Pediatric Orthopedics for Primary Healthcare: Evidence-Based Practice [1 ed.] 3030652130, 9783030652135

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Pediatric Orthopedics for Primary Healthcare Evidence-Based Practice Sattar Alshryda Lisa Jackson Nandu Thalange Ali AlHammadi Editors

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Pediatric Orthopedics for Primary Healthcare

Sattar Alshryda  •  Lisa Jackson Nandu Thalange • Ali AlHammadi Editors

Pediatric Orthopedics for Primary Healthcare Evidence-Based Practice

Editors Sattar Alshryda Head of Trauma & Orthopaedics Surgery Al Jalila Children’s Speciality Hospital Dubai United Arab Emirates Nandu Thalange Consultant Paediatric Endocrinologist Department of Medical Subspecialties Al Jalila Children’s Hospital Dubai United Arab Emirates

Lisa Jackson Associate Professor of Family Medicine College of Medicine Mohammed Bin Rashid University of Medicine and Health Sciences Dubai United Arab Emirates Ali AlHammadi Director of Clinical Planning and Strategic Partnerships Al Jalila Children’s Speciality Hospital Dubai United Arab Emirates

ISBN 978-3-030-65213-5    ISBN 978-3-030-65214-2 (eBook) https://doi.org/10.1007/978-3-030-65214-2 © The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2021 This work is subject to copyright. All rights are solely and exclusively licensed by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

To children for all the joy and hope that they bring to our life. Sattar Alshryda To my children Harold and Lucy, the MBRU family and my wonderful family medicine colleagues in the UK and United Arab Emirates. Lisa Jackson I would like to thank my dear colleagues past and present, and the children and families who are my teachers and remind me every day why I am a doctor. Nandu Thalange To my parents for their continuous love and support. Ali AlHammadi

Foreword

I am honoured to be asked to write a foreword for this book. As a paediatric orthopaedic surgeon married to a family doctor, we saw the need for such a book many years ago—but with four children of our own, we never seemed to find time ourselves to write one! In many medical schools there is less time devoted to orthopaedics as a whole than there used to be. Very little of that ‘orthopaedic time’ is spent looking at the problems of children or learning to understand what is normal in the musculoskeletal development of children. Considering that musculoskeletal conditions account for a significant proportion of family doctor consultations, there is clearly a mismatch between training and the real-life practice it is supposed to be a preparation for. This book will increase mutual understanding and knowledge and improve communication between family doctors and paediatric orthopaedic surgeons. As a consequence, it will benefit children and for that the authors are to be congratulated. Each speciality can learn useful skills from the other. There is an old joke that says that when orthopaedic surgeons talk about a holistic approach, they are thinking about the whole bone. The most memorable humour contains a grain of truth, and this highlights our tendency to focus down on the tree rather than standing back to see the whole wood. Within this book, paediatric orthopaedic surgeons can learn how to see orthopaedic conditions holistically within the context of the other issues in the child’s life. The family doctor may also gain much from the text. Within a family doctor’s practice, children’s orthopaedic conditions are only a small part. Some important conditions with life-altering consequences (such as slipped upper femoral epiphysis) may only present once, insidiously, in a family doctor’s career. It is vital that when it does, the diagnosis is considered, and referral initiated. This is a huge diagnostic challenge, but not an impossible one, if we learn the lessons in this book.

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Foreword

The book also goes some way to demystify the terminology of orthopaedics— the language that is as foreign to medical colleagues as a cardiology report is to us! I am sure this book will be successful, and deservedly so. It just remains for me to extend my thanks to all the contributors, on behalf of the readership. 

Richard Montgomery, FRCS (Ed), FRCS (Eng) British Limb Reconstruction Society London, UK British Society of Children Orthopaedic Surgeons London, UK British Society for Surgery in Cerebral Palsy Lincoln, UK Council of British Orthopaedic Association London, UK Royal College of Edinburgh Edinburgh, Scotland Intercollegiate Specialty Board in T&O Surgery (FRCS T&O Exam Board) London, UK

Foreword

Where is the Wisdom we have lost in Knowledge? Where is the Knowledge we have lost in Information? Where is the Information we have lost in the Internet. (Apologies to T.S. Eliot!)

I felt honoured and privileged when Lisa and Nandu Thalange approached me to write a foreword for this textbook, given our shared passion for primary care (family medicine), neuromusculoskeletal problems in children and medical education! I am deeply grateful to the editorial team who live by the philosophy of lifelong learning. I would hope that my experience of learning, sharing and working in and across continents for nearly half a century, as a specialist in orthopaedics and as a generalist family physician, would be relevant in writing this foreword. Paediatric orthopaedics as a subspeciality has grown and developed immensely over the last two decades. The term orthopedia was coined by Nicolas Andry (a French Professor of Medicine in Paris) in 1741, from two Greek words orthos (straight) and pais (Child) in his treatise On the Art of Correcting and Preventing Deformities in Children. About 25% of all consultations in primary care are for musculoskeletal problems. Paediatric orthopaedics demands a deeper knowledge of the normal and abnormal growth and development and understanding of the musculoskeletal system, beyond clinical anatomy. This book is the distilled product of the experience of both primary care physicians and secondary care specialists. Experience goes beyond exposure, encompassing knowledge, understanding, application of knowledge in different settings, analysing outcomes and reflecting on them and synthesising internally. Every contributor has been carefully chosen by the editorial team. Every chapter has been written by the collaboration, communication and commitment of community-based and hospital-based specialists, from different continents, covering the journey of the patient from his home to the hospital settings. This nosegay of experience from multinational authors has been thoughtfully tied together by the four editors ensuring relevance to primary care physicians. The book is divided into a general section of 13 chapters and a regional section of 7 chapters, addressing the common problems by anatomical region. Real-world case histories add relevance to the primary care practice. For example in Chapter one, Case study 1.1 is an excellent example of real-world problems in the context of primary care. The author then explores the semantics of terminology that underpins the clinical problem. The relevant ix

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illustrations, photographs and tables enhance understanding. Another useful theme is the Abstract and Key words, which focuses on the salient contents of what follows. It enables the reader to pause and review what is already known about the problem, before perusing the rest of the chapter and finally asking the question ‘What next?’ This helps the reader to progress further by accessing the web-based resources in a more focused way. The idea for the book was born prior to the global Covid-19 pandemic. The development and completion of the book, despite the global pandemic, by 38 contributors and four editors is a formidable achievement. The comprehensiveness makes this book go beyond ‘must-read’ to ‘must-have’ in primary and secondary care clinics globally. I am confident that this textbook will not only be valuable to primary care physicians, but to all clinicians dealing with orthopaedic problems in children, in any setting. 

Sidha Sambandan, FRCS, FRCGP, PGDipMSMed Norwich, Norfolk, United Kingdom

Foreword

The speciality of general practice/family medicine is a challenging one intellectually because it uses knowledge from all the medical specialities to care for patients across the lifecycle and with any condition. Recognition of the normal, helping people to tolerate uncertainty as problems are diagnosed and treated and also understanding the aftercare of hospital interventions are all part of the role of primary care physicians and their teams. In many countries, the first point of care for children as well as adults is in family medicine, which again means that family doctors will need to understand the normal development of the human skeleton and related problems. This book covers the whole range of issues from normal development to serious pathology such as tumours, and each chapter’s co-authorship by a generalist and an orthopaedic specialist ensures that its contents are relevant to the role of primary health care. It provides in-depth knowledge to support those undertaking speciality training for family medicine, but also provides updates and revision material useful to practising family doctors. Some of the topics covered may be quite rare in clinical practice, but all are potential presentations that a GP will see from time to time: so the text is also useful as a reference source when patients with paediatric orthopaedic problems attend. I congratulate the authors for having the vision to write such a text; to the readers for being professionally committed to this important area of clinical practice; and to all those who practise family medicine, as doing the right thing for your child patients may make a difference to the rest of their lives. Thank you for this book. Amanda Howe, OBE, FRCGP, FMedSci Norwich, Norfolk, United Kingdom

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Preface

Children’s orthopaedics is challenging even for experts in the field. There is a wide spectrum of normal and abnormal musculoskeletal conditions in children, and the first port of call for most families is their general practitioner. At one end of the spectrum, are normal variants that require simple reassurance, and at the other, very serious and even life-threatening conditions such as bone cancer. There is a real shortage of information, specifically geared for the needs of primary care. We attempt to address this deficiency in a comprehensive, evidence-­ based and readily comprehensible textbook that gives GPs and others working on the primary care front line the information required to navigate the complex field of children’s orthopaedic problems. More than that, we seek to imbue a sense of confidence and partnership, recognising the critical role of the GP in the management of musculoskeletal conditions. Doctors’ training has changed dramatically over the last decade. With the introduction of shorter and more specialised competency-based training, few doctors in primary care will acquire paediatric orthopaedic training. We hope this textbook will provide a definitive ‘state-of-the-art’ guide to fill this gap. The book’s contents are divided into general and regional orthopaedics. The depth of knowledge is appropriate to the general practitioner level with additional specialist information which may aid explanation and planning discussions between the primary caregiver, children and carers. Each chapter is written jointly by an orthopaedic surgeon and a primary care physician, copiously illustrated with clinical photographs, charts and radiographs with concise, evidence-based guidance and information. We hope you enjoy the fruits of our labours!  Sattar Alshryda, MBChB, MRCS, FRCS(T&O), MSc, PhD  Lisa Jackson, FRCGP, FHEA, PGCertMedEd  Nandu Thalange, FRCP, FRCPCH, FHEA Dubai, United Arab Emirates  Ali Alhammadi, MBBS

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Contents

Part I General Orthopaedic Terminology ��������������������������������������������������������������������������������   3 Liam Walker and Ling Hong Lee Normal Development ����������������������������������������������������������������������������������������  23 Wafa Binfadil, Tahani Al Ali, and Samantha Ismaile General Assessment ������������������������������������������������������������������������������������������  39 Sonia Chaudhry and Zarana R. Swarup Normal Variants ������������������������������������������������������������������������������������������������  63 Tahani Al Ali, Jihad Saeed, and Sattar Alshryda Musculoskeletal Infection����������������������������������������������������������������������������������  87 Stephanie N. Moore-Lotridge, Michael A. Benvenuti, Isaac P. Thomsen, and Jonathan G. Schoenecker Musculoskeletal Tumors������������������������������������������������������������������������������������ 113 Mohamed Ahmed Mashhour Metabolic Bone Disease ������������������������������������������������������������������������������������ 145 Ahmed Nugud, Alaa Nugud, Sattar Alshryda, and Nandu Thalange Neuromuscular Conditions ������������������������������������������������������������������������������ 171 Samena Chaudhry, Heather Read, and Sattar Alshryda Relevant Syndromes������������������������������������������������������������������������������������������ 207 Sarah Rubin and Jan Sochon-Smith Musculoskeletal Dysplasias ������������������������������������������������������������������������������ 231 Sania Shahid and Deborah M. Eastwood Limb Deformity�������������������������������������������������������������������������������������������������� 263 Mohamed Kenawey, Zullie Ali, and Farhan Ali Child Safeguarding�������������������������������������������������������������������������������������������� 283 Themistoklis Tzatzairis, Maria Nivatsi, and Claudia Maizen

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 Chronic Pain in Children���������������������������������������������������������������������������������� 293 Thanthullu Vasu and Alwyn Abraham Part II Region Specific Topics Paediatric Hips �������������������������������������������������������������������������������������������������� 309 Lydia R. Belcher, Michael A. Benvenuti, Perry L. Schoenecker, and Jonathan G. Schoenecker Paediatric Knee Disorders�������������������������������������������������������������������������������� 349 Sumukh Khandekar and Stan Jones Paediatric Foot and Ankle�������������������������������������������������������������������������������� 377 Om Lahoti and Nisha Patel Paediatric Spine ������������������������������������������������������������������������������������������������ 403 Hayder Saleh Al-Saadi and Firas Dakhil-Jerew Paediatric Shoulder Disorders�������������������������������������������������������������������������� 429 David Hawkes, H. S. Lloyd, and Matthew Nixon Paediatric Elbow������������������������������������������������������������������������������������������������ 451 Robert Wilson and Neil Wilson Paediatric Hand and Wrist ������������������������������������������������������������������������������ 473 Anastasios Chytas and Gillian Smith

Contributors

Alwyn Abraham  University Hospitals of Leicester NHS Trust, Leicester, UK Tahani Al Ali  Al Jalila Children’s Speciality Hospital, Dubai, United Arab Emirates Farhan  Ali  Royal Manchester Children’s Hospital, Manchester University NHS Foundation Trust, Manchester, UK Zullie  Ali  Royal Manchester Children’s Hospital, Manchester University NHS Foundation Trust, Manchester, UK Hayder Saleh Al-Saadi  Spinal Surgeon, Rashid Hospital, Dubai, United Arab Emirates Sattar Alshryda, MBChB, MRCS, FRCS(T&O), MSc, PhD  Head of Trauma & Orthopaedics Surgery, Al Jalila Children’s Speciality Hospital, Dubai, United Arab Emirates Anastasios Chytas  Royal Manchester Children Hospital, Manchester, UK Lydia R. Belcher  Paediatric Orthopaedics Vanderbilt University, Monroe Carrell Jr Children’s Hospital, Nashville, TN, USA Michael A. Benvenuti  Vanderbilt University Medical Center, Nashville, TN, USA Monroe-Carrel Children’s Hospital, Pediatric Orthopaedics and Infectious Disease, Nashville, TN, USA Wafa Binfadil  Al Jalila Children’s Speciality Hospital, Dubai, United Arab Emirates Samena Chaudhry  Royal Stoke University Hospital, Stoke-on-­Trent, UK Sonia Chaudhry  University of Connecticut School of Medicine, Farmington, USA Pediatric Orthopaedic and Hand Surgeon, Connecticut Children’s Medical Center, Hartford, USA Firas Dakhil-Jerew  Australian GP Group, Sydney, Australia Matthew Debenham  Middlemore Hospital, Auckland, New Zealand Deborah  M.  Eastwood  Great Ormond St Hospital and the Royal National Orthopaedic Hospital, Stanmore, UK University College London, London, UK xvii

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Contributors

David Hawkes  Trauma and Orthopaedics Registrar, Countess of Chester Hospital, Chester, UK Orthopaedic Department, Countess of Chester Health Park, Chester, UK Samantha Ismaile  The Higher Colleges of Technology, Dubai, United Arab Emirates Stan Jones  Al Ahli Hospital, Doha, Qatar Sumukh Khandekar  Al Ahli Hospital, Doha, Qatar Mohamed  Kenawey  Royal Manchester Children’s Hospital, Manchester University NHS Foundation Trust, Manchester, UK Om Lahoti  King’s College Hospital, London, UK Ling  Hong  Lee  Consultant Trauma and Orthopaedics/Paediatric Orthopaedics, Sunderland Royal Hospital, Sunderland, UK H. S. Lloyd  Mosgiel Health Centre, Mosgiel, New Zealand Best Practice Advocacy Centre (BPAC), Dunedin, New Zealand Department of General Practice and Rural Health, University of Otago, Dunedin, New Zealand Mohamed  Ahmed  Mashhour  Department of Orthopedic Surgery, Faculty of Medicine, Ain Sham University, Orthopedic Oncology Unit, Cairo, Egypt Orthopedic Surgery, Mohammed Bin Rashid University of Medicine and Health Sciences (MBRU), Dubai, United Arab Emirates Claudia Maizen  Royal London Hospital, Paediatric Orthopaedics, London, UK Stephanie  N.  Moore-Lotridge  Vanderbilt University Medical Center, Nashville, TN, USA Monroe-Carrel Children’s Hospital, Pediatric Orthopaedics and Infectious Disease, Nashville, TN, USA Maria  Nivatsi  Pediatric Department, University Hospital of Alexandroupolis, Alexandroupolis, Greece Matthew  Nixon  Trauma and Orthopaedic Consultant, Countess of Chester Hospital, Chester, UK Ahmed Nugud  Al Jalila Children’s Speciality Hospital, Dubai, United Arab Emirates Alaa  Nugud  Latifa Women’s and Children’s Hospital, Dubai Health Authority, Dubai, United Arab Emirates Nisha Patel  Urgent Care Center – Emergency Department, Kings College Hospital, London, UK Heather Read  Royal Hospital for Children, Glasgow, UK

Contributors

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Sarah Rubin  Village Community Medical Centre, Derby, UK Jihad Saeed  Al Jalila Children’s Speciality Hospital, Dubai, United Arab Emirates Perry L. Schoenecker  Paediatric Orthopaedics Washington University, St. Louis Children’s Hospital, St. Louis, MO, USA Jonathan  G.  Schoenecker  Vanderbilt University Medical Center, Nashville, TN, USA Monroe-Carrel Children’s Hospital, Pediatric Orthopaedics and Infectious Disease, Nashville, TN, USA Zille S. Shah  Al Kowthar Practice, Oldham Primary Care, Manchester, UK Sania Shahid  Latifa Women and Children Hospital, Dubai, United Arab Emirates Gillian Smith  Great Ormond Street Children Hospital, London, UK Jan Sochon-Smith  Village Community Medical Centre, Derby, UK Zarana  R.  Swarup  Pediatrician, Children’s Hospital of Philadelphia Primary Care, Philadelphia, PA, USA Nandu  Thalange, FRCP, FRCPCH, FHEA  Consultant Paediatric Endocrinologist, Department of Medical Subspecialties, Al Jalila Children’s Hospital, Dubai, United Arab Emirates Isaac P. Thomsen   Vanderbilt University Medical Center, Nashville, TN, USA Monroe-Carrel Children’s Hospital, Pediatric Orthopaedics and Infectious Disease, Nashville, TN, USA Themistoklis  Tzatzairis  Royal London Hospital, Paediatric Orthopaedics, London, UK Thanthullu Vasu  University Hospitals of Leicester NHS Trust, Leicester, UK Liam Walker  GP Registrar, Health Education England North East, Newcastle, UK Neil Wilson  Consultant, Royal Hospital for Children, Glasgow, UK Robert Wilson  GP, Kenmure Medical Practice, Glasgow, UK

Part I General

Orthopaedic Terminology Liam Walker and Ling Hong Lee

1

Introduction

We begin with a clinical vignette which includes several ‘orthopaedic’ terms commonly used in a paediatric injury. Common terms are underlined and further explained below. Case Study 1 Amy

A 12-year-old Amy attends the Emergency Department with an injury to her left forearm. She fell from a climbing frame at school an hour earlier. Nurse Matthew notes that Amy is in pain and her left forearm is deformed. He quickly establishes the history and applies a backslab to her arm once confirming absence of other injury and no wound in the forearm. An x-ray shows displaced transverse fracture of the midshaft of ulna and radius. The Orthopaedic team decides to take Amy to theatre to treat the fracture. They are satisfied with a closed reduction and immobilise Amy’s arm in an above elbow cast. By the next day, Amy remains comfortable and therefore is discharged home. At one-week follow-up, x-ray shows the fracture reduction has unfortunately displaced significantly. Amy again is taken to theatre. An attempt at closed reduction is not successful because of interposition of soft tissue at the fracture site. Therefore, an open reduction is performed. Flexible nails are used to stabilise the fracture”.

L. Walker GP Registrar, Health Education England North East, Newcastle, UK L. H. Lee (*) Consultant Trauma and Orthopaedics/Paediatric Orthopaedics, Sunderland Royal Hospital, Sunderland, UK e-mail: [email protected] © The Author(s), under exclusive license to Springer Nature Switzerland AG 2021 S. Alshryda et al. (eds.), Pediatric Orthopedics for Primary Healthcare, https://doi.org/10.1007/978-3-030-65214-2_1

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1.1

L. Walker and L. H. Lee

Backslab

It is an incomplete, usually half or 3-quarter circumference of the body part, splint using Plaster of Paris longitudinal slabs. It is applied over a layer of rolled wool and secured with another layer of dressing such as crepe bandage. It is often named according to the body part that it is protecting such as a below elbow backslab, an above elbow backslab (Fig. 1), a below knee backslab or an above knee backslab. Volarslab is the name given to the slab that is applied on the front rather than the back of a body part.

Fig. 1  Common casts in clinical pratice. Image 1: below elbow backslab; image 2: below elbow full cast; image 3: above elbow backslab; image 4 above elbow full cast; image 5: below knee backslab; image 6: below knee full cast; image 7: Sarmiento or patella tendon bearing cast (or PTB); image 8: above knee full cast

Orthopaedic Terminology

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Backslab and Volarslab are often used initially to splint fractures and injuries to provide comfort and prevent further displacement of fractures while accommodating any further swelling secondary to injuries. Backslab on the leg does not provide enough rigidity to allow even protected weight bearing. Eg, below knee backslab vs below knee cast.

1.2

Cast

This is a complete circumferential splint using Plaster of Paris or synthetic material such as fibreglass or polyester variant. The cast is stronger than a backslab and provides more protection for fractures. If fractures are stable or have united, the orthopaedist may allow the patient to walk with the cast. This may be referred to as a walking cast or weight bearing cast. These casts are often strengthened further to allow weight bearing (Fig. 2).

1.3

Fractures

A fracture is defined as a break in continuity of bone, either complete or incomplete. Incomplete fracture is common in paediatrics. ‘Greenstick’ - Imagine trying to snap a green fresh twig with your thumbs—half of the far side will break but the near side is bent but not broken. The bone cortex at the tension (far) side fractures, cortex at the compression (near) side remains intact but deformed (Fig. 3). Fig. 2  This child has bilateral below knee full cast. Feel the splint—if it feels hard all around then it is a cast. If there is an ‘empty gutter’ feeling along the front or back of the limb, it is a backslab

Fig. 3  Radiograph showing a greenstick fracture. R Radius. Arrow showing greenstick fracture. U Ulna bone is bent due to plastic deformation in this 7-year-old boy. Normal ulna bone is straight

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Fig. 4  Buckle fracture of the distal radius. It is also called torus fracture due its similarities to the torus of the Roman columns (bottom right image). There can be very little pain, swelling and deformity in an incomplete fracture. Trace the outline of the bone; look for discontinuity of the cortex or irregularity

‘Buckle’—AKA ‘torus’ fracture. Gentle attempt to bend a green fresh twig without snapping it; a small bending force will cause the compressed side to buckle. The bony cortex is compressed and bulges (buckle) (Fig. 4). ‘Plastic deformation’—Imagine bending a long liquorice candy stick without it snapping. Instead of fracturing, long bone deforms due to the force of injury. There is no cortical fracture or buckling (Fig. 3). Closed fracture is a fracture where there is no communication between the atmosphere and fracture. In contrast to an open fracture where the bone communicates with the atmosphere. Skin abrasion overlying a fracture needs careful inspection to exclude a minute puncture caused by a bone spike. Spectrum ranges from exposed bones or joints which are clearly visible to the naked eye, or puncturing of the skin at time of injury and reducing back spontaneously under the skin. In such a case, a minute puncture continues to ooze venous blood (Fig. 5).

Orthopaedic Terminology

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1

2

3

Fig. 5  Open fractures. Image 1 shows a puncture wound of forearm fracture whereas images 2&3 showed larger wound

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L. Walker and L. H. Lee Salter - Harris classification of physeal fractures Normal

Type I

Type II

Type III

S

A

L

Straight across

Above

Lower or Below

Type IV

T Two or Through

Type V

E

R

ERasure of grouth plate or CRush

Fig. 6  Salter-Harris classification

Gustilo classified open fractures into 3 types [1]: • Type I: wound ≤1 cm, minimal contamination or muscle damage • Type II: wound 1-10 cm, moderate soft tissue injury • Type III is subdivided into A, B and C: –– Type IIIA: large wound with extensive soft-tissue damage but there is adequate tissue for primary coverage. –– Type IIIB: wound requires soft tissue coverage (rotational or free flap) –– Type IIIC: There is a vascular injury requiring vascular repair, regardless of degree of soft tissue injury Fractures can also be described according to the site of fracture, into midshaft facture (in the middle of the diaphysis), metaphyseal fractures, physeal fractures (involving the physis or the growth plate) and intra-articular fracture. Eighteen percent of all fractures in children are physeal fractures and these have extra importance as they can interfere with bone growth leading to shortening or deformity [2]. Various classification systems based on radiographs are used to describe physeal fracture such as Salter-Harris [3], Ogden [4] and Peterson [5]. The Salter-Harris classification (Fig.  6) based on 5 fracture patterns is the one most widely used. This classification not only helps guide the choice of treatment but also predicts the prognosis. Fractures are also described according to the fracture configuration into transverse, oblique, spiral, multi-fragmentary and segmental fractures (Fig. 7). Fracture pattern suggests the causal mechanism and severity of energy involved. For example, multi-fragmentary (also called Comminuted) fracture is a type of fracture when a bone breaks into more than two fragments. This takes a significant degree of force and is therefore usually found in association with high-impact trauma (Fig. 7). Undisplaced fractures are fractures with bony fragments remaining aligned in their normal positions. Displaced fractures are the ones where fragments have moved apart, by force of the injury, gravity or pull of muscles attached to the fragments. Displacement on imaging (e.g. X-ray) can be described using terms such as translation, angulation, rotation and altered length.

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Fig. 7  Fracture pattern, from left transverse, oblique, spiral, multi-fragmentary, segmental

Fracture stability refers to the resistance of a particular fracture to displacement. A fracture needs stability especially during the initial healing process (haematoma, soft callus) to avoid unsatisfactory healed position (malunion) and non-union. Fracture stability can be judged by several features such as fracture configuration, size of fragments, mechanism of injury and muscle attachments. Unstable fractures, especially those involving articular surface, usually require surgical fixation and protected weight bearing postoperatively. Undisplaced and minimally displaced fractures are often treated with a backslab or a cast without the need for reduction whereas substantially displaced fractures require correction. The fracture is manipulated manually aiming to return the fragments to their normal position maintaining normal alignment of the limb. This correction is commonly called reduction. There are two types of reduction: closed reduction is when reduction is performed without incision to the skin overlying the fracture. Reduction is achieved ‘indirectly’ by manual external manipulation. Open reduction is when the position of fracture is not correctable by closed reduction, an incision is made over the fracture to ‘directly’ visualise the fracture through the wound. Reduction can then be achieved by internal manipulation directly on the fragments. Most fracture reduction in children is performed under general anaesthesia unless the child is mature and compliant for the procedure to be performed under local anaesthesia (eg. finger phalanx displaced fracture can be manipulated with digital ring block and then stabilised with a simple splint). Fractures require stabilisation (or fixation) after reduction to maintain fracture position while fracture repairs or heals. Whilst in clinical practice ‘fixation’ implies surgery, technically slabs, casts, splints and braces are also ‘fixation’ devices but non-invasive. Fixation devices are divided into two types: 1. External fixation—surgical device is constructed outside of limb as a type of skeletal stabilisation using pins, wires and screws implanted into the bone penetrating the skin and fixed together externally by bars and other devices. These

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can be used to treat fractures and correct bony deformities. External fixators are further divided based on their shapes into (a) Monolateral (unilateral or monpolar) frame (Fig. 8) (b) Bilateral (bipolar) frame (c) Circular frame • Mechanical (Ilizarov frame) • Computerised (Hexapod) such as Taylor-Spatial frame (TSF) or TL-Hex. (Fig. 9) Fig. 8 Monolateral external fixator used to stabilise knee dislocation

Fig. 9  Circular frame using with 6 struts (blue arrow and red numbers). This is why it is called a Hexapod (see chap. 11 for more details)

4 5

3

6 1

2

Orthopaedic Terminology

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Fig. 10  Fracture fixation using wires. This configuration of fixation is called tension band wires

Internal fixation—surgical devices that are applied directly on the bone surface, ‘buried’ within the surgical wound. Wound is either directly over the site of the device, or at either end of the bone if device inserted using a less-invasive method. Several internal fixation devices that are in common use: (a) Wires (Fig. 10) (b) Screws (Fig. 11) (c) Plates and screws (Fig. 11) (d) Nails • Rigid (Fig. 12) • Flexible Flexible nails are the more commonly used nails in children. As the name implies they are flexible (can be bent) to allow insertion away from joints or growth plates. They also called Nancy nails referring to the place where first clinically used. One or two nails are inserted from one end of the bone into the medullary canal and across the fracture site into the canal of the other fragment. At the insertion site, nail is cut proud off the bone but buried under skin to facilitate removal after fracture heals. In Amy’s case above, her fractures were stabilised using flexible nails that were introduced through small wounds at the wrist and or elbow for insertion of the flexible nails into radius and or ulna respectively (Fig. 13).

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L. Walker and L. H. Lee

1

3

2

4

Fig. 11  Top panel shows medial epicondyle fracture that was fixed with a single screw. Bottom panel shows a femoral fracture that was fixed with a plate and screws

Orthopaedic Terminology Fig. 12  A radiograph shows a rigid nail and wires used to stabilise a fracture in a child with osteogenesis Imperfecta

13

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L. Walker and L. H. Lee

Fig. 13  Displaced fracture of radius and ulna (A AP view, B Lateral view) C—1 week after closed reduction. Surgery involves open reduction of both radius and ulna using separate incisions over the fractures (yellow lines). It was found that muscle tissue was interposed between the fragments preventing optimum closed reduction. Flexible nails were inserted through smaller/percutaneous incisions from proximal ulna and distal radius (blue lines)

Most child bone fractures heal quickly and any residual deformity usually corrects gradually over time. This gradual correction of bone deformity is called remodelling (Fig. 14). Some fractures may take a longer time to heal and a very few may not heal at all. The former is called “delayed union” and the latter is called non-­ union. Several methods can be used to prompt bone healing in delayed or non-­ union. One of the commonest methods is using bone graft which is defined as a bone component implanted into bone or joint to stimulate bone formation, enhance fracture healing, or provide structural stability. Bone component includes bone marrow, cancellous, cortical bone or segment of bone with vascular supply (vascularised bone graft). Bone graft is classified into:

Orthopaedic Terminology

15

Fig. 14  Remodelling of fracture bony deformity over time. This child has almost completely remodelled his femoral deformity over 2 years

1. Autologous bone graft—harvested from same individual. e.g. segment of femur to pelvis during hip reconstruction. 2. Allograft—donor graft from the same species. 3. Xenograft—donor graft from a different species, e.g. bovine. 4. Bone-graft substitute—synthetic, e.g. calcium sulphate, ceramic composites. Case Study 2 Ali

Ali is a 7-year-old boy who is known to have Osteogenesis imperfecta (OI). He has had 5 tibial bone fractures over the last 3 years with minimal trauma. He was started on bisphosphonate treatment and received it for nearly 2 years now but unfortunately still suffered from fractures even whilst on treatment. Therefore, the surgeon discussed with Ali and his family about an operation to insert an intramedullary growing rod as prophylaxis against further fractures. Operation was performed under general anaesthesia with no complications. Postoperatively, he did well and was discharged after 2 days. Two weeks later, he was reviewed in OPD.  His x-ray was satisfactory and his wounds healed well. The surgeon planned to review Ali again 4 weeks later.

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L. Walker and L. H. Lee

Fig. 15  A child with osteogenesis imperfecta treated with an intramedullary growing rod (Fassier-­ Duvall type). The rod consists of two parts; female part (blue arrows) and the male part (red arrows) that slide along each other like a car aerial to allow lengthening. The rod is anchored by threads to the upper and lower epiphyses passing through the physis (green dashed arrows)

In children, the bones grow at the growth plates (physis) which is the cartilaginous segment between the epiphysis and metaphysis. The bone growth requires special consideration when treating the bones of children. For Ali to have a successful prophylactic splinting, the device should grow with him. There are several available devices which allow bone growth whilst providing structural support for the bone which they are inserted in. Ali was treated with an intramedullary telescoping nail (Fig.  15). Such nails comprise of two telescoping segments, similar to a telescoping umbrella shaft or a car aerial. The nail does not grow but it lengthens as the child grows. Although these

Orthopaedic Terminology

17

Fig. 16  A child with early onset scoliosis that was treated with VEPTER

nails are also called growing nails or growing rods, telescoping nail is a better name to differentiate them for other types of nails that can “grow”. Growing rods, nails or similar devices refer to a new generations of implants that can get longer (or shorter) mechanically or electronically pushing the attached body parts apart. Several applications of such devices are well established in pediatric orthopaedics. In the context of spine surgery, such as congenital scoliosis, vertebrae are connected with rods that are intentionally longer than the spinal segments which the rods are fixed to. As the child grows, rods are loosened regularly, e.g. 6 monthly intervals, to allow distraction and retightened under a short general anaesthesia using small wounds. In scoliosis with severe chest wall deformity, growing rods can

18

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L. Walker and L. H. Lee

b

c

Fig. 17  Lengthening of femur using the principle of distraction histogenesis. Gap between osteotomised bone increases (a, b) as the intramedullary lengthening nail is distracted. Bony callus (arrow) at end of lengthening

be attached to the ribs instead of the vertebrae (e.g. VEPTR; vertical expandable prosthetic titanium rib, Fig. 16). Newer designed rods incorporate magnetic component to allow the use of an external device to achieve lengthening or shortening therefore avoiding the need for further surgery or anaesthesia (Fig. 17). Growing rods or nails have also been utilised in long bones lengthening and deformity correction. The long bone is osteotomized (osteotomy; cut) and the growing nail is inserted and attached on either side of the cut. After a week or so, the nail is lengthened remotely and the two bone fragments move away from each other increasing the bone length (Fig. 17). The gap between the two fragments is gradually filled with newly generated bone. This process of generating new bone in response to gradual increase in tension is called distraction osteogenesis. The term has been recently changed to distraction histogenesis to reflect that other tissues, not just bone, also regenerate during the bone lengthening process. The speed and direction of bone growth can be influenced by surgical procedure to the physis. Temporary (slowing) or permanent (arresting) effects on the growth of the whole physis can shorten long bones and can reduce leg length discrepancy. Temporary slowing of the growth plate can be achieved by placing a screw across the physis on either side of the long bone or more commonly using a two-holes plate with a screw on either side of the growth plate (Fig. 18). One of the commonest implant used for this purpose is the “8-plate”, creatively named as it looks like the figure of 8 (Fig. 19).

Orthopaedic Terminology

19

Fig. 18  A child with leg length discrepancy (the left lower limb is longer than the right). Length equalisation was achieved using 8-plates to distal femur and proximal tibia Fig. 19  The 8-plate that is commonly used for guided growth

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Fig. 20  A child with marked genu valgum who was treated with guided growth. An 8-plate is inserted on the medial side of distal femur physis. This is then removed 1  year later when the deformity corrects to allow remaining growth of the physis until skeletal maturity

Slowing the growth of one side (medial or lateral) can reduce angular deformity such as valgus or varus deformities (Fig. 20). Epiphysiodesis is the term used to describe slowing or stopping the growth of the whole physis whereas hemiepiphysiodesis is the term used for slowing or stopping one side only of the physis.

Case Study 3

Alisha is a 9-year-old girl who has diagnosis of diplegic cerebral palsy. Her walking ability has been deteriorating and she is getting tired easily. She used to walk for 30 minutes easily without getting tired but now she has to stop after 15 minutes. Parents also noticed that her knees are more bent when she walks than before. She underwent gait analysis and her pediatric orthopaedic surgeons advised to change her AFOs to GRAFO.

