elevation) 50° With the palm facing medially the patient moves the arm upwards through 180° to a vertical position above the head and then backwards through this plane. Abduction—180° Adduction—50° (from neutral position) Abduction, which is initiated by the deltoid (first 15%), is possible only if the arm is fully

externally rotated. It is a key combined glenohumeral and scapulothoracic movement, which should reach 180°, and these components should be differentiated if the movement is limited. This is done by fixing the scapula with one hand, holding the scapula at its inferior angle and noting the degree of movement of each component (initial glenohumeral range 85–100°). Look for the presence of a painful arc, which occurs usually between 60° and 120° of abduction (see FIG. 52.4 ).

FIGURE 52.4 The painful arc syndrome The commonest cause is supraspinatus tendinopathy. Other causes include infraspinatus tendinopathy and subacromial bursitis (milder degree). Internal rotation—90° External rotation—90° These movements are tested with the arm by the side and the elbow flexed to 90° with the palm facing medially. The hand is carried outwards to test external rotation and inwards towards the abdomen for internal rotation. 2 Resisted movements3 Resisted movements (isometric contractions of a muscle) are important ways of testing capsulitis and for pinpointing tenderness of muscle insertions around the shoulder joint, and no examination of the shoulder is complete without them (see TABLE 52.4 ). Abduction (supraspinatus test). With the arm abducted to no more than 15° the patient pushes the elbow away from the side while the examiner’s hands resist and prevent the movement, holding for 5 seconds. Compare both sides and note any reproduction of the patient’s pain. A better and more specific test for supraspinatus impingement is testing resisted elevation in the ‘emptying the can’ position (90° of abduction, 30° horizontal flexion and full internal rotation). Internal rotation (subscapularis test). The examiner stands behind the patient and grasps the

palmar surface of the patient’s wrists (with the arm by the side and elbow at 90°). The patient attempts to move the forearm internally (medially) against resistance. External rotation (infraspinatus test). With the examiner and patient adopting a similar position to that for internal rotation, the examiner grasps the dorsal surface of the forearm near the wrist and asks the patient to press outwards, using the forearm as a lever to produce external rotation. This test is also positive for a C5 nerve root lesion. 3 Special tests Supraspinatus/infraspinatus rapid differentiation test. A quick test that helps to differentiate between a lesion of either of these tendons causing a painful arc syndrome is the ‘thumbs up/thumbs down’ abduction test. To test the supraspinatus, perform abduction with thumbs pointing upwards, and then with the thumbs pointing downwards to test the infraspinatus. Long head of biceps test. The best test is opposed forward elevation of the arm with the elbow at right angles and forearm supinated (Speed test). A positive test is reproduction of pain in the bicipital groove. Another useful test is resisted supination at the wrist (Yergason test). The brachial plexus tension test. This sequence of movements, devised by Elvey,7 tests the nerve roots and sheaths of the brachial plexus without implicating the cervical spine and the glenohumeral joint. The upper cervical roots of the plexus are sometimes injured in accidents, so this test is an effective differentiation test. Impingement test for supraspinatus lesions. See later in this chapter. Page 619

Investigations Shoulder pain occasionally benefits from appropriate investigations, including: ESR (especially for polymyalgia rheumatica)/CRP rheumatoid factor and anti-CCP serum uric acid (acute pain) ECG (if IHD suspected) radiology: X-ray of a specific part of the shoulder—AC joint, axillary view of glenohumeral joint (best view to show osteoarthritis) X-ray of cervical spine and chest (if relevant) radionuclide bone scan—to assess bone tumours

shoot-through axillary views (posterior dislocation) high-resolution ultrasound—modern techniques make this an appropriate test to assess shoulder pain due to rotator cuff lesions, especially tears and capsulitis, especially if surgery is contemplated. arthrogram of shoulder (beware of false negatives) CT scan (limited use) MRI—a useful imaging method but not routinely required except for the unstable joint arthroscopy

Shoulder tip pain Pain at the shoulder tip may be caused by local musculoskeletal trauma or inflammation or can be referred from blood or other irritants in the peritoneal cavity. Referred causes where the pain is unchanged by shoulder movement include: peptic ulceration diaphragmatic irritation (e.g. pneumonia) ruptured viscus (e.g. perforated ulcer) intraperitoneal bleeding (e.g. ruptured spleen) pneumothorax/pneumonia post laparoscopy (intraperitoneal gas) myocardial infarction/pericarditis ectopic pregnancy gall bladder disease

Shoulder pain in children Shoulder pain in children is not a common presenting problem but the following require consideration: septic arthritis/osteomyelitis swimmer’s shoulder (supraspinatus dysfunction)

Swimmer’s shoulder Although it occurs in adults, shoulder pain is the most common complaint in swimmers in the teenage years (over 12 years of age). American studies of college and national competition swimmers showed 40–60% had suffered significant pain.8,9 Swimmer’s shoulder, which is a form of rotator cuff tendinopathy, is considered to be associated with abnormal scapular positioning and cervicothoracic dysfunction and occurs in the supraspinatus tendon where an avascular zone is compressed by the greater tuberosity when the arm is abducted and relieved when adducted. Swimmers’ shoulders are forced through thousands of revolutions each day, so the susceptible area tends to impinge on the coracoacromial arch, leading to the impingement syndrome, which can progress with continued stress and age.10

Management Early recognition is important. Discuss training program with coach. Consider alteration of technique. Application of ICE after each swim. Use NSAIDs. Avoid corticosteroid injections. Refer for physiotherapy for scapular stabilisation and cervicothoracic mobilisation.

Shoulder pain in adults As a rule most of the shoulder problems increase with age. Special features in the elderly are: polymyalgia rheumatica (increased incidence with age) supraspinatus tears and persistent ‘tendinitis’ other rotator cuff disorders stiff shoulder due to adhesive capsulitis osteoarthritis of AC and glenohumeral joints cervical dysfunction with referred pain the avascular humeral head

Since the rotator cuff is prone to degeneration with age, there is a high incidence of rotator cuff tears in the elderly that are mostly asymptomatic.

The avascular humeral head The humeral head may become avascular after major proximal humeral fractures. With Page 620 experience, it is usually possible to predict the fractures at special risk. Early humeral head replacement with a prosthesis can lead to excellent pain relief and to a return of good function. Once the head has collapsed, there is secondary capsular contracture. Prosthetic replacement of the head is then rarely associated with an adequate return of joint movement. Thus, early referral of comminuted proximal humeral fractures for an expert opinion in all age groups is good practice. Early replacement can improve the functional outcome.11

Rotator cuff tendinopathy6 Rotator cuff tendinopathy, also referred to as ‘the subacromial impingement syndrome’, is the commonest cause of shoulder pain. It can be associated with inflammation (tendinitis), a tear in a tendon (degeneration), calcification, amyloidosis or impingement under the acromion. It may involve one tendon, usually the supraspinatus, or more of the rotator cuff tendons. It is most frequently encountered in young people engaged in sport involving overhead activities and people over 50 years, in whom rotator cuff tears occur most often. The diagnosis can usually be made on the history and physical examination. A comparison between rotator cuff disease and capsulitis is presented in TABLE 52.5 . Table 52.5

Is it rotator cuff disease or capsulitis?11,12 Rotator cuff disease

Adhesive capsulitis

Pain

Often severe Night pain Inability to sleep on affected side

Often very severe Night pain Inability to sleep on affected side

Onset

Gradual or sudden Rapid onset suggests calcific tendinitis

Usually gradual

Movement

Painful arc Aggravated by certain movements

Marked by stiffness in all directions

Supraspinatus tendinopathy

Supraspinatus tendinopathy can vary in intensity from mild to extremely severe. The severe cases usually involve calcification (calcific periarthritis) of the tendon, which has a very rapid onset, and spread to the subacromial bursa (subacromial bursitis).

The impingement tests5 These are effective tests for supraspinatus lesions as they force impingement of the rotator cuff and bursa under the acromion. One of these tests is the ‘emptying the can’ resistance test. The arm is placed in the ‘emptying the can’ position (90° of abduction, 30° of horizontal flexion and full internal rotation). Elevation of the arm is resisted against the therapist’s downward push. This also tests the strength of supraspinatus. It has a sensitivity of 90% and specificity of 54% for supraspinatus tendinitis in blinded trials.13 Another is the ‘drop arm’ test, where the arm is abducted to 90° and the patient asked to descend the arm slowly and in a controlled manner. A positive result is sudden dropping with or without pain. Impingement can also be tested in external rotation when the arm is abducted to 90° and externally rotated. Other tests include those of Neer and Hawkins.6

Treatment12 Systematic reviews to date have a lack of sufficient information to provide conclusive evidencebased recommendations for treatment.14 For analgesia, paracetamol taken orally is first line and if inadequate, NSAIDs alone or in combination.12 Corticosteroid injections and physiotherapy could improve range of movement. Experienced therapists believe that peritendon and subacromial corticosteroid injections are efficacious in selected patients. Rest during the acute painful phase Analgesics and NSAIDs (up to 4 weeks) Peritendon or subacromial injection (if no tears on ultrasound) Physiotherapy—an active program including scapular stabilising exercises and rotator cuff strengthening Surgery—consider after 3–6 months, usually subacromial decompression, sometimes excision of calcium

Injection technique An infiltration into the subacromial space is a simple procedure that, once learned, is usually straightforward. Approach laterally through the deltoid into the space beneath the acromion—if too high the bony acromion impedes the needle, so angle it lower. There should be no resistance to depressing the plunger, which is almost painless. Once the general injection is mastered, the ideal injection is a specific injection into the tendon. As a rule the therapeutic result is quite dramatic after one or two days of initial discomfort (often severe). The tendon can be readily palpated as a tender cord anterolaterally as it emerges from

beneath the acromion to attach to the greater tuberosity of the humerus. This identification is assisted by depressing the shoulder via a downward pull on the arm and then externally and internally rotating the humerus. This manoeuvre allows the examiner to locate the tendon readily. Page 621

Method Identify and mark the tendon. Place the patient’s arm behind the back, with the back of the hand touching the far waistline. This locates the arm in the desired internal rotation and forces the humeral head anteriorly. Insert a 23-gauge 32-mm needle under the acromion along the line of the tendon, and inject around the tendon just under the acromion (see FIG. 52.5 ). If the gritty resistance of the tendon is encountered, slightly withdraw the needle to ensure that it lies in the tendon sheath. The recommended injection is 1 mL of a soluble or long-acting corticosteroid with 5 mL of 1% lignocaine.

FIGURE 52.5 Injection placement for supraspinatus tendinopathy. Green needle: standard subacromial space. Grey needle: adjacent to subacromial tendon insertion.

Persistent supraspinatus tendinopathy There are three factors to consider with this problem: all of them may potentially be referred for surgery.

1. A very tight subacromial space. Consider referral for subacromial decompression by division of the thickened coracoacromial ligament. Even in younger patients this procedure (with or without acromioplasty) may be indicated for those with pain persisting beyond 12 months. 2. Rotator cuff tear or degeneration. In middle-aged and elderly patients, persisting tendinitis is usually due to rotator cuff tear and degeneration, an underdiagnosed condition. Surgery aims to repair an early tear. 3. Calcification of the tendon. This problem usually settles but occasionally surgical intervention is necessary.

Typical pain profile—supraspinatus tendinopathy (subacromial impingement) Site:

the shoulder and outer border of arm; maximal over deltoid insertion

Radiation:

to elbow

Quality:

throbbing pain, can be severe

Frequency:

constant, day and night

Duration:

constant

Onset:

gradual or sudden, such as straining the shoulder (e.g. dog on leash, working under car, fall onto outstretched arm)

Offset:

nil

Aggravation:

specific movements, putting on shirt, toilet activity, lying on shoulder (unable to sleep)

Relief:

analgesics only

Associated features:

trigger point over supraspinatus origin

Examination (typical features):

Diagnosis:

painful resisted abduction painful arc positive impingement test positive ‘emptying the can’ sign clinical, but high-resolution ultrasound if doubtful

Other rotator cuff lesions The patient may present with dominant signs of subscapularis or infraspinatus lesions, or a combination of two or three tendinous lesions, including the supraspinatus. This problem could be confused with milder adhesive capsulitis, hence the value of investigations such as ultrasound.

Management A subacromial space injection of 1 mL of corticosteroid and 2–3 mL of 1% local anaesthetic, using the posterior approach, generally achieves a good result for multiple affected rotator cuff tendons with or without subacromial bursitis (aim for only one injection). Relative rest is advisable. Page 622

Method With the patient sitting upright the large posterior gap between the medial acromial ridge and the humeral head is identified by palpation from behind. The needle (23-gauge, 32 or 38 mm long) is inserted into this gap just inferior to the acromion. The solution should flow into this space without resistance.

Rotator cuff tears Asymptomatic rotator cuff tears are common, being present in 4% of people 45–50 years Pain on outside hip referred to as far as foot Pain on lying on hip at night Pain climbing stairs, getting in and out of car Limp Localised tenderness on outer border of thigh

Treatment12 Page 655 A trial of NSAIDs (weigh the risks) is worthwhile and physiotherapy involving hipstrengthening exercises is first-line treatment. Injection therapy, including under ultrasound guidance, is also very effective and facilitates exercises. However, it is usually a self-limiting condition in the majority of patients.

Method of injection without ultrasound Determine the points of maximal tenderness over the trochanteric region and mark them. (For tendinopathy, this point is immediately over or above the superior aspect of the greater trochanter—see FIG. 54.8 ). Inject aliquots of a mixture of 1 mL of long-acting corticosteroid with 4–5 mL of local anaesthetic into the tender area, which usually occupies an area similar to that of a standard marble. The needle may be ‘fanned’ by repeatedly almost withdrawing then re-angling.

FIGURE 54.8 Injection technique for gluteus medius tendinopathy (into area of maximal tenderness) The injection may be very effective for a variable time. Follow-up management includes sleeping with a small pillow under the involved buttock, sleeping on a sheepskin rug, and stretching the gluteal muscles with knee–chest exercises, which are the key to relief.12 Advise the patient to walk with the feet turned out—‘the Charlie Chaplin gait’. One or two repeat injections over 6 or 12 months may be required. Surgical intervention such as iliotibial band release ± bursectomy may be necessary. Local application of ice and massage therapy may provide relief. Massaging the site with fingers, a soft drink bottle or a tennis ball (while lying on the side) may also be effective.

Fascia lata syndrome Pain in the lateral thigh can be caused by inflammation of the fascia lata. It is often due to overuse or weak musculature around the hip. Treatment is relative rest and physiotherapy.

Ischial bursitis Tailor’s bottom or ‘weaver’s bottom’, which is occasionally seen, is a bursa overlying the ischial tuberosity. Irritation of the sciatic nerve may coexist and the patient may appear to have sciatica.

Clinical features Severe pain when sitting, especially on a hard chair Tenderness at or just above the ischial tuberosity

Treatment Infiltration into the tender spot of a mixture of 4 mL of 1% lignocaine and 1 mL of LA corticosteroid (avoid the sciatic nerve) Foam rubber cushion with two holes cut out for the ischial prominences

Snapping or clicking hip (coxa saltans)13 Some patients complain of a clunking, clicking or snapping hip, either palpable and/or audible. This represents an annoying problem that may cause pain in the groin or thigh. It is more common in females with a wide pelvis.

Causes A taut iliotibial band (tendon or tensor fascia femoris) slipping backwards and forwards over the prominence of the greater trochanter (most common) or The iliopsoas tendon snapping across the iliopectineal eminence at the anterior brim of the pelvis The gluteus maximus sliding across the greater trochanter Joint laxity FAI and/or hip labral tears

Treatment The basics of treatment are: explanation and reassurance exercises to stretch the iliotibial band13 Occasionally surgery is necessary to lengthen the iliotibial band. Exercises The patient lies on the ‘normal’ side and flexes the affected hip, with the leg straight Page 656 and a weight around the ankle (see FIG. 54.9 ), to a degree that produces a stretching sensation along the lateral aspect of the thigh. Suitable for taut iliotibial band. This iliotibial stretch should be performed for 1–2 minutes, twice daily.

FIGURE 54.9 One treatment for the clicking hip

Piriformis tendinopathy This syndrome is caused by pressure on the sciatic nerve due to its aberrant course through the piriformis muscle. Symptoms include the gradual onset of deep-seated buttock pain, pain on and just after sitting, difficulty climbing stairs and sciatica-like symptoms centred on the buttocks. There is buttock point tenderness and pain on resisted rotation of the hip. Treatment is based on isometric stretching exercises under physiotherapist supervision.

When to refer Clinical evidence or suspicion of severe childhood disorders: DDH, Perthes disease, septic arthritis, SCFE or osteomyelitis Undiagnosed pain, especially night pain Any fractures or suspicion of fractures such as impacted subcapital fracture or stress fracture of the femoral neck True claudication in the buttock, whether it is vascular from aortoiliac occlusion or neurogenic from spinal canal stenosis Disabling osteoarthritis of the hip not responding to conservative measures; excellent results are obtained from surgery to the hip Any mass or lump

Practice tips Training on a plastic DDH model may help practitioners master the manoeuvres for examining the neonatal hip.

True hip pain is usually felt in the groin, medial thigh and medial aspect of the knee. The name of the FABER test is an acronym for Flexion, Abduction, External Rotation of the hip. Night pain adds up to inflammation, bursitis or tumour. The hip joint can be the target of infections such as Staphylococcus aureus, tuberculosis or inflammatory disorders such as rheumatoid and the spondyloarthropathies, but these are rare numerically compared with osteoarthritis. Psoas-related pain is an important differential diagnosis of anterior hip pain.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Bursitis and tendinitis of outer hip Hip: osteoarthritis

Resources Healthy Hips Australia: www.healthyhipsaustralia.org.au/.

References 1

Cyriax J. Textbook of Orthopaedic Medicine, Vol. 1 (6th edn). London: Balliere Tindall, 1976: 568–94.

2

Wood T (Coordinator). Sports Medicine. Check Program 453. Melbourne: RACGP, 2009: 11–13.

3

Limb conditions [published 2017]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2017. www.tg.org.au, accessed October 2019.

4

Anonymous. The Eastern Seal Guide to Children’s Orthopaedics. Toronto: Eastern Seal Society, 1982.

5

Robinson MJ. Practical Paediatrics (5th edn). Melbourne: Churchill Livingstone, 2003: 239–40.

6

Larkins PA. The little athlete. Aust Fam Physician, 1991; 20: 973–8.

7

Young D, Murtagh J. Pitfalls in orthopaedics. Aust Fam Physician, 1989; 18: 654–5.

8

Wood TQ, Young DA. Labral tears: understanding the significance of rim lesions. Medicine Today, 2008; 9: 71–5.

9

Wall PD et al. Surgery for treating hip impingement (femoroacetabular impingement). Cochrane Database Syst Rev, 2014; Issue 8: Art No. CD010796.

10

Paoloni J. Hip and groin injuries in sport. Medical Observer, 9 March 2007: 27.

11

Walsh MJ, Solomon MJ. Trochanteric bursitis: misnomer and misdiagnosis. Medicine Today, 2006; 7(12): 62–3.

12

Mellor R et al. Exercise and load modification versus corticosteroid injection versus ‘wait and see’ for persistent gluteus medius/minimus tendinopathy (the LEAP trial): a protocol for a randomised clinical trial. BMC Musculoskelet Disord, 2016; 17: 196.

13

Sheon RP, Moskowitz RW, Goldberg VM. Soft Tissue Rheumatic Pain (2nd edn). Philadelphia: Lea & Febiger, 1987: 211–12.

Page 657

55 Pain in the leg

Thou cold sciatica Cripple our senators, that their limbs may halt As lamely as their manners. WILLIAM SHAKESPEARE (1564–1616), TIMON OF ATHENS Pain in the leg has many causes, varying from a simple cramp to an arterial occlusion. Overuse of the legs in the athlete can lead to a multiplicity of painful leg syndromes, ranging from simple sprains of soft tissue to compartment syndromes. A major cause of leg pain lies in the source of the nervous network to the lower limb, namely the lumbar and sacral nerve roots of the spine. It is important to recognise radicular pain, especially from L5 and S1 nerve roots, and also the patterns of referred pain, such as from apophyseal (facet) joints and sacroiliac joints (SIJs).

Key facts and checkpoints Always consider the lumbosacral spine, the SIJs and hip joints as important causes of leg pain. Hip joint disorders may refer pain around the knee only (without hip pain). Nerve root lesions may cause pain in the lower leg and foot only (without back pain). Nerve entrapment is suggested by a radiating burning pain, prominent at night and worse at rest. Older people may present with claudication in the leg from spinal canal stenosis or arterial obstruction or both. Acute arterial occlusion to the lower limb requires relief within 4 hours (absolute limit of 6 hours). The commonest site of acute occlusion is the common femoral artery. Varicose veins can cause aching pain in the leg.

A diagnostic approach A summary of the diagnostic strategy model is presented in TABLE 55.1 Table 55.1

.

Pain in the leg: diagnostic strategy model

Probability diagnosis Muscle cramps Nerve root ‘sciatica’ Osteoarthritis (hip, knee) Exercise-related pain (e.g. Achilles tendinitis), muscular injury (e.g. hamstring, gastrocnemius, soleus) Serious disorders not to be missed Vascular: peripheral vascular disease arterial occlusion (embolism) thrombosis popliteal aneurysm deep venous thrombosis iliofemoral thrombophlebitis Neoplasia: primary (e.g. myeloma) metastases (e.g. breast to femur) Infection: osteomyelitis septic arthritis erysipelas lymphangitis gas gangrene Pitfalls (often missed) Osteoarthritis hip Osgood–Schlatter disorder Spinal canal stenosis → neurogenic claudication Herpes zoster (early) Greater trochanteric pain syndrome Nerve entrapment, e.g. meralgia paraesthetica

‘Hip pocket nerve’: from wallet pressure Iatrogenic: injection into nerve Sacroiliac disorders Sympathetic dystrophy (causalgia) Peripheral neuropathy Rarities: osteoid osteoma polymyalgia rheumatica (isolated) Paget disease popliteal artery entrapment tabes dorsalis ruptured Baker cyst Seven masquerades checklist Depression Diabetes Drugs (indirect) Anaemia (indirect) Spinal dysfunction Is the patient trying to tell me something? Quite possible. Common with work-related injuries. Page 658

Probability diagnosis Many of the causes, such as foot problems, ankle injuries and muscle tears (e.g. hamstrings and quadriceps), are obvious and common. There is a wide range of disorders related to overuse syndromes in athletes. A very common cause of acute severe leg pain is cramp in the calf musculature, the benign nature of which escapes some patients as judged by middle-of-the-night calls. One of the commonest causes is nerve root pain, invariably single, especially affecting the L5 and S1 nerve roots. Tests of their function and of the lumbosacral spine for evidence of disc disruption or other spinal dysfunction will be necessary. Should multiple nerve roots be involved, other causes, such as compression from a tumour, should be considered. Remember that a spontaneous retroperitoneal haemorrhage in a patient on anticoagulant therapy can cause nerve root pain and present as intense acute leg pain. The nerve root sensory distribution is presented in FIGURE 55.1 .

FIGURE 55.1 Dermatomes of the lower limb, representing approximate cutaneous distribution of the nerve roots Other important causes of referred thigh pain include ischiogluteal bursitis (weaver’s bottom) and gluteus medius tendinitis or trochanteric bursitis.

Serious disorders not to be missed Neoplasia

Malignant disease, although uncommon, should be considered, especially if the patient has a history of one of the primary tumours, such as breast, lung or kidney. Such tumours can metastasise to the femur. Consider also osteogenic sarcoma and multiple myeloma, which are usually seen in the upper half of the femur. The possibility of an osteoid osteoma should be considered with pain in a bone relieved by aspirin.

Infections Severe infections are not so common, but septic arthritis and osteomyelitis warrant consideration. Superficial infections such as erysipelas and lymphangitis occur occasionally.

Vascular problems Acute severe ischaemia can be due to thrombosis or embolism of the arteries of the lower limb. Such occlusions cause severe pain in the limb and associated signs of severe ischaemia, especially of the lower leg and foot. Chronic ischaemia due to arterial occlusion can manifest as intermittent claudication or rest pain in the foot due to small vessel disease.1 Various pain syndromes are presented in FIGURE 55.2 . It is important to differentiate vascular claudication from neurogenic claudication (see TABLE 55.2 ).

FIGURE 55.2 Arterial occlusion and related symptoms according to the level of obstruction Table 55.2

Comparison of the clinical features of neurogenic and vascular claudication Neurogenic claudication Spinal canal stenosis

Vascular claudication Aortoiliac arterial occlusive disease

Age

Over 50 Long history of backache

Over 50

Pain site and radiation

Proximal location, initially lumbar, buttocks and legs Radiates distally

Distal location Buttocks, thighs and calves (especially) Radiates proximally

Type of pain

Weakness, burning, numbing or tingling (not cramping)

Cramping, aching, squeezing

Onset

Walking (uphill and downhill) Distance walked varies Prolonged standing

Walking a set distance each time, especially uphill

Relief

Lying down Flexing spine (e.g. squat position) May take 20–30 minutes

Standing still—fast relief Slow walking decreases severity

Associations

Bowel and bladder symptoms

Impotence Rarely, paraesthesia or weakness

Peripheral pulses Lumbar extension

Present Aggravates

Present (usually) Reduced or absent in some, especially after exercise No change

Neurological

Saddle distribution Ankle jerk may be reduced after exercise

Note: abdominal bruits after exercise

Cause

Physical examination

Diagnosis confirmation

Radiological studies EMG

Duplex ultrasound Ankle brachial index Arteriography

Venous disorders The role of uncomplicated varicose veins as a cause of leg pain is controversial. Nevertheless, varicose veins can certainly cause a dull aching ‘heaviness’ and cramping, and can lead to painful ulceration. Superficial thrombophlebitis is usually obvious, but it is vital not to overlook deep venous thrombosis. These more serious conditions of the veins can cause pain in the thigh or calf. Page 659

Pitfalls There are many traps and pitfalls in the painful leg. Herpes zoster at the pre-eruption phase is an old trap and more so when the patient develops only a few vesicles in obscure parts of the limbs. As the population ages we can expect to encounter more cases of spinal canal stenosis, secondary to the degenerative changes. The early diagnosis can be difficult, and buttock pain on walking has to be distinguished from vascular claudication due to a high arterial obstruction. The many disorders of the SIJ and hip region can be traps, especially the poorly diagnosed yet common gluteus medius tendinitis. Another more recent phenomenon is the ‘hip pocket nerve syndrome’, where a heavy wallet crammed with credit cards can cause pressure on the sciatic nerve. One of the biggest traps, however, is when hip disorders, particularly osteoarthritis, present as leg pain, especially on the medial aspect of the knee. Nerve entrapments (see FIG. 55.3 ) are an interesting cause of leg pain, although not as common as in the upper limb. Some entrapments to consider include: lateral cutaneous nerve of thigh, known as meralgia paraesthetica common peroneal nerve posterior tibial nerve at ankle (the ‘tarsal tunnel’ syndrome) obturator nerve, in obturator canal femoral nerve (in inguinal region or pelvis)

FIGURE 55.3 Distribution of pain in the leg from entrapment of specific nerves; the sites of entrapments are indicated by an X Then there are the rare causes. One overlooked problem is complex regional pain syndrome I (sympathetic dystrophy), which may follow even minor trauma to the limb. This ‘causalgia’ syndrome manifests as burning or aching pain with vasomotor instability in the limbs. The essential feature is the disparity between the intensity of the pain and the severity of the inciting injury.

General pitfalls Overlooking beta blockers and anaemia as a precipitating factor for vascular claudication Overlooking hip disorders as a cause of knee pain

Page 660

Mistaking occlusive arterial disease for sciatica Confusing nerve root syndromes with entrapment syndromes

Seven masquerades checklist The outstanding cause of leg pain in this group is spinal dysfunction. Apart from nerve root pressure due to a disc disruption or meralgia paraesthetica, pain can be referred from the apophyseal (facet) joints. Such pain can be referred as far as the mid-calf (see FIG. 55.4 ).

FIGURE 55.4 Possible referred pain patterns from dysfunction of an apophyseal joint, illustrating pain radiation patterns from stimulation by injection of the right L4–5 apophyseal joint Source: Reproduced with permission from C Kenna and J Murtagh. Back Pain and Spinal Manipulation. Sydney: Butterworths, 1989

The other checklist conditions—depression, diabetes, drugs and anaemia—can be associated with pain in the leg. Depression can reinforce any painful complex. Diabetes can cause discomfort through a peripheral neuropathy that can initially cause localised

pain before anaesthesia predominates. Drugs such as beta blockers, and anaemia, can precipitate or aggravate intermittent claudication in a patient with a compromised circulation.

Psychogenic considerations Pain in the lower leg can be a frequent complaint (maybe a magnified one) of the patient with non-organic pain, whether depressed, malingering or having a conversion reaction. Sometimes regional pain syndrome (reflex or post-traumatic) is incorrectly diagnosed as functional.

The clinical approach Careful attention to basic detail in the history and examination can point the way of the clinical diagnosis.

History In the history it is important to consider several distinctive aspects, outlined by the following questions. Is the pain of acute or chronic onset? If acute, did it follow trauma or activity? If not, consider a vascular cause: vein or artery; occlusion or rupture. Is the pain ‘mechanical’ (related to movement)?

Page 661

If it is unaltered by movement of the leg or a change in posture, it must arise from a soft tissue lesion, not from bone or joints. Is the pain postural? Analyse the postural elements that make it better or worse. If worse on sitting, consider a spinal cause (discogenic) or ischial bursitis. If worse on standing, consider a spinal cause (instability) or a local problem related to weight-bearing (varicose veins). If worse lying down, consider vascular origin, such as small vessel peripheral vascular disease. If worse lying on one side, consider greater trochanteric pain syndrome. Pain unaffected by posture is activity-related. Is the pain related to walking? No: Determine the offending activity (e.g. joint movement with arthritis).

Yes: If immediate onset, consider local cause at site of pain (e.g. stress fracture). If delayed onset, consider vascular claudication or neurogenic claudication. Is the site of pain the same as the site of trauma? If not, the pain in the leg is referred. Important considerations include lesions in the spine, abdomen or hip and entrapment neuropathy. Is the pain arising from the bone? If so, the patient will point to the specific site and indicate a ‘deep’ bone pain (consider tumour, fracture or, rarely, infection) compared with the more superficial muscular or fascial pain. Is the pain arising from the joint? If so, the clinical examination will determine whether it arises from the joint or juxtaposed tissue. Page 662

Examination The first step is to watch the patient walk and assess the nature of any limp (see CHAPTER 28

).

Note the posture of the back and examine the lumbar spine. Have both legs well exposed for the inspection. Inspect the patient’s stance and note any asymmetry and other abnormalities, such as swellings, bruising, discolouration or ulcers and rashes. Note the size and symmetry of the legs and the venous pattern. Look for evidence of ischaemic changes, especially of the foot. Palpate for local causes of pain and if no cause is evident examine the spine, blood vessels (arteries and veins) and bone. Areas to palpate specifically are the ischial tuberosity, trochanteric area, hamstrings and tendon insertions. Palpate the superficial lymph nodes. Note the temperature of the feet and legs. Perform a vascular examination, including the peripheral pulses and the state of the veins if appropriate. If evidence of peripheral vascular disease (PVD), remember to auscultate the abdomen and adductor hiatus, and the iliac, femoral and popliteal vessels. A neurological examination may be appropriate, particularly to test nerve root lesions or entrapment neuropathies. It is worthwhile developing a routine for rapidly assessing the motor function of L5 (e.g. hallux dorsiflexion) and S1 (e.g. walking on tiptoes), as sciatica presents so commonly. Examination of the joints, especially the hip and SIJs, is very important.

Investigations A checklist of investigations that may be necessary to make the diagnosis is as follows: FBE and ESR radiology: leg X-rays, especially knee, hip plain X-ray of lumbosacral spine CT scan of lumbosacral spine ultrasound or MRI of greater trochanteric area MRI scan of lumbosacral spine bone scan electromyography vascular: arteriography duplex ultrasound scan ankle brachial index venous pool radionuclide scan contrast venography air plethysmograph (varicose veins) D-dimer test

Leg pain in children Aches and pains in the legs are a common complaint in children. The most common cause is soreness and muscular strains due to trauma or unaccustomed exercise. A rare cause of bilateral leg pain in children is leukaemia. Consider osteomyelitis (see CHAPTER 58 ). It is important to consider child abuse, especially if bruising is noted on the back of the legs.

‘Growing pains’

So-called ‘growing pains’, or idiopathic leg pain, is thought to be responsible for up to 20% of leg pain in children.2 Such a diagnosis is vague and often made when a specific cause is excluded. It is usually not due to ‘growth’ but related to excessive exercise or trauma from sport and recreation, and probably emotional factors. The pains are typically intermittent and symmetrical and deep in the legs, usually in the anterior thighs or calves. Although they may occur at any time of the day or night, typically they occur at night, usually when the child has settled in bed. The pains usually last for 30–60 minutes and tend to respond to attention such as massage with an analgesic balm or simple analgesics (refer to CHAPTER 84 ).

Serious problems It is important to exclude fractures (hence the value of X-rays if in doubt), malignancy (such as osteogenic sarcoma, Ewing tumour or infiltration from leukaemia or lymphoma), osteoid osteoma, osteomyelitis, scurvy and beriberi (rare disorders in developed countries) and congenital disorders such as sickle-cell anaemia, Gaucher disease and Ehlers–Danlos syndrome.

Leg pain in adults The older the person, the more likely it is that arterial disease with intermittent claudication and neurogenic claudication due to spinal canal stenosis will develop. Other important problems of the elderly include degenerative joint disease, such as osteoarthritis of the hips and knees, muscle cramps, herpes zoster, Paget disease, polymyalgia rheumatica (affecting the upper thighs) and sciatica.

Spinal causes of leg pain Problems originating from the spine are an important, yet at times complex, cause of pain in the leg. Important causes are:

Page 663

nerve root (radicular) pain from direct pressure referred pain from: disc pressure on tissues in front of the spinal cord apophyseal joints SIJs spinal canal stenosis causing claudication Various pain patterns are presented in FIGURES 55.3

and 55.4

.

Nerve root pain Nerve root pain from a prolapsed disc is a common cause of leg pain. A knowledge of the dermatomes of the lower limb (see FIG. 55.1 ) provides a pointer to the involved nerve root, which is usually L5 or S1 or both. The L5 root is invariably caused by an L4–5 disc prolapse and the S1 root by an L5–S1 disc prolapse. The nerve root syndromes are summarised in TABLE 55.3 . Table 55.3

Comparison of neurological findings of the neurological levels L3, L4, L5 and S17

Nerve Pain root distribution (see FIG. 55.1 ) L3 Front of thigh, inner aspect of thigh, knee and leg

Sensory loss

Motor function

Reflex

Anterior aspect of thigh

Extension of knee

Knee jerk

L4

Anterior thigh to front of knee

Lower outer aspect of thigh and knee, inner great toe

Flexion, adduction of knee, inversion of foot

Knee jerk

L5

Lateral aspect of leg, dorsum

Dorsum of foot, great toe, 2nd, 3rd and 4th

Dorsiflexion of great toe and ankle

Tibialis posterior (clinically impractical)

S1

of foot and great toe

toes, anterolateral aspect of lower leg

Buttock to back of thigh and leg, central calf, lateral aspect of ankle and sole of foot

Lateral aspect of ankle, foot (4th and 5th toes)

Plantar flexion of ankle and toes, eversion of foot

Ankle jerk

A summary of the physical examination findings for the most commonly involved nerve roots is presented in TABLE 55.3 .

Sciatica See CHAPTER 28 . Sciatica is defined as pain in the distribution of the sciatic nerve or its branches (L4, L5, S1, S2, S3) caused by nerve pressure or irritation. Most problems are due to entrapment neuropathy of a nerve root, in either the spinal canal (as outlined above) or the intervertebral foramen. It should be noted that back pain may be absent and peripheral symptoms only will be present.

Treatment A protracted course can be anticipated, in the order of 12 weeks (see CHAPTER 28 ). The patient should be reassured that spontaneous recovery can be expected. As for low back pain, systematic reviews of various interventions for sciatica (advice, or physical or medical therapy) have failed to find any particular method that impresses. Bearing that in mind, a trial of conservative treatment might involve the following:

back care education relative bed rest if very painful only (2 days is optimal)—a firm base is ideal return to activities of daily living ASAP analgesics (avoid narcotic analgesics if possible) NSAIDs3 (mild global improvement; 2 weeks is reasonable) basic exercise program, including swimming some find traction helpful (even intermittent manual), although a Cochrane review suggests otherwise.4 Referral to a therapist of your choice (e.g. physiotherapist) may be advisable. Conventional spinal manipulation is usually contraindicated for radicular sciatica. If the sciatica is not responding or the circumstances demand more active treatment, an epidural corticosteroid injection has been shown to slightly reduce pain and disability in the short term.5 Surgical consultation is indicated for a severe or progressive neurological deficit. Lumbar spinal canal stenosis (LSS)6 LSS is narrowing of the spinal canal resulting in pressure on the sciatic nerve roots and possibly the spinal canal and cord. It typically occurs in people aged over 50 years with a history of spinal degenerative changes, including osteoarthritis (see FIG. 28.9 ), but LSS may be congenital. The symptoms are as outlined for neurogenic claudication in TABLE 55.2 . Pain, which is usually bilateral, is in the buttocks, thighs and legs—proximal radiating distally. Some 50% remain clinically stable, 25% deteriorate and about 25% improve with time. Conservative treatment includes relative rest, specific exercises, analgesics and agents for neuropathic pain. There is no evidence of the value of epidural corticosteroid injections or oral corticosteroids. Surgical consultation is indicated for severe or progressive neurological deficit or progressive pain. The risk of surgical complications increases with age. The standard surgery is spinal decompression.

Referred pain Referred pain in the leg can arise from disorders of the SIJs or from spondylogenic disorders. It is typically dull, heavy and diffuse. The patient often uses their hand to describe its distribution compared with their use of fingers to point to radicular pain.

Spondylogenic pain Non-radicular or spondylogenic pain originates from any of the components of the vertebrae (spondyles), including joints, the intervertebral discs, ligaments and muscle attachments. An

important example is distal referred pain from disorders of the apophyseal joints, where the pain can be referred to any part of the limb as far as the calf and ankle but most commonly to the gluteal region and proximal thigh (see FIG. 55.4 ). Another source of referred pain is that caused by compression of a bulging disc against Page 664 the posterior longitudinal ligament and dura. The pain is typically dull, deep and poorly Page 665 localised. The dura has no specific dermatomal localisation, so the pain is usually experienced in the low back, sacroiliac area and buttocks. Less commonly, it can be referred to the coccyx, groin and both legs down to the calves. It is not referred to the ankle or the foot.

Sacroiliac dysfunction This typically causes a dull ache in the buttock, but it can be referred to the iliac fossa, groin or posterior aspects of the thighs (see CHAPTER 54 ). It rarely radiates to or below the knee. It may be caused by inflammation (sacroiliitis) or mechanical dysfunction. The latter must be considered in a postpartum woman presenting with severe aching pain present in both buttocks and thighs.

Nerve entrapment syndromes Entrapment neuropathy can result from direct axonal compression or can be secondary to vascular problems, but the main common factor is a nerve passing through a narrow rigid compartment where movement or stretching of that nerve occurs under pressure.

Clinical features Pain at rest (often worse at night) Variable effect with activity Sharp, burning pain Radiating and retrograde pain Clearly demarcated distribution of pain Paraesthesia may be present Tenderness over nerve May be positive Tinel sign (tap posterior tibial nerve behind medial malleolus)

Meralgia paraesthetica This is the commonest lower limb entrapment and is due to the lateral femoral cutaneous nerve of the thigh being trapped under the lateral end of the inguinal ligament, 1 cm medial to the

ASIS.8 The nerve is a sensory nerve from L2 and L3. It occurs mostly in middle-aged people, due mainly to thickening of the fibrous tunnel beneath the inguinal ligament, and is associated with obesity, pregnancy, ascites or local trauma such as belts, trusses and corsets. Its entrapment causes a burning pain with associated numbness and tingling (see FIG. 55.3 ). The distribution of pain is confined to a localised area of the lateral thigh and does not cross the midline of the thigh.

Differential diagnosis L2 or L3 nerve root pain (L2 causes buttock pain also) Femoral neuropathy (extends medial to midline)

Treatment Injection of corticosteroid medial to the ASIS, under the inguinal ligament Surgical release (neurolysis) if refractory Treat the cause (e.g. weight reduction, constricting belt, corset) Note: Meralgia paraesthetica often resolves spontaneously.

Peroneal nerve entrapment The common peroneal (lateral popliteal) nerve can be entrapped where it winds around the neck of the fibula or as it divides and passes through the origin of the peroneus longus muscle 2.5 cm below the neck of the fibula. It is usually injured, however, by trauma or pressure at the neck of the fibula.

Symptoms and signs Pain in the lateral shin area and dorsum of the foot Sensory symptoms in the same area Weakness of eversion and dorsiflexion of the foot (described by patients as ‘a weak ankle’)

Differential diagnosis L5 nerve root (similar symptoms)

Treatment

Shoe wedging or other orthotics to maintain eversion Neurolysis is the most effective treatment

Tarsal tunnel syndrome This is an entrapment neuropathy of the posterior tibial nerve in the tarsal tunnel beneath the flexor retinaculum on the medial side of the ankle. The condition is due to dislocation or fracture around the ankle or tenosynovitis of tendons in the tunnel from injury, or rheumatoid arthritis and other inflammations.

Symptoms and signs A burning or tingling pain in the toes and sole of the foot, occasionally the heel Retrograde radiation to the calf, perhaps as high as the buttock Numbness is a late symptom

Page 666

Discomfort often in bed at night and worse after standing Removal of shoe may give relief Sensory nerve loss variable, may be no loss Tinel test (finger or reflex hammer tap over nerve below and behind medial malleolus) may be positive Tourniquet applied above ankle may reproduce symptoms The diagnosis is confirmed by electrodiagnosis.

Treatment Relief of abnormal foot posture with orthotics Corticosteroid injection into tunnel Decompression surgery if other measures fail

Vascular causes of leg pain Occlusive arterial disease Risk factors for peripheral vascular disease (for development and deterioration): smoking

diabetes hypertension hypercholesterolaemia family history atrial fibrillation (embolism) Aggravating factors: beta-blocking drugs anaemia

Acute lower limb ischaemia Sudden occlusion is a dramatic event that requires immediate diagnosis and management to save the limb.

Causes Embolism—peripheral arteries Thrombosis: major artery, popliteal aneurysm Traumatic contusion (e.g. postarterial puncture) The symptoms and signs of acute embolism and thrombosis are similar, although thrombosis of an area of atherosclerosis is often preceded by symptoms of chronic disease (e.g. claudication). The commonest site of acute occlusion is the common femoral artery (see FIG. 55.5 ).

FIGURE 55.5 Common sites of acute arterial occlusion

Signs and symptoms—the 6 Ps Pain Pallor Paraesthesia or numbness Pulselessness Paralysis ‘Perishing’ cold

The pain is usually sudden and severe and any improvement may be misleading. Sensory changes initially affect light touch, not pinprick. Paralysis (paresis or weakness) and muscle compartment pain or tenderness are most important and ominous signs. Other signs include mottling of the legs, collapsed superficial veins and no capillary return. If the foot becomes dusky purple and fails to blanch on pressure, irreversible necrosis has occurred. Note: Look for evidence of atrial fibrillation.

Examination of arterial circulation This applies to chronic ischaemia and also to acute ischaemia. Page 667

Skin and trophic changes Note colour changes, hair distribution and wasting. Note the temperature of the legs and feet with the backs of your fingers. Palpation of pulses It is important to assess four pulses carefully (see FIG. 55.6 ). Note that the popliteal and posterior tibial pulses are difficult to feel, especially in obese subjects.

FIGURE 55.6 Sites of palpation of peripheral pulses in the leg Femoral artery. Palpate deeply just below the inguinal ligament, midway between the ASIS and the symphysis pubis. If absent or diminished, palpate over abdomen for aortic aneurysm. Popliteal artery. Flex the leg to relax the hamstrings. Place fingertips of both hands to meet in the midline. Press them deeply into the popliteal fossa to compress artery against the upper end of the tibia (i.e. just below the level of the knee crease). Check for a popliteal aneurysm (very prominent popliteal pulsation). Posterior tibial artery. Palpate, with curved fingers, just behind and below the tip of the medial malleolus of the ankle. Dorsalis pedis artery. Feel at the proximal end of the first metatarsal space just lateral to the extensor tendon of the big toe. Oedema Look for evidence of oedema: pitting oedema is tested by pressing firmly with your thumb for at least 5 seconds over the dorsum of each foot, behind each medial malleolus and over the shins. Postural colour changes (Buerger test)

Raise both legs to about 60° for about 1 minute, when maximal pallor of the feet will develop. Then get the patient to sit up on the couch and hang both legs down.8 Note: Comparing both feet, check the time required for return of pinkness to the skin (normally less than 10 seconds) and filling of the veins of the feet and ankles (normally about 15 seconds). Look for any unusual rubor (dusky redness) that takes a minute or more in the dependent foot. A positive Buerger test is pallor on elevation and rubor on dependency and indicates severe, chronic ischaemia. Auscultation for bruits after exercise Listen over abdomen and femoral area for bruits. Note: Neurological examination (motor, sensory, reflexes) is normal unless there is associated diabetic peripheral neuropathy.

Treatment Golden rules: Occlusion is usually reversible if treated within 4 hours (i.e. limb salvage). It is often irreversible if treated after 6 hours (i.e. limb amputation). Unfractionated heparin (immediately) 80 U/kg IV Emergency embolectomy (ideally within 4 hours): under general or local anaesthesia through an arteriotomy site in the common femoral artery embolus extracted with Fogarty balloon or catheter or Stenting of vessels (less invasive and less expensive) Angioplasty Arterial bypass if acute thrombosis in chronically diseased artery In selected cases, thrombolysis with streptokinase or urokinase appropriate Amputation (early) if irreversible ischaemic changes

Page 668

Lifetime anticoagulation will be required Note: An acutely ischaemic limb is rarely life-threatening in the short term. Thus, even in the extremely aged, demented or infirm, a simple embolectomy is not only worthwhile but also is usually the most expedient treatment option.

Chronic lower limb ischaemia Chronic ischaemia caused by gradual arterial occlusion can manifest as intermittent claudication, rest pain in the foot or overt tissue loss—ulceration, gangrene. Intermittent claudication is a pain or tightness in the muscle on exercise (Latin claudicare, to limp), relieved by rest. Rest pain is a constant severe burning-type pain or discomfort in the forefoot at rest, typically occurring at night when the blood flow slows down. The main features are compared in TABLE 55.4 Table 55.4

.

Comparison between intermittent claudication and ischaemic rest pain

Quality of pain

Intermittent claudication Tightness/cramping

Ischaemic rest pain Constant ache

Timing of pain (typical)

Daytime; walking, other exercise

Night-time; rest

Tissue affected

Muscle

Skin

Site

Calf > thigh > buttock

Forefoot, toes, heels

Aggravation

Walking, exercise

Recumbent, walking

Relief

Rest

Hanging foot out of bed; dependency

Associations

Beta blockers Anaemia

Night cramps Swelling of feet

Intermittent claudication The level of obstruction determines which muscle belly is affected (see FIGS 55.2

and 55.6

Proximal obstruction (e.g. aortoiliac) Pain in the buttock, thigh and calf, especially when walking up hills and stairs Persistent fatigue over whole lower limb Impotence is possible (Leriche syndrome)

Obstruction in the thigh Superficial femoral (the commonest) causes pain in the calf (e.g. claudication at 200–500 m), depending on collateral circulation

).

profunda femoris → claudication at 100 m multiple segment involvement → claudication at 40–50 m

Causes Atherosclerosis (mainly men over 50, smokers) Embolisation (with recovery) Buerger disease: affects small arteries, causes rest pain and cyanosis (claudication uncommon) Popliteal entrapment syndrome (20°

Source: Adapted from Litt8

Is there an underlying fracture? For a severe injury the possibility of a fracture—usually of the lateral malleolus or base of fifth metatarsal—must be considered. If the patient is able to walk without much discomfort straight after the injury a fracture is unlikely. X-ray according to the Ottawa ankle rules. Indications for X-ray include:6 inability to weight-bear immediately after injury marked swelling and bruising soon after injury marked tenderness over the bony landmarks marked pain on movement of the ankle crepitus on palpation or movement point tenderness over the base of the fifth metatarsal special circumstances (e.g. litigation potential)

Ottawa rules for ankle and foot X-ray7 These rules are a quick and reliable method of selecting which patients with ankle and foot injuries need X-rays to exclude a fracture. Ankle injury An X-ray of the ankle is necessary when the patient has pain over the medial or lateral malleolar zone and any one of the following findings: there is bone tenderness on palpation of the distal 6 cm of the fibula (posterior tip of lateral malleolus) there is bone tenderness on palpation of the distal 6 cm of the tibia (posterior tip of medial malleolus)

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there was an inability to bear weight (walk four steps) both immediately after injury and during the clinical examination Foot injury Refer for a foot X-ray (suspected midfoot fracture) if there is pain in the midfoot and any one of: bone tenderness at fifth metatarsal base bone tenderness at the navicular bone

inability to weight-bear immediately after injury and when seen

Management The treatment of ankle ligament sprains depends on the severity of the sprain. Most grade I and II sprains respond well to standard conservative measures and regain full, pain-free movement in 1–6 weeks, but controversy surrounds the most appropriate management of grade III sprains. A 2002 Cochrane Systematic Review revealed that functional recovery for grade III strains was quicker in those treated by rehabilitation compared with surgery.9 Grade I–II sprains R

=

Rest the injured part for 48 hours, depending on disability

I

=

Ice pack for 20 minutes every 3–4 hours when awake for the first 48 hours

C

=

Compression bandage (e.g. crepe bandage)

E

=

Elevate to hip level to minimise swelling

A

=

Analgesics (e.g. paracetamol ± codeine)

R

=

Review in 48 hours, then 7 days

S

=

Special strapping

Use partial weight-bearing with crutches for the first 48 hours or until standing is no longer painful, then encourage early full weight-bearing and full range of movement. This can be followed by isometric exercises.10 Use warm soaks, dispense with ice packs after 48 hours. Walking in sand (e.g. along the beach) is excellent rehabilitation. Aim towards full activity by 2 weeks. Grade III sprains Initial management includes RICE (as above), analgesics and an X-ray to exclude an associated fracture. Refer the patient with a complete ankle tear.

Red flag

Be aware of the serious Lisfranc ligament disruption of the forefoot.

Heel pain Important causes of heel pain in adults (see FIG. 57.4

)11 include:

Achilles tendon disorders: tendinopathy/peritendinitis bursitis: postcalcaneal, retrocalcaneal tendon tearing: partial, complete bruised heel tender heel pad: usually atrophy also inflammation neuropathies (e.g. diabetic, alcoholic) tenosynovitis (FHL, FDL) ‘pump bumps’—exostoses near achilles insertion due to stiff backs of shoes plantar fasciitis periostitis calcaneal apophysitis peroneal tendon dislocation nerve entrapments tarsal tunnel medial calcaneal nerve nerve to abductor digiti minimi

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FIGURE 57.4 Important causes of the painful heel Ultrasound examination is useful to differentiate the causes of Achilles tendon disorders.

Achilles tendinopathy11 The pathology is a combination of degenerative and inflammatory changes due to overuse and may occur either in the tendon itself or in the surrounding paratendon. It presents with tendon pain during and after weight-bearing activities with a tender local swelling of the tendon. The latter is called peritendinitis rather than tenosynovitis because there is no synovial sheath.

Management Relative rest Course of NSAIDs for acute pain Heel padding Consider heel ‘raisers’ Consider continuous topical glyceryl trinitrate as patches Physiotherapy for stretching and an eccentric exercise program11 Physiotherapy

Complete rupture of Achilles tendon11 Clinical features Sudden onset of intense pain Person usually falls over Feels more comfortable when acute phase passes Development of swelling and bruising Some difficulty walking, especially on tiptoe

Diagnosis Palpation of gap (best to test in first 2–3 hours as haematoma can fill gap) Positive Thompson test: compression of the calf muscles reveals an absent plantar reflex on the affected side (kneel on chair facing backwards)

FIGURE 57.5 Calf squeeze test for ruptured Achilles tendon: (a) intact tendon, normal plantar flexion, (b) ruptured tendon, foot remains stationary

Treatment Early surgical repair (within 3 weeks)

Partial rupture Similar history and clinical findings. Localised tenderness and very tender defect about the size of the tip of the finger. Refer for evaluation—if gap, early surgical exploration and repair. Conservative treatment if no gap, including heel raise and crutches early.

Achilles tendon bursitis Bursitis can occur at two sites: posterior and superficial—between skin and tendon deep (retrocalcaneal)—between calcaneus and tendon (see FIG. 57.4

)

The former occurs mainly in young women from shoe friction and is readily palpated. Tenderness from the deep bursitis is elicited by squeezing in front of the tendon with the thumb and index finger: a swelling may be seen bulging on either side of the tendon. Page 704

Treatment Avoid shoe pressure (e.g. wear sandals) 1–2 cm heel raise inside the shoe Apply local heat and ultrasound NSAIDs Inject corticosteroid into bursa with a 25 gauge needle

Fat pad disorders A tender heel pad or cushion causes a dull throbbing pain under the heel. It is localised more proximal to that of plantar fasciitis. Once established, it is very difficult to treat. The fat pad, which consists of globules of fat encapsulated in multiple U-shaped septa, acts as a

hydraulic shock absorber on heel strike. It also contains significant nerve endings.11 It can undergo atrophy, especially in the elderly, and also become inflamed.

Treatment Reduction of aggravating activity Weight loss (if applicable) Simple analgesics Orthotic (cushioning heel cup) ± foam insert Good footwear Problems are treated with an orthotic or an insert and good footwear. Corticosteroids should be avoided as they can accelerate the atrophy.12

Plantar fasciitis This common condition (also known as ‘policeman’s heel’) is characterised by pain of gradual onset on the plantar aspect of the heel, especially on the medial side; it usually occurs about 5 cm from the posterior end of the heel, although it can be experienced over a wide area beneath the heel. The pain radiates into the sole.

Clinical features Pain: under the heel first steps out of bed relieved after walking increasing towards the end of the day worse after sitting May be bilateral—usually worse on one side Typically over 40 years Both sexes Sometimes history of injury or overuse Few studies have investigated relationship to footwear

Signs12 Tenderness: localised to medial tuberosity may be more posterior may be lateral may be widespread not altered by tensing fascia (but this action may cause pain) Heel pad may bulge or appear atrophic Crepitus may be felt No abnormality of gait, heel strike or foot alignment Patient often obese

Treatment Plantar fasciitis not associated with one of the spondyloarthritides tends to heal spontaneously in 12–24 months. It has a variable response to treatment with NSAIDs, injections, ultrasound and insoles. Rest from long walks and from running is important. Systematic reviews to date indicate that taping is effective for short-term relief. Plantar fascia-stretching exercises, when combined with prefabricated insoles and a short course of NSAIDs, are effective for short-term and longterm pain relief.7 Another systematic review supports a conservative approach based on maximising comfort during the 3-month period of considerable discomfort.9

Protection Symptomatic relief is obtained by protecting the heel with an orthotic pad to include the heel and arch of the foot (e.g. Rose insole). Otherwise, a pad made from sponge or sorbo rubber that raises the heel about 1 cm is suitable. A hole corresponding to the tender area should be cut out of the pad to avoid direct contact with the sole. The aim is to get all of the foot to take the stress.

Injection technique Disabling plantar fasciitis can be treated by injecting local anaesthetic and long-acting corticosteroid into the site of maximal tenderness in the heel. However, a Cochrane review suggested minimal benefit, and only for the first month.13 The injection is painful: an alternative is to inject the corticosteroid after a posterior tibial nerve block.

‘Cracked’ heels

Soak feet for 30 minutes in warm water containing an oil such as Alpha Keri or Derma Oil. Pat dry, then apply a cream such as Nutraplus (10% urea) or Eulactol heel balm. Use hydrocortisone 0.5% cream for resistant cases. For severe cases, use sorbolene cream with 20% glycerol and 30% urea (test skin sensitivity first). Page 705

Arthritic conditions Arthritis of the foot or ankle is a rather meaningless diagnosis and specificity is required. Typical sites of arthritic targets are shown in FIGURE 57.6 .

FIGURE 57.6 Typical sites of arthritic causes of podalgia on skeleton of right foot (plantar aspect)

Osteoarthritis

Osteoarthritis may occur in any of the joints of the foot, but it commonly involves the first MTP joint, leading to hallux rigidus. It can affect the subtalar joint, but the ankle joint proper is usually not affected by osteoarthritis.

Hallux rigidus Osteoarthritis of the first MTP joint can lead to gradual loss of motion of the toe and considerable discomfort. Roomy protective footwear and relative rest is the basis of treatment, coupled with daily self-mobilisation (stretching toe into plantar flexion morning and night). Other measures include manipulation under general anaesthesia or surgery (arthrodesis or arthroplasty) for severe cases.

Rheumatoid arthritis Rheumatoid arthritis is typically a symmetrical polyarthritis presenting with pain in the MTP joints. It may also affect the ankle, midtarsal and tarsometatarsal joints. The interphalangeal joints are seldom affected primarily. It causes pain and stiffness under the balls of the feet, especially first thing in the morning.

Gout Gout typically affects the first MTP and should be considered with the sudden onset of pain, especially in the presence of redness, swelling and tenderness. It can affect any synovial joint and occasionally may be polyarticular. Gout is often dismissed by the patient as a ‘sprain’. A history of alcohol consumption or diuretic treatment is relevant (see CHAPTER 25 ).

Spondyloarthritides This group of arthritic disorders (reactive arthritis, ankylosing spondylitis, psoriatic arthritis and arthritides associated with chronic bowel disorders) may involve peripheral joints. Other foot involvement includes plantar fasciitis, Achilles tendinitis and sausage-shaped toes due to tenosynovitis, and arthritis of the proximal interphalangeal joints.

General foot disorders ‘Burning’ feet It is not uncommon for people, especially the elderly, to present with the complaint of ‘burning’ feet. A careful history is needed to elicit exactly what they mean by ‘burning’—is it real pain, a cold sensation or paraesthesia? A checklist of causes is as follow: vascular: ischaemic rest pain from small vessel disease, chilblains or other cold reaction,

functional vasospasm (Raynaud phenomenon) diabetic neuropathy tarsal tunnel syndrome (see CHAPTER 55

)

complex regional pain syndrome I or II Morton neuroma (localised pain between toes) psychogenic, especially anxiety It is worth considering tarsal tunnel syndrome if there is anterior burning pain in the forefoot with associated aching in the calf. It is usually present in menopausal women and worse at night. It is caused by entrapment of the posterior tibial nerve near the medial malleolus, and may be associated with rheumatoid arthritis. Treat with physiotherapy, a medial arch support and a corticosteroid injection before contemplating surgery. Page 706

Foot strain Foot strain is probably the commonest cause of podalgia. A foot may be strained by abnormal stress, or by normal stress for which it is not prepared. In foot strain, the supporting ligaments become stretched, irritated and inflamed. It is commonly encountered in athletes who are relatively unfit or have a disorder such as flat feet, or in obese adults.

Symptoms and signs Aching pain in foot and calf during or after prolonged walking or standing Initial deep tenderness felt on medial border of plantar fascia (see FIG. 57.7 Worse with new shoes, especially a change to high heels

)

FIGURE 57.7 Typical sites of important causes of podalgia (other than arthritis): right foot

Acute foot strain Acute ligamentous strain, such as occurs to the occasional athlete or to the person taking long unaccustomed walks. It is usually self-limiting and recovers rapidly with rest.

Chronic foot strain Foot strain will become chronic with repeated excessive stress or with repeated normal stress on a mechanical abnormality. A common consequence is an everted foot, leading to flattening of the longitudinal arch on weight-bearing. It is important to establish whether the symptoms commenced after the patient began wearing a different type of footwear.

Treatment The treatment is basically the same as that of the adult flat foot. Acute strain is treated with rest and by reducing walking to a minimum. Try the application of cold initially and then heat. The

management of chronic strain is based on an exercise program and orthotics, including arch supports, to correct any deformity. Aching feet Avoid wearing high heels. Wear insoles to support the foot arch. Perform foot exercises. Soak the feet in a basin of warm water containing therapeutic salts (Epsom salts is suitable). Massage feet with baby oil followed by a special ribbed wooden foot massager.

Flat feet (pes planus) Flat feet are normal in young children. No treatment is required in flat feet in which the arch is restored by standing on tiptoe (see CHAPTER 85 ). If painful, treat with exercises and insoles. Refer if concerned. Hind foot fusion can be performed for severe pain.

Claw foot (pes cavus) High foot arch is usually of congenital origin. It may be secondary to various neurological conditions (and previously polio). The foot is inflexible and the toes may be ‘hammer’ or clawed. Treatment includes special orthotics with good shock-absorbing properties, appropriate footwear, foot exercises and padding under the metatarsal heads. Operative treatment involves soft tissue release or arthrodesis to strengthen toes.

Disorders of the ankle tendons Inflammation of a tendon sheath surrounding the ankle may result from repetitive overuse, trauma such as a sprained ankle or unaccustomed stress, including sporting injuries. Tenosynovitis commonly involves the tibialis posterior tendon over the medial compartment or the peroneal tendons over the lateral compartment. It may also affect the tibialis anterior and extensor digitorum longus tendons. Friction at the point where the tendons become angulated at the ankle causes the inflammation. The patient presents with pain, swelling and restricted movement. On examination there is swelling and tenderness where the tendon bulges out from behind and below the malleolus. If necessary, the diagnosis can be confirmed by ultrasound or MRI imaging.

Page 707

Complications include tenovaginitis, weakness, ganglion formation, subluxation or dislocation and rupture.

Treatment of tendinitis includes partial immobilisation (rarely in a cast) or an orthotic device to support the arch of the foot. A carefully directed injection of corticosteroid into the tendon sheath can be very effective.

Peroneal tendinitis This occurs along the course of the tendon from behind the lateral malleolus to the outer side of the foot and is common in athletes and ballet dancers. Pain is reproduced on palpation, on stretching the tendons by passive inversion of the foot or by resisting eversion of the foot.

Peroneal tendon dislocation This is most commonly a dislocated leg tendon as a result of forceful dorsiflexion. An audible painless snapping sensation may be experienced. Surgical repair is necessary.

Tibialis posterior tendinopathy This is a common problem, especially in middle-aged females, in ballet dancers and in those with pes planus with a valgus deformity. The tendon (see FIG. 57.8 ), which is an invertor of the foot, is attached to the navicular tuberosity.14

FIGURE 57.8 Medial aspect of the foot illustrating tendon disorders: tendinopathy of tibialis anterior and tibialis posterior

Clinical features11,14 Pain and a feeling of weakness in the medial ankle and foot Pain aggravated by standing and walking Standing on toes is painful and difficult Pain on palpation anterior and inferior to the medial malleolus

Pain on stretching into eversion, and resisted active inversion May cause tarsal tunnel syndrome

Diagnosis Ultrasound examination—but MRI is the gold standard for delineating tendon tears and inflammation.15

Treatment This is basically conservative with a good outcome in 12–24 months. Orthotic correction (bilateral) with semi-rigid orthosis to support faulty arch Exercises under physiotherapist guidance Remedial massage Consider ultrasonic guided injection of corticosteroids into tendon sheath (but best avoided) and a surgical opinion for failed conservative management.

Tibialis posterior tendon dislocation This can occur with forceful ankle dorsiflexion and inversion. The patient usually experiences pain and cannot weight-bear. The dislocated tendon may be seen overlying the medial malleolus. Immediate surgical repair is recommended.

Tibialis posterior tendon rupture Rupture of the tibialis posterior tendon after inflammation, degeneration or trauma16 is a relatively common and misdiagnosed disorder, especially in middle-aged females. It causes collapse of the longitudinal arch of the foot, leading to a flat foot.5 It is uncommon for patients to feel obvious discomfort at the moment of rupture. They may subsequently present with the sudden appearance of an ‘abnormal’ flat foot. There is gross eversion of the foot. A simple test is the ‘too many toes’ test whereby more toes are seen on the affected side when the feet are viewed from about 3 m behind the patient.5 The single heel raise test is also diagnostic. The most useful investigation is an ultrasound examination. Minor cases can be treated conservatively with orthotics, but severe problems respond well to surgical correction. Page 708

Sesamoiditis1 The two sesamoids that lie beneath the head of the first metatarsal may develop painful conditions such as chondromalacia, osteoarthritis and stress fractures. A special ‘sesamoid’ Xray assists diagnosis. A painful callus can develop over here in the elderly. Well-designed insoles are usually effective, as is surgical excision for persistent problems.

Metatarsalgia1 Metatarsalgia is a symptom rather than a disease and refers to pain and tenderness over the plantar heads of the metatarsals (the forefoot). Causes include foot deformities (especially with depression of the transverse arch), leading to painful strain, arthritis of the MTP joints, trauma, Morton neuroma, Freiberg disorder and entrapment neuropathy. However it can occur in normal feet after prolonged standing. Depression of the transverse arch results in abnormal pressure on the second, third and fourth metatarsal heads with possible callus formation. Repetitive foot strain, pes cavus and high heels may cause a maldistribution of weight to the forefoot. Treatment involves treating any known cause, advising proper footwear and perhaps a metatarsal bar. Flat-heeled shoes with ample width seldom cause problems in the metatarsal region.

Stress fractures17 Clinical features The aches or pains may be slow in onset or sudden Common in dancers, especially classical ballet, and in unfit people taking up exercise Examination is often unhelpful: swelling uncommon15 Routine X-rays often unhelpful A bone scan is the only way to confirm the suspected diagnosis Basis of treatment is absolute rest for 6 or more weeks with strong supportive footwear A walking plaster is not recommended

Avulsion fracture of base of fifth metatarsal Known also as a Jones fracture, it is usually a traumatic fracture but can be a stress fracture and associated with severe ankle sprains.

March fracture of metatarsal

Stress or fatigue fracture of the forefoot usually involves the neck of the second metatarsal (sometimes the third). Swelling typically occurs along the bone shaft. X-ray changes are often delayed. Treat conservatively with support from a firm elastic bandage; avoid painful activities. Resolution may take many months.

Tarsals, especially navicular Stress fracture of the navicular, which is a disorder of athletes involved with running sports, presents as poorly localised midfoot pain during weight-bearing. Examination and plain X-ray are usually normal. It is a recently recognised serious disorder due to the advent of nuclear bone scans and CT scans. A protracted course of treatment can be expected.

