The Neurosurgical Consult Book (Jan 5, 2023)_(032375614X)_(Elsevier) 9780323756143

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1GITAL VERSION

THE NEUROSURGICAL CONSULT BOOK y>dif4«d

EdhjOrt .

Risheng Xu t Jordmo Rincon-Torroeilo ! Ann uu

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THE NEUROSURGICAL CONSULT BOOK Edited By RISHENG XU, MD/PHD | JORDINA RINCON-TORROELLA, MD | ANN LIU, MD The Johns Hopkins Department of Neurosurgery, Baltimore, Maryland, USA



ISBN: 978-0-323-75614-3

Copyright © 2023 by Elsevier, Ltd. All rights reserved.

No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Details on how to seek permission, further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/ permissions. This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein).

Notice Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds or experiments described herein. Because of rapid advances in the medical sciences, in particular, independent verification of diagnoses and drug dosages should be made. To the fullest extent of the law, no responsibility is assumed by Elsevier, authors, editors or contributors for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein.

Content Strategist: Humayra R Khan Senior Content Development Specialist: Shilpa Kumar Project Manager: Haritha Dharmarajan Design: Patrick Ferguson Illustration Manager: Anitha Rajarathnam Marketing Manager: Kate Bresnahan Printed in India Last digit is the print number:

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CONTRIBUTORS

Nancy Abu-Bonsrah, MD

Jawad M. Khalifeh, MD

A. Karim Ahmed, MD

Jennifer E. Kim, MD

Tej D. Azad, MD/MS

Ryan P. Lee, MD

Lydia Ju-mi Bernhardt, MD

Kurt Lehner, MD

James Feghali, MD

Ann Liu, MD

Landon J. Hansen, MD/PhD

Daniel Lubelski, MD

Alice L. Hung, MD

Andrew Luksik, MD

Brian Y. Hwang, MD

Dimitrios Mathios, MD

Wataru Ishida, MD

Jose Luis Porras, MD

Christina Jackson, MD

Jordina Rincon-Torroella, MD

Christopher M. Jackson, MD

Yuanxuan Xia, MD

Yike Jin, MD

Risheng Xu, MD/PhD

Brendan F. Judy, MD

Wuyang Yang, MD/MS

The Johns Hopkins Department of Neurosurgery Baltimore, Maryland, USA

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ACKNOWLEDGMENTS

Similar to our daily clinical practice, this book has been a collective effort that could not have been completed without the help of many. We are grateful to our resident colleagues, who offered their precious and limited free time to author the different chapters of this book. We give special thanks to the section editors for their subspecialty expertise in guiding and reviewing the content for each section. We want to thank all our faculty members and mentors. Neurosurgery is, in many ways, an apprenticeship, and we are forever grateful for their generosity in sharing their experience and knowledge. This book could not have been completed without their patients. Finally, we thank Elsevier for their role in neurosurgical education around the world and for supporting this endeavor.

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To all the neurosurgery residents who work tirelessly to take care of their patients, and to our loved ones for their unparalleled support.

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FOREWORD

Over the past decade, there have been unprecedented advances in the understanding and care of neurosurgery patients. We have seen marked improvement in care and outcomes based on these advances. The Neurosurgical Consult Book contributes a vital and up-to-date, critically important, easily accessible resource not only for neurosurgery trainees, but also for all practitioners at every stage of all their careers. The content of these chapters is the labor of love of the residents of the Johns Hopkins Neurosurgery Program, conceived originally for the express purpose of helping their peers. Inspired by the recognition that early learning relies on the experience of those who preceded them, Drs. Jordina Rincon-Torroella, Ann Liu, and Risheng Xu set out to organize their consult experiences in a manner easily transferrable to their junior residents. Their committed pursuit quickly evolved into a scholarly group effort with concerted input by our residents from various training years and the support of our Johns Hopkins Neurosurgery faculty. With such wideranging input across the neurosurgical subspecialties, the final product has the potential to serve as a valuable reference for all neurosurgical residents, early practice neurosurgeons preparing for oral board examinations, medical students, and advanced practice providers, as well as neurosurgeons across the spectrum of career stages and practice environments. This case-based learning tool stands out from the wide gamut of available educational resources due to its unique structure and organization. The flow of content in each chapter is designed to guide a variety of learners through the comprehensive management of various problems that begin as a hospital-based consult typically encountered in neurosurgical practice. The included details are thorough and time-sensitive, providing trainees with a practical framework to formulate efficient triage plans while on call, beginning with “walking thoughts” on the way to the patient’s bedside, key images that guide the decision-making, and the reasoning for the timely key assessment of “operative or not.” Furthermore, comprehensive “learning points” are included to confer upon the reader the specific management concerns and relevant prognosis for future treatment planning. When time is limited, a reference such as this, which helps focus on the pertinent management considerations is extraordinarily useful. The inclusion of references is particularly valuable to those preparing for their ABNS Oral Board examinations and those who wish to explore the relevant literature. We hope you will find this book to be a useful resource. Judy Huang, MD and Henry Brem, MD Baltimore, Maryland

xiii

PREFACE

We present The Neurosurgical Consult Book, a book written by residents for residents and other first-line health care providers for neurosurgical patients. Neurosurgery residency is hard. This is not a secret. But as residents, we are not afraid to face hardship, long hours, or patients in critical condition. The mastery of clinical care and surgical management of the neurosurgical patient is a combination of detailed study of the neurosurgical anatomy as well as operative techniques. In many aspects, though, this career is still based on apprenticeship from our more senior colleagues and attendings. Many surgical management and clinical skills are learned outside the operating room, including two of the most important lessons: how to stabilize a critically ill patient and when not to operate. Detailed and exhaustive guidelines are available these days in print and online. However, we felt that we were missing a straightforward and succinct resource that could clearly present a case encountered in real life, like a page or phone call. Several years ago, after a long day at the hospital as junior residents, the three of us went to grab a bowl of ramen amid a cold Baltimore winter. We have routinely used these meals to debrief and share our learning, mishaps, and victories during residency. In this instance, we had the idea of putting together our experiences during junior residency as a guide for future generations, a resource for quick review that could be used when running around the hospital on call. And this is how The Neurosurgical Consult Book was born. Now, after a concerted and collaborative effort from the three of us and our co-residents, we present to you our shared experience on how to triage the initial presentation of the neurosurgical patient, from pediatrics to trauma, tumor, spine, vascular, and cases of postoperative complications. We have included radiological pearls, essential clinical trials and guidelines, how we post cases for the operating room, and much more. We hope you enjoy this book and that it helps you in the care of neurosurgical patients, no matter your background or experience. Risheng, Jordina, and Ann

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“The Neurosurgical Consult Book is a wonderful compendium of real-world cases that neurosurgeons at every level of training can expect to encounter when on-call. The case descriptions are concise, relevant, and well referenced. I regret that I did not have access to this resource when I was starting out in neurosurgery. Congratulations to co-editors Rincon-Torroella, Xu, and Liu, on their accomplishment!”

AVIVA ABOSCH, MD, PHD

Professor and Chair Nancy A. Keegan and Donald R. Voelte, Jr. Chair in Neurosurgery Department of Neurosurgery University of Nebraska Medical Center “Illustrating key clinical principles with surgical efficiency, this book is the essential companion of the Neurosurgery resident.”

LOLA B. CHAMBLESS, MD, FAANS

Associate Professor of Neurological Surgery Residency Program Director, Neurological Surgery Vanderbilt University Medical Center “I predict this will be an indispensable reference for trainees in neurosurgery.”

EDWARD CHANG, MD

Jeanne Robertson Distinguished Professor Joan and Sanford I. Weill Chair of the Department of Neurological Surgery University of California, San Francisco “I still remember my early days as a resident when consults on top of all the other on-call burdens sometimes broke the back. Now, The Neurosurgical Consult Book provides residents a library of the common consults with quick summaries of presentations, imaging findings, clinical signs, management plans, and learning points. This handbook will surely find its way into the pockets of neurosurgery residents.”

MICHAEL T. LAWTON, MD

President and CEO Professor and Chair, Neurosurgery Chief, Neurovascular Surgery Barrow Neurological Institute “Whether you’re in the midst of a busy day on-call or between cases, this book will provide succinct, highly relevant background and management pearls on neurosurgical conditions at all levels.”

MICHAEL LIM, MD

Professor and Chair, Department of Neurosurgery Professor, by courtesy, of Medicine (Oncology), Neurology, Otolaryngology, and Radiation Oncology Stanford University School of Medicine Center for Academic Medicine

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xvi “The Neurosurgical Consult Book is a step-by-step guide to diagnosing and managing the breadth of common neurosurgical consults seen by front-line providers. Importantly, it is written by residents for residents. Each chapter starts with a “consult page” and takes the reader through imaging, assessment, and triage management decisions for common neurosurgical consults and presentations. The cases and chapters focus on an actual consult patient seen by the neurosurgery residents at Johns Hopkins University. This is an outstanding book that fills a critical niche and will be a ‘must-have’ in resident call rooms for years to come.”

WILLIAM J. MACK, MD, MS, FAANS, FACS, FAHA

Vice Chair, Academic Affairs Professor of Neurosurgery Neuroscience Graduate Program Keck School of Medicine University of Southern California

“I vividly remember the first time I was the first provider to go down to the emergency room to evaluate a patient after a gunshot wound to the brain. I still feel the thumping of my heart and the cold sweat as I was reaching my destination to do a rapid examination, review of imaging, and rapid communication with those caring for the patient. The mastery of clinical examination and a meticulous understanding of neurosurgical pathology is a continuous process, and it starts even before our residency and continues way after we finish our training. This book illustrates the importance of these consults for all of us and, most importantly, for the well-being of our patients. From that first experience, as I was rushing to do my emergency consult, I took important lessons that have stayed with me forever—I know you will take teachings from this book that will serve you well in your future careers, and they will be crucial to further strengthen the sacred commitment we have for our patients.”

ALFREDO QUIÑONES-HINOJOSA, MD, FAANS, FACS

William J. and Charles H. Mayo Professor Chair, Neurologic Surgery

“Neurosurgical consultations abound throughout residency, fellowship, and beyond for all neurosurgeons. While it is impossible to know and see everything throughout one’s career, this book offers highly practical information on the differential diagnosis, work-up and investigation, and management of patients presenting with neurosurgical disorders of all types. An especially valuable resource for junior trainees, and one which I wish I had at my fingertips back then!”

JAMES T. RUTKA, MD, PHD

Neurosurgeon, University of Toronto Editor in Chief, Journal of Neurosurgery “The Neurosurgical Consult Book is a must-have for any provider who is seeing neurosurgical patients in the acute setting. The authors of this text have provided the most up-to-date guidelines for managing neurosurgical scenarios while keeping the images and recommendations practical and digestible for the reader. I anticipate that this book will help consolidate the neurosurgical libraries of many providers into a single reference used on a routine basis.”

DANIEL M. SCIUBBA, MD, MBA

Professor & Chair of Neurosurgery, Zucker School of Medicine at Hofstra/Northwell Senior Vice President, Neurosurgery Co-Chair of the Neuroscience Institute, Northwell Health

xvii “The career path in neurosurgery is long and sometimes lonely. Countless nights faced with patients at their darkest moments and the need to make split-second decisions can all make for a stressful existence. This book serves not as an exhaustive reference regarding the minutiae of any particular case but rather a working guide on how to approach neurosurgical problems in general. Calm, cool, and collected decision-making with a commonsense approach is a guiding principle in our specialty, especially while seeing consults. This book will serve as the voice of reason to prepare the next generation of neurosurgeons to think about all problems in a broad sense while maintaining their confidence as they care for our patients and their families.”

NICHOLAS THEODORE, MD, FACS, FAANS

Donlin M. Long Professor Professor of Neurosurgery, Orthopedics & Biomedical Engineering Director, Neurosurgical Spine Program Co-Director, Carnegie Center for Surgical Innovation Co-Director & Founder, HEPIUS Lab Johns Hopkins University

C H A P T E R

1

Fall With Gradual Drowsiness Yuanxuan Xia, MD n

Jennifer E. Kim, MD

Consult Page 3 year old girl status post fall with head strike, worsening GCS, CT with large brain bleed

Initial Imaging

A

B

Figure 1.1 Head CT without contrast axial (A) and coronal (B) views demonstrate a large 8.5 cm right parietal epidural hematoma with 1 cm midline shift. Note the ominous “swirl sign” or areas of hypodensity in the hyperdense clot, which is concerning for active extravasation.

Walking Thoughts n n n n

n n n n n

2

What is the patient’s current GCS score and neurological exam? Does the patient have any other injuries? Is the patient hemodynamically stable? What is her past medical history? Does she have any bleeding disorders or take any anticoagulants or antiplatelet agents that need to be reversed? Is there any concern for non-accidental trauma? What labs have already been ordered or drawn? What interventions including hyperosmotic therapies have already been administered? When did she last have anything to eat or drink? Are the parents/legal guardians available?

1—Fall With GraDual DroWsiness

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History of Present Illness A 3 year old otherwise healthy female presents to the emergency department (ED) after a fall with posterior head strike. Per her parents who are at bedside, the patient tripped on a ball and fell backward onto the ground with immediate crying. They deny any loss of consciousness and report that the patient quickly returned to her neurological baseline. However, over the next couple of hours, she became less interactive and was taken to the nearest ED for evaluation. Per the ED team, she was initially awake and moving all extremities to command. She then had one episode of non-bilious, non-bloody emesis and acutely deteriorated to a GCS of 8 (E2V2M4). She was intubated for airway protection, and an emergent head CT demonstrated a 3.5 cm right parietal, mixed density epidural hematoma (EDH) concerning for hyperacute on acute blood products, with 1 cm midline shift and evidence of early transtentorial herniation.

Vital Signs T 36.9°C, HR 107 (normal 70-130), RR 33 (normal 20-34), BP 77/45 (normal SBP 80s-100s)

Pertinent Labs Na 141, Cr 0.3, Hgb 11.4, Plt 410, INR 1.05, PT 10.6, aPTT 26.3

Glasgow Coma Scale Motor: 2 Verbal: 1 Eye opening: 1 Intubated GCS Total: 4T

Physical Exam Intubated, sedation held for exam Normocephalic, atraumatic Right pupil unreactive and dilated, left pupil 2 mm and reactive to light Corneal, cough, and gag reflexes present Extensor posturing with stimulation

Triage Management This is a pediatric patient who presents after head trauma with a classic “lucid interval” followed by progressive altered mental status, now obtunded and requiring intubation. Her exam is notable for a fixed and dilated right pupil and decerebrate posturing. These findings are highly concerning for an acute intracranial process causing brain compression and herniation. Head CT shows a very large right parietal epidural hematoma with a positive “swirl sign” (Figure 1.1), which are hypodense areas within a hyperdense hematoma, suggestive of active bleeding.5 The blood exhibits the classic “lentiform” shape of an epidural hematoma that does not cross suture lines. It is causing significant mass effect with 1 cm midline shift, and there is both clinical and radiographic evidence of herniation. Given these findings, the patient requires emergent surgery for hematoma evacuation and brain decompression. In the meantime, bedside interventions to decrease intracranial pressure should be initiated emergently, and lines (IV and arterial lines) should be placed in preparation for the operating room. The patient will need to be admitted to the pediatric critical care unit after surgery. This is an emergent, operative consult.

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SECTION I—TRAUMA

Assessment This is a 3 year old female with no significant past medical history who presents after a fall with a lucid interval followed by rapid neurological deterioration. Head CT shows a large right parietal EDH with radiographic and clinical signs of herniation.

Plan n n n

n n

n

Elevate head of bed to ≥ 30° Hyperventilate with end tidal CO2 goal 25-35 mmHg4 Initiate osmotic therapy3 n Hypertonic saline 6.5-10 mL/kg with serum sodium goal between 145 and 160 mEq/L n Mannitol (more commonly used in adults) 1 g/kg with serum osmolality goal < 320 mOsm Insert arterial line and Foley catheter Emergent surgery for right sided craniotomy and epidural hematoma evacuation n Posting highlights: n Horseshoe headrest (or clamp in adults) n Burr hole drill and craniotome n Holemaker for tack-up sutures n For anesthesia: antibiotics, levetiracetam, mannitol, pCO2 goal 25-35 mmHg (can be normalized once hematoma is evacuated), no need for steroids Admission to pediatric critical care unit and nonaccidental trauma workup after surgery

LEARNING POINTS

• EDH often presents with a characteristic lucid interval followed by rapid neurological deterioration due to elevated intracranial pressure.6

• The majority of EDHs are arterial in origin, classically due to a laceration or avulsion of the middle meningeal artery. A more slowly progressing EDH may also result from a torn venous sinus, which may need surgical repair. Venous phase vascular imaging is recommended in cases where the EDH overlies or is in close proximity to a venous sinus. • Clinical and radiographic indications for surgical intervention include:1,7 • Altered mental status, rapidly worsening GCS, or GCS ≤ 8 • Signs of increased intracranial pressure, pupillary abnormalities, focal neurological deficits • EDH > 10 mm in size • Mass effect with midline shift • Surgery is usually indicated, but those who do not require surgery should be closely monitored in an ICU setting and undergo a follow-up head CT within 6-8 hours to assess for interval changes.1 Conservative management may be considered if a patient has mild symptoms and meets the criteria below: • EDH volume < 30 mL • Thickness < 15 mm • Midline shift < 5 mm • GCS > 8 without focal neurological deficit • It is critical to minimize the time between neurological symptoms and surgical intervention, as delays in decompression are associated with poorer overall prognosis. In adults, this time interval should be less than 2 hours.1,2 • Remember to evaluate for skull fractures on imaging before placing a patient in a skull clamp for surgery. Pinning a patient with a pre-existing fracture can lead to additional fractures and intracranial injury, and compromise the mechanical integrity of the clamp system.

1—Fall With GraDual DroWsiness

References 1. Bullock MR, Chesnut R, Ghajar J, et al. Surgical management of acute epidural hematomas. Neurosurgery. 2006;58(3 suppl):S7–iv. 2. Gerlach R, Dittrich S, Schneider W, Ackermann H, Seifert V, Kieslich M. Traumatic epidural hematomas in children and adolescents: outcome analysis in 39 consecutive unselected cases. Pediatr Emerg Care. 2009;25(3):164–169. 3. Kochanek PM, Carney N, Adelson PD, et al. Guidelines for the acute medical management of severe traumatic brain injury in infants, children, and adolescents--second edition. Pediatr Crit Care Med. 2012;13(suppl 1):S1–S82. 4. Kukreti V, Mohseni-Bod H, Drake J. Management of raised intracranial pressure in children with traumatic brain injury. J Pediatr Neurosci. 2014;9(3):207–215. 5. Osborn AG. Craniocerebral trauma. In: Diagnostic Neuroradiology. St. Louis: Mosby; 1994:204–205. 6. Sartor K, Haehnel S, Kress B. Direct Diagnosis in Radiology. Brain Imaging. New York: Georg Thieme Verlag; 2008. 7. Schutzman SA, Barnes PD, Mantello M, Scott RM. Epidural hematomas in children. Ann Emerg Med. 1993;22(3):535–541.

C H A P T E R

2

Dizziness With Syncope And Fall Yuanxuan Xia, MD n

Jennifer E. Kim, MD

Consult Page 77 year old woman with syncope and fall 3 days ago with intracranial bleed on CT

Initial Imaging

A

B

C

Figure 2.1 Axial (A), coronal (B), and sagittal (C) views of a head CT without contrast shows a small right parietal subarachnoid hemorrhage without evidence of skull fractures or mass effect.

Walking Thoughts n n n n

n n n

What was the etiology of her syncopal event? Why is she presenting 3 days after the fall? What is the patient’s baseline functional status and neurological exam? What is her current neurological exam? Is the pattern of the subarachnoid hemorrhage concerning for an underlying aneurysm or vascular abnormality? Does the patient take any anticoagulation? If so, have reversal agents been given? Are coagulation labs available or pending? Are there additional cervical spine or traumatic injuries that must be considered?

History of Present Illness A 77 year old woman with a history of carpal tunnel syndrome, gout, chronic pain, and coronary artery disease (CAD) on aspirin (ASA) 81 mg daily, presents to the emergency department (ED) with a left elbow gout flare. She incidentally reports that 3 days ago, she had dizziness, lost consciousness

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2—DizzineSS With Syncope AnD FAll

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and fell, hitting her head. She denied any headache at the time of the fall. Given this clinical history, a head CT was ordered which shows a small right parietal subarachnoid hemorrhage. The patient is currently focused on pain from her left arm and only endorses baseline left-sided weakness and paresthesias that have been ongoing for the past year. She denies any headaches, nausea, vomiting, vision changes, seizure-like activity, or speech changes. She reports that she last took her ASA earlier this morning and denies using other anticoagulation or antiplatelet medications.

Vital Signs T 36.9°C, HR 91, BP 119/83, SpO2 96% on room air

Pertinent Labs Na 142, Glu 85, Plt 201, INR 1.1, PT 11.0

Physical Exam Alert & oriented to self, place, and year Pupils equal, round, and reactive to light Extraocular movements intact bilaterally, tongue midline, face symmetric Right upper extremity 5/5 strength Left upper extremity 4/5 grip strength, exam pain-limited but at baseline Bilateral lower extremities 5/5 strength Sensation intact to light touch throughout

Triage Management The patient has an incidental traumatic subarachnoid hemorrhage (tSAH) without midline shift or mass effect. She is at her neurological baseline and not endorsing any headache. The convexity location of her bleed suggests a traumatic etiology rather than an aneurysmal cause. Given the small size of the hemorrhage and lack of new neurological symptoms since the traumatic event,6,8 she does not require platelet transfusion for aspirin reversal.9 Because she takes ASA81, a six hour interval head CT could be performed to evaluate for hemorrhagic progression.9 This is a nonurgent, non-operative consult.

Assessment This is a 77 year old female with a history of chronic pain, left arm gout, and CAD on daily ASA81 presenting with left elbow pain likely due to a gout flare but noted to have dizziness and syncope 3 days ago. She is GCS15, with no focal neurological deficits on exam. Head CT demonstrates an isolated, focal right parietal convexity tSAH without mass effect, with low anticipated risk for expansion.

Plan No acute neurosurgical intervention indicated at this time n Syncope workup per the ED team or primary team n Consider repeat head CT at 6 hours from initial scan n Hold ASA81 n Follow-up in neurosurgery outpatient clinic in 2 weeks n

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SECTION I—TRAUMA LEARNING POINTS

• Radiologic findings that are suggestive of traumatic etiology of subarachnoid hemorrhage include: localized bleeding in superficial sulci, adjacent skull fracture, cerebral contusion, or external evidence of traumatic injury.10 • Non-contrast head CT can detect nearly all cases of intracranial bleeding within 48 hours of the inciting event.1,2,5 • There is a growing body of literature that suggest a benign course for mild cases of isolated tSAH (with a presenting GCS of 13-15) that requires no neurosurgical intervention or ICU admission.7 In patients with a nonfocal, reassuring examination, routine repeat cranial imaging may be low yield.4 However, patients on anticoagulation or antiplatelet medications are at higher risk and warrant closer monitoring. The timing and need for repeat imaging is variable and provider-dependent. • Moderate to severe tSAH, multifocal tSAH, polytrauma, depressed mental status, or decline in neurological exam may require admission to the ICU due to concern for brain swelling and elevated intracranial pressure (ICP). Subsequent management may include intubation for airway protection and ICP monitor. • The use of antiepileptics for seizure prophylaxis in tSAH has not been well studied. In cases of severe traumatic brain injury, antiepileptics are recommended to decrease the incidence of early posttraumatic seizure (within 7 days).3 The choice of agent, duration, and dosing may vary depending on provider. Due to our patient’s reassuring GCS and the potential side effects with antiepileptics, seizure prophylaxis may not be warranted. The timing of re-initiation of anticoagulants and antiplatelets in tSAH has not been well • studied and is at the discretion of the provider, balancing the indication for anticoagulation with severity of bleed and clinical status.

References 1. Barber S, Gasco J, Nader R, et al. Subarachnoid hemorrhage and Vasospasm. In: Gasco J, Nader R, eds. The Essential Neurosurgery Companion. 1st ed. 2012. (Thieme). 2. Boesiger BM, Shiber JR. Subarachnoid hemorrhage diagnosis by computed tomography and lumbar puncture: are fifth generation CT scanners better at identifying subarachnoid hemorrhage? J Emerg Med. 2005;29(1):23–27. 3. Carney N, Totten AM, O’Reilly C, et al. Guidelines for the management of severe traumatic brain injury, 4th ed. Neurosurgery. 2017;80(1):6–15. 4. Joseph B, Aziz H, Pandit V, et al. A three-year prospective study of repeat head computed tomography in patients with traumatic brain injury. J Am Coll Surg. 2014;219(1):45–51. 5. Latchaw RE, Silva P, Falcone SF. The role of CT following aneurysmal rupture. Neuroimaging Clin. 1997;7(4):693–708. 6. Naidech AM, Liebling SM, Rosenberg NF, et al. Early platelet transfusion improves platelet activity and may improve outcomes after intracerebral hemorrhage. Neurocritical Care. 2012;16(1):82–87. 7. Nassiri F, Badhiwala JH, Witiw CD, et al. The clinical significance of isolated traumatic subarachnoid hemorrhage in mild traumatic brain injury: a meta-analysis. J Trauma Acute Care Surg. 2017;83(4):725–731. 8. Nishijima DK, Zehtabchi S, Berrong J, Legome E. Utility of platelet transfusion in adult patients with traumatic intracranial hemorrhage and preinjury antiplatelet use: a systematic review. J Trauma Acute Care Surg. 2012;72(6):1658–1663. 9. Schnüriger B, Inaba K, Abdelsayed GA, et al. The impact of platelets on the progression of traumatic intracranial hemorrhage. J Trauma. 2010;68(4):881–885. 10. van Gijn J, Rinkel GJ. Subarachnoid haemorrhage: diagnosis, causes and management. Brain. 2001;124(Pt 2):249–278.