Orthopaedic Terminology

1

21

2

3

Fig. 21  various types of AFOs. Image 1 is solid AFO, image 2 hinged or articulated AFO and image 3 Ground reaction AFO (GARFO)

Orthoses and splints are commonly used in pediatric orthopaedic patients particularly those with cerebral palsy. They are prescribed to achieve a specific purpose such as to support, protect or improve certain functions. They can be used on a short or a long term basis. These will be discussed further in relevant chapters. Orthoses are named relative to the part that they splint or support. • AFO = Ankle-Foot Orthosis (Fig. 21) • KAFO = Knee-Ankle-Foot Orthosis • HKAFO = Hip-Knee-Ankle-Foot Orthosis Some patients are suitable to have an articulated (hinged) AFO to allow a degree of movement in the ankle. Dorsi/plantarflexion. Temporary or long term, uni- or bilateral. In this chapter, we covered most of the relevant terms that are commonly used by pediatric orthopaedic surgeons. A few other terms and definitions will be covered in other chapters.

References 1. Gustilo RB, Anderson JT. Prevention of infection in the treatment of one thousand and twenty-­ five open fractures of long bones: retrospective and prospective analyses. J Bone Joint Surg Am. 1976;58(4):453–8. 2. Alshryda S, Jones S, Banaszkiewicz P. Postgraduate Paediatric Orthopaedics: the Candidate's guide to the FRCS (Tr and Orth) examination. Cambridge: Cambridge University Press; 2014. 3. Salter R, Harris W.  Injuries involving the epiphyseal plate. J Bone Joint Surg Am. 1963;45A:587–622. 4. Ogden JA.  Injury to the growth mechanisms of the immature skeleton. Skelet Radiol. 1981;6(4):237–53. 5. Peterson CA, Peterson HA. Analysis of the incidence of injuries to the epiphyseal growth plate. J Trauma. 1972;12(4):275–81.

Normal Development Wafa Binfadil, Tahani Al Ali, and Samantha Ismaile

1

Introduction

Many parents’ initial concerns are related to growth. Understanding normal child growth is key to detect health problems early or alternatively, to offer reassurance. In our experience distrust can arise from premature re-assurance or failure to detect problems early. It is not always easy to keep the right balance between re-assuring parents confidently and avoiding unnecessary follow-up or investigations. It is often repeated that children are not small adults. Growth is one very obvious difference. Children grow at varying rates throughout infancy, childhood and adolescence. In this chapter, we will focus on normal musculoskeletal development in children; emphasising key milestones, the magnitude of variations and lay the groundwork for the following two chapters, Chap. 3: General Assessment and Chap. 4: Normal Variants.

2

Understanding Normality

The concept of normality has always been a subject of debate and what is normal and abnormal is not always clear. Often there is an overlap. This creates some uncertainty during the initial assessment. Medical data such as weight, height or length or laboratory values follow a normal distribution. This means that the majority of measurements fall within a small range of values. Normally distributed data results in a more or less bell-shaped graph (Fig. 1). W. Binfadil (*) · T. Al Ali Al Jalila Children’s Speciality Hospital, Dubai, United Arab Emirates e-mail: [email protected] S. Ismaile The Higher Colleges of Technology, Dubai, United Arab Emirates © The Author(s), under exclusive license to Springer Nature Switzerland AG 2021 S. Alshryda et al. (eds.), Pediatric Orthopedics for Primary Healthcare, https://doi.org/10.1007/978-3-030-65214-2_2

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Frequency

34%

34%

68% 14%

0.1%

2%

14%

95%

2%

0.1%

Measurement such as child Height

Fig. 1  Bell-shaped graph of normal distribution

Two features determine the shape of normally distributed graph: the mean: where the peak of the curve occurs and the standard deviation (SD) which indicates the spread of the bell graph. Different parameters produce different curves but all normally distributed curves share the same features: 68% of the measurements fall within 1 standard deviation (SD) of the mean, 95% within 2 SDs and 99.7% of the observations fall within 3SDs. Moreover, in normally distributed data, the mean (average of a data set), the median (the middle of a data set) and mode (the most common number in a data set) are all the same value, and they coincide with the peak of the curve (Fig. 1). Motor and social milestones, anatomical and physiological changes in healthy children are no different although the ranges (the spread or SD) of when these changes happen are wide. Although it has been conventionally accepted that if a measurement falls within 2 SD of the mean is ‘normal’, it is beyond the scope of this book to debate this. It is important to appreciate this convention in order to understand growth charts and their interpretation. Not all data are normally distributed and there are better ways to present non-­ normally distributed data. The median is better than the mean to indicate the center of non-normally distributed data set and quartiles are better than SD to indicate the spread of data set around the median. A box plot is a common way to present a non-normally distributed data. The middle of the box plots represents the median and the sides (or the bottom and top of the box) are the first and third quartiles (equivalent to the 1SD around the mean in normally distributed data). The ends of the whiskers can represent several possible alternative values such as the minimum and maximum of all of the data, two

Normal Development Fig. 2  Box plot graph with different parts explanation

25 Outliers

Upper Whisker

Third (upper) quartile

Median, middle or second quartile

First (lower) quartile

Lower Whisker Outliers

standard deviations above and below the mean of the data. Outliers may be plotted as individual points (Fig. 2). The above two methods are good to describe static data such as haemoglobin levels, or urea and electrolyte levels but they fall short when growth over time is an important consideration. Quartiles and centiles are a better method to monitor height changes over time of growth. Quartiles are values that divide data into quarters according to where the numbers fall on the number line. The four quarters that divide a data set into quartiles are: First quartile (1Q) has the lowest 25% of measurements. Second quartile (2Q) has the next lowest 25% of measurements (up to the median). Third quartile (3Q) has the second highest 25% of measurements (above the median). The highest 25% of measurements are above the third quartile. Centiles (or percentile) divided the data into 100 centiles rather than 4 quartiles and they follow the same principle. If a child’s height is on the 50th centile, it means that 50% of children at their exact age are shorter. If a child’s height is on the third centile, it means that 3% of children are shorter, although they may still fall within the ‘normal’ range (Fig. 3).

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1Q

2Q

34%

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68% 14%

0.1%

2%

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95%

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Fig. 3  A composite graph showing the concepts of bell shaped normal distribution, box plot and quartile

3

Growth Charts

Growth charts are centile charts. Head circumference, height, weight and BMI are the most common parameters that are recorded in each visit to the paediatric orthopaedic clinic. The charts are gender specific (Fig. 4). The trend is far more important than a single reading. Several orthopedic problems can be detected and monitored using growth charts. Children with skeletal dysplasia are often shorter than average. Children with Marfan, and Klinefelter syndromes are taller than normal. Prader– Willi syndrome are shorter and heavier than normal. It is important to relate a child’s growth to their expected height centile for parents. Parents’ heights represent the child’s genetic potential. A child who is shorter or taller than expected for their parents is likely to have an underlying cause. On the other hand, if a parent is unusually tall or short, they may have an autosomal dominant growth disorder. The Target, or Mid-Parental Height with the range (± 2SDs) is calculated as follows: • Boys: Average of parents’ heights +7 cm (± 10 cm). • Girls: Average of parents’ heights—7 cm (± 8.5 cm).

Normal Development

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Fig. 4  Growth chart weight and height. The chart shows a child who was short and underweight, even for her relatively short parents. Her Target Height (expected height for parents, or mid-­ parental height) is depicted by the arrow, marked MPH on the right hand margin of the growth chart. The progressive improvement in height and weight in response to growth hormone (GH) is shown. A bone age x-ray at age 10½ years (depicted by a closed triangle joined by a line to the corresponding height measurement) was used to calculate a predicted adult height (PAH), which now corresponds to her mid-parental, or target height

4

Bone Development and Growth

The skeletal system develops during the first few weeks after conception. The skeleton is formed by cartilage and connective tissue membranes by the end of the eighth week after conception [1]. Then bones start ossification by two mechanisms: 1. Intramembranous ossification 2. Endochondral ossification Intramembranous ossification refers to bone that replaces connective tissue with bony tissue. This is the main method of flat bone formation. Examples include the skull bones, facial bones and clavicles. Endochondral ossification is more common than intramembranous ossification. It happens when cartilage (hyaline) is replaced with bony tissue. The initial conversion of cartilage into bone is called the primary ossification and it happens during the third month after conception and the areas of ossification are called primary ossification centres. Hyaline cartilage is replaced by spongy bone in the diaphyseal area of bones, which is then broken down to form a medullary cavity. The epiphyseal part of the bone continues to grow. After birth, secondary ossification centers form at the epiphysis and when this is complete, a space will form between epiphysis and diaphysis, which is called the growth plate, or physis (Fig. 5).

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W. Binfadil et al. Perichondrium

a

b Hyaline cartilage Calcified matrix

Uncalcified matrix

c

Bone collar

Primary ossification center

Periosteum

Spongy bone Nutrient artery

Uncalcified matrix

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f

Articular cartilage

Spongy bone

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Secondary ossification center

Periosteum (covers compact bone)

Artery and vein (provide nutrients to bone)

Artery and vein Uncalcified matrix

Epiphyseal plate

Artery and vein (provide nutrients to bone)

Fig. 5  Normal bone development

Growth plates or physes are made of cartilage and are radiolucent, appearing as a black band on plain radiographs. The chondrocytes of growth plates are arranged in columns along the longitudinal axes of the respective bones directed towards the metaphysis where endochondral ossification takes place [2]. These longitudinal columns are divided into 4 zones (Fig. 6):

Normal Development

29

Reserve

Proliferative

Maturation

Degenerative

Provisional Calcification

Primary Spongiosa of Metaphysis

Fig. 6  The structure of growth plate

Hypertrophic

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W. Binfadil et al.

1. Reserve zone 2. Proliferative zone 3. Hypertrophic Zone 4. Zone of endochondral ossification The reserve and proliferative zones have substantially more extracellular matrix than the hypertrophic zone and thus resist shear forces easily. Therefore, most physeal fractures occur in this zone although any zone may be involved in high energy injuries. In the hypertrophic zone chondrocytes increase in size, accumulate calcium in their mitochondria and degenerate by apoptosis. In rickets, failure of apoptosis results in the characteristic metaphyseal broadening visible clinically and on radiographs. Oxygen tension becomes progressively lower as cells pass through the hypertrophic zone. The zone is further subdivided into 3 zones: 1. Maturation 2. Degeneration 3. Provisional calcification The Groove of Ranvier at the periphery of the physis contains an active group of chondrocytes and contributes to lateral growth (width) of the physis. The blood supply to the growth plates comes mainly from two sources: the epiphyseal vessels (the main source) and metaphyseal vessels. However, in the physis of the proximal femur and proximal humerus where the epiphyses are covered fully by articular cartilage, the principal supply is from the metaphyseal vessels. The blood supply to the physis can be significantly compromised by trauma that leads to epiphyseal separation, as occurs in slipped capital femoral epiphysis. The child grows through expansion at the growth plates. When skeletal maturity is reached, the growth plates close, becoming fully ossified and fused with the rest of the bone. Boys reach growth maturity at around 17 years and girls, 14 years. When assessing growing children, it is important to know the age of appearance of ossification centres and the expected age of epiphyseal closure. Figure 7 of the elbow illustrates this. There are 6 secondary ossification centres around the elbow: 1. Capitellum 2. Radial head 3. Internal (medial) epicondyle 4. Trochlear 5. Olecranon 6. Lateral epicondyle The order of the appearance of these centres on plain radiographs can be remembered using CRITOL acronym. The internal epicondyle, for example, always ossifies before the trochlear. if the internal condyle ossification centre is absent, but the trochlear is present, this might have been caused by an avulsion injury that displaced an ossification centre into the joint (Fig. 8) [3–6]. The appearance of the ossification centres and the timing of epiphyseal closure may be used to obtain a child’s physical age—or more correctly, their bone age. A plain x-ray of the hand and wrist shows a distinctive appearance at different ages.

Normal Development

8-11yrs 8-13yrs

31

5-8yrs 7-9yrs

12-14yrs 13-16yrs

1m-11m 1m-26m

14+yrs 17+yrs

7-11yrs 8-13yrs

10+yrs 12+yrs

10+yrs 12+yrs

Fig. 7  Appearance and fusion of secondary ossification centre around the elbow

Fig. 8  Internal condyle avulsion. Plain radiograph (right image) shows no obvious fracture but the medial epicondyle ossification centre is missing (red arrow) despite the trochlea being fully ossified. CT Scan (3 D reconstruction) confirmed an avulsion fracture of the internal condyle and its presence inside the elbow joint (green dashed arrow)

There are many systems of bone age assessment, but that of Greulich & Pyle (G&P) is the easiest to use in clinical practice. The G&P atlas presents typical radiographic appearances of the hand and wrist from infancy to maturity. By knowing the bone age, it is possible to estimate remaining growth and arrive at an accurate prediction of final height (See Fig. 4). Automated bone age interpretation is a relatively recent development which permits remarkable accuracy in bone age assessment (see Fig. 9).

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Fig. 9  Example of a Bone Age X-ray, with automated bone age estimation. The G&P bone age is given, along with the standard deviation score (SDS), indicating mild bone age delay, within the bounds of normal (± 2SDs). The carpal bone are less influenced by growth hormone than the long bones (phalanges, radius and ulna) so a discrepancy between carpal bone age and G&P bone age may be seen in growth hormone deficiency or precocious puberty, for example. The TW3 (Tanner-­ Whitehouse version 3) bone age is an alternative method for bone age estimation but is less useful for predicting future growth. The BHI (Bone Health Index) is a measure of bone density. In this case, the BHI SDS is −1.13, which is below average (13th percentile), but within normal limits

Normal Development

33

Case Study 1 Michaela

Michaela presented to her GP at age 7 years, 3 months with signs of early puberty. She was extremely tall, being on the 94th percentile for height, whereas her target height based on her parents corresponded to the 18th percentile. She had breast and pubic hair development (Tanner stage 3). Her bone age was 10 years 9 months and menarche (onset of menstruation) was predicted to occur within 12  months. Given her young age, it was considered appropriate to halt her progress in puberty with use of medication. An MRI scan, done to exclude a hypothalamic or pituitary abnormality, was normal. She was treated for a total of 3 years, before it was considered appropriate for her to be allowed to progress in puberty, with menarche being reached after a further 8  months, just after 11  years of age. She reached a final height of 161.4 cm, which was a little above her parental target height and significantly above her expected height without treatment.

5

Developmental Milestones

Normal development is cephalo-caudal: a child will start to control his head movement and hands before the lower limbs (i.e. crawling and walking). Table 1, shows the normal motor milestones. Table 2, shows fine motor skills and Table 3 shows personal, social and language development. For an example of a child with abnormal development, see case 2, below. Case Study 2 Arthur

Arthur presented to his GP at the age of 22 months after referral by his health visitor, with parental concerns about developmental delay. He had just started walking a month previously and his speech was limited to a small number of single words or phrases. His GP asked mother to sit him on the floor in the middle of the room and encourage him to stand up. He did so with difficulty, demonstrating Gower’s sign, where he turned himself on all-fours, extended his arms and legs and “climbed” up his own body. The GP suspected Duchenne Muscular dystrophy and referred him to the local paediatric department. Muscular dystrophy was subsequently confirmed by the finding of an elevated Creatine Kinase (19,700 U/L) and later by a dystrophin gene deletion on genetic testing.

6

Sexual Development

The beginning of sexual maturity is a very important consideration in orthopaedics as it heralds skeletal maturity. This will help decision-making when treating conditions such as scoliosis, leg length discrepancy, short stature, ligament avulsion and physeal injuries [7–9].

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Table 1  Gross motor skills Gross Motor Skills 3 months

Raise their head above plane of the body when they are in prone position Beginning to roll over Sitting with minimal support Standing with support Standing without support Walking forward without support Running & ascending stairs with support Ascending stairs without support Descending stairs without support

4–6 months 6–7 months 9–12 months 14 months 12–16 months 18 months 2 years 3 years Table 2  Fine motor skills Fine Motor Skills 3 months 6 months 9 months 12 months 12–18 months 18–24 months 2–3 years

Lip pressure and coordinate sucking and swallowing during feeds Grasp objects and feed themselves (hand to mouth) Feed themselves food, use fingers and thumb to grasp and transfer objects Pick up spoon, drink milk from a cup Feed themselves with spoon Able to build block towers and turn pages Writing skills

Table 3  Personal social and verbal skills Personal, Social and Verbal skills 2–3 months 4 months 6 months 8–10 months 10 months 12 months 12–15 months 18 months 24 months

An infant will smile when spoken to and vocalize Turn their head to sound and recognize mother Laughing and smiling Responding to “no” Waving goodbye, say “da-da” and “ma-ma” Show interest in picture books and recognize familiar objects Speak 4–5 words Achieve vocabulary of 10 words Speak 3-word sentences

Tanner’s staging (also known as Sexual Maturity Rating: SMR), is a scale used to help document the development of secondary sexual characteristics in children during puberty, as in case 1. It is based on the breast size in girls, genitalia size in boys and pubic hair in both. It helps in counselling children and parents with the anticipation of hormonal and physical changes that the child would undergo during puberty [10–13].

Normal Development

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Table 4  Tanner stage for girls Stages Pubic hair growth 1 No pubic hair 2

Minimal pubic hair at the labia

3

Darker hair appear and spread sparsely over the pubic and axilla Terminal hair that fills the entire triangle of the pubic region Pubic hair distribution of adults (hair extends to the thighs)

4 5

Breast development Papillar elevation above the level of the chest wall of the breast Breast bud development and areolar growth Enlargement of breasts and areolae Secondary areolar mound formation Mature female breasts

a Anterior

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Fig. 10  Tanner stages in girls

In females, the average age of onset of puberty is 8 to 13 years with menarche around 12.5 years of age. Peak height velocity is achieved between Tanner two and three—typically around 11–12 years. (Table 4 and Fig. 10) In males, the onset of puberty ranges from 9 to 14 years, with peak height velocity achieved in Tanner Stage 3, and spermarche at stage four. (Table 5 and Fig. 11).

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Table 5  Tanner stage for boys Stages Pubic hair growth 1 No pubic hair 2

Sparse minimal pubic hair

3

Pubic hair is darker and continuous over the scrotum and pubis Terminal hair that fills the entire triangle of the pubic region Hair distribution is adult like in quantity and type, may spread to the thighs, facial hair development begins

4 5

1

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Genitalia development No penile or testicular enlargement Slight testicular and scrotal enlargement Further growth of the penis and testicles Continued penis and testicular enlargement Adult like with regards to size and shape

3

5

Fig. 11  Tanner’s stages of development of secondary sexual characteristics: male

7

Summary

An understanding of the means by which we categorise and measure development and growth through understanding of normal variation is important to the proper assessment of children. Unfortunately, there is no substitute for learning the key developmental milestones. When combined with an understanding of bone physiology, this aids the physician in the assessment of the child—in particular the use of growth charts and bone age to predict future growth. Sexual maturity is the final phase of normal childhood development, bringing with it the end of the journey through childhood and adolescence.

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References 1. Herring JA. Tachdjians. Pediatric orthopaedics. Philadelphia: Saunders Elsevier; 2013. 2. Alshryda S, Jones S, Banaszkiewicz P. Postgraduate paediatric orthopaedics: the Candidate’s Guide to the FRCS (Tr and Orth) Examination. Cambridge: Cambridge University Press; 2014. 3. Alshryda S, Tsang K, Dekiewiet G.  In: Alshryda S, Huntley JS, Banaszkiewicz P, editors. Paediatric orthopaedics: an evidence-based approach to clinical questions: Springer; 2016. 4. Osbahr DC, Chalmers PN, Frank JS, Williams RJ, Widmann RF, Green DW. Acute, avulsion fractures of the medial epicondyle while throwing in youth baseball players: a variant of Little League elbow. J Shoulder Elb Surg. 2010;19(7):951–7. 5. Potenza V, Farsetti P, Caterini R, Bisicchia S, De Luna V, Ippolito E. Neglected fracture of the medial humeral epicondyle that was entrapped into the elbow joint: a case report. J Pediatric Orthopaedics B. 2010;19(6):542–4. 6. Wilson NIL, Ingram R, Rymaszewski L, Miller JH. Treatment of fractures of the medial epicondyle of the humerus. Injury. 1988;19(5):342–4. 7. Clark KD, Tanner S. Evaluation of the Ottawa ankle rules in children. Pediatr Emerg Care. 2003;19(2):73–8. 8. Westberry DE, Davids JR, Shaver JC, Tanner SL, Blackhurst DW, Davis RB. Impact of ankle-­ foot orthoses on static foot alignment in children with cerebral palsy. J Bone Joint Surg Am. 2007;89(4):806–13. 9. Westberry DE, Davids JR, Cross A, Tanner SL, Blackhurst DW. Simultaneous biplanar fluoroscopy for the surgical treatment of slipped capital femoral epiphysis. J Pediatr Orthop. 2008;28(1):43–8. 10. Bruserud IS, Roelants M, NHB O, Madsen A, Eide GE, Bjerknes R, et  al. References for ultrasound staging of breast maturation, tanner breast staging, pubic hair, and menarche in Norwegian girls. J Clin Endocrinol Metab. 2020;105(5):1599–607. 11. Campisi SC, Marchand J, Siddiqui FJ, Islam M, Bhutta ZA, Palmert MR.  Can we rely on adolescents to self-assess puberty stage? J Clin Endocrinol Metab. 2020;105(8):2846–56. 12. Koopman-Verhoeff ME, Gredvig-Ardito C, Barker DH, Saletin JM, Carskadon MA. Classifying pubertal development using child and parent report: comparing the pubertal development scales to tanner staging. J Adolescent Health: official publication of the Society for Adolescent Medicine. 2020;66(5):597–602. 13. Sonntag B, Eisemann N, Elsner S, Ludwig AK, Katalinic A, Kixmuller D, et al. Pubertal development and reproductive hormone levels of singleton ICSI offspring in adolescence: results of a prospective controlled study. Hum Reprod. 2020;35(4):968–76.

General Assessment Sonia Chaudhry and Zarana R. Swarup

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Introduction

Musculoskeletal assessment of the child is an important skill for both generalists and subspecialists alike, in order to detect orthopaedic pathology during well-child, screening and focused complaint visits. From the newborn to the young adult, musculoskeletal conditions have variable presentations. Conditions such as infant brachial plexus palsy injury and humerus fractures present with obvious abnormality. Other issues, such as developmental hip dysplasia or a tethered cord, can be subtle. It is important to specifically examine for these conditions, as morbidity increases with late recognition. However, for other conditions, such as adolescent idiopathic scoliosis, there is debate as to the cost-effectiveness of routine screening. While the musculoskeletal history and exam are small components of a general practitioner’s wide range of tasks during a patient visit, a knowledge of the presentation of orthopaedic conditions in childhood is of key importance in order to identify problems requiring management or referral, as well as to reassure parents when appropriate.

S. Chaudhry (*) University of Connecticut School of Medicine, Farmington, USA Pediatric Orthopaedic and Hand Surgeon, Connecticut Children’s Medical Center, Hartford, USA Z. R. Swarup Pediatrician, Children’s Hospital of Philadelphia Primary Care, Philadelphia, PA, USA © The Author(s), under exclusive license to Springer Nature Switzerland AG 2021 S. Alshryda et al. (eds.), Pediatric Orthopedics for Primary Healthcare, https://doi.org/10.1007/978-3-030-65214-2_3

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History

In all fields of medicine, a thorough history will usually identify the diagnosis, or at least generate a focused differential. For many orthopaedic conditions, antenatal, neonatal, and developmental history will be germane. Additionally, family and social history are important especially for early childhood and adolescent conditions.

2.1

Antenatal History

Starting with pregnancy history, factors affecting intrauterine environment should be recorded. Gestational diabetes has been implicated in conditions such as VACTERL association and sacral agenesis. It can also lead to higher birth weights, a risk factor for brachial plexus palsy of birth (BPPB). The family should be asked if this was the mother’s first pregnancy, as a smaller in utero environment is associated with “packaging” disorders such as developmental hip dysplasia (DDH), congenital muscular torticollis (CMT), and calcaneovalgus foot deformity. Quickening, or fetal movements, should be asked about, as failure to experience this around 4–5 months of gestation may suggest myopthic diseases such as arthrogryposis or spinal muscular atrophy. Fetal position is also relevant, as being in the breech position is a risk factor for CMT, BPPB, and Developmental Dysplasia of the Hip (DDH). Breech positioning during latter portions of pregnancy, even if not the final position at birth, is such a high risk factor for DDH that one should consider routine ultrasound screening of the hips in such cases, even with a normal hip exam. Antenatal use of any potential teratogenic substances should be specifically inquired about. Historically, thalidomide was an anti-nausea medication used in the 1960s that lead to thousands of cases of phocomelia- shortened upper limbs with a flipper-like appearance. Current teratogens include methotrexate, certain anti-­ epileptics, notably sodium valproate, warfarin, and angiotensin converting enzyme inhibitors, to name a few. Substance use such as alcohol can lead to fetal alcohol syndrome, with orthopaedic manifestations including joint contractures, congenital fusions of the spine, radial aplasia, radioulnar synostosis, hip dislocations, pectus excavatum, and myelodysplasia [1]. Non-orthopaedic manifestations can include facial dysmorphism, abnormal growth, and central nervous system (CNS) dysfunction. Tobacco use is a risk factor for clubfoot [2]. Exposure to illnesses, especially in the TORCHES group (toxoplasmosis, rubella, cytomegalovirus, herpes, and syphilis) should be inquired about as they can be associated with CNS defects and lead to neuromuscular conditions, such as cerebral palsy (CP), with orthopaedic manifestations. Certain orthopaedic conditions may be detected on ultrasound, and prenatal orthopaedic consultation may be offered. Abnormal limb lengths, such as with congenitally short femur, will have no immediate requirement for orthopaedic involvement, however parents can be mentally prepared for the care requirements in the

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future. Other findings, such as clubfoot, should prompt orthopaedic consultation as soon as convenient within a few weeks of birth to allow evaluation and treatment. While occasionally prenatal ultrasound findings can be erroneous, such as in the case of a flexible metatarsus adductus foot positioned in equinus and therefore mimicking a clubfoot, this represents a small percentage of cases, and parents generally appreciate being prepared for the worst rather than being caught unaware.

2.2

Birth History

The family should be asked about the nature of the child’s birth. Prolonged labor is associated with CNS dysfunction such as in CP, while shoulder dystocia is a risk factor for BPPB. Vaginal delivery versus Caesarean section, along with the reason for the latter, should be determined. While a family may not be cognizant of fetal presentation, breech positioning is a common cause for Caesarian section and this detail should prompt consideration of DDH. Birth weight is important, as large babies are at risk for BPPB and low birth weight children are at risk for a multitude of conditions including CP, metabolic bone disease, and neonatal infections. The amount of time the infant spent in the hospital is relevant as well, as NICU stay and oxygen requirements can have CNS implications. Untreated jaundice leading to kernicterus and athetoid pattern CP is mostly historical with the widespread use of rhesus immune globulin treatment of maternal-fetal Rhesus incompatibility and phototherapy for jaundice. Birth records should be examined for any red flags. Abnormal muscle tone, contractures, or asymmetrical movements are all concerning. Depending on orthopaedic specialist availability in the delivery facility, consultation may have already taken place to explore such findings. Certain orthopaedic conditions represent true emergencies, such as constriction bands threatening necrosis. Others are visually striking, as in the case of severe calcaneovalgus foot or knee hyperextension, but clinically benign and often safely treated with observation and casting, respectively. Asymmetrical arm movement concerning for fracture or BPPB will often prompt early workup with radiographs. Low tone, clubfoot, hip instability, and congenital contractures are examples of conditions that can safely wait 1–2 weeks for specialist evaluation.

2.3

Neonatal and Infant History

Milestones are a sensitive marker for general health and development. When assessing a child of any age, asking about delayed motor milestones can point to a wide range of orthopaedic pathology. Head control should be achieved by 3–6 months, independent sitting by 7  months, crawling by 9–12  months, and walking by 12–18  months. Delayed head control and sitting are concerning for hypotonia. Crawling is a complex movement performed in different patterns by children, or

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skipped completely in some children, who go from sitting to walking without this intermediate stage. Delayed walking, however, should prompt investigation into central causes such as CNS dysfunction, more peripheral pathology such as DDH, or muscular dystrophy. Patients with CP will almost always have a history of delayed milestones and other red flags such as seizures or persistent infantile reflexes. A child with hemiplegic pattern CP may develop early hand preference. Ambidexterity is typical until at least 2–3 years of age [3]. Several points are important to keep in mind when considering the assessment of delayed milestones. The first is that the above time guidelines are averages. While the absence of walking by 18 months is very concerning to parents, delays until age 2 may be normal. From an orthopaedic standpoint, the child with delayed ambulation warrants a thorough history and physical accompanied by a simple one-view AP pelvis radiograph. Even findings such as asymmetric sitting or gentle spinal curves can be investigated with noninvasive methods in infancy in order to avoid unnecessary radiation with full-length spinal films that expose the radiosensitive soft tissues of the thorax and abdomen at this young age. MRI with sedation exposes infants to anesthesia at a young age and may not change immediate management. Ultrasound is a superb tool in the workup of the infant spine, particularly within the first 3  months, before ossification of the spinal processes impede imaging. This modality can detect congenital scoliosis and tethered cords without radiation or sedation [4]. In the setting of positive orthopaedic findings and delayed milestones involving mobility, in the hopes of reassuring parents I will often tell them that there is nothing that an orthopaedic surgeon or any other practitioner can do to make a child ambulate. The drive to explore one’s environment and mobilize will arise from the CNS, and nothing, including deformities or even the absence of a limb, will stop a child from doing this if he/she is physically capable of doing so. If CNS dysfunction is the cause, there is often little that can be done. In this situation, the role of the orthopaedic surgeon is to optimize mechanics, and so aid locomotion, whether relocating a hip or correcting a clubfoot.

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Childhood History

A child’s personal health history will be relevant for certain orthopaedic conditions. There is a spectrum of bone health dependent on calcium and vitamin D intake [5], though true nutritional rickets is rare with most modern diets. However, decreased outdoor activity, use of sun protection, and restrictive diets may predispose children to osteopenia, fractures and even rickets. Additionally, immunization history is imperative. The alarming trend of delayed or deferred vaccinations is a reminder not to assume that regular wellness checks have included vaccinations. Polio, for example, will cause necrosis of anterior horn cells in the spinal column along with motor nuclei in the brainstem, leading to a flaccid type paralysis. Tetanus vaccines and booster dates should be known in the case of injuries penetrating the skin.

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Major illnesses should be elucidated, even if completely resolved. Untreated Group A streptococcus can initially cause a sore throat, but progression to rheumatic fever can cause a migratory arthritis involving many joints. Any picture concerning for single or multiple joint inflammation or infection should prompt questioning of historical illnesses, both viral and bacterial. Staphlococcus is the most common bacterial infection. Lyme disease from borrelia burgdorferi is also quite prevalent, particularly near wooded areas. One should include an age-appropriate sexual history, which may require questioning the patient away from caregivers, as conditions such as gonococcal urethritis can cause late mono- or poly-articular septic arthritis. Post-infectious syndromes can range from isolated synovitis of joints to general malaise. Parsonage-Turner syndrome, or brachial neuritis, has a dramatic presentation of shoulder pain that progresses to a flaccid paralysis of the upper extremity that can follow infections or vaccinations. Fortunately, its natural history is favorable. Major illnesses can also explain radiographic findings. Harris growth-arrest lines may be noted on radiology reports of long bones, and generally indicate a period of halted growth where calcium continued to be deposited without actual elongation of bone, resulting in radiodense lines about the growth plate or physis. They are found with resumed growth after injury. Multiple unexplained growth arrest lines are concerning for non-accidental trauma, however the body will also have stunted bone growth during a major illness or in the case of multiple occult fractures as with osteogenesis imperfecta. In addition to a general medical history, trauma and surgical histories are particularly germane to orthopaedics. Asking about prior trauma such as fractures will often prompt parents to recall limb injuries that they thought were contusions but “didn’t heal right,” and post traumatic deformity or stiffness is then noted. Surgical history, both emergent and elective, is important. Neuromuscular patients used to be subjected to “birthday surgery,” or frequent surgeries even yearly to address tight muscles and joints. The trend has shifted towards single-event multilevel surgery (SEMLS), combining procedures whenever possible, to minimize multiple anaesthetics and interference with childhood activities.

3.1

Family History

Family history is important for identification of genetic conditions as well as managing parental anxiety. Conditions detectable from infancy, such as DDH and clubfoot, have small genetic components. Scoliosis has a genetic component as well, and despite natural history studies demonstrating even large curves not to be functionally limiting and be minimally causative of back pain [6], families will often attribute back problems to untreated or mismanaged scoliosis. Other musculoskeletal conditions have stronger inheritance patterns. It is important not to make assumptions about what may seem to be obvious treatment goals. Postaxial polydactyly type B (duplicated small finger with small soft tissue bridge), for example, is autosomal dominant with strong penetrance. Parents with duplicated

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fingers may not be interested in removal of the extra digit for their children as in many cultures this is considered auspicious, or parents may want the children to be “like them.” Similarly, achondroplasia is an autosomal dominant form of dwarfism with morbidity such as spinal stenosis, angular limb deformities, and short stature with functional implications, however parents may be pleased to have children with family characteristics. Similarly, pointing out syndromic facial features untactfully can be offensive to parents who may attribute such characteristics to family traits as opposed to genetic disease. Lastly, there is a genetic component to the appearance of limbs within the normal range. Whether a child in-toes, walks straight, or out-toes after initial childhood remodeling is thought to be similar to how parents’ limbs are oriented. The same goes for flexible flat feet or high arches. Two salient points on these factors are as follows: [1] This does not mean that the conditions are benign, as out-toeing or high arches are more likely to be pathologic than their counterparts; and [2] parents may feel that their child’s “deformity” is being ignored or downplayed as they had treatment such as braces, custom arch supports, or special shoes that are not being ordered. The latter can be addressed by reassurance that large series of natural history and comparative studies have shown these interventions not to affect final position of the limb. We now know that with these treatments, correction was due to spontaneous and predictable remodeling with growth, and functional outcome is not affected even when these conditions are not “fixed”. The indication for workup and treatment of these conditions is limited to patients with asymmetry, pain, or functional limitations, with some exceptions—for instance high arches, causing pes cavus foot deformity, is associated with neurologic conditions, such as Charcot-­ Marie Tooth disease.

3.2

Social History

An age-appropriate social history helps guide assessment and treatment plans. A mantra in pediatric orthopaedics is the “4–2 Rule,” or doing things for a patient and not to a parents. Knowledge of a child’s school achievements, hobbies, patterns of activities of daily living, and functional goals are key to focusing on addressing things to enhance their lives. For special needs children, therapy and equipment available at school and at home should be ascertained. In the older child, substance use especially tobacco should be determined, as it affects tissue healing. Specifically, fracture consolidation is delayed with both active and passive tobacco exposure [7].