Calcaneum Stress fractures of the os calcis usually have an insidious onset. Osteoporosis is a predisposing factor, as is an increased training program.17

Morton neuroma15 Morton interdigital neuroma is probably misdiagnosed more often than any other painful condition of the forefoot. It is not a true neuroma but a fibrous enlargement of an interdigital nerve, and its aetiology is still uncertain. It is related to overuse and inappropriate footwear. The diagnosis is made on clinical grounds. An ultrasound examination may detect a neuroma.

Clinical features Usually presents in adults 1000 U/L). A result >125 U/L suggests active disease.6 Note: Calcium and phosphate normal. Plain X-ray: dense expanded bone—best seen in skull and pelvis.

Note: Can mimic prostatic secondaries, so every male Pagetic patient should have a DRE and serum PSA test. Bone isotopic scans: useful in locating specific areas. Watch for the uncommon complication of osteogenic sarcoma. Note: Screen siblings and children every 5 years after the age of 40.6

Treatment5,6 The major goals are relief of pain, normal biochemical indices of bone turnover and prevention of long-term complications (e.g. deafness, deformities). Treatment is required for symptomatic features such as bone pain and neurological complications, and for selected asymptomatic patients such as those with active disease and 35) If zoledronic acid is inappropriate, use: pamidronate disodium 30–60 mg IV infused over 2–4 hours or risedronate 30 mg (o) daily for 2 months (on empty stomach) Repeated doses may rarely be required in severe cases as judged by symptoms and disease activity (e.g. monitoring with serum ALP usually 6 months post-therapy and then 2 yearly; Xrays also help monitor response).

Leg swelling Diagnostic features and pitfalls Not all swollen legs require investigation and treatment. The significance of leg swelling varies according to the age group, whether it is bilateral or unilateral, and whether the onset is sudden or gradual (see TABLE 58.2 ). If the onset of oedema is acute (often 40 years presenting with painless unilateral leg oedema. A drug history is essential as several drugs can cause oedema. Pitting oedema is a feature of venous thrombosis or insufficiency, not lymphatic obstruction. Table 58.2

Leg and ankle swelling: diagnostic strategy model

Probability diagnosis Physiological: dependency/gravitational prolonged sitting, standing, walking hot weather pregnancy mechanical (e.g. constricting clothing) Chronic venous insufficiency (varicose veins) Congestive cardiac failure Drugs (e.g. calcium antagonists, NSAIDs, steroids, glitazones, beta blockers) Local trauma Obesity Serious disorders not to be missed Vascular: deep venous thrombosis (DVT) inferior vena cava thrombosis thrombophlebitis Infection: cellulitis tropical infections (e.g. filariasis, hookworm) Cancer: obstruction from pelvic cancer localised malignancy Other: kidney disease (e.g. nephrotic syndrome)

liver disease (e.g. cirrhosis) skin allergy (e.g. angioneurotic oedema) Pitfalls (often missed) Idiopathic (periodic or cyclic) oedema Protein-losing enteropathy (e.g. Crohn) Lipoedema (fat and fluids) of legs Factitious oedema Rarities: malnutrition lymphoedema: primary or secondary Seven masquerades checklist Diabetes Drugs (multiple) Thyroid/endocrine (hypothyroidism, Cushing syndrome) Anaemia

Investigations Select from these first-line tests: urinalysis (?albumin) FBE and ESR serum UEC, LFTs, glucose TSH level ultrasound (DVT screen) other radiographs (e.g. CT scan, venogram)

Calf swelling of sudden onset Causes to consider: acute arterial occlusion ruptured Baker cyst ruptured medial head of gastrocnemius

DVT (usually gradual) cellulitis/erysipelas compartment syndrome Pain accompanies most of these conditions but the absence of pain does not exclude DVT or thrombophlebitis. Refer to CHAPTER 55 .

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Lipoedema Lipoedema (also spelled lipedema) is the development of painful bilateral leg swelling that does not involve the feet (whereas in lymphoedema the swelling starts with the most distal part of the foot). Lipoedema is abnormal fatty tissue.

Clinical features Exclusive to obese women; hereditary Typically affects the thighs, buttocks and lower legs (sometimes the arms) Spares the feet Bilateral and symmetrical distribution of fat The legs are often painful and bruise easily The Stemmer sign (the ability to pick up a fold of skin at the base of the large toe) is usually negative8 It is difficult to treat. The focus is on changes to diet, exercises, massage and bandage compression.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Deep venous thrombosis Paget disease of bone Parkinson disease

Resource A physician’s guide to the management of Paget disease of bone. Brooklyn NY: The Paget

Foundation. Available from: https://www.paget.org/index-php/healthcareprofessionals/pagets-disease-of-bone/126-a-physicians-guide-to-the-management-of-pagetsdisease-of-bone.html.

References 1

Horne M. Gait and postural disorders. Monash University Neurology Notes, 1996: 1–4.

2

Talley NJ, O’Connor S. Clinical Examination: A Systematic Guide to Physical Diagnosis (7th edn). Sydney: Churchill Livingstone/Elsevier, 2014: 459–61.

3

Gwee A, Rimer R, Marks M, eds. Paediatric Handbook (9th edn). Melbourne: Blackwell Science, 2015: 227–8.

4

Bone and joint infections [published 2019]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2019. www.tg.org.au, accessed October 2019.

5

Ralson D, Langston AL, Reid IR. Pathogenesis and management of Paget disease of bone. Lancet, 2008; 372(9633): 155–63.

6

Paget disease of bone [published 2019]. In: Therapeutic Guidelines. [digital]. Melbourne: Therapeutic Guidelines Limited; 2019. www.tg.org.au, accessed October 2019.

7

Diu P, Juergens C. Clinical approach to the patient with peripheral oedema. Medicine Today, October 2009; 10 (10): 37–42.

8

Piller N, Birrell S. Lymphoedema: how to treat. Australian Doctor, 6 June 2003: I–VIII.

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59 Palpitations

The most important requirement of the art of healing is that no mistakes or neglect occur. There should be no doubt or confusion as to the application of the meaning of complexion and pulse. These are the maxims of the art of healing. HUANG TI (THE YELLOW EMPEROR) (2697–2597 BCE) Palpitations are an unpleasant awareness of the beating of the heart. By definition it does not always imply ‘racing’ of the heart but any sensation in the chest, such as ‘pounding’, ‘flopping’, ‘skipping’, ‘jumping’, ‘thumping’ or ‘fluttering’ of the heart. The problem requires careful attention and reassurance (if appropriate) because heartbeat is regarded as synonymous with life. To the practitioner it may simply represent anxiety or it could be a prelude to a cardiac arrest. In many circumstances prompt referral to a cardiologist is imperative.

Key facts and checkpoints The symptom of palpitations is suggestive of cardiac arrhythmia but may have a non-cardiac cause. Palpitations not related to emotion, fever or exercise suggest an arrhythmia. Symptomatic premature ventricular beats/complexes (ventricular ectopics) are common, and atrial fibrillation is the most common sustained cardiac arrhythmia (1– 2% of population), while cardiologists claim that the commonest is the symptomatic ventricular ectopic beat.1 The commonest cause of an apparent pause on the ECG is a blocked premature atrial beat/complex (atrial ectopic). A 12-lead electrocardiographic diagnosis is mandatory. If the cause is not documented, an ambulatory electrographic monitor (e.g. Holter) may be used. Consider myocardial ischaemia as a cause of the arrhythmia. Consider drugs as a cause, including prescribed drugs and non-prescribed drugs such as alcohol, caffeine and cigarettes.

Common triggers of paroxysmal supraventricular tachycardia (PSVT) include anxiety and cigarette smoking. The commonest mechanism of any arrhythmia is re-entry. Ask patients to tap out the rate and rhythm of their abnormal beat.

A diagnostic approach A summary of the diagnostic strategy model is presented in TABLE 59.1 significant causes of palpitations. Table 59.1

Palpitations: diagnostic strategy model

Probability diagnosis Anxiety Premature beats (ectopics) Sinus tachycardia Drugs (e.g. stimulants) Supraventricular tachycardia Serious disorders not to be missed Myocardial infarction/angina Arrhythmias: atrial fibrillation/flutter ventricular tachycardia bradycardia sick sinus syndrome torsade de pointes Long QT syndrome Wolff–Parkinson–White (WPW) syndrome Electrolyte disturbances: hypokalaemia hypomagnesaemia hypoglycaemia (type 1 diabetes) Pitfalls (often missed) Fever/infection Pregnancy

, which includes

Menopause (sudden vasodilatation) Drugs (e.g. caffeine, cocaine) Mitral valve disease Aortic incompetence Hypoxia/hypercapnia Rarities: tick bites (T1–5) phaeochromocytoma Seven masquerades checklist Depression Diabetes (indirect) Drugs Anaemia Hyperthyroidism Spinal dysfunction UTI (possible) Is the patient trying to tell me something? Quite likely. Consider cardiac neurosis, anxiety.

Probability diagnosis If the palpitations are not caused by anxiety or fever, the common causes are sinus tachycardia and premature complexes/ectopics (atrial or ventricular). Sinus tachycardia, which by definition is a rate of 100–160/min (bpm), may be precipitated by emotion, stress, fever or exercise. PSVT and atrial fibrillation are also quite common arrhythmias. Sinus tachycardia can be differentiated clinically from PSVT in that it starts and stops more gradually than PSVT (abrupt) and has a lower rate of 100–150 compared with 160–220. Important causes of tachyarrhythmias are: ischaemic heart disease, especially acute coronary syndromes hypertension heart failure mitral disease thyrotoxicosis

atrial septal deficit

Serious disorders not to be missed It is vital not to overlook acute coronary syndromes as a cause of the arrhythmia manifesting as palpitations. About 25% of infarcts are either silent or unrecognised. Sinister life-threatening arrhythmias are: ventricular tachycardia atypical ventricular tachycardia (torsade de pointes) sick sinus syndrome (SSS) complete heart block It is also important not to miss:

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hypokalaemia hypomagnesaemia

Pitfalls There are many pitfalls in the diagnosis and management of arrhythmias, especially in the elderly, where symptoms of infection may be masked. Palpitations associated with the menopause can be overlooked. Valvular lesions, usually associated with rheumatic heart disease, such as mitral stenosis, and aortic incompetence may cause palpitations. The rare tumour, phaeochromocytoma, presents with palpitations and the interesting characteristic of postural tachycardia (a change of more than 20 beats/ min). The toxin from tick bites in dermatomes T1– 5 can cause palpitations.

General pitfalls Misdiagnosing PSVT as an anxiety state Overlooking a cardiac arrhythmia as a cause of syncope or dizziness Overlooking atrial fibrillation (AF) in the presence of a slow heartbeat Overlooking mitral valve prolapse in someone, especially a middle-aged woman presenting with unusual chest pains and palpitations (auscultate in standing position to accentuate click(s) ± murmurs)

Red flags for palpitations

Lightheadedness Chest pain New onset of irregular heart rhythm Heart rate >120 or 50) Anorexia, nausea, urticaria Diarrhoea, headache, tinnitus

Disopyramide Procainamide Quinidine

Ib

Lignocaine Mexiletine

IV use 200 mg tid

Nausea, dizziness, tremor Nausea, vomiting, tremor, dizziness

Ic

Flecainide

100 mg bd

Nausea, dizziness, rash

II

Beta blockers (slows AV conduction)

Various

Fatigue, insomnia, nightmares, hypotension, bronchospasm Avoid in asthmatics

III

Potassium channel blockers (prolongs action potential) Amiodarone Sotalol

SVT: 200 mg daily VT: 400 mg daily 80–160 mg bd

Rash, pulmonary fibrosis, thyroid, hepatic and CNS effects As for beta blockers

IV

Calcium-channel blockers Verapamil Diltiazem

(SR) 160–480 mg daily (CR) 180–360 mg daily

Constipation, dizziness, hypotension Hypotension, headache

Note: Sotalol is a beta blocker and thus is a class II and III agent. All drugs are taken orally unless IV indicated. Adenosine and digoxin are not classified.

Management strategies Treat the underlying cause. Give appropriate reassurance. Provide clear patient education. Explain about the problems of fatigue, stress and emotion. Advise moderation in consumption of tea, coffee, caffeine-containing soft drinks and alcohol.

Page 727 Page 728

Advise about cessation of smoking and other drugs.

Sinus bradycardia Sinus bradycardia can be a normal occurrence, but look for causation (e.g. hypothyroidism, myocardial ischaemia and drugs). Correct the cause. Treatment is required only if symptomatic, which is uncommon at rates >40–45 bpm. Use IV atropine or isoprenaline if acute treatment is required. However, mild or transient bradyarrhythmias may be asymptomatic or even

physiological, such as in a healthy athlete. Palpitations are not a feature but they can cause dizziness, fatigue or syncope (e.g. Stokes–Adams attack—transient bradycardia due to complete heart block).

Stokes–Adams attack Sudden onset without warning Patient falls to ground Collapse with loss of consciousness Pallor and still as if dead with slow or absent pulse

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Recovery—in seconds back to normal Patient flushes as pulse increases Refer for management as attacks may be recurrent

Premature (ectopic) complexes Premature (ectopic) atrial complexes These are usually asymptomatic. Management is based on reassurance. Check lifestyle factors such as excess alcohol, caffeine, stress and smoking; avoid precipitating factors. Drug treatment is rarely required and should be avoided if possible. At present there is no ideal anti-ectopic agent. They may be a forerunner of other arrhythmias (e.g. PSVT, atrial fibrillation). For intolerable symptoms give:6 atenolol or metoprolol 25–100 mg (o) daily or verapamil SR 160–480 mg (o) daily Page 730

Premature (ectopic) ventricular complexes

These are also usually asymptomatic (90%). They occur in 20% of people with ‘normal’ hearts. Symptoms are usually noticed at rest in bed at night. Check lifestyle factors as for atrial premature beats. Drugs that can cause both types of premature beats include digoxin and sympathomimetics. Look for evidence of ischaemic heart disease, mitral valve prolapse (especially women), thyrotoxicosis and left ventricular failure. Ventricular premature complexes may be a forerunner of other arrhythmias (e.g. ventricular tachycardia). If symptomatic but otherwise well with a normal chest X-ray and ECG, reassure the patient. Drug therapy: never commence drug therapy without performing an echocardiograph. This will help to guide the choice of agent. Class 1 agents can make the arrhythmia worse or even life-threatening if there is reduced ventricular function. If considering therapy, refer to a cardiologist. For troublesome symptoms, the beta blockers atenolol or metoprolol can be used, e.g. atenolol 25–100 mg (o) daily.

Supraventricular tachycardia9,10 SVT can be paroxysmal or sustained. Rate is 160–220/min. There are at least eight different types of SVT with differing risks and responses to treatment. PSVT commonly presents with a sudden onset in otherwise healthy young people. Passing copious urine after an attack is characteristic of PSVT. Look for predisposing factors such as an accessory pathway and thyrotoxicosis. Approximately 60% are due to atrioventricular (AV) node re-entry and 35% due to accessory pathway tachycardia (e.g. WPW).10 Look for evidence of accessory pathways after reversion because accessory pathways can lead to sudden death (avoid digoxin in WPW). Consider SSS in a patient with SVT and dizziness.

Wolff–Parkinson–White syndrome The structural basis for the arrhythmia of SVT in WPW syndrome is an accessory pathway that bypasses the AV node. A typical ECG shows a short PR interval and slurred upstroke of the QRS complex (delta wave). Patients are prone to sudden attacks of SVT. Up to 30% of patients will develop atrial fibrillation or flutter. Even one episode of PSVT requires consideration for radiofrequency ablation.11

Management of PSVT 1. Vagal stimulation can be attempted. Carotid sinus massage was the treatment of choice, but it has fallen out of favour because of the potential for stroke. A popular method is to blow hard into the end of an empty 20 mL plastic syringe to move the plunger. Other methods of vagal stimulation include: Valsalva manoeuvre (easiest for patient) self-induced vomiting ocular pressure (avoid) cold (ice) water to face or swallowing ice immersion of the face in water 2. If vagal stimulation fails, give adenosine IV (try 6 mg first over 5–10 seconds, then 12 mg in 2 minutes if unsuccessful, then 18 mg in 2 minutes if necessary and well tolerated). Second-line treatment is verapamil or metoprolol. Verapamil IV 5–10 mg over 2 minutes. If ineffective but well tolerated, repeat dose after 30 minutes. Metoprolol IV 2.5–5 mg over 2 minutes. If ineffective but well tolerated, repeat dose after 10 minutes.10 Precautions: Adenosine causes less hypotension than verapamil but may cause bronchospasm in asthmatics, as may metoprolol Use only if narrow QRS and BP >80 mm Hg Carefully monitor blood pressure AVOID verapamil if taking beta blockers and persistent tachycardia with QRS complexes >0.14 s (suggests ventricular tachycardia) 3. In the rare event of failure of medical treatment, refer for consideration of DC cardioversion or

overdrive pacing.

Prevention and maintenance To prevent recurrences (frequent episodes) use first-line atenolol, metoprolol, sotalol or verapamil. Second-line: add flecainide (only if echocardiography shows no structural heart damage). If these agents fail, consider amiodarone. Radiofrequency catheter ablation, which is usually curative, is indicated for frequent attacks not responding to medical therapy.

Carotid sinus massage1 Carotid sinus massage causes vagal stimulation and its effect on SVT is all or nothing. It slows the sinus rate and breaks the SVT by blocking AV nodal conduction. In general, right carotid pressure tends to slow the sinus rate and left carotid pressure tends to impair AV nodal conduction. Never massage both sides simultaneously. As a rule, avoid this method and use one of the simpler alternatives.

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Precautions Avoid in the elderly (risk of embolism or bradycardia).

Ventricular tachycardia Management requires special supervision.8,11 It may be non-sustained, where drug treatment such as beta blockers and amiodarone may be used for symptomatic cases, or sustained, which may result in cardiac arrest. An implantable cardioverter defibrillator or radiofrequency catheter ablation may be used.

Torsades de pointes This uncommon condition is potentially lethal as it may occasionally be prolonged or jump into ventricular fibrillation with sudden death. It is associated with disorders that prolong the QT interval. Treatment involves cessation of drugs that may prolong the QT interval, correcting electrolyte imbalance (especially potassium), cardiac pacing and IV magnesium sulphate or IV isoprenaline.8

Atrial fibrillation Facts and figures A common problem (9% incidence in the over-65 age group).7 It usually presents with an irregular ventricular rate of about 160–180 bpm in untreated patients with a normal AV node.

Apart from acute AF (new onset 30 ml/min) dabigatran, apixaban or rivaroxaban or warfarin—start with a low dose (e.g. 2–4 mg) and maintain a relatively low INR of 2–3 with regular checks. (It is the appropriate agent for valvular AF.) Do not use aspirin, clopidogrel or ticagrelor for this purpose.

Rate control Aim for a ventricular rate >50 bpm and 2000 per mL of urine (light microscopy) representing the occasional RBC on microscopic examination. Joggers and athletes engaged in very vigorous exercise can develop transient microscopic or even macroscopic haematuria. Microscopic (asymptomatic haematuria) can be classified as either: glomerular (from kidney parenchyma): common causes are IgA nephropathy and thin membrane disease4 or non-glomerular (urological): the common causes are bladder cancer, benign prostate hyperplasia and urinary calculi Common sources of macroscopic haematuria are the bladder, urethra, prostate and kidney.5 Macroscopic haematuria occurs in 70% of people with bladder cancer and 40% with kidney cancer.5 Common urological cancers that cause haematuria are the bladder (70%), kidney (17%), kidney pelvis or ureter (7%) and prostate (5%).6 It is important to exclude kidney damage, so patients should have blood pressure, urinary protein (ACR) and plasma creatinine levels measured as a baseline. All patients presenting with macroscopic haematuria or recurrent microscopic haematuria require judicious investigation, which may involve both radiological investigation of the upper urinary system and visualisation of the lower urinary system to detect or exclude pathology.

The clinical approach History Is it really haematuria? In many patients the underlying disorder may be suspected from a detailed enquiry about associated urinary symptoms. The presence of blood can be verified rapidly by microscopy so that red discolouration due to haemolysis, beetroot or red food dye can be discounted. The time relationship of bleeding is useful because, as a general rule, haematuria occurring in the first part of the stream suggests a urethral or prostatic lesion, while terminal haematuria suggests bleeding from the bladder. Uniform haematuria has no localising implications. It is most unusual for haematuria to cause anaemia unless it is massive. Massive haematuria is a feature of radiation cystitis. Painful haematuria is suggestive of infection (including sexually acquired urethritis), urethral caruncle, calculi or kidney infarction, while painless haematuria is commonly associated with infection, trauma, tumours or polycystic kidneys. Loin pain can occur as a manifestation of nephritis and may be a feature of bleeding in cancer of the kidney or polycystic kidney. A drug history is relevant, especially with anticoagulants and cyclophosphamide. A diet history should also be considered. It is worth noting that large prostatic veins, secondary to prostatic enlargement located at the bladder neck, may rupture when a man strains to urinate. A summary of the diagnostic strategy model for haematuria is presented in TABLE 65.2

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Table 65.2

Haematuria: diagnostic strategy model

Probability diagnosis Infection: cystitis urethritis pyelonephritis prostatitis Calculi—kidney, ureteric, bladder Serious disorders not to be missed Cardiovascular: kidney infarction

kidney vein thrombosis prostatic varices Neoplasia: kidney tumour urothelial: bladder, kidney, pelvis, ureter prostate cancer Severe infections: infective endocarditis kidney tuberculosis blackwater fever Glomerulonephritis (e.g. post-streptococcal, IgA nephropathy) Kidney papillary necrosis Other kidney disease, e.g. polycystic kidneys, medullary sponge kidney Pitfalls (often missed) Urethral prolapse/caruncle Pseudohaematuria (e.g. beetroot, porphyria) Haemorrhagic cystitis Benign prostatic hyperplasia Trauma: blunt or penetrating Foreign bodies Bleeding disorders Vigorous exercise Radiation cystitis Menstrual contamination Rarities: hydronephrosis Henoch–Schönlein purpura bilharzia schistosomiasis polycystic kidneys benign renal masses endometriosis (bladder) systemic vasculitides Seven masquerades checklist Drugs (cytotoxics, anticoagulants) UTI Is the patient trying to tell me something?

Consider artefactual haematuria.

Key questions Have you had an injury such as a blow to the loin, pelvis or genital area? Is the redness at the start or end of your stream or throughout the stream? Have you noticed any bleeding elsewhere, such as bruises or nose bleeds? Have you experienced any pain in the loin or abdomen? Have you noticed any burning or frequency of your urine? Have you had any problems with the flow of your urine? Have you been having large amounts of beetroot, red lollies or berries in your diet? Could your problem have been sexually acquired? Have you been overseas recently? Have you been exposed to chemicals (e.g. dyes, rubber) in your work? Have you been aware of any other symptoms? Do you engage in strenuous sports such as jogging? Have you had any kidney problems in the past? Are you taking blood-thinning drugs?

Examination The general examination should include looking for signs of a bleeding tendency and anaemia, and recording the parameters of temperature, blood pressure and the pulse (see FIG. 65.2 ). The heart should be assessed to exclude atrial fibrillation or infective endocarditis with emboli to the kidney, and the chest should be examined for a possible pleural effusion associated with perinephric or kidney infections.

FIGURE 65.2 Features to consider in the physical examination of the patient with haematuria The abdomen should be examined for evidence of a palpable enlarged kidney or spleen. The different clinical findings for an enlarged left kidney and spleen are shown in TABLE 65.3 . Kidney enlargement may be due to kidney tumour, hydronephrosis or polycystic disease. Splenomegaly suggests the possibility of a bleeding disorder. Table 65.3

Differences between spleen and left kidney on abdominal examination

Palpable upper border

Spleen Impalpable

Left kidney Palpable

Movement with inspiration

Inferomedial

Inferior

Notch

Yes

No

Ballotable

No

Yes

Percussion

Dull

Resonant (usually)

Friction rub

Possible

Not possible

The suprapubic region should be examined for evidence of bladder tenderness or enlargement. In men the prostate should be examined rectally to detect benign or malignant enlargement or tenderness from prostatitis. In women, consider a pelvic examination to search for possible pelvic masses. The urethral meatus should be inspected to exclude a urethral caruncle (‘raspberry tumour’) or urethral prolapse. Page 787

Investigations It is important to identify the cause, especially if a possible sequela is impaired kidney function. Urinalysis by dipstick testing (note: high vitamin C intake can interfere). Urine microscopy: formed RBCs in true haematuria red cell morphology can assist in determining whether the source is glomerular or urinary tract red cell casts and dysmorphic red cells indicate glomerular origin Urinary culture: early culture is important because of the common association with infection and consideration of early treatment with antibiotics. If tuberculosis is suspected, three early morning urines should be cultured for tubercle bacilli. Urinary cytology: this test, performed on a urine sample, may be useful to detect malignancies of the bladder and lower tract but is usually negative with kidney cancer. Sensitivity can be increased by testing mid morning or random specimens from three separate voids. Blood tests: appropriate screening tests include a full blood count, ESR and basic kidney function tests (urea and creatinine). If glomerulonephritis is suspected, antistreptolysin O titres and serum complement levels should be measured. PSA is appropriate if prostate cancer is suspected. Radiological techniques—available tests include:7 intravenous urography (IVU); intravenous pyelogram (IVP)—previously the key investigation, largely superseded by CT ultrasound (less sensitive at detecting LUT abnormalities) CT (with or without contrast), e.g. KUB (kidney, ureter, bladder) MRI

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kidney angiography retrograde pyelography Direct imaging techniques: these include urethroscopy, cystoscopy and ureteroscopy. Unless a renal cause is identified, referral to urologist for consideration of cystoscopy is advisable. Kidney biopsy: indicated if glomerular disease is suspected.

Red flags for urgent surgical intervention Anuria Single kidney Bilateral obstruction Concurrent UTI and urolithiasis

Pseudohaematuria Pseudohaematuria is red urine caused by pigments other than red blood cells that simply stain the culture red. Causes include: anthocyanins in food (e.g. beetroot, berries) red-coloured confectionery porphyrins free haemoglobin (e.g. haemoglobinuria) myoglobin (red-black colour) drugs (e.g. pyridium, phenolphthalein—alkaline urine)

Exercise haematuria Exercise or sports haematuria is the passage of a significant number of red cells in the urine during or immediately after exercise, particularly long runs with under-hydration. It has been recorded in a wide variety of athletes, including swimmers and rowers. Dipstick testing is usually positive in these athletes. Despite the theory that it is largely caused by the posterior wall of the bladder impacting repetitively on the base of the bladder during running, there are other possible

factors and glomerular disease must be excluded in the athlete with regular haematuria, especially if dysmorphic red cells are found on microscopy.

Artefactual haematuria Macroscopic haematuria can be a presenting symptom of people with Munchausen syndrome or those seeking opioids by simulating renal colic. If suspected, it is wise to get these people to pass urine in the presence of an appropriate witness before examining the urine.

Urethral caruncle This is a benign granulomatous tumour about the size of a pea in the distal urethra. Almost exclusive to postmenopausal women, it is very tender and bleeds easily. The main symptom is haematuria. It may require cystoscopy and biopsy for diagnosis. Treatment includes warm salt baths and oestrogen creams. Otherwise obliteration by laser vaporisation, cryotherapy, cautery or even surgical excision is used.

Bladder cancer8 Bladder cancer is the seventh most common malignancy, with 90% being transitional cell carcinomas. Other forms include squamous cell carcinoma and adenocarcinoma. Smoking is the most common association. There is little evidence that drinking chlorinated water can predispose.9

Clinical features Haematuria/microhaematuria Irritative symptoms: frequency, urgency, nocturia Dysuria

Diagnosis Urine cytology: three specimens Cystoscopy and biopsy Imaging of upper tracts: ultrasound, CT IV urethrogram is the gold standard Differential diagnosis is haemorrhagic cystitis

Management Treatment depends on the staging and grading.

The common carcinoma in situ is treated with intravesical BCG immunotherapy. This 6-week course and follow-up if necessary leads to 60–75% remission. Other intravesical agents used include various cytotoxics (e.g. mitomycin C). Other treatments include surgery such as tumour resection plus intravesical agents, bladder resection (partial or total) and radio–chemotherapy. Regular surveillance, which may be lifelong, is essential.

Glomerulonephritis10 Glomerulonephritis means kidney inflammation involving the glomeruli. It can be simply classified into: acute nephritic syndrome: haematuria + dysmorphic RBCs/casts + hypertension nephrotic syndrome: oedema + hypoalbuminaemia + proteinuria

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asymptomatic kidney disease DxT haematuria + dysmorphic RBCs/casts + hypertension → acute nephritic syndrome

The main causes of glomerulonephritis–nephritic syndrome are: IgA nephropathy (Berger disease—most common) anti-glomerular basement membrane disease (has an AD genetic link), aka Goodpasture disease post-streptococcal glomerulonephritis systemic vasculitis—ANCA associated others, e.g. SLE

Nephritic syndrome Feature is haematuria + dysmorphic RBCs and casts (microscopy) plus one of following: proteinuria (mild to moderate) hypertension

oedema ↑ s. creatinine oliguria

IgA nephropathy Typically presents as haematuria (smokey urine) in a young male adult at the time of or within 1–2 days of a mucosal infection (usually throat, influenza or URTI) and persists for several days. Other presentations are as incidentally found microscopic haematuria or as previously unsuspected chronic kidney failure. Due to deposition of IgA antibody complexes in the glomeruli, it runs a variable course, but prognosis is usually good. There is no specific treatment to date but immune suppression may be used. Refer suspected cases immediately. Diagnosis is by biopsy.

Acute post-streptococcal glomerulonephritis Typically seen in children (>5 years), especially in Aboriginal and Torres Strait Islander communities following GABHS throat infection or impetigo. Presents after a gap of 1–2 weeks.

Clinical features Irritable, lethargic, sick child Haematuria: discoloured urine (‘Coke’ urine) Peri-orbital oedema (may be legs, scrotum) Rapid weight gain (from oedema) Scanty urine output (oliguria) Hypertension → may be complications

Usual course Oliguria 2 days Oedema and hypertension 2–4 days Invariably resolves Good long-term prognosis

Diagnosis

GABHS antigens Blood urea, creatinine, C3&4 (complement), ASOT, anti-DNase B

Treatment Hospital admission Bed rest Strict fluid balance chart Daily weighing Penicillin (if GABHS +ve) Fluid restriction Low protein, high carbohydrate, low salt diet Antihypertensives and diuretics (as necessary) Follow-up: monitor BP and kidney function. Regular urinalysis (microscopic haematuria may last for years). DxT discoloured urine + peri-orbital oedema + oliguria → poststreptococcal glomerulonephritis

Proteinuria Proteinuria is an important and common sign of kidney disease. The protein can originate from the glomeruli, the tubules or the LUT. Healthy people, however, do excrete some protein in the urine, which can vary from day to day and hour to hour; hence the value of collecting it over 24 hours or comparing the albumin against the standard rate of filtered creatinine (i.e. ACR). While proteinuria can be benign, it always requires further investigation. Important causes of proteinuria are presented in TABLE 65.4 . Table 65.4

Important causes of proteinuria

Transient Contamination from vaginal secretions Urinary tract infection Pre-eclampsia

Note: These all require exclusion and follow-up. Kidney disease Glomerulonephritis Nephrotic syndrome Congenital tubular disease, e.g. polycystic kidney kidney dysplasia Acute tubular damage Kidney papillary necrosis, e.g. analgesic nephropathy diabetic papillary necrosis Overflow proteinuria, e.g. multiple myeloma, monoclonal gammopathy Systemic diseases/conditions affecting the glomeruli: diabetes mellitus hypertension SLE pre-eclampsia malignancy drugs (e.g. penicillamine, gold salts) amyloid vasculitides No kidney disease Orthostatic proteinuria Exercise Emotional stress Fever Cold exposure Postoperative Acute medical illness (e.g. heart failure) Page 790

Key facts and checkpoints11 The amount of protein in the urine is normally less than 100 mg/24 hours. Greater than 300 mg/24 hours is abnormal in children and adults.

If accompanied by dysmorphic haematuria or red cell casts, this tends to confirm glomerular origin. Routine dipstick testing will detect levels greater than 300 mg/24 hours (ACR of 30– 300 mg/g) only and thus has limitations.12 In diabetics, microalbuminuria is predictive of nephropathy and an indication for early blood pressure treatment.

Urine albumin–creatinine ratio (ACR) Remember the rough ‘rule of 3’ for ACR levels (units are mg/g):13 300 is severe albuminuria.

If proteinuria is confirmed on repeated dipstick testing it should be measured more accurately by measuring the albumin–creatinine ratio (ACR), which is preferable to a 24-hour urinary protein as it avoids finicky 24-hour collections. Refer to CHAPTER 79 . High values require referral for investigation. The minimum investigations are microurine and assessment of kidney function (eGFR). Nephrotic range proteinuria (>3 g/24 hours) is due to one or other form of glomerulonephritis in over 90% of patients.10 Possible contamination from vaginal secretions or from a low UTI needs to be excluded.

Orthostatic proteinuria Orthostatic proteinuria is the presence of significant proteinuria after the patient has been standing but is absent from specimens obtained following recumbency for several hours, such as an early morning specimen. It occurs in 5–10% of people,6 especially during their adolescent years. In the majority it is of no significance and eventually disappears without the development of significant kidney disease. However, in a small number the proteinuria can foreshadow serious kidney disease.

Diabetic microalbuminuria The presence of protein in the urine is a sensitive marker of diabetic nephropathy, so regular screening for microalbuminuria in those with diabetes is an important predictor of nephropathy

and other possible complications of diabetes. The use of antihypertensives, particularly ACEi and ARBs, at the microalbuminuria stage may slow the development of overt nephropathy.

Consequences of proteinuria While proteinuria is usually simply a marker of kidney disease, heavy proteinuria in excess of 3 g/24 hours may have severe clinical consequences, including oedema, intravascular volume depletion, venous thromboembolism, hyperlipidaemia and malnutrition. Minimal change glomerulonephritis is the commonest cause of the nephrotic syndrome in childhood and accounts for about 30% of adult nephrotic syndrome.8 It is steroid responsive.

Nephrotic syndrome10,11 DxT proteinuria + generalised oedema + hypoalbuminaemia → nephrotic syndrome

Nephrotic syndrome occurs at any age but is more common in children and has primary and secondary causes that require elucidation. Page 791

Clinical features Proteinuria >3 g/day (+++ or ++++1 on dipstick) Swelling of eyelids and face Generalised oedema, especially peripheral oedema Hypertension Hypoalbuminaemia 4.5 mmol/L Waxy pallor Normal BP Dyspnoea Frothy urine Predisposes to sepsis (e.g. peritonitis, pyelonephritis, thromboembolism).

Causes

1 in 3 (approx.): systemic kidney disease (e.g. diabetes, SLE, amyloid, hepatitis B/C) 2 in 3 (approx.): minimal change disease (most common) idiopathic nephrotic syndrome (based on kidney biopsy) focal glomerular sclerosis membranous nephropathy membranoproliferative glomerulonephritis Others: drugs, malignancy, infection, e.g. malaria

Glossary of terms Functional incontinence Loss of urine secondary to factors extrinsic to the urinary tract (e.g. dementia, endocrine causes). Nocturnal enuresis (or bed-wetting) Involuntary urine loss during sleep. Overactive bladder (detrusor instability) The most common cause of urge incontinence; synonymous with an irritable or unstable bladder; characterised by involuntary bladder contractions, resulting in a sudden urge to urinate, usually accompanied by frequency and nocturia, with or without incontinence. Overflow incontinence Escape of urine following poor bladder emptying. Stress incontinence The involuntary loss of urine on coughing, sneezing, straining or lifting, or any factor that suddenly increases intra-abdominal pressure. Urge incontinence Involuntary loss of urine associated with urgency. Urinary incontinence The involuntary loss of urine during the day or night.

Treatment Immediate referral to renal physician or unit Bed rest Treat causative disorder

Diet modification: low fluid, protein consideration, low salt Medication may include: diuretics ACE inhibitors or ARBs prednisolone pneumococcal vaccination penicillin statins aspirin

Urinary Incontinence A summary of the types of incontinence and their causes is presented in TABLE 65.5 Table 65.5

.

Types of incontinence and their implied causes

Type of incontinence

Likely cause

Stress incontinence

Pelvic floor or sphincter weakness

Urge incontinence

Detrusor overactivity or low compliance

Mixed urinary incontinence

Both pelvic floor weakness and detrusor overactivity

Quiet dribble incontinence

Variable, may be due to overflow (i.e. incomplete emptying)

Continuous leakage

Fistula, ectopic ureter, patulous urethra

Reflex incontinence (without warning)

Neuropathic bladder

Female urinary incontinence Urinary leakage affects around 37% of adult women in Australia.14 Despite its prevalence, many women will not seek treatment. The most common types are stress incontinence, urge incontinence and mixed (both stress and urge).

Assessment The basic assessment of the person with incontinence requires a careful history and examination, exclusion of infection and the keeping of a micturition or bladder chart. Use of a severity index questionnaire is very helpful. Drugs that adversely affect urinary function are presented in TABLE 65.6 . If central nervous system pathology is suspected, referral to a neurologist is required. Page 792

Table 65.6

Drugs that can cause or aggravate incontinence

ACE inhibitors phenoxybenzamine (Dibenyline) prazosin labetalol Bladder relaxants → overflow incontinence: anticholinergic agents tricyclic antidepressants Bladder stimulants → urge incontinence: cholinergic agents caffeine Sedatives → urge incontinence: antidepressants antihistamines antipsychotics hypnotics tranquillisers Others → urge incontinence: alcohol loop diuretics (e.g. frusemide), other diuretics lithium

Investigations Urine microscopy and culture is important to exclude infection, haematuria and glycosuria. Urinary tract ultrasound is often appropriate to measure the post residual volume (>100 mL is abnormal). Urodynamic studies may be required to dispel doubt about the diagnosis, especially with suspected voiding difficulty, neuropathy, failed treatment or when considering surgery.

Management Stress incontinence17 Pelvic floor muscle training (see below) Weight reduction if obese Vaginal oestrogen therapy may help some postmenopausal women Referral for surgical therapy is appropriate if symptoms are severe or unresponsive to conservative treatment

Causes of incontinence A mnemonic: DIAP2E2RS2:15,16 D = Delirium/dementia I = Infection of urinary tract A = Atrophic urethritis P = Pharmacological (e.g. diuretics) P = Psychological (e.g. acute distress) E = Endocrine (e.g. hypercalcaemia, diabetes insipidus) E = Environmental (e.g. unfamiliar surrounds) R = Restricted mobility S = Stool impaction S = Sphincter damage or weakness

The severity index questionnaire How often do you experience urine leakage? 0 = never 1 = less than once a month

2 = one or several times a month 3 = one or several times a week 4 = every day and/or night How much urine do you lose each time? 1 = drops or little 2 = more The total score is the score for the first question multiplied by the score for the second question. 0 = dry, 1–2 = slight, 3–4 = moderate, 6–8 = severe

Pelvic floor muscle training18,19 Evidence is strong. Compared to placebo or no treatment, women with stress incontinence given pelvic floor training were eight times more likely to report a ‘cure’ (56% vs 6%). Best in motivated young women with stress incontinence. At least 3 months trial with supervision (physiotherapist or continence nurse). Basic techniques: Advise the patient to pull up her pelvic muscles to imagine herself stopping passing urine (or controlling diarrhoea) and hold the ‘squeeze’ for a count of 10. Repeat many times daily. Refer to the Patient Education hand-out at the end of this chapter. Urge incontinence Lifestyle intervention: Alter fluid intake—decrease to reduce urinary frequency, increase to improve urine concentration Reduce intake of bladder irritants—caffeine, alcohol, carbonated beverages Avoid constipation Bladder retraining includes pelvic floor muscle therapy, a scheduled voiding program with gradual increases in the duration between voids, urge suppression techniques with distraction or relaxation

a basic first step is to delay voiding when possible and wait for the urge to pass best performed with supervision of a physiotherapist or continence nurse Anticholinergic/antispasmodic drugs (see below)

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Vaginal oestrogen therapy may help some post menopausal women Intravesical treatment with botulinum toxin (second line) Neuromodulation—electrical stimulation of the sacral nerve (third line) Anticholinergic/antispasmodic drugs18 These may be worth a trial for overactive bladder or urge incontinence. They are less effective for stress incontinence: oxybutynin 2.5–5 mg (o) bd or tds oxybutynin transdermal patch 3.9 mg (top) twice weekly solifenacin 5–10 mg (o) daily tolterodine 2 mg (o) bd darifenacin 7.5–15 mg (o) daily

Bladder dysfunction (in women during night) Women with urethral syndrome constantly wake at night with urge to micturate but produce only a small dribble of urine. Instruct patient to perform a pelvic tilt exercise by balancing on upper back, lifting her pelvis with knees flexed and holding position for 30 seconds Squeeze pelvic floor inwards (as though holding back urine or faeces) Repeat a few times

Uterovaginal prolapse20 Uterovaginal prolapse is very common, eventually affecting 50% of parous women. The main complaint is of ‘heaviness’ in the vagina and a sensation of ‘something coming down’. Relevant symptoms that are of considerable distress (depending on the type of prolapse) include voiding difficulties, urinary stress incontinence, faecal incontinence, incomplete rectal emptying and recurrent cystitis. Backache is a common associated symptom, usually relieved by lying down.

Classification of prolapse See FIGURE 65.3

.

Cystocele—bladder descends into vagina Urethrocele—urethra bulges into vagina Rectocele—rectum protrudes into vagina Enterocele—loop of small intestine bulges into vagina (usually posterior wall) Uterine—uterus and cervix descend towards vaginal introitus: first degree—cervix remains in vagina second degree—cervix protrudes on coughing/straining third degree (procidentia)—uterus lies outside vagina

FIGURE 65.3 Uterovaginal prolapse

Examination This is best performed with women in the left lateral position using a Sims speculum or posterior blade of the Graves speculum. Ask the patient to cough or bear down (several times)—observe anterior, posterior and lateral vaginal walls and descent of cervix.

Management As a rule asymptomatic prolapse does not need invasive treatment, just basic reassurance and education, including pelvic floor exercises (see earlier in this chapter). Consider referral to a physiotherapist. Lifestyle measures include optimal nutrition, weight loss if obese, smoking

cessation and exercise. Aggravating comorbidities such as constipation, atrophic vaginitis and COPD require optimal treatment. Consider topical oestrogens in post menopausal women.

Prevention Promote optimal obstetric management, especially postpartum exercises, lifelong pelvic floor exercises, ideal weight and sensible bladder and bowel function.

Ring pessaries Pessaries are an option for those who are poor anaesthetic risks, too frail for surgery, don’t want surgery, are young and have not completed their family or are awaiting surgery. The correctsized pessary needs to be fitted individually. Topical oestrogens will improve comfort. The pessary needs to be cleaned or changed every 4–6 months. Page 794

Surgery Refer to a gynaecological surgeon if a woman who is fit for surgery has symptomatic prolapse that warrants surgery, especially with associated voiding problems or obstructed defecation. The principles of reconstructive pelvic surgery are to: reposition pelvic structures to normal anatomical relationships restore and maintain urinary and/or faecal continence maintain sexual function correct coexisting pelvic pathology Options include repair procedures (vaginally, sometimes abdominally, per laparoscopy), colpo/vaginal suspension and hysterectomy (vaginal or abdominal).

Patient education resource Hand-out sheet from Murtagh’s Patient Education 8th edition: Incontinence of urine

References 1

Cormack J, Marinker M, Morrell D. Practice. A Handbook of Primary Medical Care. London: Kluwer-Harrap Handbooks, 1980: 3.51: 1–10.

2

Michels TC, Sands JE. Dysuria: evaluation and differential diagnosis in adults. Am Fam

Physician, 2015 (1 Nov); 92(9): 778–86. 3

Kincaid-Smith P, Larkins R, Whelan G. Problems in Clinical Medicine. Sydney: MacLennan & Petty, 1990: 105–8.

4

Mathew T. Microscopic haematuria: how to treat. Australian Doctor, 27 April 2007: 27– 34.

5

Walsh D. Symptom Control. Boston: Blackwell Scientific Publications, 1989: 229–33.

6

George C. Haematuria and proteinuria: how to treat. Australian Doctor, 15 March 1991: I– VIII.

7

Choyke PL. Radiologic evaluation of hematuria: guidelines from the American College of Radiology’s appropriateness criteria. Am Fam Physician, 2008 (1 Aug); 78(3): 347–52.

8

Gray S, Frydenberg M. Bladder cancer: how to treat. Australian Doctor, 21 November 2008: 29–36.

9

Chlorine and cancer. Updated 9 September 2019. Cancer Council Western Australian. Available from: https://www.cancerwa.asn.au/resources/cancermyths/chlorine-cancermyth/, accessed March 2021.

10

Faull R. Glomerulonephritis: how to treat. Australian Doctor, 8 February 2002: I–VIII.

11

Thomson N. Managing the patient with proteinuria. Current Therapeutics, 1996; 9: 7–28.

12

Wen CP et al. Urine dipstick to detect trace proteinuria: an underused tool for an underappreciated risk marker. Am J Kidney Dis, 2011 Jul; 58(1): 1–3.

13

Albuminuria categories in CKD. ACR. National Kidney Foundation. Available from: https://www.kidney.org/kidneydisease/siemens_hcp_acr, accessed March 2021.

14

Key statistics on incontinence. Continence Foundation of Australia. Available from: https://www.continence.org.au/about-us/our-work/key-statistics, accessed March 2021.

15

Jayasuriya P. Urinary incontinence: how to treat. Australian Doctor, 11 May 2001: I–VIII.

16

Whishaw DMK. Urinary incontinence in the frail female: how to treat. Australian Doctor, 25 July 2008: 29–36.

17

Qaseem A et al. Nonsurgical management of urinary incontinence in women: a clinical practice guideline from the American College of Physicians. Ann Intern Med, 2014; 161(6): 429–40.

18

Haylen B. Advances in incontinence treatment. Australian Doctor, 10 September 1999: 66–70. Mazza D. Women’s Health in General Practice (2nd edn). Sydney: Elsevier Australia,

19

2011: 288–94.

20

Haylen B. Pelvic organ prolapse. Australian Doctor, 21 February 2014: 23–30.

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66 Visual failure

All those, therefore, who have cataract see the light more or less, and by this we distinguish cataract from amaurosis and glaucoma, for persons affected with these complaints do not perceive the light at all. PAUL OF AEGINA (615–690 CE) The commonest cause of visual dysfunction is a simple refractive error. However, there are many causes of visual failure, including the emergency of sudden blindness, a problem that requires a sound management strategy. Apart from migraine, virtually all cases of sudden loss of vision require urgent treatment. The ‘white’ eye or uninflamed eye presents a different clinical problem from the red or inflamed eye.1 The ‘white’ eye is painless and usually presents with visual symptoms and it is in the ‘white’ eye that the majority of blinding conditions occur.

Criteria for blindness and driving This varies from country to country. The WHO defines blindness as ‘best visual acuity less than 3/60’, while in Australia eligibility for the blind pension is ‘bilateral corrected visual acuity less than 6/60 or significant visual field loss’ (e.g. a patient can have 6/6 vision but severely restricted fields caused by chronic open-angle glaucoma). The minimum standard for driving is 6/12 (Snellen system) in the better eye or bilaterally. Commercial licence standards are stricter (see Austroads guidelines).

The clinical approach History The history should carefully define the onset, progress, duration, offset and the extent of visual loss. An accurate history is important because a visual defect (particularly unilateral) may only just have been noticed by the patient, even if it has been longstanding. Two questions need to be answered:

Is the loss unilateral or bilateral? Is the onset acute, or gradual and progressive?

Key facts and checkpoints The commonest cause of blindness in the world is trachoma. The other major causes of gradual blindness are cataracts, onchocerciasis and vitamin A deficiency.2 In Western countries, the commonest causes are senile cataract, glaucoma, agerelated macular degeneration, trauma and diabetic retinopathy.2 The commonest causes of sudden visual loss are migraine and transient occlusion of the retinal artery (amaurosis fugax).3 ‘Flashing lights’ are caused by traction on the retina and may have a serious connotation: the commonest cause is vitreoretinal traction, which is a classic precursor to retinal detachment. The presence of floaters or ‘blobs’ in the visual fields indicates pigment in the vitreous: causes include vitreous haemorrhage and vitreous detachment. Posterior vitreous detachment is the commonest cause of the acute onset of floaters, especially with advancing age. Retinal detachment has a tendency to occur in short-sighted (myopic) people. Suspect a macular abnormality where objects look smaller or straight lines are bent or distorted.

The distinction between central and peripheral visual loss is useful. Central visual loss presents as impairment of visual acuity and implies defective retinal image formation (through refractive error or opacity in the ocular media) or macular or optic nerve dysfunction. Peripheral field loss is more subtle, especially when the onset is gradual, and implies extramacular retinal disease or a defect in the visual pathway. It is important to differentiate the central field loss of macular degeneration from the hemianopia of a CVA. A drug history is very important (see TABLE 66.1 ). Tuberculosis treatment with ethambutol or quinine/chloroquine can be oculotoxic. The family history is relevant for diabetes, migraine, Leber hereditary optic atrophy, Tay–Sachs disease and retinitis pigmentosa. Page 796

Table 66.1

Systemic drugs that can cause ocular side effects

Alcohol/ethanol/methanol Amiodarone Antihypertensives e.g. β Blockers Bisphosphonates Chloroquine/hydroxychloroquine/quinine Cyclosporin Cytotoxic agents, e.g. vincristine Corticosteroids Disulfiram Erectile dysfunction agents Ethambutol Indomethacin Nitrofurantoin Phenothiazines Phenytoin Tacrolimus Tamsulosin Tamoxifen Tetracyclines, e.g. minomycin Thiazides Tricyclics Topiramate

Questions directed to specific symptoms Presence of floaters → normal ageing (especially ≥55 years) with posterior vitreous detachment or may indicate haemorrhages or choroiditis Flashing lights → normal ageing with posterior vitreous detachment or indicates traction on the retina (?retinal detachment) Coloured haloes around lights → glaucoma, cataract

Zigzag lines → migraine Vision worse at night or in dim light → retinitis pigmentosa, hysteria, syphilitic retinitis Headache → temporal arteritis, migraine, benign intracranial hypertension Central scotomata → macular disease, optic neuritis Pain on moving eye → retrobulbar neuritis Distortion, micropsia (smaller), macropsia (larger) → macular degeneration Visual field loss: central loss—macular disorder total loss—arterial occlusion peripheral loss It is worth noting that if a patient repeatedly knocks into people and objects on a particular side (including traffic accidents), a bitemporal or homonymous hemianopia should be suspected.

Diseases/disorders to exclude or consider Diabetes mellitus Giant cell (temporal) arteritis Hypopituitarism (pituitary adenoma) Cerebrovascular ischaemia/carotid artery stenosis (emboli) Multiple sclerosis Cardiac disease (e.g. arrhythmias, and SBE—emboli) Anaemia (if severe can cause retinal haemorrhage and exudate) Marfan syndrome (subluxated lenses) Malignancy (the commonest cause of eye malignancy is melanoma of the choroid)

Examination The same principles of examination should apply as for the red eye. Testing should include: visual acuity (Snellen chart)—with pinhole testing

pupil reactions, to test afferent (sensory) responses to light confrontation fields (using a red pin) fundus examination with dilated pupil (ophthalmoscope), noting: the red reflex appearance of the retina, macula and optic nerve Depending on circumstances, also consider: Amsler grid (or graph paper) colour vision (Ishihara chart) tonometry

General examination General examination should focus on the general features of the patient, the nervous system, endocrine system and cardiovascular system.

Slit-lamp examination (biomicroscopy) Usually performed with fluorescein dye, it provides a precise stereoscopic view of the eyelids, conjunctiva, cornea, iris, sclera, anterior chamber (measures depth), lens, anterior vitreous and retina. It is very useful for identifying corneal lesions, uveitis and scleritis. Page 797

Perimetry Various defects in the visual fields are depicted in FIGURE 66.1

.

FIGURE 66.1 Diagrammatic representation of important causes of sudden painless loss of vision (right side) and typical defects in the visual fields (left side)

Investigations Depending on the clinical examination, the following tests can be used selectively to confirm the diagnosis: blood tests: full blood (?anaemia, lead poisoning, leukaemia) ESR (?temporal arteritis) blood sugar (?diabetes mellitus) temporal artery biopsy (?temporal arteritis) CT/MRI scan (?CVA, optic nerve lesions, space-occupying lesions)

formal perimetry and Bjerrum screen fluorescein angiography (?retinal vascular obstruction, diabetic retinopathy) visual evoked responses (?demyelinating disorders) carotid Doppler ultrasound B-scan ocular ultrasound

Visual failure in children There are long lists of causes for visual failure or blindness in children. An approximate order of frequency of causes of blindness in children is cortical blindness, optic atrophy, choroidoretinal degeneration, cataract and retinopathy of prematurity. Almost half the causes of blindness in Western nations are genetically determined, in contrast to the nutritional and infective causes that predominate in developing countries.4 About 3% of children will fail to develop proper vision in at least one eye. The eyes of all babies should be examined at birth and at 6 weeks.

Amblyopia Amblyopia, referred to as ‘lazy eye’, is defined as a reduction in visual acuity due to abnormal visual experience in early childhood. It is the main reason for poor unilateral eyesight until middle age and is usually caused by interference with visual development during the early months and years of life. The common causes are: strabismus large refractive defect, especially hypermetropia congenital cataract

Principles of management5 Most cases are treatable. Early diagnosis and intervention is fundamental to achieving useful vision. No child is too young to have the visual system assessed. The good eye should be patched in order to utilise the affected eye. Remove a remedial cause such as strabismus.

Page 798

Correct any refractive error, usually by prescription of glasses.

Some important guidelines in children Referral Refer if any of the following are present in infants: nystagmus a wandering eye a lack of fixation, or lack of following movements photophobia opacities (seen with ophthalmoscope set on +3, held 30 cm from baby’s eye) delayed development

Strabismus The two serious squints are the constant and alternating ones, which require early referral. Transient squint and latent squint (occurs under stress, e.g. fatigue) usually are not a problem. Always refer children with strabismus (squint) when first seen to exclude ocular pathology such as retinoblastoma, congenital cataract and glaucoma, which would require emergency surgery. Children with strabismus (even if the ocular examination is normal) need specialist management because the deviating eye will become amblyopic (a lazy eye with reduced vision, i.e. ‘blind’) if not functioning by 7 years of age. The younger the child, the easier it is to treat amblyopia; it may be irreversible if first detected later than school age. Surgical correction of a true squint is preferred at 1–2 years of age. (See also CHAPTER 85 .)

Cataracts Children with suspected cataracts must be referred immediately; the problem is very serious as the development of vision may be permanently impaired (amblyopia).2 Cataracts are diagnosed by looking at the red reflex and this should be a routine part of the examination of a young child. Common conditions causing cataracts are genetic disorders and rubella but most causes are unknown. Rarer conditions, such as galactosaemia, need to be considered.

Refractive errors Refractive errors, with the error greater in one eye, can cause amblyopia. Detection of refractive errors is an important objective of screening.

Retinoblastoma Retinoblastoma, although rare, is the commonest intra-ocular tumour in childhood. It must be excluded in any child presenting with a white pupil. Such children also have the so-called ‘cat’s eye reflex’. In 30% of patients the condition is bilateral with an autosomal dominant gene being responsible.

Visual failure in the elderly Most patients with visual complaints are elderly; failing vision affects perception of the environment and the ability to communicate effectively. Typical causes are cataracts, vascular disease, macular degeneration, chronic simple glaucoma and retinal detachment. Retinal detachment and diabetic retinopathy can occur at any age, although they are more likely with increasing age. Macular degeneration in its various forms is the commonest cause of visual deterioration in the elderly. For the elderly with cataracts the decision to operate depends on the patient’s vision and their ability to cope. Most patients with a vision of 6/18 or worse in both eyes usually benefit from cataract extraction, but some can cope with this level of vision and rely on a good, well-placed (above and behind) reading light.6 Sudden loss of vision in the elderly is suggestive of temporal arteritis or vascular embolism, so this problem requires immediate attention.

Floaters and flashes (photopsia) When the vitreous gel shrinks as part of the normal ageing process, it tugs on the retina (rods and cones), causing flashing lights. When the gel separates from the retina, floaters (which may appear as dots, spots or cobwebs) are seen. Floaters are more commonly seen with age, but are also more common in people who are myopic or who have had eye surgery such as removal of cataracts. It is important to consider retinal detachment but if floaters remain constant there is little cause for concern. The appearance of a fresh onset of flashes or floaters is of concern, with the two important causes being retinal detachment and posterior vitreous detachment. Page 799

Haloes Haloes around lights may result from cataracts, acute angle-closure glaucoma, chronic glaucoma, dry eyes and corneal haziness including excessive tears and/or mucus or drugs, e.g. chloroquine, digoxin.

Refractive errors Indistinct or blurred vision is most commonly caused by errors of refraction—the commonest being myopia.

In the normal eye (emmetropia) light rays from infinity are brought to a focus on the retina by the cornea (contributing about two-thirds of the eye’s refractive power) and the lens (one-third). Thus, the cornea is very important in refraction; abnormalities such as keratoconus may cause severe refractive problems.6 The important clinical feature is that where there is a refractive error only, the use of a simple ‘pinhole’ in a card will usually improve blurred vision or reduced acuity.1

Pinhole test The pinhole reduces the size of the blur circle on the retina in the uncorrected eye. A pinhole acts as a universal correcting lens. If visual acuity is not normalised by looking through a card with a 1 mm pinhole, then the defective vision is not solely due to a refractive error. The pinhole test may actually help to improve visual acuity with some cataracts. Further investigation is mandatory.

Myopia (short/near-sightedness) Close objects appear clearly but far objects are blurred. The image is focused in front of the retina. This is usually progressive in the teens. Highly myopic eyes may develop retinal detachment, macular degeneration or glaucoma.

Management Glasses with a concave lens Contact lenses Consider radial keratotomy or excimer laser surgery

Hypermetropia (long/far-sightedness) The image is focused behind the retina. This condition is more susceptible to closed-angle glaucoma. In early childhood it may be associated with convergent strabismus (squint), and spectacle correction alone may straighten the eyes. It is mostly overcome by the accommodative power of the eye, though it may cause reading difficulty. Typically, the long-sighted person needs reading glasses at about 30 years. A positive converging (convex lens) is used for correction.

Presbyopia The process of accommodation is required for focusing closer objects. This process, which relies on the action of ciliary muscles and lens elasticity, is usually affected by ageing, so that from the age of 45 close work becomes gradually more difficult.6 There is a need for near correction with loss of accommodative power of the eye in the 40s.

Astigmatism This results from non-spherical (variable) curvature of the lens or cornea. This creates the need for a corrective lens that is more curved in one meridian than another because the cornea does not have even curvature. If uncorrected, this may cause headaches of ocular origin. Conical cornea is one cause of astigmatism.

Keratoconus Keratoconus is a bulging, slowly progressive thinning and distortion of the cornea, leading to loss of visual acuity—commonly irregular astigmatism. It usually appears between the ages of 10 and 25 and seems to be genetically determined. Frequent changes of glasses is a feature and contact lenses may help. If not, corneal transplant surgery may be necessary.

Cataract The term ‘cataract’ describes any lens opacity. The symptoms depend on the degree and the site of opacity. Cataract causes gradual visual loss with normal direct pupillary light reflex. The prevalence of cataracts increases with age: 65% at age 50–59, and all people aged over 80 have opacities.3 Significant causes of cataracts are presented in TABLE 66.2 and causes of progressive visual loss in TABLE 66.3 . Page 800

Table 66.2

Causes of cataracts

Advancing age Systemic disease, e.g. diabetes mellitus, myotonic dystrophy Smoking cigarettes Steroids (topical or oral) Radiation: long exposure to UV light or X-rays TORCH organisms → congenital cataracts Trauma Uveitis Significant alcohol consumption Malnutrition Dystrophia myotonica Galactosaemia → congenital cataract

Progressive bilateral visual loss

Table 66.3

Progressive bilateral visual loss

Globe

Chronic glaucoma Senile cataracts

Retina

Macular degeneration Retinal disease: diabetic retinopathy retinitis pigmentosa choroidoretinitis

Optic nerve

Optic neuropathies Optic nerve compression (e.g. aneurysm, glioma) Toxic damage to optic nerves

Optic chiasma

Chiasmal compression: pituitary adenoma, craniopharyngioma, etc.

Occipital cortex

Tumours Degenerative conditions

Note: Unilateral causes (e.g. cataract, refractive errors, uveitis, glaucoma, progressive optic atrophy and tumours) can affect the second eye.

Typical symptoms: reading difficulty difficulty in recognising faces problems with driving, especially at night difficulty with television viewing reduced ability to see in bright light may see haloes around lights The type of visual distortion seen by patients is illustrated in FIGURE 66.2

.

FIGURE 66.2 Blurred vision: appearance of a subject through the eyes of a person with cataracts Photo courtesy Allergan Pharmaceuticals

Examination Reduced visual acuity (sometimes improved with pinhole) Diminished red reflex on ophthalmoscopy A change in the appearance of the lens The red reflex and ophthalmoscopy The ‘red reflex’ is a reflection of the fundus when the eye is viewed from a distance of about 60 cm (2 feet) with the ophthalmoscope using a zero lens. This reflex is easier to see if the pupil is dilated. Commencing with the plus 15 or 20 lens, reduce the power gradually and, at plus 12, lens opacities will be seen against the red reflex, which may be totally obscured by a very dense cataract. The setting up of the ophthalmoscope to examine intra-ocular structures is illustrated in FIGURE 66.3 .

FIGURE 66.3 Settings of the ophthalmoscope used to examine intra-ocular structures

Management Advise extraction of the cataract when the patient cannot cope. Contraindications for extraction include intra-ocular inflammation and severe diabetic retinopathy. There is no effective medical treatment for established cataracts. The removal of the cataractous lens requires optical correction to restore vision and this is usually performed with an intra-ocular lens implant. Full visual recovery may take 2–3 months. Complications are uncommon yet many patients may require YAG laser capsulotomy to clear any opacities that may develop behind the lens implant. Postoperative advice to the patient Avoid bending over for a few weeks. Avoid strenuous exercise. The following drops may be prescribed: steroids (to reduce inflammation) antibiotics (to avoid infection) dilators (to prevent adhesions)

Prevention Sunglasses, particularly those that wrap around and filter UV light, may offer protection against cataract formation. Page 801

Glaucoma

Glaucoma, which is caused by raised intra-ocular pressure, is categorised as open-angle or closed-angle and further as primary or secondary and acute or chronic. Open-angle glaucoma, also known as chronic simple glaucoma, is the commonest cause of irreversible blindness in middle age.1 At a very late stage, it presents as difficulty in seeing because of loss of the outer fields of vision due to optic atrophy (see FIG. 66.4 ). Acute glaucoma, on the other hand, has a relatively rapid onset over a few days.

FIGURE 66.4 Typical visual field loss for chronic simple glaucoma; a similar pattern occurs with retinitis pigmentosa and functional visual loss (previously termed ‘hysteria’)

Clinical features (chronic glaucoma) Familial tendency No early signs or symptoms Central vision usually normal Insidious progressive restriction of visual field resulting in ‘tunnel vision’

Investigations Tonometry Upper limit of normal is 22 mmHg Ophthalmoscopy

Optic disc cupping >30% of total disc area

Screening Adults 40 years and over: 2–5 yearly (at least 2 yearly over 60) Start about 30 years, then 2 yearly if family history

Management Treatment can prevent visual field loss Medication (for life) usually selected from:7 timolol or betaxolol drops bd Note: These beta blockers can cause systemic complications, e.g. asthma latanoprost (or other prostaglandin analogue) drops, once daily pilocarpine drops qid dipivefrine drops bd brimonidine drops bd acetazolamide (oral diuretics) Surgery or laser therapy for failed medication

Retinitis pigmentosa Primary degeneration of the retina is a hereditary condition characterised by a degeneration of rods and cones associated with displacement of melanin-containing cells from the pigment epithelium into the more superficial parts of the retina.

Typical features Begins as night blindness in childhood Visual fields become concentrically narrowed (periphery to centre), i.e. tunnel vision Blind by adolescence (sometimes up to middle age) Irreversible course—may be delayed by vitamin A8

Examination (ophthalmoscopic)

Irregular patches of dark pigment, especially at periphery Optic atrophy → disc pallor

Intra-ocular foreign body A small metal chip may penetrate the eye with minimal pain and the patient may not present with an ocular problem until the history of injury is long forgotten. If infection does not supervene, presentation may be delayed for months or years until vision deteriorates due to metal degradation. The iris becomes rust-brown. It is important to X-ray the eye if it has been struck by a hammered fragment or if in any doubt at all about the mechanism of the injury.1

Chronic uveitis Pain and redness may be minimal with this chronic inflammation, which often accompanies chronic systemic conditions (e.g. sarcoidosis). If untreated, visual loss often develops from secondary glaucoma and cataract. The pupil is bound to the lens by synechiae and is distorted. Treatment may involve long-term topical corticosteroids.

HIV infection AIDS is associated with serious ocular complications, including Kaposi sarcoma of the Page 802 conjunctivae, retinal haemorrhage and vasculitis.3 Another problem is ocular cytomegalovirus infection, which presents as areas of opacification with haemorrhage and exudates.

Sudden loss of vision This problem is alarming and distressing to the patient; considerable empathy is needed. Initial presentation may confuse with seemingly inappropriate behaviour; be very careful before diagnosing as psychogenic in origin. A comparison of bilateral and unilateral causes of sudden loss of vision is presented in TABLE 66.4 , and the diagnostic strategy model in TABLE 66.5 . A simplified classification is: unilateral:

retinal detachment retinal artery occlusion retinal vein thrombosis temporal arteritis

optic neuritis migraine

bilateral:

bilateral optic nerve lesion functional (‘hysteria’, conversion reaction)

Table 66.4

Causes of sudden loss of vision7 Unilateral Permanent

Bilateral

Transient

Vascular causes

Occipital cortex ischaemia Pituitary apoplexy Homonymous hemianopia—vascular

Amaurosis fugax Transient ocular ischaemia Retinal emboli Malignant hypertension

Central retinal artery occlusion Central retinal vein occlusion Vitreous haemorrhage Ischaemic optic neuropathy

Other causes

Bilateral optic neuritis Toxic damage to optic nerve: methanol ethanol tobacco lead Leber optic atrophy Quinine poisoning of retina Cerebral oedema Occipital lobe trauma Craniopharyngioma Functional (‘hysterical’)

Acute angle closure glaucoma Uhthoff phenomenon Papilloedema Posterior vitreous detachment

Optic neuritis Retinal detachment Optic nerve compression Carcinomatous optic neuropathy Intra-ocular tumour

Table 66.5

Acute or subacute painless loss of vision:

Table 66.5

Acute or subacute painless loss of vision: diagnostic strategy model

Probability diagnosis Amaurosis fugax Migraine Retinal detachment ‘Wet’ macular degeneration Serious disorders not to be missed Cardiovascular: central retinal artery occlusion central retinal vein occlusion hypertension (complications) CVA Neoplasia: intracranial tumour intra-ocular tumour: primary melanoma retinoblastoma metastases Vitreous haemorrhage AIDS Temporal arteritis Acute glaucoma Benign intracranial hypertension Pitfalls (often missed) Papilloedema Optic neuritis Intra-ocular foreign body Posterior uveitis Seven masquerades checklist Diabetes (diabetic retinopathy) Drugs (quinine) Thyroid disorder (hyperthyroidism) Is this patient trying to tell me something? Consider ‘hysterical’ blindness, although it is uncommon.