C H A P T E R

3

Fall And Altered Mental Status Risheng Xu, MD/PhD n

Jennifer E. Kim, MD

Consult Page 69F altered after a fall, CT brain bleed.

Initial Imaging

A

B

Figure 3.1 Axial head CT without contrast (A) demonstrates an 8 mm left sided, holohemispheric subdural hemorrhage with 7 mm midline shift. There are no skull fractures. CT c-spine without contrast (B) shows a C6 spinous process fracture not extending into the facets.

Walking Thoughts n n n n n

n n

What is the current GCS of the patient? Is she able to protect her airway? What is the baseline function of the patient? Does the patient take anticoagulation? If so, have reversal agents been given? Are coagulation labs available or pending? What are the patient’s medical comorbidities which may make surgery risky (e.g. pacemaker, chronic heart failure [CHF], severe chronic obstructive pulmonary disease [COPD])? What was the mechanism of the fall? Are there concomitant cervical spine injuries? Does this patient need to go to the operating room emergently?

9

10

SECTION I—TRAUMA

History of Present Illness A 69 year old female with history of vasculopathy with multiple non-cardiac stents (on aspirin 325 mg and clopidogrel 75 mg daily, last taken today), severe COPD, CHF (unknown ejection fraction but not on diuretics), and ethanol abuse who presents after a fall today with progressive altered mental status. Head CT demonstrates a large left sided subdural hematoma (SDH) with mass effect and midline shift; cervical spine CT demonstrates a C6 spinous process fracture. The daughter reports that the patient had a fall this morning 4 hours prior to presentation and hit her head. She did not have loss of consciousness initially and was able to tell her husband to help her up. She went to take a nap and became difficult to arouse. The daughter was eventually able to wake her with sternal rub. She was incoherent and not oriented to self, place, or time. The daughter denies any seizure-like activity before or after the fall. After arrival at the hospital, the patient became more agitated and confused.

Vitals Signs T 36.9°C, HR 107, BP 126/92, SpO2 93% on room air

Pertinent Labs Na 128, Plt 147, INR 1.0, PT 1.0, aPTT 25.4

Glasgow Coma Scale Motor: 6 Verbal: 3 Eye opening: 4 GCS Total: 13

Physical Exam Eyes open spontaneously, attends Confused speech, word finding difficulty Alert & oriented to self, place and year with choices Pupils equal, round, and reactive to light Extraocular movements intact Able to wiggle fingers to command bilaterally Able to wiggle toes to command bilaterally Cervical collar present

Triage Management The patient has a large left SDH with associated 7 mm midline shift. She is confused and clearly not at her neurological baseline. She takes dual antiplatelet therapy and is at high risk for progression of continued intracranial bleeding and continued neurological decline. At baseline, the patient is physically active and was taking aquatic classes. After extensive discussion with family, the decision is made to pursue surgical management of the left SDH. In order to prepare the patient for surgery, the functional platelet defect will be corrected with platelet transfusion. She will be started on prophylactic antiseizure medication. Given the patient is still oriented, awake and following commands with GCS13, the patient does not require emergent intubation in the emergency department. Because the patient has a cervical spine injury, anesthesia will be notified, and the cervical collar will remain in place during intubation in the operating room. Her small spinous process fracture with minimal involvement of the lamina will be managed conservatively with rigid cervical collar for 6 weeks and outpatient follow-up. Her sodium is 128; the chronicity of this is unknown and may be related to her ethanol abuse, so correction of her hyponatremia will be gradual. This is an urgent and operative consult.

s

11

Assessment This is a 69 year old woman with a history of vasculopathy and multiple non-cardiac stents (on aspirin and clopidogrel), severe COPD, CHF, and ethanol abuse who presents with progressive altered mental status after a fall today with CT demonstrating left SDH with 7 mm midline shift and a C6 spinous process fracture. After discussion with the family, the decision is made for surgical management of the acute SDH.

Plan n n n n n n

n n n

Administer 1 unit of platelets and type and cross an additional unit for the OR Levetiracetam 1 g now and 1 g every 12 hours thereafter Maintain rigid cervical collar on at all times Start normal saline, will continue to trend sodium every 6 hours Insert arterial line and Foley catheter Emergent surgery for left hemicraniotomy vs. hemicraniectomy for subdural hematoma evacuation n Duraplasty and bone flap removal will depend on the severity of underlying brain edema and/or provider preference n Posting highlights: n Skull clamp (or horseshoe headrest) n No intraoperative neuromonitoring n Burr hole drill and craniotome n Dural substitutes n For anesthesia: antibiotics, levetiracetam, platelets. Can consider judicious use of mannitol if there is concern for herniation. pCO2 30 mmHg until brain is decompressed Admission to the neurocritical care unit after surgery Syncopal workup and tertiary trauma survey after surgery Hold aspirin and clopidogrel; timing of re-initiation will depend on indication and discussion with prescribing provider

LEARNING POINTS

• According to the Surgical Management of Traumatic Brain Injury Author Group1: • SDH with thickness greater than 10 mm or a midline shift greater than 5 mm on CT scan should be surgically evacuated, regardless of the patient’s GCS.

• All patients with acute SDH in a coma (GCS score less than 9) treated nonoperatively should undergo intracranial pressure (ICP) monitoring.

• Patients with GCS < 9 with a SDH less than 10 mm thick and a midline shift less than 5 mm should undergo surgical evacuation of the lesion if the GCS score decreased between the time of injury and hospital admission by 2 or more points and/or if the ICP exceeds 20 mmHg despite maximal medical intervention. • Timing of surgery is thought to be an important predictor of outcome in SDH evacuation. In one New England Journal of Medicine study3, patients who underwent surgery within the first four hours had a 30% mortality rate, as compared with 90% in those who had surgery after four hours. • In the patient with an acute SDH, care must be taken to evaluate both the head and spine for associated neck injury. • If the patient will be placed in a skull clamp for surgery, it is critical to evaluate for skull fractures to avoid pinning and possibly worsening a fracture. • Any coagulopathy should be addressed before going to the OR either with medication or blood products (Table 3.1)3. • When family is available, discussion regarding goals of care and aggressiveness of intervention is imperative to assess if surgery is in line with the patient’s wishes or advanced directive.

12

SECTION I—TRAUMA

TABLE 3.1 n Antithrombotic Agents Commonly Encountered in the Surgical Setting Half-life, h

Strategy for reversal

Last dose before operation

Antithrombin III mediated inhibition of Factor Xa and Factor IIa

1-2

Protamine sulfate

Intravenous: 2-6 h Subcutaneous:12-24 h

Low-molecularweight heparins; Enoxaparin

Antithrombin III mediated selective inhibition of Factor Xa

2

Protamine sulfate (only partially effective)

24 h

Warfarin

Vitamin K antagonist

20-60

Vitamin K; 4-factor 5 d PCC; FFP

Dabigatran

Direct thrombin inhibitor

8-15

Idarucizumab; 4-factor PCC; hemodialysis

24-48 h

Rivaroxaban

Direct factor Xa inhibitor

5-13

4-factor PCC

24-48 h

Apixaban

Direct factor Xa inhibitor

12

4-factor PCC

24-48 h

Aspirin

Cyclooxygenase inhibitor

3-10

Platelet transfusion 7 d

Clopidogrel

Irreversible ADP receptor antagonist

8

Platelet transfusion 5-7 d

Prasugrel

Irreversible ADP receptor antagonist

7

Platelet transfusion 5-7 d

Ticagrelor

Reversible and noncompetitive ADP receptor antagonist

9

Platelet transfusion 5-7 d

Agent

Mechanism of action

Unfractionated heparin

  

  

References 1. Bullock, et al. Surgical management of acute subdural hematomas. Neurosurgery. 2006;58(3 suppl):S16– S24; discussion Si-iv. 2. Hornor, et al. American College of Surgeons’ guidelines for the perioperative management of antithrombotic medication. J Am Coll Surg. 2018;227(5):P521–P536. 3. Seelig, et al. Traumatic acute subdural hematoma: major mortality reduction in comatose patients treated within four hours. N Engl J Med. 1981;304(25):1511.

C H A P T E R

4

Unresponsive After MVC Landon J. Hansen, MD/PhD n

Jennifer E. Kim, MD

Consult Page STAT MVC trauma, 2Y with GCS3

Initial Imaging

A

B

C

Figure 4.1 Axial head CT without contrast demonstrates a minimally displaced left frontal fracture (A) without evidence of large intracranial hemorrhage (B, C).

Walking Thoughts n n n n n n n n n

What is the patient’s current neurological exam? Is the patient intubated? Why is the patient’s GCS so poor? Is the patient currently paralyzed or sedated? Do the imaging findings explain the physical exam findings? Does the patient have any other injuries? Does the patient have any lab abnormalities that can explain his poor GCS? What immediate steps need to be taken to medically optimize the patient? Is family present? What, if any, surgical interventions could improve outcome or management? Does the patient need additional imaging?

History of Present Illness A 2 year old male with no past medical history presents as a restrained back seat passenger in a car that rear-ended another at an approximate speed of 40-50 miles per hour. The patient 13

14

SECTION I—TRAUMA

had been properly restrained but the lower car seat anchors broke on impact and the car seat was found in the front seat of the vehicle. He was unresponsive with a reported GCS of 3 in the field with unequal pupils and was intubated at the scene. Intraosseous access was obtained en route to the emergency department (ED). Head CT in the ED revealed a minimally displaced left frontal bone fracture without evidence of additional intracranial abnormality.

Vital Signs T 35°C, HR 112 (normal 80-130), RR 23 (normal 25-35), BP 97/62 (normal SBP 80s-100s), SpO2 98% on ventilator

Pertinent Labs Na 140, Hgb 10.2, Plt 402, INR 1.2, PT 11.9, aPTT 20.0

Glasgow Coma Scale Motor: 4 Verbal: 1 Eye Opening: 1 GCS Total: 6T

Physical Exam Intubated, paralytic reversed, sedation held for exam Left frontal scalp laceration, multiple abrasions Hard cervical collar in place Pupils round, right 1 mm, left 3 mm, minimally reactive bilaterally Positive corneal reflexes bilaterally No appreciable cough or gag reflex Right upper extremity withdraws to stimulation Left upper extremity extends to stimulation Bilateral lower extremities withdraw

Triage Management The patient has a minimally displaced left frontal bone fracture, without evidence of intracranial hemorrhage. His neurological exam is poor, out of proportion to his head CT findings. The primary trauma survey was otherwise negative for systemic injuries. There are no laboratory abnormalities that point to a metabolic etiology for his profound altered mental status. There is high suspicion for diffuse axonal injury (DAI) and elevated intracranial pressure (ICP) given the mechanism of injury and clinical presentation. He will need emergent placement of an ICP monitor. In the meantime, he requires bedside interventions to decrease intracranial pressure including hyperosmolar therapy and hyperventilation. He will be admitted to the pediatric intensive care unit for ICP monitor placement and will subsequently require MRI of the brain (Figure 4.2) and cervical spine to evaluate for CT-occult brain injury and ligamentous cervical spine injury. He will be maintained in a rigid cervical collar. This is an emergent consult that requires intervention.

15

4—UnresponsiVe After MVC

A

B

C

Figure 4.2 Axial T2-weighted brain MRI (A) demonstrates a large hyperintensity of the left posterior midbrain. Diffusion weighted sequences show areas of restricted diffusion around grey-white matter boundaries (B) and brainstem (C). These findings are consistent with DAI.

Assessment This is a 2 year old male who presents after a high-speed motor vehicle collision. He was reportedly GCS 3 in the field, intubated at the scene, and GCS 6T upon arrival to the ED. Head CT shows a left frontal minimally displaced fracture and bilateral traumatic subarachnoid hemorrhages. Due to his poor exam in the absence of a space occupying lesion or hematoma on imaging, there is concern for DAI and elevated ICPs. He will need an ICP monitor to evaluate and guide further medical management. Procedure risks and benefits were discussed with the patient’s father who understood and wished to proceed.

Plan n n n n n n

n n

n

Pediatric dosing of prophylactic antiepileptic medication Avoid hypotension Cervical collar in place at all times Elevate head of bed to 30 degrees or more Hyperventilation with pCO2 goal 30-35 mmHg Hyperosmotic therapy: n Hypertonic saline with serum sodium goal 145-160 n In adults, can consider the use of mannitol Arterial line and foley catheter placement; consider central line placement Emergent right frontal ICP monitor placement. Since the patient will require a left frontal laceration repair, both procedures can be done in the operating room. In an adult, this procedure is usually done at the bedside. n Posting highlights: n Horseshoe headrest n Pediatric cranial perforator drill n Intracranial pressure monitor device n For anesthesia: antibiotics, antiseizure medication, blood products on hold, pCO2 goal 30-35, no need for steroids Post-operative admission to the pediatric critical care unit

16

SECTION I—TRAUMA

TABLE 4.1 n Recommendations from Brain Trauma Foundation Guidelines Recommended: • Use of ICP monitor, and treatment of ICP to 72 hours) serum Na > 170 mEq/L due to complications of thrombocytopenia and anemia, and avoid a sustained serum Na > 160 mEq/L due to complication of deep vein thrombosis

• Analgesics, sedatives, and neuromuscular blocking agents

• Use of mannitol

• Use of extraventricular drain • Maintain CPP > 40 • Use of hypertonic saline boluses (3%) for acute use, or continuous infusion hypertonic saline, or bolus of 23.4% hypertonic saline for refractory ICP • Seizure prophylaxis treatment for 7 days • Moderate (32-33°C) hypothermia is suggested for ICP control

• Bolus administration of midazolam and/or fentanyl during ICP crises due to risks of cerebral hypoperfusion

• Prophylactic severe hyperventilation to a pCO < 30 mmHg in the initial 48 hours after injury

• Prolonged continuous propofol infusion • Corticosteroids

• High-dose barbiturate therapy in hemodynamically stable patients with highly refractory intracranial hypertension

• Levetiracetam vs. phenytoin for seizure prophylaxis

• Prophylactic moderate (32-33°C) hypothermia is not recommended over normothermia

• Decompressive craniectomy for herniation or refractory ICP

  

  

LEARNING POINTS

• DAI occurs as the brain shifts and rotates inside the skull during trauma and is the result of shearing forces along the grey-white matter junction. It is most commonly a result of motor vehicle collisions and is seen in approximately half of all severe head traumas.1 • DAI is often not evident on CT or MRI during the initial trauma evaluation. Punctate hemorrhages, if present, could be detected by either modality, but with greater sensitivity using MRI, which also has better sensitivity for detecting non-hemorrhagic lesions.3,4 CT scan is the most urgent imaging modality for immediate management of severe TBI.2 • Brain MRI with diffusion sequences is the preferred modality to evaluate for DAI and will show areas of restricted diffusion and flair abnormality predominantly around the boundaries of the subcortical white matter, the corpus callosum, and the brainstem.1,3 • Severe traumatic brain injury is defined as any patient with GCS ≤8. ICP monitor placement is recommended for cases of severe traumatic brain injury or with signs of impending herniation unless there is a major contraindication. Monitoring and treatment of intracranial hypertension is associated with improved outcomes in cases of severe TBI.2 Table 4.1 summarizes the 2019 guidelines for the management of pediatric severe TBI.2 •

References 1. Gentry L. MR imaging of head trauma: review of the distribution and radiopathologic features of traumatic lesions. Am J Roentgenol. 1988;150:663–672. 2. Kochanek PM, Tasker RC, Carney N, et al. Guidelines for the management of pediatric severe traumatic brain injury, third edition: update of the brain trauma foundation guidelines, executive summary. Pediatr Crit Care Med. 2019;20:280. 3. Parizel P, Ozsarlak O, Van Goethem J, et al. Imaging findings in diffuse axonal injury after closed head trauma. Eur Radiol. 1998;8:960. 4. Provenzale J. Imaging of traumatic brain injury: a review of the recent medical literature. Am J Roentgenol. 2010;194:16–19.

C H A P T E R

5

Unresponsive After Gunshot Wound To Head Landon J. Hansen, MD/PhD n

Jennifer E. Kim, MD

Consult Page TRAUMA; 24M gunshot wound to the head, GCS 7 in field

Initial Imaging

A

B

Figure 5.1 Head CT without contrast sagittal (A) and coronal (B) views show a gunshot wound with the bullet tract entering near the vertex and bone and bullet fragments extending through the bilateral parietal and frontal cortices down to the area of the right thalamus/basal ganglia. There are scattered foci of pneumocephalus and hemorrhage.

Walking Thoughts n n n n n n

n n

n n

What is the current GCS and neurological exam? Is the patient hemodynamically stable? Are there any additional injuries or other gunshot wounds (GSW)? Is the patient on anticoagulation? If so, have reversal agents been given? Are coagulation labs available or pending? What is the appropriate imaging modality to determine extent of injury to the superior sagittal sinus? What interval imaging needs to be performed? Would the patient benefit from emergent surgical decompression or an intracranial pressure monitor (ICPm)? What is the patient’s prognosis? What medical management needs to be initiated? 17

18

SECTION I—TRAUMA

History of Present Illness A 24 year old male presents to the emergency department via ambulance as an emergent trauma with a GSW to the top of his head. He was reported to be moving minimally in the field with a GCS of 7. He was intubated prior to arrival. Head CT (Figure 5.1) demonstrated bullet entry near the vertex with the trajectory crossing the superior sagittal sinus and bullet fragments extending into the right thalamus/basal ganglia. Extensive comminuted skull fractures involving bilateral temporal bones are seen with bone fragments scattered in the parietal and frontal cortices and subcortical white matter including the corpus callosum. Head CT angiogram (CTA) and venogram (CTV) demonstrated narrowing of the distal anterior cerebral artery (ACA) and middle cerebral artery (MCA) branches, as well as non-opacification of a 2.8-cm segment of the superior sagittal sinus below the bullet entry site (Figure 5.2). The trauma team has evaluated the patient at bedside and he has no other injuries on examination and pan-body imaging.

A

B

Figure 5.2 Sagittal CTA (A) and CTV (B) demonstrates a 2.8 cm length of non-opacification of the superior sagittal sinus concerning for injury vs. thrombosis.

Vital Signs T 37.4°C, HR 74, RR 18, BP 137/91, SpO2: 100% on ventilator

Pertinent Labs Na 141, Hgb 8.5, Plt 277, INR 1.4, PT 14.3, aPTT 29.6

Glasgow Coma Scale Motor: 2 Verbal: 1 Eye Opening: 1 GCS Total: 4T

Physical Exam Intubated, sedation/paralytics held Not over-breathing the ventilator Pupils round, large, sluggishly reactive No corneal reflex

5—UNRESPONSIVE AFTER GUNSHOT WOUND TO HEAD

19

Weak cough and gag reflexes present Extending in bilateral upper extremities Minimal movement in bilateral lower extremities Large open head wound with brain matter herniating from the vertex of the head

Triage Management The patient has an isolated penetrating gunshot wound to the head with the bullet entering at the vertex over the superior sagittal sinus. A head CT without contrast is not considered sufficient to determine injury to the superior sagittal sinus or other large vessels, therefore a CTA/CTV was obtained, confirming sinus injury (Figure 5.2). Due to his poor neurological exam, lack of a space-occupying hematoma, and sinus involvement, the patient is deemed unlikely to benefit from surgical intervention, and maximal medical management is pursued. To treat his presumed elevated ICP secondary to brain swelling, the patient immediately receives two rounds of 23% hypertonic saline and 1 g/kg of mannitol. He is maintained on infusions of hypertonic saline and propofol. He has no other injuries or gunshot wounds. The patient will receive antibiotics for his penetrating head injury, and the scalp wounds will need to be irrigated thoroughly and closed. The patient is started on IV phenytoin for seizure prophylaxis. He will need to be admitted to the neurocritical care unit with plan for bedside ICPm placement to help guide medical management. This is an emergent consult that requires immediate intervention.

Assessment This is a 24 year old male presenting with a GSW to the top of his head, initially GCS 7 and now GCS 4T. Head CT demonstrates a bullet trajectory crossing the superior sagittal sinus with extensive skull fractures and the bullet fragments embedded in the right thalamus/ basal ganglia. Head CTA and CTV show a major superior sagittal sinus injury. At this time, there is no space occupying hematoma or unilateral swelling that warrants emergent surgical evacuation or decompressive craniectomy. However, the patient is at high risk for elevated ICPs from the penetrating injury and will need ICP monitoring to help direct further medical management.

Plan n n n

n n n n n n n n n n n

n

Admission to the neurocritical care unit Neurological exams every hour Trend sodium every 6 hours, Na goal >155, with hypertonic saline infusion and boluses as needed Monitor hemoglobin levels and transfuse for a goal of at least 7 g/dL Systolic blood pressure 70% of patients dying at the scene of the accident. Of those who make it to a trauma center, greater than 50% die in the acute care setting and less than half of patients who make it to the hospital warrant neurosurgical intervention. Overall mortality is greater than 90%. Post-resuscitation GCS score is the strongest predictor of mortality and favorable outcome.1 • Skull fracture with dural sinus injury is often a source of major hemorrhagic blood loss. Injury to the dural venous sinuses poses an immediate risk of hemorrhage, as well as subsequent risk of edema due to venous congestion, and is generally associated with poor outcomes.2,6 • Overall, traumatic venous sinus injury poses a mortality risk of >40%. Injury to the anterior and middle portions of the superior sagittal sinus poses lower risk than injury to the posterior third, where mortality approaches 100%.3,7,8 • Retained bullet fragments do not pose an infection risk. Bullet fragments should only be removed if surgery is indicated for surgical debridement or removal of hematoma, and if the bullet is in the vicinity and can be removed without damaging any critical structures. Abscess formation, focal seizures, infection, or migration are additional indications for bullet removal.5,9 • Although rare (4% of cases), bullet fragments can migrate into critical structures or CSF spaces resulting in hydrocephalus. This can be monitored with serial imaging.9,10 • If the injury is so severe that there is active extravasation, surgery may be too risky. The alternative is to perform an emergent laceration repair with a tight head wrap to tamponade the hemorrhage and attempt hemodynamic stabilization. • Pentobarbital coma can be used to control refractory elevated ICP in patients with severe traumatic brain injury (TBI), as was eventually used in this case. Intractable intracranial hypertension despite optimal medical management may require decompressive craniectomy.4 • Of note, although patients with intracranial GSW have a poor prognosis, this patient had a remarkable recovery. He did require a temporary tracheostomy and percutaneous gastric tube placement, but after a long hospitalization and intense rehabilitation therapy, the patient recovered to a normal level of consciousness and speech function, full strength in his upper extremities, and a new baseline of lower extremity paresis. This emphasizes the importance of early intervention. Table 5.1 summarizes guidelines for management of severe TBI. •

21

5—UNRESPONSIVE AFTER GUNSHOT WOUND TO HEAD

TABLE 5.1 n Based on the Guidelines for Management of Severe Traumatic Brain Injury Recommended: Level Ila • Large (> 12 x 15 cm) unilateral frontotemporoparietal decompressive craniectomy for herniation or refractory ICP • Seizure prophylaxis treatment for 7 days • Basal caloric replacement by 5th day Level llb • ICPm use in severe TBI, treat ICP > 22 • High-dose barbiturate therapy in hemodynamically stable patients with highly refractory intracranial hypertension • Maintain CPP > 60-70 mmHg • Transgastric jejunal feeds if ventilated Level Ill • EVD in patients with initial GCS < 6 • Antimicrobial-impregnated EVD catheter to prevent infection • Low dose heparin or LMWH may be used for DVT prophylaxis albeit with risk of increased hemorrhage • Jugular bulb monitoring of arteriovenous oxygen, maintain venous O sat > 50% • Maintain SBP > 100 mmHg for age 50-69; and > 110 mmHg for ages 15-49 and > 70 years old

Not recommended:

Insufficient data to support recommendation:

Level I • Use of corticosteroids

• ICPm in all patients with GCS 3-8 and abnormal CT

Level Ila • Bifrontal decompressive craniectomy (not found to improve outcome, though will decrease ICP and ICU stay) • Povidone-iodine oral care (increased risk of aspiration pneumonitis) • Prophylactic phenytoin or valproate for late (> 7 days) post-traumatic seizures

• ICPm in patients with normal CT and age > 40 with motor posturing

Level IIB • Early, prophylactic hypothermia • Prolonged prophylactic hyperventilation to pCO < 25 mmHg • Barbiturate infusion for burst suppression as prophylaxis against development of intracranial hypertension • Prolonged continuous propofol infusion

• Mannitol 0.25 to 1 g/kg for elevated ICP. If no ICPm use only for signs of transtentorial herniation or progressive neurological deterioration

Level Ill • Aggressive attempts to maintain CPP > 70 mmHg with fluids and vasopressors (risk of respiratory failure)

• Hyperventilation as a temporizing measure for reduction of ICP • Avoidance of hyperventilation for 24 h after injury due to presumed decreased CBF

• Levetiracetam vs. phenytoin for seizure prophylaxis • No preferred agent, dose, or timing of pharmacologic DVT prophylaxis

  

  

References 1. Aarabi B, et al. Predictors of outcome in civilian gunshot wounds to the head. J Neurosurg. 2014;120:1138–1146. 2. Benifla M, et al. Dural sinus obstruction following head injury: a diagnostic and clinical study. J Neurosurg Pediatr. 2016;18:253–262. 3. Bizhan A, Mossop C, Aarabi JA. Surgical management of civilian gunshot wounds to the head. Handb Clin Neurol. 2015;127:181–193. 4. Carney N, et al. Guidelines for the management of severe traumatic brain injury, fourth edition. Neurosurgery. 2017;80:6–15. 5. Hammon WM. Analysis of 2187 consecutive penetrating wounds of the brain. J Neurosurg. 1971;34:142–144. 6. Heary RF, Hunt CD, Krieger AJ, et al. Nonsurgical treatment of compound depressed skull fractures. J Trauma. 1993;35:441. 7. Kim YS, et al. Traumatic dural venous sinus injury. Korean J Neurotrauma. 2015;11:118–123. 8. Meier U, et al. The traumatic dural sinus injury: a clinical study. Acta Neurochir (Wien). 1992;119(1–4):91–93. 9. Negrotto M, et al. Multidirectional spontaneous migration of intracranial bullet: a case report and literature review. Neurosurgery Case Review. 2019;2:019. 10. Zafonte R, et al. Moving bullet syndrome: a complication of penetrating head injury. Arch Phys Med Rehabil. 1998;79:1469–1472.