3.3

History of Present Illness

The chief complaint and from whom it originated is a key component to a successful visit. A complaint brought up by a child will differ from that of a parent, grandparent, teacher, or coach. A direct complaint of knee pain from a child in a specific location, for example, is distinct from a parent noticing the child avoiding activities

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involving the knee or a coach noticing a limp. Avoidant behavior can often be mistaken for a medical problem by caregivers. Similarly, in-toeing may be perceived to be causing falls by a grandparent or parent, but further questioning will elucidate that the child is able to keep up with their friends and has had no injuries or limitations from their condition. Validation is important regardless of the complaint or its source. The history of present illness should include details of symptom onset, the settings in which it manifests, and any prior treatments. The mnemonic “PQRST” is useful for detailing pain, referring to provocation/palliation, quality/quantity, region/radiation, severity, and timing. Pain worse in the morning is concerning for inflammatory conditions such as juvenile rheumatoid arthritis, whereas night pain is more common in general as this is when a child is less distracted and tends to notice his/her pain more. Red flag symptoms should be specifically asked about for any musculoskeletal complaint. These include unexplained fevers or chills, constitutional symptoms, unintended weight loss, or pain waking a patient up at night. These symptoms warrant further investigation as a significant percentage of neoplasias such as childhood leukemias will present as isolated bone pain [8].

4

Physical Exam

The physical exam is a key component of the general orthopaedic assessment. Here we will review musculoskeletal exam principles in conjunction with the orthopaedic components of newborn, elementary, and adolescent exams for screenings as well as common complaints at those ages. The focused exam pertinent to individual conditions will be covered in subsequent chapters.

4.1

Musculoskeletal Exam Basics

The general orthopaedic exam should begin with inspection. This requires visualization of the limbs as well as the axial spine, therefore a sleeveless shirt and shorts are ideal, and examination of the feet with the socks off is mandatory for a comprehensive exam. Skin lesions such as café au lait spots or neurofibromas can point to diagnoses such as McCune Albright Syndrome or neurofibromatosis (Fig.  1). Additionally, skin ulcerations or red spots indicate abnormal load bearing such as in pathologic flat foot with medial arch lesions, ulcers, or dry callused knees from crawling in the cases of neuromuscular or arthrogrypotic patients not using ambulatory devices at home. Bony protuberances may be noted, as in the case of multiple hereditary exostoses (Fig. 2). By convention, deformities are defined by their distal segment. For example, a Boxer’s fracture of the fifth metacarpal neck is most often displaced and angulated volar or palmar, as the distal segment is more prominent in the palm. Alternatively, one can define where the apex of the deformity is, such as an “apex volar” distal radius wrist fracture when the fracture is angulated into extension.

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Fig. 1  Café-au-lait spots. Right; ragged border ‘Coast of Maine’ pattern Café-au-lait spots in a patient with McCune-Albright syndrome in comparison to the smooth border ‘Coast of California’ seen in neurofibromatosis (left)

Fig. 2  Bony protuberances in children with isolated bony exstosis

Varus and valgus are indicators of coronal plane alignment, i.e. looking at a patient from the front or back. Valgus angulation is the distal segment angled away from the plane of the body while varus is angulation towards. The joint referenced can be stated before the description of the direction, such as coxa (hip), cubitus (elbow), or genu (knee). In the foot, adductus is similar to varus, as in metatarsus adductus or varus. It is important to keep in mind that these are relative terms. The mature hip has 120° neck-shaft angle, or proximal femoral varus, so any increase in that angle towards 180° is termed coxa valga despite still being in varus, and a decreased neck-shaft angle is termed coxa vara. Similarly, the average carrying angle of the fully extended elbow is 16° of valgus [9], so a straight ulno-humeral angle is in relative cubitus varus. Coronal plane angulation, both clinically and radiographically, is affected by joint positioning. In the case of the elbow, flexion will exaggerate the apparent valgus angulation of the elbow (Fig. 3), which can mask cubitus varus. Similarly, rotation of the hips can alter the angular appearance of the knees. External rotation at the hips will minimize genu valgum, or cause apparent genu varum. Internal

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Fig. 3 (a) Carrying angle (asterisk) of the elbow should be assessed in full extension where it averages 16° of valgus. (b) Elbow flexion exaggerates the apparent valgus angle of the elbow (alpha)

rotation will do the opposite. It is therefore important to measure lower limb alignment with the patellae facing directly forward (Fig. 4), as opposed to standing with the feet straight. One can “eyeball” angular deformities by referencing 0° as neutral, 90° as a right angle, with estimation of common angles in between such as 30–45-60°. Alternatively, a goniometer can be used to measure angulation from specific bony landmarks or along the limb axis. Bony landmarks are more accurate, as in the case of genu valgum or knock-knees, copious soft tissues around the thigh will often accentuate this angle if measured along the apparent axis (Fig. 4), while the true femoral axis is obtained from the anterior inferior iliac spine to the patellar midpoint.

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Fig. 4  The tibiofemoral angle drawn along the long axis of the limbs shows alignment to be in overall valgus. Erroneous measurement is avoided by ensuring the patellae are facing forward and using bony landmarks as opposed to “eyeballing” the limb axis. The coronal plane alignment of the lower limb should be assessed with the patella facing forward. The examiner’s fingers at the top are palpating the patella to face forward while the left hand at the bottom controls limb rotation

Sagittal plane angulation, or the side view, is equally important. By convention, 0° is fully extended and 180° would be full flexion; the latter never achieved due to the soft tissues. While Blount’s disease is commonly thought of as excessive bowing of the legs, there is often a procurvatum or apex anterior angulation component at the proximal tibia and internal rotation from the posteromedial growth disturbance (Fig. 5). Conversely, an apex posterior angulation is present at the posterior distal tibia in the case of the calcaneovalgus foot of the newborn. Most deformities are multiplanar, or rather in one plane that does not fit our standard 2D planes. The true extent of the deformity is minimized unless assessed perpendicular to the plane of deformity. Distal tibial bowing, for example, is most often posteromedial, but can be

General Assessment Fig. 5  Blount’s disease is a good example of multiplanar deformity. It is commonly thought of as excessive bowing of the legs (top row images), but there is often a procurvatum (middle row images) and internal rotation (bottom row images)

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anterolateral or anteromedial. The implications are great; as posteromedial bowing tends to be an isolated self-resolving condition occasionally resulting in limb length discrepancy. Anterolateral and anteromedial bowing, however, are associated with tibial pseudarthrosis and fibular deficiency, respectively, both of which will often require multiple surgical procedures or even amputation. Lastly, the axial plane, or rotation, should not be ignored. This is the hardest to measure as it can be masked with joint mobility, and radiographs do not capture it, instead requiring axial cuts on CT or MRI. Scoliosis is a great example of a multiplanar deformity, often most marked in the axial plane. This is why a well-balanced curve can be masked by a compensatory curve, but the Adams forward bend test will reveal a rotational deformity in the form of rib prominence, since they are attached to the vertebrae. Similarly, children can rotate their lower extremities at the hip in order to mask in-toeing or out-toeing, commonly known as “doctor walking,” and avoid any feared interventions. The most common cause of the flat foot is explained by axial plane rotation. Patients will subconsciously mask a tight tendo-achilles by externally rotating the hips and walk on the medial border of their foot in order to get the foot flat on the ground. This avoids toe walking for a more efficient gait, but over time can lead to knee and foot discomfort if the tendo-achilles is not stretched (Fig. 6).

4.2

Infant Exam

The musculoskeletal exam of the infant is of great import in recognizing orthopaedic pathology early. As the neurologic system is intimately associated, many exam components are similar. Overall muscle tone, arousability, and age-appropriate infant reflexes should be assessed. The infant should be fully undressed. Starting peripherally, limbs should be examined for overall symmetry of girth and length. The hands and feet should be examined for the correct number, size, and mobility of digits. Duplicated, short, incompletely separated, or angulated digits are common. The presence of skin creases indicates intrauterine motion, therefore the absence of skin creases indicates underlying joint and muscle abnormalities with loss of motion, often seen in contracture syndromes such as arthrogryposis (Fig. 7 image 1). Abnormal creases may represent constriction bands, in which case perfusion distal to the band should be assessed to ensure the limb is not threatened (Fig. 7 image 2). Shoulders and elbows have physiologic newborn contractures limiting full passive range of motion until about 3 months of age, similar to lower extremity physiologic contractures (except for hip external rotation contractures which persist the longest). The key to detecting abnormal contractures or paralysis is in noting asymmetry of movement, both active and passive. Decreased spontaneous movement of an arm can have several causes including clavicle fracture, humerus fracture, congenital radial nerve palsy of the newborn, and BPPB. While most of these conditions will resolve spontaneously without sequelae, the latter should prompt referral

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Fig. 6  Patients subconsciously mask a tight tendo-achilles by externally rotating the hips and walk on the medial border of their foot in order to get the foot flat on the ground (images 1 & 2). This avoids toe walking for a more efficient gait, but over time can led to mid foot break (Talo-­ navicular joint subluxation red solid arrow in image 5). This was corrected by lengthening the tendon to restore good ankle dorsiflexion (image 4) and correct foot alignment and reducing talo-­ navicular joint (image 3 & 6)

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Fig. 7  Image 1 shows lack of creases over the finger knuckles with joint contracture in a child with arthrogryposis. Image 2 shows constriction band syndromes with normal peripheral circulation

to a specialist to begin guided range of motion exercises and early surgical exploration should it not improve. In the lower extremity, the feet should be supple with good hindfoot and subtalar motion. The typical intrauterine positioning will give nearly all infants metatarsus adductus, however if the lateral border is passively flexible into a straight position, this will resolve to a large degree, with residual varus well tolerated (Fig.  8). Abnormally bean-shaped rigid feet are concerning for clubfoot, versus the more rare everted “Persian slipper” type foot in congenital vertical talus. A foot with its dorsum plastered up against the anterior tibia is typical of calcaneovalgus foot, and while it can look like a dislocation needing acute treatment, it is often self-limiting with the only sequelae being a flexible flat foot in many cases. Newborn knees should be contracted into flexion and should be examined for gentle passive extension. Contraction past 45° is abnormal and may indicate a congenital knee flexion contracture or congenital patellar dislocation. A hyperextended knee is seen in congenital knee hyperextension, subluxation, or dislocation, all of which are usually amenable to serial casting. Bilateral knee hyperextension is typically syndromic, but even unilateral cases have high associations with DDH, clubfoot abnormalities, and an anterior cruciate ligament (ACL)-deficient knee (Fig. 9). The hips will likewise have flexion/external rotation contractures into the “frog” or “human” position, which is protective of the joint and should be allowed to resolve spontaneously. The hip exam should reveal symmetric knee heights when the infant is supine and balanced on an even surface holding the thighs straight up with the hips flexed to 90°. A shorter knee, or Galeazzi sign, most likely represents a dislocated hip as the femoral head may be resting posterior to the acetabulum, or more rarely a shorter femur. Hip abduction should be symmetric and near full or 90°, i.e. being able to get the outer knees touching the exam table. Limited hip

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Fig. 8  Foot positioned position in metatarsus varus (a) showing curved lateral border (solid arrow) and first ray in varus (dashed arrow). The hindfoot should be flexible into valgus, and the lateral border able to be passively straightened (b) as seen here against the examiner’s thumb Fig. 9  congenital knee hyperextension that requires serial casting

abduction is initially caused by mechanical impingement of the dislocated hip and later by contracture of the adductor muscles. Instability is tested with manoeuvers such as the Ortolani to relocate the dislocated hip and the Barlow to dislocate the unstable but reduced hip. Only one can be positive, depending on whether the hip

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rests inside or outside the acetabular socket. Asymmetric thigh folds and hip clicks are less specific for DDH, however any suspicion based on physical exam, history (particularly of breech positioning), or strong parental concern should prompt workup with ultrasound, as this is a low cost and noninvasive test. The hips should continue to be assessed at every visit until the child is walking. Once ambulatory, “walking hip dysplasia” will present as a Trendelenburg gait and/or limb length discrepancy in the fully dislocated hip, and is much harder to treat. Dysplastic but located hips will develop early arthritis. Moving up the axial skeleton, the back should be examined for cutaneous indicators of occult spinal dysraphism such as skin dimpling or hair tufts (Fig. 10). The spine is C shaped in the sagittal plane in infancy, developing its curves after sitting and standing. Eyes should be assessed for equal tracking to each side, and passive neck rotation and tilt should be checked to evaluate for torticollis (Fig. 11). A tight fibrotic sternocleidomastoid is often the culprit and usually improves with home stretching and physical therapy. Failure to improve, presence of a distinct mass, or other concerning factors should prompt referral to a specialist as occasionally botulinum toxin or surgical release is indicated.

a

b Fig. 10  Image a shows a deep sacral dimple. Image b shows skin tag overlying spina bifida

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Fig. 11 Congenital muscular torticollis

4.3

Toddler/Elementary Exam

After infancy, the musculoskeletal component of the well child visit becomes simplified. A normal child meets age appropriate milestones, has no persistent primitive reflexes, has a symmetrical normal gait and does not favor either upper extremity. A quick series of activities to test lower extremity neurologic and musculoskeletal function can be integrated into a short visit as follows: getting off the exam table, hopping on each foot, regular/toe/heel/lateral border/tandem (heel-to-toe) walking, and performing a full squat and rise without assistance [2]. Common musculoskeletal concerns by caretakers at this age include frequent falls, flat feet, toe walking, in-toeing, and bowed legs. Frequent falling should be further clarified. Comparison to peers of a similar age is helpful, as is asking about the child keeping up with others their age. A thorough age-appropriate neurologic exam should assess tone, coordination, muscle strength and fatigability, and persistent abnormal neurologic reflexes such as a Babinski’s sign or clonus. Any regression of milestones or change in the developmental curve should prompt concern for conditions such as tethered cord or Rett’s syndrome. Tethered cord should also be on the differential for toe walking, resistant clubfoot deformity, or other neuromuscular conditions that are not following the expected patterns. Proximal muscle weakness is concerning for myopathy. Flat feet are almost universal when beginning ambulation, as arch development occurs with further growth. In fact, early or prominent arches that do not stretch out

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b

Fig. 12  Image a: While weight bearing in the foot flat position, the heel rests in valgus (a) as shown by the tibiocalcaneal angle drawn here (solid white lines). On toe rise (b), a flexible foot should demonstrate heel inversion into varus (solid white lines)

with weight bearing are more concerning as the caval foot can be associated with neurologic conditions such as Charcot Marie Tooth Disease and other Hereditary Sensory Motor Neuropathies. Evaluation should include assessment for a tight tendo-achilles, flexible ankle on toe-rise (Fig. 12), and pliable foot without ulcerations, callosity, or other indicators of abnormal weight bearing. Lack of ankle eversion is concerning for tarsal coalition, or peroneal spastic flatfoot, but tends to be symptomatic in the early teen years. Beyond invasive measures, no special devices can induce an arch in the flexible flatfoot, and in fact the lack of support such as walking barefoot promotes strengthening of the muscles and ligaments that aid arch development. Parents can be reassured that even if a flat foot does persist, almost half of the adult population has what is considered a flat foot, and the majority are asymptomatic. Toe-walking is a common pattern observed in toddlers. Important details include whether it is unilateral or bilateral, constant or intermittent, if there is a positive family history, persistence beyond age 4, and if it is new in onset or has been present since ambulation began. Unilateral findings or new onset cases are especially concerning for neurologic etiology such as hemiplegia or a tethered cord. A thorough neurologic exam is indicated. Specifically, the child should be requested to walk

General Assessment Fig. 13  When the foot is allowed to evert while assessing for tendoachilles tightness, dorsiflexion can occur through the midfoot (solid arrow) instead of tibiocalcaneal motion. This is termed a “midfoot break” and allows foot flat weight bearing despite having a tight tendoachilles (dashed arrow). Image b: Inverting the hindfoot locks midfoot (solid arrow), forcing ankle dorsiflexion to occur through stretch of the tendo-achilles (dashed line). This is termed a “runner’s stretch” and is used to stretch the tendo-achilles

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with a heel toe pattern as well as a heel walk to see if they have the coordination and range of motion to perform this. If they revert to toe-walking only when distracted, this is more reassuringly habitual or idiopathic rather than pathologic. Ankle range of motion should be assessed with the heel held in neutral to slight varus to lock the foot. In ankle valgus, dorsiflexion can be achieved through a midfoot break rather than true ankle or tibiotalar motion (Fig. 13). Additionally, the Silverskiold test will assess for a tight gastrocnemius versus the entire tendo-achilles. It is positive if ankle dorsiflexion is limited with the knee extended but improves with knee flexion, as the latter relaxes the gastrocnemius that crosses both the ankle and knee joints. With red flags ruled out, parents can observe for improvement or trial walking casts. In-toeing is a common parental observation. The newborn will appear this way due to metatarsus adductus, where the key examination finding to reassure parents that this is physiologic rather than pathologic is the suppleness of the foot and ankle as noted earlier. With onset of ambulation, the hip external rotation contracture starts to resolve, which will unmask physiologic internal tibial rotation and femoral anteversion. These rotational axes will continue to remodel into more external rotation until final rotational profile is achieved, typically between 5–7  years of age. Persistent internal tibial torsion (ITT) is typically the cause of in-toeing prior up to 4 years of age, and can be assessed by examination of the thigh-foot angle (TFA), or relation of the axis of the foot compared to the axis of the thigh as viewed with the hips and knees flexed to 90°. A TFA of 15° external rotation is normal, whereas

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smaller or negative angles are considered ITT and larger angles external tibial torsion (ETT). ETT is more concerning, being associated with pathologic conditions such as myelodysplasia or contributing to patellar malalignment and knee pain. After age 4, femoral anteversion is often the cause of in-toeing. Assessing the rotational axis of the femur is most accurately performed by having the patient prone on the table, palpating the greater trochanter (GT) while rotating the leg with the knee flexed 90° to feel when the GT is most prominent and directly facing lateral, and seeing the angle subtended by the tibia and a straight vertical line. This should average 15° of anteversion or internal rotation of the leg, with a higher angle representing excessive femoral anteversion. This can be challenging to perform, therefore another useful exam is performed prone on the exam table or supine on caregiver’s lap, and rotating the legs in each direction while holding the knees flexed to 90°. Passive rotation should be symmetric and have a total arc of about 100°. Internal rotation beyond about 60° indicates excessive femoral anteversion. It should be noted that similar to the flat foot, nothing beyond corrective osteotomy has been shown to change one’s rotational alignment. This is only indicated when the degree of in-toeing is such that it is causing tripping over one’s own feet such that injury or limitations have occurred. Mild in-toeing is actually favorable for patellofemoral mechanics and tends to be present in many higherlevel athletes. Bowed legs, or limbs with a varus tibiofemoral angle, are nearly universal at birth, but appear more so with the onset of standing upright and ambulating. Limbs will spontaneously realign to neutral just after age 2. Children then display increasing valgus angulation until about age 4 when the valgus begins to decrease to its final alignment at maturity, generally achieved by age 6–7 and averaging 5–7° of valgus. The assessment of excessive bowing or knock knees, includes examination of the femur and tibia for bowing within the long bones themselves, versus angulation at the joint surfaces (in the adult, nearly all the genu valgum results from angulation at the distal end of the femur at the condyles). Abnormal knee laxity is best assessed by stressing the knee into varus and valgus while in slight flexion of about 15–30°. Perhaps the most important finding indicative of pathology is observation of a varus thrust during ambulation. Pathologic bowing, or Blount’s disease, should be suspected when tibia vara fails to improve or worsens after age 2. Referral for orthopaedic evaluation by age 3 is important as intervention for true infantile tibia vara is more easily achieved when addressed prior to age 4. Adolescent Blount’s disease is a distinct entity that will be covered elsewhere. Lastly, part of the general assessment of all children relating to musculoskeletal health is assessment for nutrition, activity, and fracture risk. Children that are obese are at higher risk for fractures requiring surgery. Children that have had one fracture are at higher risk for a second fracture, and multiple fractures may indicate poor bone health and/or risky behavior. Caregivers should be asked about nutrition and achieving the recommended intake of calcium and vitamin D along with sunlight exposure. Vitamin D levels can be checked or supplementation started empirically. Caregivers should also be educated on playground safety along with activity-­specific recommendations. Playground equipment where a resulting fall would be more than

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1.5 m or onto a hard surface is considered unsafe [10]. Helmets and wrist guards should be worn when appropriate. The American Academy of Orthopaedic Surgeons has position statements on activities such as trampolining, where the recommendation include waiting until 6 years of age, having supervision, and limiting the number of children using a trampoline concomitantly [11]. Additionally, the frequently injured child should also prompt consideration of abuse in the form of neglect or non-accidental trauma. Certain fracture patterns such as transphyseal distal humerus, spiral humerus, femur under age 3, and any corner metaphyseal configurations should prompt automatic referral for further investigation.

4.4

Adolescent Exam

The assessment of the adolescent’s musculoskeletal system should consist of appropriate screening as well as a focused exam for particular complaints. Scoliosis screening is controversial, however if screening is to be done, recommendations are to perform it at ages 10 and 12 for girls (Year 5 and 7 generally), and age 13 or 14 for boys (Year 9 or 10). The goal is to recognize the curve prior to the adolescent growth spurt, which is when angulation is likely to progress most rapidly. Bracing may mitigate progression though this is highly dependent on brace compliance (variable in females and low in males). Inspection for shoulder asymmetry and coronal plane curve should be performed with the patient standing and viewed from the back. It is important to ensure the pelvis is level by palpating the iliac crests in order to rule out the contribution of any limb length discrepancy. If there is uncertainty, a supine exam with the legs fully extended can be checked for symmetric leg lengths as judged by heel position. If a 2 cm leg length discrepancy is noted, for example, the patient can then be examined with a 2  cm block under the shorter leg to level the pelvis for the spine exam. Families can also be reassured that limb length discrepancies under 2 cm tend not to be symptomatic and generally do not warrant treatment. Thoracic kyphosis can be deceiving, as patients can appear overly hunched and yet the majority of adolescent curves are hypokyphotic. The presence of spinopelvic balance, referring to whether their head is centered above their pelvis, is important for satisfaction with one’s appearance. A 30° S-shaped curve can have a subtle appearance due to a balancing compensatory curve, while a 10 ° C-shaped curve can involve significant clinical deformity as it will pitch the patient’s head and shoulder girdle to one side. Forward bend will reveal posterior rib prominence from the rotational component. Suspicion for scoliosis should prompt referral to an orthopaedic surgeon. Neurologic exam and radiographic imaging is warranted. Deep tendon reflexes, namely patellar tendon and Achilles, should elicit symmetrically brisk responses. The absence of sustained ankle clonus over 4 beats and presence of symmetric abdominal reflexes (movement of the umbilicus towards a light stroke moving outward to each quadrant) point away from underlying cord abnormalities. Nonetheless, advanced imaging with MRI will be indicated for any curve with atypical features to rule out neurologic involvement.

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Back pain is a frequent complaint. The PQRST pain qualifiers should be ascertained, as well as red flag symptoms discussed earlier. Additionally, loss of bowel or bladder control, should be specifically questioned. Examination should begin with inspection and palpation for any point tenderness, usually in areas of muscle spasm. Forward bend should be performed to assess hamstring tightness, which can be recorded as the distance from the fingertips to the toes with the knees fully extended. Back extension, lateral bend, and rotation should be assessed. Unilateral pain with extension combined with lateral bend is concerning for spondylolysis, as this position puts pressure on the pars (the part of the vertebra between the superior and inferior articular processes). Gymnasts and wrestlers who repetitively hyperextend their backs are at risk for this. Imaging confirmation of this, however, needs to be correlated clinically as 5% of the asymptomatic population will demonstrate a pars defect on imaging [12, 13]. Athletes require special consideration as they are at risk for a subset of conditions that may not manifest until later stages. Overuse injuries will manifest as insidious onset of pain that may worsen acutely. The musculoskeletal system can remodel in response to repetitive stresses, as seen with glenohumeral internal rotation defect (GIRD) from repetitive throwing in baseball players. With repetitive shoulder external rotation for the “wind-up” phase of throwing, the anterior shoulder capsule stretches, the posterior capsule contracts, and the humerus remodels into excessive external rotation in order to accommodate these stresses. Examination of the shoulder for passive range of motion will reveal this condition. Open growth plates are similarly vulnerable. Gymnasts and other athletes who repetitively load and hyperextend their wrists can suffer distal radial physeal stress syndrome, with widening and potential premature closure of the growth plate leading to ulnar overgrowth. These patients will present with physeal tenderness in the absence of fracture, or pain in the dorsoradial wrist that may mimic that of an occult ganglion cyst. Ulnar sided wrist pain is a more common complaint, especially amongst female adolescents. Patients should be examined for triangular fibrocartilage complex and extensor carpi ulnaris tenderness. Open physes also put patients in this age group at risk for apophysitis, or inflammation where a tendon inserts. One of the most common locations for this is the tibial tubercle, where jumping activities can lead to inflammation of the patellar tendon insertion on its growth center—Osgood-Schlatter’s Disease. Examination of the knee will reveal a prominent and tender tibial tubercle. If point tenderness is instead on the inferior patellar pole, this is termed Sinding-Larsen-Johansson Syndrome, or Jumper’s Knee. Anterior knee pain with findings of tenderness with patellar compression is more concerning for patellofemoral pain syndrome, especially in patients with an increased Q angle (subtended by the femoral axis from ASIS to patella, and along the patellar tendon to its insertion on the tubercle). The widening of the hips with pelvic maturation increases the lateral pull on the patella, which can change forces across the anterior knee. In addition to pain and inflammation associated with skeletal growth, rapid growth spurts put children at risk for contractures. While the physes are programmed to elongate, the soft tissues lengthen only in response to the stretch they receive from their increased excursion. Achilles tightness will often occur and can lead to knee and foot

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pain. This can be assessed for with the “runner’s stretch,” or seeing how much ankle dorsiflexion the patient can achieve when the foot is neutral to slightly internally rotated, preventing the midfoot break (Fig. 13). If they cannot achieve 10° of dorsiflexion, a stretching program may be appropriate. Patients with muscle weakness, such as CP or BPPB, will be especially at risk for contractures with growth, as their muscles will not receive the normal amount of stretching it takes to lengthen them. Thorough examination for increasing joint tightness should be undertaken, as serial casting may be needed intermittently to mitigate how contracted they are at skeletal maturity. Referral to orthopaedic specialists or therapists may be appropriate in the absence of examination findings in other high-risk patient populations. There is an increasing rate of anterior cruciate ligament injuries in teenage athletes, especially female soccer players, and neuromuscular/proprioceptive training has been shown to improve biomechanics [14]. Similarly, obese patients or those with a history of repetitive ankle sprains are at risk for additional ankle injuries, and therapy can decrease reinjury rates [15]. Females should be questioned about their diet and menses, as the female athlete triad (osteoporosis, anorexia, amenorrhea) can severely affect bone health at a time when females should be building up their bone mass.

Case Study 1 Terri

Terri was a fit and active 12-year old girl. Her mother noticed that her back was asymmetric when she was swimming on holiday. She took Terri to her GP who confirmed that Terri had scoliosis and noted a kyphotic deformity which became more pronounced on bending forward. She appeared to have minor leg length discrepancy. She stood with level shoulders and level pelvis. Lower limb reflexes were normal. The GP additionally noted 6 or 7 café-au-­ lait patches, and axillary freckling. She referred Terri to orthopaedics for management of scoliosis and to paediatrics for possible diagnosis of neurofibromatosis. At her orthopaedic assessment, radiological imaging showed a classical upper thoracic kyphoscoliosis with Cobb angles measuring 60 degrees’ scoliosis (T5-T9), and 76 degrees’ kyphosis (T4-T12). Right anteroposterior side bending views showed more than 50% correction of his deformity suggesting a relatively flexible curve. MRI scan showed the tip of the T8 rib-head had dislocated dorsally into the spinal canal, although there was no intraspinal anomaly such as dural ectasia, cord compression or myelomalacia changes however the spinal canal appeared to be very narrow at the apex centred at T7/T8. There was a neurofibroma at T5 in the concavity of the scoliosis measuring approximately 2.4 cm. Terri underwent surgery through a posterior approach. Instrumentation was performed from T1 to L1 and the neurofibroma excised. The surgery was uneventful. Post-operatively, she recovered well without any neurological deficit. Subsequently, the diagnosis of neurofibromatosis Type 1 was confirmed by genetic testing and she was referred to the regional neurofibromatosis clinic.

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Summary

The general assessment of the child’s musculoskeletal system is a combination of the neurologic and orthopaedic history and physical examination. The case of Terri, above, illustrates the importance of this approach. Awareness of common conditions for each age group is important, and the adage “the eyes only see what the mind knows” comes to mind. Knowledge of age-appropriate screening exams, such as performance of the infant hip exam until walking commences, and assessment for motor milestones and persistent primitive reflexes will catch most orthopaedic conditions in infancy. As children become more verbal and can offer subjective complaints to add to caregiver concerns, ruling out red flag symptoms and performing a focused exam of the involved area will guide management. Lastly, even in the absence of specific positive findings, a feeling of things being “not quite right” should prompt further action, whether it be close follow-up, a second opinion, referral to a specialist, or workup with imaging or lab studies. While the tincture of time does help most conditions, certain pathologies are time-sensitive, and even confirmation of lack of pathology will be worth the reassurance providers and caregivers alike.

References 1. Spiegel PG, Pekman WM, Rich BH, Versteeg CN, Nelson V, Dudnikov M. The orthopaedic aspects of the fetal alcohol syndrome. Clin Orthop Relat Res. 1979 Mar;139:58–63. 2. Herring JA. Tachdjian’s pediatric Orthopaedics. Elsevier Health Sciences. 2013:1. 3. Scharoun SM, Bryden PJ. Hand preference, performance abilities, and hand selection in children. Front Psychol. 2014;5:82. 4. Kang YR, Koo J.  Ultrasonography of the pediatric hip and spine. Ultrasonography 2017 Feb 22. 5. Golden NH. Abrams SA, COMMITTEE ON NUTRITION. Optimizing bone health in children and adolescents. Pediatrics. 2014 Oct 1;134(4):e1229–43. 6. Weinstein SL, Zavala DC, Ponseti IV. Idiopathic scoliosis: long-term follow-up and prognosis in untreated patients. J Bone Joint Surg Am. 1981 Jun;63(5):702–12. 7. Scolaro JA, Schenker ML, Yannascoli S, Baldwin K, Mehta S, Ahn J.  Cigarette smoking increases complications following fracture: a systematic review. J Bone Joint Surg Am. 2014 Apr 16;96(8):674–81. 8. Sinigaglia R, Gigante C, Bisinella G, Varotto S, Zanesco L, Turra S. Musculoskeletal manifestations in pediatric acute leukemia. J Pediatr Orthop. 2008 Jan;28(1):20–8. 9. Goldfarb CA, Patterson JMM, Sutter M, Krauss M, Steffen JA, MD LG. Elbow radiographic anatomy: measurement techniques and normative data. Journal of Shoulder and Elbow Surgery Elsevier Ltd. 2012 Sep 1;21(9):1236–46. 10. Howard AW.  The effect of safer play equipment on playground injury rates among school children. Can Med Assoc J. 2005 May 24;172(11):1443–6. 11. Julitz P. Trampolines and Trampoline Safety. 2015 Dec 10;1–2. 12. Hu SS, Tribus CB, Diab M, Ghanayem AJ. Spondylolisthesis and spondylolysis. Instr Course Lect. 2008;57:431–45. 13. Sousa T, Skaggs DL, Chan P, Yamaguchi KT, Borgella J, Lee C, et al. Benign natural history of Spondylolysis in adolescence with midterm follow-up. Spine Deformity. 2017 Mar;5(2):134–8. 14. Hewett TE, Ford KR, Xu YY, Khoury J, Myer GD. Effectiveness of neuromuscular training based on the neuromuscular risk profile. Am J Sports Med. 2017 Apr;1:363546517700128. 15. Van Reijen M, Vriend I, Zuidema V. The “strengthen your ankle” program to prevent recurrent injuries: a randomized controlled trial aimed at long-term effectiveness. J Sci 2016.

Normal Variants Tahani Al Ali, Jihad Saeed, and Sattar Alshryda

1

Background

Musculoskeletal development in children varies widely even at the same age (Fig. 1). These normal variations often cause parents to worry and seek medical advice. The followings conditions are common reasons for parents visits to primary healthcare and pediatric orthopaedic clinics. 1. Flat feet 2. In-toeing (Pigeon gait) 3. Bowed legs (Genu –varum) and Knock knees (Genu valgum)

2

Flexible Flat Feet (Pes Planus)

Issues with the appearance of the foot are the most common reasons prompting medical attention for musculoskeletal problems in children with 90% related to flat feet [1]. The feet of all neonates and toddlers look flat (Fig. 2) when they stand for several reasons: 1. Most tarsal bones are still cartilaginous (Fig. 3) 2. Young children have intrinsic laxity of ligaments and tissues 3. Neuromuscular control and muscle strength in children is still developing 4. Young children possess a fat pad beneath the medial longitudinal arch which bulges medially on standing T. Al Ali (*) · J. Saeed Al Jalila Children’s Speciality Hospital, Dubai, United Arab Emirates e-mail: [email protected]; [email protected] S. Alshryda Head of Trauma & Orthopaedics Surgery, Al Jalila Children’s Speciality Hospital, Dubai, United Arab Emirates e-mail: [email protected] © The Author(s), under exclusive license to Springer Nature Switzerland AG 2021 S. Alshryda et al. (eds.), Pediatric Orthopedics for Primary Healthcare, https://doi.org/10.1007/978-3-030-65214-2_4

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Fig. 2  Flatfoot on standing but the medial arch is visible on dangling the foot

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Fig. 3  Plain radiograph of an infant. Only two out of 7 tarsal bones are ossified and the rest are made of cartilage

All these lead to the flattening of the foot when weight bearing [2]. However, the arch is visible on dangling the leg free or standing on tip toes. Several studies examined normal arch development in children [3, 4]. Staheli and colleagues reported on the development of 442 normal children. They found that the longitudinal arch develops during the first 6 to 8 years of life with a wide range of normal variation [5]. About 45% 3–6  year-olds, and 15% of older children [6] exhibit developmental flexible flat feet. However, pathological flat feet may be a sign of underlying musculoskeletal disorders: 1. Tarsal Coalition 2. Congenital vertical talus

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3. Neuromuscular feet (as in Cerebral palsy and spina bifida) 4. Tibialis posterior dysfunction (most common in old people) 5. Accessory navicular bone (share some path-anatomical features of tibialis posterior dysfunction) 6. Trauma (including iatrogenic such as overcorrected club feet) 7. Tight gastrocnemius and soleus muscles and subsequent mid-foot break (becoming very common with increasingly sedentary life styles in children-see chap. 16: the foot and ankle) It is crucial to differentiate pathological flat foot. Three important signs (red flags) are considered when assessing flat feet in children: 1. Rigidity 2. Severity 3. Pain The developmental flexible flat foot, as the name implies is flexible, whereas flat feet from pathological causes are typically rigid, or inflexible and often painful. On standing, the normal child has a flexible flat foot with the heel in valgus and sagging

Fig. 4  Flexible flat feet. The heels move into varus (red solid arrow) and the medial arches (green dashed arrow) on tip toeing

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of the medial arch. However, on standing on tip-toes, the heel moves into a varus position and the medical arch is easily visible (Fig. 4). If the above can be demonstrated clinically, then the flat foot is flexible and it is reassuring; however, if the above does not happen, the flat foot is considered rigid. A rigid flat foot (38.5 °C), 2. Inability to bear weight, 3. ESR >40 mm/h, 4. WBC >12,000 /mm The predicted probability of septic arthritis in the presence of one predictor only was 3%, two predictors 40%, three predictors 93% and four predictors 99.6%. Kocher et al. validated his criteria prospectively in a later study and showed relatively similar findings [69]. Caird et al. noted that the level of CRP (>20 mg/l) was another independent predictor for septic arthritis of the hip [70]. Table 2 summarizes the three studies’ findings, though other authors have used these criteria and found the predictive value to be less reliable [71].