A flow chart for the diagnosis of painless loss of vision is presented in FIGURE 66.5

.

FIGURE 66.5 Diagnosis of painless loss of vision Source: Reproduced with permission of Dr J Reich and Dr J Colvin

Amaurosis fugax Amaurosis fugax is transient loss of vision (partial or complete) in one eye due to transient

occlusion of a retinal artery. It is painless and lasts less than 60 minutes. It is usually caused by an embolus from an atheromatous carotid artery in the neck. The most common emboli are cholesterol emboli, which usually arise from an ulcerated plaque.7 Other causes include emboli from the heart, temporal arteritis and benign intracranial hypertension. Other symptoms or signs of cerebral ischaemia, such as transient hemiparesis, may accompany the symptom. The source of the problem should be investigated. The risk of stroke after an episode of amaurosis fugax appears to be about 2% per year.7

Transient ocular ischaemia Unilateral loss of vision provoked by activities such as walking, bending or looking upwards is suggestive of ocular ischaemia.7 It occurs in the presence of severe extracranial vascular disease and may be triggered by postural hypotension and ‘stealing’ blood from the retinal circulation.

Retinal detachment9 Retinal detachment may be caused by ocular trauma, thin retina (myopic people), previous surgery (e.g. cataract operation), choroidal tumours, vitreous degeneration or diabetic retinopathy. Page 803

Clinical features Sudden onset of floaters, flashes or black spots Blurred vision in one eye becoming worse A dark shadow peripherally, progressing centrally over days/weeks Partial or total loss of visual field (total if macula detached) Ophthalmoscopy may show detached retinal fold as large grey shadow in vitreous cavity.

Management Immediate referral for sealing of retinal tears Small holes treated with laser or freezing probe Pneumatic retinopexy is an option True detachments usually require surgery

Vitreous haemorrhage Haemorrhage may occur from spontaneous rupture of vessels, avulsion of vessels during retinal

traction or bleeding from abnormal new vessels.6 Associations include ocular trauma, diabetic retinopathy, tumour and retinal detachment.

Clinical features Sudden onset of floaters or ‘blobs’ in vision May be sudden loss of vision Visual acuity depends on the extent of the haemorrhage; if small, visual acuity may be normal Ophthalmoscopy may show reduced light reflex: there may be clots of blood that move with the vitreous (a black swirling cloud).

Management Urgent referral to exclude retinal detachment Exclude underlying causes such as diabetes Ultrasound helps diagnosis May resolve spontaneously Bed rest encourages resolution Surgical vitrectomy for persistent haemorrhage

Central retinal artery occlusion The cause is usually arterial obstruction by atherosclerosis, thrombi or emboli. There may be a history of TIAs. Exclude temporal arteritis (immediately measure ESR).

Clinical features Sudden loss of vision like a ‘curtain descending’ in one eye (same as amaurosis fugax, but doesn’t resolve) Vision not improved with 1 mm pinhole Usually no light perception

Ophthalmoscopy Initially normal May see retinal emboli or pale swelling

Classic ‘red cherry spot’ at macula

Management Urgent referral to an ophthalmologist but if seen early, or opthalmologist delayed, use this procedure within 30 minutes: massage globe digitally through closed eyelids (use rhythmic direct digital pressure for at least 5 minutes)—may dislodge embolus distally rebreathe carbon dioxide (paper bag) or inhale special CO2 mixture (carbogen)

Page 804

intravenous acetazolamide (Diamox) 500 mg refer urgently (less than 6 hours)—exclude temporal arteritis Prognosis is poor. Significant recovery is unlikely unless treated immediately (within 30 minutes).

Central retinal vein thrombosis Thrombosis is associated with several possible factors, such as hypertension, diabetes, thrombocytopenia, glaucoma and hyperlipidaemia. It usually occurs in the elderly.

Clinical features Sudden loss of central vision in one eye (if macula involved): can be gradual over days Vision not improved with 1 mm pinhole Ophthalmoscopy shows swollen disc and multiple retinal haemorrhages, ‘stormy sunset’ appearance.

Management Refer to an ophthalmologist. No immediate treatment is effective. The cause needs to be found first and treated accordingly. Some cases respond to fibrinolysin treatment. Laser photocoagulation may be necessary in later stages if neovascularisation develops, to prevent thrombotic glaucoma. Intravitreal injection of a monoclonal antibody is also an option.

Macular degeneration There are two age-related types: exudative or ‘wet’ (acute), and pigmentary or ‘dry’ (slow onset).

Page 805

‘Wet’ MD is caused by choroidal neovascular membranes that develop under the retina of the macular area and leak fluid or bleed. It is a serious disorder.

‘Dry’ MD (9 out of 10 cases of MD) develops more slowly and is always painless. More common with increasing age (usually over 60), when it is termed ‘age-related MD’, and in those with myopia (relatively common). May be familial.

Clinical features Sudden fading of central vision (see FIG. 66.6

)

Distortion of vision Straight lines may seem wavy and objects distorted Use a grid pattern (Amsler chart): shows distorted lines Central vision eventually completely lost Peripheral fields normal

FIGURE 66.6 Appearance of a subject through the eyes of a person with agerelated macular degeneration Photo courtesy Allergan Pharmaceuticals

Ophthalmoscopy White exudates, haemorrhage in retina Macula may look normal or raised

Management

No treatment is available to reverse MD. However ‘wet’ MD should be referred urgently for treatment to slow its progression: regular intravitreal injection of antivascular endothelial growth factor drugs (e.g. ranibizumab, bevacizumab) into the vitreous humour.10 The Age-Related Eye Disease Study provided confirmatory evidence that the chronic pigmentation type responds to free-radical treatment with the antioxidants vitamins A, C, E and zinc using beta-carotene, 15 mg; vitamin C, 500 mg; vitamin E, 400 IU; and 80 mg zinc oxide.11 Advise patient to cease smoking if applicable.12 Low vision aids my be beneficial.

Drusen Drusen are small yellow deposits under the retina composed of lipids, a fatty protein. They are part of ageing and harmless per se, but having drusen increases a person’s risk of developing dry MD.

Temporal arteritis With temporal arteritis (giant cell arteritis) there is a risk of sudden and often bilateral occlusion of the short ciliary arteries supplying the optic nerves, with or without central retinal artery involvement.13

Clinical features14 Usually older person: over 65 years Sudden loss of central vision in one eye (central scotoma) Can rapidly become bilateral Associated temporal headache or jaw claudication (around 60%) Temporal arteries tender, thickened and non-pulsatile (around 30%) Visual acuity—blurred, diplopia; severely impaired (around 20%) Afferent pupil defect on affected side Usually elevated ESR >40 mm Ophthalmoscopy shows optic disc swollen at first, then atrophic. The disc may appear quite normal.

Management Other eye must be tested Immediate corticosteroids (60–100 mg prednisolone daily for at least 1 week)

Biopsy temporal artery (if there is a localised tender area)

Retinal migraine Migraine may present with symptoms of visual loss (‘aura’). Associated headache and nausea may be absent. Clinical features Zigzag lines or lights Multicoloured flashing lights Unilateral or bilateral field deficit Resolution within a few hours

Posterior vitreous detachment The vitreous body collapses and detaches from the retina. It may lead to retinal detachment.

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Clinical features Sudden onset of floaters Visual acuity usually normal Flashing lights indicate traction on the retina Management Refer to an ophthalmologist urgently. An associated retinal hole or detachment needs exclusion.

Optic (retrobulbar) neuritis Causes include multiple sclerosis, neurosyphilis and toxins. A significant number of cases eventually develop multiple sclerosis.

Clinical features Usually a woman 20–40 years (multiple sclerosis) Loss of vision in one eye over a few days

Retro-ocular discomfort with eye movements Variable visual acuity Usually a central field loss (central scotoma) Afferent pupil defect on affected side

Ophthalmoscopy Optic disc swollen if ‘inflammation’ anterior in nerve Optic atrophy appears later Disc pallor is an invariable sequel

Management Test visual field of other eye MRI Most patients recover spontaneously but are left with diminished acuity Intravenous steroids hasten recovery and have a protective effect against the development of further demyelinating episodes

Corneal disorders People with corneal conditions typically suffer from ocular pain or discomfort and reduced vision. The common condition of dry eye may involve the cornea while contact lens disorders, abrasions/ulcers and infections are common serious problems that threaten eye sight. Inflammation of the cornea—keratitis—is caused by factors such as ultraviolet light, e.g. ‘arc eye’, herpes simplex, herpes zoster ophthalmicus and the dangerous ‘microbial keratitis’. Bacterial keratitis is an ophthalmological emergency that should be considered in the contact lens wearer presenting with pain and reduced vision. Topical corticosteroids should be avoided in the undiagnosed red eye. Refer to CHAPTER 40

for corneal lesions.

Pitfalls Mistaking the coloured haloes of glaucoma for migraine. Failing to appreciate the presence of retinal detachment in the presence of minimal visual impairment.

Omitting to consider temporal arteritis as a cause of sudden visual failure in the elderly. Using eyedrops to dilate the pupil (for fundal examination) in the presence of glaucoma.

When to refer Most problems outlined need urgent referral to an ophthalmologist, especially retinal detachment. Acute visual disturbance of unknown cause requires urgent referral. Any blurred vision—sudden or gradual, painful or painless—especially if 1 mm pinhole fails to alter visual acuity. Refer all suspicious optic discs. Cataracts when visual impairment seriously affects everyday activities.

Practice tips Tonometry is advised routinely for all people over 40 years; those over 60 years should have tests every 2 years. Any family history of glaucoma requires tonometry from 40 years onwards. Sudden loss of vision in the elderly suggests temporal arteritis (check the ESR and temporal arteries). It requires immediate institution of high-dose steroids to prevent blindness in the other eye. A time-scale guide showing the rate of visual loss is presented in TABLE 66.6 . Temporal arteritis is an important cause of retinal artery occlusion. Suspect field defect due to chiasmal compression if people are misjudging when driving. Pupillary reactions are normal in cortical blindness. Central retinal artery occlusion may be overcome by early rapid lowering of intraocular pressure. Retinal detachment and vitreous haemorrhage may require early surgical repair. Keep in mind antioxidant therapy (vitamins and minerals) for chronic macular degeneration. Consider multiple sclerosis foremost if there is a past history of transient visual

failure, especially with eye pain. If the person develops an eye symptom after using a hammer, always X-ray if no metal fragment can be seen on examination.

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Table 66.6

Time-scale guide for rate of visual loss3,7

Sudden: less than 1 hour Amaurosis fugax Central retinal artery occlusion Hemianopias from ischaemia (emboli) Migraine Vitreous haemorrhage Acute angle glaucoma Papilloedema Within 24 hours Central retinal vein occlusion Functional (hysteria) Less than 7 days Retinal detachment Optic neuritis Acute macular problems Up to several weeks (variable) Choroiditis Malignant hypertension Gradual Compression of visual pathways Chronic glaucoma Cataracts Diabetic maculopathy Retinitis pigmentosa Macular degeneration Refractive errors

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Cataracts Colour blindness Floaters and flashes Glaucoma Macular degeneration

References 1

Colvin J, Reich J. Check Program 219–220. Melbourne: RACGP, 1990: 1–32.

2

Hopcroft K, Forte V. Symptom Sorter (4th edn). Oxford: Ratcliffe Publishing, 2010: 138.

3

Thomson J et al. Cataracts. Prim Care, 2015 Sept; 42(3): 409–23.

4

Robinson MJ, Roberton, DM. Practical Paediatrics (5th edn). Melbourne: Churchill Livingstone, 2003: 756–70.

5

Cole GA. Amblyopia and strabismus. In: MIMS Disease Index (2nd edn). Sydney: IMS Publishing, 1996: 20–4.

6

Elkington AR, Khaw PT. ABC of Eyes. London: British Medical Association, 1990: 20– 38.

7

Li T et al. Comparative effectiveness of first-line medications for primary open-angle glaucoma: a systematic review and network meta-analysis. Ophthalmology, 2016; 123(1): 129–40.

8

Berson EL et al. Association of vitamin A supplementation with disease course in children with retinitis pigmentosa. JAMA Ophthalmol, 1 May 2018; 136(5): 490–5.

9

Jalali S. Retinal detachment. Community Eye Health, 2003; 16(46): 25–6.

10

Hodge C, Ng D. Eye emergencies. Check Program 400. Melbourne: RACGP, 2005: 1–34.

11

Bunting R, Guymer R. Treatment of age-related macular degeneration. Aust Prescr, 2012; 35: 90–3.

12

Age-Related Eye Disease Study Research Group. A randomized, placebo-controlled,

clinical trial of high-dose supplementation with vitamins C and E, beta-carotene, and zinc for age-related macular degeneration and vision loss. Arch Ophthalmol, 2001; 119: 1417– 36. 13

Singh A et al. Visual manifestations in giant cell arteritis: trend over 5 decades in a population-based cohort. J Rheumatol, 2015; 42(2): 309–15.

14

Gonzalez-Gay MA et al. Giant cell arteritis and polymyalgia rheumatica: an update. Curr Rheumatol Rep, 2015 Sept; 17(2): 6.

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67 Weight change

Everything in excess is opposed to nature. HIPPOCRATES, 460–370 BC In Australia, as in most other developed nations, the population health issue of having too much body weight has far outstripped the health issue of being underweight. The vexing problem of obesity is covered in the chronic disease section (see CHAPTER 80 ). However, the relatively short-term gain or loss of weight presents a significant diagnostic challenge in general practice, particularly as so many underlying conditions can result in weight change.

Weight gain Key facts and figures Two-thirds of the Australian population are overweight or obese and only 2–4% underweight.1 Less than 1% of obese people have an identifiable secondary cause of obesity.2 Two conditions causing unexplained weight gain that can be diagnosed by the physical examination are Cushing syndrome and hypothyroidism. After pregnancy, obesity may result from a failure to return to prepartum energy requirements. Even small weight losses are effective in preventing diabetes and improving the cardiovascular risk profile.3

A diagnostic approach A summary of the diagnostic strategy model is presented in TABLE 67.1

.

Table 67.1

Weight gain: diagnostic strategy model

Probability diagnosis Exogenous obesity Alcohol excess Drugs Serious disorders not to be missed Cardiovascular: cardiac failure Hypothalamic disorders (hyperphagia): craniopharyngiomas optic gliomas Liver failure Nephrotic syndrome Pitfalls (often missed) Pregnancy (early) Endocrine disorders: hypothyroidism Cushing syndrome insulinoma acromegaly hypogonadism hyperprolactinaemia polycystic ovarian syndrome Idiopathic oedema syndrome Klinefelter syndrome Congenital disorders: Prader–Willi syndrome Laurence–Moon–Bardet–Biedl syndrome Seven masquerades checklist Depression Drugs Thyroid disorder (hypothyroidism) Is the patient trying to tell me something? Yes: the reasons for obesity should be explored.

Probability diagnosis The outstanding cause of weight gain in exogenous obesity is excessive calorie intake coupled with lack of exercise, and influenced heavily by socio-environmental factors (see CHAPTER 80 ).

Serious causes not to be missed It is important not to misdiagnose hypothalamic disorders, which may result in hyperphagia and obesity. Injury to the hypothalamus may occur following trauma and encephalitis and with a variety of tumours, including craniopharyngiomas, optic gliomas and pituitary neoplasms. Some of these tumours may cause headaches and visual disturbances. It is also important not to overlook major organ failure and kidney disorders as a cause Page 809 of increased body weight, especially cardiac failure, liver failure and the nephrotic syndrome. The associated increase in body water needs to be distinguished from increased body fat. Consider the obesity hypoventilation syndrome (Pickwickian syndrome) in those with BMI >40 kg/m2.

Pitfalls Endocrine disorders The endocrine disorders that cause obesity include Cushing syndrome, hypothyroidism, insulinsecreting tumours and hypogonadism. They should not represent difficult diagnostic problems. An insulin-secreting tumour (insulinoma) is a very rare adenoma of the B cells of the islets of Langerhans. The main features are symptoms of hypoglycaemia and obesity.

Congenital disorders The rare congenital disorders that cause obesity, such as Prader–Willi and Laurence–Moon– Bardet–Biedl syndromes, should be easy to recognise in children (see CHAPTER 23 and later in this chapter).

Chromosomal abnormalities An important abnormality to bear in mind is Klinefelter syndrome (XXY karyotype), which affects one out of every 400–500 males. The boys show excessive growth of long bones and are tall and slim. Without testosterone treatment they become obese as adults. Some girls with Turner syndrome (XO karyotype) may be short and overweight.

Some gender pointers

Consider polycystic ovarian syndrome in women and obstructive sleep apnoea in obese men.

Seven masquerades checklist The important masquerades include hypothyroidism and drug ingestion. Hypothyroidism is usually not associated with marked obesity. Drugs that can be an important contributing factor include tricyclic (and other) antidepressants, corticosteroids, pizotifen, the antipsychotics, depot progesterone and insulin. Obesity (overeating) may be a feature of depression, especially in the early stages.

Psychogenic considerations An underlying emotional crisis may be the reason for the overweight person to seek medical advice. It is important to explore diplomatically any hidden agenda and help them to resolve any conflict.

The clinical approach A careful history is very valuable in ascertaining food and beverage intake and perhaps giving people insight into their calorie intake, since some deny overeating or will underestimate their food intake.3 Enquire about gynaecological and family history, e.g. diabetes, cardiac disease.

Relevant questions Do you feel that you have an excessive appetite? Tell me in detail what you ate yesterday. Give me an outline of a typical daily meal. Tell me about snacks, soft drink and alcohol that you have. What exercise do you get? Do you have any special problems, such as getting bored, tense and upset or depressed? What drugs are you taking?

Examination In the physical examination it is very important to measure body weight and height (and calculate BMI), waist circumference and assess the degree and distribution of body fat and the overall nutritional status. For a discussion of anthropometric measurements and their interpretation, see CHAPTER 80 . Record blood pressure and test the urine with dipsticks. Keep in mind that a standard blood pressure cuff on a large arm may give falsely elevated values.

Remember the rare possibilities of Cushing syndrome, acromegaly and hypothyroidism. Search for evidence of atherosclerosis and diabetes, and for signs of alcohol abuse. An extensive working up of the CNS is not indicated in obesity without the presence of suspicious symptoms such as visual difficulties.

Important investigations Lipid profile Glucose (fasting) and/or HbA1c if significant weight gain EUC, LFTs

Investigations to consider Thyroid function tests Cortisol (if hypertensive) Testosterone (suspected sleep apnoea) ECG and chest X-ray (older than 40)

Weight gain in children Various studies have found that approximately 10% of prepubertal and 15% of adolescent age groups are obese.4 Obesity in children is a BMI for age >95th percentile while overweight is >85th percentile. There is a risk of obesity-associated diseases and carrying the problem into adulthood, with a greater risk of obesity, premature death and disability.

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Raising the issue with parents and child requires sensitivity and discretion. Parents often blame obesity in children on their ‘glands’, but endocrine or metabolic causes are rare and can be readily differentiated from exogenous obesity by a simple physical examination and an assessment of linear growth. Children with exogenous obesity tend to have an accelerated linear growth whereas children with secondary causes are usually short.

Congenital or inherited disorders Prader–Willi syndrome The characteristic features are bizarre eating habits (e.g. binge eating), obesity, hypotonia, hypogonadism, intellectual disability, small hands and feet and a characteristic facial appearance (narrow bifrontal diameter, ‘almond-shaped’ eyes and a ‘tented’ upper lip). Progressive obesity

results from excessive intake in addition to decreased caloric requirements (see CHAPTER 23

).

Laurence–Moon–Bardet–Biedl syndrome The characteristic features are obesity, intellectual disability, polydactyly and syndactyly, retinitis pigmentosa and hypogonadism.

Beckwith–Wiedemann syndrome Characteristics include excessive growth, macrosomia, macroglossia, umbilical hernia and neonatal hypoglycaemia. Children appear obese as they are above the 95th percentile by 18 months of age. Intelligence is usually in the normal range.

Endocrine disorders Endocrine disorders in children that can rarely cause obesity include hypothyroidism (often blamed as the cause but seldom is), Cushing syndrome, insulinomas, hypothalamic lesions, Fröhlich syndrome (adiposogenital dystrophy) and Stein–Leventhal syndrome (PCOS) in girls.

Managing obesity in children Childhood obesity usually reflects an underlying problem in the family system. It can be a very difficult emotional problem in adolescents, who develop a poor body image. An important strategy is to meet with family members, determine whether they perceive the child’s obesity as a problem and whether they are prepared to solve the problem. The family dynamics will have to be assessed and strategies outlined. This may involve referral for expert counselling. It is worth pointing out that children eat between one-third and two-thirds of their meals at school, so schools should be approached to promote special programs for children who need weight reduction. Conventional therapy by dietary modification, increasing energy expenditure by increasing activity, reducing sedentary behaviour, behaviour modification and family involvement is recommended (see CHAPTER 80 ). The best outcomes are achieved with a specialist team working with the whole family.5 Some authorities emphasise that weight maintenance rather than weight loss is appropriate since many children will ‘grow into their weight’.6

Weight gain in adults Cushing syndrome Cushing syndrome is the term used to describe the chemical features of increased free circulating glucocorticoid. The most common cause is iatrogenic with the prescribing of synthetic corticosteroids. The spontaneous primary forms such as Cushing disease (pituitary-dependent hyperadrenalism) are rare. As the disorder progresses the body contour tends to assume the often

quoted configuration of a lemon with matchsticks (see CHAPTER 14

).

Clinical features Change in appearance Central weight gain (truncal obesity) Hair growth and acne in females Muscle weakness Amenorrhoea/oligomenorrhoea (females) Thin skin/spontaneous bruising Polymyalgia/polydipsia (diabetes mellitus) Insomnia Depression

Signs Moon face ‘Buffalo hump’ Purple striae Large trunk and thin limbs: the ‘lemon with matchsticks’ sign Refer for diagnostic evaluation, including plasma cortisol and overnight dexamethasone suppression tests. Untreated Cushing syndrome has a very poor prognosis, with premature death from myocardial infarction, cardiac failure and infection; hence, early diagnosis and referral is essential.

Oedema Oedema (dropsy) is an excessive accumulation of fluid in tissue spaces. It may be generalised or localised—peri-orbital, peripheral or an arm (lymphoedema, refer to CHAPTER 58 ).

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Generalised oedema The site of generalised oedema is largely determined by gravity. It is due to an abnormal excess

of sodium in the body, which leads to accumulation of water. The causes can be generally divided into two groups—oedema associated with a decreased plasma volume and oedema associated with an increased plasma volume (see TABLE 67.2 ). Table 67.2

Causes of generalised oedema

Decreased plasma volume Hypoalbuminaemia (e.g. nephrotic syndrome, chronic liver disease, malnutrition) Increased plasma volume Congestive cardiac failure Chronic kidney failure Drugs (e.g. corticosteroids, NSAIDs, certain antihypertensives, oestrogens, lithium, others) Idiopathic oedema

Diagnosis Clinical examination, including urinalysis, is usually sufficient to establish the cause of the oedema. In other cases, investigation of kidney or liver function may be required.

Treatment Treat the cause where known Salt (sodium) restriction Diuretics: a loop diuretic (e.g. frusemide) a potassium-sparing diuretic (e.g. spironolactone)

Idiopathic oedema Idiopathic oedema, also known as cyclical or periodic oedema, is a common problem and the diagnosis is made on a characteristic history: exclusive to women may be cyclical or persistent usually unrelated to menstrual cycle

excessive diurnal weight gain (worse on prolonged standing) abdominal bloating may affect hands and face as well as feet often made worse by diuretics may be associated with headache, depression, tension Treatment of this condition is difficult. Most diuretics can aggravate the problem. Supportive stockings and a nutritious diet (with restricted sodium intake) are recommended as first-line treatment. A trial of spironolactone is often recommended.

Swelling (puffiness) of the face and eyelids The causes are similar to those for generalised oedema. Important specific causes to consider are: kidney disease (e.g. nephrotic syndrome, acute nephritis) hypothyroidism Cushing syndrome and corticosteroid treatment mediastinal obstruction/superior vena cava syndrome angio-oedema skin sensitivity (e.g. drugs, cosmetics, hair dryers) carotico-cavernous fistula

Swelling of the legs Refer to CHAPTER 58

.

‘Cellulite’ ‘Cellulite’ refers to a characteristic form of dimpling seen in the subcutaneous tissues of hips, buttocks and thighs of females. The dimpling pattern is related to the manner of attachment of fibrous septae that contain the fat. Many patients seek advice about ‘cellulite’ in the buttocks and thighs in particular. Explain that the best way to overcome it is to maintain an ideal weight. If overweight, lose it slowly and exercise to improve the muscle tone in the buttocks and thighs.

Weight loss

In family practice complaints of loss of weight are more frequent than complaints about being too thin. Of great significance is the problem of recent loss of weight. A very analytical history is required to determine the patient’s perception of weight loss. The equivalent problem in children is failure to gain weight or thrive. Weight loss is an important symptom because it usually implies a serious underlying disorder, either organic or functional. It may or may not be associated with anorexia and thus diminished food intake. Page 812

Key facts and checkpoints Any loss of more than 5% of normal body weight is significant. The most common cause in adults of recent weight loss is stress and anxiety.7 Serious organic diseases to consider are: malignant disease diabetes chronic infections (e.g. tuberculosis) thyrotoxicosis The most important variable to consider in evaluating weight loss is appetite. Eating and weight go hand in glove. Two conditions commonly associated with weight loss are anaemia and fever; they must be excluded. Early detection of eating disorders improves outcome.

A diagnostic approach A summary of the diagnostic strategy model is presented in TABLE 67.3 Table 67.3

Weight loss: diagnostic strategy model (other than deliberate dieting or malnutrition)

Probability diagnosis

.

Stress and anxiety (e.g. redundancy, relationship breakdown) Depressive illness Non-coping elderly/dementia Eating disorders: anorexia nervosa/bulimia Chronic congestive heart failure Malignant disease, including: stomach pancreas lung myeloma caecum lymphoma Chronic infection: HIV infections (AIDS, AIRC) tuberculosis hidden abscess infective endocarditis brucellosis others, e.g. overseas acquired infection Pitfalls (often missed) Drug dependence, esp. alcohol Malabsorption states: intestinal parasites/infestations coeliac disease Other GIT problems Chronic kidney failure Connective tissue disorders (e.g. SLE, RA) Dementia Rarities: malnutrition Addison disease hypopituitarism Seven masquerades checklist Depression Diabetes Drugs Anaemia

Thyroid disorder (hyperthyroidism) UTI Is the patient trying to tell me something? A possibility. Consider stress, anxiety and depression. Anorexia nervosa and bulimia are special considerations.

Probability diagnosis Excluding planned dietary restriction, psychological factors are the most common cause, particularly recent stress and anxiety.7 Elderly people with adverse psychological factors, neglect and possibly drug effects can present with wasting.

Serious disorders not to be missed Many of the problems causing weight loss are very serious, especially malignant disease.

Malignant disease Weight loss may be a manifestation of any malignancy. With cancer of the stomach, pancreas and caecum, malignant lymphomas and myeloma, weight loss may be the only symptom. Occult malignancy must be regarded as the most common cause of unexplained weight loss in the absence of major symptoms and signs. The mechanisms may be multiple, with anorexia and increased metabolism being important factors.

Chronic infections These are now less common but tuberculosis must be considered, especially in people from less developed countries. Some cases of infective endocarditis may progress only very slowly with general debility, weight loss and fever as major features.8 Other infections to consider are brucellosis, and protozoal and systemic fungal infection. Infection with HIV virus must be considered, especially in high-risk groups.

Pitfalls Drug dependency, including alcohol and narcotic drugs, must be considered, especially Page 813 when the problem may result in inappropriate nutrition. Apart from malignant disease there is a whole variety of gastrointestinal disorders that require consideration—these include malabsorption states, gastric ulceration, and intestinal infestations, especially in people returning from a significant stay in tropical and under-developed countries. Addison disease (see CHAPTER 14

) can be very difficult to diagnose. Symptoms include

excessive fatigue, anorexia, nausea and postural dizziness. Hyperpigmentation is a late sign.

Seven masquerades checklist Depression and the endocrine disorders---diabetes and hyperthyroidism---are important causes.

Diabetes Unintended weight loss is a particular issue with type 1 diabetes; always be aware of the dangerous ketoacidosis as a first presentation. The triad is thirst + polyuria + weight loss.

Hyperthyroidism This is usually associated with weight loss although in some, such as an elderly male, it may not be obvious. An important clue will be weight loss in the presence of an excellent appetite, which helps distinguish it from a psychoneurotic disturbance.

Depression Weight loss is a common feature of depression and is usually proportional to the severity of the disease. In the early stages of depression, weight gain may be present but when the classic loss of the four basic drives (appetite, energy, sleep and sex) becomes manifest, weight loss is a feature.

Drugs Any prescribed drugs causing anorexia can cause weight loss. Important drugs include digoxin, narcotics, cytotoxics, NSAIDs, some antihypertensives and theophylline. Be mindful of inappropriate use of thyroxine and laxatives.

Red flag pointers for weight loss Weight loss per se is a big red flag. Rapid weight loss with malaise Acid dental erosion on surfaces of upper teeth: think bulimia Weakness and malaise in young females: consider eating disorder and hypokalaemia Evidence of abuse in a child

Psychogenic considerations

Weight loss is a feature of anxiety as well as depression. Some patients with psychotic disturbances, including schizophrenia and mania, may present with weight loss. Anorexia nervosa is quite common, particularly in females between the ages of 12 and 20 years. The main differential diagnosis is hypopituitarism, although anorexia nervosa can cause endocrine disturbances through the hypothalamic pituitary axis.

The clinical approach History It is important to document the weight loss carefully and evaluate the patient’s recordings. The same set of scales should be used. It is also important to determine the food intake. However, in the absence of an independent witness such as a spouse or parent, this can be difficult. Food intake may be diminished with psychogenic disorders and cancer but increased or steady with endocrine disorders, such as diabetes and hyperthyroidism, and with steatorrhoea. FIGURE 67.1 shows the possible causes of weight loss.

FIGURE 67.1 Weight loss: causes to consider

General questions Exactly how much weight have you lost and over how long? Have you changed your diet in any way? Has your appetite changed? Do you feel like eating? Have your clothes become looser? What is your general health like? Do you feel uptight (tense), worried or anxious? Do you get very irritable or tremulous? Do you feel depressed? Do you ever force yourself to vomit? Are you thirsty? Do you pass a lot of urine? Do you have excessive sweating? Do you experience a lot of night sweats? What are your motions like? Are they difficult to flush down the toilet? Do you have a cough or bring up sputum? Do you get short of breath? Do you have any abdominal pain? Are your periods normal (for females)? What drugs are you taking? How many cigarettes do you smoke?

Examination A careful general examination is essential with special attention to: vital parameters, e.g. BMI, temperature, BP

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the thyroid and signs of hyperthyroidism the abdomen (check liver, any masses and tenderness) rectal examination (test stool for occult blood) reflexes look for acid dental erosion on surface of upper teeth (bulimia)

Investigations Basic investigations include: haemoglobin, red cell indices and film white cell count ESR/CRP thyroid function test random blood sugar EUC, LFTs chest X-ray urine analysis faecal occult blood Others to consider: upper GIT (endoscopy or barium meal) HIV serology ultrasound of abdomen (or CT if suspected abnormality not found) colonoscopy tumour markers, e.g. CA-125, CEA

Weight loss in children Weight loss in children can be considered as:

1. failure to thrive (FTT): the child up to 2 years below 3rd percentile (refer to CHAPTER 84

)

2. weight loss in a child after normal development

Loss of weight in the older child Page 815 Acute or chronic infections are the most common causes of weight loss in children beyond infancy.9 In acute infections the weight loss is transient, and once the infection clears the child generally regains the lost weight. In chronic infections signs may be more difficult to detect (e.g. urinary tract infection, pulmonary infection, osteomyelitis, chronic hepatitis). In common with the younger child who fails to thrive, the older child may be suffering from malabsorption syndrome, chronic infection of the urinary tract or a rare chromosomal or metabolic disorder.10 Tuberculosis, diabetes and malignant disease may present as weight loss and it is necessary to exclude organic disease before considering the more common emotional disorders.

Eating disorders in the adolescent Concerns about body image and dieting are very common among young women in modern society, and to an increasing extent in young men. Among these dieters 5–10% become abnormally preoccupied with dieting and slimness and progress to the eating disorders of anorexia nervosa and bulimia. They often have extremely low self-esteem and feel ineffective. They tend to be perfectionists with obsessive–compulsive traits. A history of childhood sexual abuse may be relevant. Media images alone do not cause eating disorders but have a role— genetic vulnerability, temperament, psychological and environmental factors also mediate these illnesses: ‘Genes load the gun—the environment pulls the trigger’. The DSM-5 criteria for diagnosing these disorders, which have serious physical and psychological consequences, are presented in TABLE 67.4 . The differential diagnosis of anorexia nervosa includes most of the problems listed in TABLE 67.4. Table 67.4

DSM-5 criteria for diagnosing anorexia nervosa and bulimia

Anorexia nervosa A

Restriction of energy intake relative to requirements leading to significantly low body weight in context of age, sex and physical health. That weight is less than minimally normal or expected

B

Intense fear of gaining weight or becoming fat, despite current underweight status

C

Disturbance of body image (body size or shape) or persistent lack of recognition of seriousness of low body weight

Types

Restricting type—no binge eating or purging binge eating/purging type

Bulimia nervosa A

Recurrent episodes of binge eating, that is: 1. eating in a discrete period of time an abnormal quantity of food compared with the average person 2. a sense of lack of control during the binge

B

Recurrent inappropriate compensatory behaviour to prevent weight gain (e.g. self-induced vomiting; misuse of laxatives, diuretics, enemas etc.); fasting or excessive exercise

C

A and B both occur, on average, at least twice a week for 3 months

D

Self-evaluation is unduly influenced by body shape and weight

E

Does not occur exclusively during periods of anorexia nervosa

Types

Purging, non-purging (e.g. fasting, excessive exercise)

The history11 These patients are often secretive, tend to minimise their symptoms and may be in denial of their problem. GPs are encouraged to engage these patients and keep in close contact with them, referring them as necessary. The validated screening tool SCOFF is recommended.

The SCOFF screening tool S C O F F

Do you make yourself Sick because you feel uncomfortably full? Do you worry you have lost Control over how much you eat? Have you recently lost more than 6 kg (One stone) in a 3-month period? Do you believe yourself to be Fat when others say you are too thin? Would you say Food dominates your life?

If the patient answers Yes to two or more questions, there is a high index of suspicion for an eating disorder, warranting a more detailed assessment.

Anorexia nervosa See FIGURE 67.2.

FIGURE 67.2 18-year-old adolescent with severe anorexia nervosa (BMI 7.7). This patient survived after care by her GP. Photo and history courtesy Dr MM O’Brien

Anorexia nervosa is a syndrome characterised by the obsessive pursuit of thinness Page 816 12 through dieting with extreme weight loss and disturbance of body image. The main symptoms are anorexia and weight loss. The mortality rate may be as high as 18%. It has the highest mortality and suicide rate of any psychiatric disorder.11

Typical features Up to 1% incidence among schoolgirls aged 1613 Bimodal age of onset: 13–14 and 17–18 years11 Unknown cause Poor insight Severe emaciation

Amenorrhoea Loss of body fat Sallow, dry and scaly skin, hair loss Increased lanugo body hair BMI 35 who are resistant to simple weight control measures.5 Those with obesity and associated medical problems such as angina or severe osteoarthritis

who require relatively rapid weight reduction. Possibility of endocrine or congenital cause of weight gain or loss. Any unexplained weight loss, especially if an endocrine cause or malignancy is suspected and can’t be identified. Weight loss related to a serious psychological illness or eating disorder.

Practice tips Ask patients what they really believe is the cause of their weight gain or loss. An anxiety state and hyperthyroidism can be difficult to differentiate as causes of weight loss. Perform thyroid function tests. Laboratory tests are rarely needed to establish the diagnosis of an eating disorder. Hormonal levels return to normal following weight gain. A high index of suspicion by the family doctor is required to diagnose eating disorders. Think of it in a mid-teen female; weight loss through dieting; wide fluctuation in weight; amenorrhoea and hyperactivity.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition:

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Eating disorders Obesity: how to lose weight wisely BMI calculator

Resources NHMRC Obesity guidelines. Available from: www.health.gov.au/internet/main/publishing.nsf/Content/obesityguidelines-index.htm. RACGP Red book: the 5 As approach. Available from: www.racgp.org.au/yourpractice/guidelines/snap/2-approach-to-preventive-care-in-general-practice/21-the-5as/. WHO MONICA project. MONICA Monograph and Multimedia Sourcebook. Geneva: WHO, 2003.

References 1

Funder J. Weighing it up. RACGP: Good Practice, 2014; 4: 13.

2

Papdakis MA, McPhee SJ. Current Medical Diagnosis and Treatment (52nd edn). New York: McGraw-Hill Lange, 2013: 1259.

3

Caterson ID. Weight management. Australian Prescriber, 2006; 29: 43–7.

4

Tunnessen WW Jr. Signs and Symptoms in Paediatrics (2nd edn). Philadelphia: JB Lippincott, 1988: 33–41.

5

Cardiovascular [published 2018]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited. Available from: www.tg.org.au, accessed October 2019.

6

Buckley N (Chair). Australian Medicines Handbook. Adelaide: Australian Medicines Handbook Pty Ltd, 2018: 547.

7

Hopcroft K, Forte V. Symptom Sorter (4th edn). Oxford: Radcliffe Publishing, 2010: 226– 7.

8

Beck ER, Francis JL, Souhami RL. Tutorials in Differential Diagnosis (2nd edn). Edinburgh: Churchill Livingstone, 1988: 117–20.

9

Tunnessen WW. Symptoms and Signs in Paediatrics (2nd edn). Philadelphia: Lippincott, 1988: 25–8.

10

Robinson MJ, Roberton DM. Practical Paediatrics (5th edn). Melbourne: Churchill Livingstone, 2003: 140–4.

11

Redston S et al. ‘Help us, she’s fading away’. How to manage the patient with anorexia nervosa. Aust Fam Phys, 2014; 43 (8): 531–6.

12

Young D. Eating disorders. In: Jones R et al, Oxford Textbook of Primary Medical Care. Oxford: Oxford University Press, 2004: 972–5.

13

Smink FR et al. Epidemiology, course and outcome of eating disorders. Curr Opin Psychiatry, 2013; 26(6): 543–8.

14

Fisher MM et al. Characteristics of avoidant/restrictive food intake disorder in children and adolescents: a ‘new disorder’ in DSM-5. J Adolesc Health, 2014; 55(1): 49–52.

15

Papadakis MA, McPhee SJ. Current Medical Diagnosis and Treatment. New York: McGraw-Hill Lange, 2013: 1262.

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Part 4 Mental health Page 820

68 Depression and other mood disorders

Though I am often in the depths of misery, there is still calmness, pure harmony and music inside me. I see paintings or drawings in the poorest cottages and the dirtiest corners. And my mind is driven towards these things with an irresistible momentum. VINCENT VAN GOGH, BIPOLAR SUFFERER (1853–1890) A mood disorder, also referred to as an affective disorder, is an emotional condition that significantly impacts on a person’s mood and related functions. The combination of involved symptoms leads to a predominant mood state that is abnormal in quality, duration or both.

Classification: A classification of the types of mood disorders is presented in TABLE 68.1 . There are two basic groups ranging from the very low mood of depression to the elevated mood of mania. Table 68.1

Classification of mood disorders DSM-5

Mood disorders: major depressive illness hypomanic episode manic episode Depressive disorders: major depressive disorder

persistent depressive episode disruptive mood regulation, e.g. temper tantrums premenstrual dysphoric disorder depressive disorder due to another medical condition substance-induced mood disorder other specified or non-specified disorder Bipolar and related disorders: bipolar 1 disorder bipolar 2 disorder cyclothymic disorder

The DSM-5 classification1 divides depressive disorders into major depressive disorder (MDD), disruptive mood dysregulation disorder, persistent depressive disorder (PDD) and premenstrual dysphoric disorder. Other ‘specified’ and ‘unspecified’ disorder categories allow for diagnosis of those patients who fall short of the various diagnostic criteria.2 Refer to CHAPTER 10 on depression.3 MDD is subclassified with coded course or severity specifiers. These include mild, moderate or severe (see TABLE 68.2 )—with psychotic features, in partial remission and in full remission. Non-coded specifiers can also be used, including ‘with anxious distress’, ‘with mixed features’, ‘with melancholic features’, ‘with atypical features’, ‘with moodcongruent psychotic features’, ‘with catatonia’, ‘with peripartum onset’ and ‘with seasonal pattern’.

Table 68.2

Symptom cluster Mood

Classification of severity of depressive illness, based on clinical features Mild Lowered mood Reduced joy Crying Anxiety Irritability

Moderate Reduced interest in things Reduced pleasure in things Reduced reactivity

Severe No interest in things No pleasure in things No reactivity

Depressive thought

Loss of confidence

Pessimistic about the future Feeling worthless or a failure Paranoid ideas

Hopeless, see no future, selfreproach, guilt, shame Consider illness a punishment Paranoid or nihilistic delusions

Cognition

Minor forgetfulness or lack of concentration

Indecisiveness Forgetfulness

Unable to make decisions Slowed mentation, seems cognitively impaired (pseudodementia)

Somatic

Low drive Loss of interest in food Lowered libido Mild initial insomnia; wake 1–2 times a night

Low energy, drive Eat with encouragement; mild weight loss Loss of libido Initial insomnia; wake several times a night

No energy, drive Unable to eat; severe weight loss No libido Psychomotor retardation or agitation Sleep only a few hours

Social

Mild social withdrawal

Apathy and social withdrawal Work impairment

Apathy and social withdrawal Marked work impairment Poor self-care

Suicidality

Life not enjoyable, not worth living

Thoughts of death or suicide

Evidence of intent to suicide (plans, attempts, etc.)

In a general practice setting, having a checklist of symptoms to work through with a patient can be a useful part of the assessment of the depressed patient. However, the DSM criteria can be too rigid for general practice, with many patients whom GPs recognise as having psychological issues not meeting DSM-5 criteria.4 As Ian Hickie explains, ‘Primary care psychiatry is not specialist psychiatry in general practice’.5

Types of mood disorders

Depressive disorders Major depressive episode Major depressive episode (MDE) or unipolar depression which manifests with at least five of the features described in the DSM-5 diagnostic criteria (see CHAPTER 10 where the clinical features and management of depression is covered in more detail).

Major depressive disorder (MDD) Page 821 Major depressive disorder (MDD) or unipolar depression is also referred to as major depression or clinical depression. It manifests as the presence of a single major depressive episode without a manic or psychotic presentation at any one point in time. The two key criteria for MDD in the DSM-5 are a pervasive depressed mood and marked loss of interest or pleasure (otherwise referred to as anhedonia) persisting for at least two weeks along with other criteria (see CHAPTER 10 ).

Major depression with psychotic features Major depression with psychotic features, where there is both psychotic and depressive features at one point in time.

Adjustment disorder with depressed mood Adjustment disorder with depressed mood is a period of distress and emotional disturbance following a significant stressful life event (reactive depression, e.g. loss of employment). It is a less severe form of depression without sufficient criteria for major depression, and is very common. The symptoms may resolve spontaneously or benefit from short-term counselling. Its duration is usually no longer than 6 months. There is no evidence that antidepressants are helpful.

Melancholic depression (melancholia) This is basically general MDD but is a more severe form where symptoms such as anhedonia and psychomotor retardation are prominent. The term is not currently used often by psychiatrists and other therapists.

Recurrent brief depression There is a high prevalence in general practice of patients with recurrent episodes of short duration, about 3 to 7 days, as often as monthly. Premenstrual dysphoria may be a factor. As a rule antidepressants are ineffective. Lithium is an alternative medication for long-term use. Management is based on psychotherapy, especially CBT.

Bereavement Page 822 This is a reactive depression to the death of a loved one. The expression and duration vary considerably among cultural groups. The diagnosis of MDD is usually not given until symptoms are present for 2 months after the loss. See CHAPTER 4 .

Perinatal depression This term recognises depressive symptoms throughout pregnancy and in the postnatal phase, where the disorder ranges from normal ‘baby blues’ to postnatal adjustment disorder, and finally to the severe postnatal (or postpartum) depression. It affects about 10–20% of mothers.

Postnatal blues The ‘blues’ are a very common problem occurring in up to 80% of women that arises in the first 2 weeks (usually 3–10 days after childbirth) but lasts only about 4–14 days. Clinical features are feeling flat or depressed, mood swings, irritability, feeling emotional (e.g. crying easily) and inadequate, and lacking confidence.

Postnatal adjustment disorder Occurs in the first 6 months; similar symptoms to the ‘blues’; anxiety with handling baby; psychosomatic complaints; fearful of criticism.

Postnatal depression Some women develop a very severe depression after childbirth. Always consider it in the frequent attender. Symptoms are present for at least 2 consecutive weeks, with onset in the first few days postpartum. This occurs in 10–30% of women in the first 6–12 months (usually first 6 months, peaks about 12th week); anxiety and agitation common; marked mood swings; poor memory and concentration; typical features of depression. Note: Beware of puerperal psychosis with onset usually within the first 2 weeks.

Postpartum psychosis The most common postpartum psychosis is an affective disorder: mania or agitation depression. It demands urgent attention. Symptoms that appear within the first month include unusual behaviour, agitation, delirium, hallucinations, mania and suicidal ideation. It is rare, occurring in about 1:500 births.

Dysthymic disorder or persistent depressive disorder

(PDD) Refers to longstanding chronic depressed mood of relatively mild severity for at least 2 years. The depressed mood is accompanied by two or more of the symptoms outlined in the DSM-5 criteria. Antidepressants are less predictably effective than in major depression. Note: Included in DSM-5.

Double depression This is defined as a moderate depressed mood (dysthymia) that lasts for at least 2 years.6

Disruptive mood dysregulation disorder This is a depressive disorder of children up to 18 years of age who exhibit persistent irritability and frequent episodes of extreme behavioural control and uncontrollable social behaviour without any significant provocation. Note: Included in DSM-5.

Premenstrual dysphoric disorder This diagnosis is based on the presence of a cluster of affective behavioural and emotional symptoms in the week preceding the onset of menstruation, followed by the resolution of these symptoms after onset. The symptoms must include at least five depressive symptoms (see CHAPTER 10 ). Note: Included in DSM-5.

Dysphoria This is a profound state of unease or generalised dissatisfaction with life (as opposed to euphoria). It may accompany depression, anxiety, physical discomfort, menstruation or unhappiness with one’s biological sex or usual gender role (gender dysphonia).

Atypical depression This is different to the persistent sadness of typical depression, where the individual’s mood improves with a pleasurable event.

Catatonic depression An uncommon and severe form of major depressive episode involving disturbance of motor behaviour where the person is mute and has severe psychomotor retardation with purposeless or bizarre movements. Grimacing, echolalia and echopraxia may feature. Catatonic symptoms can also appear in schizophrenia or a manic episode.

Seasonal affective disorder

Seasonal affective disorder (SAD), or ‘winter blues’ or winter depression, is a recurrent Page 823 depressive disorder seen in people living in cold climates where winters are bleak and dark. It appears in the autumn or winter and usually resolves in spring. The diagnosis is based on at least two episodes occurring in colder months and none at other times over a 2-year period or longer. Features of depression include sleeping difficulty, sadness, lethargy, irritability and anxiety, while atypical symptoms include somnolence and increased appetite (carbohydrate craving). Treatment is based on psychotherapy, phototherapy and medication such as the SSRIs. Refer to: www.sada.org.uk.

Bipolar disorders Bipolar is a broad term to describe a recurrent illness with episodes of either abnormal high mood (mania) or low mood (depression), with return to normal function in between. The swing in moods in bipolar disorders (formerly manic depression disorders) is illustrated in FIGURE 68.1 . It affects 1–2% of the population, while BP-NOS may affect between 2–5% (refer to CHAPTER 69 for features and management).

FIGURE 68.1 Bipolar disorder (manic depression): possible mood swings

Bipolar I disorder Bipolar I disorder has one fully fledged manic or mixed episode and usually depressive episodes.

Bipolar II disorder Bipolar II disorder is defined as a major depressive episode with at least one hypomanic episode

lasting a minimum of 4 days but no classic manic episodes.

Hypomania Hypomania is the term used to describe the symptoms of mania that are similar to but less severe (without criterion C) and of shortened duration. The subsequent major depressive (cyclothymic) phase is associated with a high risk of suicide.

Cyclothymic disorder Cyclothymic disorder is a form of bipolar disorder consisting of recurrent hypomanic and dysthymic episodes without full manic or major depressive episodes.

Dysphoric mania Dysphoric mania or mixed episode during manic episodes patients may also experience depressive symptoms.3

Rapid cyclic bipolar disorder Where 4 or more episodes of depression, mania or mixed episodes occur in a 12-month period.3

Bipolar disorder not otherwise specified (BP-NOS) Also known as ‘subthreshold’ bipolar, this is where the patient has some symptoms of the bipolar spectrum but does not conform to the DSM-5 diagnosis (see CHAPTER 69 ).

Community mood disorders prevalence study An interesting study conducted over 9 years commencing in 1985 among young American adults involved a selection of demographic and health characteristics.7 Lifetime prevalence based on six mood measures were estimated and showed the following distribution. 1. Major depressive episode (MDE): 8.6% 2. Major depressive disorder with severity (MDS-s): 7.7% 3. Dysthymia: 6.2% 4. MDE with dysthymia: 3.4% 5. Any bipolar disorder: 1.6% 6. Any mood disorder: 11.5%

Key points Mood disorders are marked emotional disturbances consisting of prolonged periods of profound sadness, excessive joyousness or both, or variations of both, especially with depression. Diagnosis is based on analysis of a requisite number of mood disorder symptoms as presented by the DSM-5 classification. These disorders tend to have a hereditary basis which should be addressed in the patient’s history. With all overt disorders, depression or mania, we must be very mindful of the importance of being alert for the risk of suicide.

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References 1

American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders (5th edn). Washington DC: American Psychiatric Association, 2003.

2

World Health Organization. The ICD-10 Classification of Mental and Behavioural Disorders. Geneva: WHO, 1992.

3

Psychotropic [updated 2021]. Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2021. www.tg.org.au, accessed 2019.

4

Wilhelm K et al. Who can alert the general practitioner to people whose common mental health problems are unrecognised? Med J Aust, 2008; 188(12): 114–18.

5

Hickie I. Primary care psychiatry is not specialist practice in general practice. Med J Aust, 1999; 170: 171–3.

6

Austin M-P, Highett N. Guidelines Expert Advisory Committee. Clinical practice guidelines for depression and related disorders. A guideline for primary care health professionals. Melbourne: Beyond Blue, 2011.

7

Jonas BS, Brody B, Roper M, Warren WE. Prevalence of mood disorders in a national sample of young American adults. Social Psychiatry and Psychiatric Epidemiology, 2013; 38(11): 618–24.

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69 The disturbed patient

There is not a sight in nature so mortifying as that of a Distracted Person, when his imagination is troubled, and his whole soul is disordered and confused. JOSEPH ADDISON (1672–1719) The disturbed and confused patient is a complex management problem in general practice. The cause may be a single one or a combination of several abnormal mental states (see TABLE 69.1 ).1 The cause may be an organic mental disorder, which may be a long-term insidious problem such as dementia, or an acute disorder (delirium), often dramatic in onset. On the other hand, the cause of the disturbance may be a psychiatric disorder such as panic disorder, mania, major depression or schizophrenia. Table 69.1

A general classification of psychiatric disorders1

Organic mental disorders: acute organic brain syndrome (delirium) chronic organic brain syndrome (dementia) Psychoactive and substance use disorders: toxic states drug dependency withdrawal states Schizophrenic disorders Mood disorders: major depression bipolar (manic depressive) disorder adjustment disorders with depressed mood dysthymia

Anxiety disorders: generalised anxiety disorder panic disorder obsessive–compulsive disorder phobic disorders post-traumatic stress disorder Disorders specific to children Other disorders: postpartum psychiatric illness eating disorders personality disorders body dysmorphic disorder

Key facts and checkpoints Depression affects 15% of people over 65 and can mimic or complicate any other illness, including delirium and dementia.1 Elderly patients with depression are at a high risk of suicide. Always search vigorously for the cause or causes of delirium. Seeing patients in their home is the best way to evaluate their problem and support systems. It allows opportunities for a history from close contacts and for checking medication, alcohol intake and other factors. The diagnosis of dementia can be overlooked: a Scottish study showed that 80% of demented patients were not diagnosed by their GP.2 Patients with a chronic brain syndrome (dementia) are at special risk of an acute brain syndrome (delirium) in the presence of infections and many prescribed drugs.1 Consider prescribed and illicit substances, including the severe anticholinergic delirium syndrome. The key feature of dementia is impaired memory. The two key features of delirium are disorganised thought and attention.

Hallucination guidelines: auditory: psychoses, e.g. schizophrenia visual: almost always organic disorder olfactory: temporal lobe epilepsy tactile: cocaine abuse, alcohol withdrawal

The manifestations of the disturbance are many and include perceptual changes and hallucinations, disorientation, changes in consciousness, changes in mood from abnormally elevated to gross depression, agitation and disturbed thinking, including delusions.

A diagnostic approach A summary of the diagnostic strategy model for the disturbed or confused patient is presented in TABLE 69.2 . Page 826

Table 69.2

The disturbed mind: diagnostic strategy model

Probability diagnosis The 4 Ds: dementia delirium (look for cause) depression drugs: toxicity, withdrawal Serious disorders not to be missed Cardiovascular: CVAs cardiac failure arrhythmia acute coronary syndromes Neoplasia: cerebral cancer (e.g. lung) Severe infections:

septicaemia HIV infection infective endocarditis Hypoglycaemia Bipolar disorder/mania Schizophrenia states Anxiety/panic Subdural haematoma Pitfalls (often missed) Illicit drug withdrawal Fluid and electrolyte disturbances Faecal impaction (elderly) Urinary retention (elderly) Hypoxia Pain syndromes (elderly) Rarities: autoimmune encephalitis hypocalcaemia/hypercalcaemia kidney failure hepatic failure prion diseases (e.g. Creutzfeldt–Jakob disease) Seven masquerades checklist (all possible) Depression Diabetes Drugs Anaemia Thyroid disorder Spinal dysfunction (severe pain in elderly) UTI Is the patient trying to tell me something? Consider anxiety, depression, emotional deprivation or upset, change in environment, borderline personality disorder, serious personal loss.

Glossary of terms

Alzheimer disease A term used for both senile and presenile dementia, which has characteristic pathological degenerative changes in the brain. Cognition The mental functions of perception, thinking and memory. It is the process of ‘knowing’. Compulsions Repeated, stereotyped and seemingly purposeful actions that the person feels compelled to carry out but resists, realising they are irrational (most are associated with obsessions). Confusion Disorientation in time, place and person. It may be accompanied by a disturbed conscious state (see TABLE 64.1 , CHAPTER 64 ). Conversion The process by which thoughts or experiences unacceptable to the mind are repressed and converted into physical symptoms. Delirium (also termed ‘toxic confusional state’) A relatively acute disorder in which impaired consciousness is associated with abnormalities of perception or mood. Delusions Abnormal, illogical or false beliefs that are held with absolute conviction despite evidence to the contrary. Dementia An acquired, chronic and gradually progressive deterioration of memory, intellect and personality. Presenile dementia or early-onset dementia is dementia under 65 years of age. Senile dementia refers to older patients (usually over 80 years). Dissociation A psychological disorder in which unpleasant memories or emotions are split off from consciousness and the personality and buried into the unconsciousness. Depersonalisation An alteration in the awareness of the self—the person feels unreal. Hallucinations Disorders of perception quite divorced from reality. Features: mostly auditory or visual a false perception—not a distortion perceived as normal perceptions independent of the person’s will Illusions False interpretations of sensory stimuli such as mistaking people or familiar things. Obsessions Recurrent or persistent thoughts, images or impulses that enter the mind despite efforts to exclude them.

Somatisation The conversion of mental experiences or states into bodily symptoms, with no physical causation.

Probability diagnosis Page 827 The diagnosis depends on the age and presentation of the patient. In a teenager the probable causes of acute confusion or irrational behaviour include drug toxicity or withdrawal, schizophrenia, severe depression or a behavioural disorder.

It is the elderly who commonly present with confusion. The questions that must be asked are: Is the problem one of the 4 Ds—dementia, delirium, depression or drugs—or something else? If delirium is the problem, what is the cause? Depression affects 15% of people over 65 and can mimic other causes of confusion and behavioural disturbance. Significant prescribed drugs include hypnotics, sedatives, oral hypoglycaemics, antihypertensives, digoxin, antihistamines, anticholinergic drugs and antipsychotics.

Serious disorders not to be missed There are many serious underlying disorders that must be considered, especially with delirium (see TABLE 69.3 ). Cerebral organic lesions, including space-occupying lesions (e.g. cerebral tumour, subdural haematoma), severe infection (systemic or intracerebral) and cancer at any site, especially lung, breast, bowel or lymphoma, must be ruled out. Table 69.3

Important causes of delirium (typical examples of each group)

Drug intoxication and drug sensitivity Anticholinergics Antidepressants Sedatives Alcohol, opioids, etc. Withdrawal from substances of abuse and prescribed drugs Alcohol Opioids Amphetamines Cannabis

Sedatives and anxiolytics Infections Specific:

Urinary tract Lower respiratory (e.g. pneumonia) Otitis media Cellulitis

Intracranial:

Meningitis Encephalitis

Systemic:

Infective endocarditis Septicaemia HIV Other viral infections Malaria

Metabolic disturbances Uraemia, hepatic failure Electrolyte disturbances Dehydration Endocrine disturbances Diabetic ketoacidosis, hypoglycaemia Hypothyroidism/hyperthyroidism Nutritional and vitamin deficits Vitamin B complex deficiency (esp. B6, B12) Wernicke encephalopathy Hypoxia Respiratory failure, cardiac failure, anaemia Vascular CVA Acute coronary syndromes Head injury and other intracranial problems Seizures Complex partial seizures ‘Subtle’ causes Pain (e.g. herpes zoster) Emotional upset

Environmental change Peri-operative/anaesthetic effect Faecal impaction Urinary retention

The sudden onset of delirium may suggest angina, myocardial infarction or a cerebrovascular accident. Twenty per cent of patients with delirium also have underlying heart failure.3

Pitfalls There are many pitfalls, especially with drug toxicity or withdrawal from the so-called illicit drugs. In the elderly in particular, fluid and electrolyte disturbances, such as dehydration, hypokalaemia, hyponatraemia and hypocalcaemia, can cause delirium. Bowel disturbances such as faecal impaction or constipation can cause delirium and incontinence of both faeces and urine.

Seven masquerades checklist All the following disorders can cause disturbed or confused behaviour, particularly in the elderly: depression: a very important cause of ‘pseudodementia’ drugs: toxicity or withdrawal (see TABLE 69.4

)

diabetes: especially hypoglycaemia, which can occur with type 2 anaemia: often from self-neglect or chronic blood loss thyroid disorders: both hyperthyroidism and hypothyroidism can present with disturbed behaviour; ‘myxoedemic madness’ may be precipitated by atropine compounds urinary tract infection: causes or contributes to 20% of cases of hallucinations or illusions2 spinal dysfunction: with its many severe pain syndromes, such as sciatica, can be a significant factor Page 828

Table 69.4

Prescribed drugs that can cause delirium

Anticholinergic: antiparkinsonian (e.g. benztropine) tricyclic antidepressants

Tranquillisers and hypnotics: major tranquillisers (e.g. chlorpromazine) minor tranquillisers (e.g. diazepam) hypnotics lithium Anti-epileptics Antihistamines 1 and 2 Antihypertensives Corticosteroids Cardiac drugs: digoxin diuretics beta blockers Opioids Sympathomimetics

Psychogenic factors Apart from the primary psychiatric disorders of anxiety, depression, mania and schizophrenia, relatively simple and subtle social problems, such as loneliness, boredom, a domestic upset, financial problem or similar issues, can trigger a confusional state.

The clinical approach History Developing rapport with the disturbed or confused patient is essential and can be helped by a warm handshake or a reassuring pat on the shoulder. The basis of the history is a careful account from relatives or witnesses about the patient’s behaviour. When communicating with the patient, speak slowly and simply (avoid shouting), face the patient and maintain eye contact. Important features are the past history and recent psychosocial history, including recent bereavement, family upsets and changes in environment. Search for evidence of depression and note any organic symptoms such as cough, constipation and so on.

Mental status examination The most practical bedside screening test of mental function is the Mental Status Questionnaire of Kahn and colleagues,4 which includes 10 simple questions.

1. What is the name of this place? 2. What city are you in now? 3. What year is it? 4. What month is it? 5. What is the date today? 6. What year were you born? 7. When is your birthday? 8. How old are you? 9. Who is the prime minister/president? 10. Who was the prime minister/president before him? (Interpretation: normal 9–10; mildly impaired 8–9; confused/demented 7 or less.) Other MMSEs are presented in CHAPTER 125

.

Examination The patient’s general demeanour, dress and physical characteristics should be noted at all times. Assess the patient’s ability to hear, see, speak, reason, obey commands, stand and walk. Any problems related to the special senses can cause confusion. Look for features of alcohol abuse, Parkinson disease and hypothyroidism. Examine the neurological system and keep in mind the possibility of a subdural haematoma, which may have followed a forgotten fall. Don’t omit the rectal examination to exclude faecal impaction, melaena, cancer and prostatomegaly (in males), and also check the bladder for evidence of chronic retention.

Investigations Investigations to consider for delirious or demented patients (unknown cause): urinalysis and microscopy cultures of blood and urine total and differential blood count; ESR

blood glucose urea and creatinine and electrolytes calcium and phosphate vitamin D

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thyroid function tests liver function tests serum vitamin B12 and folate levels ECG/troponin (?acute coronary syndrome) chest X-ray cerebral CT scan, especially non-contrast CT syphilis serology HIV arterial blood gases

Behavioural emergencies: management of the acutely disturbed patient1 Delirious or psychotic patients can be paranoid and respond defensively to the world around them. This behaviour can include aggressive and violent behaviour, resulting in danger to themselves, their friends and family and to their medical attendants. Dangerousness should be assessed from features such as the patient’s past history (especially previous dangerous behaviour), age, sex, recent stress, victim behaviour, muscle bulk, presence of weapons, degree of overactivity and the manner of handling of the present distress by others. The patient may be in a state of acute panic and trying to flee a situation or in an agitated psychotic state prepared to confront the situation. It should be emphasised that most violent individuals are not mentally ill. Most cases require an injection (the ideal intravenous administration can be extremely difficult and hazardous), which is often interpreted as a physical attack. It may not be possible to diagnose the cause of the problem before giving the injection.

Approach to management

Assess the environment and don’t move into the patient’s space until in a position of control. React calmly. Communicate calmly and simply. State your task firmly and simply. Try to control the disturbed patient gently. Ensure the safety of all staff and make certain that heroics are not attempted in dangerous circumstances. An adequate number of staff to accompany the doctor is essential—six is ideal (one for immobilisation of each limb, one for the head and one to assist with drugs).1 Patients should be placed on the floor in the prone position.

Principles of sedative administration1 Use the safest possible route of administration whenever possible (i.e. oral in preference to parenteral but often impractical). Intravenous administration has the lowest margin of safety. Parenteral administration should be restricted to severely disturbed patients. Closely monitor vital signs during and after sedative administration. Avoid intramuscular diazepam because of poor absorption. Be cautious of intravenous midazolam in such patients because of the risk of respiratory depression. Avoid benzodiazepines in patients with respiratory insufficiency. Haloperidol is an alternative. Patients have died from cardiopulmonary arrest after repeated sedative administration (especially benzodiazepines), so intensive monitoring is essential. Monitor the following adverse effects: respiratory depression hypotension dystonic reactions, including choking neuroleptic malignant syndrome

Treatment options1 The treatment in acute medical and psychiatric settings depends on the appropriate mode of

administration. Benzodiazepines are generally the drugs of first choice over antipsychotics in tranquillisation.5 However, the IV route allows titration to the desired degree of sedation and has a more immediate effect.

Intravenous medication diazepam or midazolam 5 mg IV initially then 2.5–5 mg increments IV, repeated every 3–4 minutes until required level of sedation (rousable drowsiness) is reached—up to a maximum of 20–30 mg, when specialist advice is needed, especially if further boluses are necessary and/or droperidol 5–10 mg IV or olanzipine

Intramuscular medication If this route considered appropriate: midazolam 5–10 mg IM or (if history of benzodiazepine tolerance) droperidol 5–10 mg IM

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or olanzapine 5–10 mg repeated every 2–4 hours or combination midazolam/droperidol (These first two injections can be repeated in 20 minutes if required. Droperidol is similar to haloperidol but more sedating. Keep in mind the rare but potentially fatal laryngeal dystonia with high doses—cover with benztropine 2 mg IM.)

Oral medication (if considered appropriate) diazepam 5–20 mg (o), repeated every 2–6 hours (max. 120 mg/24 hours) or lorazepam 1–2 mg (o), repeated every 2–6 hours (max. 10 mg/24 hours) If sedation is not achieved, add an antipsychotic medication, e.g. olanzapine 5–10 mg initially or risperidone 0.5–1 mg initially.

Postdisturbance evaluation Determine the likely cause, such as: acute organic brain syndrome: toxic causes, infection alcohol or drugs (illicit or prescribed): intoxication, withdrawal manic illness severe depression schizophrenic syndrome severe panic

Postoperative cognitive dysfunction and dementia This occurs in up to 12% of apparently previously cognitively normal patients who show a decline of cognitive function, especially in memory and executive function. The study6 shows that it can occur in surgery other than cardiac surgery, even with regional anaesthesia. It is more common in those over 65 years. It is usually self-limiting and lasts 1–12 months.