C H A P T E R

6

Neck Pain After Syncopal Fall Yuanxuan Xia, MD n

Jennifer E. Kim, MD

Consult Page A 75 year old man with syncope and fall with dens fracture

Initial Imaging

A

B

Figure 6.1 Coronal (A) and sagittal (B) views of a cervical spine CT without contrast demonstrate a type II odontoid fracture with no displacement or retropulsion.

Walking Thoughts n n n n n n

n

22

Does the patient have any other injuries? Is the patient hemodynamically stable? What is his neurological exam? Is he wearing a cervical collar? Is the fracture unstable? Will he need surgery? What are his medical comorbidities? Does he have any bleeding disorders? Does he take any anticoagulants or antiplatelet agents that need to be reversed? What labs have already been sent? What labs are pending?

6—Neck PAiN AFter SyNcoPAl FAll

23

History of Present Illness A 75 year old male with no significant past medical history presents to the emergency department after an unwitnessed syncopal fall. The patient lives independently at home where he serves as the primary caregiver for his daughter with cerebral palsy. He was walking down the stairs to grab a drink for his daughter when he slipped and fell to the bottom. He endorses transient loss of consciousness but was able to call emergency medical services after regaining consciousness. He currently endorses neck, right elbow, and right flank pain, but denies any weakness, numbness, tingling, or bowel and bladder difficulties. He has never had a syncopal fall before and denies any prodromal symptoms such as headache or chest pain. He takes aspirin 81 mg daily for general cardiac prophylaxis, and last took it this morning, approximately eight hours prior to presentation. CT of the head, chest, abdomen, and pelvis was performed and demonstrates a type II odontoid fracture (Figure 6.1), a nondisplaced right zygomatic arch fracture, and a right flank hematoma. The trauma and neurosurgery services were consulted in this context.

Vital Signs T 36.6°C, HR 77, RR 21, BP 148/78, SpO2: 98% on room air

Pertinent Labs Hgb 13.3, WBC 9.8, Plt 174, Glu 104 Troponin 0.03

Physical Exam Hard cervical collar in place Alert & oriented to self, place, and year Pupils equal, round, and reactive to light Extraocular movements intact bilaterally, tongue midline, face symmetric Bilateral upper extremities: 5/5 deltoid/biceps/triceps/wrist flexion/wrist extension/interosseous Bilateral lower extremities: 5/5 hip flexion/knee flexion/knee extension/dorsiflexion/plantar flexion/toe extension Sensation intact to light touch throughout Negative Hoffman’s reflexes bilaterally No ankle clonus bilaterally No hyperreflexia Downgoing toes bilaterally No tenderness to midline palpation throughout the spine Right flank ecchymosis with palpable subcutaneous hematoma Minor abrasions over the right eyebrow, elbow, and knee

Triage Management This patient presents with an odontoid (dens) fracture but is neurologically intact despite endorsing minimal neck pain. It is important to note on imaging that the fracture is nondisplaced, with no angulation, distraction, or retropulsion. However, type II odontoid fractures are unstable and usually warrant operative management.4 Surgery is recommended but given the patient’s presentation and ongoing right flank hematoma, he requires preoperative clearance from the trauma and medicine services. A syncope work-up with an electrocardiogram, basic blood work (e.g. complete blood count and basic metabolic panel), and an echocardiogram will be performed. His aspirin is

24

SECTION I—TRAUMA

held, and a hard cervical collar (e.g. Miami J) must be maintained at all times. An MRI of the cervical spine could be obtained to evaluate for the presence of epidural hematoma and ligamentous injury. This is a non-urgent but operative consult.

Assessment This is a 75 year old male with no significant past medical history who presents after a fall with loss of consciousness. CT of the cervical spine demonstrates a type II odontoid fracture. His other injuries include a nondisplaced right zygomatic arch fracture and a right subcutaneous flank hematoma. The patient takes aspirin 81 mg daily for general prophylaxis and last took it this morning. He has minimal neck pain but currently denies any weakness, numbness, tingling, or bowel/ bladder complaints. Fortunately, he is neurologically intact.

Plan n n n n n n n

n

n

Admission to the neurosurgical or trauma floor Hold aspirin Hard cervical collar (e.g. Miami J) at all times Serial hemoglobin checks and abdominal binder for flank hematoma Otolaryngology/plastic/maxillofacial surgery evaluation for zygomatic arch fracture Medicine evaluation for preoperative clearance and syncope workup Consider MRI of the cervical spine to evaluate for epidural hematoma or ligamentous injury Consider CT angiogram of the neck to evaluate for vertebral artery anatomy for operative planning Planning for operative management once preoperative clearance, syncope workup, and imaging is performed

LEARNING POINTS

• In the younger population, odontoid fractures are usually the result of high energy trauma (e.g. motor vehicle collision); in the elderly, most are secondary to low-energy impact falls and reduced bone density.3 • Odontoid fractures are categorized into three types based on the common Anderson and D’Alonzo classification scheme (Table 6.1, Figure 6.2).1,5 • Cervical spine CT is the imaging modality of choice. CT angiogram may also be ordered to better evaluate the vertebral arteries when posterior instrumentation is planned. • Surgical treatment options include:6 • Anterior fusion procedures including odontoid screw (anterior odontoid osteosynthesis) and transoral odontoidectomy. • Posterior instrumented fusion of C1-C2 (this can reduce lateral cervical rotation by up to ∼50%). • Odontoid fracture management in the elderly is challenging.2 Elderly patients do not tolerate rigid halo vest immobilization and have increased mortality rates up to ∼26%. Rigid cervical collars are an alternative, but exhibit lower rates of fusion. Overall, surgery is preferred when possible;6 conservative management may be otherwise considered in poor surgical candidates. • Normal odontoid process anatomical variants that mimic odontoid fractures include:4 • Type I mimic: persistent ossiculum terminale (ossification centers of the odontoid tip and odontoid process fail to fuse during development). • Type II mimic: os odontoideum (ossification centers of the odontoid process and C2 vertebral body fail to fuse during development).

6—Neck PAiN AFter SyNcoPAl FAll

TABLE 6.1 n Anderson and D’Alonzo Classification for and Management of Odontoid Fractures1,5 Type

Fracture Location

Stability

Management

I

Rostral tip of odontoid process

Stable

• Conservative management • Hard cervical collar (e.g. Miami J) for 6-12 weeks achieves fusion in nearly 100% of cases

II

Base of odontoid process

Unstable

• Surgical management when possible • Optional conservative management: - Halo vest immobilization for younger (≤50 years) patients - Hard cervical collar for elderly patients who are poor surgical candidates

III

C2 vertebral body ± C2 facets

Usually stable

• Conservative or surgical management • Hard cervical collar (e.g. Miami J) achieves fusion in 50-65% of cases • Halo vest immobilization achieves fusion in up to ∼85-99% of cases

  

Type I

Type II

Type III

Figure 6.2 Schematic of Type I, II, and III odontoid fractures.

References 1. Anderson LD, D’Alonzo RT. Fractures of the odontoid process of the axis. 1974. J Bone Joint Surg Am. 2004;86(9):2081. 2. Guan J, Bisson EF. Treatment of odontoid fractures in the aging population. Neurosurg Clin N Am. 2017;28(1):115-123. 3. Ochoa G. Surgical management of odontoid fractures. Injury. 2005;36(suppl 2):B54–B64. 4. Robinson AL, Möller A, Robinson Y, Olerud C. C2 fracture subtypes, incidence, and treatment allocation change with age: a retrospective cohort study of 233 consecutive cases. BioMed Reserach International. 2017:8321680. 2017. 5. Ryken TC, Hadley MN, Aarabi B, et al. Management of isolated fractures of the axis in adults. Neurosurgery. 2013;72(suppl 2):132–150. 6. Wagner SC, Schroeder GD, Kepler CK, et al. Controversies in the management of geriatric odontoid fractures. J Orthop Trauma. 2017;31(suppl 4):S44–S48.

C H A P T E R

7

Neck Pain After Trauma Wuyang Yang, MD/MS n

Jennifer E. Kim, MD

Consult Page 60 year old male pedestrian struck, found to have C1 burst fracture

Initial Imaging

A

B

C

Figure 7.1 Axial views of a CT c-spine show a posterior arch fracture with right posterolateral C1 lateral mass displacement (A) and an anterior arch fracture (B). Sagittal view demonstrates the posterior and anterior displacement of the right lateral mass burst fracture (C).

Figure 7.2 Coronal view of a CT c-spine demonstrates 8 mm of overhang of the right C1 lateral mass on the C2 lateral mass (concern for TAL injury per the Rule of Spence).

26

a

27

Walking Thoughts n n n n n n n

n

What is the mechanism of injury? Does the patient have any neurological deficits? What is the exact fracture pattern on imaging? Is this a stable or unstable fracture? Are there any other injuries, including other spinal fractures? Is a hard cervical collar in place? Does the patient need an MRI to evaluate for ligamentous injury or epidural hematoma? Is there concern for vertebral artery injury and has a CT angiogram (CTA) been obtained to evaluate the vasculature? Is the patient a surgical candidate? Is he on any anticoagulant or antiplatelet agents?

History of Present Illness A 60 year old male with a history of hypertension, hyperlipidemia, and left eye blindness presents to the emergency department (ED) after being struck by a motor vehicle at an unknown speed. He was reportedly GCS 15 on arrival, moving all extremities in the trauma bay, and complaining of neck pain. Initial pan-body imaging demonstrated multiple maxillofacial fractures and concern for an expanding 6 by 4 cm retropharyngeal, prevertebral hematoma. Due to concern for impending airway compromise, the patient was emergently intubated by the otolaryngology team in the ED. CT c-spine revealed a C1 burst fracture with C1 on C2 lateral mass subluxation. CTA of the neck was performed and did not reveal any dissection of the carotid or vertebral arteries. The patient takes aspirin 81 mg daily.

Vital Signs T 37.6°C, HR 72, RR 16, BP 106/47, SpO2; 99% on ventilator

Pertinent Labs Na 137, Hgb 12.7, Plt 221, INR 1.0, PT 10.0, aPTT 20.7

Physical Exam Intubated, sedation held Hard cervical collar in place Eyes open to voice Left surgical pupil; right pupil round and reactive Right peri-orbital swelling and right facial laceration Following commands in all extremities Bilateral upper extremities: 5/5 deltoid/biceps/triceps/wrist flexion/wrist extension/interosseous Bilateral lower extremities: 5/5 hip flexion/knee flexion/knee extension/dorsiflexion/plantar flexion/toe extension Negative Hoffman’s reflexes bilaterally No ankle clonus bilaterally

Triage Management The patient has an unstable atlas fracture that requires surgery; however, his management is complicated by his multiple injuries. Imaging reveals a type 4 Jefferson fracture with an intact odontoid process and a normal atlanto-dens interval (ADI). However, there is 8 mm of overhang of the

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SECTION I—TRAUMA

right C1 lateral mass on the C2 lateral mass, suggestive of transverse atlantal ligament (TAL) injury per the Rule of Spence. The patient appears to be neurologically intact and is moving all muscle groups with good strength. At this time, his prevertebral hematoma and airway protection takes precedence, and he will be admitted under the trauma service to the surgical ICU for close monitoring and possible embolization of his prevertebral arteries for his expanding retropharyngeal hematoma. He should be on strict spinal precautions with a rigid cervical collar on at all times.

Assessment This is a 60 year old male with a history of hypertension, hyperlipidemia, and left vision loss, presenting after being struck by a motor vehicle. Imaging reveals a type 4 Jefferson fracture with posterolateral displacement of the right C1 lateral mass, concerning for transverse ligament injury. The patient also has a prevertebral hematoma with active contrast extravasation and possible airway compression, and has been intubated for airway protection. A hard cervical collar is in place and the patient has no focal neurological deficits on exam. Management of his airway and mediastinal injuries are of higher priority in the acute management of this patient. Neurosurgery will continue to follow the patient for operative planning once he is stabilized.

Plan Admission to the surgical ICU under the trauma service No acute neurosurgical intervention given his active prevertebral hematoma n Neurological exams every hour n Strict spinal precautions, including a rigid cervical collar (e.g. Miami J) in place at all times and logrolling to turn the patient n MRI c-spine without contrast to evaluate for ligamentous disruption and epidural hematoma n Otolaryngology/plastic surgery/maxillofacial consultation for facial fractures n Operative planning for spinal stabilization pending medical stability n n

LEARNING POINTS

• Initial assessment of atlas fractures should be focused on determining whether the fracture is stable or unstable. There are multiple classifications of C1 fractures, but the Jefferson classification is most widely used (Table 7.1).1 • Measurements to consider include: • ADI (normal range is 96% successful healing rates. • Combined anterior and posterior arch fractures of the atlas without TAL injury can be managed by external cervical immobilization devices for 10-12 weeks. • Combined anterior and posterior arch fractures of the atlas with TAL injury can be treated with halo orthosis for 12 weeks alone, or surgical management, which is typically C1-2 stabilization and fusion. • Timing of surgery is also dependent on the overall condition of the patient. If the patient is neurologically intact but has other, more urgent, medical issues, the patient can be kept in a cervical immobilization device until medically stabilized/optimized.

29

a

TABLE 7.1 n Jefferson Classification of C1 Fractures Type

Description

Stability

Type 1

Isolated posterior arch fracture

Stable

Type 2

Isolated anterior arch fracture

Stable

Type 3

Both anterior and posterior arch fracture, usually bilateral

Stability depends on integrity of transverse ligament

Type 4

Lateral mass fracture

Unstable

  

References 1. Jefferson G. Fracture of the atlas vertebra. Report of four cases, and a review of those previously recorded. BJS (British J Surgery). 1919;7(27):407–422. 2. Kakarla UK, Chang SW, Theodore N, Sonntag VKH. Atlas fractures. Neurosurgery. 2010;66(suppl_3): A60–A67. 3. Ryken TC, Aarabi B, Dhall SS, et al. Management of isolated fractures of the atlas in adults. Neurosurgery. 2013;72(suppl_3):127–131. 4. Spence KFJ, Decker S, Sell KW. Bursting atlantal fracture associated with rupture of the transverse ligament. J Bone Joint Surg. 1970;52(3):543–549.

C H A P T E R

8

Back Pain After Work Accident Brian Y. Hwang, MD n

Jennifer E. Kim, MD

Consult Page 38M pw severe pain with severe trauma to the back

Initial Imaging

A

B

Figure 8.1 Sagittal CT of the thoracic spine without contrast demonstrates (A) fracture-dislocation involving all 3 columns of T11-T12 with a teardrop fracture of T12, disruption of the supero-anterior endplate of the T12 vertebral body, posterior displacement of the T12 vertebral body, markedly enlarged T11-T12 interspinous process distance, and (B) bilateral perched facets. The findings are consistent with an unstable flexiondistraction injury, or soft-tissue Chance fracture.

30

31

8—BAck PAin After Work Accident

* A

B

Figure 8.2 STIR MRI of the thoracic spine (A) demonstrates disruption of the anterior and posterior longitudinal ligaments (yellow arrow) as well as the posterior ligamentous complex (asterisk). Axial T2-weighted MRI at the level of T12 (B) shows moderate spinal canal stenosis.

Walking Thoughts n n n n

n

n

What was the mechanism of injury? Was it witnessed? What treatments have been given en route to the hospital? Has the patient been evaluated by the trauma team? What other injuries does he have, and which is the most critical? Is he hemodynamically stable? Any concern for intracranial or cervical/lumbar spine injury? What is his current clinical and neurological status? Is he on any anticoagulants or antiplatelet agents?

History of Present Illness A 37 year old male construction worker with no significant medical history presents with severe mid- and low-back pain after an accident at work an hour ago. A 7000 pound excavator tipped over, hit him across the neck, pushed him into a 3 feet ditch, and pinned him to the ground in a flexed position. He was extracted by emergency medical services and brought to the emergency department. He denies loss of consciousness. He was unable to move his legs for several minutes before regaining full strength. Currently, he has a 10 out of 10 burning, throbbing pain originating from the mid- and low-back that wraps around to the front. He denies leg pain, paresthesias, weakness, bowel or bladder incontinence, or saddle anesthesia. He is not on any anticoagulant or antiplatelet medications. His last oral intake is a glass of water 8 hours ago.

Vital Signs T 37.7°C, HR 62, RR 20, BP 130/84, SpO2 96% on 2 L/min O2

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Pertinent Labs Hgb 15.3, Plt 294, INR 0.9, aPTT 23.7

Physical Exam Alert & oriented to self, place, and year, in mild distress from back pain Hard cervical collar in place Bilateral upper extremities 5/5 strength in all muscle groups Bilateral lower extremities 5/5 strength in all muscle groups Sensation to light touch and pinprick grossly intact throughout, no sensory level Proprioception intact No clonus, no Hoffmann’s sign 2+ reflexes throughout Downgoing toes Rectal tone present Intact perineal and perianal sensation  

Thoracolumbar Injury Classification and Severity Scale (TLICS) (Table 8.1) Morphology: 4 Integrity of posterior ligamentous complex: 3 Neurological status: 0 Total score: 7

TABLE 8.1 n Thoracolumbar Injury Classification and Severity Score (TLICS) I

Morphology

Compression Burst Translation/rotation Distraction

1 2 3 4

CT

II

Integrity of Posterior Ligamentous Complex

Intact Suspected Injured

0 2 3

CT/MRI

III

Neurological Status

Intact Nerve Root Complete Cord Incomplete Cord Cauda Equina

0 2 2 3 3

Physical Examination

Surgery

0-3 4 ≥5

Nonoperative Indeterminate Operative

Management Recommendation   

  

33

8—BAck PAin After Work Accident

Triage Management Based on the mechanism of injury, he is a high-risk trauma patient. The patient requires thorough primary and secondary assessments by the trauma team. Flexion-distraction injuries of the spine are commonly associated with intra-abdominal injuries (50%) that can present in a delayed fashion.1 The patient has an unstable thoracic spinal injury with a high risk of neurological deterioration. The patient is also at an elevated risk of intracranial and cervical injuries. He requires a pan neuroaxis CT if it has not been done already. The emergency department and the trauma team should use full spinal precautions during their evaluation and management. The cervical collar will be applied until it can be safely cleared. He will also be monitored closely for spinal/ neurogenic shock. This is an urgent, operative consult.

Assessment This is a 37 year old male who presents with a severe traumatic unstable T11-T12 soft-tissue Chance fracture with ASIA E and TLICS score of 7. He is currently neurologically intact and hemodynamically stable with a chief complaint of thoracic radicular pain. If he is cleared by the trauma team from other life-threatening injuries, he will need urgent surgery to stabilize his thoracic spine.

Plan Full trauma evaluation Strict spinal precautions (hard cervical collar, log-roll, use of spine board for transfer, bed rest, flat) n Arterial line and foley placement; consider central line if vasopressors are needed n Mean arterial pressure >85 for spinal cord perfusion n Pre-operative labs, including type and screen; reserve 2 units of red blood cells for surgery n Upon trauma clearance, urgent surgery for posterior thoracolumbar fusion with reduction of kyphotic deformity and facet dislocation (Figure 8.3) n n

A

B

Figure 8.3 Postoperative lateral (A) and anteroposterior (B) x-rays of the thoracolumbar spine demonstrates instrumentation from T11 to L1.

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SECTION I—TRAUMA

Posting highlights: Jackson table n Intraoperative neuromonitoring: MEP/SSEP/EMG n Fluoroscopy, intra-operative CT n High-speed drill n Spinal fusion instrumentation n Bone allograft n For anesthesia: mean arterial pressure >85 for spinal cord perfusion, particularly during induction Admission to the neurocritical care unit after surgery

n

n

n

LEARNING POINTS

• In cases of severe trauma, evaluation of concomitant injuries is paramount. Thus, assume that there is a cervical spine injury until proven otherwise.

• Chance fractures result from a flexion-distraction mechanism. They are generally a threecolumn injury with compression of the anterior elements and distraction of the posterior elements. There are bony and soft-tissue variants that can co-exist in the same patient. • Before neurosurgical intervention, always consider the ABC’s of trauma (airway, breathing, circulation). Regardless of neurological status, patients must be hemodynamically stable before surgery is considered. Until surgery, strict spinal precautions must be maintained. • Patients with injuries at the T6 level and above must be closely monitored for signs of neurogenic shock including hypotension, abnormal heart rate, and respiratory difficulty. Maintaining good spinal cord perfusion with elevated mean arterial pressure is essential during this period. The Thoracolumbar Injury Classification and Severity (TLICS) score is a guide to managing • patients with thoracolumbar injuries (Table 8.1).2

References 1. Bourne JT, Baker ADL, Khatri M. A combined bony and soft tissue, thoracic chance fracture: late displacement following conservative treatment. Case Reports in Orthopedics. 2017; 2017:6528673. 2. Lee JY, Vaccaro AR, Lim MR, et al. Thoracolumbar injury classification and severity score: a new paradigm for the treatment of thoracolumbar spine trauma. J Orthop Sci. 2005;10:671–675.

C H A P T E R

9

Left Arm And Neck Pain After Car Accident Brian Y. Hwang, MD n

Jennifer E. Kim, MD

Consult Page 73yF passenger in an MVC p/w neck and LUE pain. L C6-7 perched facet on CT.

Initial Imaging

A

B

C

Figure 9.1 Midline sagittal (A) CT of the cervical spine without contrast demonstrates 3 mm anterior subluxation of C6 over C7 and splaying of the posterior elements. Left sagittal view (B) shows the left inferior articular facet of C6 is perched on the fractured superior articular facet of C7. On the axial view (C), the left C7 facet fracture extends through the transverse process and into the transverse foramen of C7 (arrow).

Walking Thoughts n n n n n n

What was the mechanism of the accident? Was the patient wearing a seat belt? Has the patient been evaluated by the trauma team? What other injuries does she have, and which is the most critical? Any concern for intracranial injury? What is her current clinical and neurological status? Is she hemodynamically stable? Does she have any previous medical problems, surgeries, or medications (e.g. anticoagulants and antiplatelets) that complicate her management?

History of Present Illness A 72 year old female in good health presents with neck and left arm pain after a motor vehicle accident 3 hours prior. She was a belted passenger of a sedan when her vehicle rear-ended 35

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another at approximately 20 miles per hour. She felt her neck snap forward. She denies loss of consciousness or head strike. She immediately felt severe neck pain radiating down to her left arm and fingertips. She was brought to her local emergency department, where she was found to have an “abnormal x-ray” of her neck, and then transferred to our tertiary trauma center. She currently reports 4 out of 10 neck pain, tingling and numbness radiating down the left arm into her fingers, and mild weakness in her left arm. She denies pain, abnormal sensation or weakness anywhere else and reports no new bowel or bladder dysfunction since the accident. She denies any anticoagulant or antiplatelet use. The trauma team has assessed her, and she has no other injuries. Her head CT is unremarkable.