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Treatment and Pathology Management

6.1

Antibiotics

Antibiotics are critical in musculoskeletal infections. Antibiotic use is guided by clinical evaluation and culture results. However, initial therapy is guided by clinical experience and understanding of local patterns of antibiotic resistance. When infection is suspected, antibiotic therapy should not be withheld prior to obtaining cultures, since prior administration of antibiotics does not reduce culture yields from the site of infection [72, 73]. • Selection of antibiotic is dependent on both the suspected pathogen and patient factors. –– Neonates are at an increased risk of exposure to hospital acquired pathogens, such as MRSA, Candida, Enterobacteriaceae, or group B streptococcus [74–76]. –– For children under 5 years of age, treatment for Kingella kingae should be considered.

Table 2  Diagnostic criteria to differentiate between septic arthritis and transient synovitis Predictors Fever (>38.5 °C), Inability to bear weight, ESR >40 mm/h, WBC >12,000 /mm Fever (>38.5 °C), CRP (>20 mg/l)

No. of predictors 0 1 2 3 4 5

Predicted Probability of Septic Arthritis (%) Kocher 1999 Kocher 2004 Caird 2005 0.2 2 16.9 3 9.5 36.7 40 35 62.4 93.1 72.8 82.6 99.6 93 93.1 97.5

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The duration of antibiotics administered is dependent on the institutional experience, the patient’s response, as well as the tissue type affected. • Traditionally, 2–4 weeks of IV antibiotics are used for osteomyelitis, followed by oral antibiotics for a total of 6–8 weeks, however, shorter durations of IV antibiotics (e.g. until fever has resolved, exam is improving, and CRP has decreased by 50%), followed by 2–4  weeks of oral antibiotics [77] have been shown to be effective. • In cases of septic arthritis and pyomyositis, treatment regimens are shorter (e.g. 2–3 weeks of high-dose oral antibiotic after the initial intravenous antibiotics and surgical drainage).

6.2

Indications for Operative Management

In general, any patient who is physiologically unstable secondary to a musculoskeletal infection should be considered for urgent surgical debridement, or even amputation in cases of life-threatening infection such as necrotizing fasciitis [37]. From there, indications for surgery become less defined. In general, abscesses will not resolve on their own, thus drainage and debridement is required. Drainage and debridement can be done at the bedside or with a small radiographically guided procedure in some cases, however, when the infection lies deeper within tissues, behind vital structures, involves multiple tissues, or has formed more complex fluid collections, operative debridement is necessary [78]. Septic arthritis of large joints requires urgent surgical intervention within 24 hours to clear the infection, reduce the inflammation and prevent further cartilage damage.

6.3

Operative Techniques

Septic Arthritis: Septic arthritis is treated with irrigation and debridement through the most direct access to the joint. For example, the hip is drained through the anterior approach [79]. However, if the infection involves multiple tissues, the choice of approach should consider not only access to the joint space, but also access to the affected musculature or bone. Septic arthritis of larger joints can be accomplished arthroscopically with excellent results [80–82]. In most cases of septic arthritis, a drain is left in place and removed 2–3 days after surgery once inflammatory markers have decreased and the patient has improved clinically. Persistent clinical symptoms and elevated inflammatory markers after 48 hours suggests recurrence, inadequate debridement, adjacent infection or need for alternate antibiotic therapy. Osteomyelitis: Intra-osseous, sub-periosteal, or extra-periosteal infection usually requires aspiration of any visible abscess (usually under ultrasound guidance) and surgical debridement to debulk the infection and necrotic bone (Fig. 13). Pyomyositis: The first line of treatment is antibiotic administration and surgical intervention is based upon severity and response to treatment. Upon identification

Musculoskeletal Infection Fig. 13  Aspiration and Surgical treatment of sub-periosteal osteomyelitis. (a & b) MRI imaging of a sub-periosteal abscess. (c & d) Aspiration of soft tissue abscess prior to the initial incision, followed by (e-h) collection of intra-­ operative bone cultures and swabs

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of abscess formation and location by MRI, surgical debridement and irrigation is performed [83]. When the obturator musculature situated deep within the pelvis is infected, (> 60% of cases [32]), a medial approach is utilized to safely access the obturator musculature through either the adductor brevis or the obturator foramen.

6.4

Post-Operative Care

Once treatment has been initiated, serial measures of the APR (usually CRP and ESR) are the most clinically informative post-operative tests. A 50% decline in the maximum CRP may be used as an indicator for transition or oral antibiotics and discharge [84]. Patients may require physiotherapy treatment and mobility aid in the initial period.

Case Study 1

Connor, a previously fit and well five-year-old boy was seen by his general practitioner with a one-week history of high fever, resolving sore throat and a two-day history of a painful limp. It was becoming increasing painful, and he could only walk a few steps, with difficulty, and required a push chair. He was mildly febrile 37.9 °C, pulse 164/min, a respiratory rate of 25/min and normal oxygen saturation. Blood pressure was 124/68  mmHg. He had red eyes, flushed cheeks and a red inflamed throat. His left leg was passively abducted and externally rotated. Passive hip flexion and rotation was painful with a very limited range of movement. His GP referred Connor to the pediatric team at his local hospital. The history and physical findings were confirmed, and he had baseline investigations. His full blood count showed Hb 118  g/L, White cell count 26.4  ×  109/L, Neutrophils 25.6 × 109/L, Lymphocytes 0.5 × 109/L. Inflammatory markers were raised with a C-reactive protein of >250 mg/L and Erythrocyte sedimentation rate of 42 mm/hr. He was referred to Orthopaedics and an urgent hip ultrasound was performed which showed a significant left hip effusion. The hip was aspirated and washed out under general anesthesia. The resultant synovial fluid was sent for culture. He was subsequently commenced on intravenous benzylpenicillin and flucloxacillin. Over the next 24  hours, He remained febrile, with temperatures up to 38.5 °C. His case was discussed with the consultant microbiologist and antibiotics were changed to clindamycin and co-amoxiclav to cover group A beta haemolytic streptococcus infection. His throat swab subsequently grew scanty group A streptococcus, as did his blood culture and synovial fluid. In view of persistent elevation of inflammatory markers with fever, he underwent hip MRI on day 4, which showed

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diffuse marrow edema within the left femoral head and neck, as well as the acetabulum with residual joint effusion. However, there was no overt evidence of abscess or bone collection. After 5 days, his fever started to resolve and he made a gradual recovery, with resolution of his CRP and ESR. He remained on intravenous antibiotics for a total of 14 days before being discharged on oral antibiotics for a further 4 weeks.

7

Putting it all Together

Given the challenging nature of pediatric musculoskeletal infections and the potential for adverse medical and musculoskeletal outcomes, a thorough understanding of the epidemiology and optimal clinical evaluation practices are essential. As highlighted throughout, the acute phase response, if too exuberant may lead to adverse outcomes such a thrombosis and avascular necrosis, potentially leading to loss of joint function and abnormal limb development (Fig. 3). Hence, rapid diagnosis, appropriate antibiotics and, if required, surgical intervention are essential to ensure optimal recovery. Once treatment has been initiated, serial assessment of CRP & ESR is invaluable in monitoring treatment response and directing optimal care for the patient.

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10. O'Keefe RJ.  Fibrinolysis as a target to enhance fracture healing. N Engl J Med. 2015;373(18):1776–8. 11. Yuasa M, Mignemi NA, Nyman JS, Duvall CL, Schwartz HS, Okawa A, et al. Fibrinolysis is essential for fracture repair and prevention of heterotopic ossification. J Clin Invest. 2015;125(8):3117–31. 12. Benvenuti M, An T, Amaro E, Lovejoy S, Mencio G, Martus J, et al. Double-edged sword: musculoskeletal infection provoked acute phase response in children. Orthop Clin North Am. 2017;48(2):181–97. 13. Wang CL, Wang SM, Yang YJ, Tsai CH, Liu CC.  Septic arthritis in children: relationship of causative pathogens, complications, and outcome. J Microbiol Immunol Infect. 2003;36(1):41–6. 14. Blyth MJ, Kincaid R, Craigen MA, Bennet GC.  The changing epidemiology of acute and subacute haematogenous osteomyelitis in children. J Bone Joint Surg Br. 2001;83(1): 99–102. 15. Peltola H, Kallio MJ, Unkila-Kallio L.  Reduced incidence of septic arthritis in children by Haemophilus influenzae type-b vaccination. Implications for treatment. J Bone Joint Surg Br. 1998;80(3):471–3. 16. Conrad DA. Acute hematogenous osteomyelitis. Pediatr Rev. 2010;31(11):464–71. 17. Sonnen GM, Henry NK. Pediatric bone and joint infections. Diagnosis and antimicrobial management. Pediatr Clin N Am. 1996;43(4):933–47. 18. Kremers HM, Nwojo ME, Ransom JE, Wood-Wentz CM, Melton LJ, Huddleston PM, 3rd. Trends in the epidemiology of osteomyelitis: a population-based study, 1969 to 2009. J Bone Joint Surg Am 2015;97(10):837–845. 19. Karwowska A, Davies HD, Jadavji T. Epidemiology and outcome of osteomyelitis in the era of sequential intravenous-oral therapy. Pediatr Infect Dis J. 1998;17(11):1021–6. 20. Khachatourians AG, Patzakis MJ, Roidis N, Holtom PD. Laboratory monitoring in pediatric acute osteomyelitis and septic arthritis. Clin Orthop Relat Res. 2003;409:186–94. 21. Jackson MA, Nelson JD. Etiology and medical management of acute suppurative bone and joint infections in pediatric patients. J Pediatr Orthop. 1982;2(3):313–23. 22. Green NE, Edwards K.  Bone and joint infections in children. Orthop Clin North Am. 1987;18(4):555–76. 23. Saavedra-Lozano J, Mejias A, Ahmad N, Peromingo E, Ardura MI, Guillen S, et al. Changing trends in acute osteomyelitis in children: impact of methicillin-resistant Staphylococcus aureus infections. J Pediatr Orthop. 2008;28(5):569–75. 24. Sattar Alshryda JSH, Paul A.  Banaszkiewicz Paediatric Orthopaedics: An evidence-based approach to clinical questions. 1st ed. Springer International Publishing; 2016. 543 p. 25. Frank G, Mahoney HM, Eppes SC. Musculoskeletal infections in children. Pediatr Clin N Am. 2005;52(4):1083–106. ix 26. Street M, Puna R, Huang M, Crawford H.  Pediatric acute Hematogenous osteomyelitis. J Pediatr Orthop. 2015;35(6):634–9. 27. Yagupsky P, Erlich Y, Ariela S, Trefler R, Porat N. Outbreak of Kingella kingae skeletal system infections in children in daycare. Pediatr Infect Dis J. 2006;25(6):526–32. 28. Browne LP, Mason EO, Kaplan SL, Cassady CI, Krishnamurthy R, Guillerman RP. Optimal imaging strategy for community-acquired Staphylococcus aureus musculoskeletal infections in children. Pediatr Radiol. 2008;38(8):841–7. 29. Karmazyn B, Kleiman MB, Buckwalter K, Loder RT, Siddiqui A, Applegate KE. Acute pyomyositis of the pelvis: the spectrum of clinical presentations and MR findings. Pediatr Radiol. 2006;36(4):338–43. 30. Marin C, Sanchez-Alegre ML, Gallego C, Ruiz Y, Collado E, Garcia JA, et  al. Magnetic resonance imaging of osteoarticular infections in children. Curr Probl Diagn Radiol. 2004;33(2):43–59. 31. Theodorou SJ, Theodorou DJ, Resnick D. MR imaging findings of pyogenic bacterial myositis (pyomyositis) in patients with local muscle trauma: illustrative cases. Emerg Radiol. 2007;14(2):89–96.

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32. Mignemi ME, Menge TJ, Cole HA, Mencio GA, Martus JE, Lovejoy S, et al. Epidemiology, diagnosis, and treatment of pericapsular pyomyositis of the hip in children. J Pediatr Orthop. 2014;34(3):316–25. 33. Bureau NJ, Chhem RK, Cardinal É. Musculoskeletal infections: US manifestations. Radiographics. 1999;19(6):1585–92. 34. Ibrahim LF, Hopper SM, Donath S, Salvin B, Babl FE, Bryant PA. Development and validation of a cellulitis risk score: the Melbourne ASSET score. Pediatrics. 2019;143(2):e20181420. 35. Hysong A, Posey S, Blum D, Benvenuti M, Benvenuti T, Johnson S, et al. Necrotizing fasciitis: pillaging the acute phase response. J Bone Joint Surg Am. 2020. 36. McHenry CR, Brandt CP, Piotrowski JJ, Jacobs DG, Malangoni MA. Idiopathic necrotizing fasciitis: recognition, incidence, and outcome of therapy. Am Surg. 1994;60(7):490–4. 37. Lee A, May A, Obremskey WT. Necrotizing soft-tissue infections: An Orthopaedic emergency. J Am Acad Orthop Surg. 2019;27(5):e199–206. 38. Wong CH, Chang HC, Pasupathy S, Khin LW, Tan JL, Low CO.  Necrotizing fasciitis: clinical presentation, microbiology, and determinants of mortality. J Bone Joint Surg Am. 2003;85-A(8):1454–60. 39. McCarthy JJ, Dormans JP, Kozin SH, Pizzutillo PD. Musculoskeletal infections in children: basic treatment principles and recent advancements. Instr Course Lect. 2005;54:515–28. 40. Hankins CL, Southern S.  Factors that affect the clinical course of group a beta-haemolytic streptococcal infections of the hand and upper extremity: a retrospective study. Scand J Plast Reconstr Surg Hand Surg. 2008;42(3):153–7. 41. Bingol-Kologlu M, Yildiz RV, Alper B, Yagmurlu A, Ciftci E, Gokcora IH, et al. Necrotizing fasciitis in children: diagnostic and therapeutic aspects. J Pediatr Surg. 2007;42(11):1892–7. 42. Brook I.  Aerobic and anaerobic microbiology of necrotizing fasciitis in children. Pediatr Dermatol. 1996;13(4):281–4. 43. Eneli I, Davies HD.  Epidemiology and outcome of necrotizing fasciitis in children: an active surveillance study of the Canadian Paediatric surveillance program. J Pediatr. 2007;151(1):79–84. e1 44. Tang JS, Gold RH, Bassett LW, Seeger LL. Musculoskeletal infection of the extremities: evaluation with MR imaging. Radiology. 1988;166(1 Pt 1):205–9. 45. Kavanagh KT, Abusalem S, Calderon LE. The incidence of MRSA infections in the United States: is a more comprehensive tracking system needed? Antimicrob Resist Infect Control. 2017;6:34. 46. Dantes R, Mu Y, Belflower R, Aragon D, Dumyati G, Harrison LH, et al. National Burden of invasive methicillin-resistant Staphylococcus aureus infections, United States, 2011. JAMA Intern Med. 2013;173(21):1970–8. 47. Tom S, Galbraith JC, Valiquette L, Jacobsson G, Collignon P, Schøheyder HC, et al. Case fatality ratio and mortality rate trends of community-onset Staphylococcus aureus bacteraemia. Clin Microbiol Infect. 2014;20(10):O630–O2. 48. Rozgonyi F, Kocsis E, Kristóf K, Nagy K. Is MRSA more virulent than MSSA? Clin Microbiol Infect. 2007;13(9):843–5. 49. Peltola H, Vahvanen V. A comparative study of osteomyelitis and purulent arthritis with special reference to aetiology and recovery. Infection. 1984;12(2):75–9. 50. Nade S. Acute septic arthritis in infancy and childhood. J Bone Joint Surg Br. 1983;65(3):234–41. 51. Soto-Hall R, Johnson LH, Johnson RA.  Variations in the intra-articular pressure of the hip joint in injury and disease. A probable factor in avascular necrosis. J Bone Joint Surg Am. 1964;46:509–16. 52. Moore-Lotridge SN, Gibson BHY, Duvernay MT, Martus JE, Thomsen IP, Schoenecker JG. Pediatric musculoskeletal infection: an update through the four pillars of clinical care and immunothrombotic similarities with COVID-19. J POSNA. 2020;2(2). 53. Capitanio MA, Kirkpatrick JA.  Early roentgen observations in acute osteomyelitis. Am J Roentgenol Radium Therapy, Nucl Med. 1970;108(3):488–96. 54. Morley JJ, Kushner I.  Serum C-reactive protein levels in disease. Ann N Y Acad Sci. 1982;389(1):406–18.

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5 5. Gabay C, Kushner I. Acute-phase Proteins e LS 2001. 56. Gabay C, Kushner I. Acute-phase proteins and other systemic responses to inflammation. N Engl J Med. 1999;340(6):448–54. 57. Wirtz DC, Heller K-D, Miltner O, Zilkens K-W, Wolff JM. Interleukin-6: a potential inflammatory marker after total joint replacement. Int Orthop. 2000;24(4):194–6. 58. Oda S, Hirasawa H, Shiga H, Nakanishi K, K-i M, Nakamua M. Sequential measurement of IL-6 blood levels in patients with systemic inflammatory response syndrome (SIRS)/sepsis. Cytokine. 2005;29(4):169–75. 59. Pape H-C, van Griensven M, Rice J, Gänsslen A, Hildebrand F, Zech S, et al. Major secondary surgery in blunt trauma patients and perioperative cytokine liberation: determination of the clinical relevance of biochemical markers. J Trauma Acute Care Surg. 2001;50(6):989–1000. 60. Ridker PM, Rifai N, Stampfer MJ, Hennekens CH.  Plasma concentration of interleukin-6 and the risk of future myocardial infarction among apparently healthy men. Circulation. 2000;101(15):1767–72. 61. Markanday A, editor. Acute phase reactants in infections: evidence-based review and a guide for clinicians. Open forum infectious diseases: Oxford University Press; 2015. 62. Kamath S, Lip GYH.  Fibrinogen: biochemistry, epidemiology and determinants. QJM: An International Journal of Medicine. 2003;96(10):711–29. 63. Vigushin DM, Pepys MB, Hawkins PN. Metabolic and scintigraphic studies of radioiodinated human C-reactive protein in health and disease. J Clin Invest. 1993;91(4):1351–7. 64. Paakkonen M, Kallio MJ, Kallio PE, Peltola H.  Sensitivity of erythrocyte sedimentation rate and C-reactive protein in childhood bone and joint infections. Clin Orthop Relat Res. 2010;468(3):861–6. 65. Bottiger LE, Svedberg CA.  Normal erythrocyte sedimentation rate and age. Br Med J. 1967;2(5544):85–7. 66. Sox HC Jr, Liang MH. The erythrocyte sedimentation rate. Guidelines for rational use. Ann Intern Med. 1986;104(4):515–23. 67. Michail M, Jude E, Liaskos C, Karamagiolis S, Makrilakis K, Dimitroulis D, et al. The performance of serum inflammatory markers for the diagnosis and follow-up of patients with osteomyelitis. Int J Low Extrem Wounds. 2013;12(2):94–9. 68. Kocher MS, Zurakowski D, Kasser JR. Differentiating between septic arthritis and transient synovitis of the hip in children: an evidence-based clinical prediction algorithm. J Bone Joint Surg Am. 1999;81(12):1662–70. 69. Kocher MS, Mandiga R, Zurakowski D, Barnewolt C, Kasser JR. Validation of a clinical prediction rule for the differentiation between septic arthritis and transient synovitis of the hip in children. J Bone Joint Surg Am. 2004;86-A(8):1629–35. 70. Caird MS, Flynn JM, Leung YL, Millman JE, D'Italia JG, Dormans JP. Factors distinguishing septic arthritis from transient synovitis of the hip in children. A prospective study. J Bone Joint Surg Am. 2006;88(6):1251–7. 71. Luhmann SJ, Jones A, Schootman M, Gordon JE, Schoenecker PL, Luhmann JD. Differentiation between septic arthritis and transient synovitis of the hip in children with clinical prediction algorithms. J Bone Joint Surg Am. 2004;86-A(5):956–62. 72. Benvenuti MA, An TJ, Mignemi ME, Martus JE, Thomsen IP, Schoenecker JG. Effects of antibiotic timing on culture results and clinical outcomes in pediatric musculoskeletal infection. J Pediatr Orthop. 2016. 73. Zhorne DJ, Altobelli ME, Cruz AT. Impact of antibiotic pretreatment on bone biopsy yield for children with acute hematogenous osteomyelitis. Hosp Pediatr. 2015;5(6):337–41. 74. Frederiksen B, Christiansen P, Knudsen FU.  Acute osteomyelitis and septic arthritis in the neonate, risk factors and outcome. Eur J Pediatr. 1993;152(7):577–80. 75. Ish-Horowicz MR, McIntyre P, Nade S. Bone and joint infections caused by multiply resistant Staphylococcus aureus in a neonatal intensive care unit. Pediatr Infect Dis J. 1992;11(2):82–7. 76. Wong M, Isaacs D, Howman-Giles R, Uren R. Clinical and diagnostic features of osteomyelitis occurring in the first three months of life. Pediatr Infect Dis J. 1995;14(12):1047–53.

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77. Castellazzi L, Mantero M, Esposito S.  Update on the Management of Pediatric Acute Osteomyelitis and Septic Arthritis. Int J Mol Sci 2016;17(6). 78. Mignemi M, Copley L, Schoenecker J. Evidence-based treatment for musculoskeletal infection. Paediatric Orthopaedics: Springer; 2017. p. 403–18. 79. Souza Miyahara H, Helito CP, Oliva GB, Aita PC, Croci AT, Vicente JR.  Clinical and epidemiological characteristics of septic arthritis of the hip, 2006 to 2012, a seven-year review. Clinics (Sao Paulo). 2014;69(7):464–8. 80. Edmonds EW, Lin C, Farnsworth CL, Bomar JD, Upasani VV. A medial portal for hip arthroscopy in children with septic arthritis: a safety study. J Pediatr Orthop. 2018;38(10):527–31. 81. Nusem I, McAlister A. Arthroscopic lavage for the treatment of septic arthritis of the hip in children. Acta Orthop Belg. 2012;78(6):730–4. 82. Sanpera I, Raluy-Collado D, Sanpera-Iglesias J. Arthroscopy for hip septic arthritis in children. Orthop Traumatol Surg Res. 2016;102(1):87–9. 83. Spiegel DA, Meyer JS, Dormans JP, Flynn JM, Drummond DS. Pyomyositis in children and adolescents: report of 12 cases and review of the literature. J Pediatr Orthop. 1999;19(2):143–50. 84. Chou AC, Mahadev A. The use of C-reactive protein as a guide for transitioning to Oral antibiotics in pediatric Osteoarticular infections. J Pediatr Orthop. 2016;36(2):173–7.

Musculoskeletal Tumors Mohamed Ahmed Mashhour

1

Introduction

The word “tumor”, “mass”, “lump”, or “neoplasm” are all synonyms for the same diagnosis that causes severe anxiety to patients and their caregivers. In a world which is interconnected with advanced medical knowledge available to everyone, there are even greater expectations of health care professionals to provide a premium and advanced healthcare service that meets international standards. A proper understanding and awareness of common bone tumors and accurately differentiating the benign from the malignant is crucial. A physician who understands the risk factors in a simple benign lesion could prevent a pathological fracture from developing. Early detection of a malignant tumor could contribute significantly towards the preservation of a limb from amputation and enhance survival.

1.1

Definition of a Tumor

A tumor is defined as an abnormal mass of tissue that results when cells divide more than they should or do not die when they should. They can present in continuity with the tissue that are originated from or tumor cells can migrate and settle in various part of the body (Metastases). Tumors may be benign (not cancer), or malignant (cancer).

M. A. Mashhour (*) Department of Orthopedic Surgery, Faculty of Medicine, Ain Sham University, Orthopedic Oncology Unit, Cairo, Egypt Orthopedic Surgery, Mohammed Bin Rashid University of Medicine and Health Sciences (MBRU), Dubai, United Arab Emirates © The Author(s), under exclusive license to Springer Nature Switzerland AG 2021 S. Alshryda et al. (eds.), Pediatric Orthopedics for Primary Healthcare, https://doi.org/10.1007/978-3-030-65214-2_6

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Bone and Soft Tissue Tumors

Bone tumors are unique in 3 aspects: 1. Bones consist of several types of cells other than bone cells (See Table 1) 2. The high vascularity of bones renders them a common site for secondary metastasis from other tissues 3. The existence of bone tumor-like conditions (see Table 2)

1.3

Tumor Staging

Bone tumors are either benign or malignant. A staging system for bone tumors is essential to define the nature of the pathology, surgical guidelines, and determine the prognosis. It helps physicians to properly prioritize their patients and give those in need the appropriate care. The Enneking staging system for bone tumors, Table 3, is the widely accepted and used system for the staging of benign and malignant musculoskeletal neoplasms and is adopted by the Musculoskeletal Tumor Society [1].

Table 1  Musculoskeletal tumors and their cells of origin Cells Hematopoietic

Benign

Chondrogenic

Osteochondroma Chondroma Chondroblastoma Chondromyxoid fibroma

Osteogenic

Osteoid osteoma Osteoblastoma

Unknown origin

Giant cell tumor (fibrous) histiocytoma

Fibrogenic

Fibroma (Metaphyseal fibrous defect, non-ossifying fibroma) Desmoplastic fibroma (LG)

Notochordal Vascular

Hemangioma

Lipogenic Neurogenic

Lipoma Neurilemoma

Malignant Myeloma Lymphoma Primary chondrosarcoma (LG) Secondary chondrosarcoma (LG) Dedifferentiated chondrosarcoma (mixed) Mesenchymal chondrosarcoma Clear cell chondrosarcoma Osteosarcoma (HG) Parosteal osteosarcoma (LG) Periosteal osteosarcoma (IG) Ewing tumor (HG) Malignant giant cell tumor (HG) Adamantinoma (LG) Fibrosarcoma (HG) Malignant fibrous histiocytoma (HG) Chordoma Hemangioendothelioma (LG) Hemangiopericytoma Liposarcoma (Mixed)

LG low grade, IG intermediate garde, HG high grade and mixed = mixed of high and low grades

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Table 2  Bone tumor-like conditions Conditions 1. Eosinophilic granuloma 2. Osteomyelitis 3. Avulsion fractures 4. Aneurysmal bone cyst 5. Fibrous dysplasia 6. Osteofibrous dysplasia

Description Highly destructive lesion with a well-defined margin, cortex may be destroyed and there is soft tissue swelling. Self-limiting, steroid, radiotherapy, curettage and bone graft.

75% in 45°, or > 35° in severe cases. It is challenging due to bone fragility, and use of donor bone allograft rather than an iliac crest autograft may be required due to paucity of bone. Anterior Spinal Fusion is rarely indicated because of surgical complexity, severe blood loss and high rates of complications. Treatment of Basilar Invagination is required where this is a risk of cervical myelopathy, due to spinal cord impingement. Decompression and posterior fusion via a transoral approach is usually employed.

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Fig. 12  The use of telescopic rod (Fassier Duvall rod) in strengthening long bones in children with osteogensis imperfecta

References 1. Gómez-Alonso C.  Paediatric metabolic bone disease: a lifetime ahead. Adv Ther. 2020;37:38–46. 2. Jones M, Boon W, McInnes K, et al. Recognizing rare disorders: aromatase deficiency. Nat Rev Endocrinol. 2007;3:414–21. 3. Norman AW. The history of the discovery of vitamin D and its daughter steroid hormone. Ann Nutr Metab. 2012;61:199–206. 4. Pai B, Shaw N. Understanding rickets. Paediatr Child Health. 2016;25(7):325–31.

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5. International Society for Clinical Densitometry. Skeletal Health Assessment in Children From Infancy to Adolescence. 2019. 6. Sarraff V, Hogler W. Osteoporosis in children. Eur J Endocrinol. 2015;173:R185–97. 7. Bachrach LK, Catherine M, Gordon and Section on Endocrinology. Bone densitometry in children and adolescents. Pediatrics. 2016;138:e20162398. 8. Ralston SH, Gaston MS. Management of Osteogenesis Imperfecta. Front Endocrinol. 2020;

Neuromuscular Conditions Samena Chaudhry, Heather Read, and Sattar Alshryda

1

Introduction

The integrity of the musculoskeletal system is critical to function. Children cannot walk properly without an intact neuromuscular system. Joints sublux, bones twist and spines curve without proper support from healthy and strong muscles. Cerebral palsy (CP) is an umbrella term and constitutes a large proportion of neuromuscular practice by paediatric orthopaedic surgeons. The orthopaedic knowledge and skills gained from treating children with CP are used to treat other neuromuscular conditions, but it is important to understand the differences in management which relate to whether a condition is progressive (such as Duchenne muscular dystrophy) or non-progressive (such as cerebral palsy, or post-traumatic brain or spinal cord conditions).

S. Chaudhry (*) Royal Stoke University Hospital, Stoke-on-Trent, UK e-mail: [email protected] H. Read Royal Hospital for Children, Glasgow, UK e-mail: [email protected] S. Alshryda Head of Trauma & Orthopaedics Surgery, Al Jalila Children’s Speciality Hospital, Dubai, United Arab Emirates e-mail: [email protected] © The Author(s), under exclusive license to Springer Nature Switzerland AG 2021 S. Alshryda et al. (eds.), Pediatric Orthopedics for Primary Healthcare, https://doi.org/10.1007/978-3-030-65214-2_8

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Cerebral Palsy Case Study 1: Rosalind

Rosalind’s family moved to a new area and came to see her GP due to mild pain in her right hip and limping secondary to a short right lower limb. She had been born prematurely and had mild Cerebral Palsy (GMCFS II). She was of normal intelligence and attended mainstream school. Clinical examination showed that she had mild thoraco-lumbar scoliosis and wasted glutei and quadriceps on the right side. She had apparent shortening of the right leg by 2 centimetres. She walked with in-toeing of the right side. She stood with a pelvic tilt. There was a limited right hip abduction and 30° of fixed flexion deformity of the right side. Galeazzi test was positive. She was then examined while lying in a prone position. Duncan-Ely test was positive indicating a tight rectus femoris muscle on the right side. Internal rotation of the right hip was 75° whereas the external rotation was 25° in comparison to 60° and 40° of the left side respectively. Her medical history was complex. She was born preterm at 32 weeks’ gestation because of antepartum haemorrhage. She did not cry immediately and required some suction and stimulation before she started breathing. All investigations were satisfactory including hips ultrasound. She had delayed motor milestones and favoured her left side, and it was her orthopaedic surgeon who suggested that she had cerebral palsy. A neurologist subsequently confirmed she had mild right hemiplegic cerebral palsy. Thereafter, Rosalind was seen regularly in the Cerebral Palsy clinic and received regular physiotherapy. Eventually, she was able to start walking independently at the age of 2 years. Unfortunately, she then lapsed from follow-up until she was seen again aged 9 years with a painful limp which interfered with her daily activities. Radiographs showed a subluxed right hip with an underdeveloped acetabulum. She underwent hip surgery. Adductor longus and gracilis were short and were lengthened. A femoral varus and external derotation osteotomy was performed and the hip reduced. The acetabulum contained fibro-fatty tissue with otherwise healthy triradiate cartilage. Peri-acetabular osteotomy was performed to improve the joint stability and femoral head coverage. Postoperatively a hip spica was applied for 6  weeks. Unfortunately, she lapsed from follow-up again. Cerebral palsy is defined as a motor disorder which is due to a permanent, non-­ progressive injury to the developing brain which occurs before the age of 2 years [1, 2]. The brain damage is static but the clinical features change with growth [3–5]. Table 1 summarised the common causes of CP. The incidence of cerebral palsy is increasing slightly. Recent reports suggest that the incidence is between 2.4 and 2.7 per 1000 live births [6]. Peri-natal care advances including cooling may improve outcomes [7].

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Table 1  Causes of Cerebral Palsy Prenatal 1. Placental insufficiency 2. Toxaemia 3. Smoking 4. Alcohol 5. Drugs 6. Infection such as toxoplasmosis, rubella, CMV and herpes II (TORCH)

Perinatal 1. Prematurity (most common) 2. Anoxic injuries 3. Infections 4. Kernicterus 5. Erythroblastosis fetalis

Postnatal 1. Infection (meningitis, CMV, rubella) 2. Head trauma including non-accidental injury

The clinical manifestations of cerebral palsy depend on which size and location of the brain lesion. The range of clinical manifestations are wide extending from a young child who has normal intellect but may tip-toe walk, to a child who has epilepsy, is non-verbal and uses a wheelchair for mobility [8–10]. Cerebral palsy may be classified according to topographical (anatomical) distribution, clinical features and functional ability of the child [11].

2.1

Topographical (Anatomical) Classification (Fig. 1)

• • • •

Monoplegia (single limb) Hemiplegia (one side of body) Triplegia (three limbs—hemiplegia superimposed on diplegia) Diplegia (usually both lower limbs involved with only mild upper limb involvement) • Quadriplegia or ‘Total body involvement’

2.2

Clinical (Physiological) Classification

This classification is based on the affected muscles behaviour in response to the brain damage. The following types are recognised. • Spastic: increased muscle tone in response to limb movement. This is clinically evident when you ask an ambulant child to run. Spasticity is caused by damage to the motor cortex and pyramidal tracts damage and is the most common sub-­ type of CP • Dyskinetic: abnormal movements caused by damage to the extra-pyramidal system and basal ganglia. Several types have been described: –– Athetoid: slow writhing movements of the fingers, hands, and the rest of upper limb. The mouth and lower limbs may also be involved –– Ballismus and hemiballismus: Infrequent jerky, purposeless movements –– Chorea: Random movements of the limbs that increase during rest and may improve with movement –– Dystonia: Involuntary sustained muscle contraction that result in abnormal posture. The muscle tone fluctuates and often increases with effort and emotion

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DIPLEGIA

All four limbs are involved.

All four limbs are involved. Both legs are more severly affected than the arms.

HEMIPLEGIA

TRIPLEGIA

MONOPLEGIA

One side of the body is affected. The arm is usually more involved than the leg.

Three limbs are involved, usually both arms and a leg.

Only one limbs is affected, usually an arm.

Fig. 1  Anatomical classification of children with cerebral palsy

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Classification tree for sub-types of Cerebral Palsy Is there persisting increased muscle tone in one or more limbs? Y

N

Are both sides of the body involved?

Is the tone varying? N

Y

Spastic Bilateral

N

N

Y

Spastic Unilateral

Dyskinetic CP*

Non-classifiable

Is there generalised hypotonia with signs of ataxia? Y

Ataxic CP Reduced activity - tone tends to be increased

Dystonic CP*

N

Non-classifiable

Increased activity - tone tends to be decreased

Choreo-Athetotic CP*

SCPE Collaborative Group. Surveillance of cerabral palsy in Europe: a collaboration of cerebral palsy surveys and registers. Developmental Medicine and Child Neurology. 2004;24:816-24.

Fig. 2  Classification tree for sub-types of cerebral Palsy

• Ataxic: problems with balance and coordination caused by damage to the cerebellum • Mixed Surveillance of Cerebral Palsy in Europe (SCPE), which is a collaboration of professionals working with CP registers in Europe with the aim to research and monitor trends in CP, recommended a classification tree which is included in Fig. 2 [2].