Acute organic brain syndrome (delirium) The many labels of acute organic brain syndrome include: delirium acute confusional state toxic confusional state confusional episode acute brain syndrome

Main clinical features Clouding of conscious state Disorientation Impaired attention Impaired memory

Global cognitive defect—onset over days/hours Refer to the box.

DSM-5 criteria for delirium5 Diagnosis of delirium requires evidence of: A Disturbance of consciousness, attention and awareness B Clinical features appearing over a short period C A change in cognition: perceptual disturbance incoherent speech disorientation memory impairment/deficit D E

A & C not better explained by another disorder Evidence of a cause

Other clinical features1 The patients are usually elderly. Anxiety and agitation can be severe but in hypoactive deliria (usually due to metabolic disturbance) the conscious state can vary from drowsiness to coma. Odd behaviour with mood swings can occur. Psychotic symptoms can occur. Delusions are usually fleeting. The disturbance is usually worse at night and may be aggravated by sedation. Visual hallucinations are a feature of alcohol withdrawal. Attacks on bystanders may result (uncommon). Always seek a cause.1 A list of causes is presented in TABLE 69.3 are:

. The most important causes

infections (usually in urinary tract, lungs or ear, or systemic in young or elderly)

prescribed drugs Anticholinergic delirium Consider this cause (from drugs with anticholinergic properties or illicit substances). Features include hyperactivity, marked thought disorder, vivid visual hallucinations and very disturbed behaviour. Differential diagnosis of delirium Page 831 In the earlier stages it may mimic the various psychiatric disorders, including anxiety, depression, various hallucinatory states, particularly agitated schizophrenia (rarely), extreme manic states, complex partial seizures, dementia. Consider deafness. Delirium is common in the hospital setting, especially in patients ≥65 years of age.

Investigations Investigations are those listed under ‘The clinical approach’ earlier in the chapter.

Treatment Principles: Acute delirium is a medical emergency. Establish normal hydration, electrolyte balance and nutrition. Consider alcohol withdrawal and give a trial of thiamine when the cause of delirium is unknown. Attend to helpful environmental factors (e.g. calm atmosphere, a night-light, orientation clues, presence of friends and relatives). Give oxygen if hypoxic, e.g. respiratory distress. Medication Medication1 may not be needed, but it will be if there are symptoms of anxiety, aggression or psychosis (doses for a fit adult). A single dose is usually adequate.1 For anxiety and depression: midazolam 1.25–5 mg IM For psychotic behaviour: haloperidol 0.5 mg (o) as a single dose

or olanzapine 2.5 mg (o) daily as a single dose If oral administration is not possible or when parenteral medication is required (cover with benztropine 2 mg (o) or IM): haloperidol 0.5 mg IM as a single dose or olanzapine 2.5 mg IM as a single dose For anticholinergic delirium: tacrine hydrochloride 15–30 mg with caution by slow IV injection (an antidote) Note: For hypoxia, give oxygen. Avoid benzodiazepines, especially in children and in patients with respiratory insufficiency. Consider necessity for pain relief. Use lower doses of parenteral medications in the very old and frail.

Dementia (chronic organic brain syndrome) Dementia or neurocognitive disorder is an important diagnosis to consider in the elderly patient. The DSM criteria for dementia are presented in CHAPTER 125 . The main feature of dementia is impairment of memory, especially recent memory, when the person cannot remember what has happened a few hours (or even moments) earlier but may clearly remember the events of the past. The more serious behavioural changes encountered with dementia tend to occur in the advanced stages. However, these disturbances may be precipitated by illness such as infections, emotional upset and drugs. These serious disturbances include: uninhibited behaviour hallucinations (generally uncommon) paranoid delusions If a stable patient becomes acutely disturbed, delirium should be suspected.

Presenile dementia—Alzheimer type The main features are: onset in late 50s and early 60s insidious onset early loss of short-term memory progressive decline in intellect death in 5–10 years more common in Down syndrome

Differential diagnosis of dementia There are two approaches to the differential diagnosis, including consideration of the classic causes of disturbed behaviour as summarised in the mnemonic in TABLE 69.5 .7 Table 69.5

Differential diagnosis of dementias

D

=

Delirium drugs (see toxic)

E

=

Emotional disorder = depression endocrine = thyroid

M

=

Memory = benign forgetfulness

E

=

Elective = anxiety disorders/neuroses

N

=

Neurological: CVA head trauma

T

=

Toxic: drugs/medication metabolic disease

I

=

Intellect—low or retarded

A

=

Amnesic disorders—Korsakov syndrome

S

=

Schizophrenia (chronic)

Source: Reproduced with permission from McLean S. Is it dementia? Aust Fam Physician, 1992; 21: 1762–6.

However, the foremost differential diagnosis should be ‘pseudodementia’ caused by severe depression. A simple comparison between schizophrenia and dementia is shown in TABLE 69.6

Table 69.6

.

Comparison of schizophrenia and dementia

Onset

Dementia Middle-aged or elderly

Schizophrenia Young

Memory

Always impaired

Usually unaffected

Delusions

Rare

Frequent

Hallucinations

Uncommon

Frequent

Thought broadcasting

Never

Frequent

A vigorous search for a possible cause of dementia is warranted since there are a significant number of reversible causes. In particular, it is important to exclude the psychiatric conditions that may mimic dementia.

Treatment1 To control psychotic symptoms or disturbed behaviour:

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risperidone 0.5–2 mg (o) daily or olanzapine 2.5–10 mg (o) daily in 1 or 2 doses To control symptoms of anxiety and agitation: oxazepam 7.5 mg (o) 1 to 4 times daily. Avoid benzodiazepines for more than 2 weeks For depression—administer antidepressants Treat any folate and vitamin D deficiency

The acute psychotic patient Acute psychosis is the presence of the mental state where appreciation of reality is impaired as

evidenced by the presence of typical psychotic symptoms such as delusions, hallucinations, mood disturbance and bizarre behaviour.8 Refer to the diagnostic strategy for hallucinations in TABLE 69.7 . Table 69.7

Hallucinations: diagnostic strategy model

Probability diagnosis Drugs (illicit or prescribed) Alcohol (acute or chronic) Schizophrenia Febrile delirium Affective (mood) disorders Drug withdrawal (incl. alcohol, hypnotics) Dementias (esp. Lewy body) Serious disorders not to be missed Vascular: cerebrovascular disease migraine (luminous) Infections: encephalitis/meningitis septicaemia any serious febrile illness Tumours: cerebral tumours cancer treatment Other: hypoxia liver failure metabolic/electrolyte imbalance dehydration Pitfalls (often missed) Major depression Extreme fatigue Vitamin deficiency (esp. B group) Seizure disorders (esp. complex partial) Rarities: narcolepsy

post-concussion bereavement multiple sclerosis Seven masquerades checklist Depression Diabetes Drugs (iatrogenic/social, illicit) Thyroid/other endocrine (hypothyroid) Urinary tract infection (esp. elderly) Is the patient trying to tell me something? Consider conversion disorder (hysteria); fabrication.

The differential diagnoses of patients presenting with psychoses is presented in TABLE 69.8 Table 69.8

.

Causes of psychoses8

Functional psychoses: schizophrenia schizophreniform disorder (shorter duration of symptoms) schizoaffective disorder (core symptoms of schizophrenia + mood symptoms) bipolar mood disease (depressed or manic phase) Drug-induced psychoses Organic-based psychoses Other: delusional disorder (paranoid psychoses) brief psychotic disorder (rapid resolution) folie à deux (psychosis occurring simultaneously in two close associates)

Early diagnosis Early recognition of a psychosis, particularly schizophrenia, is extremely important, as early intervention leads to improved outcomes. Early or prodromal symptoms include the following: social withdrawal reduced attention and concentration

reduced drive and motivation

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depressed mood anxiety irritability/agitation suspiciousness sleep disturbance deterioration in role functioning It is appropriate to ask the correct questions in order to elicit psychotic symptoms. These are presented in TABLE 69.9 . Table 69.9

Questions for eliciting psychotic symptoms

Anxiety

Have you been feeling especially nervous or fearful? Have you felt tense and shaky, or experienced palpitations?

Depressed mood

Have you been feeling sad or ‘down in the dumps’ recently, not enjoying activities as much as before?

Elevated mood

Have you been feeling especially good in yourself, more cheerful than usual and full of life?

Auditory hallucinations

Do you hear voices of people talking to you even when there is no one nearby?

Thought insertion

Have you felt that thoughts are being put into your mind? Do you experience telepathy?

Thought withdrawal

Have you experienced thoughts being taken out of your mind?

Thought broadcasting

Have you felt that other people are aware of your thoughts?

Thought echo

Have you experienced voices or people echoing your thoughts?

Delusion of control

Have you felt under the control or influence of an outside force?

Delusions of reference

Do programs on the television or radio hold special meaning for you?

Delusions of persecution

Do you feel that you are being singled out for special treatment? Is there a conspiracy against you?

Delusions of grandeur

Do you feel special, with unusual abilities or power?

Delusions of guilt

Do you believe that you have sinned or have done something deserving punishment?

Source: Reproduced with permission from Keks N , Blashki G. The acutely psychotic patient: assessment and initial management. Aust Fam Physician, 2006; 35 (3): 90–4.

DSM-5 key diagnostic criteria5—schizophrenia A

Two or more of the following, each present for a significant portion of time during a 1-month period

B C D E

Social, learning or occupational dysfunction Continuous signs of disturbance for at least 6 months No evidence of other psychoses, e.g. bipolar Not attributable to effects of substance abuse or other medical condition

Schizophrenia and associated disorders The term ‘schizophrenia’ (Bleuler, 1911) refers to a group of severe psychiatric illnesses characterised by severe disturbances of emotion, language, perception, thought processes, volition and motor activity. The causes of schizophrenia disorders are unknown, but genetic factors and drug abuse are implicated.

Signs and symptoms of schizophrenia Positive delusions hallucinations thought disorder disorganised speech and behaviour Negative

flat affect poverty of thought lack of motivation social withdrawal reduced speech output Cognitive distractibility impaired working memory impaired executive function (e.g. planning) impaired insight Mood mania (elevation) depression Other features include: bizarre behaviour subject to tension, anxiety or depression deterioration in work and study performance peak incidence 15–25 years9—smaller peak at 40 years lifetime prevalence 1 in 100 equal sex incidence high risk of suicide

Differential diagnosis Organic factors need to be excluded, especially drugs: amphetamines hallucinogens (e.g. LSD)

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marijuana Also consider complex partial seizures and a personality disorder. Other psychoses are presented in TABLE 69.8 . A comparison of delirium, dementia and functional psychosis is presented in TABLE 69.10

Table 69.10

.

Comparison of the clinical features of delirium, dementia and acute functional psychoses9

Feature Onset

Delirium Rapid

Dementia Slow— insidious

Acute psychosis Rapid

Duration

Hours to weeks

Months to years

Depends on response to treatment

Course over 24 hours

Fluctuates— worse at night

Minimal variation

Minimal variation

Consciousness

Reduced

Alert

Alert

Perception

Misperceptions common, especially visual

Misperceptions rare

May be misperceptions

Hallucinations

Common, visual (usually) or auditory

Uncommon

Common, mainly auditory

Attention

Distractable

Normal to impaired

Variable—may be impaired

Speech

Variable, may be incoherent

Difficulty finding correct words

Variable: normal, rapid or slow

Organic illness or drug toxicity

One or both present

Often absent

Usually absent

Management Drug treatment is only a part of total management. Explanation and appropriate reassurance to the family with patient and family supportive care is obviously essential. Supportive psychotherapy is important in all phases. A team approach is necessary to cope with the disorder, which usually has a devastating effect on the family. Referral for specialist care is appropriate.

Acute phase Hospitalisation usually necessary Drug treatment for the psychosis1 Drug treatment may include the first-generation (typical or conventional) antipsychotics such as haloperidol and chlorpromazine, which are effective for managing the ‘positive’ symptoms, or the second generation (atypical) antipsychotics such as risperidone, olanzapine, quetiapine, clozapine, amisulpride and aripiprazole, which in addition are more effective at treating the ‘negative’ and other symptoms of schizophrenia.10 The usual practice rule is to start with a second-generation antipsychotic at a low dose and titrate upwards at a rate and to a level that is optimal for the patient. Patients with a first psychotic episode may respond to lower than usual doses.5 1. When oral medication is possible, first-line treatment (for the first episode) is one of (with starting doses):1,11,12 amisulpride 100 mg nocte asenapine 5 mg sublingual bd aripiprazole 10 mg once daily olanzapine 5 mg nocte paliperidone CR 3 mg nocte quetiapine 50 mg bd → 200 mg bd (by day 5) sertindole 4 mg daily risperidone 0.5–1 mg nocte → 2 mg nocte ziprasidone 40 mg bd → 80 mg bd (risk of ↑ QT) zuclopenthixol 20 mg nocte Page 835

Practice tip Typical initial oral therapy: olanzapine 5 mg or

risperidone 0.5 mg

If response is inadequate in 3 weeks, increase the dose according to prescribing guidelines. If no response after 4–6 weeks consider a change to:13 an alternative second-generation agent (above) or a first-generation antipsychotic such as: chlorpromazine 200 mg once daily → 500 mg haloperidol 1.5 mg once daily → 7.5 mg trifluoperazine 2 mg bd 2. Parenteral medication should be avoided if possible in acute care, but if required:1,12 haloperidol 2.5–10 mg IM initially, up to 20 mg in 24 hours, depending on the response or olanzapine 5–10 mg IM initially (do not use with benzodiazepines concurrently) add benztropine 1–2 mg (o) bd (to avoid dystonic reaction) or zuclopenthixol acetate 50–150 mg IM as a single dose If dystonic reaction: benztropine 1–2 mg IV or IM If very agitated use: diazepam 5–10 mg (o) up to 40 mg/day or 5–10 mg IV Chronic phase Long-term antipsychotic medication is recommended to prevent relapse.1 Examples of oral medication regimens:12,13

olanzapine 5–10 mg (o) nocte or risperidone 0.5–1 mg (o) bd, up to 2–4 mg or quetiapine 150 mg (o) bd Aim for lowest possible dose to maintain control. Chlorpromazine is not recommended for long-term use because of photosensitivity reactions. Use depot preparations if compliance is a problem (usually test dose first).1,11 Examples include: haloperidol decanoate 50 mg IM initially, then 50–200 mg every 4 weeks or flupenthixol decanoate 10 mg IM initially, then 20–40 mg every 2–4 weeks or risperidone 25–25 mg IM initially, then every 2 weeks, titrated to clinical response or zuclopenthixol 100 mg IM initially, then titrated to 200–400 mg every 2–4 weeks Tips with depot preparations: Start with IM test doses and then titrate to recommended controlling levels (half or full starting dose). May take 2–4 months to produce a stable response, so oral supplements may be necessary. Not as effective as oral therapy. Give as deep IM injection with 21 gauge needle in buttock. Use lowest possible dose to avoid tardive dyskinesia. Reassess at least every 3 months. Closely monitor patient for movement disorders. Drug-resistant schizophrenia14,15

Consider other causes (e.g. substances abuse). ECT may help the agitated patient, especially if catatonic. Consider a trial of clozapine (12.5 mg (o) bd initially, increasing to 200–600 mg daily), with strict monitoring for blood dyscrasias and cardiotoxicity, or olanzapine (5–20 mg daily). A trial of adjunctive ECT should be considered in patients who fail to respond to clozapine.

Movement disorders from antipsychotic medication1 Acute dystonias Usually bizarre muscle spasms affect face, neck, tongue and trunk Oculogyric crises, opisthotonos and laryngeal spasm Treatment: benztropine 1–2 mg IV or IM Akathisia Subjective or objective motor restlessness of feet and legs Generally later onset in course of treatment Treatment: reduce dosage until akathisia less troublesome or substitute thioridazine can use oral propranolol, diazepam or benztropine as a short-term measure Parkinsonian Seen relatively early in treatment

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The akinesia can be confused with drug-induced depression Treatment: use lower dose or substitute a phenothiazine in low dosage alternatively, use benztropine or benzhexol Tardive dyskinesia1 Tardive dyskinesia is a syndrome of abnormal involuntary movements of the face, mouth, tongue, trunk and limbs. This is a major problem with the use of long-term antipsychotic drugs and may occur months or years (usually) after starting treatment and with drug withdrawal.

Avoid prolonged use of metoclopramide. Differential diagnosis: spontaneous orofacial dyskinesia senile dyskinesia ill-fitting dentures neurological disorders causing tremor and chorea If drug withdrawal is ineffective, use tetrabenazine 12.5 mg (o) daily, increasing as necessary.13 The risks and benefits of continuing therapy have to be weighed. Note: Because of the difficulty with managing tardive dyskinesia, prevention in the form of using the lowest possible dosage of antipsychotic medication is essential. This involves regular review and adjustment if necessary. Neuroleptic (antipsychotic) malignant syndrome This is a potentially fatal adverse effect that can develop at any time. It develops in hours to days. Syndrome: high temperature, muscle rigidity, altered consciousness. Milder variants can occur (refer to CHAPTER 42 ). Treatment: discontinue medication ensure adequate hydration with IV fluids if life-threatening: bromocriptine 2.5 mg (o) bd, gradually increasing to 5 mg (o) tds and dantrolene 50 mg IV every 12 hours for up to 7 doses consultant referral Cardiac dysfunction Various psychotrophic agents, particularly the phenothiazines, are prone to cause the adverse effect of prolongation of the QT interval with potential severe outcomes. Patients should be monitored.

Bipolar disorder The mood disorders are divided into depressive disorders and bipolar disorders (see CHAPTER 68 ). Bipolar is a broad term to describe a recurrent illness with episodes of either mania or depression with return to normal function in between. The swing in moods in bipolar disorders (manic depressive disorders) is illustrated in FIGURE 68.1 . It affects 1% of the population.

DSM-5 criteria for a manic episode5 A B

Distinct period for at least 1 week of abnormal and persistent elevated, expansive or irritable mood Three or more of these unusual features: 1. inflated self-esteem or grandiosity 2. decreased need for sleep 3. talkative/accelerated speech 4. racing thoughts or flights of ideas 5. distractability as reported or observed 6. increased goal-directed activity or psychomotor agitation 7. excessive activity with ‘painful’ consequences

C D

Marked impaired social or occupational functioning or need for hospitalisation or psychotic features Episode not due to substance abuse or other medical condition

A–D = a manic episode: at least one in a lifetime for diagnosis

Bipolar I disorder has one fully fledged manic or mixed episode and usually depressive episodes. Bipolar II disorder is defined as a major depressive episode with at least one hypomanic episode lasting a minimum of 4 days but no classic manic episodes. The symptoms of mania may appear abruptly, usually in the teens or young adulthood. Typical inherent features, in addition to the above, include: reckless behaviour, overspending

hasty decisions (e.g. job resignation, hasty marriages) impaired judgment increased sexual drive, energy and activity poor insight into the problem variable psychotic symptoms—paranoia, delusions, auditory hallucinations Note: The peak onset is in early adult life. The exact cause is unknown, but there is a strong hereditary basis. Episodes may be precipitated by stress.

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‘Hypomania’ is the term used to describe the symptoms of mania that are similar to but less severe (without criterion C) and of shorter duration. The subsequent major depressive phase is associated with a high risk of suicide. Good questions to ask the patient with suspected ‘bipolar’ disorder: How have you been feeling in yourself? Have you felt especially good about yourself? Do you feel that you are special or have special powers? Have you been spending more than usual? Have you been needing less sleep than usual?

Management of acute mania1,16 This is a medical emergency requiring hospitalisation for protection of both family and patient. Involuntary admission is usually necessary. It may be a first episode or a relapse due to poor treatment compliance or substance abuse. A recent meta-analysis indicates that antipsychotics are the most efficacious drugs.

Treatment First line:1 olanzapine 5 mg (o) nocte initially, then increasing to 10 mg or risperidone 1 mg (o) nocte initially, then increasing to 2 mg Second line:

haloperidol or a second-generation antipsychotic, e.g. aripiprazole1,17 quetiapine, asenapine or lithium carbonate 750–1000 mg (o) daily in 2 or 3 divided doses increasing according to serum levels (warn patients and family about toxicity) or (a mood stabilising agent) sodium valproate 200–400 mg (o) bd initially or carbamazepine 100–200 mg (o) bd initially If a parenteral antipsychotic drug is required: haloperidol 5–10 mg IM or IV Repeat in 15–30 minutes if necessary (risk of tardive dyskinesia). Change to oral medication as soon as possible. Failure to respond to treatment:18,19,20 ensure maximum concentration of first drug switch to a different drug, e.g. olanzapine to lithium combine drugs, e.g. second-degree antipsychotic + lithium ECT is of proven benefit for recalcitrant problems Remember to provide supportive psychotherapy with appropriate psychosocial interventions. Prophylaxis for recurrent bipolar disorder Over 90% will have a recurrence at some time: consider medication if two or more episodes of either mania or depression in the previous 4 years. Recommended prophylactic agents5 lithium carbonate 125–500 mg (o) bd then adjusted or second-generation antipsychotic agent or (if depression prominent)

lamotrigine or carbamazepine or sodium valproate Use long-term lithium (e.g. 3–5 years). Target plasma level for maintenance is usually 0.6–0.8 mmol/L. A US study recommended lithium as the prime mood stabiliser.17 If poor response, use another agent. Unwanted side effects of lithium include: a fine tremor muscle weakness weight gain gastrointestinal symptoms hypothyroidism nephrotoxicity With anti-epileptics, adjust dosage according to clinical response and toxicity. Management of bipolar depression11,18 This is a difficult component to treat and antidepressants should not be used alone.12 Many mood-stabilising agents appear to have a bimodal (antidepressant and antimania) effect and can be useful in the absence of classical antidepressants.16 A recommended regimen is: an antidepressant (e.g. SSRI, SNRI or MAOI) plus lithium, valproate, carbamazepine, quetiapine, lamotrigine or olanzapine (one of these used for prophylaxis). Avoid using antidepressants alone. Antidepressants are usually withdrawn within 1–2 months because of a propensity to precipitate mania. ECT is an effective treatment for bipolar depression while psychological therapies such as CBT and psychoeducation have proven efficacy.

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Bipolar I patients usually recover but proceed to have further episodes of depression or mania.12 Liaison with family and carers

Promote a caring support and pyschoeducation program for patient and family. Educate about the patient’s ‘relapse signature’ for a manic or depressive episode.

Body dysmorphic disorder5 Body dysmorphic disorder, which is a type of somatoform anxiety disorder, is characterised by a preoccupation with the belief that some aspect of physical appearance is abnormal, unattractive or diseased. The person’s concern and distress is out of proportion to any imagined or actual defect and usually not amenable to reassurance. This preoccupation causes significant functional impairment. The condition rarely presents directly and may be over-represented in the area of dermatology or plastic surgery. It begins in late childhood or early adolescence. The person’s focus is on the face, head or secondary sexual characteristics. Patients may be helped by counselling and psychotherapy including CBT. There is clinical evidence that SSRIs help if the symptoms suggest an obsessive–compulsive disorder. An antipsychotic agent may help where beliefs are delusional or in the context of a psychotic disorder.

Depression Depression is very common and presents in a great range of severity. In the context of ‘the disturbed patient’ depression can be confused with dementia or a psychosis, particularly if the following are present: psychomotor agitation psychomotor retardation delusions hallucinations

Assessment1 The following questions need to be addressed: Is the depression primary (i.e. not secondary to another psychiatric condition such as schizophrenia or anxiety disorder)? Is it part of a bipolar disorder? Has there been a previous manic or hypomanic episode? If so, a different approach to treatment is required. Is the depression caused by another illness or physical factor (e.g. hypothyroidism, cerebrovascular disease or medication)? Is the patient psychotic?

Is the patient a suicide risk? The treatment of depression is presented in CHAPTER 10

.

Psychoactive substance use disorders It is important for the GP to be aware of the effects of self-administration of psychoactive substances, especially their toxic or withdrawal effects. They form a significant consideration for the differential diagnosis of disturbed patient behaviour. The following substances can cause these effects.

Alcohol Toxic and withdrawal effects, including delirium tremens, are outlined in CHAPTER 12 . Abrupt withdrawal can cause symptoms ranging from tremors, agitation and dysphoria (feeling thoroughly miserable) to fully developed delirium tremens. Epileptic seizures may also occur.

Barbiturate dependence Tolerance and symptoms on withdrawal are the main features. Barbiturate withdrawal is a very serious, life-threatening problem and may be encountered in elderly people undergoing longstanding hypnotic withdrawal. Symptoms include anxiety, tremor, extreme irritability, twitching, seizures and delirium.

Management1 Undertake withdrawal with medical supervision as an inpatient. Transfer the patient to phenobarbitone or diazepam. phenobarbitone 120 mg (o) hourly until sedation or phenobarbitone 30 mg for each 100 mg of shorter-acting barbiturate reduce the dose gradually over 10–14 days or diazepam 20–40 mg orally, daily reduce the dose gradually over 10–14 days

Benzodiazepine dependence

Withdrawal symptoms in the dependent patient include anxiety, restlessness, irritability, palpitation and muscle aches and pains, but delirium and seizures are uncommon except with very high doses. Refer to CHAPTER 70 for the adverse effects of benzodiazepines. The shorter the half-life, the greater the dependence. Withdrawal is best achieved by supervising a gradual reduction in dosage aided by relaxation techniques and behavioural strategies to help patients cope with insomnia and anxiety.

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Refer to CHAPTER 12 for the effects of opioid dependence, stimulant substance abuse, hallucinogen abuse and cannabis use and dependence.

Psychiatric disorders of childhood and adolescence1 The following disturbance problems do occur and must be taken seriously, especially the potential for suicide in the second decade. Many of these disorders are presented in more detail in CHAPTER 87 .

Attention deficit hyperactivity disorder Clinical features: short attention span distractibility overactivity impulsiveness antisocial behaviour

Depression Major depression follows the same criteria as for adults. Suicidal ideation has to be considered and taken very seriously if present. Imipramine is probably the drug of choice.

Bipolar disorders Mania is seldom diagnosed before puberty. Adolescents may present (uncommonly) with symptoms of mania or hypomania.

Schizophrenia and related disorders

Schizophrenia is rare before puberty. The criteria for diagnosis are similar to adults: delusion thought disorder hallucinations 6 months or more of deterioration in functioning

Autism Aggression and irritability can be a feature, especially during adolescence.

Tourette syndrome Behavioural problems can be part of this syndrome, which requires the attention of an experienced consultant.

Obsessive–compulsive disorders In about one-third of cases the onset is between 5 and 15 years of age.

When to refer21 Indications for referral to a psychiatrist: severe depression high suicide risk actual suicide attempt: recent or in the past suspected psychiatric disorders in the elderly: ?depression or schizophrenia; ?depression or dementia failure to improve with treatment poor family and social supports

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Anger management

Anxiety disorder Bipolar disorder Dementia Depression Schizophrenia

Resources Psychotropic guidelines [updated 2021]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2021. www.tg.org.au, accessed January 2021.

References 1

Psychotropic [updated 2021]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2021. www.tg.org.au, accessed April 2020.

2

Biro G. Dementia. Australian Doctor Weekly, 1990; 16 February: I–VIII.

3

Biro G. Delirium in the elderly. Australian Doctor Weekly, 1989; 1 December: I–VIII.

4

Kahn RL et al. Brief objective measures of the determination of mental status in the aged. Am J Psychiatry, 1960; 117: 326–9.

5

Kupfer DJ (Chair). Diagnostic and Statistical Manual of Mental Disorders (5th edn). Washington DC: American Psychiatric Publishing, 2013.

6

Needham NJ, Webb CE, Bryden DC. Postoperative cognitive dysfunction and dementia: what we need to know and do. British J Anaesthesia, December 2019; 119(Suppl. 1): i115–i125.

7

McLean S. Is it dementia? Aust Fam Physician, 1992; 21: 1762–6.

8

Keks N, Blashki G. The acutely psychotic patient: assessment and initial management. Aust Fam Physician, 2006; 35(3): 90–4.

9

Norman T, Judd F. Schizophrenia. In: MIMS Disease Index (2nd edn). Sydney: IMS Publishing, 1996: 455–7.

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10

Lovric K. Schizophrenia: update. Medical Observer, 17 September; 2004: 31–2.

11

Blashki G, Judd F, Piterman L. General Practice Psychiatry. Sydney: McGraw-Hill, 2007: 189–90.

12

Buckley N (Chair). Australian Medicines Handbook. Adelaide. Australian Medicines Handbook Pty Ltd, 2018: 839–44.

13

Hartling L et al. Antipsychotics in adults with schizophrenia: comparative effectiveness of first-generation versus second-generation medications: a systematic review and metaanalysis. Ann Intern Med, 2012; 157(7): 498–511.

14

McEvoy JP et al. Effectiveness of clozapine versus olanzapine, quetiapine, and risperidone in patients with chronic schizophrenia who did not respond to prior atypical antipsychotic treatment. Am J Psychiatry, 2006; 163(4): 600–10.

15

Leucht S et al. Antipsychotic drugs versus placebo for relapse prevention in schizophrenia: a systematic review and meta-analysis. Lancet, 2012; 379(9831): 2063–71.

16

Smith LA et al. Pharmacological intervention for acute bipolar mania: a systematic review of randomised placebo-controlled trials. Bipolar Disorders, 2007; 9(6): 551–60.

17

Sachs GS. A 25-year-old woman with bipolar disorder. JAMA, 2001; 285: 454–62.

18

Lovic K. Bipolar affective disorder: update. Medical Observer, 25 November 2005: 25–8.

19

Cipriani A et al. Comparative efficacy and acceptability of antimanic drugs in acute mania: a multiple-treatments meta-analysis. Lancet, 2011; 378(9799): 1306–15.

20

Geddes JP et al. Lithium plus valproate combination therapy versus monotherapy for relapse prevention in bipolar I disorder (BALANCE): a randomised open-label trial. Lancet, 2010; 375(9712): 385–95

21

Biro G. Suicide. Australian Doctor Weekly, 1991; 26 April: I–VIII.

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70 Anxiety disorders

Anxiety is a thin stream of fear trickling through the mind. If encouraged, it cuts a channel into which all other thoughts are drained. ARTHUR SOMERS ROCHE (1883–1935) Anxiety is a state of nervousness or uneasiness in response to an actual or perceived stressor. It has three essential components: psychological (feelings, e.g. frightened),1 somatic (physical, e.g. palpitations) and cognitive (thoughts, e.g. loss of control). Anxiety is a normal human physiological response that helps us respond to potential threats or dangers. We cannot get by without it. With an increase in anxiety, our performance should increase accordingly (e.g. preparing for exams). When we have too much anxiety, however, a further increase in anxiety will lead to our performance dropping off, described by the Yerkes–Dodson curve (see FIG. 70.1 ). The optimal place to be on the curve is to the left of the peak, so we have a positive response in our performance to an increase in stress. Being at the peak or to the right will make us vulnerable to stress.

FIGURE 70.1 Yerkes−Dodson curve Anxiety becomes a problem when the stressor overwhelms us, resulting in poor performance, or if the unwanted consequences of the anxiety response give us undesirable physiological consequences. The stressor causes limbic activation, which in turn leads to autonomic and neuro–endocrine activity (down-regulation) that causes various physiological responses, including activation of the hypothalamic–pituitary axis. Blood flows from the gut to the skeletal muscles, smooth muscle contracts in the bowels and increases nausea, muscle tension increases, pupils dilate, the heart rate increases and blood pressure rises. These changes account for various somatic symptoms commonly seen in anxiety disorders (e.g. tension headaches, palpitations, eye strain and irritable bowel-like symptoms). In high stress situations, a flight–fright–freeze response can ensue. The physiological role of anxiety and the way in which excess anxiety can cause these physical symptoms can be explained to patients suffering from anxiety disorders—and the GP is very well placed to conduct such psycho-education. Patients suffering from somatic symptoms are often unaware of the physiological consequences of anxiety and usually find this clarification comforting. It can help motivate patients to pursue strategies to reduce them.

Prevalence and classification of anxiety Anxiety disorders affect 14% of the population,2 with many people fulfilling the criteria for multiple anxiety disorders and/or a common codiagnosis of depression.3 The criteria for defining anxiety, as for many mental health disorders, have broadened over time, so it is difficult to compare historic prevalence figures. Specific phobia is the most commonly reported anxietyrelated diagnosis (1 in 5 women and 1 in 10 men), and PTSD is the most common disorder (over 6%).2 Anxiety disorders listed in the DSM-5 are:4 separation anxiety disorder selective mutism specific phobia social anxiety disorder (social phobia) panic disorder panic attack (specifier) agoraphobia generalised anxiety disorder substance/medication-induced anxiety disorder

illness anxiety disorder anxiety due to another medical condition other specified anxiety disorder unspecified anxiety disorder Other conditions discussed in this chapter include:

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obsessive–compulsive disorder body dysmorphic disorder (see CHAPTER 69

)

post-traumatic stress disorder acute stress disorder adjustment disorder with anxious mood somatic symptom disorder

Generalised anxiety disorder Generalised anxiety comprises excessive anxiety and worry about various life circumstances and is not related to a specific activity, time or event such as trauma, obsessions or phobias. It affects up to 5% of the population. There is an overlap between generalised anxiety disorder (GAD) and other anxiety disorders. General features: persistent unrealistic and excessive anxiety worry about a number of life circumstances for 6 months or longer

Diagnostic criteria for generalised anxiety disorder Three or more of: restless, ‘keyed up’ or ‘on edge’ easily fatigued difficulty concentrating or ‘mind going blank’ irritability

muscle tension sleep disturbance

Clinical features Psychological Apprehension/fearful anticipation (even of dying) Irritability Exaggerated startle response Sleep disturbance and nightmares Impatience Panic Sensitivity to noise Difficulty concentrating or ‘mind going blank’ Physical Motor tension: muscle tension/aching tension headache trembling/shaky/twitching restlessness tiredness/fatigue Autonomic overactivity: dry mouth palpitations/tachycardia sweating/cold, clammy hands flushes/chills

difficulty swallowing or ‘lump in throat’ diarrhoea/abdominal distress frequency of micturition difficulty breathing/smothering feeling dizziness or lightheadedness Symptoms and signs according to systems Neurological: dizziness, headache, trembling, twitching, shaking, paraesthesia Cardiovascular: palpitations, tachycardia, flushing, chest discomfort Gastrointestinal: nausea, indigestion, diarrhoea, abdominal distress Respiratory: hyperventilation, breathing difficulty, air hunger Cognitive: fear of dying, difficulty concentrating, ‘mind going blank’, hypervigilance

Diagnosis of generalised anxiety disorder The diagnosis is based on: history—it is vital to listen carefully to what the patient is saying exclusion of organic disorders simulating anxiety by history, examination and appropriate investigation, e.g. TFTs, ECG, CXR, drug screen exclusion of other psychiatric disorders, especially depression and adjustment disorder with anxious mood (comorbidity is a feature of anxiety) Note: Anxiety and major depression often coexist. Main differential diagnoses (note that this conforms to the seven masquerades list): depression substance abuse—alcohol or drug (including benzodiazepine) dependence/withdrawal hyperthyroidism angina and cardiac arrhythmias iatrogenic drugs caffeine intoxication

These organic (medical) causes need to be ruled out.

Important checkpoints These self-posed questions should be considered by the family doctor before treating an anxious patient:

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Is the anxiety primary or secondary? Is this hyperthyroidism? Is this depression? Is this normal anxiety? Is this mild anxiety or simple phobia? Is this moderate or severe anxiety? Is this an adjustment disorder?

Management The management applies mainly to generalised anxiety, as specific psychotherapy is required in other types of anxiety. Much of the management can be carried out successfully by the family doctor using brief counselling and support. Cognitive behaviour therapy (CBT), in which maladaptive thinking, feelings, perceptions and related behaviours are identified, assessed, challenged and modified, can be of considerable benefit.5 Exercise, both low or high intensity, has been shown to decrease anxiety symptoms. Hence psychological therapy and non-drug strategies are first-line therapy for most anxiety disorders.6 Table 70.1

Significant differential diagnoses of anxiety

Psychiatric disorders Depression Drug and alcohol dependence/withdrawal Benzodiazepine dependence/withdrawal Personality disorder, e.g. borderline Schizophrenia

Acute or chronic organic brain disorder Early dementia Organic disorders Drug-related: amphetamines bronchodilators caffeine excess ephedrine/pseudoephedrine levodopa thyroxine Cardiovascular: angina cardiac arrhythmias mitral valve prolapse Endocrine: hyperthyroidism phaeochromocytoma carcinoid syndrome hypoglycaemia insulinoma Neurological: epilepsy, especially complex partial seizures acute brain syndrome Respiratory: asthma acute respiratory distress pulmonary embolism

Principles of management6,7 Psychological interventions (e.g. ‘life coaching’ and CBT) are first line. Give careful explanation and reassurance: explain the reasons for the symptoms

be aware that patients often ‘worry about worrying’ (e.g. that anxiety is dangerous, that they are going crazy or ‘losing it’)7 reassure the patient about the absence of organic disease (can only be based on a thorough examination and appropriate investigations) direct the patient to appropriate resources to give insight and support Provide practical advice on ways of dealing with the problems. Advise on the avoidance of aggravating substances such as caffeine, nicotine and other drugs. Advise on general measures such as stress management techniques, relaxation programs, mindfulness and regular exercise and if possible organise these for the patient (don’t leave it to the patient). Advise on coping skills, including personal and interpersonal strategies, to manage difficult circumstances and people (in relation to that patient). Provide ongoing supportive psychotherapy. TABLES 70.2

and 70.3

Table 70.2

list a number of good sources of reliable information about anxiety.8

Australian websites providing information about anxiety

Provider and hosting organisation Beyond Blue, The National Depression Initiative

Website www.beyondblue.org.au/home www.youthbeyondblue.com (for young people aged 12–25 years)

Black Dog Institute, Prince of Wales Hospital, University of New South Wales

www.blackdoginstitute.org.au www.biteback.org.au (for young people aged 12–18 years)

Centre for Clinical Interventions, Government of Western Australia, Department of Health

www.cci.health.wa.gov.au

Clinical Research Unit for Anxiety and Depression (CRUfAD), St Vincent’s Hospital, Sydney

www.crufad.org

ReachOut Australia

http://au.reachout.com (for young people aged 14–25 years)

years)

Table 70.3

Openly accessible Australian-based interactive internet programs for anxiety

Organisation and program name CRUfAD clinical programs

Website www.crufad.org*

e-hub Australian National University, MoodGYM

www.moodgym.anu.edu.au

*in-house clinical support is supplied, but costs apply

Pharmacological treatment9,10 The key principles of using medication for anxiety disorders are: Non-pharmacological management is first line for a reason. Do not rush into using medication and repeatedly reassess whether to continue prescribing. Of the medications, SSRIs are regarded as first line and other antidepressants such as SNRIs, e.g. duloxetine and venlafaxine, and the tetracyclic mirtazapine have shown some benefit in anxiety disorders (see TABLES 70.4 and 70.5 ), but their benefits are not as long-lasting as psychological and behavioural approaches.11,12 Assess efficacy of antidepressants after at least 12 weeks (in contrast to 6–8 weeks when treating major depression) and, if of clear benefit, treat for at least 6 months.

Page 844

The cessation of an SSRI commonly causes withdrawal symptoms. A common trap (for doctor and/or patient) is to interpret these symptoms as a recurrence of the underlying anxiety and as evidence that the drug continues to be required. Propranolol is of benefit in social anxiety disorder, particularly with anticipated stressful events (e.g. public speaking, presenting at work events). Benzodiazepines have a limited role in anxiety disorders and should not be recommended. If used, they should be reserved for people who have not responded to at least 2 therapies (e.g. psychological therapy and antidepressant) and used only in the short term (stop within 6 weeks), e.g. diazepam 2–5 mg (o), as a single dose. They can also be used for specific phobias (e.g. fear of flying, agoraphobia and MRI machines). Consider buspirone, which has negligible potential for tolerance or dependence.

Table 70.4

Dosage recommendations for SSRIs in anxiety disorders11

Drug citalopram

Initial dose Maximum dose 10 mg 40 mg

escitalopram

5 mg

20 mg

fluoxetine

10 mg

80 mg

fluvoxamine

50 mg

300 mg (split dose to bd over 150 mg)

paroxetine

10 mg

60 mg

sertraline

25 mg

200 mg

Table 70.5

Initial medication options for anxiety disorders11

Condition Generalised anxiety disorder

Medication options SSRI, duloxetine (30–120 mg), venlafaxine controlled release (75–225 mg)

Panic attack

none, psychological intervention

Panic disorder

SSRI, venlafaxine controlled release (75–225 mg)

Obsessive– compulsive disorder

SSRI

Agoraphobia (without panic)

none

Social anxiety disorder (generalised)

SSRI, venlafaxine controlled release (75–225 mg)

Social anxiety disorder (nongeneralised)

propranolol 10–40 mg orally, 30–60 minutes before the social event or performance

Specific phobias

not recommended

Post-traumatic stress disorder

SSRI, mirtazapine

Note: Not every SSRI has Australian TGA approval for use for each anxiety disorder; the TGA website can provide up-to-date information on this

changing landscape. There is insufficient evidence to differentiate between efficacy of the individual SSRIs.

Panic attack9 Page 845 A panic attack is defined4 as a discrete period of intense fear or discomfort in which four (or more) of the following symptoms develop abruptly and reach a peak within 10 minutes:

shortness of breath or smothering sensations dizziness, unsteady feelings, lightheadedness or faintness palpitations or accelerated heart rate trembling or shaking sweating feeling of choking nausea or abdominal distress depersonalisation or derealisation numbness or tingling sensations (paraesthesia) flushes (hot flashes) or chills chest pain or discomfort fear of dying fear of going crazy or of doing something uncontrolled the 3 Cs—chest pain, chills, choking Organic disorders that simulate a panic attack are hyperthyroidism, phaeochromocytoma and hypoglycaemia. Note: A single panic attack is not synonymous with panic disorder, which is characterised by recurrent panic attacks. Some 40% of young people have had at least one spontaneous panic attack. Panic disorder, which affects 2-3% of the population, is when there are recurrent attacks that are followed by at least a month of worrying about future attacks and/or the consequences of them. Panic disorder can occur with or without associated agoraphobia, though >90% of people with agoraphobia develop it as a result of recurrent panic attacks.11

Management Reassurance, explanation and support (as for generalised anxiety). This is the mainstay of

treatment. A patient who is experiencing a panic attack should be taught breathing techniques to help control hyperventilation (e.g. timing breaths, breathing through nose, slow inspiration, measured medium-sized breaths). Relaxation techniques can also be employed, such as progressive muscle relaxation, and patients can teach themselves these techniques via online resources (see TABLES 70.2 and 70.3 ). Rebreathing into a paper bag is rarely indicated in a 10,11 general practice setting as the hyperventilation has usually settled by the time the patient presents. The above breathing techniques can be used by the patient anywhere and are more socially acceptable than breathing noisily into a paper bag when an attack is feared. The danger in a panic attack is the danger to the self by the self 9 (e.g. fleeing into danger, nonintentional overdose). Cognitive behaviour therapy (CBT) (See CHAPTER 4

.)

CBT aims to reduce anxiety by teaching patients how to identify, evaluate, control and modify their negative, fearful thoughts and behaviour. If simple psychotherapy and stress management fail, then patients should be referred for CBT, usually to a psychologist (or occasionally psychiatrist), although some GPs have a particular interest in providing CBT. Patients’ fears, especially if irrational, need to be clearly explained by the therapist, examined rationally and challenged, then replaced by positive calming thoughts. Pharmacological treatment Pharmacological treatment is rarely of benefit in the acute attack, as the attacks occur too quickly for their effect to be of use. For ongoing treatment of panic disorder with or without agoraphobia, there is little good quality evidence comparing medication to CBT.10 Continual use of benzodiazepines (BDZs, e.g. alprazolam or clonazepam) has previously been utilised in panic and other anxiety disorders but is now no longer recommended.11 Problems associated with benzodiazepine use include: impaired alertness, oversedation dependence increased risk of accidents adverse effects on mood and behaviour interaction with alcohol and other drugs potential for abuse and overdose risks during pregnancy and lactation

muscle weakness sexual dysfunction diminished motivation lowered sense of competency lowered self-esteem Some principles of using BDZs in anxiety disorders11 are: always check for a history of problem alcohol or drug use be wary of prescribing to unfamiliar patients, especially if asking for a particular drug by name (may indicate drug-seeking behaviour) carefully discuss the potential for addiction with the patient avoid using short-acting drugs as they are the most highly addictive

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prescribe only small quantities of medication at a time use only as short-term treatment ensure regular review of the patient and continuity of care If already being used, BDZs should be tapered very slowly (this may take 6–12 months or longer). A benzodiazepine withdrawal syndrome, which can include rebound anxiety, depression, confusion, insomnia and seizures, may occur (see CHAPTER 69 ). However, the doctor’s fear of being responsible for a withdrawal syndrome has also been used as leverage by those seeking drugs. If in doubt, seek specialist drug and alcohol advice.

Phobic disorders In phobic states, the anxiety is related to specific situations or objects out of proportion to the apparent stimulus. Phobic disorders include agoraphobia, social anxiety disorder (otherwise known as social phobia) and specific phobias. Patients avoid these objects or situations, become anxious when they anticipate having to meet them and/or endure them with intense distress. Common phobias are spiders, people and social situations, flying, open spaces, confined spaces, heights, cancer, thunderstorms, death and heart disease. The problem is seldom encountered in practice and there is usually no call for drug therapy.

Agoraphobia Avoidance includes staying away from many situations where there are issues of distance from home, crowding or confinement. Typical examples are travel on public transport, crowded shops

and confined places. Patients fear they may lose control, faint and suffer embarrassment.

Social anxiety disorder Social anxiety disorder (social phobia) is experienced in anxiety-provoking social situations in which the person feels subject to critical public scrutiny (e.g. canteens, restaurants, staff meetings, speaking engagements). It can either be generalised (fear of numerous social situations, including both performance and interactional situations) or non-generalised (fear of one or just a few situations or performance type). The treatments for the two subtypes are quite different (see TABLE 70.5 ). The sufferer may be a shy, self-conscious, premorbid personality. Social phobias, including performance anxiety and symptoms, are often related to sympathetic overactivity.

Management The basis of treatment for all phobias is psychotherapy that involves behaviour therapy (e.g. graduated exposure therapy) and cognitive therapy.

Illness anxiety disorder Also referred to as hypochondriasis or health disorder, it implies worrying excessively about being or becoming seriously ill despite no actual physical symptoms. Treatment is counselling through stress management, including relaxation techniques.

Obsessive–compulsive disorder (OCD) Anxiety is associated with obsessive thoughts and compulsive rituals. The obsessions are recurrent and persistent intrusive ideas, thoughts, impulses or images that are usually resisted by the patient. Compulsions are repetitive, purposeful and intentional behaviours conducted in response to an obsession to prevent a bad outcome for the person (e.g. excessive washing of the genitals). Mild obsessional or compulsive behaviour can be regarded as normal in response to stress.

Management Optimal management is a combination of psychotherapeutic—particularly CBT—and pharmacological treatment, namely: cognitive behaviour therapy for obsessions exposure and response prevention for compulsions an SSRI is first line if pharmacological required

Body dysmorphic disorder The person with this disorder has an exaggerated preoccupation with an imagined defect in appearance (see CHAPTER 69 ). Patients may be helped by counselling and psychotherapy.

Post-traumatic stress disorder (PTSD) Page 847 PTSD is defined somewhat differently, in terms of time lapses from the traumatic event. It refers to a similar constellation of symptoms that persist for more than 1 month after exposure:

acute PTSD: duration of symptoms 2 per month Symptoms regularly with exercise

≥80%

SABA prn

500 mcg fluticasone >320 mcg ciclesonide

tightness on

ciclesonide

waking Limitation of physical activity

If moderately severe inflammatory airways disease is not treated (with inhaled corticosteroids) there is the risk of fixed irreversible airways obstruction from submucosal fibrosis. Choose initial treatment appropriate to the above. Avoid the use of LABA on its own (i.e. without ICS), as it is associated with an increased risk of asthma death. Document evidence in notes. Identify management goals in collaboration. Provide clear written patient information. Educate and review regularly. Goals of management: absent or minimal daytime symptoms and no nocturnal symptoms; restore normal airway function (>80% of predicted) maintain best possible lung function at all times—keep asthma under control reduce morbidity control asthma with the use of regular anti-inflammatory medication and relieving doses of β2agonist when necessary Long-term goals: achieve use of the least drugs, least doses and least side effects reduce risk of fatal attacks reduce risk of developing irreversible abnormal lung function

Good asthma control Minimal symptoms day and night

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No nocturnal waking due to asthma No limitation of normal activity or exercise Minimal need for reliever medication No exacerbations Normal or near-normal lung function (FEV1 and/or PEFR >80% of predicted or best) No side effects of medication

Questions to assess asthma control1 Ask about: limitation of daily activities shortness of breath sleep disturbance use of reliever medication perceived level of asthma control

Pharmacological agents to treat asthma Simple classification Reliever = bronchodilator Preventer = anti-inflammatory Symptom controller = long-acting β2-agonist (LABA)

It is useful to teach patients the concept of the ‘preventer’ and the ‘reliever’ for their asthma treatment. The pharmacological treatment of asthma is summarised in TABLE 73.3 .

8

ogical treatment of asthma

Generic types

Examples

Salbutamol

Ventolin

Salmeterol

Serevent

Terbutaline

Bricanyl

Eformoterol

Foradile, Oxis

Indacaterol

Onbrez

Vehicle of administration Aerosol Dry Nebulising (metered Oral powder Injection solution dose (inhalation) inhalation) ✓

✓

✓

✓

✓

✓

✓

✓

✓

✓

✓

✓

✓

Adrenaline Ipratropium bromide

Atrovent

Theophylline

Nuelin

✓

✓ ✓ ✓

Aminophylline ✓

✓ ✓

✓

Sodium cromoglycate

Intal

Nedocromil sodium

Tilade

✓

Beclomethasone

QVAR

✓

✓

Budesonide

Pulmicort

✓

✓

Ciclesonide

Alvesco

Fluticasone

Flixotide

✓

✓ ✓

✓

✓

(Seretide)

✓

✓

e (Flutiform)

✓

de (Symbicort)

✓

ABA) + preventer (ICS)—fixed combination MDI

✓ ✓

Breo ellipta) ✓

‘Preventer’ drugs: anti-inflammatory agents8 These medications are directed towards the underlying abnormalities—bronchial hyper-reactivity and associated airway inflammation. Treatment with a ‘preventer’ is recommended if asthma episodes are >3/week or those who use SABA >3 times a week.

Corticosteroids Inhaled (ICS) Types: beclomethasone budesonide ciclesonide (single daily dose) fluticasone Dose range: microgram ranges differ according to drug; aim for lowest effective dose Availability: MDI Turbuhaler Autohaler Accuhaler Frequency: once or twice daily (adherence may be greater with once daily) Side effects: oropharyngeal candidiasis, dysphonia (hoarse voice)—less risk with once daily ciclesonide bronchial irritation: cough adrenal suppression (doses of 2000 mcg/daily; sometimes as low as 800 mcg)

Note: Rinse mouth with water and spit out after using inhaled steroids. ICSS have a flat dose–response curve so there are diminishing returns in prescribing above beclomethasone or budesonide 1000 mcg/day or fluticasone 500 mcg/day. For newly diagnosed patients with mild-to-moderate asthma ‘start low and step up prn’ (e.g. 250–400 mcg/day).6 Step down the dose when safe to do so. Note: Most adults and older adolescents with asthma should be on long-term inhaled corticosteroid therapy.1 Oral Prednisolone is used mainly for exacerbations. It is given with the usual inhaled corticosteroids and bronchodilators. Dose: up to 1 mg/kg/day (usual max. 50 mg) for 3 days to 2 weeks Side effects: these are minimal if drug is used for short periods long-term use has significant side effects: osteoporosis, glucose intolerance, adrenal suppression, thinning of skin and easy bruising Note: Short-term oral corticosteroids can be ceased abruptly without tapering. Clinical trials are tending to favour shorter courses.

Cromones These are sodium cromoglycate (SCG) and nedocromil sodium. SCG is available as dry Page 873 capsules for inhalation, metered dose aerosols and a nebuliser solution. They are often inhaled via a spacer in children. Adverse effects are uncommon; local irritation may be caused by the dry powder. Systemic effects do not occur. Nedocromil is used for frequent episodic asthma in children over 2 years of age for the prevention of exercise-induced asthma and the treatment of mild-to-moderate asthma in some adults. The initial dose is 2 inhalations qid. Adverse effects are uncommon.

Leukotriene antagonists Page 874 These drugs (in Australia, primarily montelukast) are very useful for seasonal asthma and aspirin-sensitive asthma and reduce the need for inhaled steroids or offer an alternative for those who cannot tolerate ICSS or have trouble using an inhaler. Favourable evidence is based on a small number of trials only, mostly in children but some adults benefit.9 Montelukast is taken as a 5 or 10 mg chewable tablet once daily.

Indications for preventive therapy10 Guidelines for introducing preventive asthma therapy in adults and children include any of the following: requirement of β2-agonist >2 days per week or >1 canister every 3 months (excluding preexercise) symptoms (non-exercise) >2 times per week between attacks any symptoms during the night or on waking spirometry showing reversible airflow obstruction during asymptomatic phases asthma significantly interfering with physical activity despite appropriate pre-treatment asthma attacks ≥twice per month infrequent asthma attacks but severe or life-threatening

‘Reliever’ drugs or bronchodilators The three groups of bronchodilators are: the β2-adrenoceptor agonists (β2-agonists)—short acting (SABA) and long acting (LABA) methylxanthines—theophylline derivatives anticholinergics

β2-agonists These drugs stimulate the β2 adrenoreceptors and thus relax bronchial smooth muscle. Inhalation is the preferred route of delivery; the vehicles of administration include metered dose inhalation, a dry powder, and nebulisation where the solution is converted to a mist of small droplets by a flow of oxygen or air through the solution. Oral administration of β2-agonists is not recommended. The inhaled drugs produce measurable bronchodilation in 1–2 minutes and peak effects by 10–20 minutes. The traditional agents such as salbutamol and terbutaline are short-acting preparations. The new longer-acting agents (LABA) include salmeterol, eformoterol and vilanterol. LABAs should always be used in combination with an ICS, not as monotherapy.11

Theophylline derivatives

These oral drugs may have complementary value to the inhaled agents but tend to be limited by side effects and efficacy.

Anti-IgE monoclonal antibodies These newer agents (e.g. omalizumab) bind IgE without activating mast cells. They are directed for use in patients >12 years of age with moderate to severe allergic eosinophilic asthma who have been treated by ICS and who have raised serum IgE levels. They are given by SC injection and the PBS stipulates specialist initiation.

Antibiotic use for chronic asthma12 Antibiotics are not recommended apart from clinical evidence of super-respiratory infection. Trials of daily oral azithromycin have provided weak evidence of possible benefit in reducing exacerbations of asthma and COPD, but this has not been recommended in clinical practice.13

Starting treatment Current treatment supports the initial treatment (summarised in TABLE 73.2 ) of a SABA with low to moderate doses of ICS with estimated equivalent doses shown in the table. Initiate therapy sufficient to achieve best lung function promptly. Wean inhaled corticosteroids to the minimum dose needed to maintain adequate asthma control.

Prophylactic agents This term is reserved for those medications that are taken prior to known trigger factors, particularly for exercise-induced asthma.

Exercise-induced asthma (options) β2-agonist inhaler (puffer): two puffs 5 minutes immediately before exercise last 1–2 hours. LABA such as salmeterol and eformoterol are more effective if used with ICS. SCG or nedocromil, two puffs Combination β2-agonist + SCG (5–10 minutes beforehand) Montelukast 10 mg (less in children ≥2 years) oral daily or 1–2 hours beforehand Paediatricians often recommend a non-drug warm-up program as an alternative to medication.

Ongoing management The three-step asthma control plan14,15

The National Asthma Council of Australia has developed the following follow-up plan, summarised in FIGURE 73.3 .

FIGURE 73.3 Asthma: ongoing management steps Source: Reproduced with permission from NPS Medicine Wise

Step 1: Assess asthma symptom control and identify the patient’s risk factors. Assess asthma symptom control over the previous 4 weeks. Assess the patient’s risk factors. Exclude factors contributing to poor control before intensifying preventer treatment: check adherence check inhaler technique check inhaler device is appropriate consider that symptoms may be due to alternative or comorbid diagnoses Step 2: Treat and adjust to achieve good control. All patients should have a reliever inhaler for as-needed use.

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Most can achieve well-controlled asthma with low-dose ICS. Trial low-dose ICS before ICS/LABA fixed combination therapy.16 Reserve ICS/LABA as a later option. This combination is too readily used in Australia. Where appropriate, step down treatment. Schedule follow-up visit. See TABLE 73.3

.

Step 3: Review response and monitor to maintain control. Review diagnosis and treatment regularly. Monitor to maintain control. A general management plan for chronic asthma is summarised in FIGURE 73.4

.

FIGURE 73.4 Stepped approach to adjusting asthma medication in adults This figure comes from the National Asthma Foundation Australia and is based on Australian Treatment Standards and medications available in Australia and not designed or intended for international use.

Practice tips For breakthrough asthma or persistent poorly controlled asthma with poor compliance switch to combined medication (e.g. Seretide MDI Accuhaler or Symbicort).

Correct use of the asthma MDI (puffer) Did you know that:

faulty inhaler technique occurs in at least one-third of users? in faulty technique, up to 90% of the medication sticks to the mouth and does not reach the lungs? it is the inhalation effort—not the pressure from the aerosol—that gets the medication to the lungs? it is important to instruct patients properly and check their technique regularly?

The two main techniques The open-mouth technique and the closed-mouth technique are the main methods, and both are effective but the closed-mouth technique is preferred. Both techniques are suitable for most adults. Most children from the age of 7 can learn to use puffers quite well. The closed-mouth technique See FIGURE 73.5

.

FIGURE 73.5 Using the metered dose inhaler: the closed-mouth technique Instructions for patients: 1. Remove the cap. Shake the puffer vigorously for 1–2 seconds. Hold it upright (canister on

top). 2. Place the mouthpiece between your teeth (do not bite it) and close your lips around it. 3. Breathe out slowly and gently to a comfortable level.

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4. Tilt your head back slightly with your chin up. 5. Just as you then start to breathe in (slowly) through your mouth, press the puffer firmly, once. Breathe in as far as you can over 3–5 seconds. (Do not breathe in through your nose.) 6. Remove the puffer from your mouth and hold your breath for about 10 seconds; then breathe out gently. 7. Breathe normally and then repeat the inhalation if you need to. Extra points The usual dose of standard MDI is one or two puffs (adult) every 3–4 hours for an attack (four puffs in children). If you do not get adequate relief from your normal dose, contact your doctor. It is quite safe to increase the dose, such as to 4–6 puffs. If you are using your inhaler very often, it usually means your other asthma medication is not being used properly.

Autohaler The Autohaler is a breath-activated MDI which can improve lung deposition in patients with poor inhaler technique.

Turbuhaler The Turbuhaler is a dry powder delivery system that is widely used as an alternative to the MDI. It is a breath-activated device. Other dry powder devices are the Accuhaler and Diskhaler.

Spacers versus nebulisers Both MDIs via a spacer and dry powder inhalers are at least as effective as a nebuliser for treating acute exacerbations in both adults and children.17 They are considerably cheaper and more readily available than an electrically powered device.

Summary of devices Breath-activated MDIs: Autohaler Breath-activated dry powder inhalers: Accuhaler, Aerolizer, Diskhaler, Rotahaler, Spinhaler, Turbuhaler Large volume spacer: Nebuhaler, Volumatic Small volume spacer: Aerochamber, Breath-A-Tech

Dangerous asthma Failure to recognise the development of a severe attack has cost the lives of many asthmatics. A severe attack can start suddenly (even in mild asthmatics) and catch people by surprise.

High-risk patients People who have experienced one or more of the following are more likely to have severe attacks: previous severe asthma attack previous hospital admission, especially admission to intensive care hospital attendance in the past 12 months long-term oral steroid treatment carelessness with taking medication night-time attacks, especially with severe chest tightness recent emotional problems frequent SABA use Early warning signs of a severe asthma attack: symptoms persisting or getting worse despite adequate medication increased coughing and chest tightness poor response to two inhalations benefit from inhalations not lasting 2 hours

increasing medication requirements sleep being disturbed by coughing, wheezing or breathlessness chest tightness on waking in the morning low PEFR readings

Thunderstorm asthma18 A likely mechanism is that this occurs when the high winds preceding a storm whip up Page 877 massive quantities of rye grass pollens which absorb moisture and rupture, releasing allergens. People most vulnerable to an asthma attack are those with a history of allergy and poorly controlled asthma in particular. When an attack is imminent, at-risk people should stay inside with windows and doors closed, take preventer medication, follow action plans and ensure reliever drugs are readily available. Treatment is with a salbutamol spray followed by corticosteroids. Seasonal allergic rhinitis patients not on constant intranasal corticosteroids (INCS) should start taking these before the pollen season and continue until pollen levels abate. For those with asthma include a combination of an INCS and intranasal antihistamine if symptoms are severe or not controlled by INCS alone.1

Dangerous signs Marked breathlessness, especially at rest Sleep being greatly disturbed by asthma Asthma getting worse quickly rather than slowly, despite medication Feeling frightened Difficulty in speaking; unable to say more than a few words Pulsus paradoxus Exhaustion and sleep deprivation Drowsiness or confusion Chest becoming ‘silent’ with a quiet wheeze, yet breathing still laboured Cyanosis Chest retraction Respiratory rate greater than 25 (adults) or 50 (children)

Pulse rate >120 beats/min Peak flow 6 yo) Add: ipratropium bromide (nebuliser) oral prednisolone (when required)

When to refer If you are doubtful about the diagnosis For advice on management when asthmatic control has failed or is difficult to achieve

Practice tips

Reassure the patient that 6–10 inhaled doses of a β2-agonist is safe and appropriate for a severe attack of asthma. It is important to achieve a balance between undertreatment and overtreatment. Beware of patients, especially children, manipulating their peak flow meter results. Get patients to rinse out their mouth with water and spit it out after inhaling corticosteroids. Patients who are sensitive to aspirin/salicylates need to be reminded that salicylates are present in common cold cure preparations and agents such as Alka-Seltzer.2 Aspirin-sensitive asthma usually manifests late in life with associated rhinitis. It cross-sensitises with NSAIDs. Possible side effects of inhaled drugs can be reduced by always using a spacer with the inhaler, using the medication qid rather than bd, rinsing the mouth, gargling and spitting out after use, and using corticosteroid-sparing medications.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Asthma Asthma: correct use of your aerosol inhaler Asthma: dangerous asthma

References 1

National Asthma Council of Australia. Australian Asthma Handbook (Version 10). Melbourne, 2014: updated 2017. Available from: www.asthmahandbook.org.au.

2

Seale JP. Asthma. In: MIMS Disease Index. Sydney: IMS Publishing, 1991–92: 59–65.

3

Australian Bureau of Statistics. Causes of death data: Asthma deaths (customised report), 2019. Available from: https://www.nationalasthma.org.au/living-withasthma/resources/health-professionals/reports-and-statistics/asthma-mortality-statistics, accessed April 2021. Australian Bureau of Statistics. Prevalence of asthma, by age and sex, 2014–15. Available

4

from: www.aihw.gov.au/reports/asthma-other-chronic-respiratory-conditions/asthma/data, accessed March 2018.

5

Global strategy for asthma management and preventing: global initiative for asthma (GINA). Updated 2009. Available from: www.ginasthma.com.

6

Rees J, Price J. ABC of Asthma (2nd edn). London: BMJ Publishing Group, 1989: 1–34.

7

National Asthma Council Australia. Australian Asthma Handbook. Use and care of spacers. Available from: www.asthmahandbook.org.au/management/devices/spacers, accessed February 2021.

8

Asthma [published 2020]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2020. www.tg.org.au, accessed February 2021.

9

Ducharme F, Hicks GC. Anti-leukotriene agents compared to inhaled corticosteroids in the management of recurrent and/or chronic asthma. In: The Cochrane Library, Issue 1, 2002. Oxford: Update Software.

10

Improve asthma control with six-step management plan. NPS News, 2002; 23: 1–6.

11

Worsnop C. Combination inhalers for asthma. Australian Prescriber, 2005; 28: 26–8.

12

Gibson PG et al. Efficacy of azithromycin on asthma exacerbations and quality of life in adults with persistent uncontrolled asthma (AMAZES): a randomised, double-blind, placebo-controlled trial. Lancet, 12 Aug 2017; 390(10095): 659–68.

13

McMullan BJ, Mostaghim M. Prescribing azithromycin. Australian Prescriber, 2015; 38: 87–9.

14

Global strategy for asthma management and prevention. Bethesda MD: Global Initiative for Asthma, 2012.

15

Asthma: steps to control. NPS Medicine Wise, April 2014: 1–6.

16

Ni Chroinin M et al. Addition of inhaled long-acting beta2-agonists to inhaled steroids as first line therapy for persistent asthma in steroid-naive adults and children. Cochrane Database Syst Rev, 2009; October: CD005307.

17

Cates CJ et al. Holding chambers versus nebulisers for beta-agonist treatment of acute asthma. In: The Cochrane Library, Issue 2, 2002. Oxford: Update Software.

18

Lindstrom SJ et al. Thunderstorm outbreak of November 2016: a natural disaster requiring planning. MJA, 18 September 2017; 207(6): 235–7.

19

Gibson PE et al. Barriers to improved asthma outcomes. Cochrane Database Syst Rev, 2003; July: CD001117.

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74 Chronic obstructive pulmonary disease

Tobacco drieth the brain, dimmeth the sight, vitiateth the smell, hurteth the stomach, destroyeth the concoction, disturbeth the humors and spirits, corrupteth the breath, induceth a trembling of the limbs, exsiccateth the windpipe, lungs and liver, annoyeth the milt, scorcheth the heart, and causeth the blood to be adusted. TOBIAS VENNER (1577–1660), VIA RECTA AD VITAM LONGAM Chronic obstructive pulmonary disease (COPD) is described by the Global Initiative for Chronic Obstructive Lung Disease (GOLD) as a: common, preventable and treatable disease characterised by non-fully reversible persistent airflow limitation that is usually progressive and associated with an enhanced chronic inflammatory response in the airways and the lung to noxious particles or gases. It is basically a structural disease. Exacerbations and comorbidities contribute to the overall severity in individual patients.1 COPD typically affects middle-aged and older people with the usual age of onset in the fifth and sixth decades. It is the fourth leading cause of death and the third leading burden of disease in Australia, affecting 12.4% of Australians between 45 and 70 years. Early diagnosis and treatment is important to outcome and the provisional diagnosis should be based on the clinical features of breathlessness and cough in a smoker or ex-smoker. Cigarette smoking is undoubtedly the major cause of both chronic bronchitis and emphysema, although only 10–15% of smokers develop the diseases.2 Chronic bronchitis is defined as >3 episodes per year for 2 years while emphysema is destruction of alveoli. The clinical differences between asthma and bronchitis are summarised in CHAPTER 38 . Patients experiencing overlap of asthma and COPD should be identified and treated differently to patients with either condition alone. Lifelong passive smoking exposure increases COPD risk by 2.2 to 4.0 times. FIGURE 74.1

illustrates the influence of smoking on lung function.