Vital Signs T 36.7°C, HR 113, RR 18, BP 149/70, SpO2 99% on room air

Pertinent Labs Hgb 13.2, Plt 214, INR 1.0, aPTT 28.4

Glasgow Coma Scale Motor: 6 Verbal: 5 Eye opening: 4 GCS Total:15

Physical Exam Alert, awake, oriented to self, place, and year In mild distress from neck pain Hard cervical collar in place Pupils equal, round, and reactive to light Extraocular movements intact, face symmetric, tongue midline Deltoid

Biceps

Triceps

Wrist Flexion

Wrist Extension

Grip

 

 

 

 

Bilateral lower extremities 5/5 in all muscle groups Decreased sensation (20% subjective reduction) to light touch and pinprick in a left C6, C7 distribution but otherwise sensation intact No clonus or Hoffmann’s sign Downgoing toes bilaterally 2+ reflexes throughout Rectal tone present Intact perineal/perianal sensation

9—Left Arm ANd NeCk PAiN After CAr ACCideNt

37

 

Triage Management This patient is hemodynamically stable, but her exam is notable for a left C7 paresis and paresthesias in a left C6-C7 distribution. She has an unstable flexion-rotation cervical injury that necessitates strict cervical spinal precautions. An MRI of her cervical spine may be obtained to confirm ligamentous injury and assess for hematoma or disc herniation. Because the fracture extends into the left transverse foramen, she will also need a CT angiogram (CTA) of her neck to evaluate for potential vessel injury. She will require urgent surgery for reduction, decompression, and stabilization of her perched facet. This is an urgent, operative consult.

Additional Imaging

Figure 9.2 Axial CT angiogram of the neck demonstrates that the vertebral arteries (arrows) are anterior to the vertebral body at the C6-C7 level. The vertebral arteries are patent and without evidence of dissection or stenosis.

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SECTION I—TRAUMA

Figure 9.3 Sagittal T2-weighted MRI of the cervical spine without contrast demonstrates rupture of the posterior longitudinal ligament, ligamentum flavum, and interspinous ligament at the C6-C7 level. There is no cord signal abnormality or significant spinal canal stenosis.

Subaxial Injury Classification and Severity Scale (SLICS) (Table 9.1) Morphology: 4 Integrity of Discoligamentous Complex: 2 Neurological Status: 1 Total score: 7 TABLE 9.1 n Subaxial Cervical Spine Injury Classification System (SLICS) SLICS Three Independent Predictors I

Morphology

No abnormality Compression Burst Distraction Rotation/translation

0 1 2 3 4

CT

II

Integrity of Discoligamentous Complex

Intact Indeterminate Disrupted

0 1 2

CT/MRI

III

Neurological Status Intact Nerve Root Complete Cord Incomplete Cord Continuous cord compression in the setting of neuro deficit

0 1 2 3 +1

Physical Examination

0-3 4 ≥5

Nonoperative Indeterminate Operative

Management Recommendation   

  

Surgery

9—Left Arm ANd NeCk PAiN After CAr ACCideNt

39

Assessment This is a 72 year old female who presents with C6-C7 subluxation, unilateral left C6 perched facet, and left C7 facet fracture after a motor vehicle accident. Her exam is notable for triceps paresis and radicular symptoms in a left C6-C7 distribution. MRI of her cervical spine without contrast is notable for posterior longitudinal ligament, ligamentum flavum, and interspinous ligament rupture at C6 and C7. Her neck CTA is unremarkable. She has no other injuries and denies anticoagulant or antiplatelet use. She has an unstable cervical injury (SLICS score of 7) that requires surgery. Given the patient’s age and the injury pattern, traction and closed reduction of the perched facet may be less effective in providing decompression of the nerve root, fusion, and durable stability.

Plan Hard cervical collar at all times Pre-operative labs, including type and screen; reserve 2 units of red blood cells for surgery n Urgent OR planning for posterior cervical decompression and C5-T2 fusion (Figure 9.4) n Posting highlights: n Mayfield head clamp n Intraoperative neuromonitoring: MEP/SSEP/EMG n Spinal fusion instrumentation n High-speed drill and/or ultrasonic bone scalpel n Fluoroscopy n n

A

B

Figure 9.4 Anteroposterior (A) and lateral (B) cervical spine x-rays demonstrate C5-T2 instrumentation with reduction of the left C6-C7 perched facet and subluxation.

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SECTION I—TRAUMA

LEARNING POINTS

• Facets are the major stabilizers of the cervical spine, particularly in flexion and rotation. Facet fractures and dislocations (perched or jumped) can be subtle. Always closely evaluate the spinolaminar and posterior spinous lines on sagittal films. • Perched facets occur when the inferior articular process sits on top of the superior articular process of the vertebra below. Jumped or locked facets occur when the inferior articular process sits anterior to the superior articular process of the vertebra below. • These facet injuries can be managed with either closed reduction and bracing or open reduction and internal fixation. Treatment selection depends on the patient history and exam, injury severity, and presence of associated spinal injuries. In unilateral facet injuries, high body mass index, fracture dislocation >2 mm, and presence of radiculopathy are associated with high risk for failure of non-operative management.1 • In cases of trauma, when pinning for cervical spine surgery, ensure there are no skull fractures prior to pinning. The Subaxial Cervical Spine Injury Classification System (SLICS) is a guide for managing • patients with subaxial cervical spine injuries (Table 9.1).2

References 1. Vaccaro AR, Hulbert RJ, Patel AA, et al. The subaxial cervical spine injury classification system: a novel approach to recognize the importance of morphology, neurology, and integrity of the disco-ligamentous complex. Spine. 2007;32:2365–2374. 2. van Eck CF, Fourman MS, Abtahi AM, Alarcon L, Donaldson WF, Lee JY. Risk factors for failure of nonoperative treatment for unilateral cervical facet fractures. Asian Spine J. 2017;11:356–364.

C H A P T E R

10

Back Pain After Low-Impact Trauma Jennifer E. Kim, MD

Consult Page 57F sp fall, CT T12 burst fracture.

Initial Imaging

A

B

P

Figure 10.1 Sagittal (A) and axial (B) non-contrast lumbar spine CT depicts T12 vertebral body (arrow) burst fracture with approximately 30% height loss and 4 mm posterior retropulsion.

Walking Thoughts What was the mechanism of injury? Does the patient have any neurological deficits? n What kind of pain does the patient have? n What is the baseline function and ambulatory status of the patient? n Has the trauma team evaluated the patient? n Is there concern for concomitant cervical or lumbar injuries? Is the patient in a cervical collar? n n

41

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SECTION I—TRAUMA

History of Present Illness A 56 year old female with a history of osteoporosis on calcium and zoledronic acid presents after a fall from seated position. The patient was on a boat when a wave bounced her up in the air and she landed on her buttocks. She denied head strike or loss of consciousness. She had immediate and persistent low back pain but was able to get up and ambulate independently after the fall. She currently denies any radicular pain, numbness, paresthesias, weakness, or bowel or bladder problems including retention or incontinence. CT of the lumbar spine without contrast demonstrates an acute burst fracture of the T12 vertebral body with approximately 30% height loss and 4 mm of posterior retropulsion with mild narrowing of the spinal canal at T11-T12. The trauma team has evaluated her, and she has no other injuries.

Vital Signs T 35.6°C, HR 80, BP 110/62, SpO2 98% on room air

Pertinent Labs Na 143, Hgb 10.3, Plt 343, INR 1.1, aPTT 27.6

Physical Exam Alert, awake Oriented to self, hospital, and date and year Hard cervical collar in place Bilateral upper extremities 5/5 strength in all muscle groups Bilateral lower extremities 5/5 strength in all muscle groups 2+ reflexes throughout No pathologic clonus or Hoffman’s sign Downgoing toes Sensation intact to light touch throughout

Triage Management The patient is neurologically intact (no focal deficits, full strength in all muscle groups with no signs of myelopathy or radiculopathy). Depending on the institution and availability of MRI, as well as surgeon’s preference, an MRI of the thoracic and lumbar spine without contrast may be ordered to assess for ligamentous disruption or underlying pathology. The patient is also hemodynamically stable, and her pain has been well controlled with oral pain medications, so she could be observed in the emergency department while further studies are obtained. This is a nonurgent, but potentially operative consult pending imaging.

Assessment This is a 56 year old female with a history of osteoporosis who presents with a fall from seated position and imaging concerning for an acute burst fracture of the T12 vertebral body without significant height loss and minimal retropulsion. There is no severe spinal canal stenosis. A thoracolumbar MRI without contrast was ultimately completed, which does not show any

10—BACK PAIN AFTER LOW-­IMPACT TRAUMA

43

evidence of underlying pathological lesion, ligamentous disruption, or significant cord compression. The patient is neurologically intact, and her pain is reasonably controlled with oral pain medications. Her TLICS (Thoracolumbar Injury Classification and Severity) Score is 2 indicating a stable injury (morphology: 2, integrity of posterior ligamentous complex: 0, neurological status: 0).

Plan n n

n

n

n

No acute neurosurgical intervention needed at this time Recommend a thoracolumbar brace for comfort (e.g. thoracic lumbar sacral orthosis/ TLSO); some surgeons may request that the patient wears the brace at all times when out of bed Early mobilization with counseling of spinal precautions (no twisting, bending, or lifting) Patient can be discharged from the emergency department with outpatient follow-up and standing/flexion-extension x-rays of the thoracolumbar spine Outpatient evaluation and management of osteoporosis

LEARNING POINTS

• Traumatic compression fractures are usually the result of a high-energy axial load. Burst fractures are a category of compression fractures that result in fracture of the posterior vertebral body cortex with bony retropulsion into the spinal canal. • There are several classification systems that have been developed to describe thoracolumbar fractures e.g. Denis, Magerl, and TLICS.2 • The TLICS score is designed to standardize clinical (operative vs. non-operative) management (Table 10.1). This treatment algorithm takes into account injury or fracture morphology, neurological deficits, as well as injury of the posterior ligamentous complex (PLC). A score of 4 favors instability requiring operative intervention.3 • The TLICS score later led to the AOspine Thoracolumbar Spine Injury Classification system which aims to standardize severity designation by taking into more detailed account fracture morphology, neurological deficits, and ligamentous injury.4 • Traditionally, conservative management included early bracing with TLSO or other extension brace. However, recent evidence suggests that bracing may not be necessary in patients without neurological deficits.1 The decision to recommend orthosis should be made on a case-by-case basis. • MRI may be indicated to assess for ligamentous compromise, as well as to evaluate for underlying pathology such as malignancy that predisposed the patient to a pathological fracture. • Fracture from low-impact or minimal trauma (defined as fragility fractures) are concerning for osteoporosis. Outpatient work-up for bone density abnormalities includes blood work (calcium, vitamin D, parathyroid hormone, testosterone level for males) and DEXA scan (Dual-energy X-ray absorptiometry). • Fractures at the thoracolumbar junctional level, such as this case, may need closer followup to monitor for the development of instability or deformity (e.g. kyphosis or scoliosis), which could warrant operative management.

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TABLE 10.1 n Thoracolumbar Spine Injury Classification Score (TLICS)

Injury morphology Compression Burst Translation/rotation Distraction PLC integrity Intact Suspected or indeterminate injury (edema/enhancement) Definite injury (discontinuity) Neurological Intact involvement Nerve root compression Cord/conus injury – complete Cord/conus injury – incomplete Cauda equina syndrome TLICS SCORE 4 Operative

1 2 3 4 0 2 3 0 2 2 3 3

  

  

References 1. Bailey CS, Urquhart JC, Dvorak MF, et al. Orthosis versus no orthosis for the treatment of thoracolumbar burst fractures without neurologic injury: a multicenter prospective randomized equivalence trial. Spine J. 2014;14(11):2557–2564. 2. Ghobrial GM, Maulucci CM, Harrop JS. Evaluation, Classification, and Treatment of Thoracolumbar Spine Injuries. Youmans and Winn Neurological Surgery. 7th ed. Philadelphia, PA: Elsevier; 2017:253– 2545.e2. 3. Vaccaro AR, Lehman RA, Hurlbert RJ, et al. A new classification of thoracolumbar injuries: the importance of injury morphology, the integrity of the posterior ligamentous complex, and neurologic status. Spine. 2005;30(20):2325–2333. 4. Vaccaro AR, Oner C, Kepler CK, et al. AOSpine thoracolumbar spine injury classification system: fracture description, neurological status, and key modifiers. Spine. 2013;38(23):2028–2037.

C H A P T E R

11

Vomiting And Neck Pain After Motor Vehicle Accident Jordina Rincon-Torroella, MD n

Jennifer E. Kim, MD

Consult Page 20M, MVC, here w/ confusion, vomiting and neck pain

Initial Imaging None

Walking Thoughts n n n n n

Have the ABCs been assessed? Has the patient been examined by the trauma team? What imaging is available? Is there an intracranial bleed causing his symptoms? Is the patient wearing a hard cervical collar?

History of Present Illness A 20 year old male is brought in by emergency medical services (EMS) as a trauma after a motor vehicle accident. He was an unrestrained driver at a moderate speed and was T-boned on the driver’s side with airbag deployment. The patient had a brief loss of consciousness after which he was able to self-extricate. Per EMS, he was ambulatory at the scene but confused. On arrival to the emergency room, the patient is GCS14 and combative with several episodes of emesis. On secondary survey, the trauma team noted cervical spine pain to palpation.

Vital Signs T 36.9°C, HR 90, BP 138/52, SpO2 99% on room air

Pertinent Labs In process

45

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SECTION I—TRAUMA

Glasgow Coma Scale Motor: 6 Verbal: 4 Eye opening: 4 GCS Total: 14

Physical Exam Alert, oriented to self, place, and year Left frontal abrasion Hard cervical collar in place Pupils equal, round, and reactive to light Extraocular movements intact, face symmetric, tongue midline No pronator drift Bilateral upper extremities 5/5 in all muscle groups Bilateral lower extremities 5/5 in all muscle groups Sensation intact to light touch throughout No Hoffman’s or clonus Downgoing toes Pain to palpation of lower cervical spine No seatbelt sign

Triage Management For any trauma patient, ABCs are key in the initial assessment. The patient seems to be oxygenating well at room air with good hemodynamics. His blood pressure and heart rate are stable. Given the mechanism of injury, he should be evaluated by the trauma surgery service for primary, secondary, and tertiary surveys. Since he is hemodynamically stable and GCS14, the patient can remain in observation in the emergency room while further studies are obtained. He is combative and vomiting, so a head CT should be prioritized to rule out an intracranial cause. This is an urgent consult.

Initial Plan Trauma evaluation (if not already obtained) Urgent CT of the head and cervical spine without contrast n Rigid cervical collar until clearance obtained n Basic bloodwork CBC, BMP, coagulation factors n Blood alcohol levels and urine toxicology n Nausea control and hydration n Avoid sedating medications until head imaging completed n NPO/IV fluids n n

11—VoMitiNg ANd Neck PAiN After Motor Vehicle AccideNt

47

Imaging

A

B

Figure 11.1 Axial head and maxillofacial head CT without contrast. A. Parenchymal window demonstrates no evidence of intracranial abnormalities. B. Bony window shows no evidence of orbital, maxillofacial, calvarial or skull base fractures.

A

B

Figure 11.2 CT of the cervical spine without contrast. A. Sagittal view demonstrates an acute fracture involving the C7 spinous process (arrow) without malalignment or loss of vertebral height. B. Axial view showing a linear nondisplaced fracture of the spinous process (arrow) without extension into the lamina.

Assessment This is a 20 year old male who presents after a motor vehicle accident with temporary loss of consciousness, combativeness, vomiting, and neck pain. Head CT is unremarkable but CT of the cervical spine shows a C7 spinous process fracture without involvement of the lamina, consistent with a Clay-shoveler’s fracture. He has no focal neurological findings, weakness, or sensory changes. His combativeness and nausea after head trauma with negative imaging findings are concerning for concussion.

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SECTION I—TRAUMA

Plan No acute neurosurgical intervention No need for repeat head imaging n Hard cervical collar at all times for 6 weeks with follow-up in clinic n If stable, the patient can be discharged with post-concussion recommendations. If there is any concern for persistent symptoms, he can be admitted overnight for one day of observation. n n

LEARNING POINTS Concussion1,2 Concussion is defined as symptomatic traumatic brain injury with negative imaging findings. Current recommendations for concussion symptoms are based on the 2008 Clinical Policy for adult mild traumatic brain injury (TBI) compiled by the Center for Disease Control (CDC) and American College of Emergency Physicians (ACEP). These guidelines are centered on adults (≥16 years of age) with nonpenetrating head trauma who present to the emergency room within 24 hours of injury and with GCS of 14 or more. The most important points are: • Head CT is recommended for those patients with positive loss of consciousness, posttraumatic amnesia, or one of the following symptoms: >60 years of age, headache, nausea, memory loss, seizure, GCS 3 cm) with

12—HeADAcHe, NAuSeA, AND SpeecH Difficulty

A

53

B

Figure 12.2 An axial head CT without contrast (A) demonstrates evidence of hyperostosis and possible tumor invasion into the overlying bony calvarium with irregular and osseous infiltration (arrow). An axial CT angiogram (B) shows displacement of the anterior cerebral arteries due to mass effect from the tumor.

significant peritumoral edema and associated mass effect on the surrounding brain. Given the patient’s relatively young age, large tumor size, associated cerebral edema, significant mass effect, and the fact that she is symptomatic, surgery is recommended for diagnosis, treatment of mass effect, and symptomatic relief. The patient’s MRI further demonstrates enhancement in the subgaleal space and the skull adjacent to the tumor (Figure 12.1B). A CT can be performed to better evaluate bony involvement, which in her case, shows osseous infiltration of the tumor (Figure 12.2A). Extensive osseous involvement may require cranioplasty during surgical resection. The patient is started on dexamethasone in the ED. Although she has not experienced another episode of speech difficulty, she may have had a seizure and thus is started on levetiracetam. This is a nonemergent but operative consult.

Assessment This is a 51 year old female with no past medical history who presents with a newly diagnosed large right frontal extra-axial lesion in the setting of work up for headache, nausea, and possible seizure. Her MRI scan is most consistent with a large meningioma and shows significant surrounding edema and midline shift. The patient’s neurological exam is intact, and she will require surgery.

Plan n

n n

Surgical planning for right sided craniotomy versus craniectomy for tumor resection, diagnosis, and decompression Continue levetiracetam, dexamethasone, and proton pump inhibitor Because the patient is neurologically intact, she can be discharged with outpatient surgical planning for the near future

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LEARNING POINTS

• The management of a patient presenting with a new brain mass begins with a detailed clinical history. Including the onset, duration, and quality of symptoms. The clinical exam includes a full neurological assessment of mental status and cranial nerve, motor, sensory, and cerebellar function. Any deficits found on the neurological assessment are correlated with tumor location, associated peritumoral edema, and vascular involvement.2 • Meningiomas are the most common primary intracranial tumor and are classified as WHO grade 1, 2 (atypical), and 3 (anaplastic). Grade 2 and 3 meningiomas have a higher tendency to recur and in rare occasions can metastasize.5 The differential includes hemangiopericytoma and dural-based metastases. • Radiation-associated meningiomas are more likely to be atypical, malignant, and multifocal compared to sporadic meningiomas. • In one of the largest systematic reviews in the literature, the preoperative seizure rate was 29.2% in patients with supratentorial meningiomas. Seizure freedom after surgery was achieved in 69.3% of patients with preoperative seizures. However, new postoperative seizures were seen in 12.3% of patients.1 Tumors with significant edema are associated with an increased risk of seizures. Seizure prophylaxis is frequently not started on patients with posterior fossa meningiomas. • In patients with peritumoral edema leading to focal neurological deficits or symptoms of increased intracranial pressure, corticosteroids can be administered to decrease cerebral edema. However, many patients with meningiomas may have very little to no associated vasogenic edema, even in cases of large tumors. When indicated, dexamethasone can be started at a dose of 4 mg every 6 hours. Postoperatively, the steroids may be tapered or discontinued. • A head CT may be used to evaluate intraosseous involvement, hyperostosis, or calcifications within the tumor. This is important to predict the need for reconstructive techniques. • For meningiomas in the parasagittal region abutting the superior sagittal sinus, additional vascular imaging such as MR or CT venogram can be obtained to further evaluate the relationship of the tumor to the superior sagittal sinus and the extent of sinus invasion. The neurosurgical community is moving away from sinus sacrifice due to the high risk of venous infarction, instead favoring sinus preservation with a small residual that can be managed with adjuvant radiation or repeat surgery.3 • Management options depend on the patient’s clinical presentation, imaging characteristics, tumor growth patterns, and other medical history and comorbidities (Figure 12.3). In the asymptomatic patient, observation is generally favored especially in elderly patients greater than 85 years of age. Indications for surgical decompression include symptomatic tumors, tumors of significant size with substantial peritumor edema, or rapidly enlarging tumors. • The Simpson scale was designed to classify tumor resection and provides a tool to study recurrence rate.6 • Preoperative embolization is rarely recommended. Cerebral angiography can be used to confirm sinus occlusion before sinus sacrifice in selected cases, and an enlarged artery of Bernasconi-Cassinari can be visualized in tentorial meningiomas. • So far, there is no role for chemotherapy. • The recurrence rate is highly variable depending on treatment modality, tumor location, extent of resection, and grade.4 General recommendations for meningioma follow up are shown in Table 12.13 •

12—HeADAcHe, NAuSeA, AND SpeecH Difficulty

Figure 12.3 Schema of meningioma management. GTR: gross total resection.

TABLE 12.1 n European Association of Neuro-Oncology (EANO) guidelines for the follow-up of meningiomas patients. Asymptomatic

First MRI in 6 months Then yearly for 5 years Then biannually

WHO grade 1 (gross total resection)

Baseline MRI within 48h or 3 months post operative Then yearly for 5 years Then biannually

WHO grade 1 (subtotal resection)

Baseline MRI within 48h At 6 months At 12 months Then annually

WHO grades 2 or 3

Every 6 months or every 3 months for rapidly progressing tumors

  

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References 1. Englot DJ, Magill ST, Han SJ, Chang EF, Berger MS, McDermott MW. Seizures in supratentorial meningioma: a systematic review and meta-analysis. J Neurosurg. 2016;124(6):1552–1561. 2. Fogh SE, Johnson DR, Barker 2nd FG, et al. Case-based review: meningioma. Neurooncol Pract. 2016;3(2):120–134. 3. Goldbrunner R, Minniti G, Preusser M, et al. EANO guidelines for the diagnosis and treatment of meningiomas. Lancet Oncol. 2016;17(9):e383–e391. 4. Mendenhall WM, Morris CG, Amdur RJ, Foote KD, Friedman WA. Radiotherapy alone or after subtotal resection for benign skull base meningiomas. Cancer. 2003;98(7):1473–1482. 5. Rogers L, Barani I, Chamberlain M, et al. Meningiomas: knowledge base, treatment outcomes, and uncertainties. A RANO review. J Neurosurg. 2015;122(1):4–23. 6. Simpson D. The recurrence of intracranial meningiomas after surgical treatment. J Neurol Neurosurg Psychiatry. 1957;20(1):22–39.

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Episodes Of Foul Smell And Bad Taste Lydia Ju-mi Bernhardt, MD n

Jordina Rincon-Torroella, MD

Consult Page 25M transfer from outside hospital with brain mass. MRI pending.

Initial Imaging None

Walking Thoughts n n n n n n

n

What are the patient’s symptoms and presentation? What medical comorbidities does the patient have? What is the GCS of the patient? What workup has been completed so far? Where is the mass located and what are the important surrounding structures? Does the patient need surgery? Does he need emergent surgery or can this be deferred electively? Is the patient at risk for further seizures?

History of Present Illness A 25 year old previously healthy male presented to an outside hospital with two days of severe nausea and lightheadedness. He also complained of several episodes of sudden-onset foul smell and bad taste, with each episode lasting for 60 seconds and self-resolving. He denied any associated loss of consciousness. Per report, a head CT at his local emergency department (ED) showed a well-circumscribed hypodense lesion in the posterior left frontal lobe without hemorrhage. He was started on levetiracetam and urgently transferred to our tertiary hospital ED. Here in our ED, he has not had any additional episodes. On questioning, he reports that he has had mild headaches for a few months. The patient denies any vision changes, weakness, numbness, or speech deficits. He denies any use of antiplatelet or anticoagulant medication including aspirin-containing headache cocktails.

Vital Signs T 36.2oC, HR 88, RR 18, BP 131/73, SpO2 100% on room air

Pertinent Labs Na 139, K 4.3, INR 1.0, aPTT 25.9 57

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Glasgow Coma Scale Motor: 6 Verbal: 5 Eye opening: 4 GCS Total: 15

Physical Exam Awake, alert, oriented to self, place, and year Correct naming of three objects and good sentence repetition Pupils equal, round, and reactive to light Visual fields intact to confrontation Extraocular movements intact, face symmetric, tongue midline No pronator drift Bilateral upper extremities 5/5 Bilateral lower extremities 5/5 Sensation intact to light touch throughout

Triage Management This is a young healthy patient who presents with a newly diagnosed brain mass. His transient episodes of foul smell and bad taste are concerning for focal seizures of the temporal lobe. The patient’s exam is intact, including good speech and no visual field cuts, and he has not had any further episodes of seizures since he was started on antiepileptic medication in the ED. He does not have current headaches, vomiting, or blurred vision that would be concerning for increased intracranial pressure. His outside hospital head CT is not available. A brain MRI is performed in the ED which shows a T2 FLAIR hyperintensity at the left frontotemporal lobe extending into the subinsular region (Figure 13.1). There is no appreciable contrast-enhancement or diffusion restriction. These radiological characteristics together with the patient’s age and presentation are consistent with a low grade glioma. He will need surgery for pathological diagnosis and decompression with the goal of achieving gross total resection while avoiding any new neurological deficits. Since his symptoms are under control with anti-seizure medication, the patient can be discharged for follow-up and elective surgery. He will continue his levetiracetam and is also started on a corticosteroid taper. The patient is recommended to return to the ED if he has any new symptoms or recurrent seizures. The risks and benefits of surgery will be discussed during his outpatient appointment with him and his family. This is a nonurgent but operative consult.