2.3

Functional Classification

The most useful development in the classification of cerebral palsy in recent years has been the creation of the Gross Motor Function Classification System (GMFCS). The GMFCS is a five-level ordinal grading system based on the assessment of self-­ initiated movement with emphasis on function with regard to sitting and walking (Fig. 3). Distinctions between levels are based on functional limitations, the need for walking aids or wheeled mobility equipment, and quality of movement according to age [12–14]. In Fig. 4 the GM-FM 66 functional ability score measures for children are shown plotted against age for each of the GMFCS levels. Curves indicate that

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CMFCS E & R between 6th and 12th birthday: Descriptors and illustrations GMFCS Level I Children walk at home, school, outdoors and in the community. They can climb stairs without the use of a railing. Children perform gross motor skills such as running and jumping, but speed, balance and coordination are limited.

GMFCS Level II Children walk in most settings and climb stairs holding onto a railing. They may experience difficulty walking long distances and balancing on uneven terrain, inclines, in crowed areas or confined spaces. Children may walk with physical assistance, a handheld mobility device or used wheeled mobility over long distances. Children have minimal ability to perform gross motor skills such as running and jumping.

GMFCS Level III Children walk using a hand-held mobility device in most indoor settings. They may climb stairs holding onto a railing with supervision or assistance. Children use wheeled mobility when traveling long distances and may self-propel for shorter distances.

GMFCS Level IV Children use methods of mobility that require physical assistance or powered moblity in most settings. They may walk for short distances at home with physical assistance or use powered mobility or a body support walker when positioned. At school, outdoors and in the community children are transported in a manual wheelchair or use powered mobility.

GMFCS Level V Children are transported in a manual wheelchair in all settings. Children are limited in their ability to maintain antigravity head and trunk postures and control leg and arm movements.

GMFCS descriptors: Palisano et al. (1997) Dev Med Child Neurol 39:214-23 CanChild: www.canchild.ca

Illustrations Version 2 © Bill Reid, Kate Willoughby, Adrienne Harvey and Kerr Graham, The Royal Children’s Hospital Melbourne ECR151050

Fig. 3  The Gross Motor Function Classification System (GMFCS) for children with cerebral palsy aged 6-12 years. Courtesy of Kerr Graham, The Royal Children’s Hospital, Melbourne

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Fig. 4  Gross Motor Development curves using GM-FM 66 scores plotted against age

motor ability peaks earlier for children of higher GMFCS Level e.g. age 3 for GMFCS 5 and age 7 for GMFCS 1 [13]. Children’s motor skills improve from birth but delayed motor milestones prompt Brain MR imaging to establish a diagnosis of cerebral palsy (Fig. 4).

2.4

Pathology

Cerebral palsy is caused by damage to the developing brain. There is debate as to when the human brain stops developing, but experts agree that the most critical period of brain development occurs in the first 2 years of life. Pathological changes consequent to the damage are described as primary, secondary or tertiary.

2.4.1 Primary Pathological Changes These changes are directly related to the loss of function of the damaged part of the brain. 1. Intellectual impairment 2. Epilepsy 3. Visual problems 4. Hearing loss

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5. Speech and communication problems 6. Swallowing difficulty 7. Feeding difficulty, failure to thrive 8. Respiratory problems 9. Incontinence 10. Neuromuscular problems: (a) Abnormal muscle tone (b) Balance problems (c) Loss of selective control (d) Pathological reflexes or persistence of primitive infantile reflexes (e) Loss of sensation

2.4.2 Secondary Pathological Changes 1. Indirect consequences of the primary pathological changes. For example, a child with learning difficulty and or visual impairment would be expected to take longer to learn how to sit or walk. 2. The interaction between growth and affected muscles. Children get taller by the longitudinal growth of their bones. Long bones have two growth centres; proximal and distal. Menelaus’s “Rule of thumb” predicts proximal femoral growth of approximately 3  mm per year whereas the distal grows10  mm per year. The proximal tibial growth centre grows 6 mm per year and the distal tibial growth centre grows 5 mm per year [15] (Fig. 5). Bones grow first and muscles follow when they stretch during play. In children with CP this normal physiological process is altered. Impairment of movement and motor control is associated with a mismatch between muscles length and bone length. Bi-articular muscles such as hamstrings, gracilis and gastrocnemius which cross more than one joint also cross multiple growth plates and are at a particular risk of becoming shortened. Altered muscle length combined with weakness, spasticity and altered motor control leads to the development of contractures, bone deformity, joint subluxation and scoliosis. A pattern of muscle dysfunction in diplegic CP in sagittal plane gait where the dominant muscles affected are described to target management [16]: • Type 1—True equinus—Gastrocnemius muscle spasticity is present causing plantarflexion at ankle and may progress to contracture Type 2—Jump knee— Proximal muscle spasticity in hamstrings and hip flexors causes flexed knee and flexed hip gait (in combination with equinus at ankle). • Type 3—Apparent equinus—Spasticity in hip flexors and hamstrings causes flexed hip and knee and foot lever remains competent maintaining plantigrade position under load (but as heel is off ground may appear at first glance to resemble equinus).Calf lengthening contra-indicated as would produce rapid descent into crouch. • Type 4—Crouch—Excessive Dorsiflexion at ankle in combination with hamstring and hip flexor spasticity and/or contracture. Spasticity or contracture of the Rectus femorii may be present and cause stiff knees (Fig. 6).

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179 3 mm

10 mm

6 mm

Hamstring muscles

gastrocnemius muscle

5 mm

Soleus muscle

Fig. 5  Menelaus “Rule of thumb” prediction of annual physeal bone growth in lower limbs in children

Mercer Rang coined the name Birthday Syndrome when children with ambulatory CP underwent cautious single operations annually to correct deformities as the results of surgery were unpredictable. Gait improvement surgery has been assisted by gait analysis technology and improved understanding of muscle pathophysiology [17]. Hip displacement is one of the most common joint displacements in children with CP (Fig. 7). It is usually attributed to spasticity and contracture of the hip adductors and flexors as well as the medial hamstrings [18]. In the presence of growth and abnormal hip posture in adduction, flexion and internal rotation the acetabular margin can erode creating a channel that is directed postero-superiorly. The triradiate cartilage widens the ‘teardrop’ appearance on radiographs [19]. Subluxation develops progressively, usually in a posterior direction with increasing lateralization and proximal migration of the femoral head. Altered forces on

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Fig. 6  Sagittal gait patterns in spastic diplegia

Fig. 7  Hip dislocation in a child with cerebral palsy

the growing femoral epiphysis result in a valgus femoral neck shaft angle developing. The head-shaft angle has also been substantiated as a predictive factor in hip displacement in children of GMFCS 3-5 levels [20]. The bony skeleton responds during growth as it is subject to limb and trunk posture, muscle length and spasticity.

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Fig. 8  Progressive loss of femoral head sphericity and operative findings of deep femoral head notching

If hip displacement occurs radiological measurements of acetabular angle and centre edge angle decrease but Reimer’s index is the most frequently used method of Orthopaedic measurement. Reimer’s index is the portion of the ossified femoral head lying lateral to Perkins line as a percentage of the total width of the ossified femoral head (see below). The femoral head shape may change, with notching presumably as a result of repeated impact against adjacent structures including the capsule, acetabular rim, abductors, and ligamentum teres (Fig. 8). Abnormal, sustained posture of the hip is implicated in the development of pelvic obliquity and subsequent scoliosis though it is sometimes difficult to ascertain the order in which these co-existent deformities occurred. The reported incidence of hip displacement in children with cerebral palsy is correlated with the severity of involvement and the ambulatory status. Children with mild involvement and who walk independently are considered to have a low incidence of hip displacement though there is a subset of hemiplegic children with hip involvement who justify more frequent hip surveillance than those with diplegia

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100 89.7

Incidence Hip Displacement (%)

90 80 69.2

70 60 50

41.3

40 30 15.1

20 0

10 0 I

II

III GMFCS levels

IV

V

Fig. 9  Incidence of hip displacement (a migration percentage of >30%) according to the Gross Motor Function Classification System (GMFCS) level

functioning at GMFCS 1/2 level. Children with more severe involvement and who are unable to walk have the greatest risk of hip displacement (GMFCS Level V) (Fig. 9) [21]. Bone and joint deformities in the foot occur frequently in children with CP.  The talonavicular joint is particularly at risk (Fig.  10, image 2). A short gastrocnemius-­soleus musculo-tendinous unit results in either toe-walking or knee hyperextension (the plantar-flexion extension couple). Joints in the feet of growing children subjected to non-physiological forces may sublux and bony modelling can occur. Changes in foot biomechanics in diplegia often result in valgus hindfoot posture, forefoot over-pronation with or without hallux valgus deformity (Fig. 10, image 1). Limbs as Levers Limbs function as levers. A joint forms the axis (or fulcrum), and the muscles crossing the joint apply the required force to produce movement or resistance. Levers are typically labelled as first class, second class, or third class. All three types are found in the body. Using the ankle joint as an example, the longer the foot, the more efficient the lever system (Fig. 11). However, maltorsion talonavicular subluxation and/or hallux valgus shorten the foot leading to biomechanical disadvantage at foot push off. Similar examples occur around the knee (Fig. 12). Lever arm syndrome is the name given to these anatomical changes which result in an inefficient anatomical lever arm system [17].

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Fig. 10  Foot deformity in children with Cerebral Palsy. Image 1 is a plain radiograph of a child with a hallux valgus deformity (curved red arrow) but normal talonavicular joint (straight green arrow). Image 2 shows a talonavicular joint subluxation (red straight arrow) and normal big toe alignment

2.4.3 Compensatory Mechanisms In the presence of a primary motor disorder characterised by spasticity, secondary changes in muscle length and bony alignment occur. Tertiary coping or compensatory strategies may be used unconsciously by a child with cerebral palsy to assist walking. For example children with a knee flexion contracture may walk on their toes even when they do not have any tendo-achilles contracture. Historically unnecessary tendoachilles lengthening led to iatrogenic crouch. Gait analysis and interpretation by an experienced multi-disciplinary team can assist in differentiating between secondary changes which may be amenable to intervention and and tertiary changes which may spontaneously revert to normal if the underlying pathology is improved .

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Body

Body

Resistance

Axis

Force

Force

Resistance

Axis

Fig. 11  The ankle joint represents a second-class lever, the weight (resistance) is located between the axis (fulcrum—the ball of the foot) and the force (the gastro soleus system)

3

Diagnosis and Evaluation

Most children with CP are diagnosed before they reach the paediatric orthopaedic surgeon; however, children with mild motor delay or gait difficulties may present to the primary healthcare or the paediatric orthopaedic surgeon. Early signs suggestive of CP in the infant are abnormal behaviour, oro-motor problems, or poor motor development. Establishing the diagnosis is important for subsequent management, particularly differentiating progressive from non-progressive neurological problems. Early involvement of paediatric neurologist and neurorehabilitation team is important for diagnosis, management, and appropriate treatment. Orthopaedic evaluation consists of the following:

3.1

History

1 . Birth & developmental history 2. Co-morbidities 3. Generate list of Orthopaedic Problems

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40 kg

40 kg

40 kg

5cm 5cm

40 kg

40 kg

120 kg

40 kg

0 cm

40 kg

5cm 5cm

15cm

Fig. 12  The effect of knee flexion contracture on the required force to stand and walk

3.2

Examination

Gait assessment (In ambulatory children). Static Physical examination of every joint (See Fig. 13–in most well-established units this is done by physiotherapist with special interest in CP) 1. Muscle tone/spasticity 2. Muscle length: Joint range of motion 3. Lever Arm: Bone alignment & Torsional profile 4. Selective control 5. Muscle strength

3.3

Investigations

1. Radiographs—Routine hip surveillance is included in NICE Guidelines on Spasticity in under 19 years. (Ref CG145) 2. Gait Analysis

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Set Realistic Goals Sixty-five percent of children with CP are ambulatory and the goals of surgical intervention include: 1. Optimization of gait efficiency (a) Optimizing energy conservation (b) Preserving or improving physical function (c) Maintaining or improving physical activities 2. Gait cosmesis: Improved appearance of gait 3. Pain relief In children who are non-ambulatory, the goals of surgical interventions are different: 1 . Preventing or relieving pain/discomfort 2. Facilitating caregiving 3. Preserving or improving quality of life

4

Management

Management of children with neuromuscular conditions in general requires a multidisciplinary approach involving: 1. Neurorehabilitation team (a) Community and hospital physiotherapists (b) Orthotists (c) Occupational therapist 2. The general practitioner 3. Neurologist 4. Orthopaedic surgeon 5. Gastroenterology team / Dietician team 6. Neurosurgery team The primary lesion in CP is static brain damage; however, the musculoskeletal problems evolve because of the interaction between growth and muscle abnormalities. The mainstay of treatment is optimising muscle function. There are six essential lines of treatment: 1. Physiotherapy 2. Orthoses 3. Assistive devices 4. Serial casting 5. Treatment of spasticity 6. Surgery

Neuromuscular Conditions Joint

R(R1)

L(R1)

Coronal

R

L

Transverse

R

L

Ankle Dorsiflexion (Knee 0)

15

-10(0)

Inversion

F

F

HBL

2IDS

2IDS

Dorsiflexion (Knee90)

20

-5(5)

Eversion

F

F

FTA

15E

15E

Plantarflexion

F

F

Midfoot break

No

Y

TMTA

NT

NT

Plantaris

No

No

Hallux valgus

No

No

Extension

F

F

Flexion

F

F

Knee

Sagittal

1 2 35°

25 ° before skeletal maturity (b) >50  ° in the thoracic spine or 40  ° in the lumbar spine (progress 1–2  ° per annum) 2. Immaturity (see above) 3. Female gender 4. Type of curve (thoracic more likely to progress than lumbar, and double curves more likely to progress than single curves There are 3 treatment options to treat scoliosis in general and AIS in particular. These are: 1. Observation (often combined with physiotherapy). 2. Bracing using orthoses 3. Corrective surgery Table 2 summarises the general guidelines of AIS treatments. Observation Observation with or without physiotherapy is indicated when Cobb’s angle is less than 25 °. Six-monthly x-rays are needed to monitor progress. The inter-­observer and intra-observer errors of Cobb’s angle measurement is 3–5  °. Therefore, an increase of more than 5 ° in Cobb’s angle is considered significant. The role of physiotherapy in treating scoliosis has been debated. The supporting evidence is weak; however, it is viewed as desirable by parents and patients.

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Fig. 12  The use of bracing to treat scoliosis

Bracing Bracing is indicated when the Cobb’s angle is from 25 ° to 45 °. It is only effective for flexible deformity in skeletally immature patient who are still growing. The main aim is to stop or slow progression, not to correct deformity. Braces can be rigid or flexible (dynamic). Rigid braces work by opposing the deforming forces through direct pressure on the trunk. Discomfort, pressure sore and muscle weakness are some of the undesirable effect. Spine x-ray with and without the brace must demonstrate more than 50% correction for a brace to work. Dynamic braces slow curve progression by retraining body into a corrective posture. The name of the braces indicates the size and shape of the brace. The two most commonly used braces are: 1. TLSO (Thoracic-Lumbar-Sacral Orthosis) such as the Boston brace and the Wilmington brace (Fig. 12). 2. CTLSO (Cervical- Thoracic-Lumbar-Sacral Orthosis) such as Milwaukee brace Several studies have shown various success rates. A 50% reduction in need for surgery with compliant brace wear of at least 13 h a day has been reported [6]. A lower chance of curve control with bracing has been shown with: 1. Curves greater than 40 ° 2. Correction of less than 50% in brace 3. Poor compliance or tolerance (brace worn fewer than 16 h per day) 4. Male gender. 5. Hypokyphosis (relative contraindication) 6. Obesity

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Surgery The essence of surgical treatment of scoliosis is fusion. The curved vertebrae are fused together so that they heal into a single, solid bone. This can be done by fusing the posterior vertebral parts (spinous process, laminae, transverse process and adjacent facet joints). This is called posterior spinal fusion (PSF). Fusing the vertebral bodies is called anterior spinal fusion (ASF). In certain scoliosis, a combined (anterior and posterior spinal fusion is required). Non-fusion scoliosis correction has been introduced in the last decade and is still being evaluated. Complications of surgery include: 1. Neurologic injury (incidence of paraplegia is 1:1000) 2. Pseudoarthrosis (failure of fusion) in (1–2%) 3. Infection (1–2%) 4. Crankshaft phenomenon (rotational deformity of the spine caused by continued anterior spinal growth after PSF. This usually occurs in very young patients when PSF is performed alone and the anterior column is allowed continued growth 5. Superior mesenteric artery (SMA) syndrome when third part of duodenum is compressed due to narrowing of the space between SMA and aorta 6. Hardware failure such metal rods breakage or bending and screws pulling out. These usually happens when the bone fusion is not complete creating an area of movement between two fused segments. This area is often referred to as pseudoarthrosis.

1.3

Neuromuscular Scoliosis

Strong muscles are important to maintain normal spinal posture. Weakness that is caused by neuromuscular diseases, such as cerebral palsy, may cause neuromuscular scoliosis. Generally neuromuscular scoliosis presents as a long curve with more vertebrae involved. It has a high risk of progression even after maturity, and is associated with pelvic obliquity (Fig. 13). Patients often have associated co-morbidities such as poor nutritional status, seizures, hydrocephalies, poor lung function and impaired immunity. Complication rates are higher. See Table 3 for a summary of the types of neuromuscular scoliosis and treatments.

1.4

Syndromic (Dystrophic) Scoliosis

When scoliosis presents as part of a syndrome, the common syndromes associated with scoliosis are 1. Marfan syndrome 2. Neurofibromatosis (NF) 3. Ehlers-Danlos syndrome 4. Osteogensis imperfecta Scoliosis is the most common skeletal manifestation in Neurofibromatosis, which is divided into 2 types 1. Non-dystrophic scoliosis which behaves like idiopathic scoliosis in curve patterns and progression

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Paediatric Spine Fig. 13  Neuromuscular scoliosis in a child with cerebral palsy. A long curve with substantial pelvic obliquity

Table 3  Classification of neuromuscular scoliosis Underlying causes Brain Spinal cord Muscular Muscles

Examples Cerebral palsy Rett syndrome Spinal muscular atrophy Poliomyelitis Spina bifida Spinal cord injuries Muscular dystrophies such as Duchene muscular dystrophy

Treatments Bracing until puberty (age 10–12) and/or wheelchair modification Surgery is indicated if curve becomes >50 ° or worsening pelvic obliquity interfering with sitting Bracing may be harmful in these conditions and surgery is indicated. In muscular dystrophy. Surgery should be considered before pulmonary function declines (curve from 20 to 30 °)

2. Dystrophic scoliosis (more common), usually has short thoracic sharply angled curve, with a greater tendency to progress, and presents a risk for developing neurological deficits (Fig. 14). Surgical correction is indicated.

1.5

Miscellaneous Types of Scoliosis

There are several other causes of scoliosis such as: 1. Degenerative scoliosis. This develops in adults due to degenerative changes in the spine. It develops gradually causing mainly lumbar scoliosis with a short curve.

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Fig. 14  Dystrophic scoliosis in a child with neurofibromatosis

2. Traumatic vertebral fracture including osteoporotic fractures. It is more common in elderly; however, it can happen in children with some intrinsic bone disease such as osteogenesis imperfecta. 3. Infective. 4. Bony Neoplasms such as osteoid osteoma or osteoblastoma. Investigations and treatments are directed toward the underlying condition.

2

Kyphosis

The word kyphosis is derived from the Greek kyphosis (humpbacked). However, it has also been widely used to describe the normal shape of the vertebral column in the sagittal plane. The vertebral column has a slight cervical lordosis, a thoracic kyphosis (20–50 °- apex between T5–8), a lumbar lordosis (20–60 °- apex L3) and a sacro-coccygeal kyphosis. This indiscriminate use of the terms kyphosis and lordosis commonly causes confusion. Kyphosis is considered abnormal when it exceeds the above values. However, to be considered as a disease that require interventions, other clinical and radiological

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features have to be fulfilled. The thoracic spine is the most common place for kyphosis. Like scoliosis, several types of kyphosis have been described: 1. Congenital 2. Developmental 3. Neuromuscular 4. Syndromic 5. Traumatic 6. Degenerative

2.1

Congenital Kyphosis

Congenital anomalies of the spine can cause scoliosis, kyphosis or both. As in scoliosis, this can be divided into: Type 1-Failure of formation Type 2-Failure of segmentation Type 3-Mixed abnormalities A wedge-shaped vertebra that is positioned posteriorly deforms the spine in a kyphotic direction (Fig. 15). This is likely to progress further with the growth of the abnormal vertebra. As kyphosis worsens, the centre of gravity of the body inexorably moves forward increasing the load on the anterior aspect of the other normal vertebrae. This impedes vertebral anterior growth and accelerates the deformity progression further [2]. Progression is highly likely in the first year of life because of rapid growth and there is the potential for spinal cord compression. Failure of segmentation deformity

Fig. 15  Congenital kyphosis secondary to posterior hemivertebra

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has a slower rate of worsening and may not become a surgical curve until adolescence. With increasing deformity, the spinal cord may become tented over the angular deformity and lead to neurological problems such as pain, numbness, difficulty walking or bowel and bladder emptying problems. Management of congenital kyphosis is not very different to congenital scoliosis. Other associated anomalies must be ruled out. Observation with serial examination and x-rays to monitor progress is required initially and may be all what is required. Casting and/or bracing is often used to delay progression but clear evidence of effectiveness is lacking. Surgery is required to deal with progressive congenital kyphosis. This could involve correction of deformity then fusion or simply in-situ fusion (with little or no correction of the spine) [7].

2.2

Developmental Kyphosis

Two types of developmental kyphosis have been described: postural and structural. Postural kyphosis is related to posture. It is flexible and will correct on standing. X-ray is normal with no vertebral abnormalities. Some patients may have tight hamstring muscles pulling the pelvis backward on sitting. This forces them to stoop forward to sit comfortably. The main core treatment is parent and patient education. A series of muscle strengthening and stretching exercises for the trunk, abdomen, shoulder girdle, and lower extremities is useful. Orthotics may be used for extreme cosmetic deformity. Surgery is not normally required [8]. Structural kyphosis arises from Scheuermann’s disease. This was first described by Holger Werfel Scheuermann, a Danish surgeon in 1920. He described a specific type of fixed angular kyphosis with anterior wedging of the vertebral bodies and irregularities of the vertebral apophyses. Although the deformity was initially described only for the thoracic spine, it also can occur in the thoracolumbar and lumbar spine. Scheuermann’s disease present with typical radiographic features: 1. More than 5  ° of anterior wedging of three consecutive adjacent vertebral bodies at the apex of the kyphosis 2. Irregular vertebral apophyseal lines combined with flattening and wedging 3. Narrowing of the intervertebral disk spaces 4. Schmorl  nodes are variably present. Schmorl nodes are intravertebral herniations of the disc cartilage, which protrude through the vertebral body endplate and into the adjacent vertebra, potentially leading to inflammation Mild Scheuermann’s disease (kyphosis 75  ° (Long-term natural history studies have shown that pain and function levels are not affected if the curve is below 75 ° [9].) 2. neurological deficit 3. spinal cord compression 4. severe pain in adults (Fig. 16)

2.3

Neuromuscular Kyphosis

Aetiology, pathology, presentation, progression and management of neuromuscular kyphosis is very similar to the neuromuscular scoliosis and they often co-­exist. Physiotherapy, bracing, wheelchair modification and surgery are the main-stay treatments. These patients should be managed by a multidisciplinary team involving

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neurorehabilitation team (physicians, physiotherapist, orthotist, occupational therapist), orthopaedic and spinal surgeons [8, 10].

2.4

Syndromic Kyphosis

Kyphosis can be a feature of a number of syndromes such as mucopolysaccharidoses. Investigations and treatments are directed toward the underlying condition. Each patient is assessed and managed on individual bases. The mainstays of treatments are the same but their feasibility and success rates vary depending on the cause.

3

Spondylolysis/Spondylolisthesis

Spondylolysis arises from a defect in the pars interarticularis, a small segment of bone that joins the posterior facet joints, which may cause one of the vertebrae to slip forward onto the bone directly beneath it - spondylolisthesis. It may lead to a deformity of the spine, narrowing of the spinal canal (central spinal stenosis), or nerve root compression (foraminal stenosis). Spondylolisthesis is most common in the low back (lumbar spine) but can also occur in the thoracic or cervical. The commonest cause is a stress fracture resulting from repetitive trauma. Sporting activities involving lumbar spine extension and twisting such as tennis, badminton, gymnastics, and American football. Spondylolisthesis may lead to spinal instability (Fig. 17). Spondylolisthesis is classified according to its aetiology into 6 types. These are summarised in Table 4 [11]. The usual presenting symptoms are persistent pain and stiffness, aggravated by activity and improved by rest. Meyerding graded spondylolisthesis into 5 grades by dividing the superior endplate of S1 into quarters and observing how far the postero-inferior corner of the L5 vertebral body slips forward on S1 (Fig. 18) [12]. Plain x-ray shows spondylolysis in only 40–60%. CT imaging is diagnostic and can show the spondylolysis and any spondylolisthesis in the supine position. Sagittal reconstructions are the best images to view. MRI scan can show any nerve root compression and any degenerative disc changes. Treatment is mainly symptomatic when the slip is less than 50%. Analgesia, physiotherapy, activity modification and rarely a short period of bracing are required. if pain continues without slippage, the pars fracture can be repaired without fusing the vertebrae together. When the slippage is more than 50%, the risk of further slippage is high and this merits vertebral fusion.

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Pars Interarticularis

Spondylolysis

Spondylolisthesis

Fig. 17 Spondylolysis/Spondylolisthesis Table 4  Spondylolisthesis classification Age (years) Pathology/Other Type I—Dysplastic Child Congenital dysplasia of S1 superior facet II—Isthmic 5–50 Predisposition leading to elongation/fracture of pars (L5-S1) III—Degenerative >40 Facet arthrosis leading to subluxation (L4-L5) IV—Traumatic Any age Acute fracture other than pars V—Pathological Any age Incompetence of bony elements VI—Postsurgical Adult Excessive resection of neural arches/facets

Normal spine

Grade 1 75%

Fig. 18  Meyerding grading of spondylolisthesis. Grade 1   100% slip (also called spondyloptosis - not in the original description)

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Infection Case Study 2 Jacob

Jacob, a 3-year-old boy, presented with irritability, apparent back pain, limp, and refusal to walk. After his symptoms failed to respond to simple analgesia, his parents took him to his GP. He was previously well, with no medical history of note. On examination, he was afebrile 36.7 °C. Jacob was crying and uncooperative, and apparently in pain. He refused to bend forward and was uncomfortable sitting, preferring to lie prone on the floor. The GP thought he was tender over the lumbar spine. He had reduced hip flexion, screaming in distress, when this was attempted. Neurological exam was very difficult as he was uncooperative. He was referred urgently to paediatrics at his local hospital. He had a slightly raised white cell count but ESR was normal. X-rays of the lumbar spine and pelvis were reported as normal. Blood culture was negative. Attempts to mobilise him through play and physical therapy were unsuccessful and he was referred to a paediatric rheumatologist for exclusion of juvenile idiopathic arthritis. Through use of play therapy and analgesia, the rheumatologist was able to conduct a full examination and noted exquisite tenderness over the L2–3 intervertabral disc. In hindsight, his spinal X-rays showed slight narrowing of the L2/3 intervertebral disc space. He arranged an urgent MRI, which showed hyperintensity of the L2/3 disc on T2-weighted images and contrast enhancement of the disc and adjacent bone marrow, with a very small epidural abscess, confirming the diagnosis of discitis. He was referred to orthopaedics for ongoing management. He was initially treated with intravenous pencillin and flucloxacillin, but lack of symptom improvement after 5  days of antibiotics led to him undergoing Disc puncture under anaesthesia. Kingella kingae was subsequently isolated on culture and K. kingae-specific quantitative PCR assays were positive in peripheral blood and throat swab. He was switched to intravenous cefuroxime, which he received for 2 weeks, followed by a prolonged course of oral antibiotics, and gradually made a full recovery.

Infection of the spine is relatively rare. The incidence is not higher than 0.3 per 100,000 [13], with iatrogenic infection secondary to spinal surgery being the commonest [14]. The posterior surgical approach carries a higher postoperative risk [15]. Other risk factors include presence of significant co-morbidities, poor nutrition, or immunity, and low socio-economic status [16]. Based on the primary or main site of infection, four categories are recognised: 1. Discitis 2. Vertebral osteomyelitis 3. Paraspinal abscess e.g. psoas abscess 4. Epidural abscess These represent a continuum and can co-exist in severe infection, or in immunocomprised patients. Discitis is more common in children than adults due to the blood supply to the disc, which, in children, extends from the cartilaginous end plate into the nucleus pulposus. This allows direct inoculation of the disc space in

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Fig. 19  An MRI image (T2 weighted) showing a high signal in L5/S1 disc and pus collecting anterior to the disc

children (Fig. 19) [17, 18]. In contrast, in adults the blood vessels extend only to the annulus fibrosis. Staphylococcus aureus is by far the commonest organism that causes spinal infection. However, tuberculosis should always be considered when the response to antibiotic is poor. It is important to identify the causative organism wherever possible. Infection of the spine presents a diagnostic challenge to primary healthcare providers. Some studies report delayed diagnosis by up to 3 months in 50% of patients [19] for several reasons: 1. Spinal infection is uncommon, and may not be suspected 2. Children may not able to verbalise their symptoms 3. Non-specific symptoms and signs mimic common viral infection in children [17, 19]. Suspected spinal infection requires urgent orthopedic referral. Essential blood tests include: 1. White cell count: increased WBC count occurs in 2/3 of patients 2. Inflammatory markers: CRP, ESR and procalcitonin (PCT) which are typically elevated 3. Blood cultures are positive in 2/3 of patients [17, 19, 20]. MRI is the gold standard for imaging of spinal infections and can differentiate infection from tumours as well as delineating detailed anatomy [21, 22]. These patients require intravenous antibiotics, guided by local antibiotic policy and culture results. Sometimes, it may be necessary to obtained CT guided or open biopsy of infected tissue for microbiological examination. Most antibiotic policies recommend antibiotics for a minimum of 6 weeks for pyogenic osteomyelitis, with the first 2 weeks given intravenously [19, 23, 24].

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Surgery is indicated in the following situations: 1. For drainage of large abscesses particularly if they are compressing nerves 2. Debridement of dead bone or tissue to facilitate tissue repair 3. Stabilisation where infection affects structural integrity of the spine. Spinal infection is thankfully rare, but is serious and requires prompt treatment to avoid progression to complicated infection. Awareness of the possibility and prompt referral when suspected is essential. In summary, spinal disorders are common in children. They can range from simple and self-limiting muscular sprains to life-altering problems such as deformity, tumours, infection or chronic pain. The chapter highlights these conditions and the current treatments and complications to facilitate a common language between orthopaedic surgeons and general practitioners.

References 1. Knipe H, Thuaimer A. Cobb angle. 2013. 2. Alshryda S, Jones S, Banaszkiewicz P. Postgraduate Paediatric Orthopaedics: the Candidate’s guide to the FRCS (Tr and Orth) examination. Cambridge: Cambridge University Press; 2014. 3. Mehta MH. Growth as a corrective force in the early treatment of progressive infantile scoliosis. J Bone Joint Surg Br. 2005;87(9):1237–47. 4. Mehta MH. The rib-vertebra angle in the early diagnosis between resolving and progressive infantile scoliosis. J Bone Joint Surg Br. 1972;54(2):230–43. 5. Dubousset J, Herring JA, Shufflebarger H.  The crankshaft phenomenon. J Pediatr Orthop. 1989;9(5):541–50. 6. Alshryda S, Tsang K, Dekiewiet G.  In: Alshryda S, Huntley JS, Banaszkiewicz P, editors. Paediatric orthopaedics: an evidence-based approach to clinical questions. Berlin: Springer; 2016. 7. SRS.  Congenital Kyphosis 2020. Available from: https://www.srs.org/patients-and-families/ conditions-and-treatments/parents/kyphosis/congenital-kyphosis Accessed 20 Mar 2020. 8. Herring JA, editor. Tachdjians, pediatric orthopaedics. Philadelphia: Saunders Elsevier; 2013. 9. Sachs B, Bradford D, Winter R, Lonstein J, Moe J, Willson S.  Scheuermann kyphosis. Follow-up of Milwaukee-brace treatment. J Bone Joint Surg Am. 1987;69(1):50–7. 10. Staheli L. Fundamentals of pediatric orthopaedics, vol. 2008. 4th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. 11. Wiltse LL, Newman PH, Macnab I. Classification of spondylolisis and spondylolisthesis. Clin Orthop Relat Res. 1976;117:23–9. 12. Meyerding HW. Spondyloptosis. Surg Gynecol Obstet. 1932;54:371–7. 13. Kang HM, Choi EH, Lee HJ, Yun KW, Lee C-K, Cho T-J, et  al. The etiology, clini cal presentation and long-term outcome of spondylodiscitis in children. Pediatr Infect Dis J. 2016;35(4):e102–e6. 14. Cooper K, Glenn CA, Martin M, Stoner J, Li J, Puckett T. Risk factors for surgical site infection after instrumented fixation in spine trauma. J Clin Neurosci. 2016;23:123–7. 15. Fei Q, Li J, Lin J, Li D, Wang B, Meng H, et al. Risk factors for surgical site infection after spinal surgery: a meta-analysis. World Neurosurg. 2016;95:507–15. 16. Miller M. Review of orthopaedics. Philadelphia: Saunders; 2008. 17. Kayser R, Mahlfeld K, Greulich M, Grasshoff H. Spondylodiscitis in childhood: results of a long-term study. Spine. 2005;30(3):318–23. 18. Avanzi O, Chih LY, Meves R, Mattos C. Tratamento da discite na criança. Rev Assoc Med Bras. 2005;51(2):113–6.

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19. Sobottke R, Seifert H, Fätkenheuer G, Schmidt M, Goßmann A, Eysel P. Current diagnosis and treatment of spondylodiscitis. Deutsches Aerzteblatt Online. 2008;105:181–7. 20. Jensen AG, Espersen F, Skinhøj P, Rosdahl VT, Frimodt-Møller N. Increasing frequency of vertebral osteomyelitis following Staphylococcus aureus bacteraemia in Denmark 1980–1990. J Infect. 1997;34(2):113–8. 21. Fuster D, Tomás X, Mayoral M, Soriano A, Manchón F, Cardenal C, et al. Prospective comparison of whole-body 18F-FDG PET/CT and MRI of the spine in the diagnosis of haematogenous spondylodiscitis. Eur J Nucl Med Mol Imaging. 2014;42(2):264–71. 22. Modic MT, Feiglin DH, Piraino DW, Boumphrey F, Weinstein MA, Duchesneau PM, et al. Vertebral osteomyelitis: assessment using MR. Radiology. 1985;157(1):157–66. 23. Sampath P, Rigamonti D. Spinal epidural abscess. J Spinal Disord. 1999;12(2):89–93. 24. Danner RL, Hartman BJ. Update of spinal epidural abscess: 35 cases and review of the literature. Clin Infect Dis. 1987;9(2):265–74.