FIGURE 74.1 Clinical trajectory of a person who quits smoking

Factors in causation Cigarette smoking (usually 20/day for 20 years or more)4 Natural fuel—wood, twigs, crop residue Air pollution (outdoor and indoor) Airway infection/chronic bronchitis Occupation: related to cadmium, silica, dusts Familial factors: genetic predisposition Alpha1-antitrypsin deficiency (emphysema) Bronchial hyper-responsiveness

Diagnosis and management of COPD Page 882 The COPDX Plan guidelines5 developed by the Australian Lung Foundation and the Thoracic Society of Australia and New Zealand provide an appropriate framework for diagnosis and management. The key recommendations are: Confirm diagnosis, Optimise function, Prevent deterioration, Develop a self-management plan and manage eXacerbations.

C—Confirm diagnosis and assess severity Symptoms Breathlessness Cough

main symptoms

Sputum production Chest tightness Wheezing Airway irritability Fatigue Anorexia

with advanced disease

Weight loss

Consider the diagnosis of COPD in all smokers and ex-smokers older than 35 years. The diagnosis of COPD rests on the demonstration of airflow obstruction. The sensitivity of the physical examination for detecting mild to moderate COPD is poor.

Signs The signs vary according to the nature of the disease and the presence of infection. Signs may be completely absent in the early stages of COPD: chronic bronchitis may present only with wheezing, while dyspnoea is a feature of associated airflow obstruction. Signs may include:

tachypnoea reduced chest expansion hyperinflated lungs hyper-resonant percussion diminished breath sounds ± wheeze ‘pink puffer’—always breathless ‘blue bloater’—oedematous and central cyanosis signs of respiratory failure signs of cor pulmonale The diagnosis is usually clinical with a history of increasing dyspnoea and sputum production in a lifetime smoker with no (or minimal) features of asthma. It is imprudent to make a diagnosis of chronic bronchitis and emphysema in the absence of cigarette smoking unless there is a family history suggestive of alpha1-antitrypsin deficiency.6 At diagnosis, up to 50% of lung function may have been lost.

Investigations Pulmonary function tests Spirometry remains the gold standard for diagnosing, assessing and monitoring COPD. Postbronchodilator forced expiratory ratio (FER) 1 month: 7.5 mg/kg (up to 500 mg) bd for 7 days erythromycin >1 month: 10 mg/kg (up to 250 mg, or 400 mg if ethylsuccinate) qid for 7 days trimethoprim + sulfamethoxazole Cough mixtures are ineffective. Good ventilation is important: avoid dust and smoke, and also emotional excitement and overfeeding during the paroxysmal phase. All infants under 6 months and older children with complications (e.g. apnoea, cyanosis, pneumonia, encephalopathy) require admission to hospital.

Treatment of contacts8 High-risk contacts of a pertussis case (those with close/household contact and who may be vulnerable to complications, or transmit to others who are vulnerable) should be treated with the same medications, dosage and duration as above. There is little evidence that prophylactic antibiotic treatment of contacts reduces secondary transmission outside of household settings (i.e. not in child-care centres, preschools, schools or workplaces). State public health officers should be contacted to determine which contacts require prophylaxis.

Prevention8 Active immunisation with pertussis vaccine is given at 2, 4 and 6 months, then at 4 years and 12– 13 years as per the NIP schedule. Other strategies to reduce the incidence of pertussis and its complications, particularly in infants, include: a ‘cocoon’ strategy of vaccinating any adults who are or will be in close contact with an infant. Fathers and other adult contacts can be vaccinated before the birth

a pertussis vaccine should be given to pregnant women between 20 and 32 weeks (which will boost maternal antibodies that are transmitted in utero to the about-to-be-newborn fetus) a single booster dose of pertussis-containing vaccine is recommended in health care workers and other adults in regular contact with young children (booster required after 10 years)

Exclusion For the patient, 5 days after commencing antibiotics; 14 days for non-immunised contacts. The family contacts should have 7 days of prophylactic antibiotics.

Herpes simplex9 Herpes simplex virus (HSV) infection is common and widespread. Primary HSV infection is usually a disease of childhood readily transmitted through direct contact, with the majority of the population being infected in early childhood. Many cases are asymptomatic or non-specific. The specific gingivostomatitis occurs in 25–30% of cases and can be severe and acute.

Clinical features Typical clinical features of the primary infection: children 1–3 years fever and refusal to feed ulcers on gums, tongue and palate prone to dehydration may be lesions on face and conjunctivae resolution over 7–14 days Pushing fluids and monitoring for dehydration is important. Topical lignocaine preparations can help. Paracetamol is often not useful, though if the child is very distressed, combined ibuprofen and paracetamol compounds can be considered. Topical antivirals usually do not contribute and are not recommended. Serious complications (seek specialist advice): encephalitis can develop in otherwise healthy children (see CHAPTER 20

Page 999

)

eczema herpeticum—children with eczema can get widespread severe herpetic lesions disseminated HSV infection in neonates (avoid contact until recovered) HSV can be a serious issue in the immunocompromised patient

Impetigo10 Impetigo (school sores) is a contagious superficial bacterial skin infection caused by Streptococcus pyogenes or Staphylococcus aureus or a combination of these two virulent organisms. There are two common forms: 1. vesiculopustular with honey-coloured crusts (either strep or staph) 2. bullous type, usually S. aureus

Treatment If mild with small lesions and a limited area: soak a clean cloth in a mixture of ½ cup white vinegar and 1 L of tepid water. Apply the compress to moist areas for 10 minutes several times a day then wipe off crusts (alternatively just use soap and water) antiseptic (povidone iodine, chlorhexidine) or mupirocin (Bactroban), a small amount tds to crusted areas for 7 days. Topical antibiotics other than mupirocin 2% (Bactroban) are not recommended general measures while still oozing or crusted: cover the sores avoid close contact with others (especially touch and bathing contact) regular handwashing use separate towels and cloths change and launder clothes and linen daily If extensive and causing systemic symptoms: flucloxacillin/dicloxacillin 12.5 mg/kg (o) up to 500 mg, 6 hourly for 10 days or cephalexin 12.5 mg/kg (o) up to 500 mg, 12 hourly for up to 10 days Likely to be S. pyogenes in remote settings, add penicillin. Boils (furunculosis) and carbuncles can use the same treatment as impetigo.

The child should be excluded from child-care settings until antibiotic treatment has commenced. Any sores on exposed skin should be covered with a watertight dressing.2

Head lice11 Head lice is an infestation caused by the louse Pediculus humanus capitis (see FIG. 86.11 ). The head louse is about 1–3 mm in length, white to grey, wingless and has a flat elongated body. They spread through close contact, and do not jump or fly. The female louse lays eggs (or ‘nits’), which are glued to the hairs a few millimetres from the scalp. The nit moves away from the scalp as the hair grows. They hatch at around 8 days, mature into adults in about 10 days and live for about a month.

FIGURE 86.11 Louse (enlarged) Head lice spread from person to person by direct contact, such as sitting and working very close to one another. They can also spread by the sharing of combs, brushes and headwear, especially within the family. Children are the ones usually affected, but people of all ages and from all walks of life can be infested. It is more common in overcrowded living conditions but is not a reflection of poor hygiene. Resistance to the usual agents is becoming a problem.

Clinical features Infestations can vary from a few dozen to hundreds of lice and eggs. Asymptomatic or itching of scalp White spots of nits can be mistaken for dandruff

Unlike dandruff, the nits cannot be brushed off Diagnosis by finding moving lice (or nits) Occasionally the eyelashes can be affected (use physical removal methods outlined below) ‘Wet combing’ (see below) improves detection rate

Treatment Treatment can be based on physical methods, chemical methods or both (the most effective).

Physical methods Wet combing: Inspection—look for both lice and nits behind and above the ears and at the back of the scalp. Treat all members of family at the same time. Have ready a fine metal nit comb (available at pharmacies), a good light, some Page 1000 paper towels or tissues and lots of cheap hair conditioner (temporarily inactivates lice). Sitting a child in front of the TV will help them keep still (it takes around 30 minutes). Wet the hair with a handful or two of conditioner. Detangle the hair and work through in sections, removing resistant nits by combing down the hair towards the shaft. Wipe the conditioner onto tissues or paper towels, where the lice and nits will be visible. Repeat the combing routine for at least 2 more consecutive nights. Repeat weekly until no lice are found on 3 consecutive nights (nits may persist but do not necessarily mean active infection —they may be empty or dead eggs). Other physical measures include: machine wash bed-linen, clothes, towels in hot water items that can’t be washed such as soft toys/helmets should be placed in an airtight plastic bag for two weeks vacuum pillows spray hairbrushes and combs with fly spray

Chemical treatments Insecticides used to treat head lice topically include:

maldison 0.5% or 1% according to instructure permethrin 1%, leave 10 minutes, repeat in 7 days pyrethrins 0.165% + piperonyl butoxide 1.65%, leave 10 minutes, repeat in 7 days These applications can be irritating to the scalp, so use caution in children prone to atopic dermatitis; following the manufacturer’s instructions carefully is important. Wet comb after each treatment. A second application 7 days after the first may be required. Many other methods are used and recommended for head lice, both commercially available or ‘home remedies’, which may or may not be useful. These include suffocating agents (e.g. mayonnaise, olive oil, petroleum jelly), natural oils and cutting the hair short (especially in boys, e.g. a No. 1 cut). Treatment failure and/or reinfestation is common. The former can be from inappropriately applied treatments, resistance to chemical treatments or misdiagnosis of old nits as active infection. Cotrimoxazole can be trialled for resistant head lice. Community or school-wide education programs can also keep infestation rates down. Notify the school when detected. No exclusion necessary after initial treatment.

Body lice Diagnosis is by demonstration of lice and nits in clothing, especially at the seams, and bed clothes. Treatment is as for head lice, applying the preparation to the entire body (with care). The fomites should be discarded or washed in a hot cycle or sealed in plastic bags for 30 days.10

Scabies Refer to pruritis (see CHAPTER 112

).

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Chickenpox (varicella) Hand, foot and mouth disease Lice: head lice Herpes simplex (cold sores) Impetigo Measles

Mumps Roseola Rubella Scabies Slapped cheek disease Whooping cough (pertussis)

References 1

National Health and Medical Research Council. The Australian Immunisation Handbook (10th edn, updated January 2014).

2

National Health and Medical Research Council. Staying Healthy in Child Care— Preventing Infectious Diseases in Child Care (4th edn), December 2005.

3

The Center for Disease Control. Measles (home page), July 2014. Available from: www.cdc.gov/measles/, accessed 27 July 2014.

4

The Center for Disease Control. Pregnancy and fifth disease, February 2012. Available from: www.cdc.gov/parvovirusB19/pregnancy.html, accessed 27 July 2014.

5

The Royal Children’s Hospital Melbourne. Clinical Practice Guidelines: Kawasaki Disease, December 2012. Available from: www.rch.org.au/clinicalguide/guideline_index/Kawasaki_Disease_Guideline/, accessed 28 July 2014.

6

American Academy of Pediatrics. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals. Pediatrics, 2004; 114(6): 1708–33.

7

The Royal Children’s Hospital Melbourne. Clinical Practice Guidelines: Whooping Cough (Pertussis), May 2014. Available from: www.rch.org.au/clinicalguide/guideline_index/Whooping_Cough_Pertussis/, accessed 28 July 2014.

8

Pertussis [published 2019]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2019. www.tg.org.au, accessed August 2020.

9

The Royal Children’s Hospital Melbourne. Clinical Practice Guidelines: HSV Gingivostomatitis, November 2013. Available from: www.rch.org.au/clinicalguide/guideline_index/HSV_Gingivostomatitis/, accessed 29 July 2014.

10 11

Impetigo [published 2019]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2019. www.tg.org.au, accessed August 2020. DermNet NZ: the dermatology resource. Available from: www.dermnetnz.org/, accessed 30 July 2014.

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87 Behavioural and developmental issues and disorders in children

Although the world is full of suffering, it is full also of the overcoming of it. HELEN KELLER, 1903 Many behavioural issues in children arise because of (and in the context of) factors such as the child’s temperament, parental schema and child–parent attachment. These factors determine, in the beginning, how the parent–child relationship will evolve, and also strongly affect the psychological development of the child.

Temperament Temperament refers to the personality characteristics you are born with—what is innate rather than learned. An awareness of the fact that children are born with characteristics over which parents have no control can be liberating, especially for parents of children with challenging temperaments. The best-known description of temperament is based on the work of Thomas and Chess from the 1970s1 (see TABLE 87.1 ). They found that about two-thirds of children can be classified into one of three categories: easy, difficult and slow to warm up. Of these, about 15% were considered easy, 70% slow to warm up and 15% difficult. A child with a difficult temperament will be emotionally labile and have difficulty coping with new experiences. A child with a slowto-settle temperament will have some difficulty coping with new experiences, but will eventually manage after repeated exposures. A child with an easy temperament will cope easily with new experiences and will have a calm nature.

Table 87.1

Easy and difficult temperament characteristics

Easy temperament characteristic Generally happy

Difficult temperament characteristic Generally serious

Goes with the flow

Inflexible

Laid-back

In-your-face

Patient

Impulsive

Flexible

Stubborn

Friendly

Shy

Calm body movements

Restless

Persistent

Gives up easily

Calm nature

Hot-tempered

Expressive

Reserved

‘Even’ mood

Up and down mood

Thick-skinned

Over-sensitive

Good concentration

Distractible

Regular body clock

Chaotic body clock

Tolerates sensations well

‘Sensory defensiveness’ (light, sounds, tastes, textures)

Easy doesn’t mean good, and difficult doesn’t mean bad. These children may just require more patience and a thoughtful and paced approach to parenting. Further, the characteristics that can make a child ‘difficult’ can be the flipside of other characteristics that lead them to achieve success (if appropriately nurtured and guided). A stubborn child may have good persistence, and a distractible child may be artistic and expressive. Of course, most children will have a few ‘difficult’ characteristics, but some will have more than others. Raising parents’ awareness of this, getting them to think about the innate characteristics of their child (which may or may not be similar to their siblings or one or both of the biological parents) and to adjust their parenting style accordingly will help them deal more effectively with the challenges of raising their child.

Parental schema Schema is the view you have of the world based on your own experiences; it is the ‘soup in your head’. Parental schema is how parents view how they should parent, and is often based on how they were brought up themselves. Parental schemas may not match, and this can cause relationship issues. Parental attitudes to issues such as communication, emotion sharing and discipline can fundamentally affect the behaviour and psychological health of a child. Parental schema can also commonly be influenced by cultural factors.

Child–parent attachment

An infant first has to learn how to connect with other minds. A child does this in their first relationship, usually with their mother. This is attachment.

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It’s important to note that attachment is an ongoing phenomenon, even into adulthood. Children attach not only to their mother, but to other people from whom they can ‘gather strength’. Children’s attachment to their two parents may differ and can also change over time. A secure attachment is achieved if a parent responds to a child’s cries promptly, consistently and appropriately. The three main ways in which attachment can go wrong are: Carer not responding—this can result in a lack of emotional depth in the child. Carer responding inconsistently—this can lead to uncertainty and anxiety in the child. Carer responding inappropriately—such as with aggression or anger. This is obviously very damaging, and happens in situations of emotional abuse, particularly with mental health or drug and alcohol issues in the carer. It creates a terrible and unsolvable paradox for children, where the person on whom they are completely reliant to teach them how to relate to the world is also a threat. Observing how carers, particularly mothers, respond and interact to their child can give the family doctor insight into this relationship. Gently advising and encouraging appropriate responses to the child’s needs can be beneficial, particularly in a mother with poor parenting capabilities and/or difficult circumstances (e.g. very young mothers, socially isolated, postnatal depression, drug using or alcoholic parents, violence in the household). In high-risk situations, further referral to support services or even protective agencies may be required (see CHAPTER 88 ).

Child behaviour and discipline2,3 Issues around child behaviour and how to deal with unwanted behaviours commonly arise and are often a source of conflict and angst. It is normal for children, especially toddlers, to have resistant and oppositional behaviour. The word ‘discipline’ comes from the Latin disciplina, which means ‘to teach’. The purpose of disciplining a child is to guide and teach, so they know what is allowed and what is not allowed, what ‘works’ in the world and what will not. Parents can teach children in a variety of ways. The most effective forms of discipline are actually praise and encouragement—of wanted behaviours. However, when children are doing something that they should not—an unwanted behaviour—they need to learn that this is the case. The challenge for parents is to get this message across to them clearly and effectively. A simple equation sums up the best way to deliver this message: unwanted behaviour = unwanted (by your child) outcome Educating parents to recognise that the thing a child desires most in the world is the attention

(this is a survival instinct) and approval of their parents will help them understand how to reinforce desired behaviours, and extinguish unwanted behaviours. That is, pay attention to when the child is behaving, and even praise particularly wanted behaviours, and ignore unwanted behaviours.

Consequences3 Consequences work only with children >3 years of age, and should be used solely in response to a small fraction of a child’s behaviour, regardless of the child’s temperament. If consequences are overused, their effectiveness diminishes and family relationships can be damaged. There are three types of consequences that work (in decreasing order of usefulness): Natural consequences: where there is a natural flow from the misdemeanour to the consequence. For example, if a child refuses to have dinner, he or she goes to bed hungry. Natural consequences have a momentum of their own and, assuming they do not result in harm, can provide many valuable lessons. Related consequences: where there is some link between the unwanted behaviour and the result. This is also sometimes called a ‘logical consequence’. For example, if a child makes a mess, he or she must clean it up. Or, if two children are fighting over a toy, the toy gets taken away for a while. Related and natural consequences allow a child to link the unwanted behaviour with the consequence. Losing a privilege: This can be a very powerful technique as it focuses the child’s Page 1003 attention but is more punitive than natural and related consequences. Also, there is no logical link between behaviour and consequence. Parents should only withdraw a privilege with care as it can cause resentment, and the child should be warned beforehand that he or she is going to lose a particular privilege for a particular unwanted behaviour. Time-outs4 are another response to unwanted behaviours. Time-outs are useful from 2–3 years of age, and can take the heat out of a situation, for both the parent and child. Time-outs should be consistent, enforced promptly and calmly, even with empathy, and short—a good rule of thumb is a minute per year of age. Responses that don’t work when disciplining children include: screaming, constantly explaining, repeatedly warning, threatening, pleading, arguing, bribing and giving in, as well as smacking.5 Most parents who are struggling with behaviour and discipline issues respond with relief when provided with a set of alternative responses for disciplining children, though care needs to be used when raising these issues, as they can be highly emotional and confronting topics for parents.

Maltreatment GPs should consider maltreatment of the child if there is behaviour change inconsistent with age

or development and which is not explained by a problem such as autism or by a stressful situation such as parental separation.

Tantrums6 Tantrums are when we lose control of our feelings and behaviour. Everyone, including adults, is capable of tantrums. Tantrums are what we use to deal with an emotionally confronting situation when we have no other option—the emotional response of last resort. They are more common in toddlers (particularly between 18 months and 3 years of age) because they have not learned any or many other emotional responses to challenging situations, such as ignoring, negotiating, reconsidering or reframing a situation. There may be a dramatic protestation to frustration such as kicking, shouting, screaming, throwing or head banging. There is a lot of misinformation about tantrums, frequently over-emphasising control rather than guidance. They may be a pointer to autism. Diverting a child from predictable tantrum triggers, ‘scaffolding’ (giving necessary support) to handle the event (e.g. a 5-minute warning), distraction or active listening in the build-up to a tantrum can be useful strategies to employ. Once a child is mid-tantrum, any attempts at negotiation will be ineffective. Afterwards, an unemotional and calm response from the parent will tell the child that tantrums don’t work (though, of course, this can be difficult for a stressed parent). Skill-building toddlers in how to handle emotionally challenging situations (i.e. expanding their repertoire beyond tantrums) is best done either before or after (‘What else could you have done?’). Rousing at a child is counter-productive; they are still learning to control their emotions, and this will just increase the risk of them becoming demoralised and losing self-esteem. Appropriate advice for parents Ignore what is ignorable; avoid what is avoidable; distract what is distractable; praise appropriate behaviour. Medication has no place in management.

Breath-holding attacks7 Breath-holding is common (around 5% of children do so at some stage), and occurs between 6 months and 6 years (peak at age 2). While frightening to watch, such attacks are harmless, and no treatment or action is required. There are two types: blue spells (more common)—these happen in response to being upset or angry. The child cries loudly then holds his or her breath at the end of expiration pale spells (white attack)—these anoxic ‘seizures’ happen in response to pain or a fright They can last from 10–60 seconds, and can cause the child to faint or, rarely, fit. They cause no harm, and parents should be reassured. Approaches to discipline should not change because of them. There is no need to blow air or splash water in the child’s face, and the child should be

treated as if the breath-holding has not occurred (i.e. no discussion or punishment).

Head banging and rocking8 Head banging and rocking, which usually occur before the child goes to sleep, are common. Head banging occurs in up to 30% of normal infants and toddlers, and rocking is even more prevalent. They are more frequent in children with developmental disability and autism, where these behaviours may be more pervasive through the day.

Features Usually starts after 8–9 months, common at 3 years and is rare after 2 years of age Usually prior to going to sleep Child usually not distressed and rarely self-injurious

Management There is no need to try to stop the behaviour, and attempting to do so is often not successful.

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Reassure parents that it’s a self-limiting behaviour and usually settles by 2–3 years. Avoid reinforcing behaviour by excessive attention or punishment. Ignore the behaviour. Place the bed or cot in the middle of the room away from a wall (reduces disruption from noise). Monitor the condition of the cot, to ensure screws and hinges are not being loosened.

Sibling rivalry Sibling rivalry is very common and can cause a lot of disruption in the family. A toddler may pinch, prod and even attempt to smother a new baby, so providing the toddler with attention from the mother and encouraging the child to see that it is his or her baby too can ease the jealousy. In school-age children the following tips are useful:9 Be fair. Avoid making comparisons between your children. Encourage the children to work out their own differences. Avoid taking sides in sibling conflicts. Set guidelines on how children can disagree and

resolve conflicts. Discourage telling tales on each other. When it is necessary to punish or reprimand, do it with the child alone in a quiet, private place. Use regular family meetings to establish family rules and negotiate conflicts.

Stuttering/stammering Stuttering (also known as stammering)10 usually begins at age 2–5 years, as children start to use more words and longer sentences. It can start suddenly or develop slowly. Up to 12% of children will stutter by the age of 4. Stutters can be repetitions (of a sound, word or phrase), prolongations of a sound (e.g. ‘aaaand’), a block (where no sounds comes out) or combinations of these. There is no known cause, though it can run in families. Stutters are not associated with other disabilities, though they may cause psychological distress for the child. They are not caused by anxiety or stress, but can be more prominent when the child is excited. A child developing a stutter should see a speech pathologist as soon as possible, as early treatment is more successful. The most common program used in Australia to treat stuttering is the Lidcombe Program, which is often successful and can even sometimes stop the stutter. However, evidence to support this and other interventions is not strong.

Habit cough This is a common problem usually affecting school-age children and occurs in the absence of underlying disease. It occurs only when the child is awake—not during sleep. There are two common types of habit cough: 1. A honking-type cough (heard from the waiting room) in teenage girls. There is no dyspnoea or sputum production. 2. A throat-clearing cough commonly seen in boys aged 7–10. This is related to a transient tic disorder. Habit cough is a diagnosis of exclusion and there should be a careful assessment to exclude other causes such as respiratory disorders or pertussis. Underlying triggers include inter-family problems and bullying or other perceived stress or anxiety, e.g. school issues. Management includes explanation, reassurance and CBT. Referral for resistant cases is appropriate. Other functional respiratory problems:

hyperventilation sighing dyspnoea vocal cord dysfunction (see CHAPTER 46

)

Sleep disorders in children See CHAPTER 60

.

Bullying11,12 There are varying figures on how common bullying is, but most suggest more than a quarter of children are significantly bullied at some stage. It is more common in the early school years, but can happen at any age. Bullying differs from conflict, in that there is an imbalance of power between the perpetrator and the victim. It is a deliberate behaviour repeatedly done and intended to harm the victim. It is a power play, constructed on contempt, not anger, and this is what can make it so damaging. Types of bullying include physical harm, verbal assaults, hidden bullying (e.g. Page 1005 exclusion or spreading rumours) and cyberbullying (now the most common form of bullying). The latter is particularly insidious as it is difficult to detect and follows the victim home and even into their bedroom via social media or phones. Bullying can lead to physical, psychological and social consequences, and in extreme cases depression and even suicide. Despite this, less than half of bullying is reported (with boys reporting less than girls) due to fear of retaliation or not being believed. Bullies themselves tend to be assertive, impulsive and aggressive, with little empathy for their victims or remorse for their actions. They often come from dysfunctional backgrounds, and their bullying tendency may have been learned from being modelled by parents or peers. If bullying is raised, the child should be believed, and a history carefully, gently and nonjudgmentally extracted. Parents should be encouraged to stay calm, involve other adults in the environment (e.g. teachers at school) and to formally address the issue after educating themselves on the best approach. There are many good resources available for this, such as the National Centre Against Bullying at www.ncab.org.au. A vigilant approach to this common and serious problem by GPs and parents should be employed, especially in those particularly vulnerable to bullying, such as children who are shy, lacking in confidence, children with disabilities or who physically or socially stand out in some way (e.g. children with speech disorders). Indicative signs to watch out for include: school phobia: sham sickness and other excuses being tense, tearful and miserable after school reluctance to talk about happenings at school

poor appetite functional symptoms (e.g. habit cough) repeated abdominal pains/headache unexplained bruises, injuries, torn clothing, damaged books lack of a close friend; not bringing peers home crying during sleep restless sleep with bad dreams appearing unhappy or depressed unexpected irritability and moods; temper outbursts Referral to a psychologist should be considered in severe cases, in those that fail to be resolved or if there are other psychological or family issues. If child abuse is suspected, appropriate action should be taken (see CHAPTER 88 ).

Behavioural and developmental disorders Developmental disability and delay The family doctor is in an ideal position to recognise and initiate evaluation of the child with a developmental disability, whether it is a physical disability or delay, an intellectual disability or a learning disability. All children with a suspected developmental delay should be promptly evaluated by a paediatrician or a multidisciplinary developmental assessment team, and the family doctor should remain a key player in the long-term management both of the child and the family. Many developmental problems will be obvious but others are subtle. Several disabilities may evade a diagnosis. Transient developmental delay may be associated with factors such as prematurity, family stress, physical illness and learning opportunities, while persistent delay can be caused by intellectual disability, cerebral palsy, autism and hearing and visual impairment. Many rare dysmorphic syndromes are becoming more recognised and defined with the rapid advances in genetics, and referral to genetic disorder units will help in getting an appropriate evaluation.

Evaluation An appropriate history includes a careful look at developmental milestones, social and behavioural issues, and family history (see CHAPTER 6 ). A new diagnosis of developmental

delay of any sort can have a massive impact on family members, especially parents, and the family doctor should keep a watchful eye on how they are coping and reacting to the diagnostic process. The physical examination includes growth parameters, assessment of all developmental domains, looking for dysmorphic features, testing of hearing and vision, examining for neurocutaneous stigmata (e.g. café-au-lait spots, neurofibromas, hypopigmented macules) and careful systems examination, including cardiac and neurological examination. Eyes, ears, mouths (including teeth), hands, feet and genitals should all be examined. Investigations are usually coordinated via specialist services, but often include bloods (including karyotyping and genetic tests), urinary metabolic screening and imaging, such as cerebral MRI (which may require a general anaesthetic).

Intellectual disability Intellectual disability (ID) is a neurodevelopmental disorder characterised by deficits in intellectual and adaptive functioning that present before 18 years of age.13 The term replaces and improves upon the older term ‘mental retardation’. The term ‘global developmental delay’ (GDD) is usually used to describe children 3/week for >12 months Conduct disorder—where there is behaviour that violates the rights of others or societal norms, such as aggression directed towards people, animals or property, often with a callous manner and lack of empathy

Tic disorders24 Tics are sudden, rapid, recurrent involuntary vocalisations or movements usually appearing in bouts that wax and wane in intensity, frequency and type of tic. They include behaviours such as grunting, blinking, shrugging shoulders, humming, yelling out a word or phrase or clearing the throat. Tic disorders are classified into:13 Tourette syndrome—motor and vocal tics for >1 year persistent motor or vocal tic disorder—motor or vocal tics for >1 year provisional tic disorder—motor and/or vocal tics for 5 years may have a place.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Attention deficit hyperactivity disorder Autism Autism: Asperger’s syndrome Bullying of children Stuttering Tantrums Tourette disorder

Resources National Disability Insurance Scheme. Ph: 1800 800 110; www.ndis.gov.au/. Amaze (Victoria). Ph: 1300 308 699; www.amaze.org.au. Autism Spectrum of Australia (Aspect). Ph: 1800 277 328; www.autismspectrum.org.au.

References 1

Thomas A, Chess S. Temperament and Development. New York: Bruner/Mazel, 1977.

2

American Academy of Pediatrics Committee on Psychosocial Aspects of Child and Family Health. Guidance for effective discipline. Pediatrics, 1998; 101: 723–28 (reaffirmed Pediatrics, 2012; 130: e467).

3

Raising Children’s Network. Consequences, June 2011. Available from: www.raisingchildren.net.au/articles/consequences.html, accessed 3 August 2014.

4

Raising Children’s Network. Time out, June 2010. Available from: www.raisingchildren.net.au/articles/time_out.html, accessed 3 August 2014.

5

The Royal Australian College of Physicians. Paediatric & Child Health Division. Position statement: physical punishment of children, July 2013. Available from: www.racp.edu.au/page/paed-policy, accessed 3 August 2014.

6

Women and Children’s Health Network. Parenting and child health: tantrums. Available from: http://www.cyh.com/HealthTopics/HealthTopicDetails.aspx? p=114&np=141&id=1775, accessed 20 November 2017.

7

The Royal Children’s Hospital Melbourne. Kids health info: breath holding. Available from: www.rch.org.au/kidsinfo/fact_sheets/Breath_holding/, accessed 18 November 2017.

8

Women and Children’s Health Network. Parenting and child health: head banging and rocking, November 2013. Available from: http://www.cyh.com/HealthTopics/HealthTopicDetails.aspx?p=114&np=141&id=2958, accessed 20 November 2017.

9

American Academy of Pediatrics. Healthy children: dealing with sibling rivalry, November 2013. Available from: www.healthychildren.org/English/family-life/familydynamics/Pages/Dealing-with-Sibling-Rivalry.aspx, accessed 9 August 2014.

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10

Raising Children’s Network. Stuttering, November 2013. Available from: www.raisingchildren.net.au/articles/stuttering_an_overview.html, accessed 9 August 2014.

11

The National Centre Against Bullying, Alannah & Madeline Foundation. Available from: www.ncab.org.au/, accessed 2 December 2017.

12

Briggs, F. Smart Parenting for Safer Kids. Melbourne: JoJo Publishing, 2011.

13

American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders (5th edn), 2013.

14

Australian Cerebral Palsy Register Group, report 2013.

15

Wray J, Williams K. The prevalence of autism in Australia. Report commissioned by the Australian Advisory Board on Autism Spectrum Disorders, 2007.

16

Wray J et al. Language disorders and autism. MJA, 2005; 182 (7): 354–60.

17

Raising Children’s Network. Children with autism, September 2016. Available from: www.raisingchildren.net.au, accessed 19 November 2017.

18

Prior M et al. The Australian Society For Autism Research. A review of the research to identify the most effective models of practice in early intervention for children with autism spectrum disorders. Report commissioned by the Australian Government Department of Families, Housing, Community Services and Indigenous Affairs (FaHCSIA), 2011.

19

The Royal Children’s Hospital Melbourne. Kids health info: ADHD—an overview, September 2012. Available from: www.rch.org.au/kidsinfo/fact_sheets/ADHD_an_overview/, accessed 13 August 2014.

20

The Royal Children’s Hospital Melbourne. Kids Health info: ADHD—ways to help children with ADHD, September 2012. Available from: www.rch.org.au/kidsinfo/fact_sheets/ADHD_ways_to_help_children_with_ADHD/, accessed 17 November 2017.

21

Disorders usually first diagnosed in childhood and adolescence [updated 2021]. In: Therapeutic Guidelines [digital]. Melbourne Therapeutic Guidelines Limited; 2021 www.tg.org.au, accessed July 2020.

22

Buckley N (Chairman). Australian Medicines Handbook. Adelaide, 2018: 864–5.

23

Fraser A, Wray J. Oppositional defiant disorder. Aust Fam Physician, 2008; 37(4): 402–5.

24

ESSTS guideline group. European clinical guidelines for Tourette syndrome and other tic disorders. Eur Child Adolesc Psychiatry, 2011; 20: 155–71.

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88 Child abuse

It is customary, but I think it is a mistake, to speak of happy childhood. Children, however, are often overanxious and acutely sensitive. Man ought to be man and master of his fate; but children are at the mercy of those around them. SIR JOHN LUBBOCK, BARON AVEBURY (1834–1913) Public and medical awareness of child abuse has markedly increased in recent decades, and the possibility of child abuse and neglect has to be kept in mind by the family doctor. It may surface in families from all walks of life and where a good trustful relationship exists between parents or children and doctor. While reporting rates continue to increase (at 34 per 1000 Australian children in 20121), the true incidence of child abuse is much greater and also difficult to assess. The various types of abuse1 (and their estimated incidence2) are shown in FIGURE 88.1

.

FIGURE 88.1 Percentage breakdown of primary substantiated harm types in Australia in 2011–20123 Source: Based on Australian Institute of Health and Welfare material.

Facts on child abuse1,3,4 The younger the child, the greater the risk. The more disabled, the more vulnerable. Indigenous children are 8 times more likely to be the subject of substantiated abuse than nonAboriginal children. The number of children who are deemed at risk and placed in out-of-home care (OOHC) has increased in recent years, with the rate in 2012 at 7.7 per 1000 Australian children nationally. Roughly half of these are in foster care, and half in relative/kinship care, with a small (5%) proportion in residential (paid workers) care.

Definitions5 Child abuse and neglect are collectively referred to as child maltreatment. This refers to any nonaccidental behaviour by parents or other adults or adolescents that is outside the norms of conduct and entails a substantial risk of causing physical or emotional harm to a child or young person. Such behaviours may be intentional or unintentional, and can be through omission (i.e. neglect) or commission (i.e. abuse). Deciding whether behaviour constitutes maltreatment can depend on: cultural issues (e.g. corporal punishment) developmental age of the child the severity, frequency and duration of the behaviour the behaviour itself, or the result of the behaviour (a definition issue)

Physical abuse (non-accidental injury) Physical abuse is defined as the non-accidental use of physical force against a child that results in harm to the child. It include behaviours such as hitting, shoving, slapping, shaking, throwing, punching, kicking, biting, burning, strangling and poisoning. Munchausen syndrome by proxy (see OTHER TYPES OF CHILD MALTREATMENT) is considered physical abuse.

Neglect

Neglect is defined as the failure by a parent/caregiver to provide a child (when they are in a position to do so) with the conditions that are culturally accepted as being essential for the child’s physical and emotional development and well-being.

Emotional maltreatment Page 1015 This is where a parent or caregiver conducts inappropriate verbal or symbolic acts towards a child, and/or there is a pattern or failure over time to provide a child with adequate non-physical nurturing and emotional availability. This can take the form of rejecting, isolating, terrorising, ignoring or corrupting, and can damage the child’s self-esteem and social functioning.

Sexual abuse Sexually abusive behaviour refers to any sexual activity between an adult and a child below the age of consent; non-consensual sexual activity between minors (e.g. a 14-year-old and a 10-yearold); or any sexual activity between a child under 18 years of age and a person in a position of power or authority (e.g. parent, teacher).2 It is sometimes difficult to determine whether a behaviour constitutes sexual abuse, depending on variables such as age, development and cultural norms. Behaviours can include fondling of genitals; masturbation; oral sex; vaginal or anal penetration by a penis, finger or any other object; fondling of breasts; voyeurism; exhibitionism; or exposure to pornography. In contrast to other forms of maltreatment, the labelling of an act as sexual abuse also depends on the relationship between perpetrator and victim (e.g. age difference, being related).

Other types of child maltreatment Munchausen syndrome by proxy—this is the term used when a parent or guardian creates an illness in a child so that the perpetrator can develop or maintain a relationship with medical staff or transfer his or her responsibilities. A ‘devoted parent’ may continually present a child for medical treatment yet deny the origin of the problem—namely, the parent. Often, the mother is the abuser. The abuse may be of physical or medical neglect. The masquerade may be simple or very sophisticated. Children may be indirectly abused by the lengthy or invasive investigations. Be cautious where there has been unexplained illness or death of a sibling. Incest—defined as intercourse between biological family members. Fetal abuse behaviour, such as smoking, drinking or illicit drug use, during pregnancy that endangers a fetus. Bullying, or peer abuse (see CHAPTER 87 Sibling abuse Witnessing community violence

)

Institutional abuse Organised exploitation (e.g. child sex rings, child prostitution) State-sanctioned abuse (e.g. female genital mutilation in parts of Africa, the Stolen Generations in Australia)

Female genital mutilation This comprises all procedures involving partial or total removal of the female external genitalia or other injuries to the female genital organs, whether for cultural or other non-therapeutic reasons (WHO definition). It is also referred to as female circumcision.

Abuse: who and why? The real cause seems to be a combination of several interrelated factors: personal, familial, social/cultural and societal stress. Abused children exist at all levels of society, although the majority of abused children who come to the attention of authorities are from families where there is high mobility, lack of education, loneliness, poverty, unemployment, inadequate housing and social isolation. Sexual abuse, occurring alone, does not follow these patterns and can occur under any socioeconomic circumstance. Both men and women physically abuse their children. While women are the parents most responsible in cases of neglect and emotional abuse (probably because of a dominant role in childcare, social and economic disadvantage and being the only one responsible for the care of children in a single parent arrangement), men are more likely to abuse their own or other’s children sexually. The child can be abused at any age (even adolescents can be victims of abuse and neglect). It is important to keep this in mind—it does happen.

Underdiagnosing and under-reporting Although the medical profession remains the foremost focus of child abuse reporting (they are the most likely to encounter injured children and are the most qualified to diagnose abuse), they still contribute only a small percentage of the total reporting to central registries.6 This could be because there is underdiagnosis of the problem, but it could also be because of under-reporting. Reasons given6 as to why GPs don’t report more cases of child abuse include: concern about drain on time and finances breaching doctor–patient confidentiality

lack of undergraduate education on the topic risk of alienation and stigmatisation to the family the feeling by some GPs that they can work on the problem with the family without outside intervention

Page 1016

a breakdown of the doctor–patient relationship uncertainty about what to do personal and legal risks (e.g. fear of court, libel suits, irate parents), especially if they ‘get it wrong’ reluctance until absolutely certain of diagnosis It will always be difficult to take the first step but it is important and it can help, no matter how small that first step is.

Interviewing parents or guardians A skilled, sensitive, diplomatic interview is fundamental to management. Guidelines include: a relaxed, non-judgmental approach sensitivity to all people involved appropriate questions—open-ended, not leading using verbatim quotes from the child where possible and waiting silently for a reaction recording notes carefully

Physical abuse7,8 Physical abuse should be suspected, especially in a child aged under 2 years, if certain physical or behavioural indicators in either the child or the parents are present. Inflammation, bruising, abrasions and lacerations are the most common presentations of the physically abused child, with equal incidence in boys and girls. As well as the consequences of the physical injuries, victims of physical abuse are more likely to develop a variety of behavioural and functional problems including conduct disorders, physically aggressive behaviour (e.g. become bullies), poor academic performance and decreased cognitive functioning. Children suffering traumatic brain injuries from physical abuse (e.g. hitting head or shaking) are at risk of disabilities including ADHD, seizures, spasticity, blindness, paralysis and developmental delay. Parents and carers can tend to misinterpret normal child behaviours (e.g. crying and tantrums) and respond to them inappropriately. A vigilant GP can actually help prevent abuse by providing

‘anticipatory guidance’ to parents on normal child behaviour, educating them on appropriate responses to behaviours and offering themselves as a resource to which parents can turn if the child’s behaviour becomes unmanageable or overwhelming. Risk factors: maternal smoking >2 siblings low infant birth weight unmarried mother poverty significant life stressors in parents caregiver role conflicts living in house with unrelated adult (50 times more likely to die from inflicted injuries)

Physical abuse indicators Accidental injuries such as bruises or fractures are common in children, but tend to follow certain patterns. A GP should be aware of cases where the injury or injuries suggest a nonaccidental cause is possible, when the history doesn’t match up with the injury or developmental age of the child, or when the behaviour of the parent and/or child is suggestive of non-accidental injury. Bruises Bruises are commonly found on the front of the body over bony prominences (e.g. forehead in toddlers, knees and shins in older children). Suspicious bruises include: finger-shaped bruises (e.g. thumb grip marks) multiple bruises/welts of different ages (the colour of a bruise doesn’t reliably indicate when it occurred) bruises in premobile children bruises away from bony prominences—face, scalp, neck, buttocks (see FIG. 88.2 earlobes, behind the ears

), genitalia,

FIGURE 88.2 Physical abuse: imprint of a boot on the buttock of a child abdominal bruising (consider damage to internal organs, including organ rupture)

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multiple bruises of uniform shape multiple bruises in clusters Scalds and burns Beware of: a scald of uniform depth, sparing flexures, uniform burn line demarcation, bilateral and no splash marks (suggests forced immersion) unusual position (e.g. back of hand, genitals) cigarette butt-type burns Fractures Look out for: metaphyseal fractures of the proximal humerus and proximal or distal tibia are highly suggestive

other fractures commonly non-accidental: rib (especially posterior), clavicle, vertebral body, sternum, scapula multiple, especially bilateral complex or multiple skull fractures Shaking and brain injury Shaking a baby is a critically dangerous thing to do, a danger often underestimated by perpetrators. The relatively large head size and weak neck muscles can easily lead to serious injury. Suspicion of traumatic brain injury should be raised when there is: unexplained encephalopathy unexplained vomiting, irritability and apnoeas altered states of consciousness neurological symptoms and signs torn frenulum or retinal damage

History Unexplained injury Different explanations offered Vague or changing history Injury unlikely to have occurred in manner stated Unreasonable delays between injury and presentation Presentation inconsistent with child’s developmental capabilities Evidence of neglect (clues include failure to thrive, dental caries, severe nappy rash, poor wound care) Remember Munchausen syndrome by proxy.

Behavioural indicators Wariness of adult contacts Inappropriate clothing (e.g. long sleeves on a hot day)

Apprehension when other children cry or shout Behavioural extremes Fear of parents Afraid to go home Child reports injury by parents or gives inappropriate explanation of injury Excessive compliance Extreme wariness Attaches too readily to strangers

Management If suspected or disclosed, a thorough assessment, with detailed documentation (including measurement and photography of bruises/other injuries) of findings should be undertaken. A detailed history and thorough examination of all areas of the body is required. In a general practice setting, getting help with this difficult situation would often be useful. Discussing (immediately) with colleagues or child protection authorities is advisable, with the safety of the child (and other cohabiting children) being ensured at all stages. Verbatim comments should be written down and, if possible, information gathered from other sources or witnesses. The family doctor should diplomatically confront the parents and/or carers and always act in the best interests of the child. Offer to help the family. An approach would be to say, ‘I am very concerned about your child’s injuries as they don’t add up—these injuries are not usually caused by what I’m told has been the cause. I will therefore seek assistance—it is my legal obligation. My duty is to help you and, especially, your child.’ Acquiring essential help Psychosocial assessment of child and family: involves social worker and multidisciplinary assessment Admission to hospital: for moderate and severe injuries Investigations done in conjunction with specialists Case conference (where appropriate) Mandatory reporting: notify child protection authorities

Emotional abuse

Physical indicators There are few physical indicators, but emotional abuse can cause delay in physical, emotional and mental development.

Behavioural indicators Extremely low self-esteem Compliant, passive, withdrawn, tearful and/or apathetic behaviour Aggressive or demanding behaviour Anxiety Serious difficulties with peers and/or adult relations Delayed or distorted speech Regressive behaviour (e.g. soiling)

Neglect Poor parenting or neglect occurs in at least 5% of children under 5 years.9

Physical indicators Consistent hunger Failure to thrive, or malnutrition Poor hygiene Inappropriate clothing Consistent lack of supervision Unattended physical problems or medical needs Abandonment Dangerous health or dietary practices

Behavioural indicators Stealing food Extending stays at school

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Consistent fatigue, listlessness or falling asleep in class Alcohol or drug abuse Child states there is no caregiver Aggressive or inappropriate behaviour Isolation from peer group

Sexual abuse5,10 Only 6% of child sexual abuse is by strangers. Most are known by parents and child; that is, familiar people in familiar environments, especially within the family. A child may disclose up to 12 times before they are believed (listen for hints). Most (but not all) of the adults who sexually abuse are men. Boys are assaulted less commonly than girls, but are less likely to disclose if they are assaulted. Adolescents are perpetrators in at least 20% of cases. Child sexual abuse is usually about power rather than sexual gratification.

Sexual abuse presents in three main ways: Disclosure: allegations by the child or an adult Injuries to the genitalia or anus Suspicious presentations, especially: genital infection (see FIG. 88.3

)

recurrent urinary infection unexplained behavioural changes/psychological disorders

FIGURE 88.3 Genital human papillomavirus infection: a sign of sexual abuse in a child Clinical indicators that may suggest child sexual abuse are presented in TABLE 88.1 Table 88.1

Clinical indicators that may suggest child sexual abuse

Child disclosing abuse (rarely invented, and the majority of presentations) Parent or adult disclosing abuse allegation Vaginal discharge Sexually transmitted infections (acquired in 5% of sexually abused children) Urinary tract infection Unexplained genital trauma Unexplained perianal trauma Overt sexual play

.

Pregnancy in an adolescent Deterioration in school work Family disruption Indiscriminate attachment Abnormal sexualised behaviour (many abused children do not do this) Poor self-esteem Psychological disorders: behaviour disturbances regression in behaviour sleep disturbances abnormal fears/reactions to specific places or persons psychosomatic symptoms anxiety lack of trust overcompliance aggressive behaviour Depression: self-destructive behaviour substance abuse suicidal tendencies Non-specific physical problems: abdominal pain enuresis (especially secondary) encopresis Examination (abnormal findings uncommon) Genital trauma Perforated hymen/lax vagina (may be normal variant) Perianal trauma Vaginal discharge Look for semen and STIs

Clinical approach Ideally, the child should be assessed by experienced medical officers at the regional sexual assault service, so the temptation for the inexperienced GP to have a quick look should be

resisted. For the practitioner having to assess the problem, a complete medical and social history, including a behavioural history, should be obtained prior to examination. The child’s history must be obtained carefully, honestly, patiently and objectively, without leading the child. The history is more important than the physical findings as there are no abnormal physical findings in many confessed cases.

Examination Page 1019 A parent or legal guardian must give informed consent before the child is physically examined. It is recommended that the physical examination of any child suspected of being sexually abused is performed according to the VFPMS proforma (see: www.rch.org.au/vfpms) and ideally by a paediatrician or forensic physician experienced in the area of sexual abuse.11

Point of caution: Perianal erythema due to streptococcal infection (GABHS) (see FIG. 88.4 threadworms and non-specific vulvovaginitis (see CHAPTER 99 ) can be misinterpreted as sexual abuse.

) or

FIGURE 88.4 Perianal dermatitis with erythema caused by group A betahaemolytic streptococcal infection

The crisis situation It is important to realise that the child will be in crisis. Children are trapped into the secrecy of sexual abuse, often by a trusted adult, through powerful threats of the consequences of disclosure. They are given the great responsibility of keeping the secret and holding the family together or disclosing the secret and disrupting the family. A crisis occurs when these threats become reality.

Management It is important to act responsibly in the best interests of the child. When we encounter real or suspected child abuse, immediate action is necessary. The child needs an advocate to act on its behalf and our intervention actions may have to override our relationship with the family. Some golden rules are: Never attempt to solve the problem alone. Do not attempt confrontation and counselling in isolation (unless under exceptional circumstances). Seek advice from experts (only a telephone call away). Avoid telling the alleged perpetrator what the child has said. Refer to a child sexual assault centre or Protective Services Unit where an experienced team can take the serious responsibility for the problem.

Page 1020

Supporting the child Acknowledge the child’s fear and perhaps guilt. Assure the child it is not his or her fault. Tell the child you will help. Obtain the child’s trust. Tell the child it has happened to other children and you have helped them.

Prevention of child abuse Prevention of abuse, particularly self-perpetuating abuse, can be helped by creating awareness through media attention, programs in schools and the community in general, and increased knowledge and surveillance by all professionals involved with children. Clear guidelines on reporting and the accessibility of child abuse clinics are important for the strategies to be effective. Teaching children how to protect themselves offers the greatest potential for

prevention.4

Counselling the secondary victims Non-offending parents, who are the secondary victims of the abused child, will require help and guidance from their family doctor on how to manage the crisis at home.10 Parents should be advised to reassure the child of support and safety and to maintain usual routines. The child should be allowed to set the pace, without overattention and pressure from the parents. Siblings should be informed that something has happened but that the child is safe. Ensure that the child will inform if the perpetrator attempts further abuse. Parents need substantial support, including alleviation of any guilt. An unhappy consequence of the crisis is the problem of broken relationships, which may involve the separation of the child from the family. At least one hitherto unsuspecting parent will be devastated if a parent is responsible for the abuse. The sexually abused child needs to be living with a protective parent or carer, with the abusive parent living separately.

Support for doctors The attending doctor also requires support, and sharing the problem with colleagues, mentors and family is recommended. Some helpful guidelines are as follows. Carefully record all examination findings (take copious notes). Always keep to the facts and be objective. Do not become emotionally involved. Work with (not for) the authorities. Avoid making inappropriate judgments to the authorities (e.g. do not state ‘incest was committed’, but rather say ‘there is evidence (or no evidence) to support penetration of …’). If called to court, be well prepared; rehearse presentation; be authoritative and keep calm without allowing yourself to be upset by personal affronts. The main difficulty in diagnosing child abuse is denial that it could be possible.

Adult survivors of child abuse9 All forms of child abuse can result in PTSD (from a single incident) or complex trauma (cumulative). Because of the high rate of child abuse, much of it unreported and hidden away for many years, adult survivors of child abuse are likely to be presenting to GPs on a frequent basis, often unknowingly.

The effect is more likely to be noticed in adulthood. We may encounter the sequelae of the issue, for the first time, in women in their early 30s and men in their late 60s.12 Childhood trauma, especially in the first few years during formative brain development, can lead to lifelong problems with dealing with stress. Unresolved trauma restricts the capacity to respond flexibly to life challenges. Patients may present with diverse and puzzling symptoms, including medically unexplained symptoms. Substance abuse, mood disorders and other psychological impairments may result. A GP should be aware of this possibility, and screen for trauma from child abuse in patients presenting with such issues. Take particular care with physical examination, particularly in sensitive areas or involving internal examination, and realise that many patients on whom we are doing Pap tests or rectal examinations may be survivors of child abuse. Procedures can also cause pain, and may re-traumatise such a patient. Practising in a ‘trauma informed’ manner involves paying attention to the way we perform a service or procedure, as well as what it comprises, and being sensitive to the context of what may have come before. Trauma from previous child abuse can often be helped by appropriately targeted counselling and psychological therapies.

Basic rules Suspect child abuse. Recognise child abuse. Consult the child protection authorities. Page 1021

Mandatory reporting In most states of Australia and in many areas throughout the world it is mandatory to notify the relevant statutory authorities about suspected child abuse. All family doctors should become familiar with the appropriate local legislation.

Practice tips and guidelines A child’s statement alleging abuse should be accepted as true until proved otherwise. Children rarely lie about sexual abuse. False allegations, however, are a sign of family disharmony and an indication that

the child may need help. Do not insist that the child has ‘got it wrong’, even if you find the actions by the alleged perpetrator unbelievable. Do not procrastinate—move swiftly to solve the problem. The genitalia are normal in the majority of sexually abused children. Be supportive to the child by listening, believing, being kind and caring.

When to refer Unless there are exceptional circumstances, referral to an appropriate child abuse centre, where an expert team is available, is recommended. If doubtful, relatively urgent referral to a paediatrician is an alternative. There are also the following support services: National Child Abuse Helpline. Ph: 1800 99 10 99, email: [email protected] White Ribbon Australia: www.whiteribbon.org.au 1800RESPECT. Ph: 1800 737 732

Resources The Royal Children’s Hospital Melbourne, Clinical Practice Guidelines: Child Abuse. Available from: https://www.rch.org.au/clinicalguide/guideline_index/Child_abuse/, accessed March 2021.

References 1

Australian Institute of Family Studies. Child abuse and neglect statistics, May 2013. Available from: www.aifs.gov.au/cfca/pubs/factsheets/a142086/, accessed 18 August 2014.

2

Australian Institute of Family Studies. The prevalence of child abuse, April 2017. Available from: www.aifs.gov.au/cfca/pubs/factsheets/a144254/index.html, accessed March 2021.

3

Australian Institute of Health and Welfare. Child Protection Australia 2011–2012, March 2013. Available from: https://www.aihw.gov.au/reports/child-protection/child-protectionaustralia-2011-12/contents/table-of-contents, accessed March 2021.

4

Briggs F. Smart Parenting for Safer Kids. Melbourne: JoJo Publishing, 2011.

5

Australian Institute of Family Studies. What is child abuse and neglect? September 2018.

Available from: www.aifs.gov.au/cfca/pubs/factsheets/a142091/, accessed March 2021. 6

Bird S. Child abuse: mandatory reporting requirements. Aust Fam Physician, 2011; 40(11): 921–6.

7

Kellogg N. Evaluation of suspected child physical abuse. Pediatrics, 2007; 119: 1232–41.

8

Smith A. Nonaccidental injury in childhood. Aust Fam Physician, 2011; 40(11): 858–61.

9

Blue Knot Foundation [formerly Adults surviving child abuse (ASCA)]. ASCA factsheet for general practitioners: understanding complex trauma, 2014. Available from: https://www.blueknot.org.au/Resources/Videos/Primary-Care-Practitioners, accessed March 2021.

10

Kellogg N. The evaluation of sexual abuse in children. Pediatrics, 2005; 116: 506–12.

11

Gwee A, Rimer R, Marks M. Paediatric Handbook (9th edn). Oxford: Wiley-Blackwell, 2015: 417–25.

12

Paulton R, Moffitt TE, Silva PA. The Dunedin Multidisciplinary Health and Development Study: overview of the first 40 years, with an eye to the future. Soc Psychiatry Psychiatr Epidemiol, 2015; 50(5): 679–93.

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89 Emergencies in children

We can say with some assurance that, although children may be the victims of fate, they will not be the victims of our neglect. JOHN F KENNEDY (1917–1963) Important serious emergencies in children include: trauma, especially head injuries and intra-abdominal injuries painful conditions swallowed foreign bodies (FB) respiratory problems: – bronchial asthma – epiglottitis – croup – inhaled FB – acute bronchiolitis severe gastroenteritis septicaemia (e.g. meningococcal septicaemia) myocarditis immersion poisoning bites and stings

seizures febrile convulsions sudden infant death syndrome (SIDS) and apparent life-threatening episode (ALTE) child abuse: emotional physical sexual neglect potential psychogenic disturbances anxiety/hyperventilation suicide/parasuicide

Survey by age group The author’s study analysed emergencies into three groups:1 preschool (0–5 years), primary school (6–12), adolescence (13–17). The commonest emergency calls in the 0–5 years group were poisoning, accidents and violence, dyspnoea, fever/rigors, convulsions, abdominal pain, earache, vomiting. In the 6–12 years age group: accidents and violence, dyspnoea, abdominal pain, vomiting, acute allergy, bites and stings, earache. In the 13–17 years age group: accidents and violence, abdominal pain, psychogenic disorders, acute allergy, bites and stings, epistaxis.

The signs and symptoms of a serious illness Babies who are febrile, drowsy and pale are at very high risk and require hospital admission. The busy GP will see many sick children in a day’s work, especially in the winter months with the epidemic of URTIs. It is vital to be able to recognise the very sick child who requires special

attention, including admission to hospital. It is unlikely that the commonplace robust, lustily crying, hot, red-faced child is seriously ill but the pale, quiet, whimpering child spells danger. These rules are particularly helpful in the assessment of babies under six months of age.2,3 The presence of a fever in itself is not necessarily an indication of serious illness but rather that the baby has an infection.2

The features of a very sick infant include: Inactive, lying quietly, uninterested Increased respiratory rate Increased work of breathing Noisy breathing: chest wall or sternal retraction wheezes, grunting, stridor Tachycardia Sunken eyes Cold, pale skin Cold extremities Drowsiness Poor perfusion (reduced capillary refill time) Page 1023

study4

A Melbourne of the sensitivity of clinical signs in detecting serious illness in infants identified five key signs or markers: Marker Drowsiness

Risk to baby 58%

Pallor

49%

Chest wall retraction

41%

Temperature >38.9°C or 2 cm

42%

If sepsis suspected, investigate with: blood culture FBE/ESR/CRP lumbar puncture urine culture chest X-ray Serious infectious illnesses to consider include: Haemophilus influenzae type B (Hib) infection: acute epiglottitis meningitis (now uncommon since Hib immunisation) acute bacterial meningitis septicaemia: meningococcaemia toxic shock syndrome other bacterial sepsis acute viral encephalitis acute myocarditis asthma/bronchitis/bronchiolitis pneumonia intussusception/bowel obstruction/appendicitis severe gastroenteritis

Strategic approach5 It is useful to have a systematic mnemonic for appraisal of the sick child. One general pattern (ABCDEFG) for the primary survey is:

Airway Breathing Circulation Disability (neurological assessment) Exposure Fluids: in and out Glucose Another is the ABCD assessment (see below). Blood glucose should not be overlooked: normal random range children 3.5–5.5 mmol/L neonates 2.9–7 mmol/L hyperglycaemia >12 mmol/L hypoglycaemia 2 cm other than hydrocele or umbilical hernia, significant faecal blood CNS: convulsions Skin: petechial rash

Airway and breathing5 Assessment of the airway and breathing is an important barometer of life-threatening illness and impending cardiac arrest due to hypoxia. The effort and efficacy of breathing must be assessed in the sick infant and child.

Important signs to observe with indicators Inspiratory noises with obstruction bubbly noises—partial obstruction with fluid snoring—decreased level of consciousness stridor—partial obstruction to larynx or trachea Apnoea—no respiratory effort for >20 seconds Bradypnoea—respiratory rate < normal for age Tachypnoea—rate > normal for age (indicates need to ↑ O2 and ↓ CO2) Chest recession—on inspiration Tracheal tug—downward pull on inspiration Accessory muscle use Nasal flaring Gasping—severe hypoxia; impending arrest Grunting—expiration against obstructed glottis Oxygen saturation—efficacy of breathing; use oximetry only as an adjunct to clinical assessment Cyanosis

Secondary signs of worsening obstruction

Increased respiratory rate or effort

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Decreased oxygen saturation Increasing tachycardia Deterioration of colour Development of agitation or decreased level of consciousness

Pulse oximetry and oxygen saturation The pulse oximeter measures arterial saturation of arterial blood (SpO2). In healthy young people, the SpO2 should be 97–99%. The ideal value is 97–100%. The median value in neonates is 97%, in young children 98% and in adults 98%. A level less than 95% and particularly 92% is a serious concern. The aim of treatment, including for asthma, is to keep it above 94%. Indications for investigations are presented in TABLE 89.1 Table 89.1

.

Indications for investigations in the sick child2

Urine microscopy, culture and sensitivity

All with fever

Full blood examination

All 30 minutes)

Collapse in children Collapse in children is a very dramatic emergency and often represents a life-threatening event. It is important to remember that the child’s brain requires two vital factors: oxygen and glucose. There is only a 2-minute reserve once cerebral blood flow stops. Bacterial meningitis should be considered as a cause. Important causes of collapse are presented in TABLE 89.2 of collapse. Table 89.2

. Keep in mind child abuse as a cause

Collapse in children: causes to consider

Anaphylaxis

Penicillin injection Stings

Asphyxia

Near drowning Strangulation

Airways obstruction

Asthma Epiglottitis Croup Inhaled foreign body

CNS disorders

Convulsions Meningitis Encephalitis Head injury

Severe infection

Gastroenteritis → dehydration Septicaemia Myocarditis

Hypovolaemia

Dehydration (e.g. heat) Blood loss (e.g. ruptured spleen)

Cardiac failure

Arrhythmias Cardiomyopathy

Metabolic

Acidosis (e.g. diabetic coma) Hypoglycaemia Hyponatraemia

Poisoning

Drug ingestion Envenomation

SIDS

Near-miss

Functional

Breath-holding attacks Conversion reaction Vasovagal

Note: Consider child abuse.

Initial basic management6 1. Lay child on side. 2. Suck out mouth and nasopharynx. 3. Rescue breaths. 4. Intubate or ventilate (if necessary). 5. Give oxygen 8–10 L/min by mask. 6. Pass a nasogastric tube: 0–3 years 12 FG 4–10 years 14 FG 7. Attend to circulation: IV access ?give blood, Haemaccel or N saline. 8. Take blood for appropriate investigations. 9. Consider ‘blind’ administration of IV glucose. 10. A pulse oximeter is ideal. Monitor SpO2.

Cardiopulmonary resuscitation6

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Sudden primary cardiac arrest is rare in children. It is mostly due to hypoxia. Asystole or severe bradycardia is the usual rhythm at the time of arrest. The following basic life support plan should be followed: Check breathing and pulse. Inspect oropharynx and clear any debris using suction if necessary. Basic life support for infants and children is 2 breaths and 15 compressions (with 2 rescuers). Outside the hospital setting is 30:2 compression ventilation ratio, including two initial rescue breaths. The ratio of 30:2 for one rescuer is appropriate for all ages regardless of the number of revivers present (see: www.resus.org.au/policy/guidelines/index.asp). Tilt head backwards, lift chin and thrust jaw forwards (the sniffing position). Ventilate lungs at about 20 inflations/min with bag-valve-mask or mouth to mask or mouth to mouth. An Air Viva using 8–10 L/min of oxygen is ideal if available. Intubate via mouth and secure, if necessary (must pre-oxygenate). If intubation not possible, use a needle cricothyroidotomy as an emergency. Start external cardiac compression immediately if pulseless or 6 cm long, wider > 2.5 cm Magnet + metal object > 1 magnet Lead-based objects (lead toxicity if impacted) Abnormal GI tract, e.g. previous surgery, TOF

A golden rule The natural passage of most objects entering the stomach can be expected. Once the pylorus is traversed the FB usually continues. This includes: coins buttons sharp objects open safety pins glass (e.g. ends of thermometers) drawing pins Special cases are: very large coins: watch carefully hair clips (usually cannot pass duodenum if under 7 years)

Management

Manage conservatively. X-ray all children (mouth to anus, especially chest and abdomen) on presentation (the oesophagus is a concern). Investigate unusual gagging, coughing and retching with X-rays of the head, neck, thorax and abdomen (check nasopharynx and respiratory tract). Watch for passage of the FB in stool (usually 3 days). Defecate into a container. If not passed, order X-ray in 1 week. If a blunt FB has been stationary for 1 month without symptoms, remove at laparotomy.

Button and disc battery ingestion If not in stomach, these (especially lithium batteries) create an emergency if in the oesophagus because electrical current generated destroys mucous membranes and perforates within 6 hours (must be removed endoscopically ASAP). This also applies to the ear canal and nares.

Fever11 Fever, which is commonly defined as a temperature >38°C, is a significant symptom in a child, especially in neonates (see CHAPTER 42 ). If an infant aged less than 3 months presents with a fever but no focus of infection, consider bacteraemia. One recommendation is that all febrile neonates should have a full septic work-up and be admitted for parenteral antibiotics. Page 1028

Not to be missed Meningitis/encephalitis Sepsis Pneumonia Osteomyelitis/septic arthritis Pertussis Urinary infection (especially if no focus)

Febrile convulsions Diagnosis based on presence of fever, short duration and no clinical evidence of CNS pathology.

Clinical features

The commonest cause is a URTI (e.g. the common cold or similar viral syndrome). About 4 per 100 incidence in children. Rare under 6 months and over 5 years. Commonest age range 9–20 months. Recurrent in up to 50% of cases. Consider meningitis and lumbar puncture after first convulsion if under 2 years or cause of fever not obvious. Epilepsy develops in about 2–3% of such children.

Management of the convulsion (if prolonged >10–15 minutes), also for status epilepticus Undress the child to singlet and underpants or light clothing but take care to prevent the child getting cold. Maintain the airway and prevent injury. Place patient chest down with head turned to one side. Oxygen 8 L/min by mask. Give midazolam or diazepam. Give midazolam by one of four routes: IV 0.15 mg/kg, IM 0.2 mg/kg, buccal 2–5 mg/dose or (preferable) intranasal 0.3 mg/kg undiluted (use 1 mL of drops from vial) Buccal and nasal routes slower response—IM may be most practical. Give diazepam by one of two routes: IV 0.2 mg/kg, undiluted or diluted (10 mg in 20 mL N saline) or rectally 0.5 mg/kg (dilute with saline or in pre-prepared syringe) up to 10 mg or with suppository or rectal gel. Note: Although the IV route is preferred, the rectal route is ideal in a home or office situation; for

example, consider a 2-year-old child (weight 12 kg) with a persistent febrile convulsion. The dose of diazepam injectable is 0.5 mg/kg, so 6 mg (1.2 mL) of diazepam is diluted with isotonic saline (up to 10 mL of solution) and the nozzle of the syringe pressed gently but firmly into the anus and injected slowly. Observe carefully for respiratory depression. rectal paracetamol 15 mg/kg statim The anti-epileptics can be repeated in 5 minutes if fitting continues. Check blood glucose: if 8 years (take care with viscera). A good rule is the rule of 5s—5 breaths, 5 back blows, 5 chest thrusts, 5 abdominal thrusts (older child). If unresponsive, start CPR and seek help If complete obstruction—attempt removal of the FB with forceps. If unsuccessful, perform a tracheostomy or cricothyroidotomy. Note: Once an FB has passed through the larynx it is very rare for there to be an immediate threat to life, so referral is usually quite safe. Note: Do not instrument the airways if the child is coping. Bronchoscopy Bronchoscopy is necessary in almost every child where there is a strong suggestion of an inhaled FB. It is difficult and requires an expert with appropriate facilities.