Assessment This is a 25 year old previously healthy male who presents with temporal lobe/insular seizures and a new left frontotemporal brain lesion concerning for a low grade glioma. His seizures have been controlled, he is otherwise asymptomatic, and he will require surgery.

aste

A

B

C

D

59

Figure 13.1 Axial brain MRI with and without contrast shows a well-defined 5-cm mass at the left frontotemporal lobe involving the subinsular region with mild mass effect into the basal ganglia. The lesion is hypointense without contrast enhancement on T1 sequences with contrast (A) and hyperintense on T2 sequences (B). T2/ FLAIR sequences show no major surrounding edema (C) without diffusion restriction on ADC sequences (D).

Plan Discharge home with return precautions Continue levetiracetam n Dexamethasone taper over 2 weeks n Proton pump inhibitor while on steroids n Outpatient visit for surgical planning n Preoperative brain MRI with and without contrast with navigation protocol n Consider functional MRI given the location of the mass n n

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Surgical planning for left frontal craniotomy Posting highlights: n Skull clamp n Intraoperative neuromonitoring n Consider awake craniotomy n Consider intraoperative MRI n Neuronavigation n Ultrasonic aspirator n Microscope n Bipolar cautery n Intraoperative ultrasound n For anesthesia: antibiotics, dexamethasone, levetiracetam, mannitol, hyperventilation if intubated. If an awake craniotomy is performed, special coordination with anesthesia is essential. n Will require neurocritical care unit after surgery

n

Follow-Up After discussing the benefits and risks of observation versus surgical resection, the patient and his family decided to proceed with surgery. He underwent a successful left frontal craniotomy with near gross total resection. Intraoperative neuromonitoring, ultrasound, and intraoperative MRI were used to guide the resection (Figure 13.2). The patient had no postoperative deficits and was discharged home on postoperative day 2. Pathology was consistent with a diffuse astrocytoma, IDH mutant (WHO grade 2).

A

B

Figure 13.2 Intraoperative axial T1 (A) and T2 sequences (B) without contrast show good resection with a transcortical operative corridor through the frontal lobe. There is a small region of T2 signal at the anterior temporal region (arrow). Pathology demonstrated diffuse astrocytoma, IDH mutant (WHO Grade 2).

aste

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LEARNING POINTS

• Central nervous system tumors are classified by grade according to the World Health Organization (WHO) guidelines, updated in 2021 to incorporate both histological and molecular markers.5,7 • The introduction of standardized genetic analysis for brain tumors in our daily practice has changed the way we diagnose gliomas over the past decade. This was reflected in the WHO 2021 brain tumor classification. Currently, molecular markers define brain tumor diagnosis. Adult-type diffuse gliomas are now classified into three main categories: • Astrocytoma, IDH mutant • Oligodendroglioma, IDH mutant and 1p/19q codeleted • Glioblastoma, IDH wildtype • Within the IDH mutant astrocytomas, such as the present case, tumors can be WHO grades 2, 3, or 4. • LGGs present most commonly in the second through fourth decades of life with no gender preponderance. • Clinical signs and symptoms vary and are largely attributed to mass effect from invasion into surrounding parenchyma or obstructive hydrocephalus. Seizures are the presenting symptom in up to 80% of patients. These tumor-related seizures manifest as simple or complex partial seizures with or without secondary generalization, and are drug resistant in more than 50% of cases.8 Other patients may present with subtle cognitive or behavioral changes, focal neurological deficits, or clinical signs or symptoms of increased intracranial pressure, such as headache or papilledema. However, patients may also be asymptomatic, without evident abnormalities on neurological examination.6 • Distinguishing between a low grade versus a high grade lesion via CT or MRI is imprecise, but may be performed as a preliminary assessment. On CT scan, LGGs appear as diffuse areas of low attenuation. On MRI, LGGs are often homogenous with hypointensity on T1weighted sequences and hyperintensity on T2-weighted/FLAIR sequences. There is little to no mass effect or surrounding edema (FLAIR hyperintensity). They do not typically enhance with contrast. This differs from grades 3 and 4 gliomas, as higher grade lesions demonstrate increased tumor heterogeneity, contrast enhancement, restricted diffusion on diffusion-weighted imaging, and increased relative cerebral blood volume on perfusionweighted MRI. Positron emission tomography (PET) or MR spectroscopy can be used as adjunctive imaging.2,5-6 • Minimal contrast enhancement and calcifications are more characteristic of oligodendrogliomas. Oligodendrogliomas are low grade gliomas that carry the IDH mutation and 1p19q codeletion. These usually appear as subcortical masses in a young patient with seizures. Oligodendrogliomas have a better prognosis and show good response to systemic chemotherapy.3 • Generally for patients with LGG, maximum safe surgical resection is recommended over observation to improve overall survival, delay malignant transformation, and alleviate symptoms.1,2,6 Surgical adjuncts such as intraoperative neuromonitoring, ultrasound, or intraoperative MRI are useful to ensure maximal resection. Some evidence suggests that supratotal resection performed in non-eloquent areas can significantly delay malignant transformation. Intraoperative cortical and subcortical stimulation during awake craniotomy can provide a higher extent of resection and also reduce the rate of postoperative neurological deficits.4 • In selected cases of WHO grade 2 gliomas, radiation and chemotherapy may be adjuvant to surgery. Early radiotherapy leads to improvement in progression-free survival but not overall survival; however, radiation may compromise long-term neurocognitive function. For high-risk patients such as those over 40 years of age or those with partially resected tumors, chemotherapy with alkylating agents (e.g. temozolomide) improves progressionfree and overall survival.6 • Although patients with LGG have an indolent course and better survival than patients with high grade glioma, diffuse astrocytoma may eventually progress to high grade glioma with poorer survival.

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References 1. Aghi M, Nahed B, Sloan A, Ryken T, Kalkanis SN, Olson JJ. The role of surgery in the management of patients with diffuse low grade glioma: a systematic review and evidence-based clinical practice guideline. J Neurooncol. 2015;125(3):503–530. https://doi.org/10.1007/s11060-015-1867-1. 2. Delgado-López PD, Corrales-García EM, Martino J, Lastra-Aras E, Dueñas-Polo MT. Diffuse lowgrade glioma: a review on the new molecular classification, natural history and current management strategies. Clin Transl Oncol. 2017;19:931–944. https://doi.org/10.1007/s12094-017-1631-4. 3. Engelhard HH, Stelea A, Mundt A. Oligodendroglioma and anaplastic oligodendroglioma: clinical features, treatment, and prognosis. Surg Neurol. 2003;60(5):443–456. https://doi.org/10.1016/ s0090-3019(03)00167-8. 4. Eseonu CI, Rincon-Torroella J, ReFaey K, et al. Awake craniotomy vs craniotomy under general anesthesia for perirolandic gliomas: evaluating perioperative complications and extent of resection. Neurosurgery. 2017;81(3):481–489. https://doi.org/10.1093/neuros/nyx023. 5. Ferris SP, Hofmann JW, Solomon DA, Perry A, Perry A. Characterization of gliomas: from morphology to molecules; 2017:257–269. https://doi.org/10.1007/s00428-017-2181-4. 6. Forst D, Nahed B, Loeffler J, Batchelor T. Low-grade gliomas. Oncol. 2014:403–413. 7. Louis DN, Perry A, Wesseling P, et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol. 2021;23(8):1231–1251. https://doi.org/10.1093/neuonc/ noab106. 8. Rudà R, Bello L, Duffau H, Soffietti R. Seizures in low-grade gliomas: natural history, pathogenesis, and outcome after treatments. Neuro Oncol. 2012;14:55–64.

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14

Headache And Right Arm Weakness Christina Jackson, MD n

Jordina Rincon-Torroella, MD

Consult Page 70M new mass on CT

Initial Imaging

Figure 14.1 An axial head CT without contrast demonstrates a large left fronto-parieto-occipital hypodensity with surrounding edema. There is minimal midline shift.

Walking Thoughts n

n

What is the patient’s neurological exam? Does he have decreased mental status or any associated neurological deficits? How long has the patient had symptoms, and have the symptoms changed recently? 63

64

SECTION II—TUMOR n n n n

n

Has the patient had any seizures? Does the patient have a history of other malignancies? Does the patient need surgical intervention? If so, what is the timing of surgery? Does the patient have medical comorbidities which may make surgery risky (e.g. pacemaker, heart failure, severe chronic obstructive pulmonary disease)? Does this patient need to be admitted?

History of Present Illness A 70 year old male with history of Parkinson’s disease, hypertension (HTN), and diabetes mellitus (DM) presents to the emergency department (ED) with 1 week of right upper extremity weakness and headaches. He began noticing these symptoms without any inciting event. His headaches are worse in the morning and initially were responsive to acetaminophen but are now persistent. His weakness has not worsened over the past week, but has not improved, and so he came to the ED. His wife notes that the patient seems to be bumping into objects when walking. The patient denies any numbness, vision changes, seizure-like activity and does not have a history of other malignancies. The patient does not take any antiplatelet or anticoagulant medication. In the ED, a head CT demonstrated a left fronto-parieto-occipital hypodensity (Figure 14.1). Subsequent brain MRI with and without contrast (Figure 14.2) revealed a heterogeneously enhancing mass with surrounding edema and central necrosis.

Vital Signs T 37°C, HR 64, BP 139/80, SpO2 95% on room air

Pertinent Labs Na 136, Cr 1.1, INR 1.0, aPTT 25.9

Physical Exam Alert and oriented to self, place, and year Following commands briskly in all extremities Names 2 of 3 objects with good repetition Pupils equal, round, and reactive to light Extraocular movements intact Face symmetric, tongue midline Right hemianopsia to confrontation Right pronator drift Right upper extremity 4+/5 strength Right lower extremity 5/5 strength Left upper and lower extremity 5/5 strength Sensation intact to light touch throughout

Triage Management The patient presents with a newly diagnosed left fronto-parieto-occipital enhancing intra-axial mass in the setting of new right upper extremity weakness. The appearance of the mass on MRI is concerning for a high grade glioma versus a metastasis. The patient has no history of other malignancies; however, he will need a CT with contrast of his chest, abdomen, and pelvis to rule out a primary lesion. The patient demonstrates neurological deficits associated with the mass, including a right visual field cut and right upper extremity weakness. He needs surgery for diagnosis and decompression of the mass. Given that the patient is mildly symptomatic, he will be admitted

65

14—HeAdAcHe And RigHt ARm WeAkness

A

B

C

D

P

Fig. 14.2 An axial T1-weighted brain MRI with contrast (A) demonstrates an intrinsic left fronto-parieto-occipital heterogeneously enhancing mass with central areas of necrosis. An axial T2-weighted FLAIR image (B) shows surrounding T2 hyperintensity representing associated edema. There is restricted diffusion (C) and increased cerebral blood volume (D) associated with the nodular enhancement (*) along the posterior margin of the lesion (A).

to the neurosurgical floor and given corticosteroid and anti-seizure medications. Because he is not on antiplatelet or anticoagulant medications, surgical planning can be expedited during this admission. This is a nonemergent but operative consult.

Assessment This is a 70 year old male with history of Parkinson’s disease, HTN, and DM who presents with one week of right upper extremity weakness and a right visual field cut. He is found to have a left fronto-parieto-occipital heterogeneously enhancing mass concerning for a high grade glioma versus a metastasis.

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Plan n n n n n n

n

Admission to the neurosurgical floor Dexamethasone 4 mg every 6 hours Levetiracetam 1 g every 12 hours Proton pump inhibitor Brain MRI with navigation protocol in preparation for operative intervention Evaluation by medicine for preoperative surgical risk assessment given his age and comorbidities Surgical planning for a left craniotomy for tumor resection n Posting highlights: n Skull clamp n Intraoperative neuromonitoring n High speed drill and craniotome n Neuronavigation n Consider intraoperative ultrasound n At his age, the dura mater may be thin and frail; consider dural substitutes n When planning the incision and craniotomy, consider the possible need for repeat surgery for tumor recurrence n For anesthesia: antibiotics, dexamethasone, levetiracetam, mannitol, hyperventilation

Follow-Up The patient underwent a CT of the chest, abdomen, and pelvis that was negative for other lesions, which favored the diagnosis of a glioma. He underwent a successful left parietal craniotomy for tumor resection. Postoperatively, the patient did well and was discharged to rehabilitation after two days. He slowly recovered his strength over the next 2 weeks. Pathology confirmed IDH wildtype glioblastoma without MGMT-promoter methylation. The patient continued on anti-seizure medication and a slow corticosteroid taper over 2 weeks. He was seen by radiation and medical oncology for adjuvant treatment with the Stupp protocol. LEARNING POINTS

• In a patient with a newly diagnosed intra-axial brain mass, the differential diagnosis includes primary brain tumor, metastasis, brain abscess, lymphoma, and inflammatory lesion. If the patient is neurologically intact or has mild symptoms that improve with steroid treatment, surgery can be deferred to outpatient planning with short-term follow-up. • Malignant astrocytomas encompass the higher grades of astrocytomas (IDH mutant), glioblastomas (IDH wildtype), and gliosarcoma, among others. Glioblastoma is the most common primary malignancy of central nervous system in adults. • MRI features of high grade glioma include a heterogeneously enhancing mass with an area of hypointensity consistent with central necrosis. T2 FLAIR imaging usually demonstrates significant surrounding edema. Diffusion and perfusion imaging, as well as MR spectroscopy can be used as adjuncts in differentiating tumor versus infectious or inflammatory lesions. • The use of perioperative anti-seizure prophylaxis and steroids are controversial. There are currently no consistent guidelines, and the use of these medications depends on the patient’s symptoms, amount of edema, and risk of seizures.4,11 In a patient who is at low surgical risk, surgical resection is recommended for diagnosis • and decompression. Retrospective studies have demonstrated survival benefit with increased extent of resection in high grade gliomas, as long as there are no new long-term postoperative deficits.1-3,7

14—HeAdAcHe And RigHt ARm WeAkness

67

• Randomized, prospective, controlled, multi-institutional trials have led to only four treatments that are FDA approved for glioblastoma: the Stupp protocol, carmustine-loaded biodegradable polymers, bevacizumab, and electric fields generated by the NovoTTF-100A device (OPTUNETM, Novocure, Portsmouth, NH). The current standard treatment is safe gross total resection followed by the Stupp protocol—adjuvant chemoradiation with temozolomide followed by temozolomide alone.8 This can be complemented with tumor electric fields.9 • Even with optimal treatment, the median length of survival for glioblastoma is less than 2 years. • Tumor recurrence must be differentiated from radiation necrosis or pseudoprogression. Pseudoprogression primarily refers to MRI changes that mimic tumor progression with or without associated clinical symptoms. Pseudoprogression may be related to response to radiation, chemotherapy, or experimental immunotherapy. Specific diagnostic modalities for these entities are limited, and surgery is often necessary to differentiate them and guide further treatment strategies.10 • Favorable prognostic factors for high grade glioma are young age, good preoperative Karnofsky performance status score, IDH (isocitrate dehydrogenase) 1 or IDH2 mutation (as opposed to wild type), and MGMT (O6-methylguanine-DNA methyltransferase) promoter methylation.1,5 • Glioblastomas were traditionally categorized as WHO grade 4 gliomas. The 2021 WHO classification of CNS tumors was adapted to include important molecular parameters in addition to histological characteristics. Glioblastomas are now defined as diffuse gliomas that are IDH wildtype. Contrarily, diffuse gliomas that carry an IDH mutation are categorized as astrocytomas, IDH mutant. Astrocytomas, IDH mutant can be WHO grade 2, 3, or 4. Glioblastomas, IDH wildtype are considered WHO grade 4. It is important to note that the 2021 WHO brain tumor classification incorporated critical molecular markers that confer a worse prognosis to diffuse gliomas. Therefore, glioblastomas, besides being IDH wildtype, can also carry a characteristic molecular signature such as TERT promoter mutation, EGFR gene amplification, or +7/−10 chromosome copy number changes.5 • The advent of molecular markers allows for further categorization of brain tumors, with specific definitions of midline glioma and oligodendroglioma.5 • Midline glioma is defined as a diffuse glioma that appears in midline structures and carries the H3K27M mutation. This mutation is associated with a worse prognosis.5 • Oligodendrogliomas are diffusely infiltrative gliomas defined by the presence of 1p19q codeletion and IDH mutation. Depending on their histological features, they are classified as WHO grade 2 and 3 oligodendrogliomas.5 • Oligodendrogliomas have a better prognosis than astrocytomas and are much more responsive to chemotherapy. Optimal management of oligodendroglial tumors is still under evaluation in ongoing clinical trials. • WHO grade 2 oligodendrogliomas are commonly treated with surgery alone or surgery followed by adjuvant therapies. • WHO grade 3 oligodendrogliomas are commonly managed with surgery followed by adjuvant chemotherapy with PCV (procarbazine/CCNU [lomustine]/vincristine) and radiation. The use and timing of radiation is still being studied. Temozolomide is being studied and used as an alternative to PCV given its considerably better tolerance.6

References 1. Brown TJ, Brennan MC, Li M, et al. Association of the extent of resection with survival in glioblastoma: a systematic review and meta-analysis. JAMA Oncol. 2016;2(11):1460–1469. https://doi.org/10.1001/ jamaoncol.2016.1373. 2. Chaichana KL, Jusue-Torres I, Navarro-Ramirez R, et al. Establishing percent resection and residual volume thresholds affecting survival and recurrence for patients with newly diagnosed intracranial glioblastoma. Neuro Oncol. 2014;16(1):113–122. https://doi.org/10.1093/neuonc/not137.

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Grabowski MM, Recinos PF, Nowacki AS, et al. Residual tumor volume versus extent of resection: predictors of survival after surgery for glioblastoma. J Neurosurg. 2014;121(5):1115–1123. https://doi.org /10.3171/2014.7.JNS132449. 4. Jessurun CAC, Hulsbergen AFC, Cho LD, Aglio LS, Nandoe Tewarie RDS, Broekman MLD. Evidence-based dexamethasone dosing in malignant brain tumors: what do we really know? J Neurooncol. 2019;144(2):249–264. https://doi.org/10.1007/s11060-019-03238-4s. 5. Louis DN, Perry A, Wesseling P, et al. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 2021;23(8):1231–1251. doi:10.1093/neuonc/noab106. 6. Penas-Prado M, Wu J, Cahill DP, et al. Proceedings of the Comprehensive Oncology Network Evaluating Rare CNS Tumors (NCI-CONNECT) oligodendroglioma workshop. Neurooncology Advance. 2019;2(1):dz048. Published 2019 Dec 6. https://doi.org/10.1093/noajnl/vdz048. 7. Sanai N, Polley MY, McDermott MW, Parsa AT, Berger MS. An extent of resection threshold for newly diagnosed glioblastomas. J Neurosurg. 2011;115(1):3–8. https://doi.org/10.3171/2011.2.jns10998. 8. Stupp R, Mason WP, van den Bent MJ, et al. Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma. N Engl J Med. 2005;352(10):987–996. https://doi.org/10.1056/ NEJMoa043330. 9. Stupp R, Taillibert S, Kanner AA, et al. Maintenance therapy with tumor-treating fields plus temozolomide vs temozolomide alone for glioblastoma: a randomized clinical trial. JAMA. 2015;314(23):2535– 2543. doi:10.1001/jama.2015.16669. 10. Tsakiris C, Siempis T, Alexiou GA, et al. Differentiation between true tumor progression of glioblastoma and pseudoprogression using diffusion-weighted imaging and perfusion-weighted imaging: systematic review and meta-analysis. World Neurosurgery. 2020;144:e100–e109. https://doi.org/10.1016/ j. wneu.2020.07.218. 11. Wu AS, Trinh VT, Suki D, et al. A prospective randomized trial of perioperative seizure prophylaxis in patients with intraparenchymal brain tumors. J Neurosurg. 2013;118(4):873–883. https://doi. org/ 10.3171/2012.12.JNS111970.

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New Onset Seizure With Prior History Of Melanoma Alice L. Hung, MD n Jordina Rincon-Torroella, MD

Consult Page 64M with history of melanoma presents with a seizure. Head CT concerning for mass/swelling

Initial Imaging

A

B

Figure 15.1 Axial head CT without contrast on initial consult in the ED demonstrates (A) a large 3.5 by 3 cm left parietal mixed-density lesion with (B) surrounding hypodensity consistent with edema.

Walking Thoughts What is the patient’s current neurological exam and mental status? What is the semiology of his seizure? Is he still seizing? n What is his melanoma history? Does he have any prior metastasis? n What is his baseline level of independence and function? What is his life expectancy? n Does he have any other medical comorbidities? n Does the patient take any anticoagulant or antiplatelet medications? n n

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History of Present Illness A 64 year old male with a prior history of metastatic melanoma currently on immunotherapy presents to the emergency department (ED) after a generalized tonic-clonic seizure. Earlier in the day, the patient noted new right-hand clumsiness while cooking. His wife states that this then evolved into a full body shaking episode with unresponsiveness lasting a few minutes. He did not have any urinary or bowel incontinence, or tongue biting. He currently denies any headaches, nausea, vomiting, or speech deficits. His wife states that he is now at his neurological baseline. He was initially diagnosed with melanoma 3 years prior after a suspicious lesion was found in his groin. The lesion was resected at that time, and the patient declined adjuvant therapy. One year prior to his current presentation, he was found to have multiple metastases to the lungs. He underwent a right middle lobectomy of the lung and after his thoracotomy, he was started on nivolumab for BRAF-negative melanoma. He has not received radiation or had new metastatic lesions on his last oncological follow-up. Head CT without contrast in the ED demonstrates a new left parietal mixed density lesion with surrounding edema, suggestive of a mass.

Vital Signs T 37.1°C, HR 52, BP 145/75, SpO2 97% on room air

Pertinent Labs Na 137, Glu 105, Plt 532, INR 0.9, PT 11.0, aPTT 26.2

Physical Exam Alert and oriented to self, place, and year Following commands briskly in all extremities Pupils equal, round, and reactive to light Extraocular movements intact, tongue midline, face symmetric Visual fields full to confrontation No pronator drift Bilateral upper extremities 5/5 strength Bilateral lower extremities 5/5 strength Sensation intact to light touch throughout

Triage Management The patient has a history of metastatic melanoma and presents with a generalized seizure. A head CT on arrival to the ED shows a left parietal mass. His presentation and imaging findings are concerning for a metastatic brain tumor. He requires admission for stabilization and surgical planning. Clinically, he is fully intact with no neurological deficits, and is appropriate for floor level of care. A higher level of care, such as the intensive care unit, may be considered for patients presenting with persistent altered mental status. An electroencephalogram (EEG) can be considered to assess for non-convulsive status epilepticus if the patient presents with persistent mental status changes. After the patient is stabilized clinically, a brain MRI with and without contrast will be necessary to further characterize the lesion. Furthermore, the patient will need a CT scan of the chest, abdomen, and pelvis with contrast for staging purposes. Based on the head CT, this patient has associated vasogenic edema and will benefit from corticosteroids. He is also started on antiepileptic

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medications given his seizure presentation. This patient is otherwise healthy with no other significant comorbidities that would require preoperative optimization. He will need surgery for resection and diagnosis of the mass. This is a nonemergent, but operative consult.

Follow-Up Imaging

A

B

Figure 15.2 A. Axial T1-weighted brain MRI with contrast shows a left parietal heterogeneously contrastenhancing mass. B. Axial T2-weighted FLAIR sequence shows marked surrounding vasogenic edema.

Assessment This is a 64 year old male with a history of metastatic melanoma who presents with a generalized tonic-clonic seizure and is found to have a new lesion on head CT and MRI concerning for a brain metastasis.

Plan Admit to the neurosurgical floor Corticosteroids (dexamethasone 4 mg every 6 hours) n Proton pump inhibitor n Anti-seizure medication (levetiracetam 1 g twice a day) n Brain MRI with and without contrast, with protocol for navigation planning n CT of the chest, abdomen, and pelvis with contrast for oncological staging n Consult to oncology for management of primary pathology and adjuvant therapy n Surgical planning for left craniotomy for tumor resection n Posting highlights: n Skull clamp n Neuronavigation n Can consider intraoperative neuromonitoring or awake craniotomy n n

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Figure 15.3 Post-surgical axial T1-weighted brain MRI with contrast shows interval resection of the previously seen mass without residual tumor. The pathology was consistent with metastatic melanoma.