Paediatric Shoulder Disorders David Hawkes, H. S. Lloyd, and Matthew Nixon

1

Introduction

The shoulder is a multi-axial ball and socket synovial joint and its normal function is integral to the completion of everyday activities. Pathology is therefore particularly debilitating. Audit data reviewing 193 children referred to an elective paediatric shoulder service at Central Manchester Children’s Hospital illustrates the different types of pathology and their relative frequency (unpublished data) (Table 1). In this case series shoulder instability was the most common pathology. Traumatic instability overlaps with adult medicine, although children tend to have more complex injuries and there is a high rate of recurrence following surgical intervention [1]. Nevertheless, further discussion on shoulder instability is outside the scope of this chapter. The focus here is on obstetric brachial plexus injuries and their impact on the developing shoulder; the congenital malformations Sprengel’s deformity and pseudoarthrosis of clavicle; and the overuse syndrome Little Leaguer’s Shoulder. Tumours can present with shoulder symptoms but these are considered elsewhere.

D. Hawkes (*) Trauma and Orthopaedics Registrar, Countess of Chester Hospital, Chester, UK Orthopaedic Department, Countess of Chester Health Park, Chester, UK H. S. Lloyd Mosgiel Health Centre, Mosgiel, New Zealand Best Practice Advocacy Centre (BPAC), Dunedin, New Zealand Department of General Practice and Rural Health, University of Otago, Dunedin, New Zealand M. Nixon Trauma and Orthopaedic Consultant, Countess of Chester Hospital, Chester, UK © The Author(s), under exclusive license to Springer Nature Switzerland AG 2021 S. Alshryda et al. (eds.), Pediatric Orthopedics for Primary Healthcare, https://doi.org/10.1007/978-3-030-65214-2_18

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Table 1  Paediatric shoulder pathologies Pathology Instability (traumatic/atraumatic/muscle patterning) Neuromuscular disorders (upper and lower motor neuron/myopathies) Congenital malformations (Sprengel’s deformity, congenital pseudoarthrosis of clavicle) Tumours (benign and malignant) Trauma (overuse syndromes)

2

Frequency (%) 39 17 16 15 13

Obstetric Brachial Plexus Injuries Case 1

Ashish, a 5-year-old boy was brought to his general practitioner because of multiple deformities of the left arm, dating from birth. The family had moved to the UK from India, 6 months previously. Mother had gestational diabetes, requiring insulin treatment, but otherwise pregnancy was uneventful. Ashish’s birth was complicated by shoulder dystocia and foetal distress, requiring assisted vaginal delivery using forceps. Birth weight was 4.4 Kg. He was apnoeic and bradycardic at birth requiring immediate intubation and ventilation. He was admitted to the neonatal intensive care unit (NICU) for 14 days. It had been apparent in NICU that his left arm was not moving as much and had an unusual posture, diagnosed as Erb’s Palsy. They were advised this was due to his traumatic delivery and advised that it would likely resolve within a few weeks. By 9 months, there was no improvement, so they took him to an Ayuverdic practitioner who treated him with application of healing oils and rice paste, and with electrical stimulation. However, there was no improvement. At age 2 years, he was taken to an orthopaedic specialist, who advised surgical correction of the deformity, but the parents were unable to afford the cost of the procedure. Ashish was otherwise well and developing normally. He attended school daily and growth and development were appropriate for age. On examination, his left shoulder was in abduction, with his elbow flexed at 30 ° and his wrist extended with metacarpophalangeal joints flexed with his thumb in his palm. The most disabling feature was the severe contracture of the muscles of the shoulder, elbow, forearm and hand. Whenever he moved his hand, there was simultaneous movement of the elbow and shoulder and vice versa. His GP referred Ashish to orthopaedics at his local hospital, but initial assessment concluded he need specialist paediatric expertise, and he was referred to a regional paediatric orthopaedic centre.

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When first seen, the orthopaedic surgeon noted the findings above, and observed the classical trumpet sign: on telling Ashish to lift something towards his mouth, he was unable to accomplish the manoeuvre as both the biceps and triceps were contracting simultaneously, meaning he had no useful function in the arm. A diagnosis of left total brachial plexus injury (without sympathetic nerve involvement) was made, and microsurgery was advised to improve his function. He subsequently underwent brachial plexus exploration and microsurgery. At surgery, extensive fibrosis was observed involving all the roots and trunks of the brachial plexus. After microdissection and neurolysis had freed the nerve trunks, he underwent multiple nerve transfers to achieve better shoulder external rotation and elbow flexion. To achieve finger extension he underwent tendon transfers. The postoperative period was uneventful, and he was discharged after 5  days. He returned to his local hospital for intensive physiotherapy. After 6 months he was able to open his hand and had achieved a weak but functional grip.

2.1

Background

Obstetric brachial plexus injuries (OBPI) have an incidence of just over 1 per 1000 births [2]. A significant proportion of these children recover but in approximately 20–30% of cases the injury is permanent and significant disability results [3]. The emphasis of this section is on the shoulder pathology that can develop following an OBPI, although, depending of the extent of injury elbow contractures and hand weakness can also result, as in case 1. In order to appreciate the pathology of the condition an understanding of brachial plexus anatomy and the factors pertaining to shoulder stability is essential. The brachial plexus is a network of nerves that supplies the musculature and skin of the upper limb. It forms from the ventral rami of spinal nerves C5 and T1 and the roots emerge between the anterior and middle scalene muscles. A complex network of interconnections then leads to the formation of the peripheral nerves as the plexus progresses distally towards the axilla. Figure 1 is a schematic representation of the brachial plexus. In order to appreciate the implications of a plexus injury an understanding of the root values of key upper limb muscles is required: C5 and C6 innervate supraspinatus, infraspinatus, deltoid, biceps and brachialis; C7 innervates the wrist extensors (extensor carpi radialis longus and brevis); C8 innervates the long finger flexors (flexor digitorum profundus and superficialis); and T1 the intrinsic hand muscles responsible for finger abduction and adduction. Each spinal segment gives rise to dorsal and ventral rami which subsequently come together to form the spinal nerves. The ventral rami contain the efferent (motor) nerves and the dorsal rami the afferent (sensory) nerves. The dorsal root

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Fig. 1  Schematic representation of the branches of the brachial plexus supplying muscles around the shoulder. (DSN dorsal scapula nerve, LTN long thoracic nerve, SCN suprascapular nerve, LPN lateral pectoral, MCN musculocutaneous nerve, U/LSSN upper/lower subscapular nerves, TDN thoracodorsal nervce, MPN medial pectoral nerve, A axillary)

ganglia contain the cell bodies of the sensory nerves. The nature of the brachial plexus injury in relation to the dorsal root ganglion has important prognostic implications. Pre-ganglionic rupture is avulsion of the nerve roots proximal to the ganglion and post-ganglionic rupture is avulsion distal to the ganglion. Pre-ganglionic rupture carries a poorer prognosis. There is more connective tissue binding the upper roots of the plexus (C5/C6) to the vertebral column as compared to the lower roots (C8/T1). Therefore, an injury of the upper roots is typically post-ganglionic as compared to the lower roots which tends to be pre-ganglionic. The cervical sympathetic chain lies adjacent to the vertebral column and is the route through which sympathetic nerves run from their thoracolumbar outflow to the head and neck. If the OBPI is extensive the sympathetic chain can also be involved. The risk factors for OBPI have been debated within the literature. Historically they include shoulder dystocia, high birth weight, prolonged labour, breech alignment and instrument assisted vaginal delivery [4, 5]. However, when studied alone

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Table 2  The Narakus classification of OBPIs Narakas grade 1 2 3 4

Region of plexus involved Upper trunks (C5/C6) Upper + middle trunk (C5-C7) Total plexus (C5-T1) Total plexus (C5-T1) + sympathetic chain (Horner’s syndrome)

or in combination they have not been found to be reliable predictors [4]. The mechanism of brachial plexus injury is forced lateral flexion of the cervical spine, which result in traction predominantly on the upper portion of the plexus. Consequently, injuries to the upper trunk (C5/C6) are most common, but more severe injuries can also extend to involve the middle and lower parts of the plexus. Upper trunk injuries are referred to as an Erb’s palsy, isolated lower trunk injuries (Klumpke’s palsy) do not tend to occur. The types of OBPIs have been classified by Narakas [6] (Table 2). The glenohumeral joint is inherently unstable as the spherical humeral head articulates with a relatively flat glenoid fossa. Therefore, the joint relies heavily on dynamic muscular balance to maintain stability. During arm abduction the supraspinatus forms a force couple with the deltoid which acts to limit the superior migration of the humeral head. Similarly, in the axial plane the anterior (subscapularis) and posterior (infraspinatus and teres minor) rotator cuff are balanced. A plexus injury denervates key shoulder girdle muscles which can result in an imbalance. Narakas 1 and 2 injuries cause weakness of the supraspinatus, infraspinatus, deltoid and rhomboids. Consequently, the shoulder is internally rotated due to the unopposed action of subscapularis and winging of the scapula can result due to weakness of the rhomboids. Shoulder abduction is often abnormal but this is predominantly thought to occur due to the loss of force couple between the deltoid and supraspinatus. The deltoid is supplied at multiple levels and the muscle belly at surgery is often healthy [7]. In a Narakus 3 injury there is additional weakness of the subscapularis, pectoralis major and deltoid. In this instance the shoulder is not internally rotated as both the anterior and posterior rotator cuff are weak and therefore arm is flail. In a Narakas 4 injury there is a concurrent Horner’s syndrome characterised by miosis, partial ptosis and ipsilateral forehead anhydrosis. A shoulder internal rotation contracture, which occurs as part of a Narakas 1 and 2 injury, can result in glenohumeral dysplasia if left untreated. Initially the internal rotation contracture causes retroversion and flattening of the humeral head. This then results in the glenoid becoming bi-concave and developing a false posterior facet. Subsequently the humeral head will dislocate posteriorly. In addition to the muscular paralysis, perinatal nerve injury also impacts on the longitudinal growth of the affected muscles, which also contributes to the development of the contractures and osseous abnormalities [8]. Reduction of the joint and rebalancing of the muscles can stimulate bony remodelling and encourage normal growth, particularly in younger children. Figure 2 is a schematic illustration of the development of glenohumeral dysplasia.

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Normal

Retroverted glenoid Biconcave glenoid

Fig. 2  The development of glenohumeral dysplasia as illustrated by an axial section through the scapula and humeral head

2.2

Clinical Assessment

Clinical assessment of children, especially infants, with a suspected OBPI is difficult. The child should be recently fed and examined in a relaxing warm environment. Reassessment to ensure the correct diagnosis and establish the severity of the injury is important [9]. The differential diagnoses for a flail limb include clavicle and humeral fractures and septic arthritis. The essential points to elicit in the history are the details of pregnancy and birth such as birth weight and the use of forceps to assist vaginal delivery. The posture of the limb indicates the type and severity of the injury. Upper trunk injuries are characterised by the waiter’s tip position (ERb’s palsy), with the shoulder adducted and internally rotated, the elbow extended and the wrist flexed. The limb is flail if the injuries involve the entire plexus and in this situation there may be an associated Horner’s syndrome. Asymmetric chest expansion may also be seen in these more severe injuries if there is phrenic nerve involvement. Passive range of motion of the shoulder, particularly in external rotation, requires assessment as it enables shoulder internal rotation contractures to be detected. In infancy evaluation of active range of motion is clearly challenging and it predominantly relies on observation of play and interaction. Eliciting primitive reflexes can also be helpful in provoking active movement [10]. The Active Movement Scale, initially designed for use up to the age of 1 year, assesses 15 upper limb joint movements based on the percentage of active movement a child has within their passive range [11]. The modified Mallet score, suited to children over the age of 3 years, evaluates the function of the arm during different composite movements and scores each 1–5 [12]. In older, more compliant children, it is possible to grade muscle power according to the Medical Research Council grading system.

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Evaluation of these patients is difficult. OBPI are a varied entity with a constellation of different presentations depending on the injury severity and timing of the injury. Knowledge of the common patterns and their natural history is therefore important.

2.3

Investigations

In infancy investigations are required to define the severity of OBPI and exclude the other differential diagnoses of a flail arm. Radiographs will identify fractures or dislocations within the cervical spine and fractures of the clavicle or humerus [13]. A chest x-ray, or ultrasound scan, can be used to assess phrenic nerve involvement [9, 10]. CT myelography was historically used to determine the nature of the nerve injury in relation to the dorsal root ganglion but it has the disadvantage of involving ionising radiation and intrathecal catheterisation. Therefore MRI has become the preferred investigation in many centres [13]. Neurophysiological testing is typically unhelpful [13]. In later childhood investigations are principally used to evaluate the degree of glenohumeral dysplasia. Due to the lack of ossification of the proximal humerus an arthrogram and examination under anaesthetic has a role [9]. An axial slice CT scan allows glenoid and humeral head version to be determined and it can also identify a false posterior facet on the glenoid. An MRI scan allows secondary adaptive changes around the shoulder, such as tightening of the glenohumeral and coracohumeral ligaments and elongation of a coracoid, to be defined. Joint congruity is assessed by the posterior humeral head articulation (PHHA) which assesses the percentage of the humeral head anterior to the mid axis of the glenoid fossa. The normal value is 50% with values less than this implying humeral head subluxation or dislocation [14] (Fig. 3). Anteroir

b b

A

A HH

H PH

P

a

a OBPI dysplasia

Normal

Posteroir

Fig. 3 (a) Glenoid version in relation to the spine of the scapula. (b) posterior humeral subluxation

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Management

2.4.1 Management in Primary Care Urgent referral to specialist care for appropriate assessment, investigation and management should be arranged. 2.4.2 Management in Secondary Care The management of OBPI includes primary surgery, which involves neurosurgical exploration and reconstruction of the brachial plexus and secondary surgery, which involves reduction and rebalancing of the glenohumeral joint. Historically secondary surgery was the only option but with modern techniques the results of primary surgery have improved and secondary surgery is now reserved for addressing any residual defects or for cases which present late [15].  rimary Neurosurgical Reconstruction P There is a narrow window of opportunity in which brachial plexus reconstruction can be undertaken and the timing of surgery is therefore critical. If surgery is delayed, fatty atrophy of the affected muscles renders them functionless even if they are reinnervated [15]. Evidence suggests that intervention within the first 3 months of life improves outcomes [16]. The difficulty is that this is undertaken before the opportunity for spontaneous recovery has occurred. The general consensus in the literature is that primary surgery should be performed when it is anticipated that the child will have no or inadequate spontaneous recovery [15]. In upper plexus injuries neurosurgical exploration and reconstruction is indicated if the biceps muscle has not recovered by 3 months of age [15, 17]. In some respects the decision to intervene is easier when there is a total plexus injury with a Horner’s syndrome as there is no spontaneous recovery in these cases [9]. There are predominantly 3 different techniques used for brachial plexus reconstruction. Neurolysis is the release of a nerve from any surrounding fibrous tissue. However, as a technique alone, it does not result in sustained functional improvements [18]. Nerve grafting involves resection of the neuroma-in-continuity that often develops in the affected nerve roots and replacement with a graft. The technique was pioneered by Gilbert who reported excellent results [17]. More recently Lin et al. demonstrated neuroma-in-continuity resection and nerve grafting resulted in significant improvements in the Active Movement Scores as compared to neuroloysis alone [18]. The final technique, nerve transfer, involves the re-routing of an unaffected nerve (or part of that nerve) to reinnervate a previously denervated distal nerve. A typical nerve transfer in upper trunk injuries might involve the use of a branch of the spinal accessory nerve to reconstruct the suprascapular nerve [9]. Lardak reviewed 10 patients treated with nerve transfers and demonstrated results comparable to grafting [19]. Surgery in each case is tailored based on the specific nature of the injury, typically involving a combination of all techniques.

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Secondary Shoulder Reduction and Rebalancing The results of primary brachial plexus reconstruction are poor after the age of 2  years and a different approach is required in older children [9]. This so called secondary surgery involves reducing the glenohumeral joint, releasing the contractures and performing tendon transfers to restore muscular balance. The aim is to promote osseous remodelling of the dysplastic joint and subsequently restore function. Supervised parental provided physiotherapy should be performed in all children to prevent the development of contractures and maintain range of motion whether or not they undergo further surgery. In children with an internal rotation contracture but a congruent glenohumeral joint, a subscapularis release with or without a tendon transfer is the surgery of choice. Transfer of the latissimus dorsi tendon to the greater tuberosity is the most commonly used transfer and it is effective in restoring abduction and external rotation. Ozturk, in a study of 30 patients, reported increase in mean abduction and external rotation of 138 ° and 51 ° respectively [20] and these results are echoed elsewhere in the literature [21, 22]. In established glenohumeral dysplasia the joint is incongruent and this must be addressed in conjunction with any re-balancing procedures. A stepwise approach to established glenohumeral dysplasia has been promoted by Di Mascio. Here the minimum surgery necessary to achieve a congruent and balanced joint is performed [23] (Table 3). The first step described is an open reduction, but in reality, this is never performed in isolation. A coracoid osteotomy is often necessary as it becomes elongated as part of the disease process. A release of the upper fibres of subscapularis is then performed or, if necessary, a ‘Z’ lengthening of its tendon. Some authors advocate performing the anterior release arthroscopically. Kozin reported their experience of 44 patients undergoing arthroscopic release with favourable outcomes seen with regards to glenohumeral congruity and active range of motion [14]. The humeral head flattens and retroverts in the natural history of the condition as discussed above. In this situation it is not possible to re-fashion the spherical nature of the humeral head surgically. Therefore the goal is to maintain a concentric joint reduction in order to allow remodelling to occur. A de-rotational humeral osteotomy can be performed if there is excessive humeral head retroversion. This is best performed proximal to the deltoid insertion [9]. Kambhampati performed a humeral osteotomy in 70 shoulders with established glenohumeral dysplasia when retroversion of the head was greater than 40 °. Good outcomes were seen with an increase in external rotation range of motion of 58  ° and an increase in the Mallet score. There was however a high re-operation rate [7]. Table 3  Di Mascio’s stepwise approach to treating established glenohumeral dysplasia [23]

Step Procedure 1 Open joint reduction 2 + anterior release and lengthening of subscapularis 3 + internal rotation humeral osteotomy 4 + Glenoplasty

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OBPI dysplasia

Bone block

Sub-chondral opening wedge osteotomy

Fig. 4  Bone block and sub-chondral glenoplasty for OBPI

The final step in the treatment of glenohumeral dysplasia is glenoplasty which addresses glenoid retroversion. This can either be performed with a posterior bone block, which acts as a physical barrier to dislocation, or via a subarticular opening wedge osteotomy (Fig. 4). The latter has the advantage of restoring a hyaline cartilage articular surface. Di Mascio reported their experience in 29 shoulders where glenoplasty was used to achieve a stable shoulder with improvements in the Mallet score and range of motion [23].

3

Sprengel Deformity

3.1

Background

The deformity that now carries the name of Sprengel was actually first described by Eulenberg in 1863. However, it was subsequently attributed to Sprengel after he presented a case series of 4 patients in 1891 [24]. It is a rare condition but it represents the most common congenital abnormality of the shoulder girdle [24]. The scapula migrates caudally as it develops during the third to the seventh week of gestation [24]. In Sprengel’s deformity there is arrest of both its development and normal caudal migration. As a consequence, the scapula lies in an abnormally high position and is rotated such that the inferior angle is medialised and the glenoid fossa faces inferiorly [25] (Fig. 5). The scapula itself is hypoplastic with a reduced height and it has an anteriorly curved supraspinous portion [26]. An associated omovertebral bar was found in 54% of cases in a case series by Khairouni et al. The structure, which is usually bone but can be fibrous tissue or cartilage, arises from the superomedial angle of the scapula and inserts on to a transverse or spinous process at a variable level in the cervical spine [27]. The peri-scapula muscles, particularly the trapezius and rhomboids, can be hypoplastic or fibrous. Sprengel’s deformity is often associated with other congenital abnormalities [27]. In the cervical spine it can occur with spinal synostosis and transverse process anomalies. Approximately 35% of patients with Klippel–Feil syndrome, a condition

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Fig. 5  CT Image of a child with a right sided Sprengel’s deformity

Fig. 6  Typical x-ray features in Sprengel’s deformity

primarily characterised by abnormal union of two or more cervical vertebrae, have an associated Sprengel deformity. In the thoracic and lumbar spine there can be a related spina bifida and spina bifida occulta, scoliosis or a 13th rib [25].

3.2

Clinical Assessment

Sprengel’s deformity occurs as a spectrum with cases ranging from mild to severe. Correspondingly, the age at diagnosis varies markedly, with the more severe cases tending to be diagnosed in the early years with the milder cases later [25, 27]. It is therefore essential that patients are examined fully undressed from the waist up with careful inspection, comparing both the affected and unaffected side, a requirement to detect mild cases. Patients can have a shrugged shoulder appearance [28] with a lump at the base of the neck, the size of which varies according to how far the scapula has descended [29] (Fig. 6). The cosmetic deformity seen in Sprengel’s deformity has been classified by Cavendish [30] (Table  4). This is a subjective classification based on the appearance of the shoulder during inspection. Care is needed in differentiating Sprengel’s deformity from scoliosis, with a number of children erroneously referred

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Table 4  The Cavendish classification Grade Grade 1 Grade 2 Grade 3 Grade 4

Description Shoulders level Deformity not visible when the patient is dressed Shoulders are almost level Deformity visible as a lump in the web of the neck when the patient is dressed Shoulder elevated 2–5 cm Deformity clearly visible Shoulder elevated Superomedial angle of the scapula lies near the occiput

to the spinal clinic for assessment, although, as outlined above, the conditions can occur together. Functional limitation arises due to impairment of normal scapulothoracic motion and an inferiorly angled glenoid [24, 26]. Resultantly patients have reduced range of motion in abduction [28, 29].

3.3

Investigations

Radiographs are the initial investigation. The position of the scapula in relation to the contralateral side is noted (Fig. 6). Associated abnormalities such as an omovertebral bar, scoliosis or rib abnormalities may also be seen [24]. Rigault proposed a classification based on radiographic appearances [31]. The grade is determined by the superomedial angle of the scapula and the associated vertebral level. In grade 1 the angle lies below T1, in grade 2 between T1 and C5 and in grade 3 above C5. A CT scan is necessary to fully delineate scapula morphology and an MRI scan is advocated to detect a fibrous or cartilaginous omovertebral bar. A thorough evaluation for associated abnormalities, both clinically and radiographically, is essential. Concurrent cervical spine abnormalities are the only established negative prognostic indicator following surgery and therefore prior knowledge of these is necessary to appropriately counsel the patient and parents pre-operatively.

3.4

Management

3.4.1 Management in Primary Care Mild cases, with little cosmetic concern and no functional limitation, are managed non-operatively nevertheless referral to secondary care for assessment is warranted on account of the rarity of the condition. 3.4.2 Management in Secondary Care Non-operative management with physiotherapy, to improve shoulder range of motion and prevent torticollis, is indicated for mild cases with no restriction of shoulder function and little cosmetic concern (Cavendish grade 1 and 2). The natural history of the condition has been reviewed in a case series of 16 scapulae. At a

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mean follow up of 26 years there was no significant change in range of motion or the Cavendish or Rigault grade [25]. Although long term review is prudent the condition is non-progressive. Operative intervention is reserved for those cases where there is severe cosmetic concern or functional limitation. In respect to the Rigault grade, Farsetti proposes surgery is indicated for grade 3 cases and in those grade 2 cases where the superomedial aspect of the scapula is located above the sixth rib [25]. The optimal age at which to undertake surgery remains controversial, but most authors advocate operating between 3 and 6  years old. The age of the patient at surgery has not been demonstrated to be a predictive criterion for outcome [27], however the risk of brachial plexus injury does increase in older children [26]. The principles of surgery are to lower the scapula with respect to the chest wall, restore its normal rotation and excise any omovertebral connection that may be present. Lowering the scapula to match that of the contralateral side however should be cautioned, particularly in severe cases, due to the risk of brachial plexus palsy. Some authors advocate a clavicle osteotomy which can be performed either at the time of definitive surgery or as a first stage procedure in order to reduce this risk [26]. Multiple surgical techniques have been described for Sprengel’s deformity, the most common of which are the Woodward [32] and Green procedures [33]. In the Woodward procedure the levator scapulae, rhomboids and trapezius muscles are first detached from their vertebral origin. The scapula is moved caudally and the muscles are subsequently reattached in a more inferior position. This is accompanied by excision of any omovertebral connection [32]. In a case series of 7 patients by Walstra et al. mean improvement in range of motion was 56 ° and cosmesis improved by 1 grade on the Cavendish classification [34]. Since the initial description by Woodward modifications to the procedure have been described with emphasis placed on restoring the normal rotational alignment of the scapula, as well as lowering it with respect to the chest wall. Khairouni, in a series of 19 cases, used a modification of the Woodward procedure to lower and rotate the scapula correcting the varus malalignment of the glenoid fossa. Improvements in function and cosmesis were seen in 79% of patients [27]. Similar results were reported in a case series by Nakamura et al. who placed a similar emphasis on restoring normal scapula rotation [28]. More recently, a minimally invasive approach has also been described [35]. In the Green procedure the muscles on the medial and superior aspect of the scapula are detached. Any omovertebral connection present is excised, in addition to the curved supraspinous portion of the scapula. The scapula is then moved caudally and the muscles reinserted without tension after they are lengthened as needed. The position of the scapula is then maintained by percutaneous wires or a plaster jacket [33]. Multiple modifications have again been described. Wada presented a case series of 22 patients in which the inferior angle of the scapula was positioned in a pocket developed underneath latissimus dorsi. Improvement in cosmesis and function were seen but scapula winging post-operatively complicated 3 cases [26]. A further modification was made by Bellemans in which the serratus anterior was not detached from the scapula and immediate mobilisation post-operatively was

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allowed. In 7 cases abduction improved by approximately 75  ° and subjectively outcome was excellent or good in 6 cases [29]. Scapula osteotomies have also been described as a surgical option for the treatment of Sprengel’s deformity. A vertical scapula osteotomy allows caudal displacement of the scapula [36] and the Mears technique involves excising a wedge of the superomedial aspect of the scapula [37]. Studies of surgical intervention for Sprengel’s deformity are limited to case series with no high level evidence directly comparing surgical techniques. Good improvements in function and cosmesis have been reported following both the Woodward and Green procedures and their modifications. Surgeon familiarity with a particular procedure, as influenced by their training, is arguably the more significant determinant of outcome at present.

4

Congenital Clavicle Pseudoarthrosis

4.1

Background

Congenital clavicle pseudoarthrosis (CCP) is a rare condition that was first reported in the literature by Fitzwilliams in 1910 [38]. Since then, approximately, there are only another 200 cases that have been described [39]. In the majority it is unilateral and affects the right side, although bilateral cases have been described in around 10%. Isolated left sided lesions have been known to occur, however, these are extremely rare and associated with dextrocardia or cervical ribs [40]. Two theories have been proposed to account for the pathophysiology of the condition. There are 2 primary clavicular ossification centres that develop and fuse in the 6-seventh week of intra-uterine life. The first theory purports that CCP develops due to a failure of fusion of these ossification centres [41]. Histological analysis provides some support for this theory. The bone ends are covered in hyaline cartilage with fibrocartilage in between. The organisation of chondrocytes in the hyaline cartilage is similar to that of the physis with new bone formation seen at the bone-­cartilage interface [40, 42, 43]. However, this failure of fusion theory has been questioned in the literature as the junction of the ossification centres lies between the lateral and middle third of the clavicle whereas the site of the pseudoarthrosis is found in the middle portion of the bone [44]. The alternate vascular theory advocates that the force of pulsation transmitted from the subclavian artery disrupts intramembranous ossification of the developing clavicle [45]. Advocates of this theory believe that cervical or vertically oriented ribs may predispose to the formation of CCP by exaggerating this arterial pulsation. The right subclavian artery lies higher than the left anatomically, which would apparently support the theory as there is a much higher incidence of right sided cases.

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Clinical Assessment

CCP is rarely diagnosed during the neonatal period, rather a child typically presents in the first few years of life [46]. The child is usually asymptomatic, complaining of a painless non-tender mass over the clavicle. Indeed, the first presentation may be driven by a parental cosmetic concern. There may be associated weakness with an inability to use the arm to push effectively whilst crawling. It is important to elicit any history of trauma as a clavicular fractures related to obstetric birth injury are an important differential diagnosis. On examination the enlarged bone ends at the non-union site are palpable and motion may be demonstrated between the segments. The mass may increase in size during a phase of rapid growth and it can subsequently affect the child’s posture with shortening and forward dropping of the shoulder girdle [47]. It can cause thoracic outlet obstruction, with reports of vascular and neurogenic compromise described [48]. Box 1 outlines the key differential diagnoses.

Box 1 Differential Diagnoses in CCP

• Obstetric trauma –– History of traumatic birth –– Child presents with pseudoparalysis of the arm –– Copious callus on follow up radiographs • Cleidocranial dysplasia –– Abnormality of intramembranous bone formation –– Partial or complete abscess of clavicles in addition to other skeletal abnormalities such as maxillary hypoplasia and failure of Fontanelle closure • Neurofibromatosis type 1 –– The clavicle is a rare site of dysplasia in neurofibromatosis –– Associated cutaneous manifestations such as café au lait spots and neurofibromas

4.3

Investigations

Radiographs are the initial investigation of choice and the typical features have been described by Gennaro [39]. The pseudoarthrosis is found in the middle portion of the clavicle and the bone ends are either rounded, tapered or enlarged. The proximal and distal segments commonly lie horizontally, however they can point cranially. The mean gap between the bone ends is 9.5 ± 5.6 mm, which represents 9 ± 4% of the entire length of the clavicle [39]. Figure 7 illustrates these typically radiographic features.

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Fig. 7  Typical x-ray features of a patient with CCP

4.4

Management

4.4.1 Management in Primary Care CCP is a rare condition with little clear understanding of the natural history and no consensus on management algorithms. Referral to secondary care for a specialist opinion is therefore warranted in all cases. 4.4.2 Management in Secondary Care Currently, there is no evidence to support any absolute indication for surgery. The natural history of the condition is difficult to establish as many reports in the literature are of surgical series and therefore selection bias influences the cases managed non-operatively. Surgical intervention has been advocated by some even if a child is asymptomatic due to the possibility of the development of thoracic outlet obstruction. Surgery however is not without risk and complications such as non-union, infection and brachial plexus injury have all been described. Consequently, a number of authors express reluctance towards operating when a child is asymptomatic. There is therefore little consensus with regard to the indications and indeed the optimal timing of surgery. It can however be stated that fusion can only be achieved with surgery and has never been seen spontaneously [47]. The principles of surgery are excision of the pseudoarthrosis tissue, alignment of bone ends, bone grafting and then skeletal stabilisation. A number of studies have examined the different methods of skeletal fixation, such as wires and plates (Fig. 8) however, these are limited to small case series with no high level evidence available. Molto, in a review of 6 patients, described outcomes following K-wire fixation with iliac crest bone grafting. Union was achieved in all cases but 3 developed a superficial pin site infection [47]. Chandran retrospectively reviewed 10 patients with CCP comparing outcomes following pin and plate fixation. Higher rates of infection and non-union were observed in the cases that underwent fixation with a pin [49]. Beslikas presented a case report of a 6 year old girl who was treated successfully with an external fixator and cited advantageous cosmetic results [50]. At present there is no clear consensus on the optimal method of skeletal fixation with reasonable outcomes seen following fixation with both pins and plates.

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Fig. 8  Surgical fixation of CCP with a plate and screws (a) and K-wire (b)

a

b A number of different types of bone grafting have been employed. Gennaro in a larger review of 19 cases undergoing surgical intervention compared autologous iliac crest bone grafting (n = 15) with a fibula allograft (n = 4). The rates of non-­ union were significantly higher in those treated with a fibula allograft [39]. Elliot reported the outcome of the xenograft Tutobone (bovine substitute material) in 2 cases. Treatment failure was seen in both cases with significant osteolysis and failure of graft material incorporation [51]. The evidence for the management of CCP is limited to small case series and reports. With regard to the technical aspects of surgery, management controversy still exists as to the optimal method of surgical stabilization, with little evidence to differentiate between the use of pins and plates. However, the use of bone graft does appear important with optimal results seen when using iliac crest autograft as comparted to fibula allograft and xenograft.

5

Little Leaguer’s Shoulder

5.1

Background

Little Leaguer’s shoulder (LLS) is an overuse syndrome that affects the proximal humeral physis in skeletally immature athletes. Whilst it can occur in other sports, such as tennis, most cases are seen in baseball pitchers or backstops [52]. The peak age of diagnosis is 12–13 years and the overall incidence is increasing due to the higher pitch velocities and the increased participation in sport [52]. Poor stance and core weakness leads to scapula mal-positioning and escalation of forces along the kinetic chain. This causes chronic repetitive microtrauma on the proximal humeral

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physis. Premature physeal closure and physeal fracture have been reported but these are considered extremely rare [53, 54].

5.2

Clinical Assessment

Children with LLS complain of a diffuse shoulder pain on throwing. This might subsequently progress to pain during daily activities or even at rest [52]. Shoulder examination is characterised by tenderness of the proximal humerus and there may be a reduced range of motion [52].

5.3

Investigations

Radiographs are the initial investigation of choice. The findings are physeal widening, increased sclerosis and bony fragmentation adjacent to the physis [52, 55]. The pathology tends to occur in the anterolateral zone of the physis and therefore x-rays in external rotation can highlight the abnormalities. Comparative x-rays of the contralateral shoulder can be helpful. Figure 9 illustrates the typical x-ray features of LLS. In some instances, an MRI scan may be required to exclude other shoulder pathology, which, in addition to the physeal widening, will show associated marrow oedema [55].

Fig. 9  X-ray showing the characteristic features of LLS particularly physeal widening

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Management

There is no role for surgery in the management of LLS. Physiotherapy, addressing throwing mechanics and specifically looking at the kinetic chain emphasising stance and core strength, is the treatment.