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Prevention No child 2000 IU/L, an IUP should be visible on vaginal ultrasound. If the uterus is empty, ectopic is more likely. If LIF) Often described as a ‘horse kick pain’ Pain tends to move centrally (see FIG. 95.3

)

Heavy feeling in pelvis Relieved by sitting or supporting lower abdomen Pain lasts from a few minutes to hours (average 5 hours) Patient otherwise well

FIGURE 95.3 Typical clinical features of a ruptured Graafian follicle (mittelschmerz) Note: Sometimes it can mimic acute appendicitis.

Management Explanation and reassurance Simple analgesics: aspirin or paracetamol ‘Hot-water bottle’ comfort if pain severe

Ovarian tumours

Benign ovarian tumours, particularly ovarian cysts, may be asymptomatic but will cause pain if complicated. They are common in women under 50 years of age. Ovarian cysts are best defined by transvaginal ultrasound, which can identify whether haemorrhage has occurred inside or outside the cyst.

Symptoms Pain (usually torsion or haemorrhage) Pressure symptoms (e.g. bloating, difficulty emptying bladder or bowel)

Ruptured ovarian cyst The cysts tend to rupture just prior to ovulation or following coitus.

Clinical features Patient usually 15–25 years Sudden onset of pain in one or other iliac fossa May be nausea and vomiting No systemic signs Pain usually settles within a few hours

Signs Tenderness and guarding in iliac fossa PR: tenderness in rectovaginal pouch

Investigation Transvaginal ultrasound

Management Appropriate explanation and reassurance Conservative: simple cyst 4 cm).

Diagnosis Pelvic ultrasound with transvaginal and transabdominal views Tumour markers such as CA-125, β-hCG (choriocarcinoma), human epididymis protein 4 (HE4) and alpha-fetoprotein should be measured only if the ultrasound raises suspicion of malignancy.12 Refer urgently to gynaecologist. Treatment is usually laparoscopy ± adjuvant chemotherapy.

Dysmenorrhoea Dysmenorrhoea (painful periods) may be functional in the absence of organic disease (known as primary dysmenorrhoea). Primary dysmenorrhoea is more common in adolescent women. Secondary dysmenorrhoea is painful menstruation that is due to pelvic pathology.

Primary (functional) dysmenorrhoea This is menstrual pain associated with ovular cycles without any pathologic findings. The pain usually commences within 1–2 years after menarche and becomes less severe with age. It affects about 50% of menstruating women and up to 95% of adolescents.

Clinical features Low midline abdominal pain Pain radiates to back or thighs (see FIG. 95.5

)

Varies from a dull dragging to a severe cramping pain Maximum pain at beginning of the period

May commence up to 12 hours before menses appears Usually lasts 24 hours but may persist for 2–3 days May be associated with nausea and vomiting, headache, syncope or flushing No abnormal findings on examination

FIGURE 95.5 Typical pain of dysmenorrhoea

Management Full explanation and appropriate reassurance Promote a healthy lifestyle: regular exercise avoid smoking and excessive alcohol Recommend relaxation techniques such as yoga Avoid exposure to extreme cold

Place a hot-water bottle over the painful area

Medication Options include (trying in order): simple analgesics (e.g. aspirin or paracetamol) NSAIDs naproxen 500 mg (o) initially then 250 mg every 6–8 hours (max. 1250 mg/day) or ibuprofen 200–400 mg (o) tds (max. 1600 mg/day) commence at first suggestion of pain in the first 3 days of the period combined oral contraceptive pill If not helped by these treatments, further investigation is required for a possible secondary cause.

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Secondary dysmenorrhoea Secondary dysmenorrhoea is menstrual pain for which an organic cause can be found. The pain begins as a dull pelvic ache 3–4 days before menses and becomes more severe during menstruation. Commonest causes: endometriosis, adenomyosis (a major cause) PID submucous myoma intra-uterine polyp pelvic adhesions

Investigations Investigations include ultrasound and laparoscopy. Management involves treating the cause. Consider medical management for possible endometriosis.

Endometriosis

Endometriosis is a chronic inflammatory gynaecological condition caused by hormonedependent growth of endometrial-like tissue outside of the uterus, usually on the peritoneum and ovaries.13 Deposits may be superficial or infiltrating, causing haemorrhage, adhesions and ultimately dense scar tissue changes. Endometriosis infiltrating the myometrium is referred to as adenomyosis. Endometriosis is the most common single cause of chronic pelvic pain in women.14 The average time to diagnosis is 6.5 years. Patients experience varying degrees of symptoms according to the site and severity of the endometriosis deposits. The diagnosis may be masked by the COC pill. Pregnancy is beneficial but recurrence can follow.

Clinical features15 5–10% incidence Puberty to menopause, peak 25–35 years Possible family history (3–10-fold increase risk in first-degree relatives)13 Dysmenorrhoea Gastrointestinal symptoms during menses (e.g. painful defecation, diarrhoea) Urinary symptoms: dysuria, frequency Pain may radiate to lower back, legs or rectum Subfertility Dyspareunia Non-specific pelvic pain Heavy menstrual bleeding Premenstrual spotting Acute pain with rupture of endometrioma Page 1098

DxT dysmenorrhoea + heavy menstrual bleeding + dyspareunia = abdominal/pelvic pain → endometriosis

Possible signs

Fixed uterine retroversion Tenderness and nodularity in the pouch of Douglas/retrovaginal septum Uterine enlargement and tenderness

Diagnosis Consider endometriosis in any woman with painful periods that do not respond to NSAIDs and significantly interfere with daily functioning The gold standard of diagnosis is by direct visual inspection at laparoscopy Diagnostic laparoscopy is not always required and a presumed clinical diagnosis may be appropriate14 Transvaginal pelvic ultrasound may identify adenomyosis, ovarian endometrioma or rectal endometriosis (normal findings do not exclude diagnosis)

Treatment13 Careful explanation Approach depends on the patient’s age, impact and severity of symptoms and family planning Options include analgesia, hormonal and surgical treatment Analgesia (first line): NSAIDs, paracetamol or combination Hormonal (aims to suppress the disease process): combined hormonal contraception: oral contraceptive pill or vaginal ring, consider extended or continuous use progestogens: levonorgestrel-releasing IUD 52 mg (Mirena), 5 yearly dienogest 2 mg (o) daily norethisterone 5–10 mg (o) daily (up to 10 mg bd) medroxyprogesterone acetate (depot) 150 mcg IM 12 weekly GnRH analogues, e.g. goserelin 3.6 mg SC implant every 28 days or nafarelin 200 mcg intranasally bd for up to 6 months (requires specialist advice) hormonal therapy is often used post surgery to prevent recurrence

Surgical: Laparoscopy is indicated for diagnosis and excision/ablation of disease, especially if there is associated infertility. General principle is ‘the first go is the best go’14 as there is the risk of scarring from repeated procedures. Recurrence of symptoms is common. Hysterectomy may be recommended. Many patients will develop chronic pelvic pain, which requires a multidimensional approach (see below).

Pelvic adhesions Pelvic adhesions may be the cause of pelvic pain, infertility, dysmenorrhoea and intestinal pain. They can be diagnosed and removed laparoscopically when the adhesions are well visualised and there are no intestinal loops firmly stuck together.

Chronic/persistent pelvic pain (PPP) Chronic or persistent pelvic pain (PPP) is defined as non-cyclical pelvic pain present for at least six months that is severe enough to cause functional disability or require treatment.16 PPP is a challenging presentation which can be viewed as a complex neuromuscularpsychosocial disorder. Multiple systems can be involved, including gynaecological, urological, gastrointestinal, musculoskeletal and psychological.16 There is a high incidence of associated mental illness, past trauma and post-traumatic stress disorder. It is important to identify and ensure adequate management of treatable causes. Common causes of chronic pelvic pain are listed in TABLE 95.3 . A feature of chronic pain is central sensitisation, which causes hyperalgesia and alodynia (see CHAPTER 82 ).

Table 95.3

Causes of chronic lower abdominal and pelvic pain in women2,17

Genital Endometriosis/adenomyosis Pelvic inflammatory disease (chronic; adhesions) Ovarian pathology Prolapse Vulvodynia (dysaesthetic vulvodynia, vulvar vestibular syndrome) Chronic vulvovaginal candidiasis Pudendal neuralgia

Fibromyomata (rarely) Non-genital Adhesions Inflammatory bowel disease Diverticular disease Irritable bowel syndrome Pelvic congestion syndrome Urinary disorders, e.g. interstitial cystitis

Features Affects 15–25% of women Endometriosis causes 33%, adhesions 24% Reason for up to 40% of laparoscopies, 5% of hysterectomies Possible symptoms:3 dysmenorrhoea/cyclical aggravation of pain dyspareunia difficulty inserting tampons or menstrual cups vulvovaginal irritation or pain bladder symptoms (frequency, dysuria, urgency) irritable bowel (bloating, alternating bowel habit) sensitivity to light touch in the lower abdominal or genital region musculoskeletal involvement (pain with movement or prolonged positions)

Page 1099

psychosocial difficulties (social withdrawal, hypervigilance to pain, low self-confidence)

Management3 Like all chronic pain, a multidimensional approach is indicated (see CHAPTER 82 ). A reduction of pain with improved function and well-being may be more achievable than cure. A helpful approach is to address four components of PPP—pelvic organ pain, pelvic muscle

pain, central sensitisation and psychological sequelae: Pelvic organ pain minimise the number of periods if there is dysmenorrhoea (hormonal contraceptives, GnRG agonists) hysterectomy is not considered a cure avoid repeated laparoscopies avoid bladder irritants (caffeine, acidic drinks, stay hydrated) treat symptoms of overactive bladder (see CHAPTER 65 ) treat vulvovaginal irritation (see CHAPTER 99 ) treat symptoms of irritable bowel (see CHAPTER 34 ) Pelvic muscle pain keep active, avoid aggravating activities pelvic physiotherapy botulinum toxin injection for severe cases Central sensitisation (see CHAPTER 82 ) patient education exercise relaxation strategies optimisation of sleep neuropathic pain medications—amitriptyline, SNRIs, gabapentinoids Psychological sequeale re-enagement with family, friends and community psychological therapy

Pudendal neuralgia3 Pudendal neuralgia causes a burning or sharp pain in the ‘saddle’ area, anywhere from the clitoris back to the anal area, often when sitting. It may be uni- or bilateral and may be associated with increased clitoral arousal. There is often associated bladder or bowel irritation. Causes18 Childbirth trauma Injury or pelvic/perineal trauma Gynaecological or colorectal surgery Cycling Excessive physical exercise Musculoskeletal issues Straining with bowel or bladder emptying Note: There is usually a combination of causes. Management Avoiding activities that compress the nerve, such as cycling

Modify sexual practices to avoid pain A ‘U-shaped’ foam cushion with the front and centre area cut out when sitting Pelvic physiotherapy to relax and/or stretch pelvic muscles and reduce pressure on pudendal nerve Avoid straining with bowels or bladder TENS machine Neuropathic pain medications Interventional management:18 Injections such as cortisone, hyaluronic acid, botox, platelet-rich plasma Pulsed radiofrequency treatment Pudendal nerve release surgery Implantable neuromodulation devices

When to refer All cases of ‘unexplained infertility’ Patients with suspected endometriosis, not responding to analgesia or hormonal therapy Severe pelvic pain in pregnancy

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Pregnancy of unknown location Patients with positional dyspareunia Symptomatic or complex ovarian cysts Patients with complex or chronic pelvic pain

Practice tips Think of endometriosis and ovarian cysts in any woman with lower abdominal pain. In any woman whose normal activities are disturbed by dysmenorrhoea unrelieved by NSAIDs, endometriosis should be suspected. After surgical emergencies have been excluded in a young woman with acute pelvic pain, consider PID. A positive β-hCG plus an empty uterus and an adnexal mass are the classic diagnostic features of ectopic pregnancy.

Patient education resources

Hand-out sheets from Murtagh’s Patient Education 8th edition: Dysmenorrhoea (painful periods) Endometriosis Pelvic inflammatory disease

References 1

Endometriosis Australia. Endo Facts (2020). Available from: www.endometriosisaustralia.org/research, accessed April 2021.

2

Soo Keat Khoo. Lower abdominal pain in women. Patient Management (Suppl), 1990; August: 13–23.

3

Evans S. Management of persistent pelvic pain in girls and women. Aust Fam Physician, July 2015; 44(7): 454–9.

4

Engeler D et al. European Association of Urology. Guidelines on Chronic Pelvic Pain. Available from: http://uroweb.org/wp-content/uploads/EAU-Guidelines-Chronic-PelvicPain-2015.pdf, accessed May 2021.

5

Forbes KL. Lower abdominal and pelvic pain in the female: a gynaecological approach. Modern Medicine Australia, 1991; September: 24–31.

6

Royal Australasian College of Physicians (RACP). Genital Examination of Young Girls (April 2018). Available from: https://www.racp.edu.au/docs/default-source/advocacylibrary/genital-examinations-in-girls-and-young-women-a-clinical-practice-guideline.pdf, accessed April 2021.

7

O’Connor V, Kovacs G. Obstetrics, Gynaecology and Women’s Health. Cambridge: Cambridge University Press, 2003: 325–7.

8

Porter R, Kaplan J. The Merck Manual of Diagnosis and Treatment (19th edn). Whitehouse Station, 2011: 2664–5.

9

Genital and sexually transmitted infections [published 2019]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2019. www.tg.org.au, accessed April 2021.

10

Australian Sexual Health Alliance. Australian STI Management Guidelines For Use in Primary Care. Available from: www.sti.guidelines.org.au, accessed April 2021.

11

Dynan L. Pelvic inflammatory disease. Aust Fam Physician, Nov 2006; (35)11: 858–62.

12

Yeoh M. Investigation and management of an ovarian mass. Aust Fam Physician, Jan/Feb 2015; (44)1: 48–52.

13

Endometriosis. [published 2020]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2020. www.tg.org.au, accessed May 2021.

14

Abbott J. A pragmatic approach to surgical management of endometriosis. O&G Magazine, 2019; 21(2).

15

Johnson NP, Hummelshoj L, The World Endometriosis Society Montpellier Consortium. Consensus on current management of endometriosis. Hum Reprod, 2013; 28(6): 1552–68.

16

Saha S. What else could it be? Causes of pelvic pain. O&G Magazine, 2019; 21(2).

17

Stone K. Scope of medical imaging for pelvic pain. O&G Magazine, 2019; 21(2).

18

Women’s Health & Research Institute of Australia. Pudendal neuralgia. Available from: www.whria.com.au/for-patients/pelvic-pain/pudendal-neuralgia/, accessed May 2021.

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96 Premenstrual syndrome

Shivering, lassitude and heaviness of the head denotes the onset of menstruation ... mistiness of vision is relieved by menstruation. HIPPOCRATES, 400 BCE Premenstrual syndrome (PMS) is defined as a disorder of non-specific somatic, psychological or behavioural symptoms occurring during the late luteal phase of the menstrual cycle.1 The pathogenesis of PMS is still uncertain. Among the proposed causes are pyridoxine deficiency, excess prostaglandin production, hypoglycaemia and neurotransmitter (in particular, GABA) abnormalities. However, PMS is most probably related to enhanced sensitivity to progestogen with an underlying serotonin deficiency.2

Key facts and checkpoints PMS increases in incidence after 30 years, with a peak incidence in the 30–40 years age group. PMS also occurs in the 45–50 years age group, when it may alternate with menopausal symptoms, causing clinical confusion.3 The symptoms of PMS decrease in severity just before and during menstruation. The symptoms cannot be explained by the presence of various psychological or psychiatric disorders. The severe form of PMS is classified in the Diagnostic and Statistical Manual of Mental Disorders (4th and 5th edns) as premenstrual dysphoric disorder (PMDD).

Incidence Up to 90% of women may experience premenstrual symptoms, which can vary from moderately

severe in 20–40% of women to disabling in 2–9%.4 And perhaps 2–5% of women experience symptoms so debilitating as to significantly reduce their quality of life. In this case, they are considered to have premenstrual dysphoric disorder (PMDD).1

Symptoms Various symptoms from among the 150 reported are summarised in FIGURE 96.1

FIGURE 96.1 Symptoms of premenstrual syndrome

.

Symptoms usually reach a peak during the last 5 premenstrual days and remit within a few days of menstruation. Typically, they recur in subsequent cycles, although variation between cycles is common. The most common psychological symptoms are depression and irritability, while headache, bloating and breast tenderness are the most common physical symptoms.

Classification of PMS It is convenient to classify PMS in terms of severity of symptoms.3 1. Mild: symptoms signal onset of menstruation. No medical advice sought or needed. 2. Moderate: symptoms annoying but insufficient to interfere with function at home or work. Medical advice sought in about one-third. 3. Severe: symptoms are such that functions at work or home are disrupted. Medical advice is usually sought. This disruptive form is labelled PMDD (see TABLE 96.1 ). Table 96.1

Summary of PMDD criteria*5

A

Symptoms must occur during the week before menses and start to improve within a few days after onset of menses

B

One (or more) of the following symptoms must be present and marked: 1. Affective lability 2. Irritability or anger 3. Depressed mood or dysphoria 4. Anxiety, tension or feelings of being on edge

C

One or more of the following to reach a total of 5 symptoms when combined with those from criterion B above: 1. Decreased interest in usual activities 2. Concentration difficulties 3. Marked lack of energy; lethargy 4. Marked change in appetite, overeating or food cravings 5. Hypersomnia or insomnia 6. Feeling overwhelmed or out of control 7. Other physical symptoms (e.g. breast tenderness, bloating)

D

Symptoms must interfere with work, school, productivity, usual activities or relationships

E

Symptoms must not merely be an exacerbation of another disorder such as major depression

F

Criteria for A must be confirmed by prospective daily ratings for at least two

cycles G

The symptoms are not attributable to the physiological effects of a substance or other medical condition

*Adapted from DSM-5-TR

Page 1102

Differential diagnosis3 Endometriosis Menopause Mastalgia Other causes of fluid retention—kidney or adrenal Thyroid disorder (hyper- or hypoactivity) Anaemia Polycystic ovarian syndrome Psychiatric disorders: depression, mania

Diagnosis Thorough history—including diet, exercise habits, psychosocial background, emotional influences and family history Menstrual calendar—for 3 months, showing timing of the three main symptoms3 Physical examination to exclude gynaecological, endocrine or other systemic disease; and also include: breast examination (if breast tenderness) cervical screening test Investigations (to exclude other causes): thyroid function tests full blood count electrolytes and creatinine

FSH and oestradiol—if perimenopause suspected serum androgens—if oligomenorrhoea present

Management2,4 The basic aim of management is to reassure and treat the woman in such a way that she makes changes in her lifestyle to cope with the hormonal dysfunction rather than rely on medication. The management strategies include the following, with the emphasis on lifestyle factors.

Explanation, reassurance and insight Cognitive-based therapy, which has been shown to have a positive effect in several RCTs,4 is very helpful for the patient to understand the nature of their symptoms and to receive appropriate support and empathy. The patient may wish to inform family and close friends, to encourage extra support while symptoms occur.

Keeping a diary3 Advise the patient to keep a daily diary of all her symptoms and when they occur over a 2–3 month period. This information should help her to plan around her symptoms: for example, avoid too many social events and demanding business appointments at the time when PMS symptoms are worst.

Dietary advice1 Advise the patient to eat regularly and sensibly; eat small, frequent meals and aim for ideal weight. Increase amount of low-GI complex carbohydrates, leafy green vegetables and legumes. Decrease or avoid: refined sugar, salt, alcohol, caffeine (tea, coffee, chocolate), tobacco, red meat and excessive fluid intake during premenstrual phase. Decrease total protein to 1 g/kg/day; decrease fats.6

Exercise Recommend a program of regular exercise such as swimming, aerobics, jogging or tennis. Such exercise has been proven to decrease depression, anxiety and fluid retention premenstrually.6 Page 1103

Relaxation Advise patients to plan activities that they find relaxing and enjoyable at the appropriate time. Consider stress reduction therapy, including meditation, yoga, relaxation techniques and appropriate counselling.

Appropriate dress Advise sensible dressing to manage breast tenderness and a bloated abdomen, such as a firmfitting bra and loose-fitting clothes around the abdomen.

Medication Pharmaceutical agents that have been used with success in some patients and little or no relief in others include diuretics (e.g. spironolactone), vitamins and minerals (e.g. pyridoxine and evening primrose oil), simple anti-inflammatories (e.g. aspirin, mefenamic acid) and hormonal preparations such as the combined oral contraceptive (COC). A combination of agents may have to be used. It is worth noting that progestins used alone are ineffective and may even aggravate symptoms, but can be used in conjunction with oestrogen to protect the endometrium.4 Supplements Women often enquire about the use of vitamins, minerals and herbal remedies for PMS. Evidence to support the use of complementary and alternative medicines is limited. The following may be considered:1,7 pyridoxine/vitamin B6 up to 100 mg daily (beware nerve damage to hands/feet with higher doses) elemental calcium, 1200 mg to 1500 mg daily (two randomised controlled trials have shown significant benefit) elemental magnesium up to 400 mg daily (minimal evidence) evening primrose oil 500 mg daily (no benefit over placebo) Agnus castus (Premular) is an extract of the berries from the chaste tree (small randomised controlled trials have indicated some benefit over placebo) Oral contraception4 It is appropriate to use a COC-containing ethinyloestradiol and drospirenone since a metaanalysis of drospirenone, which is a progestogen derivative of spironolactone, concluded that it was effective in reducing the severe symptoms of PMDD. Extended/continuous use or shorter hormone-free intervals may benefit mood symptoms. ethinyloestradiol 20 mcg + drospirenone 3 mg (o) once daily on days 1–24 of a 28-day cycle Moderate to severe symptoms4,8 A trial of an SSRI or SNRI is warranted in women with incapacitating PMDD who are

unresponsive to other treatments. SSRIs are most commonly used but SNRIs are also effective. No one drug is considered more effective than another. fluoxetine 20 mg mane for 14 days before the anticipated onset of menstruation4 or sertraline 50 mg daily for 14 days before the anticipated onset of menstruation

Practice tip Moderate to severe PMDD: fluoxetine 20 mg (o) or sertraline 50 mg (o) daily in morning for 14 days before anticipated onset of menstruation and through to the first full day of menses of each cycle

When to refer3 If the recommended approach of support, education, reassurance and stress management is still not effective. Consider specialist referral if symptoms are severe or do not respond to other therapies. Refer to a gynaecologist if underlying disease is suspected or proven (e.g. polycystic ovarian syndrome, endometriosis). Refer to an endocrinologist if an endocrine disorder such as adrenal, pituitary or thyroid is suspected or proven. Refer to a psychiatrist if depression worsens, is not cyclical or psychosis develops.

Practice tips Keeping a daily diary of symptoms is very helpful for both patient and clinician. Aim for lifestyle changes and commonsense non-pharmacological management. Allow at least three cycles of treatment to provide reasonable time for a particular medication to take effect. Be careful of overdiagnosing PMS and overlooking disorders such as depression, which may be exacerbated in the premenstrual phase.

Patient education resource Hand-out sheet from Murtagh’s Patient Education 8th edition:

Page 1104

Premenstrual syndrome

Resources National Association of Premenstrual Syndrome: www.pms.org.uk

References 1

Foran T. Management of premenstrual syndrome: how to treat. Australian Doctor, 28 November 2013.

2

Mazza D. Women’s Health in General Practice (2nd edn). Sydney: Elsevier, 2011: 16–21.

3

Smith M. Premenstrual syndrome. In: MIMS Disease Index. Sydney: IMS Publishing, 1991–92: 439–41.

4

Premenstrual syndrome and premenstrual dysphoric disorder [published 2020]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2020. www.tg.org.au, accessed April 2021.

5

Premenstrual dysphoric disorder. In: Diagnostic and Statistical Manual of Mental Disorders (5th edn). Arlington: American Psychiatric Association, 2013: 191–4.

6

Papadakis MA, McPhee SJ. Current Medical Diagnosis and Treatment (52nd edn). New York: The McGraw-Hill Companies, 2013: 749–50.

7

Wyatt K et al. Premenstrual syndrome. Clinical Evidence, 2000; 4: 1121.

8

Brown J et al. Selective serotonin reuptake inhibitors for premenstrual syndrome. Cochrane Database Syst Rev, 2009 Apr 15; Issue 2: Art No. CD006586 (PMID 193706 44).

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97 The menopause

Every woman should use what Mother Nature gave her before Father Time takes it away. LAURENCE J PETER, 1977

Definitions The WHO has defined the menopause as signifying the permanent cessation of menstruation, resulting from the loss of ovarian follicular activity.1 In most Western women, it occurs between the ages of 45 and 55 years, with an average age of 51.5 years.2 Premature menopause (or premature ovarian insufficiency) is menopause occurring before age 40, while early menopause occurs before 45. The term is used in a broader sense to include the perimenopausal phase when ovarian function waxes and wanes and the periods become irregular. This may last 2–5 years and sometimes longer and involves the premenopausal and menopausal phases. The postmenopause is the period following the menopause but cannot be defined until after 12 months of spontaneous amenorrhoea, except in women who have had an oophorectomy. Surgical menopause is known as bilateral oophorectomy.

Summary The climacteric can be subdivided into four phases: Phase 1 Premenopausal: up to 5 years before the last menstrual period. Phase 2 Perimenopausal: the presence of early menopausal symptoms with changes in menstrual cycle. Phase 3 Menopausal: the last menstrual period. Phase 4 Postmenopausal: the phase beginning 12 months after the last menstrual cycle.

Osteoporosis Osteoporosis, which literally means ‘porous bone’, is reduced bone mass per unit volume. Osteoporosis is usually addressed in the context of the menopause because the drop in oestrogen levels causes accelerated loss of bone mass (10% the first 5 years after menopause). Osteoporosis is diagnosed on the presence of a fragility fracture, when a fracture occurs following a fall from standing height or less, that would not be expected under normal circumstances. It is also defined by bone mineral density (BMD) as a T score of ≤–2.5 (see CHAPTER 81 ). Loss of bone density can be largely prevented by correcting oestrogen deficiency.

Physiology of the menopause provides an overview of how menopausal symptoms are related to ovarian follicular activity and hormonal activity. FIGURE 97.1

FIGURE 97.1 Schematic representation of some clinical, biological and endocrinological features of the perimenopausal and postmenopausal phases Source: Reproduced with permission from Burger H. Talking women: HRT and breast cancer risk. Medical Observer, 1 August

2008: 40.

Women are born with their total complement of ovarian primary follicles. The peak number is reached at about 20 weeks’ gestation, while in their mother’s womb, with an average number of 6 million. From this point, a female constantly loses gametes, reaching 1 million at birth and about 400 000 by puberty. A woman will ovulate 400–500 times in her lifetime and will begin the menopause transition when there are only a few thousand follicles remaining. The number of follicles declines rapidly as the menopause approaches, with an insufficient number to stimulate cyclical activity. Oestrogen levels fall and this has a positive feedback on the pituitary, increasing FSH levels to 10–15 times that of the follicular phase of the cycle, while LH levels rise about threefold. The ovary secretes minimal oestrogen but continues to secrete significant amounts of androgens. Reduced oestrogen is the main cause of menopausal symptoms such as hot flushes.

Clinical features In the early menopausal transition, the cycle length may vary by about 7 days (e.g. a 28-day cycle becomes 21). The late menopausal transition is characterised by two or more skipped menstrual cycles.4 Variability of the heaviness of periods is common and the pattern can be erratic, with prolonged bleeding episodes or scanty menses, interspersed with regular ovarian activity. Uncommonly, women may experience regular periods until complete cessation. Because small amounts of oestrogen are still being produced in the adrenal glands, Page 1106 symptoms other than cessation of periods may be mild or absent. Around 20% will have no symptoms at all, while another 20% will be severely affected, with symptoms continuing into their 60s or later.5 Up to 80% of women experience vasomotor symptoms for an average duration of 5 years (range 1–10 years).3

Hot flushes The classic vasomotor symptom of menopause is the hot flush. A hot flush lasts 1–2 minutes, begins in the chest and then spreads up over the face and body. There may be associated redness, sweats which can be drenching, panic attacks, palpitations and faintness. Fever is a differential diagnosis. During a menopausal flush, skin temperature increases, but core temperature remains stable.

Symptoms Vasomotor:1 hot flushes (80%) night sweats (70%) palpitations (30%)

lightheadedness/dizziness migraine Psychological: irritability depression anxiety tearfulness loss of concentration poor short-term memory unloved feelings sleep disturbances mood changes loss of self-confidence decline in libido Urogenital (60%): atrophic vaginitis vaginal dryness (45%); itching; burning dyspareunia/sexual dysfunction bladder dysfunction (e.g. frequency, dysuria) stress incontinence/prolapse Musculoskeletal: non-specific muscular aches non-specific joint aches and pains Skin and other tissue changes: dry skin

formication (17%) new facial hair breast glandular tissue atrophy Other: unusual tiredness headache Page 1107

Clinical approach A thorough evaluation of the patient is important, including a good history.

History Enquire about any symptoms related to oestrogen deficiency, with an emphasis on the menstrual history and vasomotor symptoms. Take a general medical and gynaecological history, including sexual history, contraception, smoking, drug and alcohol, sleep, micturition and social history, including relationships. Enquire about mental state symptoms, such as irritability, depression, anxiety and loss of self-esteem. Ask how the symptoms are affecting quality of life, particularly sleep disturbance. A symptom score card is a helpful aid. Consider all women at the menopause to be at risk for cardiovascular disease, cancer (especially breast, ovary and cervix), diabetes and osteoporosis.2 Information on family history of osteoporosis, cancer and cardiovascular disease should be sought.

Physical examination The general examination should include measurement of blood pressure, weight, height and waist circumference. Consider breast examination, abdominal palpation, vaginal examination and cervical screening test (if due).

Investigations2 Apart from a cervical screening test, the following tests should be considered: full blood count and iron studies (if heavy or abnormal menstrual bleeding) fasting lipids including HDL and fasting glucose liver function tests

thyroid stimulating hormone (TSH) urinalysis (if urinary symptoms) screening mammography (if due) transvaginal ultrasound (if abnormal vaginal bleeding) bone density study (if risk factors)

Diagnosis Menopause is a retrospective diagnosis, which can be made after 12 months of amenorrhoea in women over 50 and after 2 years in women younger than 50. The diagnosis of perimenopause is made by taking a thorough menstrual history. Investigating hormone levels is usually unnecessary and often unhelpful, with frequent fluctuations during perimenopause. Testing may be appropriate for younger patients (30).

Differential diagnosis of menopause syndrome Depression Anaemia Thyroid dysfunction Hyperparathyroidism Diabetes Medications (e.g. SSRIs) Gynaecological disorders dysfunctional uterine bleeding

Management Education and lifestyle Patients should receive adequate understanding, support and explanation, with the emphasis being that the menopause is a natural life transition. It is also important to provide education on long-term health implications, such as cardiovascular disease risk and osteoporosis. Always consider this as an opportunity to holistically review a woman’s health and reinforce the

importance of a healthy lifestyle, including: healthy, balanced diet maintain a healthy weight adequate relaxation adequate exercise (especially weight-bearing) adequate calcium intake (3 serves per day) smoking cessation safe alcohol intake

Menopausal hormone therapy Page 1108 The principle indication of menopausal hormone therapy (MHT) is for the relief of troublesome vasomotor symptoms. The regimen is tailored to the individual patient and depends on several factors, including previous hysterectomy, menopausal phase, predominant symptoms and age. MHT has also been known as hormone replacement therapy (HRT).

The Women’s Health Initiative study6 The arguably flawed Women’s Health Initiative (WHI) trial investigated the use of long-term MHT in postmenopausal women with an intact uterus using long-term combined oral oestrogen and progesterone. The study raised concerns about the safety of MHT, with an increased risk of breast cancer (1.26-fold), coronary heart disease (1.29-fold), stroke (1.41-fold) and pulmonary embolism (2.13-fold) with prolonged use greater than 5 years.7 The study also found a reduction in risk of bowel cancer and fracture in these women, and reduction of breast cancer risk in women using oestrogren-only MHT. The trial did not include people using other forms of MHT, such as patches, gels or implants. The recently published long-term follow-up of WHI participants found no difference in the rate of all-cause mortality between women randomised to MHT or placebo. Moreover, for women aged 50–59 years who were randomised to MHT, the hazard ratio for all-cause mortality during the intervention phase of the study was 0.69.8 Studies show that the influence of the WHI study has resulted in women being inappropriately discouraged from the use of MHT.9

Benefits and risks of MHT MHT is the most effective method for relieving distressing symptoms such as hot flushes, urogenital symptoms, sleeplessness and joint symptoms (level I and II evidence) (NHMRC

criteria).10 Risks differ depending on the age of the woman and the route of administration. The recent National Institute for Health and Care Excellence (NICE) guideline on menopause management concluded the following:9 MHT was the most appropriate treatment for menopausal symptoms. MHT improved bone density and reduced fracture. CVD risk was not increased among women using MHT and may be reduced (in normal target population). Venous thromboembolism (VTE) risk was increased among women using oral MHT but not among women using non-oral therapy. Breast cancer risk was not increased for women taking oestrogen but was increased with longduration use in women taking combined oestrogen and progestin therapy. The effect reduced after ceasing therapy. In regard to breast cancer risk, studies suggest that other progestogens, specifically micronised progesterone and dydrogesterone, may be associated with a lower risk than medroxyprogesterone acetate. For women with a high risk of breast cancer, even for those with BRCA mutations, there is no evidence of a greater increase in risk with MHT than that observed with MHT in the general population.11 The International Menopause Society advises that MHT carries few risks when prescribed for symptomatic women without contraindications if initiated in women aged under 60 years or within 10 years of menopause. If MHT is required beyond 60 years, oral oestrogen and tibolone are not recommended due to increased stroke risk.

Practice tip In women without contraindications, MHT carries few risks if initiated before the age of 60 or within 10 years of menopause.

Contraindications to MHT2 Important contraindications to MHT are listed in TABLE 97.1 Table 97.1

.

Contraindications (absolute or relative) to MHT11

Contraindications to MHT: breast, endometrial and other hormone-dependent cancers (current or previous) undiagnosed vaginal bleeding Relative contraindications (transdermal MHT is preferred): established cardiovascular disease venous thromboembolic disease active liver disease possibly migraine with aura Note: Hypertension is not a contraindication.

MHT regimens The appropriate regimen depends on presence of a uterus and whether the woman is perimenopausal or postmenopausal. Current regimens include the following hormonal treatments: oestrogen progestogen selective oestrogen receptor modulator (SERM) tibolone testosterone

Golden rule A progestogen must be used with oestrogen if the woman still has a uterus.

Page 1109

Oestrogen Oestrogen comes in various preparations: oral, transdermal (patches, gels) and topical vaginal preparations (see TABLE 97.2 ). Topical preparations are appropriate for women with genitourinary symptoms only and will be discussed later in the chapter. A goal of therapy is to prescribe the lowest possible dose to relieve symptoms. Oestrogenic side effects include breast

tenderness and nausea.

Table 97.2

Oestrogens used in the menopause12 Daily dose range

Trade name

Conjugated equine oestrogen (CEE)

0.3–1.25 mg

Premarin

Oestradiol

0.5–2 mg

Estrofem, Zumenon

Oestradiol valerate

0.5–2 mg

Progynova

25–100 mcg

Climara, Estraderm, Estradot

Oestradiol hemihydrate

0.5–1.5 mg

Sandrena

Oestradiol

0.75–3 mg

Estrogel

Generic name Oral preparations

Transdermal patch Oestradiol Transdermal gel

Progestogen Progestogen is given to women with a uterus and may be given continuously or cyclically (see TABLE 97.3 ). If a progestogen is not used in addition to oestrogen, many women will develop endometrial hyperplasia and there is a 5–10 times increased risk of endometrial cancer. If given cyclically, it is given for the 1st to the 14th day of the calendar month and a withdrawal bleed will occur. The intrauterine device (IUD) containing the progestogen levonorgestrel (52 mg Mirena) may also be combined with oestrogen and has the added benefits of providing contraception and managing heavy menstrual bleeding.

Table 97.3

Progestogens used in the menopause10,12

Generic name Oral preparations

Daily dose range Trade name

Medroxyprogesterone acetate

2.5–20 mg

Provera, Rolvera

Norethisterone

1.25–5 mg

Primolut N

Micronised progesterone

100–200 mg

Prometrium

20 mcg

Mirena

Intrauterine device Levonorgestrel (LNG) IUD

Micronised progesterone became available on the Australian market in 2016 and is available in oral and vaginal (pessary and gel) preparations. Mild sedation can occur and, as such, it should be taken at night.13

Selective oestrogen receptor modulator (SERM)14 The SERM bazedoxifene has been developed as an alternative to progestogen for endometrial protection. SERMs act only on oestrogen receptors, with different effects in different tissues. Bazedoxifene has an oestrogenic effect on bone and improves bone density, while exerting an anti-oestrogenic effect on the breast and endometrium. It offers superior rates of amenorrhoea compared to other MHTs and does not increase mammographic density or breast pain. Bazedoxifene is combined with conjugated oestrogen in what is called a TSEC (tissue-selective oestrogen complex). It is suitable for use in postmenopausal women. An increased risk of VTE has been reported for both oral oestrogen and SERMs and while early studies do not reveal an additive effect on the VTE risk, the true risk remains unknown. It should not be used by women at high risk of VTE. TSEC dose: CEE 0.45 mg + bazedoxifene 20 mg (o) daily

Tibolone This is a selective tissue oestrogenic activity regulator with combined oestrogenic, progestogenic and androgenic properties that can be used as an excellent alternative to conventional MHT in postmenopausal women. Positive effects are on vasomotor and urogenital symptoms, sexual function, bone density and fracture risk. It is unsuitable in perimenopausal women because of an increased risk of breakthrough bleeding. It is an appropriate alternative to oestrogen therapy for women who have undergone hysterectomy. Adverse effects with breakthrough bleeding and virilisation are a concern.10 Dose: tibolone 2.5 mg (o) daily

Page 1110

Testosterone Testosterone is usually reserved for postmenopausal women whose libido does not improve with MHT alone. Side effects include acne, increased hair growth, weight gain and fluid retention.

Long-term safety is unknown and treatment should be avoided in women with a history of a hormone dependent cancer (e.g. breast cancer). Blood testosterone levels should be tested prior to and during treatment, noting that endogenous testosterone levels do not predict response to therapy. Dose: testosterone 1% cream, starting 0.5 mL (5 mg) daily (max. dose 15 mg)

How to prescribe MHT TABLE 97.4

presents current commonly used regimens.

Table 97.4

Menopausal hormonal therapy (options)

Uterus intact

Post-hysterectomy

Transdermal O + P patch

Oral O

Transdermal O + oral P

Transdermal O

Transdermal O + LNG-IUD

Tibolone

Oral O + oral P Oral O + LNG-IUD Oral O + SERM (i.e. TSEC) Tibolone O = oestrogen P = progestogen LNG-IUD = levonorgestrel IUD SERM = selective oestrogen receptor modulator TSEC = tissue-selective oestrogen complex Source: Adapted from Jane and Davis, Figure 32

Previous hysterectomy Women do not need a progestogen and should be prescribed continuous oestrogen in either a transdermal or oral preparation. Transdermal delivery of oestrogen has a lower VTE risk.

Perimenopausal women For women whose LMP was less than 12 months ago, the progestogen should be cyclical. Use of continuous oestrogen and progestogen too soon after the LMP can result in unpredictable breakthrough bleeding. The exception is with the use of the levonorgestrel (LNG) intra-uterine system, which can be used with oral oestrogen. After 12 months of cyclical MHT, it is reasonable to transition to a continuous regimen.

Oestrogen and progesterone can be administered separately or in combined preparations. Combined cyclical preparations available in Australia are listed in TABLE 97.5 .

Table 97.5

Cyclical MHT preparations available in Australia2,12

Generic name Oral preparations

Daily dose*

Trade name

Oestradiol/ dydrogesterone

1 mg/10 mg, 2 mg/10 mg

Femoston

Oestradiol/ norethisterone

1 mg/1 mg, 2 mg/1 mg

Trisequens

50 mcg/140 mcg, 50 mcg/250 mcg

Estalis Sequi

Transdermal patch Oestradiol/norethisterone

*Progestogen for 14 out of 28 days only

Combined hormonal contraception is an appropriate alternative to cyclical MHT and can be used up to 50–51 years, provided there are no contraindications (see CHAPTER 92 ). Contraception is advisable for 12 months after the last period for women over 50 years and for 2 years for those under 50 years.

Postmenopausal women For women whose LMP was more than 12 months ago, continuous combined oestrogen plus progestogen therapy can be used (see TABLE 97.6 ).

Table 97.6

Continuous MHT preparations available in Australia2,12

Generic name Oral preparations

Daily dose

Trade name

Oestradiol/drospirenone

1 mg/2 mg, 1 mg/5 mg

Angeliq 1/2, FemostonConti

Oestradiol/norethisterone

1 mg/0.5 mg, 2 mg/1 mg

Kliovance, Kliogest

Conjugated equine oestrogen (CEE)/medroxyprogesterone acetate

0.625 mg/2.5 mg, 0.625 mg/5 mg

Premia

Transdermal patch Oestradiol/norethisterone

50 mcg/140 mcg, 50 mcg/250 mcg

Estalis Continuous

Side effects of therapy15 In the first 2–3 months, the woman may experience oestrogenic side effects, but these usually resolve or stabilise. Starting with a lower dose may minimise these side effects.

Premenstrual syndrome (in 15%) Action: decrease progestogen dose or change to alternative progestogen

Nausea and breast symptoms Cause: initial sensitivity to oestrogen Action: reduce oestrogen dose, consider SERM if breast symptoms

Bleeding problems Issue: Heavy bleeding Action: decrease oestrogen Issue: Unscheduled vaginal bleeding Action: increase progestogen, consider LNG-IUD or SERM Unscheduled vaginal bleeding occurring 3 months after initiating therapy requires formal investigation. Page 1111

Duration of treatment16 Recent guidelines published by the International Menopause Society recommend that there should be no mandatory limit to the duration of MHT. Women who are prescribed MHT should be reviewed at least annually to check on its efficacy and side effects, with treatment tailored accordingly. The mode of delivery and the type of MHT should be adjusted according to age and circumstance. Provided contraindications do not emerge, women can continue MHT as long as it addresses therapeutic goals, symptoms and quality of life.

Vaginal dryness12

While vasomotor symptoms improve over time in the majority of women, genitourinary symptoms generally do not. The first-line therapy is a non-hormonal lubricant such as Replens or K-Y gel. If these are ineffective, topical low-dose vaginal oestrogen preparations can be useful, for example: oestrogen cream or pessary (e.g. Ovestin, Vagifem) intravaginally (inserted 1–1.5 inches), daily at bedtime for 2–3 weeks, then once or twice weekly for maintenance. Topical oestrogen therapy is the preferred option for those women whose menopausal symptoms are limited to vaginal dryness and dyspareunia. Systemic absorption of oestrogen can occur and safety cannot be assured for women with contraindications to MHT. CO2 Fractional Laser is an alternative treatment available for women with contraindications to topical oestrogen or for whom topical treatments have proved insufficient.

Non-hormonal therapy regimens Lifestyle changes Discuss triggers for hot flushes that might be avoidable, including spicy food, alcohol, coffee and emotional stressors. It is advisable to wear loose-fitting clothes and dress in layers.

Medical therapy Non-hormonal, medical options for hot flushes include gabapentinoids, clonidine, oxybutynin and antidepressants. The front-line treatments are the SSRIs and SNRIs, for example:12 paroxetine 10 mg (o) daily increasing up to 20 mg daily after 1 week or venlafaxine 37.5 mg (o) daily, increasing to 75 mg

Complementary therapy Options include black cohosh, red clover, soy products and other phytoestrogens (plants containing oestrogen-like compounds). Black cohosh has been linked to rare cases of abnormal liver function, hepatitis and liver failure. The National Prescribing Service concluded that: most complementary medicine has little evidence of efficacy and poor-quality safety data. Anecdotal evidence is particularly unreliable as hot flushes improve by up to 60% with placebo, partly due to natural fluctuation in symptoms17,18

Bioidenticals These are mixtures of hormones prepared by compounding chemists and supplied as lozenges, troches and creams. There is inadequate data to show that these are effective or safe.

Premature ovarian insufficiency19 Premature ovarian insufficiency (POI) is defined as a disorder in ovarian function in any woman before the age of 40 years, irrespective of the cause. It occurs spontaneously in 1% of women before the age of 40. Causes and associations: idiopathic (most common) genetic (10%) monosomy X (Turner syndrome) fragile X mutation iatrogenic, e.g. ovarian surgery, chemotherapy, radiotherapy autoimmune associations (20%), e.g. coeliac disease, diabetes, Addison disease infection, e.g. mumps, TB, malaria, shigella metabolic disease, e.g. galactosaemia Investigation is appropriate with FSH and oestradiol levels.

Page 1112

Untreated POI is associated with increased incidence of osteoporosis, heart disease, cognitive impairment and premature death. Vasomotor symptoms may be more severe in premature menopause. The cornerstone of treatment is MHT until the median age of menopause.

When to refer Symptoms insufficiently controlled with MHT Severe symptoms when there are contraindications to MHT, especially breast cancer and history of thrombosis Side effects of MHT that are not corrected by routine measures

Practice tips Careful pretreatment assessment is important. Encourage conservative management with an emphasis on lifestyle if symptoms

are mild. Ensure the patient is informed and accepts both benefits and risks. Individualise hormonal therapy. The prime treatment for an oestrogen-deficiency disorder is oestrogen. Use oestrogen-only therapy for women without a uterus. If a uterus is present, give combined oestrogen-progestogen therapy. A SERM is a new alternative to progestogen. Use cyclical MHT in perimenopausal women and continuous MHT in postmenopausal women. Always start with a low dose of oestrogen. Allow about 6 months to stabilise with MHT. Yearly follow-up is advised. Problematic loss of libido can be treated with tibolone or testosterone. Oestrogen deficiency causes vaginal dryness, which can be treated with topical oestrogen therapy.

Patient education resource Hand-out sheet from Murtagh’s Patient Education 8th edition: Menopause

Resources The British Menopause Society: www.thebms.org.uk The Australian Menopause Society: www.menopause.org.au Jean Hailes Foundation: www.jeanhailes.org.au

References 1

Gold EB et al. Factors related to age at natural menopause: longitudinal analyses from

SWAN. Am J Epidemiol, 2013; 178: 70–83. 2

Jane FM, Davis S. A practitioner’s toolkit for managing the menopause. Climacteric, 2014; 17: 1–16.

3

Burger H. Talking women: HRT and breast cancer risk. Medical Observer, 1 August 2008: 40.

4

Mazza D. Women’s Health in General Practice (2nd edn). Sydney: Elsevier, 2011: 256–7.

5

Col NF, Guthrie JR, Politi M, Dennerstein L. Duration of vasomotor symptoms in middleaged women: a longitudinal study. Menopause, 2009; 16(3): 453–7.

6

Writing Group for the Women’s Health Initiative Investigators. Risks and benefits of oestrogen plus progestin in healthy postmenopausal women: principal results from the Women’s Health Initiative randomised controlled trial. JAMA, 2002; 288(3): 321–33.

7

Royal Australian College of General Practitioners. HRT advice. Aust Fam Physician, 2002; 31(8): 733–4.

8

Manson J et al. Menopausal hormone therapy and long-term all-cause and cause-specific mortality. The Women’s Health Initiative randomized trials. JAMA, 2017; 318: 927–38.

9

Baber R. Hormone therapy and menopause: a protracted misunderstanding explained. Medicine Today, 2017; 18(2): 23–6.

10

Vincent A, Burger H. Menopause: how to treat. Australian Doctor, 6 November 2009: 25– 32.

11

Magraith K, Stuckey B. Making choices at menopause. AJGP, July 2019; 48(7): 457-62.

12

Menopause [published 2020]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2020. www.tg.org.au, accessed April 2021.

13

Davis S. Micronised progesterone as a component of menopausal hormone therapy. Medicine Today, 2017; 18(1): 53–5.

14

Baber R. What’s new in menopausal hormone therapy: combination oestrogenbazedoxifene. Medicine Today, 2018: 19(4): 47–50.

15

Baber R. The menopause: update. Medical Observer, 28 July 2006: 31–3.

16

Magraith K, Stuckey B. An update on hormone therapy for menopause. Medicine Today, 2018; 19(1): 41–4.

17

National Prescribing Service Ltd. Managing Menopausal Symptoms, Review PPR 47, September 2009.

18

MacLennan AH et al. Oral oestrogen and combined oestrogen/progestogen therapy versus

placebo for hot flushes. Cochrane Database Syst Rev, 2004; Issue 4: Art No. CD002978. 19

Nguyen HH, Milat F, Vincent A. Premature ovarian insufficiency in general practice: meeting the needs of women. Aust Fam Physician, 2017; 46(6): 360–6.

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98 Vaginal discharge

In all cases of abnormal vaginal discharge consider the possibility of the sexually transmitted infections. DR STELLA HELEY, VICTORIAN CYTOLOGY SERVICE, 2001 Vaginal discharge is a common complaint seen by family physicians, yet it is often difficult to solve, especially if it is recurrent or persistent. Women may complain of an increase in the amount of discharge, a change in the consistency or colour and the presence of an offensive odour. It is important to make a proper diagnosis, to differentiate between normal (physiological) and pathological discharge and to be aware of the considerable variation in secretion of vaginal fluid. This variation extends to different age groups, with microbial causes less likely in prepubertal girls and the elderly, who more often present with dermatoses and symptoms related to reduced vaginal oestrogen. The differential diagnoses should include consideration of normal discharge, vaginal candidiasis, bacterial vaginosis (BV), STIs, foreign body, vulval dermatoses, atrophic vaginitis and genital tract malignancy.

A diagnostic approach A summary of the diagnostic strategy model is presented in TABLE 98.1 Table 98.1

Vaginal discharge: diagnostic strategy model

Probability diagnosis Normal physiological discharge Vaginitis: candidiasis bacterial vaginosis

.

Serious disorders not to be missed Neoplasia: cancer fistulas STIs/PID: Chlamydia trachomatis Neisseria gonorrhoeae Mycoplasma genitalium Herpes simplex—types 1 and 2 Sexual abuse, esp. children Tampon toxic shock syndrome (staphylococcal infection) Ectopic pregnancy (‘prune juice’ discharge) Pitfalls (often missed) Atrophic vaginitis Contact dermatitis (e.g. intravaginal pessaries and creams) Retained foreign objects (e.g. tampons) Erosive lichen planus Desquamative inflammatory vaginitis Latex allergy Herpes simplex virus (if causes cervicitis) Threadworms Seven masquerades checklist Diabetes Drugs Is the patient trying to tell me something? Needs careful consideration; possible sexual dysfunction.

Probability diagnosis The most common causes of vaginal discharge are physiological discharge, vulvovaginal candidiasis and bacterial vaginosis.

Physiological discharge Normal physiological discharge is usually milky-white or clear mucoid and originates from a combination of the following sources: cervical mucus (secretions from cervical glands)

vaginal secretion (transudate through vaginal mucosa) vaginal squamous epithelial cells (desquamation) cervical columnar epithelial cells resident commensal bacteria The predominant bacterial flora are lactobacilli, which produce lactic acid from glucose derived from the epithelial cells. The lactic acid keeps the vaginal pH acidic (40 years Family history of diabetes (1st degree relative with diabetes or sister with GDM)

PCOS with hyperandrogenism (clinical or biochemical) Indian Subcontinent ethnicity Previous baby >4000 g Medications, e.g. oral corticosteroids, antipsychotics Moderate risk (2 or more = high risk)

Maternal age 35–39 years BMI 25–35 Asian, Aboriginal, Torres Strait Islander, Maori, Pacific Islander, Middle Eastern or non-white African ethnicity Polycystic ovarian syndrome (androgens not elevated)

Record day of first fetal movements (i.e. ‘quickening’) (ask patient to write down the dates): primigravida: 17–20 weeks multigravida: 16–18 weeks

Fundal height The relative heights of the uterus fundus are shown in FIGURE 100.2. The uterus is a pelvic organ until the 12th week of pregnancy, after which it can be palpated abdominally. At about 20– 22 weeks it has reached the level of the umbilicus and reaches the xiphisternum between 36 and 40 weeks. From 24 weeks, measure the fundal height from the pubic symphysis and the distance in centimeters should match the number of weeks gestation. Refer for ultrasound if the fundal height is ≤3 cm than expected.4

Page 1134

FIGURE 100.2 Fundal height in normal pregnancy (in weeks); the height of the fundus is a guide to the period of gestation. Nulliparas experience lightening at about 36 weeks when the fundal height usually reverts to the 34week level.

Management of specific issues Nutrition advice A healthy diet is very important and should contain at least the following daily allowances: 1. Eat most:

fruit and vegetables (at least 4 serves) cereals and bread (4–6 serves) 2. Eat moderately: dairy products—3 cups (600 mL) of milk or equivalent in yoghurt or cheese lean meat, poultry or fish—1 or 2 serves (at least 2 serves of red meat per week) 3. Eat least: sugar and refined carbohydrates (e.g. sweets, cakes, biscuits, soft drinks) polyunsaturated margarine, butter, oil and cream Bran with cereal helps prevent constipation of pregnancy. If the ideal diet is followed, iron, vitamin and calcium supplements should not be necessary, although most patients prefer to take OTC vitamin and mineral preparations (e.g. iron, folic acid, multivitamins, iodine). Do not diet to lose weight. It is usual to gain about 12 kg during pregnancy. Less weight gain is recommended for women who are overweight pre-conception.

Healthy environment2 It is appropriate to discuss measures to avoid the following infections and toxins known to be harmful in pregnancy.

Listeria Infection in pregnancy has a fetal mortality of 30–50% Practise good personal and food hygiene; wash hands and use fresh ingredients Wash raw vegetables, fruit and herbs thoroughly prior to consumption Avoid unpasteurised dairy products, soft cheeses, cold meats, pâté, raw seafood, raw egg and chilled ready-to-eat foods Cook meat thoroughly

Mercury 2–3 serves/week of seafood is considered safe Avoid regular consumption of larger fish with higher mercury levels, e.g. orange roughy, shark (flake), marlin or swordfish

Toxoplasmosis Delegate cleaning of cat litter trays to others; if not possible, gloves should be worn during cleaning and hands washed well afterwards Ensure litter trays are emptied daily and regularly disinfected with boiling water

Cytomegalovirus and parvovirus B19 (fifth disease) Women who work with children or in the health care sector can further reduce risk by frequent handwashing and using gloves when changing nappies

Smoking, alcohol and other drugs Encourage patients to stop all recreational drugs, alcohol and tobacco. Caffeine reduction is advised, with some guidelines recommending complete avoidance, while others allow for a maximum of 200 mg/day, i.e. one cup of strong espresso-style coffee, or four cups of mediumstrength tea. Other household members should also stop smoking as passive smoking may be Page 1135 harmful to mother and child. There is convincing evidence that promotion of smoking cessation programs during pregnancy is effective, with improved outcomes, including reduction in preterm birth rates and low birthweight rate.7 Nicotine replacement therapy (NRT) should be recommended to all nicotine-dependent women who have been unable to quit using nonpharmacological approaches. We do not know a safe lower limit of alcohol consumption and fetal alcohol syndrome is a significant cause of mental impairment, so it is best to abstain. Mothers taking illicit drugs, especially opioids and amphetamines, require identification, counselling, treatment and surveillance for the neonatal abstinence syndrome in the newborn child.

Breastfeeding Mothers-to-be should be encouraged to breastfeed; however, their choice should be respected if that is not their wish. Give advice and relevant literature. They can be directed to a local nursing mothers’ group for support and guidance if necessary.

Antenatal classes Referral to therapists conducting such classes can provide advice and supervision on antenatal exercises, relaxation skills, pain relief in labour, infant care and breastfeeding. Enrolment with the partner is recommended.

Psychosocial and emotional stress Antenatal visits provide an ideal opportunity to become acquainted with the patient and explore issues that help her. Provide whole-person understanding with appropriate help and reassurance where necessary. Areas to be explored include support systems, attitudes of patient and partner to the pregnancy, issues in the relationship such as domestic violence, sexuality, expectation of labour and delivery, financial and housing issues, and attitudes of parents and in-laws.

Mental health The effect that pregnancy can have on mental health is variable and women with pre-existing mental illness should receive optimal support and close surveillance. Many treatments for mental illness can be safely used in pregnancy and women should be referred promptly if symptoms deteriorate.

Weight gain in pregnancy Although a standard weight gain is given as 12 kg over 40 weeks of pregnancy, it is common for some women in Australia to gain up to 20 kg without adverse effects.8 Less weight gain is recommended for women who are overweight pre-conception. Dieting to lose weight is not recommended during pregnancy. Encourage sensible weight control. Normal weight gain is minimal in the first 20 weeks, resulting in a 3 kg weight gain in the first half of pregnancy. From 20 weeks onwards there is an average weight gain of 0.5 kg per week. From 36 weeks the weight gain usually levels off.8

Fetal movement chart If daily fetal movements exceed 10 and the regular pattern has not changed significantly, then usually the fetus is at no risk. However, if the movements drop to fewer than 10 per day, the patient should be referred to hospital for fetal monitoring.

Vaginal bleeding in early pregnancy9 This is a common problem in the first trimester in particular. At least 10% of normal pregnancies will have an episode and about 15% of recognised pregnancies will miscarry. If the bleeding is light to moderate and the pain mild or absent the question is ‘Can a viable pregnancy continue or is there an ectopic pregnancy or a threatened miscarriage?’ 1500 IU/L transvaginal ultrasound should be arranged

gestational sac should be visualised once hCG is >1500–2000 IU/L, although variation is common 6–8 weeks: ultrasound is used to define an intra-uterine pregnancy and exclude an ectopic. A repeat ultrasound in 1 week may be required if viability is in doubt >8 weeks: normal ultrasound is reassuring since miscarriage rate is only 3% unless the amount of intra-uterine blood is large Note: Rest is not necessary for threatened miscarriage. Be aware that an incomplete abortion can cause cervical shock (pelvic pain and fainting). Products of conception must be removed from the cervical os. Anti-D is required only in the event of complete miscarriage.

Page 1136

Consider the antiphospholipid syndrome for recurrent miscarriage and arrange antibody testing (see CHAPTER 21 ). Refer to a specialist for full assessment if 3 consecutive miscarriages have occurred.

Nausea and vomiting in pregnancy10 Nausea and vomiting occur in more than 50% of women Majority of cases disappear by the end of the first trimester Mild cases can be dealt with by explanation and reassurance; it is preferable to avoid drug therapy if possible Simple measures: small, frequent meals including ginger up to 1 g daily dry crackers can be kept by the bed to consume first thing a fizzy soft drink, especially ginger drinks, may help ensure adequate hydration, including sucking ice chips and drinking small amounts of fluid avoid stimuli such as cooking smells take care with teeth cleaning rest where possible, especially later in the day, as fatigue often worsens symptoms Medication:11 pyridoxine (vitamin B6) 25 mg (half-tablet or full) bd or tds

doxylamine 25 mg tds (often sedating, consider half-tablet at night only) if still ineffective, add metoclopramide 10 mg tds if persistent nausea and vomiting, consider ondansetron 4–8 mg bd or tds (limited safety data) consider treating associated gastro-oesophageal reflux

Hyperemesis gravidarum This is severe vomiting in pregnancy, which may result in severe fluid and electrolyte depletion. It occurs in about 1 in 100 pregnancies.

Associations Normal complication Hydatidiform mole Multiple pregnancy Urinary infection

Management Test urine—MCU (microculture of urine); ketones: if +ve, admit to hospital Ultrasound examination Test electrolytes, urea, LFTs Bed rest Nil orally Fluid and electrolyte replacement Pyridoxine and doxylamine as per nausea and vomiting in pregnancy Metoclopramide 10 mg IV → 10 mg (o) tds (if necessary); if ineffective, ondansetron 4–8 mg IV 8–12 hourly

Heartburn Gastro-oesophageal reflux is a major source of discomfort to women in the latter half of pregnancy. Non-pharmacological treatment such as frequent small meals, avoidance of bending

over and elevation of the head of the bed are the mainstays of treatment. Regular use of antacids is effective (e.g. alginate/antacid liquid—Gaviscon, Mylanta Plus—10–20 mL) before meals and at bedtime. H2-receptor antagonists or PPIs may be necessary and are considered safe.

Cramps Pregnant women are more prone to cramp. If it develops they should be advised simply to place a pillow at the foot of the bed so that plantar flexion of the feet is avoided during sleep. Prolonged plantar flexion is the basis of the cramps. Quinine, including tonic water, should be avoided. There is no evidence that calcium supplements help cramps during pregnancy.12

Varicose veins Wearing special supportive pantyhose is the most comfortable and practical management, in addition to adequate rest.

Haemorrhoids Haemorrhoids in the later stages of pregnancy can be very troublesome. Emphasising the importance of a high-fibre diet to ensure regular bowel habits is the best management. Some women are prone to constipation in pregnancy and may require regular laxatives such as lactulose or macrogol powder. Painful haemorrhoids may be eased by the application of ice packs or perhaps haemorrhoidal ointments containing local anaesthetic, which are considered safe. Page 1137

Dental hygiene Dental problems can worsen during pregnancy so special care of teeth and gums, including a visit to the dentist, is appropriate. Continuation of cleaning with a softer brush is recommended.

Back pain Back pain, especially low back pain, is common during pregnancy and special back care advice can help women manage this problem, which can become debilitating. Physical therapy administered by a skilled therapist can be extremely effective for pregnant patients.

Guidelines for treatment Keep mobilisation and manipulation to a minimum. Use stretching and mobilisation in preference to manipulation. Safeguard the SIJs in the last trimester.

Encourage active exercises as much as possible. Simple paracetamol is appropriate. A brace may be of benefit. Give trigger-point injections (5–8 mL 1% lignocaine) around the SIJs if necessary.

Pubic symphysitis Pubic symphysitis is characterised by increasing difficulty sitting, moving, walking, getting in and out of cars and bed. Examination of the pelvis with AP and lateral bony pressure reproduces the pain. Treatment is use of a pelvic support garment, physiotherapy and time. Sleeping with a pillow between the knees and avoiding hip abduction may also be helpful. Most but not all settle after delivery.

Exercise guidelines Exercise advice depends on the woman’s fitness and exercise routine prior to pregnancy. General advice includes: exercise at a mild to moderate level only avoid overheating and dehydration allow for a long warm-up before exercise and a long cool-down choose low-impact or water exercise stop if there are adverse symptoms (e.g. any pain, bleeding, faintness, undue distress) avoid scuba diving and sky diving

Carpal tunnel syndrome Splinting of the hand and forearm at night might be beneficial. If needed, an injection of corticosteroid into the carpal tunnel can be very effective (check drug category for risk relative to dates). Rarely, operative division of the volar carpal ligament is necessary. Most problems subside following delivery.

Hypotension This is due to increased peripheral circulation and venous pooling. It is especially common in the first trimester. Advise to avoid standing suddenly and hot baths as these may cause syncope. Fainting may also occur when the woman lies on her back in the latter half of pregnancy (supine hypotension). It is advisable to try to lie on the left side.

Pruritus Generalised itching (pruritus gravidarum) is usually associated with cholestasis due to oestrogen sensitivity in the third trimester. Order LFTs and, if not elevated, reassure and prescribe a soothing skin preparation (e.g. aqueous cream ± glycerol). Monitor LFTs every 1–2 weeks. If LFTs markedly abnormal or if pruritus is severe, the risk of fetal death is increased and special care and assessment is required.

Obesity4 There is a well-documented increased risk of complications for women who are overweight or obese during pregnancy: large-for-gestational-age babies caesarian section stillbirth neural tube defects hypertension pre-eclampsia preterm birth gestational diabetes major depressive disorders Consider dietitian input to support controlled weight gain for overweight women during pregnancy. A pregnancy weight gain of 5–9 kg is recommended for women with a preconception BMI above 30.

Breathlessness of pregnancy13 Consider physiological breathlessness of pregnancy in a woman with unexplained dyspnoea. It starts in the second trimester, is constant and aggravated by exercise and emotional stress. No special treatment is needed or helpful. The breathlessness usually settles 6–8 weeks after delivery. Page 1138

Supplements in pregnancy Iron Iron is not routinely recommended for pregnant women who are healthy, following an optimal diet and have a normal blood test. Those at risk (e.g. with poor nutrition, vegan diet) will require supplementation.

Folic acid

Folic acid is advised for all women contemplating pregnancy, starting about 1 month prior to conception and continuing for the first trimester. Dose: 0.5 mg (o) daily.9 In those at risk (as mentioned earlier in the chapter), the dose is 5 mg per day.4

Vitamin B12 Vitamin B12 is essential for the developing fetus and if deficiency is known or suspected (e.g. vegetarian/vegan diet), test and give B12 supplementation if deficient.

Iodine It is recommended, for pregnant and lactating women and those planning a pregnancy, to take 150 mcg of supplementary iodine as soon as possible by using iodised salt for cooking and a multivitamin that includes iodine.

Vitamin D4 There may be a case for routine testing but it is advisable to test women who are dark-skinned, veiled and at risk. Recommend supplementation for women with vitamin D levels 34 weeks gestation but earlier if possible premature labour occurring between 22 and 34 weeks gestation

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition:

About your pregnancy Miscarriage Pregnancy planning

Resources NICE: www.nice.org.uk NHS Fetal Anomaly Screening Programme: www.fetalanomaly.screening.nhs.uk/ The Royal Women’s Hospital: www.thewomens.org.au MotherSafe: www.mothersafe.org.au/

References 1

Barker JH. General Practice Medicine. Edinburgh: Churchill Livingstone, 1984: 76–89.

2

Royal Australian College of General Practitioners. Guidelines for Preventative Activities in General Practice (the Red Book; 9th edn). Melbourne: RACGP, 2016.

3

The Royal Women’s Hospital (Victoria). Clinical Practice Guidelines (Professional). Available from: www.thewomens.org.au, accessed May 2021.

4

Department of Health. Clinical Practice Guidelines: Pregnancy Care. Canberra: Australian Government Department of Health, 2018.

5

The Royal Women’s Hospital (Victoria). Clinical Practice Guidelines. Antenatal care schedule—routine low risk. Available from: www.thewomens.org.au.

6

The Royal Australian and New Zealand College of Obstetricians and Gynaecologists. Prenatal screening and diagnosis of chromosomal and genetic conditions in the fetus in pregnancy, 2016. Available from: https://ranzcog.edu.au/RANZCOG_SITE/media/RANZCOGMEDIA/Women%27s%20Health/Statement%20and%20guidelines/ClinicalObstetrics/Prenatal-screening_1.pdf?ext=.pdf, accessed May 2021.