Frozen and permanent pathology specimens Hemostatic agents n For anesthesia: corticosteroids, antiepileptic medications, antibiotics, mannitol, pCO2 to 30 mmHg After surgery, the patient will be admitted to the neurocritical care unit with postoperative corticosteroids, anti-seizure medications, early mobilization, and MRI within 48 hours (Figure 15.3).

n n

n

LEARNING POINTS

• Brain metastases are the most common type of brain tumor. • Fifteen percent of patients with cancer will have a brain metastasis as a presenting symptom.4

• The most common sources of cerebral metastases by frequency are the following: lung cancer, breast cancer, renal cell cancer, gastrointestinal cancer, and melanoma.

• Historically, patients with brain metastases have a poor outcome with a median survival of 3 to 6 months after whole brain radiation therapy (WBRT).3 However, the discovery of new chemotherapies and the advent of immunotherapy has increased the number of long-term survivors. Several factors are considered in the management of metastatic brain tumors, including the • number and size of lesions, their location, and associated neurological symptoms from the lesion. Recommendations from the 2019 Congress of Neurological Surgeons Guidelines are as follows:1,2,5

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• For solitary supratentorial lesions in a non-eloquent location, surgical resection followed by radiation therapy is the current Level 1 recommendation. The radiation oncology team should therefore be consulted for patients found to have metastatic brain lesions. • For a single brain metastasis, en bloc tumor resection, as opposed to piecemeal resection, is recommended to decrease the risk of postoperative leptomeningeal disease. • Stereotactic radiosurgery (SRS) is used after surgical resection of a solitary brain metastasis to decrease local recurrence rates. • The use of SRS instead of WBRT is considered for patients with < 4 lesions and an overall volume less than 7 cc. Compared to WBRT, SRS is associated with fewer neurocognitive side effects. • In patients with multiple lesions, WBRT may be used if the total volume is > 7 cc, there are > 15 metastases, or the size or location of the metastases are not amenable to surgical resection or radiosurgery. Surgery may also be considered to reduce mass effect for symptomatic lesions. • Radiosurgery alone may be considered in patients who are poor surgical candidates or those with surgically inaccessible lesions measuring < 3 cm in maximum diameter.3 • Routine chemotherapy is not currently recommended for the treatment of all brain metastases. However, depending on the pathology and presence of other metastatic lesions in the body, this should be considered on a case by case basis. Likewise, as new targeted molecular and immunotherapy agents become available, their role in the overall management of brain metastases will also need to be addressed. Neuro-oncology should be consulted for assistance with further management. • Corticosteroids can be used for symptomatic control in patients who have masses with associated vasogenic edema (Level 3 recommendation). Dexamethasone is the treatment of choice. • Antiepileptics are not routinely recommended for seizure prophylaxis in patients with supratentorial metastases but without seizures (Level 3 recommendation).

References 1. Ammirati M, Nahed BV, Andrews D, Chen CC, Olson JJ. Congress of neurological surgeons systematic review and evidence-based guidelines on treatment options for adults with multiple metastatic brain tumors. Neurosurgery. 2019;84(3):E180–E182. 2. Gaspar LE, Prabhu RS, Hdeib A, et al. Congress of neurological surgeons systematic review and evidencebased guidelines on the role of whole brain radiation therapy in adults with newly diagnosed metastatic brain tumors. Neurosurgery. 2019;84(3):E159–E162. 3. Kalkanis SN, Kondziolka D, Gaspar LE, et al. The role of surgical resection in the management of newly diagnosed brain metastases: a systematic review and evidence-based clinical practice guideline. J Neurooncol. 2010;96(1):33–43. 4. Mintz AP, Cairncross JG. Treatment of a single brain metastasis: the role of radiation following surgical resection. JAMA. 1998;280(17):1527–1529. 5. Nahed BV, Alvarez-Breckenridge C, Brastianos PK, et al. Congress of neurological surgeons systematic review and evidence-based guidelines on the role of surgery in the management of adults with metastatic brain tumors. Neurosurgery. 2019;84(3):E152–E155.

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Altered Mental Status With Brain Lesions Dimitrios Mathios, MD n

Jordina Rincon-Torroella, MD

Consult Page 53F, history of IVDU, found unresponsive, HCT with multiple brain lesions, large vents.

Initial Imaging

A

B

Figure 16.1 An axial head CT without contrast shows a hemorrhagic 3 cm right parieto-occipital lesion with enlarged lateral ventricles (A) and a left 3.5 cm cerebellar hypodensity with surrounding edema and mild compression of the fourth ventricle (B).

Walking Thoughts n

n

n

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What is the patient’s current Glasgow Coma Scale (GCS) score? Is the patient protecting her airway? What is the cause of her unresponsiveness (e.g. a drug effect, a seizure, or acute hydrocephalus with brainstem compression)? What is the etiology of her brain lesions and what workup will she need?

s n

n

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Does she need an emergent external ventricular drain (EVD) placed? Does she need to go to the operating room emergently? Is the patient on any anticoagulant or antiplatelet therapy?

History of Present Illness A 53 year old female with a history of hypertension, intravenous drug use (IVDU), smoking, alcohol abuse with prior hospitalizations for withdrawal, and bipolar disorder presents with altered mental status. Earlier that day, she was witnessed to be walking oddly and then collapsed to the floor with full body shaking and left gaze deviation. A bystander called emergency medical services, who transported the patient to the emergency department (ED). There are no family or friends to give additional history.

Vital Signs T 36.6°C, HR 120, BP 176/100, RR 29, SpO2 93% on 100% O2

Pertinent Labs Na 138, Glu 192, Cr 1.4, Lactic acid 14.4 (normal 0.5-2.2) Hgb 10.2, WBC 18.3, Plt 203, INR 1.04, PT 1.0, aPTT 26.8 Urine toxicology screen positive for cocaine and morphine Ethanol not detected

Glasgow Coma Scale Motor: 5 Verbal: 1 Eye opening: 1 GCS Total: 7T

Physical Examination Intubated, sedation and paralytics held Hard cervical collar in place Eyes closed, not following commands Pupils equal, round, and reactive to light Corneal reflexes present bilaterally Cough and gag reflexes present Bilateral upper extremities localizing to pain Bilateral lower extremities withdrawing to pain

Triage Management The patient has a severely depressed mental status with possible etiologies including narcotic use, seizures, and/or hydrocephalus. She received emergent cardiorespiratory support with intubation in the ED. On neurological examination, she is localizing, and her brainstem reflexes are intact. An emergent head CT is concerning for a hemorrhagic right parieto-occipital and a posterior fossa mass with enlarged ventricles. Although she may need an EVD, currently her fourth ventricle

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is still patent, and she has other explanations for her altered mental status that can be addressed quickly. The most readily reversible cause is her drug use, and she receives naloxone. Her initial presentation of whole body shaking and left gaze deviation is concerning for a possible seizure due to the hemorrhagic supratentorial lesion, and she receives a lorazepam and levetiracetam load. She also receives corticosteroids (high dose dexamethasone) to treat the edema associated with her brain lesions. If these interventions do not improve her clinical exam, then it is important to consider hydrocephalus as the primary etiology, and she may need an EVD. The patient requires close monitoring with admission to the neurocritical care unit. This an emergent and possibly operative consult.

Assessment This is a 53 year old female smoker with IVDU who presents with depressed mental status and imaging concerning for right parieto-occipital and posterior fossa lesions and mild hydrocephalus. She is GCS7T on arrival, and her history is concerning for drug use and seizures. The multiple lesions involving both the supratentorial and infratentorial compartments suggest metastasis as the leading differential.

Follow-Up One hour after receiving naloxone, high-dose steroids, and antiepileptic medications, the patient’s exam improves such that she opens her eyes to voice and follows commands in all extremities. Her initial presentation was thus most likely due to her drug use and possible seizure rather than hydrocephalus.

Plan n n n n n n n n n n

Admission to the neurocritical care unit with hourly neurological exams Judicious naloxone administration for continued reversal of opioids Monitor for drug withdrawal Continue levetiracetam with electroencephalogram if mental status worsens Dexamethasone 4 mg every 6 hours for lesion-associated edema Proton pump inhibitor Minimize sedation Wean mechanical ventilation Repeat head CT at 6 hour interval to evaluate for hemorrhage and ventricular stability Once clinically stable, additional imaging to initiate oncological workup: n Brain MRI with and without contrast (with neuronavigation protocol) n CT of the chest, abdomen, and pelvis (CT CAP) with contrast

Hospital Course After the patient’s neurological exam improved, she underwent an MRI of the brain with and without contrast (Figure 16.2) and a CT CAP with contrast for workup of her brain lesions. Her brain MRI showed a 3 cm hemorrhagic right parieto-occipital mass and a 3.5 cm cystic and contrast-enhancing left cerebellar lesion. There is moderate vasogenic edema resulting in mild compression of the fourth ventricle. A CT CAP showed a left upper lobe heterogeneously enhancing mass, measuring 2.5 x 3.7 cm and extending towards the left hilum. There was also a left adrenal nodule, suspicious for a metastasis.

s

A

B

C

D

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Figure 16.2 Preoperative axial T1-weighted brain MRI with contrast shows a 3 cm hemorrhagic right parietooccipital tumor (A) and a 3.5 cm heterogeneously enhancing cystic lesion in the left cerebellar hemisphere (B). Preoperative T2-weighted brain MRI shows moderate edema at the right parieto-occipital lesion (C) and moderate vasogenic edema surrounding the cerebellar lesion resulting in compression of the fourth ventricle (D). Note that the hemorrhage is hypointense and the cystic fluid is hyperintense on the T2-weighted brain MRI.

Her exam continued to improve over time, and she was successfully extubated. Oncology was consulted and the decision was made to treat her brain lesions first given the need to control her symptoms, relieve brainstem compression, and address the hydrocephalus. Interventional pulmonology was consulted for a potential lung biopsy, which was deferred given that tissue for diagnosis would be obtained during brain surgery. The patient underwent a right parieto-occipital craniotomy and a suboccipital craniectomy for tumor resection. Pathology showed lung adenocarcinoma. The patient did well from her surgery. She was discharged from the hospital with plans for adjuvant radiation at the resection sites and follow-up with oncology for her lung primary.

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LEARNING POINTS

• Cerebral metastases are the most common brain tumor. About 8.5% of patients with lung, breast, renal, colorectal cancer, or melanoma will have brain metastases.4

• The incidence of brain metastases has been increasing over the years given better diagnostic tools and screening, as well as a result of improved survival from the primary tumors. Therefore, the management of brain metastases has also become more aggressive with surgery and stereotactic radiosurgery as first lines of treatment to improve disease control and progression-free survival.1-3 • Twenty percent of brain metastases occur in the posterior fossa, which may be associated with worse prognosis.6 Following the European Association of Neuro-Oncology (EANO) guidelines,5 surgical resection is considered in patients with: • 1 to 3 newly diagnosed metastatic lesions (especially for symptomatic lesions in eloquent areas but that are surgically resectable) • symptomatic or asymptomatic lesions of ≥ 3 cm in diameter • lesions with significant edema and/or mass effect • posterior fossa lesions with associated hydrocephalus, such as in this case • In a patient with hydrocephalus from a posterior fossa tumor, surgical resection is usually the definitive treatment for the hydrocephalus. Steroids (in mild cases) or an EVD (in more severe cases) can also be used to temporize symptoms during surgical planning. • Altered mental status can have many contributing factors, including seizures, metabolic abnormalities, drug consumption, and hydrocephalus. Pharmacological or metabolic causes that can be rapidly reversed should be addressed first, when possible. • In patients with a metastatic brain lesion that will require surgery for disease control or symptomatic relief, biopsy of the primary site can be deferred based on recommendations from the oncology team.

References 1. Aoyama H, Tago M, Shirato H, & Japanese Radiation Oncology Study Group 99-1 (JROSG 99-1) Investigators. Stereotactic radiosurgery with or without whole-brain radiotherapy for brain metastases: secondary analysis of the JROSG 99-1 randomized clinical trial. JAMA Oncol. 2015;1(4):457–464. 2. B Brennan C, Yang TJ, Hilden P, et al. A phase 2 trial of stereotactic radiosurgery boost after surgical resection for brain metastases. Int J Radiat Oncol Biol Phys. 2014;88(1):130–136. https://doi.org/10.1016/ j. ijrobp.2013.09.051. 3. Churilla TM, Chowdhury IH, Handorf E, et al. Comparison of local control of brain metastases with stereotactic radiosurgery vs surgical resection: a secondary analysis of a randomized clinical trial. JAMA Oncol. 2019;5(2):243–247. 4. Gavrilovic IT, Posner JB. Brain metastases: epidemiology and pathophysiology. J Neuro Oncol. 2005;75(1):5–14. 5. Soffietti R, Abacioglu U, Baumert B, et al. Diagnosis and treatment of brain metastases from solid tumors: guidelines from the European Association of Neuro-Oncology (EANO). Neuro Oncol. 2017;19(2):162–174. 6. Sunderland GJ, Jenkinson MD, Zakaria R. Surgical management of posterior fossa metastases. Journal Neurooncology. 2016;130(3):535–542.

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Incidental Sellar Mass After An Accident Jordina Rincon-Torroella, MD

Consult Page 23F Headache after MVC. MRI with sellar mass

Initial Imaging

A

B

C Figure 17.1 Brain MRI with and without contrast. A. Coronal T1-weighted sequence without contrast shows an isodense sellar mass. B. Coronal T1-weighted sequence with contrast shows a 2 by 2 cm homogeneously enhancing pituitary mass abutting the optic apparatus (arrow). C. Sagittal T1-weighted sequence with contrast shows the relationship of the suprasellar portion of the mass with the anterior communicating artery complex (arrow).

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Walking Thoughts n n n n n n n n n n

n

What are the patient’s vitals? What is her neurological exam? Does she have any other injuries from the motor vehicle accident? Is the sellar mass a known diagnosis or is it new? Does she have any vision loss? Are her current symptoms related to the sellar mass or is the sellar mass an incidental finding? What other symptoms does she have? Are they related to endocrine abnormalities? Is the mass causing any compression symptoms (e.g. vision loss)? Does the patient require any immediate hormone replacement? Does she need emergent surgery, or does she need a more exhaustive work-up performed as an outpatient? Is the patient on antiplatelet or anticoagulant medication?

History of Present Illness A 23 year old college student with no major medical history is brought to the emergency department (ED) by emergency medical services after a motor vehicle accident (MVA). She was a belted passenger in the front seat of a car when the car slid on ice at 40 miles per hour hitting the curb. There was no airbag deployment, but she hit her head against the window. The patient reports no loss of consciousness and was able to leave the car without assistance. A full trauma survey in the ED is negative for other injuries and cervical spine imaging is unremarkable. However, her head CT shows a sellar mass. The patient has headaches and mild nausea but denies any vision loss, tingling, numbness or weakness. Despite receiving analgesic medication, she continues to have persistent headaches, thus the ED team obtained a brain MRI which shows a 2 by 2 cm contrast-enhancing sellar mass. On further questioning, the patient reports a history of amenorrhea for 6 months and weekly headaches for the past 10 years. Her headaches are frontal and usually worsen with bright lights. These are accompanied with nausea and resolve with acetaminophen. She has a history of acne and was placed on spironolactone 6 months ago. After the spironolactone was started, her periods disappeared. The medication was stopped after 2 months, but her periods did not return until 2 weeks ago. She attributed it to the drug and has not had any further work-up. The patient denies any vision changes, fatigue, cold or heat intolerance, weight change, polyuria, polydipsia, galactorrhea, or change in size of hands and feet. She is not on anticoagulant or antiplatelet medications.

Vital Signs T 36.8oC, HR 87, BP 103/64, SpO2 99% on room air

Pertinent Labs Na 137, Glu 99, Hgb 10.5, WBC 5.4, Plt 168, INR 1.0, aPTT 25.7 Beta-hCG negative No ethanol in blood Negative urine toxicology

Physical Exam Alert and oriented to self, place, and year Following commands briskly in all extremities Pupils equal, round, and reactive to light Extraocular movements intact, face symmetric, tongue midline

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Visual fields full to confrontation No pronator drift Bilateral upper extremities 5/5 strength Bilateral lower extremities 5/5 strength Sensation intact to light touch throughout Normal facies and body habitus, normal sized hands and feet

Triage Management This patient presents with mild headache and nausea after suffering a head collision, likely related to a concussion. An initial head CT did not show any skull fractures or intracranial hemorrhage, but there was a sellar mass, which prompted the ED team to obtain a full brain MRI. The findings in the MRI are consistent with an incidental pituitary macroadenoma. There is no hemorrhage within the mass, the patient does not complain of vision changes, and her visual fields are grossly full to confrontation. Since there is no concern for pituitary apoplexy, this pituitary mass can be worked-up as an outpatient with formal ophthalmology and endocrine evaluations. The patient has no clinical signs or symptoms of a secreting pituitary adenoma (e.g. weight change, galactorrhea, change in hands and feet size); however, many patients with pituitary macroadenomas have associated panhypopituitarism requiring hormone replacement. Her pregnancy test was negative, and she has a history of amenorrhea which could be related to the spironolactone treatment, prolactin secretion, or impaired sexual hormone secretion. The endocrine team is consulted, and bloodwork is obtained to expedite the outpatient work-up. She has a normal TSH, FSH, LH, random cortisol, IGF, and a mildly elevated prolactin at 41.3 μg/liter. This is a nonurgent consult that may require elective surgery.

Assessment This is a 23 year old female with a history of acne on spironolactone and intermittent amenorrhea who presents to the ED after an MVA. She has headaches and nausea after a head collision and an incidental pituitary adenoma found on MRI.

Plan n n

n n n

n

Outpatient follow-up with neurosurgery and ENT teams for potential surgical planning Outpatient dedicated pituitary MRI and CT maxillofacial with CT angiogram of the head and neck (Figure 17.2) Outpatient follow-up with ophthalmology for formal visual fields Discharge home from the ED pending endocrine evaluation Return precautions in the event of new red flag signs such as sudden headache, vision loss, fatigue, altered mental status or hypotension Counseling on concussion symptoms and recovery

Follow-Up After their evaluation in the ED, the endocrine team felt that the patient’s slightly elevated prolactin is likely due to stalk effect and her mass is a non-functioning pituitary adenoma without panhypopituitarism. She was discharged with outpatient follow-up. The patient received formal outpatient visual field testing, which demonstrated a mild left superotemporal visual field deficit. Risks and benefits of surgical resection were discussed with the patient. Given her young age, the elevation and compression of the optic apparatus on imaging, and her ophthalmological defect, surgery was recommended. The patient underwent a successful endoscopic endonasal approach for pituitary macroadenoma resection with ENT and neurosurgery (Figure 17.3).

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* Figure 17.2 Maxillofacial CT of the head with CT angiogram of the head and neck is useful to evaluate the bony structures, pneumatization of the sella (asterisk) and the relationship of the internal carotid arteries to the tumor (arrows). This can also be very useful for intraoperative navigation.

A

B

Figure 17.3 Postoperative coronal (A) and sagittal (B) brain MRI with contrast shows excellent tumor resection with decompression of the optic apparatus.

LEARNING POINTS

• According to the Central Brain Tumor Registry of the United States (CBTRUS) between 2013 and 2017, 16.9% of all central nervous system (CNS) neoplasms were tumors of the pituitary. These are more prevalent in females and young patients. For example, 28% of CNS tumors in young adults aged 15 to 39 years are pituitary tumors.11 Pituitary tumors can include non-functioning pituitary adenomas (NFPAs), hormone• secreting or functioning pituitary adenomas, pituicytomas, and pituitary carcinomas (pituitary adenomas that metastasize). A wide variety of sellar and suprasellar lesions are in the differential (e.g. craniopharyngiomas, meningiomas, and Rathke’s cleft cysts).

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• Pituitary adenomas that are less than 1 cm are classified as microadenomas, while those that are 1 cm or more are macroadenomas. This is a distinction of size and not of hormone-secretion. • The clinical and surgical complexity of pituitary adenomas require a multidisciplinary effort with the ENT, neurosurgery, endocrine, and ophthalmology teams working together for diagnosis and management. • Routine endocrine evaluation is essential in the diagnosis and follow-up of pituitary tumors. • Elevated prolactin levels in patients with pituitary adenomas may be due to stalk effect or caused by a prolactinoma. Stalk effect is hypothesized to be the result of compression of the pituitary stalk with impairment of the dopaminergic inhibition of prolactin secretion. Stalk effect results in moderate prolactin elevation (200-250 μg/liter) often indicates a prolactinoma (Table 17.1).1,2,5,7 • Alterations in the posterior pituitary axis are not common in pituitary adenomas. If diabetes insipidus is present at diagnosis, other pathologies such as a craniopharyngioma or germinoma are higher in the differential. • Pituitary apoplexy is a neurosurgical emergency related to pituitary tumors and is discussed in Chapter 18. Nonfunctioning Pituitary Adenomas1

• NFPAs are the most common type of pituitary lesion. The differential of NFPAs includes hormone-secreting pituitary adenomas and other sellar and suprasellar lesions such as craniopharyngiomas, meningiomas, hypothalamic hamartomas, gliomas, germ cell tumors, metastases, or sinonasal malignancies. • NFPAs are usually macroadenomas and present with local compression signs such as vision loss, hypopituitarism, cranial nerve dysfunction, or headaches. Many NFPAs are also diagnosed incidentally, especially with the current widespread use of brain imaging. • The management of NFPAs includes surgical resection, observation, medical management, and/or radiotherapy. • Surgical decompression with microscopic, endoscopic, or combined approaches is highly recommended in symptomatic NFPAs. • Although controversial, radiation therapy or radiosurgery are growing alternatives in patients who are not surgical candidates or as an adjunct to surgical resection (e.g. in residual or recurrent tumors). • Management of asymptomatic NFPAs is still a matter of debate. Depending on the patient and tumor characteristics, asymptomatic NFPAs can be either followed clinically and with imaging, or undergo upfront treatment. • In endocrine evaluation, NFPAs are often associated with hypopituitarism (which may require hormone replacement) and hyperprolactinemia. • Although hyperprolactinemia in NFPAs is usually from stalk effect, it is imperative to rule out a prolactinoma even in cases that are not clinically suspicious. • When there is discrepancy between the tumor size or prolactinoma symptoms and a mildly elevated prolactin level, a diluted prolactin is recommended. • A diluted prolactin includes serial dilution of serum samples to eliminate the Hook effect. The Hook effect is a laboratory artifact that can occur when saturating the antibodies in some prolactin assays leading to a falsely low prolactin value.7 • Preoperative and follow-up ophthalmological evaluation is recommended. A significant percentage of patients without preoperative optic nerve atrophy from compression can have vision improvement after decompression. However, older patients (>65 years of age), patients with long-standing symptoms (>4 months) or with optic nerve atrophy must be warned of the lower chances of improvement. • Evaluating the relationship of the tumor with surrounding structures, tumor density, presence of cysts or hemorrhage, cavernous sinus invasion, and vascular anatomy is essential in the management and surgical planning of NFPAs.

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• A high-resolution MRI is recommended as the standard preoperative imaging for NFPAs. Different centers have dedicated pituitary protocols which may include constructive interference steady state (CISS) or fast imaging employing steady-state acquisition (FIESTA) sequences. • Dynamic imaging during contrast administration is also helpful in differentiating between the tumor, normal pituitary, and the surrounding structures. • MRI can be complemented with CT, CTA, or maxillofacial CT to evaluate the bony anatomy and position of the vascular structures. • Intraoperative navigation can be a useful adjunct during surgery. • Treatment of NFPAs may improve or worsen pituitary function requiring changes in hormone replacement. Immediate postoperative diabetes insipidus (DI) and syndrome of inappropriate antidiuretic hormone secretion (SIADH) are common and require careful monitoring of urine output and sodium levels. • Long-term endocrinological, ophthalmological, and imaging follow-up are essential in the management of NFPAs. • Recurrence of NFPAs after surgery can be as high as 44 to 75%.1 Observation, repeat resection, and/or radiation therapy/radiosurgery may be considered in the management of recurrent NFPAs. Hormone-secreting or Functioning Pituitary Adenomas2,4,8,9 (Table 17.2)

• Hormone-secreting pituitary adenomas from higher to lower incidence are prolactinomas, growth hormone (GH)–secreting tumors, adrenocorticotropic hormone (ACTH)–secreting tumors, and rarely, thyroid-stimulating hormone (TSH)–secreting tumors. Gonadotropinsecreting adenomas are extremely unusual. • Because of their hormone-secretion they are commonly diagnosed when they are small in size (as microadenomas), however they can also be macroadenomas. • These tumors are histologically benign, but their potent endocrine effects cause severe morbidity. • The main goal of treatment is to achieve biochemical remission and relieve mass effect if present. • Medical treatment is often the first line therapy for prolactinomas. Transsphenoidal surgery for adenoma resection is often chosen as the first line therapy for Cushing syndrome and acromegaly. Medical therapy can also be used as an adjunct or temporary treatment in other secreting pituitary adenomas, but is unlikely to eradicate them or result in permanent cure. • Radiation and radiosurgery are controversial adjunct therapies. Stereotactic radiosurgery results in overall faster reduction in hormone secretion and fewer adverse effects than conventional radiotherapy.