References 1. Nixon MF, Keenan O, Funk L. High recurrence of instability in adolescents playing contact sports after arthroscopic shoulder stabilization. J Pediatr Orthop B. 2015;24(3):173–7. 2. Chauhan SP, Blackwell SB, Ananth CV. Neonatal brachial plexus palsy: incidence, prevalence, and temporal trends. Semin Perinatol. 2014;38(4):210–8. 3. Pondaag W, Malessy MJ.  The evidence for nerve repair in obstetric brachial plexus palsy revisited. Biomed Res Int. 2014;2014:434619. 4. Ouzounian JG.  Risk factors for neonatal brachial plexus palsy. Semin Perinatol. 2014;38(4):219–21. 5. Zuarez-Easton S, Zafran N, Garmi G, Hasanein J, Edelstein S, Salim R. Risk factors for persistent disability in children with obstetric brachial plexus palsy. J Perinatol: official journal of the California Perinatal Association. 2017;37(2):168–71. 6. Al-Qattan MM, El-Sayed AA, Al-Zahrani AY, Al-Mutairi SA, Al-Harbi MS, Al-Mutairi AM, et al. Narakas classification of obstetric brachial plexus palsy revisited. J Hand Surg Eur Vol. 2009;34(6):788–91. 7. Kambhampati SB, Birch R, Cobiella C, Chen L. Posterior subluxation and dislocation of the shoulder in obstetric brachial plexus palsy. J Bone Joint Surg. 2006;88(2):213–9. 8. Cheng W, Cornwall R, Crouch DL, Li Z, Saul KR. Contributions of muscle imbalance and impaired growth to postural and osseous shoulder deformity following brachial plexus birth palsy: a computational simulation analysis. J Hand Surg Am. 2015;40(6):1170–6. 9. Nixon M, Trail I. Management of shoulder problems following obstetric brachial plexus injury. Shoulder Elb. 2014;6(1):12–7. 10. Duff SV, DeMatteo C. Clinical assessment of the infant and child following perinatal brachial plexus injury. J Hand Therapy: official journal of the American Society of Hand Therapists. 2015;28(2):126–33. quiz 34 11. Clarke HM, Curtis CG.  An approach to obstetrical brachial plexus injuries. Hand Clin. 1995;11(4):563–80. discussion 80-1 12. Abzug JM, Kozin SH. Evaluation and management of brachial plexus birth palsy. Orthop Clin North Am. 2014;45(2):225–32. 13. van Ouwerkerk WR. Preoperative investigations in obstetric brachial plexus palsy. Semin Plast Surg. 2005;19(1):17–23. 14. Kozin SH, Boardman MJ, Chafetz RS, Williams GR, Hanlon A.  Arthroscopic treatment of internal rotation contracture and glenohumeral dysplasia in children with brachial plexus birth palsy. J Shoulder Elb Surg. 2010;19(1):102–10. 15. Socolovsky M, Costales JR, Paez MD, Nizzo G, Valbuena S, Varone E.  Obstetric brachial plexus palsy: reviewing the literature comparing the results of primary versus secondary surgery. Child’s Nerv Syst: ChNS: Official Journal of the International Society for Pediatric Neurosurgery. 2016;32(3):415–25. 16. Terzis JK, Kokkalis ZT. Primary and secondary shoulder reconstruction in obstetric brachial plexus palsy. Injury. 2008;39(Suppl 3):S5–14. 17. Gilbert A, Tassin JL. Surgical repair of the brachial plexus in obstetric paralysis. Chirurgie; memoires de l’Academie de chirurgie. 1984;110(1):70–5. 18. Lin JC, Schwentker-Colizza A, Curtis CG, Clarke HM. Final results of grafting versus neurolysis in obstetrical brachial plexus palsy. Plast Reconstr Surg. 2009;123(3):939–48.

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19. Ladak A, Morhart M, O’Grady K, Wong JN, Chan KM, Watt MJ, et al. Distal nerve transfers are effective in treating patients with upper trunk obstetrical brachial plexus injuries: an early experience. Plast Reconstr Surg. 2013;132(6):985e–92e. 20. Ozturk K, Bulbul M, Demir BB, Buyukkurt CD, Ayanoglu S, Esenyel CZ.  Reconstruction of shoulder abduction and external rotation with latissimus dorsi and teres major transfer in obstetric brachial plexus palsy. Acta Orthop Traumatol Turc. 2010;44(3):186–93. 21. Pagnotta A, Haerle M, Gilbert A. Long-term results on abduction and external rotation of the shoulder after latissimus dorsi transfer for sequelae of obstetric palsy. Clin Orthop Relat Res. 2004;426:199–205. 22. Aydin A, Ozkan T, Onel D. Does preoperative abduction value affect functional outcome of combined muscle transfer and release procedures in obstetrical palsy patients with shoulder involvement? BMC Musculoskelet Disord. 2004;5:25. 23. Di Mascio L, Chin KF, Fox M, Sinisi M. Glenoplasty for complex shoulder subluxation and dislocation in children with obstetric brachial plexus palsy. J Bone Joint Surg. 2011;93(1):102–7. 24. Harvey EJ, Bernstein M, Desy NM, Saran N, Ouellet JA. Sprengel deformity: pathogenesis and management. J Am Acad Orthop Surg. 2012;20(3):177–86. 25. Farsetti P, Weinstein SL, Caterini R, De Maio F, Ippolito E. Sprengel’s deformity: long-term follow-up study of 22 cases. J Pediatr Orthop B. 2003;12(3):202–10. 26. Wada A, Nakamura T, Fujii T, Takamura K, Yanagida H, Yamaguchi T, et al. Sprengel deformity: morphometric assessment and surgical treatment by the modified green procedure. J Pediatr Orthop. 2014;34(1):55–62. 27. Khairouni A, Bensahel H, Csukonyi Z, Desgrippes Y, Pennecot GF. Congenital high scapula. J Pediatr Orthop B. 2002;11(1):85–8. 28. Nakamura N, Inaba Y, Machida J, Saito T. Use of glenoid inclination angle for the assessment of unilateral congenital high scapula. J Pediatr Orthop B. 2016;25(1):54–61. 29. Bellemans M, Lamoureux J. Results of surgical treatment of Sprengel deformity by a modified Green’s procedure. J Pediatr Orthop B. 1999;8(3):194–6. 30. Cavendish ME. Congenital elevation of the scapula. J Bone Joint Surg. 1972;54(3):395–408. 31. Rigault P, Pouliquen JC, Guyonvarch G, Zujovic J.  Congenital elevation of the scapula in children. Anatomo-pathological and therapeutic study apropos of 27 cases. Revue de chirurgie orthopedique et reparatrice de l’appareil moteur. 1976;62(1):5–26. 32. Woodward JW. Congenital elevation of the scapula: correction by release and transplantation of muscle origins. JBJS. 1961;43(2):219–28. 33. Green W.  The surgical correction of congenital elevation of the scapula (Sprengel’s deformity). J Bone Joint Surg [Am]. 1957;39-A:1439. 34. Walstra FE, Alta TD, van der Eijken JW, Willems WJ, Ham SJ.  Long-term follow-up of Sprengel’s deformity treated with the Woodward procedure. J Shoulder Elb Surg. 2013;22(6):752–9. 35. Soldado F, Barrera-Ochoa S, Domenech-Fernandez P, Bergua-Domingo JM, Diaz-Gallardo P, Knorr J. Endoscopic Woodward procedure for Sprengel deformity: case report. J Pediatr Orthop B. 2017;26(3):266–9. 36. McMurtry I, Bennet GC, Bradish C.  Osteotomy for congenital elevation of the scapula (Sprengel’s deformity). J Bone Joint Surg. 2005;87(7):986–9. 37. Mears DC. Partial resection of the scapula and a release of the long head of triceps for the management of Sprengel’s deformity. J Pediatr Orthop. 2001;21(2):242–5. 38. Fitzwilliams DL. Hereditary cranio – cleido-dysostosis. Lancet. 1910;176(4551):1466–75. 39. Di Gennaro GL, Cravino M, Martinelli A, Berardi E, Rao A, Stilli S, et al. Congenital pseudarthrosis of the clavicle: a report on 27 cases. J Shoulder Elb Surg. 2017;26(3):e65–70. 40. Galanopoulos I, Ashwood N, Garlapati AK, Fogg Q.  Congenital pseudarthrosis of clavicle: illustrated operative technique and histological findings. BMJ Case Rep. 2012;2012:bcr2012006908. 41. Fawcett E. The development and ossification of the human clavicle. J Anat Physiol. 1913;47(Pt 2):225–34.

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42. Hirata S, Miya H, Mizuno K. Congenital pseudarthrosis of the clavicle. Histologic examination for the etiology of the disease. Clin Orthop Relat Res. 1995;315:242–5. 43. Gomez-Brouchet A, Sales de Gauzy J, Accadbled F, Abid A, Delisle MB, Cahuzac JP.  Congenital pseudarthrosis of the clavicle: a histopathological study in five patients. J Pediatr Orthop B. 2004;13(6):399–401. 44. Ogata S, Uhthoff HK.  The early development and ossification of the human clavicle--an embryologic study. Acta Orthop Scand. 1990;61(4):330–4. 45. Lloyd-Roberts GC, Apley AG, Owen R.  Reflections upon the aetiology of congenital pseudarthrosis of the clavicle. With a note on cranio-cleido dysostosis. J Bone Joint Surg. 1975;57(1):24–9. 46. Brevaut-Malaty V, Guillaume JM. Neonatal diagnosis of congenital pseudarthrosis of the clavicle. Pediatr Radiol. 2009;39(12):1376. 47. Lorente Molto FJ, Bonete Lluch DJ, Garrido IM. Congenital pseudarthrosis of the clavicle: a proposal for early surgical treatment. J Pediatr Orthop. 2001;21(5):689–93. 48. Watson HI, Hopper GP, Kovacs P. Congenital pseudarthrosis of the clavicle causing thoracic outlet syndrome. BMJ Case Rep. 2013;2013:bcr2013010437. 49. Chandran P, George H, James LA. Congenital clavicular pseudarthrosis: comparison of two treatment methods. J Child Orthop. 2011;5(1):1–4. 50. Beslikas TA, Dadoukis DJ, Gigis IP, Nenopoulos SP, Christoforides JE. Congenital pseudarthrosis of the clavicle: a case report. J Orthop Surg (Hong Kong). 2007;15(1):87–90. 51. Elliot RR, Richards RH.  Failed operative treatment in two cases of pseudarthrosis of the clavicle using internal fixation and bovine cancellous xenograft (Tutobone). J Pediatr Orthop B. 2011;20(5):349–53. 52. Heyworth BE, Kramer DE, Martin DJ, Micheli LJ, Kocher MS, Bae DS.  Trends in the presentation, management, and outcomes of little league shoulder. Am J Sports Med. 2016;44(6):1431–8. 53. Dalldorf PG, Bryan WJ. Displaced salter-Harris type I injury in a gymnast. A slipped capital humeral epiphysis? Orthop Rev. 1994;23(6):538–41. 54. Lipscomb AB. Baseball pitching injuries in growing athletes. J Sports Med. 1975;3(1):25–34. 55. Paz DA, Chang GH, Yetto JM Jr, Dwek JR, Chung CB.  Upper extremity overuse injuries in pediatric athletes: clinical presentation, imaging findings, and treatment. Clin Imaging. 2015;39(6):954–64.

Paediatric Elbow Robert Wilson and Neil Wilson

1

Anatomy of the Paediatric Elbow

The elbow is a synovial joint which is classed functionally as a “hinge joint”. There are two separate articulating surfaces. The trochlear notch of the ulna and trochlea of the humerus; and the head of the radius and capitellum of the humerus. The joint capsule of the elbow is thickened medially and laterally to form collateral ligaments which stabilise flexion and extension movements of the elbow. The radial collateral ligament is found on the lateral side of the joint, extending from the lateral epicondyle, and blending with the annular ligament of the radius. The ulnar collateral ligament originates from the medial epicondyle and attaches to the coronoid process and olecranon of the ulna. Fat pads occur at the upper region of the elbow capsule; a posterior pad within the depths of the olecranon fossa when the elbow is flexed and an anterior pad overlying the coronoid fossa. The significance of this is reflected in their appearance radiologically if displaced by the presence of an effusion within the elbow. In such cases an occult fracture may be suspected, particularly if the posterior fat pad is elevated. The ossification centres of the elbow are of particular relevance in the paediatric setting. There are six ossification sites and the timing and sequence of their development follows a reproducible pattern [1]. The order of appearance can be recalled by a useful mnemonic, CRITOL: Capitellum first, then Radial head, Internal (medial) epicondyle, Trochlea, Olecranon, and finally the Lateral (external) epicondyle. A simple counting method can be used to remember the ages when the ossification centres appear, 1-3-5-7-9-11. This helps in the interpretation of the radiographs of the paediatric elbow. The order of ossification of the internal (medial) epicondyle and the trochlea is important. For example, if the trochlea centre can be seen on an R. Wilson GP, Kenmure Medical Practice, Glasgow, UK N. Wilson (*) Consultant, Royal Hospital for Children, Glasgow, UK © The Author(s), under exclusive license to Springer Nature Switzerland AG 2021 S. Alshryda et al. (eds.), Pediatric Orthopedics for Primary Healthcare, https://doi.org/10.1007/978-3-030-65214-2_19

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8-11yrs 9-13yrs

5-8yrs 7-9yrs

12-14yrs 13-16yrs

1m-11m 1m-26m

14+yrs 17+yrs

7-11yrs 8-13yrs

10+yrs 12+yrs

10+yrs 12+yrs

Fig. 1  age of appearance and fusion of ossification centre around the elbow. CRITOL is a useful mnemonic to remember. The sequence of appearance is more important of the age of appearance

elbow radiograph but not the internal epicondyle then it is likely a medial epicondylar fracture is present where the ossification centre has become avulsed and been displaced. The ossification centres appear later in boys than girls (Fig. 1). Extension of the elbow joint is predominantly accomplished by the triceps brachii and the anconeus; whereas, flexion is performed by the brachialis, biceps brachii and brachioradialis. In the clinical evaluation of a child following elbow injury, if a normal range of movement is found, especially full elbow extension, researchers agree that immediate radiographs are not necessary if the child can be re-examined should symptoms not resolve in a week or so [2].

2

Traumatic Disorders of the Paediatric Elbow

2.1

Pulled Elbow (Nursemaid’s Elbow)

Subluxation of the annular ligament, Pulled Elbow Syndrome or Nursemaid’s elbow is a common elbow injury in young children. Typically, cases occur in children around 2 or 3 years old, sometimes younger but rarely after 7 years of age. Many cases may be treated in Primary Care or a Minor Injuries Unit without recourse to orthopaedic involvement. The injury results from longitudinal traction to the child’s hand or wrist with the elbow extended and the forearm probably in pronation. Common incidents include lifting or swinging the child or when the child trips or falls whilst the hand is held

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NORMAL ELBOW RADIUS LIGAMENT

ULNA

PULLED ELBOW PARTIALLY SLIPPED RADIUS TORN LIGAMENT

Fig. 2  Pulled elbow occurs when the radial head slips through the annular ligament

(Fig. 2). After the initial incident any distress often subsides but the child remains reluctant to use the arm which may be held to their side, often pronated. Some limitation of the flexion-extension range may be found but trying to supinate the forearm will be sore and resisted. Some may describe shoulder discomfort or pain referred to the wrist. Radiographs are usually unremarkable and in typical cases add little and are often not necessary. The typical history is however often not described and other causes should be considered. Treatment by closed means is virtually always successful. The literature suggests ‘hyper-pronation’ to be more successful than supination [3, 4]. Generally, forearm rotation into full pronation and supination with the elbow in flexion and extension will reduce all instances. A palpable click may be felt if a hand is held over the elbow during the reduction manoeuvre and observation of the child for a period will confirm symptom resolution. Ordinarily immobilisation is unnecessary in typical first-time cases. However, if not clearly resolved rest in a sling will result in return to normal use within a day. Advice on prevention by avoiding pulling the arm is worth stressing but recurrent cases even when parents are conscientious are not

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unusual. Advice on how to perform the reduction manoeuvre to parents of those whose children experience recurrent episodes can help reduce visits to primary care physicians or emergency departments. There is a strong tendency for recurrent cases to cease, often by school age, as the children grow up. Case Study 1 Jasmine

Jasmine is a 4-year-old girl who was holding her dad’s hand while crossing the road. She tripped on the pavement and fell. Dad pulled on her hand to prevent her falling. She immediately cried and was not able to use her hand. She kept the arm internally rotated and the elbow straight. Although she was able to move her fingers, she was not using her hand. She was taken to the local hospital and was examined by a doctor who made the correct diagnosis of pulled elbow. He also offered to reduce it. He explained to the parents that she may cry when the pulled elbow is reduced but she should settle and be able to use it within a couple of hours. Parents agreed. The doctor explained to Jasmine what he would do in a way that she could understand then while he was holding the elbow in 90 degrees’ flexion, he rotated the forearm and a clunk was felt. Jasmine cried a bit but she quickly settled. Less than half an hour later, Jasmine was able to use her limb as normal.

2.2

Supracondylar Fracture of the Humerus

Fracture to the area of slim bone above the humeral condyles at the distal end of the humerus is amongst the most common fracture encountered in childhood [5]. Apart from a minority most of these fractures result in the distal portion being somewhat extended and the recognition that the opposite elbow usually has demonstrable hyperextension suggests that a fall onto an outstretched arm with that characteristic is the mechanism of injury. At initial assessment the value of the elbow extension test has been studied in children as well as adults. Patients who cannot fully extend their elbow after injury should be referred for radiography, as they have a nearly 50% chance of fracture. For those able to fully extend their elbow, radiography can be deferred if the practitioner is confident that an olecranon fracture is not present [2]. Patients who do not undergo radiography should return if symptoms have not resolved within 7–10 days. There are also those who present with elbow pain and some restriction of movement having fallen and yet no overt fracture or crack is seen on radiographs. In such images elevation of the fat pads at the elbow should be looked for (Fig. 3). The fat pads normally lie on the bone of the distal humerus at the front and back and when displaced away from their normal position by elbow joint swelling or an effusion become evident on the radiograph, producing a triangular shaped area of lesser density at the front or back of the supracondylar region. A quarter of such elbows may have occult fractures to the supracondylar region and if the symptoms correspond they are best treated as if a crack is present [6] and

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Fig. 3  Elbow x-ray showing anterior and posterior fat pad sign with no visible fracture

afforded support; generally, by a gutter splint or long arm plaster ‘back-slab’ or cast, a sling seems less effective [7, 8]. This approach does not usually need further imaging on follow-up [9]. A spectrum of injury is recognised from the most severe; those where displacement results in loss of contact between the fracture surfaces, through those where the extending force stops with the fracture surfaces maintaining some contact but leaving an extended angulation, to those where the force falls short of actual displacement but nevertheless cracks the bone. This forms the basis of a widely used classification described by Gartland which with modification [10] provides a useful guide to the treatment: the undisplaced Gartland I fracture being protected in a long arm cast or backslab, supported in a sling; the Gartland II, extended but still with some bony contact may be treated by manipulative improvement and usually percutaneous pins and cast, though for the younger child without undue elbow swelling, treatment in a cast may be used if the elbow can be flexed sufficiently—these need close monitoring. For the ‘off-ended’ Gartland III the orthopaedic surgeon will need to achieve reduction, most often done by closed manipulation but open if needed, and stabilisation with percutaneous K-wires [11] (Figs. 4 and 5). Inherent in the management of this fracture is the necessity to assess the neurovascular integrity. The hand needs to be perfused—‘pink’ and the pulses checked. The children’s game ‘rock-paper-scissors’ combined with the ‘OK’ sign can be used as a simple aide-memoir for the nerve supply to the hand and forearm: the median nerve creates the “rock position” of the pronated fist; the radial nerve extends the wrist and hand forming the “paper position”; and the ulnar nerve creates the “scissor position”, by clawing the ring and little fingers and spreading the index and middle and adducting the thumb and flexing the interphalangeal joint [12]. Creating the “OK sign” is a specific test for the anterior interosseous nerve, a branch

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Fig. 4  Gartland’s classification of supracondylar fracture

of the median nerve (Fig. 6). Efforts need to be made to identify nerve injuries and distinguish those at presentation from iatrogenic neurological deficits. Reassessment allows for a change in these aspects to be identified and addressed. Most neurological injuries recover given time but this needs to be confirmed. Healing in the supracondylar area is usually reliable and the cast and or pins are retained for 3 weeks. The child will then be left free to mobilise the elbow, perhaps with the initial support of a sling. Routine physiotherapy does not appear to be warranted [13] but swimming seems worth encouraging, especially during the initial weeks when activities with risk of further injury are inadvisable. The movement recovers mostly in the initial 6 weeks and then more slowly over most of a year [14]. Children may not necessarily regain the ‘hyperextension’ portion. Poorer outcomes occur when the elbow carrying angle is not retained and cubitus varus results (Fig. 7). Although this does not necessarily affect joint function the appearance can be unappealing but since correction by osteotomy has drawbacks a moderate degree of altered alignment may be acceptable. Overall lasting satisfaction following treatment for supracondylar fractures in children is generally good especially if a near symmetrical carrying angle in coronal plane alignment is achieved. Satisfaction is potentially less for the Gartland II injury and interestingly may not necessarily be improved by managing a greater proportion of these surgically [15].

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Fig. 5  Supracondylar humeral fracture that was treated with closed reduction with 3  k-wire stabilisation Fig. 6  The “OK” sign to test for the AIN motor supply for the long flexors of the index and thumb. The clinical photograph was taken for a child with neurovascular injury following a supracondylar humeral fracture. The nerve fully recovered as the child is able to perform the “OK” sign. There is still residual skin discolouration secondary to autonomic nervous system

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Fig. 7  Gunstock (varus deformity) following a supracondylar fracture. It can be corrected surgical for cosmetic reasons mainly

2.3

Elbow Dislocation

Elbow dislocations in children are not common but can occur, usually in the second decade of life. The most common, a posterior dislocation, results when forces disrupt the medial constraints, produce valgus instability and the proximal radius and ulna displace posteriorly or posterolaterally. This needs to be distinguished from a humeral extension supracondylar fracture. The distortion of the normal triangular relationship found on palpation of the olecranon tip to medial and lateral condyles in the dislocation may help but radiographs will clarify. Associated fractures to the medial epicondyle, radial head and neck and coronoid process or other adjacent structures should be looked for and occur in at least half of cases (Fig. 8). Successful treatment by closed reduction under good sedation or anaesthesia is usually successful. However, confirmation of a concentric reduction without interposed tissues or neurovascular injury is imperative and appropriate management of any associated fracture is key. The most frequent important complication of elbow dislocation is stiffness and accordingly immobilisation in a splint ought to be limited to a period sufficient to allow the initial swelling and discomfort to settle and then mobilisation commenced. Results in children are usually good [16] and recurrent elbow dislocation is rare but may need specialist management.

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Fig. 8  Plain radiograph showing a dislocated elbow

2.4

Lateral Condyle Humeral Fracture

Fractures of the lateral condyle region either cross the distal humeral physis (Milch I) or travel along it for a short distance to the trochlea (Milch II) and occur via two proposed mechanisms, ‘push-off’ or ‘pull-off’ and result in differing degrees of displacement (Fig. 9). In the initial stage the fracture is essentially undisplaced and the articular cartilage remains intact; in the second stage the fracture extends into the joint and displacement occurs; and the third stage is distinguished by significant fragment rotation (Jakob classification) (Fig. 10). In contrast to the supracondylar fracture, the lateral condyle fracture may not result in elbow deformity although swelling laterally with local tenderness will be helpful features. Any degree of displacement, unless minimal (often referred to as under 2 mm) in any radiograph, AP, Lateral or Oblique views [17] ought to be managed by reduction, usually by open procedure, and stabilisation, most often by K-wires. Those judged to be un-displaced may be treated in a long arm cast but management ought nevertheless to be under orthopaedic surgeons who will use early and follow-up radiographs to confirm the undisplaced nature of the injury, and ensure any change in position is recognised [18]. Should anxiety remain as to the degree of displacement, oblique x-rays are useful, as may be examination under anaesthesia with arthrogram [19, 20] and pinning of cases where stability is in doubt (Fig. 11). In general, if fixed these fractures are solved and a low threshold for operative treatment is applicable.

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A. MILCH TYPE I

B. MILCH TYPE II

Fig. 9  Milch classification of humeral lateral condyle fracture of the elbow. It is commonly misconceived that Milch I is Salter type IV fracture and Milch II is Salter type II fractures. In fact, all lateral condyle fractures involve the growth plate and should be considered to be Salter Harris IV. Milch II is more unstable and often associated with elbow dislocation (Fracture dislocation)

Fig. 10  Jakob’s classification of humeral lateral condyle fracture. Jakob I—There is an articular cartilage bridge (incomplete fracture). There is no displacement or minimal displacement of less than 2 mm displacement. Jakob II—complete fracture and the displacement is usually more than 2 mm. Jakob III—These are mal-rotated and displaced

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Fig. 11 Lateral condyle fracture that was treated with open reduction and stabilisation using K-wires

Since the injury involves the joint, once healed there may be a period of elbow stiffness which is slower to resolve than applies to the fracture in the supracondylar area [21]. A number of elbows may have a degree of lasting restriction.

2.5

Medial Epicondyle Fracture

Injuries to the medial epicondylar apophysis are commonly associated with dislocation of the elbow mostly overtly though also as an implied incident. At times the avulsed epicondylar fragment may be incarcerated within the elbow joint at reduction which may have occurred in the course of the injury or following management of an elbow dislocation (Fig. 12). Apart from elbow dislocation, the most plausible mechanisms described include avulsion and extension (valgus force) and a check for associated injures to the radial neck and olecranon is prudent. Occasionally direct muscle action in throwing may produce the injury. The most obvious surgical indication is when the medial epicondylar fragment is trapped within the joint and needs retrieval and is then generally treated with fixation (Fig. 13). Where the fragment is extra-articular, non-operative treatment in a cast may be employed and debate continues on the role of surgical fixation. Operative management appears to increase the proportion where bony union occurs yet a fibrous union can be entirely compatible with normal function [22, 23]. Paradoxically, surgery may increase the proportion who subsequently report some degree of elbow discomfort [24]. There are reports of elbow instability in cases of fibrous union in those who participate in throwing sports, and such pursuits by an individual may merit consideration in treatment options. Fracture of the Medial Condyle is to be distinguished from the epicondylar injury described above. Fracture to the medial condyle is uncommon but as the injury extends to involve the joint, in an analogous fashion to the lateral condyle fracture, a similar approach with fixation for those showing any displacement is warranted.

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Fig. 12  Medial epicondyle fragmented that is entrapped in elbow joint after reducing the dislocation (same patient in Fig. 8)

2.6

Radial Neck Fracture

In children, fractures to the proximal radius are usually physeal or metaphyseal and are termed radial neck fractures (Fig.  14). True radial head fractures are rare. Findings include tenderness and swelling over the radial head or neck region and pain exacerbated by passive forearm rotation—pronation and supination; less so by flexion and extension movements. Pain may be referred to the wrist and the physician will be further misled if only distal radiographs are undertaken. Some fractures appear to occur in association with elbow dislocation—either as the joint dislocates or as spontaneous reduction occurs, but more usually angular fractures occur due to valgus forces when the child falls onto the outstretched hand [25]. The fracture is produced by valgus injury and if the force is sufficient may be associated with olecranon fractures or medial epicondyle avulsion. The size of the force has a significant influence on the outcome. Older children and delayed treatment are also detrimental factors to a good outcome. Treatment options include immobilisation alone for those less than 2 mm translation or 30 ° angulation [26]. Displacement beyond this degree requires consideration of: immobilisation following closed reduction by manipulation [27]; percutaneous guide wire reduction; intramedullary wire reduction; or open reduction with or without fixation. By and large open reduction is associated with poorer results though this may be due to the related soft tissue injury rather than the surgical impact. Parents ought to be warned of potential residual stiffness especially in

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Fig. 13  Medial epicondyle fracture that was treated with a cannulated screw Fig. 14  Radial head fracture

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those cases with unfavourable aspects. After initial improvement if full movement is not regained, very little progress is likely after the initial 6 months.

2.7

Olecranon Injury

Minimally displaced fractures of the olecranon can be treated closed in a cast somewhat extended. Displaced fractures are better managed by operative reduction and stabilisation along tension-band principles employing K-wires or a screw and tension suture or wire in older children (Chap. 1 “Orthopaedic Terminology” in Fig. 10). The irritation from the wire however often requires its removal. Some oblique fractures due to a shear mechanism may be secured by oblique screws. Fracture combinations should be anticipated, common associations are involvement of the olecranon and medial epicondyle or the olecranon and the radial neck. True apophyseal separation of the olecranon tip is rare but chronic forms associated with sports employing repetitive elbow extension occur and cessation of the activity to allow healing is usually successful.

2.8

Monteggia Lesion

The Monteggia lesion is a fracture of the ulna and a dislocation of the radial head. The radial head dislocated in a variety of directions in association with various degrees of failure of the ulna: plastic deformation, greenstick or complete fracture and now different variations are acknowledged as ‘Monteggia equivalents’ including isolated radial head dislocation (Fig.  15). Recognition of the combination is

Fig. 15  Monteggia fractures: image with plastic deformation of the ulna; image 2 with greenstick fracture of the ulnar and image 3 is Monteggia equivalent with olecranon fracture

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important as is the form of injury sustained by the ulna. Delayed treatment or missing the injury makes for problematic management [28]. In the acute setting management is usually straightforward since ordinarily the radial head dislocation can be reduced, usually by closed means, if any deformity in the ulna is fully addressed. The stability of reduction depends on the degree of failure to the ulna, its alignment and length needs to be restored and retained till healed, and accordingly is an important guide to management; closed reduction and casting for plastic deformation and greenstick ulnar fractures; intramedullary pinning for transverse or short oblique fractures; and plating reserved for long oblique or comminuted fractures [29]. The prospect of a chronic Monteggia Lesion and its poor outcome is minimised by a meticulous approach and close initial follow up to ensure reduction maintained.

2.8.1 Congenital Elbow Conditions Two congenital abnormalities occur with any significant frequency, while both are rare they may not be recognised until after infancy. Congenital radial head dislocation, is a rare condition although the most common congenital elbow abnormality and is often bilateral (Fig.  16). A number of cases may be inherited and the condition can be associated with other developmental abnormalities. Functional issues may not be noticed initially but as the child’s activities gradually increase later presentation occurs. Parents may notice a fullness or prominence from the radial head and restricted movement. The condition needs

Fig. 16  congenital dislocation of the radial head

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Fig. 17  congenital radioulnar synostosis

to be differentiated from a chronic, missed or ‘neglected’ traumatic radial head dislocation. The radiographs in the congenital cases show dysplastic features that helps make the distinction. Treatment of this congenital condition is usually non-­operative. Reconstruction for the chronic traumatic case may be offered but the result of this approach in the congenital examples have generally been less encouraging. Congenital radioulnar synostosis, is a developmental fusion of the proximal ends of the radius and the ulna (Fig. 17). This may be bilateral but is usually isolated. The functional result depends on the position of fixed rotation that the forearm demonstrates. There may be a delay in the appreciation of the restriction due to adaption by the child using the shoulder. Functional limitation may arise, usually due to an excessively pronated position, for which shoulder movement cannot compensate. An osteotomy to reposition the forearm rotational position is the usual approach employed [30].

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Osteochondrosis and Osteochondritis Dissecans

There are two distinct elbow conditions which might be considered together and the contrasting differences noted. Both are idiopathic and affect the distal humeral physeal region with bone changes and associated inflammatory (osteochondritis) aspects resulting in epiphyseal irregularity. Panner’s disease is an osteochondrosis affecting the capitellum of the distal humerus (Fig. 18). It is typically seen in children, usually boys, aged 5–12 years

Fig. 18  Panner’s disease. Red arrows point to the areas of fragmentation

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and occurs spontaneously. It is to be distinguished from the osteochondritis dissecans seen in the teenage years since Panner’s disease occurs in a younger age group and does not relate to activities or result in loose bodies. Panner’s disease presents with intermittent pain and stiffness and sometimes swelling of the elbow lasting for several weeks, though some have much longer duration. Symptoms may be relieved by rest and aggravated by activity and palpation may reveal local tenderness. Range of movement is typically reduced especially with a lack of full extension. The diagnosis is usually made radiologically from the capitellar changes which range from sclerosis to fragmentation as patchy bone resorption occurs, changes seen as similar to Perthes Disease of the hip but without its malign effects. A recent systematic review [31] confirms that Panner’s Disease is a benign self-limiting condition and the bone changes heal in time usually with complete recovery. Accordingly, active treatment is not usually required and management includes advice and explanation. Adolescent Capitellar Osteochondritis Dissecans (OCD) of the capitellum is a localised form of avascular bone necrosis often related to repeated minor trauma. The condition presents around 12–16 years of age with a history of stiffness, pain and locking or catching symptoms to the elbow. Radiographs show localised capitellar involvement with often a loose fragment, flattening of a portion of the humeral capitellum and cyst formation (Fig. 19). MRI may help to assess if a fragment has an intact articular surface in which conservative treatment may be followed or is loose and has a less certain prognosis. Sometimes there are corresponding radial head changes. Management is based on the clinical findings with limitation of activities until healed but may include fragment

Fig. 19  Capitellar OCD

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removal if mechanical symptoms occur. Debridement and drilling of affected areas is of uncertain value and ostechondral grafting has been reported [32]. Around half of cases have joint stiffness and degenerative changes with radial head enlargement as adults.

4

Chronic Paediatric Elbow Conditions

A number of children, particularly adolescents, may present with a somewhat extended history of painful elbow on a background of pursuit of sport or other activities at various levels of intensity.

4.1

Ulnar Collateral Ligament (UCL) Injuries

These injuries are unusual until after closure of the medial epicondylar apophysis and become more prevalent in adolescent athletes as muscle mass and throwing force increase. The UCL provides valgus stability for the elbow joint and plays an important role in the biomechanics of throwing in sports such as baseball, javelin, hockey and racquet sports. In America, studies have shown that the incidence of these chronic tension injuries can be kept low if guidance on frequency of training and playing is followed [33]. Athletes present with progressive medial elbow pain related to their throwing activities which cause a decrease in power and control. Acute injuries may present with sudden medial elbow pain sustained during a particular throw and some may describe a ‘pop’ following which further throwing was not possible. The most notable findings are medial elbow tenderness and swelling. Tenderness should be sought, especially a couple of centimetres distal to the medial epicondyle. UCL tenderness can be differentiated from flexor pronator tendonitis as the latter is aggravated by actively resisting forearm pronation. Loss of elbow range of motion may be present and pain may be reproduced by asking the patient to clench their fist and valgus stress testing should be used to look for instability of the joint as compared to the other side. Radiographs may identify increased distal humeral bone density, hypertrophy of the bone, somewhat advance bone maturity, occasional avulsion fractures, ligament calcification but also helps rule out other causes of elbow pain [34]. Based on the clinical findings in individual cases, MRI scanning can be employed. Ultrasonography can allow a rapid simple assessment of the UCL if the clinical distinction between tendon rupture and tendon sprain injuries is unclear. Most cases with such medial elbow conditions will respond to conservative management: rest, judicious use of anti-inflammatories and physiotherapy though this may take some 3–6 months. Once pain and swelling have resolved, gradual return to throwing activities can be introduced. Surgical management is not usually required and usually restricted to those with problematic chronic instability and recurring pain and laxity despite a prolonged conservative approach.

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Medial Epicondyle Apophysis

These come under the generic headings of “little league elbow” or “thrower’s elbow” and is a common presentation in those participating in frequent throwing activities, such as baseball pitchers. The medial epicondyle apophysis is a secondary ossification centre which gives attachment to the ulnar collateral ligament of the elbow, pronator teres and acts as a tendon insertion site for the forearm flexors, flexor carpi radialis, flexor carpi ulnaris, flexor digitorum superficialis and palmaris longus. During the throwing motion, valgus stress is placed on the elbow and tension is generated to the medial epicondyle, medial epicondylar apophysis and ulnar collateral ligament complex; on the lateral side compression of the capitellum and radial head is experienced. Repeated stress may result in an overuse injury where tissue breakdown exceeds tissue repair. As the medial epicondyle is the last site to fuse with the humeral epiphysis the excessive valgus stress from throwing can result in prominent growth of the area. Symptoms include pain with throwing and decreased speed, accuracy and distance in the activity. Ascertain the throwing activity, its frequency, volume and types of throws as well as the level of attainment achieved. A history of chronic and progressive pain is usual, though an acute event may precipitate presentation. The elbow soft tissue structures are palpated including the ulnar collateral ligament, best felt with the elbow in 50–70 degrees of flexion. The strength of the relevant muscle groups should be assessed and neurological examination of the ulnar nerve is pertinent. A valgus force on the elbow in 20–30 degrees flexion is compared to the uninjured side when looking for instability which suggests an ulnar collateral ligament injury. This forms the valgus stress test. Radiographs may be normal or show widening of the medial condyle epiphysis. MRI scanning can identify oedema of the bone marrow, around the medial epicondylar area. If the initial symptoms have been disregarded, then persistence in the activities may lead to irregularity or fragmentation of the medial epicondyle. Activity modification is key in the management of the condition. Cessation of the activity usually allows resolution which can then be followed by gradual reintroduction. Physiotherapy can be helpful in individual cases as is attention to technique.