7

Lumley J, Oliver S, Waters E. Interventions for promoting smoking cessation during pregnancy (Cochrane Review). In: The Cochrane Library. Issue 1, 2001. Oxford: Update Software.

8

Fung P, Morrison J. Obstetric share-care. Aust Fam Physician, 1989; 18: 479–84.

9

Peat B. Antenatal care: common issues facing GPs in shared care. Medicine Today, 2001;

June: 81–5. 10

Humphrey M. Common conditions in an otherwise normal pregnancy. In: MIMS Disease Index (2nd edn). Sydney: IMS Publishing, 1996: 116–20.

11

Assessment of nausea and vomiting [published 2016]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2016. www.tg.org.au, accessed April 2021.

12

Hammer I et al. Calcium treatment of leg cramps in pregnancy. Acta Obstet Gynaecol Scand, 1981; 60: 345–7.

13

Burdon J. Respiratory medicine. Check Program 395. Melbourne: RACGP, 2005: 17–8.

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101 Postnatal care

As concerning the bringing up, nourishment and giving of suckle to the child, it shal be beste that the mother give her child sucke her selfe, for the mother’s milk is more convenient and agreeable to the infant than any other woman’s or other milke. THOMAS RAYNALDE, THE BYRTHE OF MANKYNDE (1540) Education for the puerperium and caring for the baby should begin during pregnancy so that a new mother is familiar with the basic principles of motherhood, especially infant feeding.1 The puerperium is defined as the period of approximately 42 days from the completion of the third stage to the return of the normal physiological state. It is important to educate postpartum women on care of the baby and breastfeeding, self-care, healing of the genital tract, sexual life and contraception, nutrition and what happens to their bodies and preventive issues. The uterus involutes to non-pregnant size by 6 weeks and the cervical os should be closed by 2–3 weeks post delivery.

Postnatal consultations The two-week consultation Mother: take history of birth and any perinatal complications check blood pressure, weight (BMI) assess how she is adjusting to motherhood look for signs/symptoms of postpartum depression provide encouragement and advice check breastfeeding Baby:

measure weight, height and head circumference routine baby examination by checking the following: fontanelles eyes (observation, corneal reflexes, white pupil) cardiovascular examination femoral pulses hip tests for dislocation testes fully descended genitalia anal region skin reflexes

The six-week consultation This is basically a repeat of the previous consultation—a checklist is presented in TABLE 101.1 Table 101.1

Checklist for postnatal check at 4–6 weeks

Mother Enquire about vaginal discharge (lochia) and whether ceased Ask about healing of the perineum if vaginal delivery Check for any bowel or urinary problems Check if breastfeeding and whether there are concerns Check abdomen (uterus should be impalpable) and Caesarean wound if present Check if intercourse has resumed and whether there are problems or concerns Discuss contraception options Advise on postnatal exercises Discuss adequate diet, rest and personal care Check psychological health, consider Edinburgh Postnatal Depression Scale Consider pelvic examination, checking the perineum and pelvic floor strength Cervical screening test (if due)

.

Review antenatal screening tests for follow-up action (e.g. rubella booster) Further follow-up if necessary Baby Measure weight, height and head circumference Routine examination, e.g. check for red reflex, hips, heart sounds, testes in boys Check growth and feeding Complete childhood health record Discuss immunisation schedule with parent/s

Contraception Page 1140 Lactational amenorrhoea method (LAM) (see CHAPTER 92 ) is an effective contraceptive for the first 6 months, but only if the woman remains amenorrhoeic. Because some women ovulate before their period returns, breastfeeding women are usually advised to use an additional method of contraception if they wish to avoid pregnancy.

Oral contraception Most progesterone-only contraception (i.e. the etonogestrel implant/Implanon, POP and depot medroxyprogesterone acetate) can be commenced in the immediate postpartum period. The exception is IUDs, which are considered safe from 4 weeks. The POP has been a popular choice for breastfeeding women; however, adherence may be difficult given the strict daily 3-hour timeframe for maximum efficacy. The progestogen-only pill (POP or mini-pill) norethisterone 350 mcg/day or levonorgestrel 30 mcg/day Combined hormonal contraception (COC or contraceptive vaginal ring) can be used from 6 weeks postpartum, even if breastfeeding.

After-pains1 After-pains, which are more common and most intense after the second and subsequent pregnancies, are characterised by intermittent lower abdominal pains, like period pains, which are often worse during and after feeding in the first 2 weeks. They are caused by oxytocin released from the posterior pituitary, which also causes the milk ejection (let-down) reflex of

nursing. Suspect endometritis if there is offensive lochia, fever and poor involution of uterus. Treatment, after examination, is reassurance and analgesics in the form of paracetamol every 4 hours for 3 days or as long as necessary.

Breastfeeding problems Insufficient milk supply Studies have shown that many women wean because of perceived low milk supply. It is rare that a woman is truly incapable of making sufficient milk for their infant’s needs (i.e. ‘insufficient glandular tissue’). Factors and events in the very early postnatal period can affect the establishment of maternal breast milk supply, such as separation from the infant (limiting opportunities for skin-to-skin contact), postpartum haemorrhage or factors affecting milk ejection reflex such as pain or stress. Low milk supply can also occur secondary to ‘lactation mismanagement’ such as insufficient frequency of feeds and poor latch and/or position resulting in inadequate transfer of milk.2 Important factors in establishing breastfeeding: 1. positioning and attachment of the baby on the breast—essential for adequate transfer of milk 2. the milk ejection reflex 3. supply and demand feedback—the more the breasts are emptied, the more milk is produced 4. intact milk ducts and sensory nerves 5. sufficient glandular breast tissue 6. infant being able to feed Maintenance of breast milk supply can be affected by: 1. Maternal factors hydration adequate calorie intake stress physiological diurnal/hormonal variations maternal medical conditions, eg. PCOS, anaemia, thyroid disease

2. Infant factor individual variation in metabolism age/size physiological periods of increased demand relating to growth, development and hydration/climate pathological causes of increased infant demand such as cardiac, respiratory and metabolic disorders Signs of low supply include: poor infant weight gain dark, hard, infrequent stools 24 hours or patient is unwell, commence antibiotics.13

.

Ensure good latch and positioning to promote drainage of breast and minimisation of nipple trauma. Antibiotics: resolution without progression to an abscess will usually be prevented by antibiotics: dicloxacillin 500 mg (o) qid for 5–10 days or flucloxacillin 500 mg (o) qid for at least 5–10 days or cephalexin 500 mg (o) qid for at least 5–10 days If severe cellulitis: flucloxacillin 2 g IV 6 hourly Ibuprofen or paracetamol for pain Page 1144

Instructions to patients Keep the affected breast well drained. Continue breastfeeding: do it frequently and start with the sore side or begin feeding from the normal side until the milk comes and then switch to the sore side. Heat the sore breast before feeding (e.g. hot shower or hot washcloth). Cool the breast after feeding: use a cold facewasher from the freezer. Massage any breast lumps gently towards the nipple after feeding (avoid during a feed as it may interrupt the latch and position). Empty the breast well: hand express if necessary. Get sufficient rest. Keep to a nutritious diet and drink ample fluids. Avoid restrictive bras and clothing.

Breast abscess If tenderness and redness persist beyond 48 hours and an area of tense induration develops, then a breast abscess may have formed. It can be treated with needle aspiration or may require surgical drainage under general anaesthesia.

For a description of surgical and other management refer to CHAPTER 93

.

Secondary postpartum haemorrhage14 Secondary postpartum haemorrhage is any bright bleeding from the birth canal 24 hours or more after delivery. It may vary from very slight to torrential and may occur at any time up to 6 weeks postpartum. It tends to peak at 5–10 days.

Causes Retained products of conception (PoC) Infection, especially at placental site Laceration of any part of the birth canal Coagulation disorder Rarely due to gestational trophoblastic disease No cause is found in one-third of cases (i.e. idiopathic subinvolution).

Treatment Rule: An empty and contracted uterus will not bleed. Investigation: ultrasound (?retained PoC) cervical swab for culture FBE β-hCG IV oxytocin 10 IU followed by infusion of 40 IU in Hartman solution Ergometrine 250 mcg IM or 25–50 mcg slowly IV (if continuing heavy bleeding) Consider misoprostol, 4 or 5 × 200 mcg tablets per rectum or otherwise by intramyometrial injection of prostaglandin F2 alpha (caution: specialist supervision) Exploration under general anaesthetic if blood loss >250 mL: gentle blunt curettage required in the postpartum uterus (aim to prevent uterine adhesions —Asherman syndrome)

Consider blood transfusion if Hb is 10 years), no significant surgical risk factors, a low volume stage and a low PSA and who, after being informed of the risks and benefits, prefers surgery. Approaches include radical perineal, laparoscopic and robot-assisted prostatectomy. Major complication issues include incontinence and impotence, and the risk of these will vary depending on the surgical approach and the patient’s age and health. Radiotherapy is also likely to benefit those with a long life expectancy and lowPage 1185 volume low PSA, who have a moderately (or worse) differentiated tumour and who, after being informed of the risks and benefits, prefer external beam or interstitial radiotherapy (brachytherapy). The main adverse reactions from external radiotherapy are faecal urgency and diarrhoea together with urine frequency. Impotence is common for up to 2 years after external beam radiotherapy. Active surveillance is often used for those with a low life expectancy, or those who prefer to avoid the possible complications of surgery or radiotherapy. PSA monitoring is done for surveillance, or for follow-up after treatment with curative intent. For locally advanced or metastatic disease, androgen deprivation is the cornerstone of treatment,

the options being: bilateral orchidectomy or daily anti-androgenic tablets, for example: cyproterone acetate (Androcur) flutamide (Eulexin) bicalutamide (Cosudex) abiraterone (Zytiga) or luteinising hormone releasing hormone (LHRH) agonists: depot injections of LHRH analogues, for example: goserelin (Zoladex) leuprorelin acetate (Lucrin, Eligard) Treatment combinations for low volume metastatic prostate cancer may prolong life, for example: orchidectomy plus flutamide LHRH agonists plus flutamide or bicalutamide—LHRH agonists cause an initial surge of testosterone so a preliminary anti-androgenic agent is advised to prevent a flare in the cancer. As well as androgen deprivation, radiotherapy to bony metastases or local disease and other adjuvant therapy options can be considered.

Complementary medicine Among the disorders most widely promoted as benefiting from natural remedies are those of the prostate, though none has enough evidence to warrant its recommendation.6 Herbal remedies that have been widely used include saw palmetto (Serenoa repens), stinging nettle (Urtica dioica), African prune (Pygeum africanum, syn. Prunus africana), willowherb (Epilobium) and cernilton. Saw palmetto has been used widely, especially in Germany, with initial studies suggesting efficacy in LUTS consistent with BPH. However, a recent Cochrane review concludes it is not more effective than placebo.33 BPH has been treated with isoflavone phytoestrogens. A weak oestrogen agonist effect in males

may antagonise the growth-promoting effects of androgens on the prostate. Epidemiological data indicate that in countries such as Japan where isoflavone diets are prevalent, prostatic enlargement occurs less with ageing. The most widely used source of phytoestrogens is soy protein. It is also present in lentils, chickpeas, some variations of beans and alfalfa sprouts. A variety of nutrients may play a role in the development and progression of prostate cancer. There is evidence from some epidemiological studies that selenium, leucopenes (from tomato and tomato products), vitamin D, vitamin E, calcium and green tea in the diet protect against cancer of the prostate, though their true role remains largely unclear.34 Page 1186

References 1

Healthy Male (Andrology Australia). Clinical Summary Guide 7: prostate disease 2007 (reviewed March 2018). Available from: https://www.healthymale.org.au/files/resources/prostate_disease_csg_healthy_male_2019_ 0.pdf, accessed April 2021.

2

Urinary tract infections [published 2019]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2019. www.tg.org.au, accessed April 2021.

3

Buckley N (Chair). Australian Medicines Handbook. Adelaide: Australian Medicines Handbook Pty Ltd, 2018: 558.

4

European Association of Urology. Guidelines on Chronic Pelvic Pain, 2010. Available from: www.uroweb.org/wp-content/uploads/26-Chronic-Pelvic-Pain_LR.pdf, accessed April 2021.

5

Katz DJ, Love CJ, Chung E. Lower urinary tract symptoms and benign prostatic hyperplasia: old problems, new solutions. Medicine Today, 2016; 7(11): 14–25.

6

Nickel J et al. Alfuzosin and symptoms of chronic prostatitis—chronic pelvic pain syndrome. N Eng J Med, 2008; 359: 2663–73.

7

Litwin M et al. The National Institute of Health chronic prostatitis symptom index: development and validation of a new outcome measure. Chronic Prostatitis Collaborative Research Network. J Urol, 1999; 162: 369–75. Available from: www.prostatitis.org/symptomindex.html, accessed 20 March 2014.

8

American Urological Association. Management of benign prostatic hyperplasia (revised 2010, reviewed 2014). Available from: www.auanet.org/documents/education/clinicalguidance/Benign-Prostatic-Hyperplasia.pdf, accessed April 2021.

9

Barry M et al. The American Urological Association symptom index for benign prostatic hyperplasia. The Measurement Committee of the American Urological Association. J Urol, 1992; 148: 1549.

10

Abrams P et al. Evaluation and treatment of lower urinary tract symptoms in older men. J Urol, 2009; 181: 1779.

11

Abdel-Azis S, Mamalis N. Intraoperative floppy iris syndrome. Curr Opin Opthalmol, 2009; 20: 37.

12

McConnell J et al. The long-term effect of doxazosin, finasteride, and combination therapy on the clinical progression of benign prostatic hyperplasia. N Eng J Med, 2003; 349: 2387.

13

Roehrborn C et al. The effect of dutasteride, tamsulosin and combination therapy on lower urinary tract symptoms in men with benign prostatic hyperplasia and prostatic enlargement: 2-year results from the CombAT study. J Urol, 2008; 179: 616.

14

Wasson J et al. A comparison of transurethral surgery with watchful waiting for moderate symptoms of benign prostatic hyperplasia: the Veterans Affairs Cooperative Study Group on Transurethral Resection of the Prostate. N Eng J Med, 1995; 332: 75.

15

Rassweiler J et al. Complications of transurethral resection of the prostate (TURP)— incidence, management, and prevention. European Urol, 1 February 2005; 50(5): 969–80.

16

Australian Institute of Health and Welfare. Cancer in Australia: key facts. Available from: www.aihw.gov.au/cancer/cancer-in-australia, accessed 23 April 2014.

17

Kumar P, Clark M. Clinical Medicine (7th edn). London: Saunders, 2009: 674–6.

18

Baade P et al. Communicating prostate cancer risk: what should we be telling our patients? Med J Aust, 2005; 182(9): 472–5.

19

Johns L, Heuston R. A systematic review and meta-analysis of familial prostate cancer risk. BJU Int, 2003; 91(9): 789–94.

20

Gronberg H. Prostate cancer epidemiology. Lancet, 2003; 361: 859–64.

21

Barton S, ed. Clinical Evidence. London: BMJ Publishing Group, 2001: 588–98.

22

Royal Australian College of General Practitioners. Guidelines for Preventative Activities in General Practice (the red book). Available from: https://www.racgp.org.au/download/Documents/Guidelines/Redbook9/17048-Red-Book9th-Edition.pdf, accessed April 2021.

23

Sikaris K. Prostate specific antigen. Aust Prescr, 2011; 34(6): 186–8.

24

US Preventive Services Task Force. Screening for prostate cancer: US Preventive Services Task Force Recommendation Statement. Ann Intern Med, 2008; 149(3): 185–91.

25

National Health and Medical Research Council (NHMRC). PSA testing for prostate cancer in asymptomatic men, information for health practitioners. Available from: https://www.nhmrc.gov.au/about-us/publications/prostate-specific-antigen-testing-

asymptomatic-men, accessed April 2021. 26

Prostate Cancer Foundation of Australia and Cancer Council Australia PSA Testing Guidelines Expert Advisory Panel. Draft Clinical Practice Guidelines PSA Testing and Early Management of Test-Detected Prostate Cancer. Sydney: Cancer Council Australia. Available from: http://wiki.cancer.org.au/australia/Guidelines:PSA_Testing/, accessed 12 October 2020.

27

Prostate Cancer Foundation of Australia. How is prostate cancer graded and staged? Available from: www.prostate.org.au/awareness/for-recently-diagnosed-men-and-theirfamilies/partners-and-carers/diagnosis/grading-and-staging-of-prostate-cancer, accessed 13 December 2014.

28

American Cancer Society. Grading and staging of prostate cancer. Available from: www.cancer.org/cancer/prostatecancer/detailedguide/prostate-cancer-staging, accessed 23 April 2014.

29

Whitmore W. Natural history and staging of prostate cancer. Urol Clin North Am, 1984; 11(2): 205–20.

30

Tacklind J et al. Serenoa repens for benign prostatic hyperplasia. Cochrane DatabaseSyst Rev, 2012; Issue 12: Art No. CD001423.

31

National Health and Medical Research Council (NHMRC). Clinical Practice Guidelines: Evidence-based Information and Recommendations for the Management of Localised Prostate Cancer. Available from: www.nhmrc.gov.au/guidelines/publications/cp88, accessed 23 April 2014.

32

Cancer Council Australia Advanced Prostate Cancer Guidelines Working Party. Management of locally advanced and metastatic prostate cancer. Available from: www.cancer.org.au/health-professionals/clinical-guidelines/prostate-cancer.html, accessed April 2021.

33

Sonn G et al. Impact of diet on prostate cancer: a review. Prostate Cancer P D, 2005; 8: 304–10.

34

European Association of Urology. Guidelines on Prostate Cancer, 2013. Available from: www.uroweb.org/wp-content/uploads/09_Prostate_Cancer_LR.pdf, accessed April 2021.

Page 1187

Part 9 Sexual health Page 1188

107 The subfertile couple

You notice that the tabetic has the power of holding water for an indefinite period. He is also impotent—in fact, two excellent properties to possess for a quiet day on the river. DR DUNLOP, TEACHING AT CHARING CROSS HOSPITAL, 1913 Infertility is defined as the absence of conception after a period of 12 months of unprotected sexual intercourse.1 The inability to conceive can be a very distressing and emotional problem for a couple, who need considerable care, empathy and relatively rapid investigation of their problem. In assessing a couple with the problem of subfertility, it is appropriate to involve both partners in the consultation. Human fertility depends on a complex series of events, including:2 production of gametes (sperm and oocytes) capable of fertilisation release of the oocyte into the fallopian tube after ovulation the right number of sperm have to be placed in the right place at the right time implantation and development of the embryo in the hormonally primed uterine mucosa maintenance of the growing fetus in the uterus until pregnancy is full term As a general rule, the major factors limiting fertility are approximately one-third female, onethird male and one-third combined male and female. In addition, there are couples who fulfil the three primary fertility factors (egg, sperm and tubes) but do not conceive. This is known as unexplained (idiopathic) subfertility and accounts for 10–20% of subfertile couples.

Key facts and checkpoints

Infertility affects about 10–20% (1 in 7) of couples.2 Fertility in women and men declines naturally with age. After the age of 32, female fertility decreases by 1.5% per year. Increasing female age is a common cause of decreased fertility. The main identifiable causes of male infertility are failure of spermatogenesis, failure of sperm delivery, impaired sperm quality and sperm autoimmunity.2 Female factors include ovulation dysfunction, tubal disease, uterine disorders and peritoneal pathology.3 Polycystic ovarian syndrome (PCOS) is the most common cause of ovulatory dysfunction.4 In up to 20% of couples, no apparent cause is identified (idiopathic).2 Assisted reproductive technology (ART) helps the majority of subfertile couples to achieve pregnancy, although success rates reduce with increasing maternal age.5

Physiological factors6 Male fertility Fertility in the male requires: normal hypothalamic function producing gonadotrophin-releasing hormone (GnRH) normal pituitary function producing the gonadotrophin hormones—follicle stimulating hormone (FSH) and luteinising hormone (LH) normal seminiferous tubule and Leydig cell function normal sperm transport and delivery Facts about sperm viability: the maximum number of viable sperm is found in the ejaculate after a 48-hour abstinence after entering receptive cervical mucus, sperm are capable of fertilising an egg for up to 5 to 6 days

sperm survive for less than 30 minutes in the vagina due to an acidic environment Page 1189

Female fertility Fertility in the female requires: normal function of the ovulatory cycle, which requires: normal hypothalamic-pituitary function producing the hormones GnRH, FSH and LH normal ovarian function with follicular response to FSH and LH (see FIG. 107.1

)

appropriate prolactin levels (which are normally low); excessive prolactin secretion (hyperprolactinaemia) causes anovulation normal tubal transport and access of the ovum to incoming sperm receptive cervical mucus normal uterus to permit implantation of the fertilised ovum

FIGURE 107.1 The normal ovulatory cycle: ovulation occurs at 14 days, 36 hours after the LH surge starts

Probabilities of pregnancy About 50% of couples, having unprotected intercourse at least twice a week, will probably achieve pregnancy in 6 months, 85% in 1 year and 95% in 2 years.6

Causes of infertility Significant causes of infertility are summarised in TABLE 107.1 FIGURE 107.2 .

and illustrated in

Page 1190

Table 107.1

Causes of subfertility3

Female factors Ovulation dysfunction: hypothalamic/pituitary disorders suppression of the hypothalamic-pituitary axis from stress, chronic illness, extreme exercise, malnutrition or eating disorders hyperprolactinaemia hypothalamic or pituitary tumours PCOS ovarian failure premature ovarian failure secondary to chemotherapy or radiotherapy, ovarian surgery or autoimmune disease other endocrine disorders thyroid dysfunction hyperandrogenism Tubal disease: PID previous ectopic pregnancy previous tubal ligation tubal obstruction Uterine abnormalities: fibroids endometrial polyps congenital (e.g. septate uterus, bicornuate uterus) acquired (e.g. Asherman syndrome) Peritoneal pathology: endometriosis adhesions previous peritonitis inflammatory bowel disease

Endometrial functional abnormalities: coagulation disorders or immunopathology, e.g. SLE, antiphospholipid syndrome Male factors7 Pre-testicular: hypogonadotropic disorder (e.g. Kallman syndrome) hyperprolactinaemia Testicular: congenital cryptorchidism (maldescent) inflammation (e.g. mumps orchitis, infection, trauma, torsion) antispermatogenic agents: chemotherapy drugs radiation heat Klinefelter syndrome (46 XXY) antisperm antibodies Post-testicular: sexual dysfunction retrograde ejaculation obstruction: vasectomy congenital absence of the vas deferens, consider cystic fibrosis ejaculatory duct obstruction or seminal vesicle dysfunction Couple factors Joint subfertility Psychosexual dysfunction

FIGURE 107.2 The major factors involved in subfertility Source: Adapted from Kumar and Clarke8

A diagnostic approach History Ask each partner for their age and take a general medical history, including family history, medication and drug use. Enquire about previous pregnancies in current or past relationships and outcomes.

The man Sexual function (problems with erections, ejaculation)

Age of puberty Previous testicular problems/injury (e.g. orchitis, trauma, undescended testes, torsion) Past history (PH) of STIs PH of mumps PH of urethral problems Genitourinary surgery (e.g. hernia, vasectomy reversal) Occupational history (exposure to heat, pesticides, herbicides) Medications and drug use: alcohol smoking marijuana anabolic steroids chemotherapy radiotherapy aminoglycoside antibiotics sulfasalazine cimetidine/ranitidine colchicine spironolactone antihypertensive agents narcotics phenytoin nitrofurantoin

The woman Onset of menarche

Menstrual history Symptoms of ovulation (cervical mucus changes, mittelschmerz) Symptoms of endometriosis (dysmenorrhoea, pelvic pain, dyspareunia) PH of STIs and pelvic infection Previous contraception use/IUD use PH of intra-abdominal surgery (e.g. appendicitis, ovarian cyst) PH of genitourinary surgery Medications and drug use: alcohol smoking, especially >20/day past contraception, especially depot provera anabolic steroids

Combined history Time trying to conceive Frequency and timing of intercourse Attitudes to pregnancy and subfertility Expectations for the future

Examination A general assessment of body habitus, BMI, general health and secondary sexual characteristics should be noted in both man and woman.

The man Secondary sexual characteristics; note any gynaecomastia Genitalia size and consistency of the testes—can compare to an orchidometer: normal range 15–35 mL (average 18 mL); small in Klinefelter syndrome (approx. 7–8 mL)

palpate epididymis and vas (present and non-tender is normal) evidence of varicocele PR: check prostate note penis and location of urethra (always retract the foreskin for examination)

The woman Secondary sexual characteristics Thyroid status Note skin for acne, hirsutism Vaginal and pelvic examination: assess uterus and ovaries (normal—present, mobile and non-tender) the adnexae (any masses) Page 1191

Investigations These are usually performed after referral but the family doctor should organise initial investigations to assess where to refer (e.g. andrologist, endocrinologist, gynaecologist or fertility specialist).

Initial investigations Male—semen analysis7 Collection should be made directly into a sterile container 2–3 days after sexual abstinence. If collected at home, semen should be kept at body temperature during transport to the laboratory and examined within 1 hour of collection. A repeat test in 1–3 months is indicated if the first is abnormal.7 Normal values (based on WHO criteria): volume ≥1.5 mL (average 2–6 mL) pH ≥7.2 concentration ≥15 million sperm/mL total sperm count ≥39 million

progressive motility ≥32% vitality ≥58% normal forms ≥4% leucocytes 30 nmol/L can be used to confirm ovulation androgens (free testosterone, SHBG, free androgen index (FAI), 17-OH progesterone) thyroid function tests (TSH 90 days >1 year post menarche 45 days in the first 1–3 years post menarche 35 days >3 years post menarche primary amenorrhoea by age 15 or > 3 years post breast development If only irregular cycles or hyperandrogenism are present, a pelvic ultrasound confirming the

presence of polycystic ovary morphology (at least 10 follicles in each ovary) may also confirm the diagnosis. Note: Pelvic ultrasound is not recommended 35 years Consider early referral for women with amenorrhoea or oligomenorrhoea, or for couples with a known reason for infertility.

References 1

World Health Organization. WHO-ICMART revised glossary. Definition of infertility. Available from: www.who.int/reproductivehealth/topics/infertility/definitions/en, accessed March 2018.

2

Infertility [published 2020]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2020. www.tg.org.au, accessed April 2021.

3

Burns K, Smith H. The subfertile couple: a guide to investigations. Endocrinology Today, October 2016; 5(4): 6–16.

4

Boyle J, Teede H. Polycystic ovary syndrome: an update. Australian Family Physician. October 2012; 41(10): 752–6.

5

Chambers G et al. Assisted reproductive technology in Australia and New Zealand: cumulative live birth rates as measures of success. Med J Aust, 2017; 207(3): 114–8.

6

O’Connor V, Kovacs G. Obstetrics, Gynaecology and Women’s Health. Cambridge: Cambridge University Press, 2003: 454–66.

7

Katz D, Teloken P, Shoshany O. Male infertility–the other side of the equation. Aust Fam Physician, September 2017; 46(9): 641–6.

8

Kumar PJ, Clark ML. Clinical Medicine (5th edn). London: Elsevier Saunders, 2003: 1028–30.

9

Illingworth P, Lahoud R. Investigation of the infertile couple 1. Medical Observer, 21 April 2006: 27–30.

10

Hunter T. Investigating fertility. O&G Magazine, Spring 2014; 6(3). Available from: www.ogmagazine.org.au/16/3-16/investigating-fertility, accessed April 2021.

11

The Jean Hailes Foundation for Women’s Health. PCOS. Available from: https://jeanhailes.org.au/health-a-z/pcos, accessed April 2021.

12

Family Planning NSW. Reproductive and Sexual Health: An Australian Clinical Practice Handbook (3rd edn). Sydney: Family Planning NSW, 2016.

13

Monash University. PCOS GP Tool, 2018. Available from: www.monash.edu/__data/assets/pdf_file/0010/1459243/pcos-gp-tool.pdf, accessed April 2021.

14

Craig S. A medical model for infertility counselling. Aust Fam Physician, 1990; 19: 491– 500.

15

Dyer S et al. International Committee for Monitoring Assisted Reproductive Technologies world report: assisted reproductive technology 2008, 2009 and 2010. Hum Reprod, 2016; 31: 1588–1609.

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108 Sexual health

[Drink,] sir, provokes the desire, but it takes away the performance. WILLIAM SHAKESPEARE (1564–1616), MACBETH, A CT 2, SCENE 1 Sexual health is the ability to embrace and enjoy our sexuality throughout our lives. Our sexuality is a fundamental human experience that encompasses sex, gender identities and roles, sexual orientation, eroticism, pleasure, intimacy and reproduction.1 Family doctors are often asked to provide advice and help for sexual concerns and are continually challenged to detect such problems presenting in some other guise. Since we deal with so much illness and prescribe so many drugs, we must be aware of the possible implications of their various effects on sexual health.

Sexual dysfunction2 Sexual dysfunction occurs when an individual is unable to enjoy their sexuality as they would wish. Several studies have demonstrated that sexual concerns and problems are common, with a prevalence ranging from 20–40%. Sexual difficulties are summarised in TABLE 108.1 . There is also evidence that health professionals do not discuss sexual concerns in consultations as often as patients would like. Table 108.1

Sexual difficulties and dysfunction2

Female problems: lack of libido: 4 in 10 women orgasmic difficulties: 1 in 3 women painful intercourse (dyspareunia, vaginismus): 1 in 10 women concerns about labia Male problems:

lack of libido: 1 in 4 men erectile dysfunction: 1 in 5 men premature/delayed ejaculation: 1 in 4 men anorgasmia concerns about penis Other: sexual desire discrepancy substance/medication-induced sexual dysfunction compulsive sexual behaviour

Sexual dysfunction can be the bodily expression of many sorts of distress and requires consideration of biological, psychological, sociocultural and relationship factors. The unique place of general practice and the family doctor provides ideal opportunities to address the sexual concerns of patients as the family doctor often has considerable insight into the family dynamics and first-hand perspective of the individuals involved.

Presentation of sexual concerns Although some patients may present directly with a complaint of sexual dysfunction, many will be less direct and use some other pretext or complaint as a ‘ticket of entry’ for their sexual concerns (see TABLE 108.2 ). Despite a seemingly terse approach the issue must be recognised and treated with considerable importance. This may mean scheduling an appropriate time to discuss the concerns. Table 108.2

How sexual issues may present in family practice3

Minor non-sexual complaint—‘entry ticket’ Specific sexual concern Marital or relationship problem Non-sexual problem (as perceived by the patient) Sexual enquiry as part of illness management Sexual enquiry as part of total health check-up Infertility Menopausal symptoms

The effect of illness on sexual function Doctors seldom enquire about the impact of an illness on the sexual function of patients and their partners (see TABLE 108.3 ). It is most appropriate to enquire about these issues with patients, e.g. the postmyocardial infarction patient, the postprostatectomy patient, the patient taking antihypertensives or other drugs (see TABLE 108.4 ), and the post-mastectomy or posthysterectomy patient. Diabetes deserves special attention as more than 60% of diabetic men have reported erectile difficulties.4 Page 1197

Table 108.3

Medical conditions affecting sexual performance

Cardiovascular: previous myocardial infarction angina pectoris peripheral vascular disease hypertension and its treatment Respiratory: asthma COPD Endocrine: diabetes mellitus thyroid dysfunction Cushing syndrome hypogonadism Neurological: multiple sclerosis neuropathy spinal cord lesions Parkinson disease Musculoskeletal: arthritis Psychogenic: depression anxiety PTSD

Kidney: kidney failure Urological: prostatectomy prostatitis phimosis Peyronie disorder priapism Klinefelter syndrome Hepatobiliary: cirrhosis Gynaecological: endometriosis vaginal repair/birth trauma pelvic prolapse hysterectomy vestibulodynia Chronic pain Cancer Colorectal anal pain colitis

Table 108.4

Drugs affecting sexual arousal and function

Alcohol Anticholinergics Anti-androgens Antidepressants, e.g. SSRIs, SNRIs Anti-epileptics Antihypertensives Antipsychotics Aromatase inhibitors

Benzodiazepines Cytotoxic drugs Hormone contraception, e.g. combined oral contraceptive Tamoxifen Marijuana Opioids

Taking a sexual history It is important to create a safe environment for patients to talk about their sexual behaviour and sexuality. Consider explaining why you are asking for this information and request permission to proceed. Avoid being too formal or too familiar but aim to display a wise, matter-of-fact, empathic, commonsense rapport. CHAPTER 109 covers the sexual history required when assessing for sexually transmitted infections (STIs). Be aware that many patients will have an undisclosed history of sexual abuse.

Probing questions for a suspected sexual problem How are things going in your sex life? Do you have any difficulties with your relationship? Do you have any pain or discomfort during intercourse? Have you had any difficulties with erections (men)? It’s common for people in your situation to experience sexual difficulties. Is that happening to you? Page 1198

Sexual history When a patient wishes to discuss a sexual symptom, consider the following: phases of the sexual response (interest, arousal, erectile function, orgasm) range of sexual practices including masturbation situations in which the symptoms occur associated distress with symptoms

pain during sex Further questions that may be helpful: What sex education did you have as a child at home or at school? What were your parents’ attitudes to sex? Have you experienced unwanted sexual experiences? Are you experiencing stress in any area of your life—relationship, work or home-life? Are you able to talk to your partner about sex? Do you have concerns about your sexual orientation or gender identity? A general medical history should be taken including: menstrual history past STIs chronic health issues mental health issues medication use, including contraception surgical history recreational drug and alcohol use smoking

Examination The routine medical examination should include the basics such as urinalysis, BP measurement, genital examination and neurological examination where indicated. Consider a male genital exam to check for Peyronie disease, small testicle size (may indicate hypogonadism or Kleinfelter syndrome) or retractable foreskin. Consider a female genital examination in women with dyspareunia or vaginismus.

Investigations No particular routine tests are recommended. Tests for male erectile dysfunction (impotence) are outlined later in this chapter. Tests that may help exclude significant causes of low libido are

those for diabetes, liver dysfunction, thyroid dysfunction and endocrine dysfunction. Endocrine dysfunction tests include prolactin, free testosterone, FSH, LH and oestradiol estimations. Other investigations may include pelvic ultrasonography, colposcopy or laparoscopy.

Basic sexual counselling The family doctor can learn to be an effective sex counsellor. Sex counselling can be emotionally demanding and, while good interviewing skills, interest, support and basic advice are important, additional skills are needed to be an effective counsellor. Principles for basic sexual counselling: give the patient permission to talk openly about sexual matters allow for a relaxed exchange of information dispel sexual myths and correct misunderstandings explore the patient’s anxiety and any impact on their relationship de-emphasise the modern-day obsession with performance and orgasm and emphasise the value of alternative forms of sexual expression (e.g. caressing, kissing, and manual and oral stimulation) reassure the patient that he or she is normal (where appropriate) Inappropriate doctor behaviour is presented in TABLE 108.5 Table 108.5

.

Sexual counselling: inappropriate doctor behaviour

Overfamiliarity Being too formal Being too talkative Blunt questioning Being judgmental Making assumptions about the other’s sexuality Imposing one’s own beliefs and standards Dogmatism Tackling problems beyond one’s experience

Sexual problems can be grossly underestimated. Human beings generally have a basic craving

for intimacy, touching, stroking and loving sex. Apparently harmonious relationships can lack this type of intimacy, which may lead to various psychosomatic manifestations. Ideally, the family doctor should undertake a course in sexual counselling to promote confidence in the counselling process. Patients can be taught basic methods (where appropriate) such as sensate focus, squeeze or stop–start techniques for premature ejaculation, self-exploration using Kegel pelvic floor exercises, fantasy conditioning with books or videos, use of sex toys and behaviour modification. Complex or serious problems, especially those involving compulsive sexual behaviour, demand specialist referral.

The PLISSIT counselling model The PLISSIT counselling model developed by Annon5 can be used to build the skills needed to deal with sexual problems, especially if there is a psychological element. The mnemonic PLISSIT stands for:

Page 1199

P = Permission giving LI = Limited Information SS = Specific Suggestion IT = Intensive Therapy ‘Permission giving’ allows patients to talk about sex, ask questions, feel guilty and so on. Their problems are shared with a reflective listening confidant. Most medically trained people can probably provide the limited information required about sexual physiology and behavioural patterns. ‘Specific Suggestion’ provides ideas for self-help and may include key reference books and relevant videos. With a little support and permission, the patient can take simple action to remedy or improve a problem. Intensive therapy, whether psychiatric or emotional, calls for deeper involvement and can be a dangerous area for the inexperienced. Referral to an appropriate practitioner is usually advisable.

Analogous roles of the penis and clitoris An explanation of the analogous roles of the penis and clitoris (proposed by Cohen and Cohen) may be a useful strategy for educating patients and helping them to understand the relationship of intercourse and penile and clitoris stimulation with orgasm. The simple model (see FIG. 108.1 ) can be shown to patients to explain, for example, why some women are unable to achieve orgasm by intercourse alone, especially using the conventional missionary position.3 It can readily be explained that clitoral stimulation in women is analogous to penile stimulation in men.

FIGURE 108.1 Analogous structures in male and female genitalia Source: Reproduced with permission from G and M Cohen, Canadian Family Physician, 852; 31: 767–713

Female orgasmic difficulties6 Difficulties with orgasm are common. If a person has never had an orgasm and is distressed by this, consider factors such as lack of sexual education, communication challenges with partners or inexperience with self-stimulation. A biological cause is more common in acquired anorgasmia, especially as a result of medications and with endocrine or neurological disorders. Less intense or delayed orgasms may be expected with normal ageing. If orgasm occurs during self-stimulation but not during sex with a partner, then underlying biological causes are unlikely. Difficulties within the relationship or with communication about sexual needs may be factors. A common concern of women is the inability to regularly achieve orgasm through vaginal intercourse, believing there may be a physical cause. It is important to reassure that this is normal, as the majority of women are able to achieve orgasm through clitoral stimulation rather than intercourse.

The use of the Cohen model (see FIG. 108.1 importance of clitoral stimulation.

) is very helpful in emphasising the

Page 1200

Treatment options for orgasm difficulties include:6 mindfulness interventions relationship counselling cognitive behavioural therapy aids such as books, videos or vibrators sexual therapist/specialist treatment: directed self-stimulation sensate focus exercises testosterone therapy in postmenopausal women (may improve orgasms)

Dyspareunia Painful intercourse is a source of considerable distress both physically and psychologically for the sufferer and also for her partner. Tact and sensitivity is very important in management. In particular it is helpful to keep in mind vulval vestibular syndrome, which has subtle physical signs (see CHAPTER 99 ). Important causes are listed in TABLE 108.6 . A common problem encountered is the presence of painful scar tissue following an episiotomy, especially after vaginal delivery. Women who experience deep pain with intercourse need a gynaecological referral to assess for pelvic pathology. Table 108.6

Important causes of dyspareunia

Pain worse on insertion Physiological—inadequate lubrication Vaginismus Pelvic floor muscle tightness Provoked vestibulodynia syndrome Vaginitis in chronic candidiasis Vulvar dermatoses (e.g. dermatitis, psoriasis, lichen planus) Vulvovaginal atrophy (e.g. postmenopausal) Postnatal perineal scarring Incompletely ruptured hymen

Pain worse on deep penetration Endometriosis PID Pelvic adhesions Ovarian and uterine tumours

Management of dyspareunia involves treating the organic cause and giving appropriate advice about the use of lubricants, including topical oestrogen where indicated. While this may be enough in simpler cases, it does not address the often accompanied fear of penetration,2,7 which can continue even after the cause of dyspareunia is addressed. Explain that continuing to have painful intercourse can exacerbate the problem. It is appropriate to stop sexual practices that cause pain and continue other ways of being intimate (if comfortable), until the pain is adequately treated or resolved.6

Vaginismus2 Vaginismus is the involuntary contraction of muscles around the introitus (outer third of vagina) in response to and preventing the possibility of penetration. It can be classified as primary or secondary. In primary vaginismus, tampons will probably never have been inserted. It is often related to provoked vestibulodynia (vestibular hypersensitivity—see CHAPTER 99 ). A fear of penetration can be the underlying cause and may be associated with fears of internal damage, pregnancy, learned negative attitudes to sex and past sexual trauma. The problem may respond to sensitive exploration of fears and education of the anatomy and physiology. Encourage use of lubricant and a comfortable sexual position that the woman can control, usually superior. Consider referral to specially trained physiotherapists for pelvic floor relaxation and use of vaginal trainers. Vaginal trainers can be inserted at increasing sizes for desensitisation, allowing for progressive vaginal dilation. Expert counselling is necessary if fears remain a barrier to recovery.

Low libido in females2 The Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) defines lack of libido in women as sexual interest/arousal disorder (FSAD).8 Low sexual arousal or desire is a problem when it is out of keeping with the age of context of the patient and is causing distress. Lack of libido is usually due to a combination of factors including relationship issues, illness, medications, drug and alcohol use, hormonal changes, stress and fatigue. Individuals or couples may benefit from basic sexual counselling from their GP, while others

may request input from a relationship counsellor or sex therapist. If lack of libido is secondary to menopause, a trial of menopause hormone therapy (MHT) may be useful. Tibolone is an MHT that has been shown to have benefit in managing loss of libido in some women.6 Use of transdermal testosterone is effective in postmenopausal women with low libido (see CHAPTER 97 ). Page 1201

Concerns about labia Demands for labiaplasty, the surgical reduction of the labia minora, has significantly increased in Australia. The portrayal of hairless, prepubescent external genitalia in the media has contributed to concerns about labia, although this is not representative of society.9 It is appropriate to provide sensitive education regarding the normal appearance of labia. Labia library (see: labialibrary.org.au) is a helpful resource.

Low libido in males6 Low libido in men is defined as male hypoactive sexual desire disorder (MSHDD).8 It is underreported and under-recognised. Erectile dysfunction and premature ejaculation often accompany low libido and may be contributing factors. Other contributing factors may include androgen deficiency (see CHAPTER 102 ), insomnia, obesity, illness, fatigue, depression, relationship factors and concerns regarding gender identity or sexual orientation. Address underlying conditions and consider referral for psychological therapy or to a sex therapist. Testosterone is not indicated for treatment of low libido in males unless they have androgen deficiency.

Erectile dysfunction Erectile dysfunction (impotence) is the inability to achieve or maintain an erection of sufficient quality for satisfactory intercourse. Erectile dysfunction is common. In an Australian study, the overall prevalence of erectile dysfunction was 61% (25% sometimes, 19% usually and 17% had complete erectile dysfunction). More than 20% of healthy men aged 60–65 years with no risk factors had moderate or complete erectile dysfunction.4 Traditionally, the aetiology of erectile dysfunction is classified into organic, psychogenic or mixed. In reality, however, anxiety and depression commonly accompany erectile dysfunction, irrespective of the original aetiology. Consequently, nearly all organic erectile dysfunction will eventually become ‘mixed’.10 Consider a psychological cause if onset is sudden, whereas an organic cause is more likely to be gradual.

Causes Psychogenic: related to stress, interpersonal or intrapsychic factors (e.g. depression, marital disharmony, performance anxiety) Neurogenic: disorders affecting the parasympathetic sacral spinal cord (e.g. multiple sclerosis); it usually develops gradually Vascular Diabetes Hypertension Chronic kidney disease Urological problems (e.g. Peyronie disorder, Pelvic trauma and surgery) Hormone disorder: androgen deficiency (e.g. testicular disease) hypothyroidism hyperprolactinaemia (rare) → impotence and loss of libido due to secondary testosterone deficiency Drug-induced: alcohol cocaine, cannabis nicotine (four times the risk by age 50) antihypertensive agents finasteride Ageing Unknown

Practice tip Erectile dysfunction may be the first symptom of atherosclerotic disease (e.g. CAD).

History The nature of the onset of erectile dysfunction is very important and this includes the nature of the relationship. Of particular importance is a drug history, including alcohol, nicotine, recreational drugs and pharmaceutical agents, particularly antihypertensives (beta blockers and thiazide diuretics), hypolipidaemic agents, anti-androgens (prostate cancer treatment), antidepressants, antipsychotics and H2-receptor antagonists. Ask about nocturnal and early morning erections.

Examination Genitourinary, cardiovascular and neurological examinations are appropriate, although may not reveal the diagnosis. A focused examination should include a rectal examination and examination of the vascular and neurological status of the lower limbs and the genitalia, especially the testicles and penis. Check the cremasteric and bulbocavernosus reflexes. Page 1202

Investigations First-line blood tests: morning serum total testosterone TSH prolactin luteinising hormone (LH) FSH fasting blood glucose Other blood tests to consider: LFTs, especially GGT (alcohol effect) kidney function tests lipid studies When the aetiology is unclear, specialist testing includes a penile duplex doppler ultrasound using an intracavernosal vasoactive agent.

Management Address modifiable risk factors, including medications (if feasible), psychosocial issues and lifestyle (see NEAT, CHAPTER 5 ). Management should comprise appropriate patient

education. The partner should be included in the discussions and general management process with an emphasis on bolstering the couple’s self-image, which may have been affected by feelings of rejection or avoidance.

Psychogenic disorders These involve psychotherapy and sex behavioural modification as outlined earlier in this chapter under Basic sexual counselling. Referral to a consultant may be appropriate.

Oral medication PDE-5 inhibitors: the phosphodiesterase type 5 (PDE-5) inhibitors are the first-line oral medication (see TABLE 108.7 ). They are about 70% effective but not very effective for neurogenic ED. They do not initiate an erection but enhance whatever erection the man is capable of having. Sexual stimulation is necessary. They are contraindicated if the patient has unstable angina, recent stroke or myocardial infarction. Use with nitrates should be avoided and a nitrate should never be taken within 24 hours of use. The interaction with nitrates can result in a severe and potentially fatal hypotensive response. PDE-5 inhibitors have the potential for side effects, especially headache. Treatment is not considered a failure until a full dose has been trialled 7–8 times.11 Table 108.7

Phosphodiesterase type 5 inhibitors10 Sildenafil (Viagra)

Avanafil (Spedra)

Tadalafil (Cialis)

Vardenafil (Levitra)

Dosage (mg)

25, 50, 100

50, 100, 200

5, 10, 20

5, 10, 20

Usual starting dose (1 hour pre i/c)

50 mg

100 mg

10 mg

10 mg

Peak effect

1 hour

30 to 45 minutes

2 hours

1 hour

Half-life (approximately)

4 hours

6 to 17 hours

18 hours

4 hours

Class side effects

Headache, nasal congestion, facial flushing, dyspepsia

Specific side effects

Blue vision, diarrhoea

Back pain

Myalgia, back pain

Visual disturbance

Tadalafil can be taken daily (at a lower dose, 2.5 to 5 mg) rather than intermittently for erectile

dysfunction. Use with caution in patients with renal impairment. Intrapenile injection6 Intracavernosal vasodilator injections are second-line therapy and should be supervised by an experienced practitioner. Alprostadil intracavernosal injections: self-administered after supervised teaching (use a penile model if available) start with a lower dose, 2.5–5 mcg spontaneous erection in 5–20 minutes if prolonged erection >2 hours, take 120 mg pseudoephedrine orally and have a hot shower— repeat at 4 hours if necessary (provided not hypertensive) Vacuum constriction Vacuum constriction devices (VEDs) may have a place in management, where pharmacological therapies have failed or are inappropriate. VEDs are often poorly tolerated in the long term due to side effects such as pain, inability to ejaculate, bruising, ‘hinging’ and paraesthesia.10 Surgery Malleable penile prosthesis Inflatable penile prosthesis (see FIG. 108.2

)

FIGURE 108.2 An inflatable prosthesis, showing positioning of the components Page 1203

Premature ejaculation Premature ejaculation is defined as ‘ejaculation that occurs sooner than desired’ or, more precisely, as ‘persistent or recurrent ejaculation, before, on or shortly after penetration and associated with significant personal distress’. In most cases, this occurs within 1 minute of vaginal penetration. It may not be clearly described by the patient so a careful history is necessary to define the problem. Ensure that the person is not suffering from erectile dysfunction. Both patient and partner may complain about the problem. There are many approaches to treatment but they are aimed either at prolonged ejaculatory control or at satisfactory sexual activity without preoccupation with ejaculation. Non-pharmacological treatment includes psychosexual therapy and/or behaviour therapy (including ‘stop–start’ techniques).

Pharmacological treatment SSRIs have also been reported as effective—using dapoxetine 30 mg, sertraline 50 mg or paroxetine 20 mg, all about 3 hours before intercourse.6 Some men may prefer to take SSRIs daily for managing premature ejaculation. Local anaesthetic has been suggested to reduce penile sensation; however, this tends to be counterproductive as it reduces sexual pleasure.2

Concerns about the penis In general practice it is not uncommon to counsel men and adolescent males for anxiety about the size or appearance of their penis. The presence of pearly penile papules (see CHAPTER 105 ) often requires considerable reassurance. Educate that penile size has little relationship to a partner’s satisfaction from sexual intercourse. The average adult penis length is 7.5–10.5 cm when flaccid and 12–18 cm when erect.

Gender and sexual orientation12 For some individuals, gender identity and sexual orientation are fixed and clear, while for others it may be more fluid. It is worth noting that identity, attraction and behaviour are not necessarily congruent. Biological sex is determined by sex chromosomes, reproductive organs and hormones, however, not everyone identifies with the gender assigned to them at birth. Gender identity is the internal experience of gender, and gender and sexual expression is how a person chooses to express their gender in terms of appearance and mannerisms. An Australian study found that 14.7% of women are same sex attracted, 13.5% engage in same sex behaviour, 1.2% identify as lesbian and 2.2% are bisexual; and 6.8% of men are same sex attracted, 6.0% engage in same sex behaviour, 1.6% identify as gay and 0.9% bisexual.13 It is estimated that up to 8% of Australians are gender diverse and individuals who are intersex constitute 1.7% of births in Australia. Page 1204

Glossary of terms Lesbian A woman who experiences an attraction to other women. Gay A man who experiences attraction to men. Men who have sex with men (MSM) do not always identify as gay. It can also be used as an umbrella term to describe both lesbians and gay men and perhaps bisexual people of both sexes. Bisexual A person who experiences attraction to their own as well as the opposite gender. Transgender A person whose gender identity and/or gender expression differs to their birth sex. Sexual orientation varies and is not dependent on gender identity. Trans man or FTM (female-to-male) A person assigned the female gender at birth who identifies on the male spectrum.

Trans woman or MTF (male-to-female) A person assigned the male gender at birth who identifies on the female spectrum. Intersex People who are born with genetic, hormonal or physical sex characteristics that are not typically ‘male’ or ‘female’. Causes include congenital adrenal hyperplasia and androgen insensitivity syndrome. Intersex individuals can have a broad range of gender identities and sexual orientations. Queer A political statement as well as a sexual orientation which advocates nonbinary thinking and seeing both sexual orientation and gender identity as potentially fluid. Non-binary A gender identity that is neither exclusively woman or man, is inbetween or beyond both genders. Gender-fluid A person who does not identify themselves as having a fixed gender. Cisgender A person whose gender identity corresponds to their sex at birth.

Gender incongruence14 Gender incongruence, when a person’s biological sex and gender identity do not align, is no longer considered a mental health disorder. There has been a move away from mandatory psychiatric assessment, in favour of a patient-centred, informed consent model of care. Trans, gender diverse and non-binary (TGDNB) people may present to their GPs requesting gender-affirming care, including psychological support, a request for gender-affirming hormones or transgender-specific surgeries. Providing earlier access to gender-affirming care improves the health outcomes and well-being of TGDNB people. GPs are playing an increasing role in prescribing gender-affirming hormones.

Prescribing gender-affirming hormones14 Assess and document capacity to give informed consent. Counsel regarding expectations of treatment, potential complications and side effects. Exclude contraindications to hormone therapies. Goal is to align physical appearance with gender identity. Hormone therapy should be individualised. Start with low doses and titrate gradually. Hormone therapy is usually lifelong, although some patients may choose to cease treatment.

Hormone therapy should not be relied on for contraception. Regular monitoring of hormone levels, FBE, LFTs and renal function is required. Regular clinician review is essential. Details on dosing can be found at: https://cdn.thorneharbour.org/media/documents/htprescribing-guideline-v3-aug-2020.pdf. Feminising hormones Oestradiol valerate tablets, transdermal oestradiol or oestradiol implant Early changes include calmer mood, softer skin, reduced libido, erectile dysfunction Further changes over time include body fat redistribution, reduced muscle mass, decreased testicular volume, breast development Side effects include nausea, DVT, gall stones, liver impairment and infertility Sperm cryopreservation should be discussed prior to commencement of therapy Progesterone is less frequently prescribed, lacking evidence of efficacy Anti-androgens Spironolactone or cyproterone acetate tablets Usually prescribed alongside oestradiol to reduce testosterone levels Changes include slower growth of body hair, improved acne, reduced libido, erectile dysfunction; facial hair usually persists Most common side effect is fatigue Page 1205

Masculinising hormones Testosterone injection, gel or cream Early changes include acne, increased libido, increase in clitoral size Further changes over time include amenorrhoea, body fat redistribution, muscle growth, increase in body and facial hair and voice deepening Side effects include androgenic alopecia, atrophic vaginitis, sleep apnoea and polycythaemia Voice deepening is irreversible

Menstruation and fertility return on cessation of treatment Children and adolescents Given gender identity develops in the first four years of life, children may also require genderaffirming care. For patients 10 partners in past 6 months participate in group sex use recreational drugs during sex are HIV-positive Aboriginal and Torres Strait Islander people Sex workers People who inject drugs People with a past history of STIs People who have unprotected sex with casual partners People who have unprotected sex overseas Sexual partners of a person with an STI Pregnant women (more detail in CHAPTER 100 Neonates born to infected mothers

)

Practice tip Explain and demonstrate the importance of negotiating condom use.4

Asymptomatic STI testing5 Since most STIs are asymptomatic, it is important to offer appropriate opportunistic testing to people at risk. A discrete history is also important—a detailed history and examination may serve as a barrier to proceeding to testing. Consider the following questions: Before we do a cervical screening test/prescribe your pill, would you also like a chlamydia test? It is recommended that anyone under 30 years of age who is sexually active should have an annual chlamydia test. Would you like a test today? I would like to ask you some questions about your sexual activity so we can decide what tests to do, is that okay? Patients often request to be ‘tested for everything’ and it is important to explain that certain STIs, in particular genital warts and genital herpes, are not tested for in asymptomatic people. Genital warts is a clinical diagnosis, and herpes is diagnosed on a swab of a specific lesion. Guidelines on STI testing in asymptomatic patients are summarised in TABLE 109.2 . Nucleic acid amplification tests (NAATs), such as polymerase chain reaction (PCR), should be employed on swabs in asymptomatic patients. TABLE 109.3 outlines how to test for each infection. Table 109.2

STI testing in asymptomatic patients6

Who? Young people (age 15–29)

What infection? How often? Chlamydia Annually Hepatitis B Confirm immune status*

Men who have sex with men (MSM)

Chlamydia Gonorrhoea Syphilis HIV Hepatitis A Hepatitis B Hepatitis C

Annually, 3 monthly if higher risk Confirm immune status* If HIV positive, on PrEP or history of injecting drug use

Asymptomatic people

Chlamydia

Annually

requesting STI testing

Aboriginal and Torres Strait Islander people

Gonorrhoea Syphilis HIV Hepatitis B

Frequency according to risk

Chlamydia Gonorrhoea Syphilis HIV

Annually Consider a low threshold for testing

Confirm immune status*

Rural/remote areas Confirm immune status*

Hepatitis C Trichomonas Hepatitis B People who inject drugs

Sex workers

Chlamydia Gonorrhoea Syphilis HIV Hepatitis C Hepatitis A Hepatitis B

Annually

Chlamydia Gonorrhoea Syphilis HIV Hepatitis B

Frequency according to risk/requirements Confirm immune status*

Frequency according to risk Confirm immune status*

*Vaccinate if non-immune Page 1208

Table 109.3

How to test in asymptomatic patients6

Infection Females

Specimen collection

Test

Chlamydia

Vaginal swab or first-pass urine or endocervical swab

Chlamydia NAAT (PCR)

Gonorrhoea

Vaginal swab or first-pass urine or endocervical swab and for sex workers: throat swab

Gonorrhoea NAAT (PCR)

Trichomonas

Vaginal swab or first-pass urine

Trichomonas NAAT (PCR)

Chlamydia

First-pass urine and for MSM: throat swab, rectal swab

Chlamydia NAAT (PCR)

Gonorrhoea

First-pass urine and for MSM: throat swab, rectal swab

Gonorrhoea NAAT (PCR)

Males

Blood tests in females and males Infection

Test

Syphilis HIV Hepatitis A Hepatitis B

Syphilis serology HIV Ab/Ag Anti-HAV Ig-total Anti-HBc Anti-HBs HCV Ab

Hepatitis C

Note: All swabs can be self-collected except an endocervical swab, which requires collection by the clinician. First-pass urine is preferably collected at least one hour after last passing urine and does not have to be the first urine of the day.

Testing after exposure1 Patients may present requesting STI testing following condom breakage or suspected STI exposure/contact. Postexposure prophylaxis (PEP) for HIV or hepatitis B immunoglobulin and immunisation may be appropriate within 72 hours if moderate to high risk (more detail in CHAPTER 110 ). Testing should be performed, depending on risk, at the following intervals: HIV, syphilis and hepatitis B (if not immune) baseline testing, repeat at 6 and 12 weeks chlamydia, gonorrhoea and trichomonas initial screening with swab of any involved orifice, repeat at 2 weeks after contact for definitive negative or earlier if symptomatic

Taking a sexual history1,5 A detailed sexual history may be important if a patient presents requesting STI testing or if they present with symptoms. Doctors and patients alike usually find discussing sexual matters uncomfortable, even when STI testing is requested. A skilful approach will help overcome

barriers or discomfort. A non-judgmental approach and appropriate body language will facilitate patient disclosures on sensitive sexual issues. Are you currently in a relationship? How many sexual partners have you had in the past 3–12 months? Were these casual or regular partners? Were your sexual partners male, female or both? When you had sex, was it vaginal, oral or anal sex? Did you use condoms? Have you ever been diagnosed with or thought you had an STI? For a detailed STI risk assessment: I’d like to ask you about some other activities that could increase someone’s risk of some sexually transmitted infections and blood-borne viruses, is that okay? Have any of your male partners ever had sex with men? Have you ever been paid to have sex? Have you ever paid to have sex? Have you received any tattoos overseas? Have you ever injected drugs and, if so, have you ever shared needles? Have you ever been in jail? Are you a recent migrant or refugee?

Presenting conditions1 Urethritis in men Urethritis can be classified as gonococcal or non-gonococcal urethritis (NGU). NGU is caused most commonly by Chlamydia trachomatis and Mycoplasma genitalium; however, it is common to not find any cause. Other organisms include herpes simplex virus (HSV), adenoviruses and Trichomonas vaginalis, whereas Ureaplasma urealyticum is considered normal urethral flora. Page 1209

Symptoms

The main symptoms (if present) are: a burning sensation when passing urine (dysuria) a penile discharge or leakage (clear, white or yellow) Sometimes there is no discharge, just pain. Most often the symptoms are trivial with chlamydia. Although a creamy pus-like discharge is typical of gonorrhoea (see FIG. 109.1 ), and a less obvious milky-white or clear discharge typical of chlamydia (see FIG. 109.2 ), it is often difficult to differentiate the causes from the discharge. In some males the only complaint is spots on the underpants or dampness under the foreskin.

FIGURE 109.1 Gonococcal urethritis: typical purulent discharge

FIGURE 109.2 Chlamydia urethritis: the discharge is usually milky in colour but can also be yellow

Diagnosis First-pass urine (FPU) for Chlamydia and Gonorrohoea NAAT If discharge—urethral swab for microscopy and culture

Management principles Given the prevalence of chlamydia in the community, it is recommended to treat with doxycycline 100 mg (o) bd for 7 days while waiting for test results. Use of a stat dose of azithromycin is no longer first-line management, as its use is driving mycoplasma resistance. If tests are negative and symptoms persist, consider referral for first-pass urine NAAT testing for Mycoplasma genitalium, HSV and adenovirus (if not available through general practice). Consider seeking specialist advice before treating infection other than chlamydia/gonorrhoea. Regardless of results, female partners are at greater risk of PID and require assessment.

Chlamydia

Incubation period Symptoms usually develop 1–2 weeks after intercourse, but may take longer.

Presentation 50% of men and 75% of women are asymptomatic. Genital and anorectal infections are most common, with rare infections of the eye and oropharynx. Infection in men can cause urethritis and lead to epididymo-orchitis. In women, infection can cause cervicitis with vaginal discharge and intermenstrual or postcoital bleeding. Male symptoms: urethritis (dysuria, penile discharge) testicular pain anorectal symptoms Female symptoms: dysuria intermenstrual or postcoital bleeding vaginal discharge dyspareunia, pelvic pain (due to PID) increased dysmenorrhoea anorectal symptoms

Complications Epididymo-orchitis Pelvic inflammatory disease (PID) Tubal factor infertility and ectopic pregnancy Chronic pelvic pain Perinatal conjunctivitis or pneumonia Reactive arthritis ± conjunctivitis (Reiter syndrome) Conjunctivitis

Treatment For uncomplicated genital or pharyngeal infection: doxycycline 100 mg (o) 12 hourly for 7 days (preferred) or for pregnant women or patients likely to be non-adherent to doxycycline azithromycin 1 g (o) single dose7 For anorectal infection: doxycycline 100 mg (o) 12 hourly for 7 days if asymptomatic, 21 days if symptomatic (to cover for LVG)1 Sexual intercourse must be avoided until 7 days after both partners have received treatment. Reinfection rates are high so retest after 3 months.

Gonorrhoea Gonorrhoea is most common in MSM, Aboriginal and Torres Strait Islander people living in rural and remote areas, and travellers from endemic areas.

Incubation period Gonorrhoea has a short incubation period of 2–3 days and symptoms usually appear 2–7 days after vaginal, anal or oral sex.

Presentation Up to 80% of women and 10–15% of men have no genital symptoms and most people are asymptomatic in other sites, especially the pharynx, rectum and endocervix. Upper genital infection can lead to pelvic inflammatory disease in women. Gonococcal urethritis in men is characterised by a purulent urethral discharge.

Other manifestations of gonorrhoea Epididymo-orchitis and prostatitis (males) Perinatal conjunctivitis or pneumonia Reactive arthritis ± conjunctivitis (Reiter syndrome) Conjunctivitis Disseminated disease including septic arthritis and macular rash

Investigations NAATs for gonorrhoea are highly sensitive, but false positives can occur at non-genital sites. Gonococcal culture is not as sensitive but has high specificity and allows for antibiotic susceptibility testing. If a NAAT is positive, a swab should be obtained (if not already collected) for culture to determine antibiotic susceptibility prior to commencing treatment. Page 1210

Treatment Alternative treatments are not recommended because of high levels of resistance. For uncomplicated genital, anorectal or conjunctival infection:1 ceftriaxone 500 mg IM (dissolved in lignocaine 2 mL 1%) as a single dose plus azithromycin 1 g (o) as a single dose For uncomplicated pharyngeal infection: ceftriaxone 500 mg IM (dissolved in lignocaine 2 mL 1%) as a single dose plus azithromycin 2 g (o) as a single dose Sexual intercourse must be avoided until 7 days after both partners have received treatment. Test of cure should be performed 2 weeks after treatment is completed. Repeat testing at 3 months is recommended.

Mycoplasma genitalium3 Mycoplasma genitalium should be considered in patients with persistent genital tract symptoms when chlamydia and gonorrhoea have been excluded. It has been identified as a cause of urethritis in men, proctitis, cervicitis and PID. Testing has not been widely available, although access to commercial assays is increasing. NAAT can be performed on vaginal, cervical and rectal swabs or first-pass urine. Testing is only offered to asymptomatic patients if a symptomatic partner has tested positive and there is ongoing sexual contact. Optimal treatment is still unclear with increasing macrolide resistance, which is the reason why doxycycline is now recommended for empirical treatment of NGU. Consult local guidelines or specialist advice for recommended treatment. A commonly used regime includes:

doxycyline 100 mg (o) bd for 7 days initially followed by either azithromycin 1 g (o) on the first day, then 500 mg daily for 3 days or if macrolide resistance is confirmed or suspected, moxifloxacin 400 mg orally, daily for 7 days7 Page 1211

Anorectal syndromes (proctitis) Proctitis is characterised by anal discharge, pain and bowel symptoms including tenesmus and constipation. The most common causes are HSV, N. gonorrhoea and C. trachomatis (including lymphogranuloma venerum, LGV). Other causes include M. genitalium and non-STI causes such as inflammatory bowel disease, Campylobacter and Shigella. It is important to perform an examination and collect swabs at the time. Request NAAT for HSV and C. trachomatis and culture and NAAT for N. gonorrhoea. LGV should be tested in the event of a positive chlamydia test, which requires the same treatment as symptomatic anorectal chlamydia infection. Treatment should be empirical, taking into account the clinical picture and likelihood of particular STIs in the patient. Consider a combination of ceftriaxone 500 mg (IM) stat, doxycyline 100 mg (o) bd for 14–21 days and antiviral therapy (e.g. valaciclovir 500 mg (o) bd for 5 days).7

Epididymo-orchitis Epididymo-orchitis is covered in more detail in CHAPTER 103 . Consider chlamydia and gonorrhoea in all sexually active men, especially men aged 6 months of age. Apply to whole body from jawline down (include every flexure and area), leave overnight, then wash off. Wash clothing and linen after treatment and hang in sun. Repeat treatment in 7 days. or benzyl benzoate 25% emulsion left for 24 hours before washing off. Repeat after 7 days. Wash clothing and linen after treatment (preferably on a hot cycle) and hang in sun or dry in hot clothes dryer. Repeat treatment in 7 days. The whole family and close contacts must be treated, regardless of symptoms, which can take weeks to develop. Treatment of children younger than 6 months is covered in CHAPTER 112 . Note: Persistence of the itch after treatment is common, which can take 3 weeks or more to resolve. Also prescribe a moderately potent topical corticosteroid ± an oral antihistamine for the itch. Pubic lice permethrin 1% lotion: apply to pubic hair and surrounding area, leave for a minimum of 10 minutes and then wash off or pyrethrins 0.165% + piperonyl butoxide 1.65%, apply as above9 Shaving pubic hair is also effective. Bed clothes and underwear should be washed normally in hot water after treatment. Repeat the treatment after 7 days. Sometimes a third treatment is necessary. Sexual contacts and the family must be treated (young children can be infested from heavily infested parents). Where the lice or nits are attached to eyelashes, insecticides should not

be used: apply white soft paraffin (e.g. Vaseline) liberally to the lashes bd for 8 days. Then remove the nits with forceps.