TABLE 17.1 n Prolactin Values in The Assessment of Prolactinoma Versus Stalk Effect2,5,7 200-250 μg/liter likely prolactinoma

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TABLE 17.2 n Major Characteristics of Secreting Pituitary Adenomas3,5,6,8-10 Type (% of secreting adenomas)

Goals of treatment (Biochemical remission)

Classic Symptoms

Laboratory Diagnosis

Prolactinomas (32% to 66%)

Women: amenorrhea, loss of libido, galactorrhea, and infertility Men: loss of libido,erectile dysfunction, and infertility

Elevated prolactin

Growth hormone– secreting tumors (8% to 16%)

Acromegaly: Elevated insulin-like Enlargement of growth factor the hands, 1 (IGF-1) levels feet, and and growth tongue, insulin hormone levels resistance, (less reliable) hypertension, Additional tests: arthritis, oral glucose carpal tunnel tolerance test syndrome, sleep apnea, colon tumors, cardiac disease

First line: Surgical resection Alternative/adjunct: Somatostatin analogues (octreotide and lanreotide), cabergoline, pegvisomant, radiation

Controversial but commonly used: GH nadir less than 1 μg/L (ng/mL) and IGF-1 levels to the normal age-adjusted range

Adrenocorticotropic hormone (ACTH)– secreting tumors (2% to 6%)

Cushing syndrome: Obesity, diabetes hypertension, moon facies, violaceous skin striae, ecchymoses, osteoporosis, mood disorders

First line: Surgical resection Alternative/adjunct: ketoconazole, mifepristone, pasireotide, radiation Rescue treatment: bilateral adrenalectomy (causes lifelong adrenal insufficiency, and there is risk of pituitary tumor enlargement [Nelson syndrome])

Controversial but commonly used: Early postoperative serum cortisol reaching a nadir of less than 2 μg/ dL is likely predictive of curative resection while 2-5 μg/dL is predictive of remission

Screening (at least 2 of 3 tests positive): - elevated late-night salivary cortisol level - 24-hour urinaryfree cortisol - low-dose dexamethasone suppression test To differentiate between pituitary or ectopic ACTH secretion: Highdose dexamethasone suppression and corticotropinreleasinghormone stimulation tests. Petrosal sampling in rare cases to distinguish pituitary versus ectopic source.

Treatment

First line: Normalization Dopamine agonists of serum (Bromocriptine, prolactin Cabergoline) If refractory or intolerant to medical therapy, surgical resection Alternative/adjunct: Radiation Observation if minimal symptoms and slight elevation in prolactin

Continued

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TABLE 17.2 n Major Characteristics of Secreting Pituitary Adenomas3,5,6,8-10—cont’d Type (% of secreting adenomas) Thyroid-stimulating hormone– secreting tumors (1%)

  

Goals of treatment (Biochemical remission)

Classic Symptoms

Laboratory Diagnosis

Treatment

Hyperthyroidism and goiter

Elevated serum free T4 and T3 Elevated or inappropriately “normal” (not suppressed) TSH

First line: Restore normal Antithyroid agents thyroid followed by surgical function resection Alternative/adjunct: somatostatin analogues

1.

Aghi MK, Chen CC, Fleseriu M, et al. Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on the Management of Patients With Nonfunctioning Pituitary Adenomas: Executive Summary. Neurosurgery. 2016;79(4):521–523. 2. Burke WT, Penn DL, Castlen JP, et al. Prolactinomas and nonfunctioning adenomas: preoperative diagnosis of tumor type using serum prolactin and tumor size [published online ahead of print, 2019 Jun 14]. J Neurosurg. 2019:1–8. 3. Hameed N, Yedinak CG, Brzana J, et al. Remission rate after transsphenoidal surgery in patients with pathologically confirmed Cushing’s disease, the role of cortisol, ACTH assessment and immediate reoperation: a large single center experience. Pituitary. 2013;16(4):452–458. 4. Jane JA Jr, Catalino MP, Laws ER Jr. Surgical Treatment of Pituitary Adenomas. In: Feingold KR, Anawalt B, Boyce A, et al., eds. Endotext. South Dartmouth (MA): MDText.com, Inc.; October 4, 2019. 5. Kruse A, Astrup J, Gyldensted C, Cold GE. Hyperprolactinaemia in patients with pituitary adenomas. The pituitary stalk compression syndrome. Br J Neurosurg. 1995;9(4):453–457. 6. Laws ER, Vance ML, Jane JA Jr. TSH adenomas. Pituitary. 2006;9(4):313–315. 7. Melmed S, Casanueva FF, Hoffman AR, et al. Diagnosis and treatment of hyperprolactinemia: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011;96(2):273–288. 8. Mehta GU, Lonser RR. Management of hormone-secreting pituitary adenomas. Neuro Oncol. 2017;19(6):762–773. 9. Molitch ME. Diagnosis and Treatment of Pituitary Adenomas: A Review. JAMA. 2017;317(5):516– 524. 10. Nieman LK, Biller BM, Findling JW, Murad MH, Newell-Price J, Savage MO, Tabarin A, Endocrine Society. Treatment of Cushing’s Syndrome: An Endocrine Society Clinical Practice Guideline. The Journal of clinical endocrinology and metabolism. 2015;100(8):2807–2831. 11. Ostrom QT, Patil N, Cioffi G, Waite K, Kruchko C, Barnholtz-Sloan JS. CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2013-2017. Neuro Oncol. 2020;22(12 Suppl 2):iv1–iv96.

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Sudden Headache And Vision Loss Jordina Rincon-Torroella, MD

Consult Page 45M, Headache, vomiting, sudden vision loss and suprasellar mass

Initial Imaging

Figure 18.1 Axial head CT without contrast shows a heterogeneously hyperdense sellar and suprasellar lesion concerning for a hemorrhagic pituitary lesion.

Walking Thoughts n n n n

What is the patient’s neurological exam? What are the patient’s vitals? Does the patient have a known pituitary adenoma or suprasellar mass? Are his symptoms related to the suprasellar mass or is the suprasellar mass an incidental finding (e.g. rule out stroke, elevated intracranial pressure)? 87

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Is the mass causing hydrocephalus? What is the timeline for his symptoms? Will the patient require any immediate hormone replacement? What is the patient’s sodium level? Is the patient hypotensive? Does this patient need emergent surgery? What are the patient’s medical comorbidities? Is the patient on antiplatelet or anticoagulant medications?

History of Present Illness A 45 year old male with no significant past medical history presents to the emergency department complaining of 3 days of progressively worsening headache with associated emesis and now 12 hours of blurry vision with peripheral vision loss. He reports that he also has been having both heat and cold sensitivity and overall fatigue over the past few months. He did not see a doctor for these symptoms and the suprasellar mass seen on CT here in the ED is a new finding. He denies any focal weakness, numbness, tingling, or speech difficulties. He is not on any anticoagulant or antiplatelet medications.

Vital Signs T 36.9oC, HR 90, BP 90/56, SpO2 99% on room air

Pertinent Labs Na 129, INR 1.0, PT 1.0, aPTT 25.7, Plt 168 Free T4 0.5 ng/dL (normal 0.8-1.8), TSH 0.89 μ[IU]/mL (normal 0.5-4.5), prolactin 26 ng/mL (laboratory reference 3-14.7 ng/mL), cortisol (serum) 1.7 μg/dL (laboratory reference 7-9 AM 4.6-23.4, 4-6 PM 2.7-15.9)

Physical Exam Alert and oriented to self, place, and year with choices Mildly confused Eyes open spontaneously, attends Right pupil 5 mm, left pupil 3 mm; both round and reactive to light Bitemporal hemianopsia to confrontation Mild right ptosis with limited movement of the right eye when crossing midline towards the left Tongue midline, face symmetric No pronator drift Wiggle fingers and toes to command bilaterally Sensation intact to light touch throughout

Triage Management This patient presents with headache, nausea, and vision changes with a suprasellar mass. On examination, the patient has clear bitemporal hemianopsia with a partial right cranial nerve III palsy concerning for pituitary apoplexy. He is confused but maintaining his airway well. Additionally, despite a normal electrocardiogram, his blood pressure is low with hyponatremia concerning for acute hypopituitarism. The patient needs emergent stress dose steroids and a bolus of fluids. A pituitary protocol MRI of the brain with and without contrast is performed as soon as the patient is stabilized and confirms pituitary apoplexy (Figure 18.2).

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C Figure 18.2 A. Axial T1-weighted brain MRI without contrast shows a heterogeneous hyperintensity at the sellar region concerning for pituitary apoplexy. B. Coronal pituitary protocol brain MRI with contrast shows a large hemorrhagic mass with suprasellar extension and compression of the optic nerves and chiasm. C. Postoperative coronal brain MRI with contrast shows good decompression of the optic chiasm (*).

Although basic endocrine labs were sent, the patient needs to be evaluated for pituitary dysfunction. The endocrine team is consulted for hormonal evaluation. They note a low free T4 with an inappropriately normal TSH, consistent with central hypothyroidism. His low random cortisol level and his hypotension suggest acute adrenal insufficiency. He is mildly hyponatremic and will need slow sodium replacement. His mildly elevated prolactin could be due to stalk effect given the size of the sellar/suprasellar mass. This constellation of lab values is suggestive of a non-secreting pituitary macroadenoma. Emergent endoscopic endonasal surgery for decompression of his pituitary apoplexy is recommended. The otolaryngology team is mobilized for planning of emergent surgery. Ophthalmology can be consulted for formal evaluation, but surgical evacuation takes precedence. This is an emergent and operative consult.

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Assessment This is a 45 year old male with no significant medical history who presents to the emergency department with 3 days of severe headache, emesis, blurry vision, and bitemporal hemianopsia. He is also hypotensive with hyponatremia. A dedicated pituitary brain MRI shows a hemorrhagic sellar mass. His presentation is consistent with pituitary apoplexy with adrenal insufficiency. He will need immediate hormonal replacement and emergent endoscopic endonasal surgery for decompression.

Plan n

Consult to endocrinology Start intravenous stress dose hydrocortisone 100 mg once, and then 50 mg every 6 hours n Proton pump inhibitor n Thyroid hormone replacement but one day after starting hydrocortisone to avoid worsening acute adrenal insufficiency n Additional endocrine labs (IGF-1, GH, ACTH, free testosterone) n Sodium and urine specific gravity checked every 6 hours n Close monitoring of input and output Emergent CT angiography with maxillofacial protocol can be obtained to evaluate the anatomy of the internal carotid arteries and the bony structures before surgery Emergent endoscopic endonasal transsphenoidal approach for surgical decompression n Posting highlights: n Skull clamp n Neuronavigation n Endoscopic set: endoscopes, endoscopic skull base instruments n Long high-speed drill n Tissue debrider, side-cutting aspiration device n Reconstruction material per the surgeon’s preference n For anesthesia: SBP 100-160 mmHg, stress dose steroids, antibiotics, can consider mannitol to help prevent cerebrospinal fluid leak during the suprasellar resection of the tumor Post-operative admission to the neurocritical care unit Consult to ophthalmology n

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LEARNING POINTS

• Although uncommon, pituitary apoplexy is a neurosurgical emergency. It affects 2-12% of patients with pituitary adenomas.1-2 It is a clinical diagnosis where the patient classically presents with acute vision loss, headache, and pituitary dysfunction, which can be fatal. Oculoparesis and altered mental status can also be present. • A pituitary adenoma with intratumoral hemorrhage on imaging but lack of these symptoms is not considered pituitary apoplexy. • Pituitary apoplexy can be caused by a hemorrhagic or ischemic pituitary adenoma, or in rare incidences, by an acutely ruptured cyst (e.g. Rathke’s cleft cyst). • Patients with pituitary adenomas that present with pituitary apoplexy and acute vision loss often require emergent neurosurgical management. When the patient has mild symptoms (e.g. headache alone), conservative management can be an option. • Endocrine labs to order include ACTH, cortisol, prolactin, TSH, free T4, IGF-1, LH, FSH, estrogen, progesterone (women), testosterone (men), serum sodium, and urine sodium. Comanagement of these patients with the endocrinology team is crucial. Surgical Management

Emergent endoscopic or microscopic surgical resection is the management of choice in • patients with acute and severe vision loss (24 to 72 hours), acute diplopia, or persistent altered mental status after correction of electrolyte disturbances.

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• Goals of surgery are decompression of the optic apparatus and hematoma evacuation with maximal safe tumor resection. Complete resection of the tumor is not the primary goal and can be staged with a second operation if needed.

• Visual deficits and oculoparesis are likely to improve with surgical management, but longterm hormone replacement will likely be required. Between 74% to 94% of visual field/ acuity deficits improve following surgery. Oculoparesis recovery ranges between 68% and 100%. Hormone recovery following surgery for pituitary apoplexy is uncommon with 12% to 23% of recovery cited in the literature.2,4 Medical Management

• Acute hypopituitarism with Addisonian crisis or hyponatremia may be present. Steroid replacement is essential. The steroid of choice is hydrocortisone since it has both mineralocorticoid and glucocorticoid effects. The stress dose is 100-200 mg once in the setting of an emergency followed by 50 mg IV every 6 hours which can be further tapered. Thyroid replacement is started 24 hours after hydrocortisone initiation. In patients with panhypopituitarism, it is imperative to replace cortisol prior to replacing thyroid hormone to avoid Addisonian crisis.3 • Hyponatremia due to adrenal insufficiency is relatively common in pituitary apoplexy. Acutely correcting hyponatremia is recommended if severe (Na < 125 mEq/L) or if symptoms are present (e.g. nausea, vomiting, altered mental status). To prevent central pontine myelinolysis, hyponatremia is corrected slowly (0.5 mEq per hour or no more than 12 mEq a day). Hormone replacement is the definitive treatment for hyponatremia caused by hypocortisolism. Conservative Management

• Patients with only headaches, very mild vision loss, or a subacute presentation can be treated conservatively with medical management of hormonal replacement and supportive therapy. If the prolactin is > 200 μg/L and suggestive of a prolactinoma, medical management may be particularly indicated as prolactinomas are highly responsive to dopamine agonists. • Tumor ischemia may result in tumor infarction with a decrease in tumor size. However, these patients require close monitoring since there is a small risk of reinfarction.

References 1. Barkhoudarian G, Kelly DF. Pituitary apoplexy. Neurosurgery Clinical North America. 2019;30(4):457– 463. https://doi.org/10.1016/j.nec.2019.06.001. 2. Fernandez A, Karavitaki N, Wass JA. Prevalence of pituitary adenomas: a community-based, crosssectional study in Banbury (Oxfordshire, UK). Clin Endocrinol (Oxf). 2010;72(3):377–382. https://doi. org/10.1111/j.1365-2265.2009.03667. 3. Grzywotz A, Kleist B, Möller LC, et al. Pituitary apoplexy - a single center retrospective study from the neurosurgical perspective and review of the literature. Clin Neurol Neurosurg. 2017;163:39–45. https:// doi. org/10.1016/j.clineuro.2017.10.006. 4. Rutkowski MJ, Kunwar S, Blevins L, Aghi MK. Surgical intervention for pituitary apoplexy: an analysis of functional outcomes. J Neurosurg. 2018;129(2):417–424. https://doi.org/ 10.3171/2017.2.JNS1784.

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Headache, Blurry Vision, And Intraventricular Mass Dimitrios Mathios, MD n

Jordina Rincon-Torroella, MD

Consult Page 20F with headache and dizziness with hydro on OSH CT. MRI pending.

Initial Imaging Pending

Walking Thoughts n

n

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What is the patient’s neurological exam? Does she have decreased mental status that requires an emergent intervention (e.g. external ventricular drain)? What other symptoms is she having? How long has the patient had symptoms? Have the symptoms changed recently? Is the outside hospital imaging or report available? What does it show? How severe is her hydrocephalus? What is the underlying cause of her hydrocephalus? Does the patient need surgery? If so, what is the timing of surgery?

History of Present Illness A healthy 20 year old female presents to the emergency department (ED) with 3 months of subacute headaches and one day of nausea, dizziness, and imbalance. Her headaches are holocranial and intermittent in nature but have been increasing in intensity. On further questioning, she mentions that her vision has been blurry and two weeks ago, she went to the optometrist to get new glasses. Her parents are at bedside and report that her headaches have worsened considerably over the past week. She started experiencing nausea, dizziness, and some imbalance yesterday and decided to go to the ED. She denies vomiting, speech deficits, weakness, numbness, or paresthesias. She reports taking a daily birth control pill but denies any other medications including antiplatelets, anticoagulants or aspirin-containing migraine cocktails. The head CT performed at the outside hospital is not available but reportedly showed enlarged ventricles without clear evidence of a tumor. She was transferred to our tertiary center for further work-up. A brain MRI has been ordered by the ED provider and will be performed soon.

Vital Signs T 37.3°C, HR 75, BP 117/63, SpO2 99% on room air

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Pertinent Labs Na 141, Glu 98, Plt 205, INR 0.9, aPTT 25.0 Beta hCG negative Urine toxicology negative

Physical Exam Appears uncomfortable from headaches Alert and oriented to self, place, and year Following commands briskly in all extremities Pupils equal, round, and reactive to light Extraocular movements intact Visual fields full to confrontation Tongue midline, face symmetric No pronator drift Bilateral upper extremities 5/5 strength Bilateral lower extremities 5/5 strength Sensation intact to light touch throughout Wide-based gait

Triage Management The patient has a history of subacute headaches, however, the recent development of blurry vision, nausea, and imbalance is concerning. Her pregnancy test and urine toxicology are negative, and her blood values are within normal limits. Her presentation, together with the outside hospital head CT reporting hydrocephalus, can be related to a progressive increase in intracranial pressure. She will be evaluated for papilledema. The outside hospital head CT report does not mention a mass or tumor. However, small intraventricular masses can be easily missed, especially due to the potential isodense appearance of the tumor and the typically thicker cuts of screening CT scans. A brain MRI with and without contrast is performed in the ED (Figure 19.1) demonstrating a round T1 and T2 isointense, non-contrast enhancing lesion at the third ventricle with enlarged ventricles and mild transependymal flow. This is compatible with a symptomatic colloid cyst that is obstructing cerebrospinal fluid (CSF) flow at the level of the foramen of Monro. Given the presence of transependymal flow and red flag symptoms for intracranial hypertension, she will be admitted to a monitored unit (e.g. intermediate care unit or intensive care unit) for surgical planning. Her mental status is intact, and an external ventricular drain may be deferred at this time. In the interim, she will receive nonsedating pain medication or headache cocktails as well as acetazolamide. If the patient remains stable, a brain MRI with constructive interference in steady state (CISS) sequences may be performed to better evaluate the mass and the CSF obstruction. This is an urgent and operative consult.

Assessment This is a healthy 20 year old female with subacute headaches, blurry vision, and one day of nausea, dizziness, and imbalance. A brain MRI demonstrates a third ventricular lesion consistent with a colloid cyst causing obstructive hydrocephalus.

Plan Admit to the neurosurgical unit with frequent neurological checks n Assess for papilledema n Nonsedating headache cocktail or pain medications n

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C Figure 19.1 An MRI of the brain demonstrates a small round mass at the third ventricle (arrow) that is isointense on axial T1- without contrast (A) and T2- (B) sequences and does not enhance on coronal T1-weighted MRI with contrast (C). The ventricles are enlarged with mild transependymal flow.

Consider acetazolamide MRI brain with and without contrast, with protocol for navigation planning n Consider CISS sequences (Figure 19.2) n Plan for endoscopic intraventricular approach for cyst removal n Posting highlights: n Skull clamp n Neuronavigation n High-speed drill and craniotome n Ventricular cannulation port n Endoscopic tower and intraventricular endoscopes n n

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Figure 19.2 A midline sagittal brain MRI constructive interference in steady state (CISS) sequence shows an isointense round mass at the level of the foramen of Monro (dashed circle). n n n n

Irrigation bag and endoscopic irrigation system Endoscopic instruments: monopolar, forceps, scissors Warm saline in case bleeding is encountered Always be prepared to convert to an open approach if a major complication is encountered, or for external ventricular drain placement at the end of the procedure

LEARNING POINTS

• Colloid cysts are rare and histologically benign intracranial tumors. They originate on the roof of the third ventricle and can cause progressive obstruction at the level of the foramen of Monro with resultant hydrocephalus. • Colloid cysts are often iso- or hypointense to the brain parenchyma but can be hyperintense depending on the viscosity of the cystic contents.1 • The classical presentation of colloid cysts was paroxysmal headaches associated with postural changes. However, with the current widespread use of CT and MRI, colloid cysts are often incidentally found. Intermittent chronic headaches, papilledema, ataxia and memory loss are common symptoms.1,3 In patients with enlarged ventricles and subacute symptoms without visual disturbance or papilledema, surgery can be scheduled with close outpatient follow-up. • For asymptomatic patients, treatment options include observation or surgery. The risks and benefits of each option may be discussed with the patient. • Patients with colloid cysts who are also diagnosed with hydrocephalus are at a higher risk for acute deterioration and rarely, death.2. Hydrocephalus is often treated with the removal of the cyst, and patients do not usually require postoperative external ventricular drains or shunting.1,2 • The operative technique for the treatment of colloid cysts depends on the ventricular size and the surgeon’s comfort. In general, patients with large ventricles are treated with endoscopic removal of the lesion, whereas patients with small ventricles can be treated through a microsurgical transcallosal or a transcortical transventricular approach.3,4 Memory loss is not uncommon after this type of surgery given the stretch applied over the fornices during the tumor resection. Postoperative memory deficits are often transient.1 • If the patient requires a preoperative external ventricular drain, clamping the drain for a few hours before the endoscopic procedure can be helpful. This allows for the ventricles to slightly enlarge, providing a wider surgical corridor and improving visualization. Hemorrhage during an endoscopic approach is a major concern as it can be difficult to • control and hinders visibility. If encountered, warm irrigation and patience are key.

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References 1. Desai KI, Nadkarni TD, Muzumdar DP, Goel AH. Surgical management of colloid cyst of the third ventricle--a study of 105 cases. Surgery Neurology. 2002;57(5):295–304. https://doi.org/10.1016/ s0090-3019(02)00701-2. 2. de Witt Hamer PC, Verstegen MJ, De Haan RJ, et al. High risk of acute deterioration in patients harboring symptomatic colloid cysts of the third ventricle. J Neurosurg. 2002;96(6):1041–1045. https:// doi. org/10.3171/jns.2002.96.6.10415. 3. Kone L, Chaichana KL, Rincon-Torroella J, Snyman C, Moghekar A, Quiñones-Hinojosa A. The impact of surgical resection on headache disability and quality of life in patients with colloid cyst. Cephalalgia. 2017;37(5):442–451. 4. Sethi A, Cavalcante D, Ormond DR. Endoscopic versus Microscopic transcallosal Excision of colloid cysts: a Systematic Review in the Era of Complete endoscopic Excision. World Neurosurgery. 2019;132:e53– e58.

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Progressive Neurocognitive Decline Alice L. Hung, MD n Jordina Rincon-Torroella, MD

Consult Page 27F bilateral jaw dislocation. CT with large bifrontal lesion

Initial Imaging

Figure 20.1 Outside hospital axial head CT shows a large bifrontal diffuse hypodense lesion with partial effacement of the right lateral ventricle.

Walking Thoughts What is the patient’s neurological exam? What other symptoms does the patient have? n What is the timeline of her symptoms? n n

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Does the patient have any comorbidities? Is there any personal or family history of cancer, infection, or autoimmune disease? Did the patient have a seizure causing bilateral jaw dislocation? What is her baseline functional status? What workup has been done so far? Is she on any antiplatelet or anticoagulant medications?

History of Present Illness A previously healthy 27 year old female presents to the emergency department (ED) with bilateral jaw dislocation. She is unable to provide her medical history due to her dislocated jaw; therefore, clinical information is obtained from her parents. The patient was fully independent living with her parents until two months prior when they began noticing progressive cognitive decline. They first noticed that the patient started having problems with her daily tasks. It was more difficult for her to stock shelves at work, and she began skipping steps of routine activities, such as not using soap in the shower. Two weeks ago, she started slurring her speech, perseverating, and having a difficult time focusing. They brought her to her primary care physician, who ordered bloodwork and a brain MRI. The brain MRI reportedly showed a large bifrontal lesion, and she was in the process of undergoing expeditious outpatient work-up given the concern for malignancy. This morning, the patient was found to have difficulty articulating words and was unable to fully close her mouth. She was initially taken to an outside hospital ED, where a maxillofacial CT demonstrated bilateral mandibular dislocation and a diffuse bifrontal hypodensity. The patient was then transferred to our tertiary care ED for jaw reduction. Currently, the patient denies any headaches, focal weakness, numbness, tingling, or vision changes. She reports no recent trauma and has no personal or family history of connective tissue disorder, malignancy, or inflammatory disease. Her family denies any involuntary movements, tongue biting, or loss of consciousness. She does not take any anticoagulant or antiplatelet medications.