4.3

 lecranon Impingement Syndrome (Valgus O Extension Overload)

Another condition described in young athletes performing activities with repeated extension of the elbow, such as throwing activities, racquet sports, gymnastics, boxing and swimming, resulting in the dissipation of the force when extending the elbow by a resulting shear stress posteriorly. This repeated valgus extension force overloads the area of the olecranon tip as it repeatedly impinges into the olecranon fossa resulting in  local inflammation and can proceed to involve the cartilage and bone. Occasionally osteophytes may form in the posteromedial tip of the olecranon which can impinge on the trochlea during elbow extension, these may form ‘loose’ bodies. The condition presents with pain in the posteromedial aspect of the elbow especially when throwing in the deceleration phase as the elbow straightens, such as

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during serving in overhead racquet sports, and power of delivery is reduced. Swelling of the elbow, mechanical symptoms and tenderness may be present. There may also be stiffness with lack of full extension, valgus instability because of collateral ligament insufficiency may be present. The valgus extension load test may identify pain in the posteromedial aspect as the slightly flexed elbow is forced into extension by the clinician whilst a valgus force is applied. Radiographs are usually normal but should be scrutinised for early osteophytes at the olecranon or evidence of ‘loose’ bodies. MRI scanning can be useful especially if loose bodies or chondral injuries to the trochlea are suspected and radiographs are normal. Most can be managed by advice to rest followed by activity modification and graduated return to activities. Selected cases may benefit from physiotherapy and advice on technique.

References 1. Cheng JC, Wing-Man K, Shen WY, Yurianto H, Xia G, Lau JT, et al. A new look at the sequential development of elbow-ossification centers in children. J Pediatr Orthop. 1998;18(2):161–7. 2. Appelboam A, Reuben AD, Benger JR, Beech F, Dutson J, Haig S, et  al. Elbow extension test to rule out elbow fracture: multicentre, prospective validation and observational study of diagnostic accuracy in adults and children. BMJ. 2008 Dec;337(dec09_1):a2428+. 3. Bexkens R, Washburn FJ, Eygendaal D, van den Bekerom MP, Oh LS. Effectiveness of reduction maneuvers in the treatment of nursemaid’s elbow: a systematic review and meta-analysis. Am J Emerg Med. 2017 Jan;35(1):159–63. 4. Krul M, van der Wouden JC, Kruithof EJ. Van Suijlekom-smit LWW, Koes BW. Manipulative interventions for reducing pulled elbow in young children. Cochrane Database Syst Rev. 2017 Jul;7:CD007759. 5. Abzug JM, Herman MJ. Management of supracondylar humerus fractures in children: current concepts. J Am Acad Orthop Surg. 2012 Feb;20(2):69–77. 6. Skaggs DL, Mirzayan R. The posterior fat pad sign in association with occult fracture of the elbow in children. J Bone Joint Surg Am. 1999 Oct;81(10):1429–33. 7. Ballal MS, Garg NK, Bass A, Bruce CE.  Comparison between collar and cuffs and above elbow back slabs in the initial treatment of Gartland type I supracondylar humerus fractures. J Pediatr Orthop B. 2008 Mar;17(2):57–60. 8. Oakley E, Barnett P, Babl FE. Backslab versus nonbackslab for immobilization of undisplaced supracondylar fractures: a randomized trial. Pediatr Emerg Care. 2009 Jul;25(7):452–6. 9. Al-Aubaidi Z, Torfing T. The role of fat pad sign in diagnosing occult elbow fractures in the pediatric patient: a prospective magnetic resonance imaging study. J Pediatr Orthop B. 2012 Nov;21(6):514–9. 10. Alton TB, Werner SE, Gee AO. Classifications in brief: the Gartland classification of supracondylar humerus fractures. Clin Orthop Relat Res. 2015 Feb;473(2):738–41. 11. Mulpuri K, Wilkins K. The treatment of displaced supracondylar Humerus fractures: evidence-­ based guideline. J Pediatr Orthop. 2012 Sep;32(Suppl 2):S143–52. 12. Davidson AW. Rock-paper-scissors. Injury. 2003 Jan;34(1):61–3. 13. Schmale GA, Mazor S, Mercer LD, Bompadre V. Lack of benefit of physical therapy on function following supracondylar humeral fracture: a randomized controlled trial. J Bone Joint Surg Am. 2014 Jun;96(11):944–50. 14. Spencer HT, Wong M, Fong YJ, Penman A, Silva M. Prospective longitudinal evaluation of elbow motion following pediatric supracondylar humeral fractures. J Bone Joint Surg Am. 2010 Apr;92(4):904–10. 15. Sinikumpu JJJ, Victorzon S, Pokka T, Lindholm ELL, Peljo T, Serlo W. The long-term outcome of childhood supracondylar humeral fractures: a population-based follow up study with

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a minimum follow up of ten years and normal matched comparisons. Bone Joint J. 2016 Oct;98-B(10):1410–7. 16. Josefsson PO, Johnell O, Gentz CF. Long-term sequelae of simple dislocation of the elbow. J Bone Joint Surg Am. 1984 Jul;66(6):927–30. 17. Kurtulmuş T, Sağlam N, Saka G, Avcı CCC, Uğurlar M, Türker M. Paediatric lateral humeral condyle fractures: internal oblique radiographs alter the course of conservative treatment. Eur J Orthop Surg Traumatol: orthopédie traumatologie. 2014 Oct;24(7):1139–44. 18. Pirker ME, Weinberg AM, Höllwarth ME, Haberlik A. Subsequent displacement of initially nondisplaced and minimally displaced fractures of the lateral humeral condyle in children. J Trauma. 2005 Jun;58(6):1202–7. 19. Lee DHH, Han SBB, Park JHH, Park SYY, Jeong WKK, Lee SHH. Elbow arthrography in children with an ulnar fracture and occult subluxation of the radial head. Journal of Pediatric Orthopaedics Part B/European Paediatric Orthopaedic Society, Pediatric Orthopaedic Society of North America. 2011 Jul;20(4):257–63. 20. Marzo JM, d’Amato C, Strong M, Gillespie R. Usefulness and accuracy of arthrography in management of lateral humeral condyle fractures in children. J Pediatr Orthop. 1990;10(3):317–21. 21. Bernthal NM, Hoshino MM, Dichter D, Wong M, Silva M. Recovery of elbow motion following pediatric lateral condylar fractures of the humerus. J Bone Joint Surg Am. 2011 May;93(9):871–7. 22. Kamath AF, Baldwin K, Horneff J, Hosalkar HS.  Operative versus non-operative management of pediatric medial epicondyle fractures: a systematic review. J Child Orthop. 2009 Oct;3(5):345–57. 23. Mehlman CT, Howard AW. Medial epicondyle fractures in children: clinical decision making in the face of uncertainty. J Pediatr Orthop. 2012 Sep;32(Suppl 2):S135–42. 24. Wilson NI, Ingram R, Rymaszewski L, Miller JH. Treatment of fractures of the medial epicondyle of the humerus. Injury. 1988 Sep;19(5):342–4. 25. Pring ME.  Pediatric radial neck fractures: when and how to fix. J Pediatr Orthop. 2012 Jun;32(Suppl 1):S14–21. 26. Neher CG, Torch MA. New reduction technique for severely displaced pediatric radial neck fractures. J Pediatr Orthop. 2003;23(5):626–8. 27. Augustithis GA, Huntley JS. Closed reduction of paediatric radial neck fractures. Ann R Coll Surg Engl. 2015 May;97(4):316–7. 28. David-West KS, Wilson NI, Sherlock DA, Bennet GC.  Missed Monteggia injuries. Injury. 2005 Oct;36(10):1206–9. 29. Fernandez FF, Egenolf M, Carsten C, Holz F, Schneider S, Wentzensen A. Unstable diaphyseal fractures of both bones of the forearm in children: plate fixation versus intramedullary nailing. Injury. 2005 Oct;36(10):1210–6. 30. Simcock X, Shah AS, Waters PM, Safety BDS. Efficacy of Derotational osteotomy for congenital radioulnar synostosis. J Pediatr Orthop. 2015 Dec;35(8):838–43. 31. Claessen FM, Louwerens JK, Doornberg JN, van Dijk CN, Eygendaal D, van den Bekerom MP. Panner’s disease: literature review and treatment recommendations. J Child Orthop. 2015 Feb;9(1):9–17. 32. Churchill RW, Munoz J, Ahmad CS.  Osteochondritis dissecans of the elbow. Curr Rev Musculoskelet Med. 2016 Jun;9(2):232–9. 33. Francis R, Bunch T, Chandler B. Little league elbow: a decade later. Phys Sportsmed. 1978 Apr;6(4):88–94. 34. Adams JE. Injury to the throwing arm. A study of traumatic changes in the elbow joints of boy baseball players. Calif Med. 1965 Feb;102:127–32.

Paediatric Hand and Wrist Anastasios Chytas and Gillian Smith

1

Congenital Hand Deformities

The development of the upper limb begins during the fourth week of in utero life, when a limb bud consisting of undifferentiated mesenchymal cells encased in ectoderm develops. By 9 weeks the bud has developed into an arm and hand plate with identifiable digits, and by 12 weeks the digits have completely separated. Further development is by size increase in the fully formed structures. Growth and differentiation are under the control of signal regions at the tip of the developing limb with complicated interactions and feedback systems along three axes: proximal to distal, ulnar to radial and dorsal to ventral. There is a complicated interaction between genes producing transcription factor proteins, which drive limb elongation and patterning. Anomalies in these complex processes result in anomalies in limb development. These can be the result of genetic mutation, interruption of a pathway at molecular level, or gross environmental insult. The aetiology of such insults can be environmental such as radiation, infection, and chemicals (including drugs). Congenital hand deformities were traditionally classified using the “Swanson classification” [1]. This was based on morphology but had problems as many conditions lay in more than one category. With greater understanding of limb developmental biology, the Oberg, Manske and Tonkin (OMT) classification has become the internationally recommended classification, although this still does not fit perfectly with what is seen and does not always dictate subsequent management [2]: 1. Malformations 2. Dysplasias

A. Chytas (*) Royal Manchester Children Hospital, Manchester, UK e-mail: [email protected] G. Smith Great Ormond Street Children Hospital, London, UK © The Author(s), under exclusive license to Springer Nature Switzerland AG 2021 S. Alshryda et al. (eds.), Pediatric Orthopedics for Primary Healthcare, https://doi.org/10.1007/978-3-030-65214-2_20

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3. Deformations 4. Syndromes The commonest and most important congenital conditions are described below in their morphological form:

1.1

Polydactyly

Polydactyly translates literally as “many fingers”. It represents the commonest form of congenital hand deformity. It is polygenic but the pattern of transmission appears autosomal dominant. When more than two limbs are affected, it is likely to be related to a syndrome. Polydactyly is broadly categorised, in order of frequency, as postaxial (ulnar), preaxial (radial), or central [3].

1.2

Post Axial Polydactyly

Post axial duplication is duplication of the little finger (Fig. 1). It is eight times more common than any other polydactyly, often bilateral and is more common in Africans (1:150), then Asians and less common in Caucasians (1:300), where it is more commonly syndromic. Post axial polydactyly is classified as follows (Stelling Classification). Type 1 Soft tissue connection with hand, no bony connection. Type 2 Duplicate digit/part digit articulating with normal/bifid metacarpal/phalanx. Type 3 Duplicate digit including duplicate metacarpal. Careful examination should include the orientation and active and passive ROM of the remaining fingers and inspection of the feet. Radiographs may be required for types 2 and 3.

Fig. 1 Postaxial polydactyly (type 1)

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Type 1 duplications can be managed simply with deletion/excision under local anaesthetic if less than 3 months of age, older than this it becomes difficult to do safely without general anaesthetic. Type 2 and 3 require reconstruction of functional components including ulnar collateral ligament, bony resection, tendon realignment and muscle transfer.

1.3

Central Polydactyly

Central polydactyly refers to a duplication of the index, long, or ring finger (Fig. 2). This is the least common polydactyly. It is commonly associated with a syndactyly and is usually a genetic condition. Central polydactyly is frequently bilateral but not necessarily symmetrical, with ring finger duplications the most common, followed by middle and index. With ring finger duplications there are often associated fifth toe duplications. Diagnosis is clinical and radiographic. Clinical assessment should include ROM, posture and function. Frequently there is a restricted range of motion in the middle and ring fingers with shared tendons with the additional digit and this will not be improved by surgery. These fingers usually have abnormal vasculature and sometimes three digits will share a single vessel which will make separation risky. Exact procedures are case specific and may vary from simple ray resection with intermetacarpal ligament reconstruction to complex redistribution of vasculature, tendinous, ligamentous  and skeletal elements with a resultant limitation in movement. Fig. 2  Plain x-ray shows a child with central polydactyly

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Pre-axial Polydactyly

Pre axial polydactyly is thought of as a duplication of the thumb, but, in reality is more like a split thumb with each of the duplicates incomplete (Fig. 3). It has an incidence of 0.8 per 1000 live births and may have a dominant inheritance pattern. There is a female to male ration of 2.5:1 and is more common in Asians. It can have syndromic associations especially when bilateral but is often an isolated anomaly [4]. Clinical examination needs to assess for the dominant thumb, which is usually the ulnar duplicate and for ROM and deviation at all joints of both duplicates. Radiographs are mandatory. Chromosome fragility testing for Fanconi’s anaemia is recommended. Classification of preaxial polydactyly is in fact radiographic and is based on the presence of complete/incomplete duplication of each phalanx (Wassell Classification) [5]. Type 1. Bifid DP. Type 2. Duplicate DP. Type 3. Bifid PP. Type 4. Duplicate PP—most common. Type 5. Bifid metacarpal.

Fig. 3 Pre-axial polydactyly

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Type 6. Duplicate metacarpal. Type 7. Triphalangism in either or both duplicates. Surgical management is usually delayed until after 12  months as this reduces overall anaesthetic risk, allows for full and thorough preoperative assessment and eases surgery, thanks to a larger hand size. The surgical principles are to produce a well-aligned, reasonably sized and stable thumb before the age of two whilst preserving the epiphyseal plates. Despite this, the reconstructed thumb is always smaller than the normal, contralateral thumb. Overall, joint alignment is more important than joint motion to a successful outcome, with stiffness at one thumb joint being functionally well tolerated. Surgical treatment depends largely on classification but mainly involves reconstruction of one thumb using components from the other. Occasionally it involves using half components from each duplicate (Fig. 4).

1.5

Syndactyly

Syndactyly, literally translated as—together “Syn”, finger “dactyl” is one of the most common congenital hand deformities at one per 2000 live births. It has multifactorial aetiology but some forms follow an autosomal dominant pattern. Syndactyly arises from a failure of apoptosis (Fig. 5). It is bilateral in 50% of cases, Caucasians have the highest racial predilection and there is a 2:1 male to female ratio. Although often non syndromic, associated syndromes include Poland’s, Trisomy 21, Carpenters, Holt-Oram, Pfeiffer’s and Apert’s.

Fig. 4  Wassel Classification of preaxial polydactyly

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Fig. 5  Simple syndactyly

Fig. 6  Complex syndactyly

The most common web involved is the third web (50%,) on the hand and the second web on the foot, with the first, second, and fourth webs of the hand accounting for 5%, 15% and 30% respectively. Syndactyly is classified, as either complete, with fusion at least up to the DIPJ, or incomplete. It is further classified into skin only syndactyly, termed simple, or as complex, involving both bony fusion and skin (Fig. 6). A final category of “complicated” is reserved for cases where both syndactyly and polydactyly are present [6].

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Clinical examination should assess the number of rays involved and completeness of syndactyly, the range of motion in the fingers, the identification of syndromic features and a basic assessment of upper limb function. An X-ray is mandatory to evaluate bony involvement but frequently will appear normal when clinically it is obvious that there is a skeletal join but this is still cartilaginous. Syndactyly release is aimed at between 12 and 18 months unless involving the border digits, where release is around 6  months of age to allow for differential growth. Relative contraindications include a minor degree of webbing (which is not cosmetically or functionally significant) and hypoplastic digits, where release may be functionally disadvantageous.

1.6

Constriction Ring Syndrome

These may consist of multiple amputations in multiple limbs or tight rings with or without lymphoedema and sometimes with syndactyly with proximal sinuses (acrosyndactyly) (Fig. 7). They are common and are associated with twin pregnancies and operative interventions during pregnancy. There is no genetic association and patients are usually otherwise normal—cleft lip and palate is rarely associated. Where there is vascular compromise or lymphoedema, urgent release is required. Otherwise, resection of rings later, acrosyndactyly release or free toe transfer where appropriate give good, but still aesthetically abnormal results. Frequently, these patients require multiple procedures. Functional results depend on the level of amputation but structures proximal to that level are typically normal.

Fig. 7  Constriction ring syndrome (images are courtesy of Dr. Om Lahoti, King’s College Hospital)

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Thumb Hypoplasia

Thumb hypoplasia represents a spectrum of deformity from mild deficiency to complete thumb adactyly (Fig. 8). It is considered as a radial deficiency both in terms of its genetic cause and associated syndromes. The overall incidence is approximately 1 per 100,000 live births, with an equal male to female distribution and is bilateral in 60% of patients. Associated syndromes include Holt-Oram, TAR, VACTERL and Fanconi’s anaemia. Although more severe adactylies or “floating thumbs” are diagnosed early, a mild thumb hypoplasia may go unnoticed by medical teams and parents until fine motor skills begin and the thumb is noted to be ignored or smaller than the contralateral side. All thumb hypoplasia patients should be managed in a multidisciplinary team including paediatricians due to the associations with blood dyscrasias and cardiac abnormalities. Bilateral inspection and palpation are the mainstays of examination comparing thumb size, consistency, joint stability, thenar muscle bulk and first web space. General assessment should include bilateral complete upper limb assessment for associated radial longitudinal deficiencies and to assess ipsilateral index finger active range of motion in case of future pollicisation. Radiography is mandatory and should include bilateral hands and forearms, with more proximal radiographs if anomalies are seen clinically. A normal thumb is not possible to achieve and treatments are aimed at functional improvement. If the CMC is present and stable, reconstruction of the thumb is advocated. If the CMC is absent or unstable, pollicisation is preferred to reconstruction, where culturally it is accepted. Even the best reconstructions in cases where the CMC joint is absent, currently give an inferior result to index finger pollicisation [7].

Fig. 8  Hypoplastic thumb

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Macrodactyly

The term macrodactyly is derived from macros ‘large’ and dactyl ‘finger’ (Fig. 9). It is characterised by enlargement of both soft tissue and osseous elements. Some of these cases are due to vascular malformations. Macrodactyly is now known to frequently be associated with a post zygotic mutation which affects the regulation of growth. Mutations affect the mTor pathway and many of the conditions are now grouped as dysplasias under the umbrella term of PIK3Ca related overgrowth disorders [8]. Macrodactyly is rare. There is a male predilection and most commonly involves the second ray in the foot and the median nerve distribution in the hand. Adjacent digits grow away from the most involved and where four digits are affected, there is usually limb gigantism. Management may alter according to the chronology of the overgrowth (static versus progressive), the localisation of the abnormality to anatomical area and nerve territory, the presence of features suggesting vascular abnormality (compressibility, warmth, thrill,) the degree of osseous involvement or overgrowth and systemic features including those of neurofibromatosis. Comparison radiographs are mandatory to document growth and assess for osseous involvement. Ultrasound, MRI or arteriography may be required to rule out vascular abnormality. Surgery for macrodactyly is often complex and unsatisfactory, yet psychological consequences can be severe. Social counselling and family involvement are imperative. The goals of surgery are: control or reduction in deformity with maintenance of sensibility and function, in as few procedures as possible. Often the best results Fig. 9 Macrodactyly (Alshryda, Jones and Banaszkiewicz, Postgraduate Paediatric Orthopaedics, first Edn, 2013, courtesy of Cambridge University Press)

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are with amputation, since even when longitudinal growth stops, the width continues to increase. Surgical option depends on aetiology, anatomical location, severity and subtype but include soft tissue reduction, epiphysiodesis, neurolysis, nerve excision and grafting, corrective osteotomies, arthodesis and in severe cases, amputation. In general, it is best to perform few definitive procedures, rather than multiple ones. Epiphysiodesis should be performed when the digit is of adult size. MTor inhibitors such as rapamycin hold hope for future management.

1.9

Arthrogryposis

The term arthrogryposis is derived from Arthros ‘joint’ and Gryposis ‘curved or hooked’. Arthrogryposis is a congenital disorder affecting the muscles and is thought to be secondary to abnormality in the anterior horn cells. It is properly termed “Arthrogryposis multiplex congenita” literally meaning a congenital anomaly in the newborn involving multiple curved joints. It is important to note that arthrogryposis is essentially a descriptive term and not an exact diagnosis. It has numerous underlying pathologies which broadly fall into two groups [9]: 1. Amyoplasia, is a sporadically occurring condition with hypoplastic muscles and multiple joint contractures (Fig. 10). 2. The distal arthrogryposis (DA) syndromes are often hereditary and affect hands and feet joints (Fig. 11). The overall incidence of amyoplasia is approximately 1 per 10,000 live births, although distal arthrogryposis is more common with an incidence of 1 in 3000. Clinical examination remains the best modality for establishing the diagnosis with classical presentation involving all four limbs, with multiple rigid joint deformities, defective or absent muscles groups, but normal sensation. Muscle and joint contractures result with cylindrical appearing limbs lacking skin creases. Adducted and internally rotated shoulders, extended knees or elbows and flexed wrists with limited finger movement and thumbs in palms are typical although variants may occur. A Naevus flammeus is frequently present and approximately one third of patients will have systemic involvement involving their respiratory or gastrointestinal tracts. Radiographs show little but may demonstrate poor joint development and a limited muscle shadow with a relatively thick subcutaneous fat layer. Arthrogryposis patients should be managed in a multidisciplinary team involving surgeon, therapist, paediatrician and geneticist. Early use of splints, physiotherapy and serial casting is the mainstay of treatment to encourage passive elbow flexion and wrist.

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Fig. 10  Amyoplasia, note the generalised muscle wasting and lack of skin creases over the feet, knees and elbow

Fig. 11  The distal arthrogryposis

Surgery may be reserved for cases of failed splintage or late presentation and aims to address posture and limb functional achieving improved position of the shoulder, elbow, and wrist and hand posture. Surgery, when indicated, can be staged with initial correction of deformity and contractures with later muscle transfers to achieve elbow flexion, if suitable muscles

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are available. The aim of wrist surgery is to put the wrists in a good position for keyboard use and is usually achieved by carpal wedge osteotomy and tendon transfer, but this is only appropriate if passive elbow flexion is sufficient to reach the mouth with a straighter wrist. Ongoing splintage is required to maintain the correction until skeletal maturity.

1.10

Transverse Arrest and Phocomelia

Transverse arrest is characterized by a complete absence of the upper limb distal to some point, producing an amputation stump (complete type). An absence of one portion the limb (intercalated type) is usually referred to as phocomelia (Fig. 12). Complete arrests can occur at any level but are most common in the forearm at the junction of proximal third and middle third. The incidence is unknown and appears to not be inherited. Intercalated variants (previously most commonly related to maternal thalidomide ingestion; Phocomelia) can present as “complete” with hand attached directly

Fig. 12  Phocomelia, an absence of one portion of the limb

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to torso, “proximal” with normal hand and forearm attached to torso, or “distal” with hand attached to normal upper arm at the elbow. Maternal drug history and clinical examination for classification are the main aims despite classification having no practical role in management thereafter. Prosthetics is the mainstay of treatment with limited possibilities for surgical intervention.

1.11

Brachydactyly

Brachydactyly is a general term (brachy “short” and dactyl “finger”) that refers to disproportionately short fingers or toes usually occurs as a genetic condition. Brachydactyly is therefore a general term and encompasses a number of pathological entities and geneticists have a large number of different types, most of which have good function and don’t require surgical intervention.

1.12

Symbrachydactyly

There is a unilateral underdevelopment of the bone and soft tissue elements of the hand and severely hypoplastic digits or nubbin-like remnants (Fig. 13). Blauth has subdivided this into four descriptive types: 1. Short finger type 2. Cleft hand type: absence of one or more central digits (Fig. 14) 3. Monodactylous type: absence of all digits other than the thumb 4. Peromelic type: in which there is an absence of all digits and metacarpals

Fig. 13 Symbrachydactyly; severely hypoplastic digits or nubbin-like remnants

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Fig. 14  Cleft hand type: absence of one or more central digits

Fig. 15  Chest wall abnormalities (absent pectoralis muscle) in Poland’s Syndrome

Carpal and forearm types have also been described although here there is controversy in distinguishing from transverse arrest. The severity of digital shortening is variable with mild shortening only apparent due to the loss of normal cascade and sometimes associated syndactyly. Careful examination including the length and function of brachydactylous digits and presence of remnant nail plates is mandatory for diagnosis. Examination of the shoulder, and chest wall are required as chest wall abnormalities are likely part of Poland’s Syndrome (Fig. 15). Associated abnormalities may include syndactyly, clinodactyly and symphalangism.

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The functional and aesthetic aspects of symbrachydactyly can range from mild to severe depending on the degree of shortening, status of digital remnant and number of digits involved. Hence surgical intervention varies likewise. In general, with regard to lengthening of the brachydactylous digit, the preferred treatment for short phalanges is to avoid surgery, as lengthening particularly with distraction osteogenesis is fraught with problems. Functional improvements are more predictable with soft tissue procedures including widening of web space, syndactyly release and free vascularised toe transfer for the more severely affected.

1.13

Radial Longitudinal Deficiency

Radial deficiencies consist of a spectrum of abnormalities where the dorsoradial structures in the hand and forearm have failed to adequately form. The prevalence is approximately 1:30000 live births with radial deficiencies frequently bilateral and asymmetrical. Both environmental causes (including maternal use of thalidomide), and syndromic associations are well documented. Associated syndromes include VACTERL, Holt-Oram, TAR, Fanconi’s anaemia and Trisomy 13 and 18 (Fig. 16). Fig. 16 Radial longitudinal deficiency

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Due to the associated syndromes, children with radial longitudinal deficiencies should be managed as part of a multidisciplinary team involving paediatricians and therapists. Haematological testing, cardiography, renal ultrasound and genetic screening are also mandatory. Complete physical examination is undertaken to assess syndromic associations and examination used to assess shoulder, elbow and hand function. Radiography is mandatory and should be bilateral including the digits, hand, wrist and forearm (including the elbow.) Classification is based on radiographic severity and was outlined by Bayne. • Type 1 Short distal radius (>2 mm shorter than ulna with normal proximal radius). • Type 2 Hypoplastic distal and proximal radius. • Type 3 Partial absence of radius. • Type 4 Complete absence of radius. Treatment is broadly based on the severity of deformity, although functional impairment, secondary to forearm length, wrist instability and thumb hypoplasia, is the main indication for treatment. Physiotherapy is required in all but the most minor of cases and helps to prevent progression of stiffness. In types 1 and 2, management is primarily with stretching and splintage. Severe type 2 deficiencies occasionally require more extensive surgery. In types 3 and 4, wrist instability with more severe radial deviation necessitates preoperative stretching followed by surgical realignment of the carpus on the ulna. Contraindications include major organ defects making anaesthetic risk unacceptable, inadequate elbow flexion (as discussed above), or firmly established functional patterns in adults. Preoperative stretching through the use of serial casting has now been largely superseded by external distraction. Distraction offers both osseous realignment, making wrist stabilisation easier, and soft tissue lengthening, reducing the requirement for local skin flaps to cover radial shortages and reducing the need for tendon lengthening procedures. Once adequately distracted, the realignment procedure can either be “centralisation” with the carpus repositioned over the ulna (usually by making a notch in the carpus) and stabilised with pin fixation through the third metacarpal, or “radialisation” with the carpus placed over the ulnar head and secured through the second metacarpal. Surgery may be followed by pollicisation to reconstruct an absent or hypoplastic thumb.

1.14

Ulnar Longitudinal Deficiency

Ulnar deficiencies are far less common than their radial counterparts and occur in approximately 1:100000 live births. Occurrence is sporadic, more commonly unilateral and is rarely associated with systemic conditions. Ulnar deficiencies differ from radial sided deficiencies with marked elbow pathology but a more stable wrist. Despite this, the hand and carpus are always abnormal

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with most associated with ectrodactyly, one third have syndactyly and over two thirds have thumb hypoplasias. Examination is therefore based on assessing bilateral upper limbs, with focus on elbow and hand function and identification of associated abnormalities. Radiography is mandatory for comparison and diagnosis. Classification is into four types depending on the degree of ulna hypoplasia. • Type 1 hypoplastic ulna. • Type 2 Partial absence of ulna. • Type 3 Total absence of ulna with normal radiohumeral joint. • Type 4 Total absence of ulna with synostosis of radio-humeral joint. The more common unilateral ulna deficiency patients usually function well, preferring the normal limb for one-handed tasks and adapting well for bimanual function, despite marked shortening of the limb. Surgical treatment depends on the severity with better outcomes in types 1 and 2. In such cases, surgery for associated thumb hypoplasia or syndactyly is all that is usually required. Indications for surgery in progressive or unstable type 2 and 3 deficiencies remain controversial. Excision of the ulna anlage has been advocated by some for progressive deformity. Realignment and formation of a one-bone forearm has been described but risks sacrificing forearm rotation for forearm stability. Type 4 deficiencies result in marked internal rotation of the limb which severely limits limb function. If functional compensation is not achieved through conservative measures, osteotomy with corrective external rotation is occasionally indicated.

1.15

Central Longitudinal Deficiency

Central ray deficiencies typically involve aplasia of the central digits forming what is also known as a typical cleft hand (Fig. 17). It is characterised by partial or complete absence of a central ray forming a deep v-shaped cleft. It is often bilateral, may involve both hands and feet. A family history is common, with autosomal dominant inheritance. Incidence is estimated at 1:10000 live births. Bones are either absent or malpositioned, and this can be associated with thumb duplication and central syndactyly. Central ray deficiencies can present as part of a syndrome, such as EEC syndrome or as an isolated abnormality. Radiographs may demonstrate transverse bony elements within the cleft which will require excision. Despite first web space contractures, hand function is often remarkably good. This has led it to be labelled as a “functional triumph but a social disaster.” Indications for early surgical intervention include border syndactyly and the presence of transverse bones within the cleft. Marked length discrepancies between the syndactylised digits can severely affect growth potential and transverse bone growth within the cleft results in progressive cleft widening. Closure of the cleft should only be performed if the first web is increased in size as otherwise it is doomed to failure.

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Fig. 17  Central ray deficiencies typically involve aplasia of the central digits forming what is also known as a typical cleft hand

This may involve transfer of the second metacarpal to the third metacarpal base and reconstruction of the transverse metacarpal ligament with skin adjustment to provide a good first web.

1.16

Camptodactyly

Camptodactyly is a congenital flexion deformity of the digit involving PIP joint and can be found in approximately 1% of the population. The incidence is likely an underestimate due to underreporting and some literature quotes up to 20% in adolescent females. The term is derived from the Greek ‘kamptos’ (arched) and ‘dactyl’ (finger) (Fig. 18). Camptodactyly has some chromosomal and syndromic associations but is frequently isolated and tends to have a bimodal distribution. It is commonly bilateral and is classically defined as involving the little finger, although any digit may be affected. It is classified by its distribution into: 1. Infantile (at or soon after birth) 2. Preadolescent (can be more severe and progressive) 3. Syndromic (often severe and multiple digits) In infancy, the male:female ration is 1:1, whereas the adolescent variant has a female predominance. The aetiology remains poorly understood with almost all structures crossing the volar aspect of the PIP being implicated. Abnormalities found on exploration include abnormal lumbrical insertion, short or tethered superficialis tendon, anomalous fibrous tissue and abnormalities in the retinacular or extensor system. The DIPJ is not pathological but may develop late compensatory changes.

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Fig. 18 Camptodactyly is a congenital flexion deformity of the digit involving PIP joint

Presentation of an abnormal posture involving the PIPJ requires careful history taking to help rule out non-congenital or traumatic aetiologies. Clinical assessment is focused on the digits affected and assessment with measurement of both passive and active ROM classifying the PIPJ into reducible or irreducible. When the PIPJ flexion deformity is irreducible, the suggestion is of significant joint pathology and is not likely to be correctable by surgery. Radiographic examination showing pathological changes involving PIPJ are a poor prognostic sign. Camptodactyly rarely affects function and most can be managed conservatively, particularly when contracture is less than 40°. Serial splintage and physiotherapy are the mainstays of treatment. If early correction is achieved, further splinting may be necessary in adolescence due to recurrent deformity during growth spurts. In general, surgery should be avoided if the deformity is mild and not progressive. Therefore, relative indications for surgery are functional problems (with PIPJ flexion deformity 40°+) and progressive disease when conservative therapies have failed. However surgical correction is rarely complete, particularly when radiographic evidence of PIPJ pathology is present and may result in loss of flexion as well as incomplete extension [10]. The principles of surgery if no skeletal abnormalities are present include exploration of all volar structures around the PIPJ and the release of abnormal/anomalous tissue. The principles of surgery if skeletal abnormalities are present include the above exploration as well as opening/closing wedge osteotomies but will sacrifice functional flexion for extension without increasing the arc of motion. Arthodesis is reserved for only the most severe cases when no useful function at the PIPJ can be attained but is often difficult due to the abnormal shape of the joint.

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Clinodactyly

Clinodactyly is characterised by radio-ulnar curvature of the digit distal to MCPJ and has a reported incidence between 1 and 19% of live births. It is derived from “clino” (sloped) and “dactyl” (finger). It is common and can follow an autosomal dominant pattern with variable penetrance. It may be syndromic with associated syndromes including Poland’s, Treacher-Collins, Kleinfelters and Trisomy 21. It is often bilateral and most commonly affects little finger and involves the middle phalanx. Clinical deformity results from abnormalities in the growth plate morphology of the middle phalanx. Abnormalities include a C-shaped physeal plate termed as a longitudinal epiphyseal or diaphyseal bracket. Growth in the longitudinal portion of the epiphysis leads to asymmetrical growth and progressive abnormal phalangeal morphology. At its most extreme this is seen as a delta phalanx (Fig. 19). The assessment of a clinodactyly patient should start with general review and identification of any syndromic features. The involved digit should be assessed for the degree of deformity, based on degree of angulation and the presence of shortening. Range of motion is usually normal with a bracketed epiphysis. Clinodactyly is generally painless with painful deformity suggesting trauma. Radiographic assessment in at least two views is mandatory. Classification is based on the degree of angulation and physeal and phalangeal morphology. • I—Minor angulation, normal length (very common). • II—Minor angulation, short phalanx, associated with Down’s syndrome. • III—Marked deformity, associated delta phalanx (wedge-shaped) with C-shaped physis. As the majority of clinodactyly patients have no functional deficit, reassurance is all that is required and surgery should be avoided for cosmesis only.

Fig. 19  Clinodactyly is characterised by radio-ulnar curvature of the digit distal to MCPJ

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Bracket resection and prevention of future angulation and shortening is an option in immature skeletons (