Extragenital STIs Viral hepatitis Sexual activity is a factor in the transmission of hepatitis B (in particular), hepatitis A (where faecal–oral contact is involved), hepatitis C (rare and mostly in MSM, particularly with HIV) and hepatitis D (rare, in people with hepatitis B only). Hepatitis A vaccination is recommended for men who have sex with men and people who inject drugs.6 Page 1216

Hepatitis B In Western societies, sexual transmission of HBV is a common mode of spread and there is a higher prevalence in men who have sex with men (MSM), inmates in correctional facilities and injecting drug users. It is also prevalent among Aboriginal and Torres Strait Islander people living in remote areas and among people from culturally and linguistically diverse populations. Transmission can be via: parenteral contact including intravenous needle sharing and non-sterile tattoo equipment horizontal spread via exposure to blood and bodily fluids, including semen and vaginal secretions vertical from mother to child (usually intrapartum or perinatal) Even though studies have shown minute quantities of the virus can be present in saliva, tears and breast milk, they are not considered to be in high enough levels to transmit the virus. There is no specific therapy for hepatitis B, so prevention through vaccination is important. Antiviral and immunomodulatory drugs, including entecavir, tenofovir or peginterferon alfa-2a, can be used to prevent progression to liver failure and hepatocellular carcinoma.10 See CHAPTER 47 .

Immunisation Hepatitis B immunisation is provided to all Australian children and most people born from the 1990s should have received immunisation. It should be encouraged in hepatitis B marker-free people at risk of acquiring this infection. At-risk groups include Aboriginal and Torres Strait Islander people, MSM, people who inject drugs, sex workers and close contacts of anyone with hepatitis B.

Hepatitis C Sexual transmission of hepatitis C is considered rare but can occur in men who have sex with men, especially men with HIV. Over 90% of newly acquired hepatitis C infections in Australia are attributable to injecting drug use.11

HIV infection Infection with HIV causes chronic immune deficiency which, if untreated, leads to acquired immunodeficiency syndrome (AIDS) after an average period of 10 years after infection. Male‐to‐male sex continues to be the major HIV risk exposure in Australia, reported for 68% of HIV diagnoses in 2015, with heterosexual sex reported for 20%, both male‐to‐male sex and injecting drug use for 5%, and injecting drug use for 3%.12 HIV infection is considered in more detail in CHAPTER 18

.

Pre-exposure prophylaxis (PrEP) and postexposure prophylaxis (PEP) Daily use of coformulated tenofovir and emtricitabine for HIV pre-exposure prophylaxis (PrEP) by populations at high risk of HIV infection is now recommended through the 2015 WHO guidelines.13 Postexposure prophylaxis may be indicated when there is exposure to a known or potential HIV source. Antiretroviral treatment can be taken within 72 hours of exposure to reduce the chance of infection. Consider specialist advice, as every presentation for PEP should be assessed on a caseby-case basis.

When to refer Non-gonococcal urethritis: if symptoms do not resolve with treatment Gonorrhoea: if complications or symptoms do not resolve with treatment Syphilis: early referral or discussion with a sexual health specialist/service is strongly recommended, especially in pregnancy HIV: experience in prescribing antiretroviral treatment required

Genital warts: urethral, cervical warts refractory warts

Contact tracing5 Contact tracing is the responsibility of the infected individual but the health care provider also has a responsibility to initiate the process. The person infected with an STI should be advised to inform partners that they may have been exposed and should seek testing and treatment. All current partners should be treated simultaneously. Government guidelines on contact tracing can be found at www.contacttracing.ashm.org.au, and include how far back to trace, which varies between conditions. In the case of a positive chlamydia test, it is recommended to notify partners from the previous 6 months. Assistance from the local sexual health service is recommended for HIV and syphilis because it leads to more contacts being tested and treated. There are several helpful websites, including www.letthemknow.org.au, which assist with sending anonymous emails or text messages to contacts. Patient-delivered partner therapy, which is when the treating clinician provides another prescription for the current partner without a consultation, remains controversial. It should be restricted to times when other means of contact tracing are likely to fail. Clinicians are advised to check with their local health department for advice on the current legislation. Page 1217

Table 109.5

STIs: twelve golden rules of management (Sexual Health Society of Victoria)

1

An STI can only be diagnosed if the possibility is considered.

2

An adequate sexual history is paramount.

3

A proper history and careful examination must precede laboratory investigations.

4

Remember the sexual partner(s)!

5

Treatment consists of the appropriate antibiotic in correct dosage for an adequate period of time.

6

A patient concerned about STIs is probably an at-risk patient.

7

Counselling and education are fundamental to STI management.

8

Penicillin will not cure NGU.

9

Not all vaginal discharges are thrush.

10

Multiple, painful genital ulcers are most often due to herpes simplex.

11

Prompt, accurate treatment of PID is necessary to preserve fertility.

12

Remember the three Cs—Consent, Confidentiality and Counselling—of HIV antibody testing.

Good communication Note: Tests for STIs, including the HIV antibody test, should only be performed with the patient’s knowledge and consent, and after adequate counselling. An appointment should be made to give results in person, irrespective of the results.

Patient education resources Hand-out sheets from Murtagh’s Patient Education 8th edition: Chlamydial urethritis Gonorrhoea Hepatitis B Herpes: genital herpes HIV infection and AIDS HIV postexposure prophylaxis Lice: pubic lice Pelvic inflammatory disease Warts: genital warts

Resources Australian STI Management Guidelines for use in primary care. Australian Sexual Health Alliance. Available from: www.sti.guidelines.org.au.

References

1

Australian Sexual Health Alliance. Australian STI Management Guidelines for use in Primary Care. Available from: www.sti.guidelines.org.au, accessed April 2021.

2

Royal Australian College of General Practitioners. Guidelines for Preventive Activities in General Practice (9th edn). East Melbourne: Royal Australian College of General Practitioners, 2018.

3

Melbourne Sexual Health Centre. MSHC Treatment Guidelines. Available from: www.mshc.org.au, accessed April 2021.

4

Family Planning NSW. Reproductive and Sexual Health: An Australian Clinical Practice Handbook (3rd edn). Sydney: Family Planning NSW, 2016.

5

Bateson D et al. The who, what and from where of STIs: selective testing in asymptomatic patients. Medicine Today, 2012; 13(1): 12–21.

6

NSW STI Programs Unit. STI/HIV testing tool. Available from: www.stipu.nsw.gov.au/wp-content/uploads/STI-HIV-Testing-Tool-online.pdf, accessed April 2021.

7

Genital and sexually transmitted infections [published 2019]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2019. www.tg.org.au, accessed April 2021.

8

Cunningham AL et al. Prevalence of infection with herpes simplex virus types 1 and 2 in Australia: a nationwide population based survey. Sex Transm Infect, April 2006; 82(2): 164–8.

9

Insects and mites: bites and infestations [published 2015]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2015. www.tg.org.au, accessed April 2021.

10

Hepatitis B [updated 2021]. In: Therapeutic Guidelines [digital]. Melbourne: Therapeutic Guidelines Limited; 2021. www.tg.org.au, accessed April 2021.

11

Australian Government Department of Health. Fourth National Hepatitis C Strategy 2014– 2017: 3–4. Available from: www.health.gov.au/internet/main/publishing.nsf/content/A68444CDED77B3A9CA257BF 0001CFD80/$File/Hep-C-Strategy2014-v3.pdf, accessed April 2018.

12

The Kirby Institute. HIV, viral hepatitis and sexually transmissible infections in Australia Annual Surveillance Report 2016. Sydney: The Kirby Institute, UNSW, 2016: 24.

13

Wright E, Grulich A, Roy K. Australasian Society for HIV, Hepatitis and Sexual Health Medicine HIV pre-exposure prophylaxis: clinical guidelines. Journal of Virus Eradication, 2017; 3: 168–84.

Page 1218

110 Intimate partner violence and sexual assault

I want to tell people that family violence happens to [anybody], no matter how nice your house is, no matter how intelligent you are. ROSIE BATTY, 2014 Intimate partner violence (IPV) is defined by the World Health Organization as ‘any behaviour within an intimate relationship that causes physical, psychological or sexual harm to those in the relationship’. Domestic violence emphasises the setting of the violence, whereas family violence is a broader term that includes any violence or abuse that occurs in a family, e.g. elder abuse and adolescent violence against parents.1 There is a strong gender bias in IPV. We describe the gender bias in other conditions like rheumatoid arthritis where the majority of people affected are women. In IPV, it is mainly women and children who are subjected to IPV, and it is mainly men who perpetrate IPV. Of course, not all men are violent, and some women can be violent too. However, it is important to be aware of this strong gender bias. IPV occurs in both heterosexual and same sex relationships. Due to space limitations, this chapter will focus on IPV in heterosexual relationships perpetrated by men against women and children. IPV is the most common form of violence against women. The most common form of violence against men is perpetrated by other men, usually by strangers. GPs often say we do not see many women that have experienced IPV. However, it has been estimated that full-time GPs are seeing up to five women per week who have experienced some form of IPV in the past 12 months.2 A major problem in dealing with IPV is that it is hidden and women are reluctant to disclose IPV when visiting medical practitioners. Another complicating factor is the fact that many women experiencing IPV may not even be aware that this is the case. Women who have been subjected to IPV stress the importance of a trusting doctor–patient relationship, including confidentiality and a non-judgmental attitude. We usually think of IPV in terms of physical violence but it can take many forms.3 These

include: acts of physical violence, such as slapping, hitting, kicking and beating sexual violence, including forced sexual intercourse and other forms of sexual coercion emotional (psychological) abuse, such as insults, belittling, constant humiliation, intimidation (e.g. destroying things), threats of harm, threats to take away children controlling behaviours, including isolating a person from family and friends; monitoring their movements; and restricting access to financial resources, employment, education or medical care

Key facts and checkpoints4 In Australia, 1 in 4 women are subjected to IPV or emotional abuse by an intimate partner at some time. Women are nearly three times more likely to have experienced IPV than men. Of the women who are subjected to IPV, approximately a quarter are pregnant at the time and more than half have children in their care.5 Pregnancy is a high-risk time for IPV. IPV is the greatest health risk factor for women aged 25–44. In Australia, 8 women per day are hospitalised due to IPV and one woman per week is murdered by her current or former partner. Alcohol is a factor in 45% of IPV incidents (i.e. neither the sole cause, nor an excuse).6 Leaving is the greatest time of risk—approximately two-thirds of women who are murdered by their (ex) partner are murdered around the time of leaving.7

Practice tip Safety is the highest priority when working with women who are subjected to IPV.

Possible presentations8

In women:

Page 1219

frequent presentations of non-specific symptoms (e.g. chronic pain, headaches, functional gastrointestinal disorders, sexual dysfunction) psychological problems (depression, somatiform disorders, anxiety disorders, panic attacks, PTSD, suicidal ideation) insomnia eating disorders drug and alcohol problems physical injuries: usually bruising caused by punching, kicking or biting; also fractures, burns, genital trauma pregnancy and childbirth (e.g. unintended pregnancies, forced terminations, miscarriage, poor antenatal care, low birth weight, premature labour)—it is recommended to ask all pregnant women about violence during antenatal care In children: bedwetting, sleeping disorders, anxiety, stress, depression, withdrawal aggressive behaviour and language problems at school chronic somatic problems and frequent presentations drug and alcohol abuse suicidal ideation in adolescence

Diagnosis Diagnosing the problem, which is usually ‘hidden’, can be a challenge to the GP. It is important to set aside common assumptions about the problem and have a high index of suspicion. If you suspect domestic violence—ASK! The doctor has to take the initiative because patients rarely complain about the violence.7 It is also the doctor’s responsibility to ensure the man leaves the room to talk to the woman alone. Possible questions include: How are things at home? How are things with your partner? Do you get scared or frightened of your partner?

Is there a lot of tension in your relationship? Do you feel your views are respected? Has your partner ever physically threatened or hurt you? Have you ever felt unsafe? It is advised to universally screen all pregnant women for intimate partner violence.

Practice tip Screen all pregnant women for intimate partner violence.

Barriers to communication about abuse9 Concerns about confidentiality Perceptions about doctors: do not ask directly have no time are not interested Embarrassment Fear of involving police/courts Fear of shaming family Fear of partner retaliation

Assessment Delineate the problem: pattern of violence; effect on the woman and her children; resources available to women; social/cultural environment. Examine and investigate presenting symptoms. Check for coexisting injuries (common target areas are breast, chest, abdomen and buttocks); inspect the ears, teeth and jaw. Check the patient’s general health status.

Look for signs of alcohol or drug abuse. Keep accurate records and consider taking photographs. X-rays may show old fractures.

Women who are subjected to violence Women who have been subjected to violence come from all socioeconomic and cultural groups. As a rule they enter the relationship as normal, independent and intelligent women but gradually lose self-esteem and become increasingly compliant as a coping strategy. Unfortunately, many victims are led to believe that somehow they are at fault. Groups of women at greater risk include Aboriginal and Torres Strait Islander women, young women, pregnant women, women separating from their partners, women with disability, women experiencing financial hardship and women who witnessed or were subject to violence as children.4 Most women simply want the violence to stop rather than leave their relationships. Page 1220 They are usually constrained from leaving due to concerns for their children, feeling they have nowhere to go or financial dependance. Often the reason they stay is fear of retaliation. Women may be at various stages of change in terms of taking action, in accordance with Prochaska and DiClemente’s model of change (see CHAPTER 12 , FIG. 12.4 ), and the counsellor should be aware of this process.

Men who use violence10 Men who use violence come from all walks of life and social and ethnic groups. There are often no distinguishing characteristics to identify a man who will be violent towards his partner. Men who use violence tend to minimise responsibility for their use of violence, blame the victim or external factors, and greatly under-report their use of violence. Perpetrators use abuse and violence to maintain their power and control in a relationship. This is often based on community norms and expectations, along with risk factors including mental disorders, substance abuse, unemployment, poverty and coming from a family where intimate partner abuse occurred.8 It is important to note that these risk factors are not causal.

Cycle of violence A predictable pattern that is referred to as the ‘cycle of violence’ has been identified in many intimate relationships. It is controlled by the perpetrator, while the victim feels confused and helpless. The cycle repeats itself with a tendency for the violence to increase in severity (see FIG. 110.1 ).

FIGURE 110.1 The cycle of IPV

Management The key to management is initial recognition of the problem and establishment of empathic caring and support for the woman and family. It must be emphasised that men who use violence (as in most criminal activity) do not readily change their behavioural pattern and thus there is minimal prospect of the violence decreasing unless there is a dramatic reason to change. A management strategy is presented in TABLE 110.1 . The immediate safety of women and any children is always the prime working rule. Table 110.1

Management strategy for domestic violence

Suspect domestic violence, treat any physical injuries ↓ Establish the diagnosis ↓ Assess safety and initiate safety planning

↓ Establish an empathic, trusting relationship ↓ Build the victim’s self-esteem and emphasise coping skills ↓ Make effective use of community resources: support services women’s support group domestic violence resource centre (in each capital city) social services/police social workers private counsellors and psychologists

Useful strategies11 Do: talk to the woman alone believe her validate the decision to disclose provide a strong statement that violence is never okay reassure her that it is not her fault enquire about and express concern for her safety give information (i.e. about the course of action available to her, contacts for legal advice) acknowledge the complexity of the issue respect her right to make her own decisions provide follow-up and continued support Page 1221

Harmful strategies Don’t: deny domestic violence

minimise the importance of domestic violence blame the victim treat with tranquillisers refer to a psychiatrist refer to marriage guidance, but do refer to specialist counsellors set explicit criteria/rules (takes away victim’s power yet again)

Dealing with perpetrators It is uncommon to get the cooperation of the perpetrator in the management process. The perpetrator has to admit that they have a problem before effective counselling can begin. If they do seek help they require counselling by a skilled and experienced practitioner, as treatment will be prolonged and complex. In early intervention, the GP’s role is to:10 briefly assess men prepare them to accept a referral to a behaviour change program undertake alternative interventions to decrease the risk of violence if a referral is not taken up

Safety8 Women may not feel safe to go home and may require urgent crisis referral or an urgent safety plan. When assessing a woman’s immediate safety, it is important to take the following into account: the woman’s own assessment of her safety presence of risk factors in the perpetrator, including severity and duration of previous violence, history of arrest and incarceration, drug and alcohol use, access to weapons and unemployment your own professional judgment Where you reasonably believe a patient is in imminent threat of danger, you should seek their consent to report the matter to the police. Children at risk require mandatory reporting to child protection. If a violent incident develops or is imminent, it is important to assist the victim in developing a safety plan. One such plan is: Compile a list of emergency numbers. Identify family and friends who can provide support.

Put aside emergency money. Pack a bag of clothing, medications and toiletries. Organise a safe place to store valuables and important documents. Identify a safe place for the woman to go to and how she will get there. Ask neighbours to call police if they hear any disturbance. As a general rule the most effective intervention in arresting the violence is to arrest the violent person.7 Consider legal interventions such as intervention orders, protection orders (AVOs) and restraining orders.

Continuing care11 It is important to keep in mind that the woman’s ability to change her situation may be very limited. It is also important to: assess safety regularly, checking for escalating violence provide emotional support ensure confidentiality build her self-esteem by acknowledging the coping skills she has developed empower her to take control of decision-making: ask what she needs, explore options and present services available familiarise yourself with referral services and their processes: domestic violence resource centres relationship services legal aid social workers social services/police private counsellors and psychologists have information available for her to take if appropriate offer assistance in contacting referral services if this would be of help

Sexual assault According to Australian statistics, since the age of 15 years, 1 in 5 women and 1 in 20 men have been sexually assaulted and/or threatened.4 Sexual assault is defined broadly as any unwanted sexual contact that occurs without a person’s consent and which makes the victim feel uncomfortable, frightened or threatened. The behaviour can include any activity from sexual harassment through to life-threatening rape. The majority of perpetrators of sexual assault are known to their victims and a high proportion of perpetrators are current or ex-partners. For this reason, it is important to assess whether the patient is safe to return to their accommodation. Stranger assaults only make up approximately 15% of sexual assault cases.12 Survivors of sexual assault should be allowed to accept or decline various assessment or treatment options offered by the practitioner. Many victims do not report sexual assault to police because of fear, humiliation or shame. Medical practitioners dealing with alleged sexual assault should familiarise themselves with the laws applicable in their state/territory. Self-care for clinicians is also of particular importance when dealing with this distressing problem.

Page 1222

Disclosure of sexual assault Offer and provide privacy, safety and emotional support. Take the time to explain confidentiality and its limits, so that the patient is aware that the assault will not be discussed with the police or their family without their consent. Believe them, listen to them and be non-judgmental. Five important things to say: I am sorry this happened to you. This is a crime—it was not your fault. It’s good that you have talked to me about this. I will do what I can to help you. You are safe now (if applicable).

History Most jurisdictions require that the first person who hears an allegation of sexual assault must give evidence if the complaint comes to trial, so document the exact words used. If the patient presents following recent sexual assault, the following information will help guide management:13,14

time and place of assault brief description of the assault (i.e. what went where) condom use details of the assailant preceding drug or alcohol use violence used and any injuries current contraception risk assessment for patient and any children accommodation issues When patients disclose sexual assault that took place in the past, it is not necessary or advisable to ask all the details about what happened at the first disclosure. The patient may not be ready to tell you details and doing so may easily take them back to their trauma, leading them to decompensate. Let the patient know that you are ready to listen to more details if or when they are comfortable telling you.15

Management A patient may disclose sexual assault immediately or years after the event. Management depends on when the assault occurred.

Recent sexual assault8 Management options include: forensic examination by a specialist sexual assault service, preferably within 72 hours of the assault—this is still advised if the patient is undecided about reporting the assault assessment and treatment of physical injuries emergency contraception if indicated testing for pregnancy or STIs according to need urine or blood drug testing if suspected drug-assisted sexual assault, i.e. when the patient has no memory of events and time or other suspicious circumstances—seek specialist advice address safety and supports at home—alternative accommodation may be required follow-up—patients may need to return for follow-up at 2 weeks and 3 months following STI

checks sexual assault counselling and support groups ongoing support and monitoring of symptoms of trauma

STI testing and prophylaxis16 Take swabs and/or first-pass urine for chlamydia, gonorrhoea and trichomoniasis (PCR), repeat after 2 weeks Collect swabs from the oropharynx or rectum if indicated; consider self-collection of swabs Take blood for HIV, syphilis and hepatitis B; repeat at 6 and 12 weeks STI prophylaxis Prophylactic antimicrobial therapy is generally not required If seen within 72 hours, consider prophylaxis for hepatitis B and HIV if hepatitis B non-immune, no history of vaccination or unknown status, consider hepatitis B immunoglobulin (should be given within 72 hours), along with the hepatitis B vaccine (within 14 days) consider referral for postexposure prophylaxis (within 72 hours) Page 1223

Past sexual assault The long‐term physical and psychological consequences of sexual abuse are many, often somatic or psychological, and can have devastating consequences for the victim. Disclosure to a trusted health professional may be the first step in the healing process. A sensitive response to disclosure can be critical in this process. Offer referral to an experienced therapist or sexual assault specialist counselling service, with access to up-to-date knowledge about relevant reporting and legal processes.15 Page 1224

Resources Hindmarsh E, Roberts G, eds. RACGP Manual: Abuse & Violence, Working with Our Patients in General Practice (The White Book) (PDFIMB). National Sexual Assault, Domestic Family Violence Counselling Service. 1800 RESPECT (call 1800 737 732). www.1800respect.org.au, accessed April 2021.

References 1

Long D, Lee S, Coles JY. Family violence: an illustrated guide to the terminology. Med J Aust, 2017; 207(6): 270.

2

Hegarty K, Bush R. Prevalence and associations of partner abuse in women attending general practice: a cross-sectional survey. Aust NZ J Public Health, 2002; 26(5): 437–42.

3

World Health Organization. Understanding and addressing violence against women, 2012. Available from: https://apps.who.int/iris/bitstream/handle/10665/77432/WHO_RHR_12.36_eng.pdf;jsessio nid=DAC8304B0CA9F5CDE2F35C96B4ABD0FF?sequence=1, accessed April 2021.

4

Australian Institute of Health and Welfare. Family, domestic and sexual violence in Australia. Canberra: AIHW, 2018.

5

Campo M. Domestic and family violence in pregnancy and early parenthood. CFCA, December 2015: ix–xii.

6

Mouzos J, Makkai T. Women’s experience of male violence: findings from the Australian component of the International Violence Against Women survey. Canberra: Australian Institute of Criminology, 2004.

7

Hegarty K. Domestic violence: how to treat. Australian Doctor, 20 February 2009: 29–36.

8

RACGP. Abuse and Violence: Working with Our Patients in General Practice (4th edn). Melbourne: The Royal Australian College of General Practitioners, 2014: 1–39.

9

Rodriguez MA et al. The factors associated with disclosure of intimate partner abuse to clinicians. J Fam Pract, 2001; 50(4): 338–44.

10

Hegarty K et al. Identifying and responding to men who use violence in their intimate relationships. Aust Fam Physician, April 2016; 45(4): 176–81.

11

Australian Medical Association. Supporting patients experiencing family violence, May 2015: 6–9. Available from: https://ama.com.au/article/ama-family-violence-resource, accessed April 2021.

12

Australian Bureau of Statistics. Personal Safety Survey. Canberra: Australian Bureau of Statistics, 2016. Available from: www.abs.gov.au/ausstats/[email protected]/mf/4906.0, accessed April 2021.

13

Mein JK et al. Management of acute adult sexual assault. Med J Aust, 2003; 178: 226–30.

14

Howlett M. Clinical assessment of sexual assault. O&G Magazine, Summer 2017; 19(4): 26–8.

15

Kinsella P, Uebergang M. Disclosures of sexual abuse: what do you do next? Aust Fam Physician, March 2015; 44(4): 122–4.

16

Australian Sexual Health Alliance. Australian STI management guidelines for use in primary care. Available from: www.sti.guidelines.org.au, accessed April 2021.

Page 1225

Part 10 Problems of the skin Page 1226

111 A diagnostic and management approach to skin problems

The skilful doctor knows by observation, the mediocre doctor by interrogation, the ordinary doctor by palpation. CHANG CHUNG-CHING (C. 170–196 CE) The diagnosis of skin problems depends on astute clinical skills based on a systematic history and examination and, of course, experience. If the diagnosis is in doubt, it is appropriate to refer the patient to a skilled cooperative consultant, as the referral process is an excellent educational opportunity for the GP. Another opinion from a colleague/s in a group practice is also very educative. At least, cross-referencing the skin lesion with a colour atlas facilitates the learning process.

Terminology of skin lesions Primary lesions Macule. Circumscribed area of altered skin colour (Latin for stain) without elevation 1 cm diameter (see FIG. 111.1

).

Papule. Palpable mass on skin surface 1 cm diameter. Nodule. A circumscribed, solid palpable mass >1 cm diameter (see FIG. 111.2

).

Wheal. An area of dermal oedema (can be any size), which is pale and compressible.

Angio-oedema. A diffuse area of oedema extending into subcutaneous tissue. Vesicle. A fluid-filled blister 0.5 cm diameter (see FIG. 111.3

).

).

Pustule. A visible collection of pus in the skin 1 cm diameter. Furuncle. A purulent infected hair follicle; includes: folliculitis (small furuncles) boils (larger furuncles) Carbuncle. A cluster of boils discharging through several openings. Purpura. A circumscribed deposit of blood >0.5 cm in diameter. May be palpable or nonpalpable. Petechiae. Purpuric lesions 0.9

50 to 75%

++++

>75 to 100%

Source: References for prevalence data available from [email protected] on request

Mental health The mental health effects of trauma in refugees are wide ranging and long lasting. A strong therapeutic relationship informed by trauma-sensitive care, which is patient led, and allows sensitive assessment over time, is the foundation to care. Resettlement difficulties cannot be overemphasised as a contributor to psychological stress. Newly arrived humanitarian entrants are often socially isolated and bereft of their usual family and social supports, and their fledgling communities often lack the capacity to support them. Those with intact family and social support tend to recover more quickly. Asylum seekers continue to suffer profound and ongoing uncertainty as to their future. They suffer the trauma of having to prove their experiences to disbelieving or hostile government agencies with variable access to Medicare, work rights, case support and legal support. Detention and temporary visas cause well-documented severe, and often irreparable, psychological illness. Refugees and asylum seekers may be remarkably resilient and have variable Page 1450 experiences and episodes of mental illness. Common problems are listed in TABLE 128.2 . People of refugee backgrounds may recover and settle extremely quickly once the basics of safety (housing, adequate income, the address of physical and psychological health issues, education and training for themselves and their children) have been established. Others may require long-term care with GPs or specialist mental health and/or trauma services.

Iron deficiency Iron deficiency is common in people from refugee backgrounds, particularly children and women of child-bearing age in whom universal new arrival screening is recommended. In Australian-born children with mothers of refugee backgrounds, consider screening for iron deficiency and check for excessive milk intake and developmental problems. Also check iron studies for anyone with fatigue, a history of poor nutrition, chronic gastrointestinal infections or anaemia on screening FBE. Iron deficiency is easily treated with education about dietary sources and oral replacement. Consider iron infusion in those with intolerance to oral replacement or severe iron deficiency.

Vitamin D Vitamin D deficiency is common in people from refugee backgrounds of all ages. Causes include lack of exposure to sunlight, darkly pigmented skin and low levels in breast milk. Children and adolescents are at particular risk due to rapid growth. Most children and adolescents are asymptomatic but check for signs of rickets, looking for leg bowing, delayed walking, muscle aches and leg weakness. Blood levels of < 50 nmol/L require treatment,12 which may be given in daily, weekly, monthly or 6-monthly Stoss doses if compliance is difficult. Adequate calcium intake is also important.

Vitamin B12 It is important to recognise and treat vitamin B12 deficiency to prevent neurological complications. Screening is recommended for people from Bhutan, Afghanistan, Iran and the Horn of Africa or those with food insecurity or vegans within 6 months of arrival.

Haemoglobinopathy Genetic disorders such as sickle-cell disease, alpha and beta thalaessaemia and G6PD deficiency are more common in people from refugee backgrounds. Affected people are usually asymptomatic carriers. Hb electrophoresis should be performed in pre-pregnancy screening or if microcytic or hypochromic anaemia persists after correcting for iron deficiency.

Hepatitis B The rate of hepatitis B infection in people from refugee backgrounds varies but is generally higher than that of the Australian-born community. Infection is usually asymptomatic, so screening with HBsAg, HBsAb and HBcAb is recommended for all people of refugee background, even some years after arrival if not already performed. Those with positive HBsAg should have further investigations and their household and sexual partners should be screened. Those who are not immune should have hepatitis B vaccination completed, and household and close contacts of those with infection need their immunity checked post vaccination.

Malaria Malaria is common in many refugee source countries and some countries of refugee and asylum seeker transit. A single screening thick and thin film with a rapid diagnostic test (RDT) is recommended for those travelling from or through a country with endemic malaria within 3 months of arrival. If a person from a refugee background presents with a fever of unknown origin within 12 months of travel from an endemic area, malaria should be considered and urgent 3x thick and thin films and malaria RDT ordered.

Schistosomiasis

Schistosomiasis is a chronic parasitic infection acquired from swimming or bathing in endemic fresh water in Africa, South-East Asia, parts of the Middle East and South America. There are gastrointestinal and urinary tract forms, which often remain asymptomatic until end organ damage. Screening with schistosoma serology is recommended in people from countries of high prevalence.11 Treatment with praziquantel is usually well tolerated. Ensure further investigation and follow-up of positive cases according to guidelines.13 Page 1451

Strongyloides Strongyloides is an intestinal nematode, common in refugees with a prevalence up to 11% in some groups. It is transmitted by contact with infected soil or surface water and can persist asymptomatically for decades. Mild symptoms include diarrhoea, recurrent abdominal pain, skin or respiratory symptoms. If immunocompromised, it may cause fulminant infection and death. Screening with Strongyloides Ab is recommended for all. Positive cases need further investigation with stool samples. Treatment is a short course of ivermectin according to weight, then follow-up serology at 6 and 12 months.

Intestinal parasites Gastrointestinal parasites are common in new arrivals from high prevalence backgrounds. Predeparture empirical treatment with albendazole is common. If there are gastrointestinal symptoms or eosinophillia despite empirical treatment, test stool microscopy and treat appropriately. Note: Albendazole should not be used in the first trimester of pregnancy or for those with CNS symptoms and/or a travel history compatible with neurocysticercosis.

Tuberculosis (TB) TB is a common infection in refugee source countries with active and latent (asymptomatic) forms. Active TB is screened for prior to immigration to Australia in humanitarian entrants and is most common in the first 5 years post immigration. If people from refugee backgrounds present with night-sweats, fevers, persistent cough, haemoptysis, chronic bone pains or unexplained symptoms, have a high index of suspicion for active TB. It is recommended to screen for latent TB post arrival in all who would benefit from chemoprophylaxis,13 which decreases the risk of reactivation of latent TB into the active form. Screening is with Tuberculin Skin Test (Mantoux) or IGRA. Those with positive results require examination for signs of active TB, CXR and referral for consideration of chemoprophylaxis, which is usually isoniazid for 6–9 months.

Blood-borne viruses HIV and Hepatitis C are uncommon in people from refugee backgrounds in Australia, but given

their significance and treatment options, both are recommended to be included in universal screening.13

Sexually transmitted infections Screen for STIs in those with risk factors. A sexual history should be taken sensitively and privately, with the knowledge sexual assault is common in women of refugee backgrounds.

Helicobacter Pylori infection People immigrating from non-Western countries have a higher prevalence of Helicobacter infections than the Australian population.14 These may present with upper gastrointestinal symptoms, anorexia, weight loss or failure to thrive in children. Investigate those with symptoms or a family history of gastric cancer with Helicobacter faecal Ag or urease breath test, and treat according to guidelines.

Immunisation Almost all people from refugee backgrounds arriving in Australia need catch-up vaccination. All immunisation records, including those from overseas and pre-departure records, should be reviewed and updated into the Australian Immunisation Register. Check antibodies for hepatitis B, rubella in child-bearing-aged women and varicella in those >14. All other immunisations which are not documented according to the Australian schedule for the patient’s age should be updated with a catch-up vaccines schedule. See the Australian Immunisation handbook for how to develop a catch-up schedule (https://immunisationhandbook.health.gov.au/catch-up-vaccination).

Chronic disease Some refugee source countries have a high incidence of chronic non-communicable disease (see TABLE 128.2 ). This includes obesity, type 2 diabetes, cardiovascular disease, hyperlipidaemia, hypertension, chronic lung disease and musculoskeletal complaints. People of refugee backgrounds from high prevalence countries should be screened early for diabetes and cardiovascular disease, particularly if they have additional risk factors such as increased BMI or waist circumference. Culturally tailored management and active follow-up is important in chronic disease prevention and care.

Developmental and other disabilities There has been an increase in arrivals with significant disabilities to Australia. Both children with developmental delay and adults with intellectual disability may have a significant delay to their diagnosis and management. Further paediatric and specialist assessment and early referral for additional support is recommended.

Hearing, vision and oral health Hearing and vision impairment and dental caries are common in people from refugee backgrounds. Clinical assessment of vision, hearing and dental health is recommended for all. Referral for routine dental care for all is recommended. Optometry review is recommended for African patients > 40 years and others > 50. Early referral for optometry and audiology for those with symptoms is recommended. Page 1452

Post-immigration health assessment People from refugee backgrounds have varying screening prior to arrival in Australia. It is recommended all people of refugee background have comprehensive post-arrival health assessments, preferably within 1 month of arrival (see FIGURE 128.1 ). The usual approach to history taking should be followed with an additional emphasis on psychosocial and migration history and screening for common conditions. See TABLE 128.3 . Table 128.3

Refugee health assessment

History Presenting problem/s, patient’s concerns Past medical history, including NCDs, e.g. cardiovascular, diabetes Family history, medications, allergies Migration history and pre-migration health information (including documented immunisations) Past infection or contact with TB, malaria, parasites and blood-borne viruses Review of systems (particularly respiratory and gastrointestinal) Hearing, vision and dental problems Disabilities Lifestyle/risk factors: nutrition, vitamin D risk, e.g. dark skin, lack of sun exposure smoking, alcohol, recreational drugs (consider regional use, e.g. sheesha, khat) Psychosocial/mental health settlement stressors (see TABLE 128.1 ) sleep, appetite, energy, mood, interests and ADLs, memory, concentration, ‘suddenly fearful’ relationships/family functioning Child/adolescent health: add developmental history, education, nightmares, enuresis Women’s health: add pregnancies/births, contraception, breastfeeding, cervical and breast screening, female circumcision/traditional cutting

Physical examination and conditions to consider General: fever (malaria), pallor (anaemia), BP (hypertension) Nutritional status and growth, BMI, waist–hip ratio, percentiles (children), underor overweight ENT (chronic infections, hearing impairment) Skin: rash (parasites, fungal); BCG scar Oro-dental (caries, vitamin deficiencies) Goitre (iodine deficiency) Rickets (vitamin D deficiency) Cervical, axillary and inguinal lymphadenopathy (TB and HIV) Cardiorespiratory exam (TB, COPD, CVD, RHD) Signs of chronic liver disease (malaria, HBV, schistosomiasis, TB, HIV) Musculoskeletal deformities; scars (accidents, injuries, torture) Visual acuity (e.g. refractive errors, cataract, glaucoma) Appearance, affect and behaviour (mental health issues) Investigations All: FBE, HBs sAg, HBsAb, HBcAb, Strongyloides Ab HIV, >/=15, if unaccompanied minor or clinical concerns 14 if no known Hx disease Rubella Ab women of child-bearing age Ferritin women, children and men with risk factors Vitamin D level, also check Ca, PO4 and ALP in children if risk of deficiency B12 level, arrival 35 consider if high prevalence country or other RF Syphilis serology, urine/vaginal swab chlamydia/gonorrhoea PCR, if risk STIs or request Helicobacter stool Ag or breath test, upper GI symptoms or Fam Hx gastric cancer Stool micro, if no recent albendazole, or abdominal pain/diarrhoea/eosinophil Abbreviations: NCDs = chronic non-communicable disease; ADLs = activities of daily living

Impact of COVID-19 Page 1453 COVID-19 has further disadvantaged people from refugee backgrounds in Australia. There have been barriers to accessing translated information, fears of going out and racism. Barriers to usual care have increased as has psychological distress and financial difficulties.

Many asylum seekers are facing destitution. There was no data about the prevalence of COVID19 in people of refugee backgrounds in Australia at the time of writing.

Role of general practitioner The GP is ideally placed to facilitate long-term care for patients of refugee backgrounds and their families. Allow for time, work with interpreters and consider the impact of your patient’s background on their presenting problem. Complete refugee health assessments, ensure follow-up and preventive health care and coordinate specialist and settlement services when needed. Take an incremental, patient-led approach to management and foster greatly valued therapeutic relationships.

Practice tips: ASSSK Ask about: country of origin, preferred language, year of arrival, need for an interpreter, visa type (refugee/woman at risk/orphan/spousal visa or asylum seeker) Settlement and access to services: How is settlement going, e.g. housing, financial, education and training? Other household/family members/children? What services or agencies are assisting you? Could I have their contact details? Screening, physical health issues and specialist visits: Is health screening required? If my client is not newly arrived, should I screen for common conditions? Other medical services involved, especially hospital outpatient/procedures. Psychosocial issues, separation and support: screen for mental health related issues, consider the impact of separation from/death of family and friends as a cause of presentation/illness. Support for client and clinician: What support does my client need? E.g. social worker, case support, MCHN. What support do I need? E.g. websites, colleagues, ID opinion. Kindness: ‘Every clinical encounter can be an opportunity for healing.’

Resources Australian Refugee Health Practice Guide. Primary care for people from refugee backgrounds. http://refugeehealthguide.org.au Australian Government Department of Health and Ageing. Catch-up vaccination. In: Australian Immunisation Handbook. Available from: https://immunisationhandbook.health.gov.au/catch-

up-vaccination Page 1454

References 1

United Nations High Commissioner for Refugees. Basic facts (online). Available from: www.unhrc.org.

2

Department of Home Affairs. 2018–19 Humanitarian Program Outcomes. Available from: https://www.homeaffairs.gov.au/research-and-stats/files/australia-offshore-humanitarianprogram-2018-19-glance.pdf, accessed May 2021.

3

Department of Home Affairs. Immigration detention and community statistics summary, 30 September 2020. Available from: https://www.homeaffairs.gov.au/research-andstats/files/immigration-detention-statistics-30-september-2020.pdf, accessed May 2021.

4

Department of Home Affairs. Monthly update: Onshore protection (subclass 866) visa processing, October 2020. Available from: https://www.homeaffairs.gov.au/research-andstats/files/monthly-update-onshore-protection-866-visa-processing-october-2020.pdf.

5

Department of Home Affairs. Key statistics as at 31 March 2021. Available from: https://www.homeaffairs.gov.au/about-us-subsite/files/population-and-number-of-peopleresettled.pdf, accessed May 2021.

6

Royal Australian College of General Practitioners (RACGP). Guide for clinicians working with interpreters in healthcare settings [published January 2019]. Available from: https://www.racgp.org.au/clinical-resources/clinical-guidelines/guidelines-by-topic/viewall-guidelines-by-topic/refugee-health/guide-for-clinicians-working-with-interpreters, accessed May 2021.

7

Gardiner J, Walker K. Compassionate listening: managing psychological trauma in refugees. Aust Fam Pract, April 2010; 39(4): 198–203.

8

Blue Knot Foundation. Trauma-informed care and practice. Available from: https://www.blueknot.org.au/Workers-Practitioners/For-Health-Professionals/Resourcesfor-Health-Professionals/Trauma-Informed-Care-and-practice, accessed May 2021.

9

Centre for Culture, Ethnicity & Health. Health literacy—using teach-back via an interpreter. Reviewed March 2017. Available from: https://www.ceh.org.au/resourcehub/health-literacy-using-teach-back-via-an-interpreter/, accessed May 2021.

10

Parajuli J, Horey D. How can healthcare professionals address poor health service utilisation among refugees after resettlement in Australia? A narrative systematic review of recent evidence. Aust J Prim Health, June 2019; 25(3): 205–13.

11

Cheng I-Hao et al. Refugee experiences of general practices in countries of resettlement: a literature review. British J Gen Pract, March 2015, 65(632): 171–176.

12

NPS MedicineWise. Vitamin D supplementation in musculoskeletal health: what’s new? 8 July 2019. Available from: https://www.nps.org.au/news/vitamin-d-supplementation, accessed May 2021.

13

Chaves NJ et al. Refugee Guidelines: Recommenations for comprehensive post-arrival health assessment for people of refugee-like backgrounds (2nd ed.). Expert Advisory Group. Australian Society for Infectious Disease and Refugee Health Network of Australia, 2016. Accessed May 2021.

14

Refugee Health Network of Australia. Helicobacter Pylori. In: Recommendations for comprehensive post-arrival health assessment for people from refugee-like backgrounds. Australian Refugee Health Practice Guide. Available from: http://refugeehealthguide.org.au/helicobacter-pylori/, accessed May 2021.

Page 1455

129 Travellers’ health and tropical medicine

Our lot is a perilous age … but where shall we fly to escape from pestilences that come and pestilences that do not come, from ships that bring us yellow fever, from cattle diseases that can only be exterminated by exterminating the cattle, from infectious patients whose pulses must be felt with a pair of tongs and their chests explored with tarred stethoscopes. JACOB BIGELOW, 1860

Principles of pre-travel health care Advise the patient to plan early—at least 8 weeks beforehand. Register with Smartraveller (provided by the Australian Department of Foreign Affairs and Trade (DFAT)). Advise a dental check before departure. Allow adequate time for consultation (e.g. 30–45 minutes). Individualise advice. Provide current information. Provide written as well as verbal advice. Provide a letter concerning existing medical illness and treatment. Encourage personal responsibility.

Key facts and checkpoints The main diseases facing the international traveller are traveller’s diarrhoea

(usually relatively mild) and malaria, especially the potentially lethal Plasmodium falciparum malaria. Register with Smartraveller (provided by the Australian Department of Foreign Affairs and Trade (DFAT)). Most cases of traveller’s diarrhoea are caused by enterotoxigenic Escherichia coli, Shigella sp. and Campylobacter species. Enteroinvasive E. coli (a different serotype) produces a dysentery-like illness similar to Shigella. Traveller’s diarrhoea is contracted mainly from contaminated water and ice used for beverages, washing food or utensils, or cleaning teeth. Poliomyelitis is endemic in at least 20 countries and thus immunisation for polio is still important. One bite from an infected mosquito during a single overnight stop in a malaria area can result in a possible lethal infection. Infections transmitted by mosquitoes include malaria, yellow fever, Rift Valley fever, Japanese B encephalitis, chikungunya, Zika and dengue fever. Avoiding their bites is excellent prevention. Every year approximately 1000 Australians catch malaria while travelling overseas. Malaria is a dusk-till-dawn risk only, but bites from daytime mosquitoes can cause dengue. P. falciparum malaria is steadily increasing, as is resistance to newer antimalarials. It is important for GPs to consult a travel medicine database to obtain specific information about ‘at risk’ countries. Avoid tattooing, ear-piercing, acupuncture or any skin puncturing while overseas. The commonest causes of death in travellers overseas are trauma (26%), particularly traffic accidents, and homicide (16.9%). Travellers visiting family and relatives (TVFR) in some countries are at risk of contracting preventable travel-related illnesses.

Gastrointestinal infections

The commonest problem facing travellers is traveller’s diarrhoea but other important diseases caused by poor sanitation include hepatitis A and worm infestations, such as hookworm and schistosomiasis. Contamination of food and water is a major problem, especially in third world countries. Reputable soft drinks, such as Coca-Cola, should be recommended for drinking. Indian-style tea, in which the milk is boiled with tea, is usually safe, but tea with added milk is not. The food handlers can be infected and the water used to wash food may be contaminated. Page 1456

Traveller’s diarrhoea Traveller’s diarrhoea is a special problem in Mexico, Nepal, India, Pakistan, South-East Asia, Latin America, the Middle East and Central Africa and its many colourful labels include ‘Bali Belly’, ‘Gippy Tummy’, ‘Rangoon Runs’, ‘Tokyo Trots’ and ‘Montezuma’s Revenge’. It occurs about 6–12 hours after taking infected food or water. The illness is usually mild and lasts only 2 or 3 days. It is unusual for it to last longer than 5 days. Symptoms include abdominal cramps, frequent diarrhoea with loose, watery bowel motions and possible vomiting. Very severe diarrhoea, especially if associated with the passing of blood or mucus, may be a feature of Shigella sp. or Campylobacter sp. infections and amoebiasis. Traveller’s diarrhoea is caused by a wide variety of organisms but mainly enterotoxigenic E. coli (ETEC), Campylobacter sp., Shigella sp. and Salmonella sp. Travellers are infected because they are exposed to slightly different types or strains of E. coli from the ones they are used to at home.1 Norovirus is a common cause on cruise ships.

Treatment1 Chemoprophylaxis is not recommended in healthy travellers. Refer to FIGURE 129.1

.1,2

FIGURE 129.1 Algorithm for adult travellers with acute diarrhoea Source: Locke1

The key factor in treatment is rehydration.

Mild diarrhoea Maintain fluid intake—consider Gastrolyte. Consider antimotility agents (judicious use: if no blood in stools)—avoid in children. loperamide (Imodium) 2 caps statim, then 1 after each unformed stool (max. 8 caps/day) or

diphenoxylate with atropine (Lomotil) 2 tablets statim, then 1–2 (o) 8 hourly Imodium is the preferred agent. Page 1457

Moderate to severe diarrhoea1 Consider admission to hospital if severe (toxic and febrile). Attend to hydration. Patient can self-administer antibiotic—e.g. single dose norfloxacin or azithromycin (especially in India, Nepal and Thailand). Loperamide in adults. Antibiotic: azithromycin, ciprofloxacin or norfloxacin (usually 3 days). Note: There is increasing resistance to doxycycline and cotrimoxazole, especially in South-East Asia.

Persistent diarrhoea Any travellers with persistent diarrhoea after visiting less-developed countries, especially India and China, may have a protozoal infection such as amoebiasis or giardiasis. If the patient has a fever and mucus or blood in the stools, suspect amoebiasis. Giardiasis is characterised by abdominal cramps, flatulence and bubbly, foul-smelling diarrhoea persisting beyond 2 to 4 days. Take three specimens of faeces for analysis. In some cases serology may be helpful (e.g. amoebiasis). Consider subsequent development of irritable bowel syndrome.

Treatment Giardiasis: tinidazole or metronidazole Amoebiasis: metronidazole or tinidazole Patients can self-administer these drugs and carry them if visiting areas at risk, but they can have a severe disulfiram-like adverse reaction with alcohol.

Preventive advice The following advice will help prevent diseases caused by contaminated food and water. These ‘rules’ need only be followed in areas of risk such as Africa, South America, India and other parts of Asia. Only drink purified water and only eat well-cooked food. Purify all water by boiling for 10 minutes. Adding purifying tablets is not so reliable, but if the water cannot be boiled some protection is provided by adding PurTabs (chlorine) or iodine

(2% tincture of iodine), which is more effective than chlorine—use 4 drops of iodine to 1 litre of water and let it stand for 30 minutes. Avoid ice in your drinks unless known to be safe. Drink only boiled water (supplied in some hotels) or well-known bottled beverages (mineral water, 7-Up, Coca-Cola, beer). Brush your teeth using purified water. Avoid fresh salads or raw vegetables (including watercress). Salads or uncooked vegetables are often washed in contaminated water. Bananas and fruit with skins are safe once you have peeled and thrown away the skin but care should be taken with fruit that may possibly be injected with water. Be wary of dairy products such as milk, cream, ice-cream and cheese. Avoid eating raw shellfish and cold cooked meats. Avoid food, including citrus fruits, from street vendors. Drink hot liquids wherever possible. Use disposable moist towels for hand washing. Vaccines against ETEC are in development.

Golden rule for preventing diarrhoea If you can’t peel it, boil it or cook it—don’t eat it.

Malaria Page 1458

General aspects Travellers to all tropical countries are at some risk of this protozoal infection. Malaria is endemic in 102 countries;3 2.3 billion people are at risk, with 500 million affected every year. The risk is very low in the major cities of Central and Southern America and South-East Asia but can be high in some African cities. In humans, malaria is caused by four species of plasmodium: Plasmodium vivax and P. ovale—tertian malaria

P. falciparum—malignant tertian malaria P. malariae—quartan malaria P. knowlesi—presents like vivax and falciparum: rare but can be serious Malaria is either benign (vivax, ovale) or malignant (falciparum). Resistance to many drugs is increasing: The lethal P. falciparum is developing resistance to chloroquine and the antifolate antimalarials (Fansidar and Maloprim).3 Resistance is now reported to mefloquine and artemether. Resistance is common in South-East Asia, Papua New Guinea (PNG), northern South America and parts of Africa. Chloroquine is used infrequently as it is only effective in limited areas of the world. The long-awaited vaccine will make all the complex drug management much simpler. However, it still appears to be many years away despite considerable research. Patients who have had splenectomies are at grave risk from P. falciparum malaria (PFM). People die from malaria because of delayed diagnosis, delayed therapy, inappropriate therapy and parasite–host factors. It is recommended that pregnant women and young children do not travel to malarious areas (if possible). Practitioners should follow updated recommended guidelines (e.g. WHO therapeutic guidelines (antibiotic)). DxT fever + chills + headache → malaria

Malaria risk assessment The risk of catching malaria is increased by: being in a malaria area, especially during and after the wet season a prolonged stay in a malaria area, especially rural areas, small towns and city fringes sleeping in unscreened rooms without mosquito nets over the bed

wearing dark clothing with short-sleeved shirts and shorts taking inappropriate drug prophylaxis an incomplete course of prophylaxis

Malaria prevention Travellers should be advised that malaria may be prevented by following two simple rules: 1. avoid mosquito bites 2. take antimalarial medicines regularly In order to avoid mosquito bites, travellers are advised to: keep away from rural areas and avoid outdoor activities between dusk and darkness sleep in air-conditioned or properly screened rooms use insecticide sprays to kill any mosquitoes in the room or use mosquito coils at night smear an insect repellent on exposed parts of the body; an effective repellent is diethyl-mtoluamide (Muskol, Repellem, Rid) use mosquito nets (tuck under mattress; check for tears) impregnate nets with permethrin (Ambush) or deltamethrin wear sufficient light-coloured clothing, long sleeves and long trousers to protect whole body and arms and legs when in the open after sunset avoid using perfumes, cologne and after-shave lotion (also attract insects)

Important considerations in malaria prophylaxis 1. Minimise exposure to mosquitoes and avoid bites. 2. Know areas of risk: tropical South America (southern Mexico to northern half South America) tropical Africa (sub-Sahara to northern South Africa) Nile region, including remote rural Egypt Southern Asia, especially tropical areas

3. Know areas of widespread chloroquine resistance: Asia, tropical South America (rare north of Panama Canal), sub-Sahara, East Africa 4. Consider several factors: intensity of transmission season and length of stay itinerary: urban: hotel urban: non-hotel rural: housing rural: backpacking resistance patterns host factors age pregnancy associated illness compliance 5. Know the antimalarial drugs (see TABLE 129.1

).

6. Balance risk benefit of drug prophylaxis: drug side effects versus risk of PFM. 7. Visiting areas of PFM does not automatically require the use of potentially harmful drugs.4 8. Those at special risk are pregnant women, young children and the immunocompromised. Advise against travel. 9. No drugs give complete protection.

Table 129.1

Common drugs used for malarial prophylaxis2,3,5 Adult dosage

Children’s dose

Comments

Doxycycline*

100 mg each day, 1–2 days before, during, 4 weeks after

>8 years only 2 mg/kg/day up to 100 mg

Photosensitivity reactions

Mefloquine (Lariam)* In nonresistant areas

250 mg (1 tab), once weekly, same day each week, 2– 3 weeks before, during, 4 weeks after

Not recommended 45 kg as for adults

Avoid use in resistant areas, e.g. Greater Mekong Subregion Side effects: dizziness, ‘fuzzy’ head, blurred vision, neuropsychiatric Beware of beta blockers

Atovaquone + proguanil (Malarone)*

250 mg/100 mg (1 tab) per day with food 2 days before, during, 7 days later

Paediatric formulation: 62.5 mg/25 mg 11–20 kg: 1 tablet/day 21–30 kg: 2 tablets/day 31–40 kg: 3 tablets/day >40 kg: 1 adult tab/day

Avoid in pregnant women or women breastfeeding infants 10 years since last dose if >5 years for third world travel give DT 10 years of age Routine vaccination for all Australians (National Schedule) Tetanus, diphtheria, pertussis Hepatitis B Haemophilus influenzae Measles, mumps, rubella Influenza** Pneumococcal disease**

Poliomyelitis Rotavirus Varicella Selected vaccinations based on risk Cholera Hepatitis A Japanese B encephalitis Meningococcal disease* Rabies Tick-borne encephalitis Tuberculosis (BCG if Mantoux -ve) Typhoid fever Yellow fever* Other: Preventive measures against gastrointestinal infections, mosquito bites, malaria (where applicable), sexually transmitted infections *legal requirement in some countries **recommended for older travellers in particular DT = diphtheria/tetanus combined, diphtheria at a higher dose for children. dT = booster diphtheria/tetanus with a lower diphtheria tetanus for adolescents and adults.

Compulsory immunisations The two vaccinations that may be required before visiting ‘at risk’ areas are yellow fever and meningococcus.

Yellow fever Yellow fever is a serious viral infection spread by Aedes mosquitoes and, like malaria, is a tropical disease. Milder cases may present with flu-like symptoms and relative bradycardia (Faget sign) and albuminuria. Severe cases experience these symptoms with abrupt fever then prostration, jaundice and abnormal bleeding from the gums and possibly haematemesis. Diagnosis is by ELISA testing. Yellow fever vaccination, which is the only WHO-required vaccine, is essential for travel to or through equatorial Africa and northern parts of South America, and for re-entry to Australia from those countries.

DxT fever + bradycardia + jaundice → yellow fever

One injection only is required and the immunisation is valid for 10 years. Children aged less than 9 months should not be given this vaccine. It should not be given within 3 weeks of cholera vaccine. Note: It is important to check specific country requirements in the WHO book on vaccination requirements.8 According to the WHO, a certificate against yellow fever is the only certificate that should be required for international travel. The requirements of some countries are in excess of international health regulations. However, vaccination against yellow fever is strongly recommended to all travellers who intend to visit places other than the major cities in the countries where the disease occurs in humans.

Meningococcal infection Meningitis due to this organism is a contagious lethal disease. It is common in Nepal, Mongolia, Vietnam and parts of Africa and Asia, especially in the dry season. Travellers trekking through the Kathmandu valley of Nepal and those attending the Haj pilgrimage to Saudi Arabia are at special risk and should have the vaccine. However, some countries require immunisation for entry.

Voluntary immunisation Precautions against the following diseases are recommended for those travellers who may be at special risk.2

Hepatitis A, B Hepatitis A is a common problem in rural areas of developing countries. There is a declining level of antibodies to hepatitis A in developed countries and adults are at special risk so one or two doses of hepatitis A vaccine should be given. A blood test for hepatitis A antibodies can be carried out to determine a person’s immunity. Page 1461

Prevention The rules of avoiding contaminated food and water apply (as for traveller’s diarrhoea). Hepatitis A vaccine is given as a course of two injections. Hepatitis B is endemic in South-East Asia, South America and other developing countries. Vaccination is recommended, especially for people working in such countries, particularly those

in the health care area or those who may expect to have sexual or drug contact. If patients have a ‘negative’ HBV core IgG titre, then vaccination would be worthwhile (three doses: 0, 1 and 6 months). Hepatitis E has a high mortality rate in pregnant women. The usual approach for non-immunised people is to give the combined hepatitis A and B vaccine (Twinrix) as a course of three injections.

Typhoid Typhoid immunisation is not required for entry into any country but is recommended for travel to third world countries where the standards of sanitation are low. It should be considered for travellers to smaller cities, and village and rural areas in Africa, Asia, Central and South America and Southern Europe. The parenteral (subcutaneous) vaccine can be used but the single dose typhim Vi vaccine or the oral vaccine, which have fewer side effects, are generally preferred. The oral vaccine, which is given as a series of three or four capsules, appears to afford protection for about 5 years but is contraindicated in the immunocompromised.

Cholera Cholera vaccination is not officially recommended by the WHO because it has only limited effectiveness. It is advisable for health care workers or others at risk entering an endemic area. Cholera is given as an oral vaccine (Dukoral) over 1 week prior to exposure. It is not recommended in children under 5 years or pregnant women.

Japanese B encephalitis This mosquito-borne flavivirus infection presents a real dilemma to the traveller and doctor because it is a very severe infection (mortality rate 20–40%) with high infectivity and high prevalence in endemic countries. The disease is prevalent during the wet season in the region bounded in the west by Nepal and Siberian Russia and in the east by Japan and Singapore, especially in Nepal, Myanmar, Korea, Vietnam, Thailand, China, eastern Russia and the lowlands of India. Rice paddies and pig farms are areas of risk. The usual preventive measures against mosquito bites are important. DxT febrile illness + vomiting + stupor → Japanese B encephalitis

Rabies Rabies vaccination for the rhabdovirus is recommended for some international aid workers or

travellers going to rabies-endemic areas for periods of more than 1 month or even for short periods of working with affected animals in those areas. The vaccination can be effective after the bite of a rabid animal, so routine vaccination is not recommended for the traveller. Affected animals include dogs, cats, monkeys, camels and feral (wild) animals. A traveller who sustains a bite or scratch or even is licked by an animal in countries at risk should wash the site immediately with soap or a detergent, and then seek medical help. The prebite vaccination does not remove the need for postexposure vaccination. DxT painful bite + paraesthesia + hydrophobia (pain with drinking) → rabies

Plague Plague (Black death) caused by the Gram-negative bacterium Yersinia Pestis is still prevalent in rodents in countries such as Vietnam, Brazil, Peru, Ecuador and Kenya. The three most endemic countries are Democratic Republic of Congo, Madagascar and Peru. Rats and fleas have been implicated in transmission, but spread from person to person via lice or coughing is now considered more likely. Although not compulsory, vaccination is recommended for those engaged in field operations in plague areas and rural health workers who may be exposed to infected patients. Two doses are given to adults (three to children 3 weeks) diarrhoea Usually due to Giardia lamblia, Entamoeba histolytica, Campylobacter jejuni (especially SouthEast Asia), Salmonella, Yersinia enterocolitica or Cryptosporidium.12 Stool examination (three fresh specimens): microscopy wet preparation culture Faecal Multiplex PCR (if available)

Treat pathogen (see guidelines under diarrhoea in CHAPTER 34

)

Non-pathogens such as Escherichia coli and Endolimax nana are often reported but do not treat specifically. Note: Consider exotic causes such as schistosomiasis, strongyloidiasis and ciguatera in unusual chronic post-travel ‘gastroenteritis’.

Persistent abdominal discomfort This common syndrome includes bloating, intestinal hurry and borborygmi, and often follows an episode of diarrhoea. Usually no pathogens are found on stool examination. However, giardiasis can be difficult to detect and an empirical course of tinidazole (2 g statim) is worthwhile. Any persistent problem then is a type of postinfective bowel dysfunction or irritable bowel. Reassurance is important.

Rash/other skin lesions Maculopapular: consider dengue, HIV, typhus, syphilis, arbovirus infections, leptospirosis, Q fever Petechiae: viral haemorrhagic fevers, leptospirosis, dengue Rose spots: typhoid Eschar: typhus (louse-borne, tick and scrub), anthrax Chancre: African trypanosomiasis, syphilis Page 1466

Fever Causes range from mild viral infections to potentially fatal cerebral malaria (see TABLE 129.4 ) and meningococcal septicaemia. An Australian study of fever in returned travellers13 revealed the most common diagnosis was malaria (27%) followed by respiratory tract infection (24%), gastroenteritis (14%), dengue fever (8%) and bacterial pneumonia (6%). The commonness of malaria was supported by results from the GeoSentinel Surveillance Network.14 The common serious causes are malaria, typhoid, hepatitis (especially A and B), dengue fever, amoebiasis and tuberculosis. Most deaths from malaria have occurred after at least 3 or 4 days of symptoms that may be mild. Death can occur within 24 hours. Factors responsible for death from malaria include delayed presentation, missed or delayed diagnosis (most cases), no chemoprophylaxis and old age.

Refer immediately to a specialist unit if the patient is unwell. Be vigilant for meningitis and encephalitis. Be vigilant for amoebiasis—can present with a toxic megacolon, especially if antimotility drugs are given. If well but febrile, first-line screening tests: full blood examination and ESR thick and thin films liver function tests urine for micro and culture Refer immediately if malaria is proven or if fever persists after a further 24 hours. Table 129.4

Fever and malaise in the returned traveller: diagnostic strategy model

Note: All fever in a returned traveller is malaria until proved otherwise! Probability diagnosis Viral respiratory illness (e.g. influenza, including coronavirus) Malaria Hepatitis (may be subclinical) Gastroenteritis/diarrhoeal illness Dengue Serious disorders not to be missed Malaria Tuberculosis Typhoid/paratyphoid Encephalitis Viral haemorrhagic diseases Meningococcal meningitis Melioidosis Amoebiasis (liver abscess) HIV seroconversion illness Pitfalls (often missed) Ascending cholangitis

Infective endocarditis Cytomegalovirus Epstein–Barr virus Dengue fever Lyme disease Bronchopneumonia Ross River fever Leptospirosis Rarities: Chikungunya Legionnaire disease Schistosomiasis African trypanosomiasis Typhus Yellow fever Zika virus Rift Valley fever Spotted fever Lassa fever, Hantavirus, Marburg, Ebola and other haemorrhagic fevers Note: Three causes of a dry cough (in absence of chest signs) are malaria, typhoid and amoebic liver abscess. Seven masquerades checklist Drugs (reaction to antimalarials) Urinary infection Investigations (if no obvious cause) Full blood examination Chest X-ray TB screening: TST (Mantoux); IGRA Blood culture Liver function tests Urine—micro and culture Stool—micro and culture ESR, CRP Malaria screening: rapid diagnostic test, thick and thin films, spit test Acute phase serology (hold pending convalescent serology)

Page 1467

Malaria See FIGURE 129.2

.

FIGURE 129.2 Clinical features of malaria

Incubation period: P. falciparum 7–14 days; others 12–40 days Most present within 2 months of return Can present up to 2 or more years Can masquerade as several other illnesses

Clinical features High fever, chills, rigor, sweating, headache Usually abrupt onset Can have atypical presentations (e.g. diarrhoea, abdominal pain, cough)

Other features Beware of modified infection. Must treat immediately. Delay may mean death. Typical relapsing patterns often absent. Thick smear allows detection of parasites (some laboratories are poorly skilled with thick films). Thin smear helps diagnose malaria type. If index of suspicion is high, repeat the smear (‘No evidence of malaria’ = 3 negative daily thick films). Newer tests (e.g. the malaria rapid diagnostic test, polymerase chain reaction [PCR] tests and immune chromatographic test [ICT] card tests for PFM) show promise. Cerebral malaria and blackwater fever are severe and dramatic. The Para check V test (a desktop test) is accurate and needs to be positive before prescribing artemether in some areas. The new malaria spit (saliva) test detects a protein biomarker. Page 1468

Treatment2,5 Admit to hospital with infectious disease expertise. Rule out G6PD deficiency Supportive measures, including fluid replacement Avoid the same drugs used for prophylaxis Uncomplicated malaria2 artemether + lumefantrine 20 mg + 120 mg (Riamet)

4 tablets with food at 0, 8, 24, 36, 48, 60 hours (i.e. 24 tablets) in 60 hours or atovaquone + progunail or (combination treatment) quinine sulphate 600 mg (o) 8 hourly, 7 days + doxycycline 100 mg (o) 12 hourly, 7 days or clindamycin 300 mg (o) tds, 7 days (children, pregnancy) complicated (severe): artesunate 2.4 mg/kg IV statim, 12 hours, 24 hours, then once daily until oral therapy (Riamet) is possible or quinine dihydrochloride 20 mg/kg up to 1.4 g IV (over 4 hours) then after 4-hour gap 7 mg/kg IV 8 hourly until improved (ECG/cardiac monitoring) then quinine (o) 7 days Note: Check for hypoglycaemia. Beware if antimalarial use in previous 48 hours.

Typhoid fever Incubation period 10–14 days

Clinical features Insidious onset Headache prominent Dry cough Fever gradually increases in ‘stepladder’ manner over 4 days or so Abdominal pain and constipation (early) Diarrhoea (pea soup) and rash—rose spots (late)

± Splenomegaly DxT ‘stepladder’ fever + abdominal pain + relative bradycardia → typhoid (early)

Diagnosis On suspicion—blood and stool culture Serology not very helpful

Treatment Azithromycin l g (o) for 7 days or Ciprofloxacin 500 mg (o) bd for 7–10 days

Cholera Incubation period a few hours–5 days

Clinical features Variable Subclinical Mild, uncomplicated episode of diarrhoea Fulminant lethal form with severe water and electrolyte depletion, intense thirst, oliguria, weakness, sunken eyes and eventually collapse DxT fever + vomiting + abrupt onset ‘rice water’ diarrhoea → cholera

Diagnosis Stool microscopy and culture (Vibrio cholerae)

Treatment

In hospital with strict barrier nursing IV fluid and electrolytes Azithromycin or ciprofloxacin

Viral haemorrhagic fevers These include: yellow fever, Lassa fever, etc., plus dengue fever and chikungunya.

Lassa fever, Ebola virus, Marburg virus, Hanta virus These rare but deadly tropical diseases usually commence with a flu-like illness, gastrointestinal symptoms with thrombocytopenia, anaemia and, if severe, findings consistent with disseminated intravascular coagulation leading to bleeding and possibly shock and frank haemorrhage. Hanta virus tends to cause respiratory symptoms, including cough, progressing to respiratory difficulty. Seek urgent expert help.

Ebola Incubation period: 2–16 days Transmission: direct body contact (body fluids, esp. blood, vomit from affected or dead person), infected animals, contaminated objects (needles, medical equipment) Early symptoms: constitutional (fever, malaise, headache), upper respiratory (flu-like, cough, etc.), abdominal (pain, nausea, vomiting, diarrhoea) May progress to severe symptoms as above then multiorgan failure Diagnosis: PCR, histopathology Treatment is supportive, esp. IV fluids

Dengue fever2,4 Also known as ‘breakbone’ fever, it is widespread in the south-east Pacific and endemic in Queensland. A returned traveller with myalgia and fever