Vital Signs T 36.6°C, HR 97, BP 123/95, SpO2 99% on room air

Pertinent Labs Na 139, Glu 94, Hgb 16.5, WBC 6.4, Plt 234, INR 0.8, aPTT 24.3 ESR 2, CRP 0.1 Urine toxicology negative Beta-hCG negative

Physical Exam Alert and oriented to self, place, and year to choices given inability to talk Following commands briskly in all extremities Pupils equal, round, and reactive to light Extraocular movements intact, tongue midline Unable to close her mouth Drooling with slurred speech Visual fields full to confrontation No drift

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Bilateral upper extremities 5/5 strength Bilateral lower extremities 5/5 strength Sensation intact to light touch throughout

Triage Management The patient presents with a subacute decline in functional status and has a known large bifrontal lesion. In the absence of trauma or known connective tissue disorder, bilateral jaw dislocation in the setting of a known intracranial lesion can be suspicious for seizure presentation. A brain MRI is obtained in the ED showing a bifrontal lesion with incomplete ring enhancement (Figure 20.2). She is currently stable but will require admission to evaluate for seizures and to complete a full neurological work-up. The differential includes malignancy, infection, or an autoimmune process. While malignancy is possible (such as glioblastoma or lymphoma), given the patient’s presentation and imaging characteristics of the lesion, other etiologies must be considered. The patient has been afebrile with reassuring WBC and negative inflammatory markers, making infection a less likely etiology. In a young and otherwise healthy patient, an autoimmune process is high on the differential. Given that the patient is currently stable with no signs of herniation from mass effect requiring urgent neurosurgical intervention, neurology evaluation is recommended for further management. The patient may require a brain biopsy if the work-up remains unrevealing. Of note, while lumbar puncture is routinely used in the diagnostic work-up of inflammatory and infectious diseases, careful consideration must be given in her case due to the size of the lesion and its associated mass effect. This is a non-emergent, but potentially operative consult.

Assessment This is a previously healthy 27 year old female presenting with two months of subacute functional decline and bilateral jaw dislocation in the setting of a newly diagnosed large bifrontal lesion with incomplete ring enhancement.

A

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Figure 20.2 Axial brain MRI with and without contrast demonstrates a large bifrontal lesion. A. Post-contrast sequence shows an infiltrative hypointense T1 lesion with an “open ring” or incomplete ring pattern of enhancement. B. FLAIR sequence demonstrates a large solitary hyperintense bifrontal lesion crossing the corpus callosum. Note that there is relatively little surrounding edema.

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Plan n n n n

Consultation to neurology and potential admission to the neurology team Defer initiation of antiepileptic and steroid medications to the neurology team Consultation to the ENT team for bilateral mandibular dislocation No acute neurosurgical intervention; if required, the neurosurgery team will be available for biopsy

Follow-Up The neurology team assessed the patient in the ED and felt her presentation and imaging were concerning for an inflammatory disease. She was admitted to the neurology service for further work-up. ENT was consulted for her bilateral mandibular dislocation which was managed conservatively with jaw reduction and a pressure dressing. The patient was started on levetiracetam for seizure prophylaxis and a routine electroencephalogram was obtained which showed bilateral cerebral disturbances without electrographic seizures. An extensive set of laboratories, including LDH, ESR/CRP, C3/C4, anti-dsDNA, TSH, B12, peripheral blood smear, blood flow cytometry, and inflammatory/autoimmune markers were negative. A lumbar puncture was considered to be too risky given the size of the lesion, effacement of the right lateral ventricle, and potential downward herniation with a large volume tap. Cervical and thoracic spine MRI were obtained without evidence of other demyelinating lesions. A CT of the chest, abdomen, and pelvis ruled out other primary malignancies. Given this negative work-up and the inability to obtain CSF, the neurology team requested a brain biopsy for diagnosis. Intraoperative cultures were negative. The pathology was consistent with a demyelinating process, suggestive of tumefactive demyelination. The patient was started on a 5-day course of IV solumedrol. Her clinical exam improved and a repeat brain MRI after completion of the steroid course showed near resolution of the contrast enhancement (Figure 20.3). The patient was discharged home on a course of oral steroids and levetiracetam with neurology outpatient follow-up.

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Figure 20.3 Axial brain MRI with contrast one month after corticosteroid treatment. A. Post-contrast sequence shows significant reduction in enhancement. B. FLAIR sequence demonstrates decrease in hyperintensity and size of the FLAIR signal. The mass effect on the right lateral ventricle has resolved.

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LEARNING POINTS

• Non-traumatic mandibular dislocation is very rare but can be present after an epileptic seizure or as a symptom of neurodegenerative or neurodysfunctional disorders such as Huntington’s disease or multiple sclerosis. • In atypical brain lesions resembling brain tumors in MRI, it is necessary to rule out other non-tumoral causes before proceeding to surgery. These patients receive an exhaustive work-up by the neurology team. A brain biopsy, which can be done open or stereotactically, is left as the last step if other less invasive techniques are non-diagnostic. While stereotactic biopsy can be associated with risk of hemorrhage and sampling error, the diagnostic yield has been reported to be up to 90%.6 • The patient’s clinical presentation and past medical history are essential to evaluate brain tumor mimics.7 Characteristics that suggest non-tumoral causes of brain lesions: • Young age • High-risk sexual behavior • IV drug use • Travel to countries with endemic infectious diseases or prior contact with tuberculosis • Personal or family history of autoimmune and/or inflammatory diseases • Fevers, recent dental procedures, ENT infections • Immunosuppression, including diabetes • Transient neurological deficits • Skin rashes or oral and genital ulcers • Abnormalities on body CT scan (e.g. primary cancer, inflammatory disease) Tumefactive Multiple Sclerosis

• Tumefactive multiple sclerosis presents as a large (generally >2 cm) solitary lesion with associated mass effect that resembles malignancy. It is a rare diagnosis with an estimated prevalence of 1 to 2 per 1000 cases of multiple sclerosis.2,4 • On MRI, it characteristically demonstrates contrast enhancement in an “open ring” or incomplete ring fashion. Unlike high grade gliomas, the associated edema is minimal.3,4 • Patients presenting with tumefactive multiple sclerosis will often require biopsy for tissue diagnosis. Once the diagnosis is established, initial treatment is with corticosteroids.2,3 • The majority of patients demonstrate a positive response associated with reduction of inflammation and enhancement on MRI (Figure 20.3). Long-term disease-modifying agents may be considered for patients with recurrent disease.2 • Other possible inflammatory brain lesions include Behçet’s disease or neurosarcoidosis. Other Tumor Mimics

• Intracranial infections (e.g. abscess, tuberculosis, toxoplasmosis, neurocysticercosis) can also present with ring enhancement, mass effect, and edema, resembling gliomas on imaging. These patients often have other signs of infection such as fever, chills, leukocytosis, and elevated inflammatory markers (ESR/CRP). The acuity of symptom onset and other clues, such as recent travel and/or an immunocompromised state, are also helpful to distinguish infection from neoplasm. Prompt initiation of antimicrobials is essential in these cases. However, biopsy may still be required to establish diagnosis and to obtain tissue for culturing.1,5,7 • In patients with a previously diagnosed brain tumor that has been treated with radiation, treatment necrosis is another possibility on the differential. These patients can present with worsening neurological deficits, headaches, nausea, and vomiting with imaging demonstrating an increase in lesion size, enhancement, or mass effect. While these findings are concerning for possible tumor recurrence, pseudoprogression from radiation necrosis appears similarly. Various approaches have been proposed to distinguish between the two, but the current gold standard is still tissue biopsy.1,8

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• S ubacute strokes can also have contrast enhancement and be mistaken as masses. However, they are typically confined to certain vascular distributions. Vascular neoplasms can sometimes present with hemorrhage, and initial imaging may be obscured by acute blood products. There may be associated vasogenic edema and mass effect from the underlying lesion that raises suspicion for a neoplasm.7 Repeat imaging in a delayed fashion, once the blood and inflammation have decreased, may aid in the differentiation of a vascular versus a neoplastic lesion.

References 1. Bradley D, Rees J. Brain tumour mimics and chameleons. Practice Neurology. 2013;13(6):359–371. 2. Brod SA, Lindsey JW, Nelson F. Tumefactive demyelination: clinical outcomes, lesion evolution and treatments. Mult Scler J Exp Transl Clin. 2019;5(2). 2055217319855755. 3. Given CA, 2nd, Stevens BS, Lee C. The MRI appearance of tumefactive demyelinating lesions. AJR Am J Roentgenol. 182(1), 195–199. 4. Kaeser MA, Scali F, Lanzisera FP, Bub GA, Kettner NW. Tumefactive multiple sclerosis: an uncommon diagnostic challenge. J Chiropr Med. 2011;10(1):29–35. 5. Khullar P, Datta NR, Wahi IK, Kataria S. Brain abscess mimicking brain metastasis in breast cancer. J. Egypt. Natl. Cancer Inst. 2016;28(1):59–61. 6. Lara-Almunia M, Hernandez-Vicente J. Symptomatic intracranial hemorrhages and frame-based stereo- tactic brain biopsy. Surg. Neurol. Int. 2020;11:218. 7. Omuro AM, Leite CC, Mokhtari K, Delattre JY. Pitfalls in the diagnosis of brain tumours. Lancet Neurol. 2006;5(11):937–948. 8. Verma N, Cowperthwaite MC, Burnett MG, Markey MK. Differentiating tumor recurrence from treatment necrosis: a review of neuro-oncologic imaging strategies. Neuro Oncol. 2013;15(5):515–534.

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Unresponsive Patient Ryan P. Lee, MD n

Risheng Xu, MD/PhD

Consult Page 70yo male found down with a large brain bleed.

Initial Imaging

A

B

Figure 21.1 Axial head CT without contrast (A) shows a large 6 cm hemorrhage centered at the right lentiform nucleus with extension to the thalamus and bilateral lateral ventricles. Coronal view (B) demonstrates 10 mm of midline shift and ventriculomegaly concerning for hydrocephalus.

Walking Thoughts Where is the hemorrhage? What is the mechanism of the bleed? n What is the patient’s GCS? Does the patient need airway protection? n Was the exam performed on sedation? n Are there signs of intracranial pressure (ICP) elevation or herniation? If so, have medical measures been initiated? n Is there intraventricular hemorrhage (IVH) or hydrocephalus that might require a ventriculostomy? n What is the blood pressure? Was this a hypertensive hemorrhage? n What is the patient’s medical history? Is there a history of brain metastases or vascular lesions? n n

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21—UnresPonsive Patient n

n n

105

Is the patient on any antiplatelet or anticoagulant medications? If so, have these been reversed? Is the patient a candidate for surgical evacuation or decompression? Does the patient have an advanced directive in place? Who is his healthcare agent?

History of Present Illness A 70 year old male with a history of hypertension, ischemic strokes, and prior myocardial infarction on daily aspirin 81 mg was found unresponsive at home. On the scene, he was GCS 3 with a systolic blood pressure ranging from 200-220 mmHg. He was intubated, started on a nicardipine infusion, and brought to his local emergency department. Head CT showed a 6 cm basal ganglia hemorrhage centered at the right lentiform nucleus with thalamic and intraventricular extension. He was given levetiracetam and mannitol, and transferred emergently to our institution for further management.

Vital Signs T 36.7oC, HR 72, BP 114/60 on nicardipine, MAP 78, SpO2 100% (on 40% FiO2 via endotracheal tube)

Pertinent Labs Na 139, Cr 4.5, Trop 0.05 Hgb 8.0, WBC 6.56, Plt 192, PT 10.6, INR 1.0, aPTT 25.5

Glasgow Coma Scale Motor: 5 Verbal: 1 Eye opening: 2 Intubated GCS Total: 8T

Physical Exam Intubated, sedation and paralytics held Hard cervical collar in place Eyes open to painful stimulation Pupils equal, round, reactive to light Cough, gag, and corneal reflexes present Localizing to pain in right upper extremity Withdrawing to pain in right lower extremity Minimal to no movement in left upper or lower extremity to pain No signs of external trauma

Triage Management The patient has a large right basal ganglia hemorrhage causing significant mass effect with 10 mm of midline shift and right uncal herniation. Given his history of hypertension, elevated blood pressure on arrival, and location in the basal ganglia, this is likely a spontaneous hypertensive hemorrhage. There is elevated ICP from mass effect of the hematoma and surrounding edema. Medical measures of addressing acute ICP elevation are continued during triage.

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He also has significant intraventricular extension of hemorrhage and acute hydrocephalus with a poor exam off sedation after medical resuscitation, so an external ventricular drain (EVD) may be placed emergently for ICP monitoring and CSF drainage. An attempt to reach family should be made to discuss the patient’s critical condition and obtain procedural consent. An EVD is planned unless there is a clear advanced directive against further measures (e.g. do not resuscitate orders) or the family declines. Labs show a normal platelet count, PT, INR, and aPTT. He has a history of aspirin use, so platelet transfusion can be initiated given the plan for neurosurgical intervention. In general, surgery for basal ganglia clot removal and treatment of mass effect can be considered life-saving but may not affect functional outcome. Surgical evacuation is not generally offered given the lack of evidence of efficacy in improving functional outcome. However, this is controversial and evacuation and/or hemicraniectomy are performed at some centers. This patient will be admitted to the neurocritical care unit for management of his EVD, close blood pressure control, and further work-up to rule out other causes of bleed (including a vascular lesion or tumor). This is an emergent, operative consult.

Assessment This is a 70 year old male with a history of hypertension, ischemic strokes, and prior myocardial infarction who was found unresponsive with a large hypertensive right basal ganglia hemorrhage with extension to the thalamus, associated IVH, and hydrocephalus. He has acute elevation in ICP and depressed GCS (8T). An emergent EVD is planned to treat his elevated ICP and hydrocephalus.

Plan Admission to the neurocritical care unit with neurological exams every hour Medical management of acute intracranial hypertension: hyperventilation, head of bed elevation, hyperosmolar therapy, hypertonic saline n Arterial line and foley catheter placement n Emergent placement of EVD after platelet transfusion and preprocedural antibiotics n Repeat head CT with CT angiogram after EVD placement to rule out underlying vascular malformation n Maintain ICP 50-70 mmHg n Maintain systolic blood pressure below 160 mmHg, nicardipine infusion as needed n Maintain elevated sodium goal (e.g. >145) n Maintain NPO with maintenance (isotonic or hypertonic) IV fluids n EKG, trend troponins n Consultation to neurology for antiepileptic management and management of hemorrhagic stroke if neurosurgical causes of bleed are ruled out n Consultation to nephrology for creatinine of 4.5 n Once stable, MRI brain with and without contrast to evaluate for other causes of the bleed n Possible surgical decompression if ICPs remain refractory to medical management n

n

LEARNING POINTS

• Remember to first address the ABC’s. • Initial management of acutely elevated ICP includes elevation of head of bed, hyperosmolar therapy, hypertonic saline, hyperventilation, and sedation.4

For ICH patients presenting with SBP between 150 and 220 mm Hg and without • contraindication to acute BP treatment, acute lowering of SBP to 140 mm Hg is safe and can be effective for improving functional outcome.4 It is our practice to set systolic blood pressure goals of less than 160 mmHg.

21—UnresPonsive Patient

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• Invasive ICP monitoring, usually with ventriculostomy, should be considered in patients with GCS < 8, clinical evidence of transtentorial herniation, or significant IVH or hydrocephalus.4 • Primary intracerebral hemorrhage (ICH) is typically secondary to hypertension (usually in the basal ganglia) or amyloid angiopathy (typically lobar in location).4 • CT angiography or MR angiography is recommended and is typically done as soon as the patient is stable to rule out underlying vascular etiologies of ICH.4 • If the patient is known or found to have severe thrombocytopenia or coagulopathy due to a factor deficiency, the appropriate replacement therapy of platelets or coagulation factors should be administered.1 • Platelet transfusion for history of platelet inhibitor use is generally not recommended if the patient is not undergoing surgical intervention.4 For patients undergoing surgical intervention, platelet transfusion is recommended for those with history of aspirin or ADP receptor inhibitor use.1 • All anticoagulants should be held and immediately reversed.4 • If there is neurological deterioration or elevated ICP refractory to medical management, surgical clot evacuation and/or bony decompression can be considered as a life-saving measure. As demonstrated by the STICH I and II trials, hematomas that are moderatesized and within 1 cm of the cortical surface are typically considered the best surgical candidates, as are those with GCS 9-12. Poor surgical candidates include those with hematomas so massive as to already be devastating or so small that the risk of surgery outweighs any potential benefit.5 • Catheter based clot evacuation with intraventricular alteplase, either for ICH (MISTIE I, II, III trials), or for IVH (CLEAR I, II, III trials), may be considered. The MISTIE III trial demonstrated improved survival for patients undergoing clot evacuation, but only improved functional outcomes in patients with less than 15 cc of residual hemorrhage in subgroup analysis.2 The CLEAR III trial also demonstrated improved survival for patients undergoing intraventricular clot evacuation with intraventricular alteplase but failed to demonstrate improved functional outcomes neurologically.3 • There should be discussion with the family regarding surgical intervention as life-saving measures may not improve long-term, neurologic functional outcome.

References 1. Frontera JA, Lewin 3rd JJ, Rabinstein AA, et al. Guideline for reversal of antithrombotics in intracranial hemorrhage: executive summary. A statement for healthcare professionals from the Neurocritical Care Society and the Society of Critical Care Medicine. Crit Care Med. 2016;44(12):2251–2257. 2. Hanley DF, Thompson RE, Rosenblum M, et al. Minimally invasive surgery with thrombolysis in intracerebral haemorrhage evacuation (MISTIE III): a randomised, controlled, open-label phase 3 trial with blinded endpoint. Lancet. 2019;393(10175):1021–1032. 3. Hanley DF, Lane K, McBee N, et al. Thrombolytic removal of intraventricular haemorrhage in treatment of severe stroke: results of the randomised, multicentre, multiregion, placebo-controlled CLEAR III trial. Lancet. 2017;389(10069):603–611. 4. Hemphill 3rd JC, Greenberg SM, Anderson CS, et al. Guidelines for the management of spontaneous intracerebral hemorrhage: a guideline for healthcare professionals from the American Heart Association/ American Stroke Association. Stroke. 2015;46(7):2032–2060. 5. Mendelow AD, Gregson BA, Fernandes HM, et al. Early surgery versus initial conservative treatment in patients with spontaneous supratentorial intracerebral haematomas in the International Surgical Trial in Intracerebral Haemorrhage (STICH): a randomised trial. Lancet. 2005;365(9457):387–397.

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22

Severe Headache Ryan P. Lee, MD n

Risheng Xu, MD/PhD

Consult Page 57 year old female with large cerebellar hemorrhage

Initial Imaging

Figure 22.1 Axial head CT without contrast demonstrates a 5 cm acute left cerebellar hemorrhage with associated edema, causing effacement of the fourth ventricle and mass effect on the brainstem. There is ventriculomegaly indicative of obstructive hydrocephalus.

Walking Thoughts n n n n

108

What is the mechanism of the bleed? What is the patient’s GCS? Does the patient need airway protection? Was the exam performed on sedation? Are there signs of intracranial pressure (ICP) elevation or herniation? If so, have medical measures been initiated?

22—Severe HeadacHe n

n n n n n

109

Is there intraventricular hemorrhage (IVH) or hydrocephalus that might require a ventriculostomy? What is the blood pressure? Was this a hypertensive hemorrhage? What is the patient’s medical history? Is there a history of brain metastases or vascular lesions? Is the patient on any antiplatelet or anticoagulant medications? If so, have these been reversed? Is the patient a candidate for surgical evacuation or decompression? Does the patient have an advanced directive in place? Who is her healthcare agent?

History of Present Illness A 56 year old female with a history of active heroin use and hypertension (noncompliant with medication) presents to the emergency room with sudden severe headache. She was brought by ambulance with a systolic blood pressure (SBP) over 200 mmHg. En route, she developed nausea and vomiting. She denied any antiplatelet or anticoagulant medication use. Head CT on arrival showed a large left cerebellar hemorrhage with surrounding edema, mass effect on the brainstem, and obstructive hydrocephalus. CT angiogram (CTA) of the head was negative for an underlying vascular lesion. While in the emergency room, her exam worsened, and she became progressively more somnolent.

Vital Signs T 37.4oC, HR 88, RR 15, BP 223/112, MAP 149, SpO2 97% on room air

Pertinent Labs Na 139, Cr 0.9, Trop < 0.02 AST 115 (normal 3-37), ALT 123 (normal 6-65), total bilirubin 1.3 (normal 0.2-1.2) Hgb 12.6, WBC 11.4, Plt 142, PT 13.9, INR 1.5, aPTT 25 Urine toxicology positive for codeine and morphine

Glasgow Coma Scale Motor: 4 Verbal: 2 Eye opening: 2 GCS Total: 8

Physical Exam Somnolent No sedation Eyes open to painful stimulation Pupils 3 mm, equal, round, and reactive to light Cough, gag, and corneal reflexes present Face symmetric with grimace Withdrawing to pain in all four extremities No signs of external trauma Track marks on arms and legs

Triage Management This patient is neurologically deteriorating from a large left cerebellar bleed. She should be triaged swiftly in this emergent situation. There is evidence of obstructive hydrocephalus and brainstem

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compression, both of which are likely contributing to the patient’s decline. The ABC’s of airway, breathing, and circulation must be addressed first. She is intubated for airway protection given a GCS of 8 and progressive decline. An arterial line is placed for close blood pressure monitoring, and her hypertension needs to be addressed acutely. Medical measures of addressing acute ICP elevation are initiated and continued during triage while interventions are planned. Since a CTA has already been performed and was negative for an underlying vascular lesion, this is likely a hypertensive hemorrhage. With a marked exam decline, an emergent external ventricular drain (EVD) is planned to address the hydrocephalus. This patient is also a good candidate for suboccipital craniectomy to relieve brainstem compression. If family is not present in the ED, an attempt to reach them should be made to discuss the patient’s critical condition and obtain procedural consent. There is no history of antiplatelet or anticoagulant medication to reverse. Her platelet count is normal, but her INR is slightly elevated at 1.5 and there are abnormalities on the liver function panel, suggestive of liver dysfunction causing coagulopathy. Given her planned interventions, it is reasonable to address this with vitamin K or factor replacement emergently. This is an emergent, operative consult.

Assessment This is a 56 year old female with a history of active heroin use and uncontrolled hypertension who presents with a left cerebellar hemorrhage causing brainstem compression and acute obstructive hydrocephalus. A history of hypertension, high blood pressure on arrival, and negative CTA make a hypertensive origin almost certain. A history of heroin abuse, abnormal liver function tests, and an INR of 1.5 indicate possible coagulopathy. Her exam is declining rapidly, and she will require emergent external ventricular drain placement and suboccipital craniectomy for brainstem decompression. Consent for intervention was obtained from the patient’s adult son, who understood the critical condition and the risks and benefits of the proposed procedures.

Plan n

n n n n n n n

n n n

In the ED, intubation and rapid medical management of acute intracranial hypertension: hyperventilation, head of bed elevation, hyperosmolar therapy, hypertonic saline Arterial line and foley catheter placement Maintain systolic blood pressure below 160 mmHg—nicardipine infusion as needed Maintain NPO with maintenance IV fluids Vitamin K administration and factor replacement Emergent placement of EVD with preprocedural antibiotics EVD leveled at 15-20 mmHg with special care to avoid significant CSF drainage Emergent suboccipital decompressive craniectomy after placement of EVD n Posting highlights: n Prone positioning with skull clamp n No intraoperative neuromonitoring n High speed drill n Dural substitutes n Hemostatic agents n For anesthesia: antibiotics, mannitol 1 g/kg, pCO2 25-30 mmHg until brain is decompressed Admission to the neurocritical care unit with hourly neurological exams Maintain elevated sodium goal (e.g. >145) Consult to neurology for management of hemorrhagic stroke

22—Severe HeadacHe

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LEARNING POINTS

• Remember to first address the ABC’s of airway, breathing, and circulation. • Initial management of acutely elevated ICP includes elevation of head of bed, hyperosmolar therapy, hypertonic saline, hyperventilation, and sedation.3

• For hemorrhagic stroke patients presenting with SBP between 150 and 220 mmHg and without contraindication to acute BP treatment, acute lowering of SBP to 140 mmHg is safe and can be effective for improving functional outcome.3 It is our practice to set SBP goals of less than 160 mmHg. • Invasive ICP monitoring, usually with ventriculostomy, should be considered in patients with GCS 150, and mannitol 1g/ kg. Discuss with family the possible need for hemicraniectomy as a life saving measure if the patient continues to deteriorate. The patient’s values and wishes should be discussed when considering this procedure, as hemicraniectomy can reduce mortality but often leaves patients with severe disabilities. If the patient and/or family are agreeable, consent should be obtained. If the patient worsens neurologically despite maximal medical management of ICPs, and the patient/family agrees, an emergent left hemicraniectomy will be performed. n Posting highlights: n Skull clamp or horseshoe n No intraoperative neuromonitoring n Burr hole drill and craniotome n Dural substitutes n Hemostatic agents n For anesthesia: antibiotics, mannitol 1g/kg, levetiracetam 1g, pCO2 25-30 mmHg until brain is decompressed

Follow-Up The patient’s neurological exam progressively declined despite sodium augmentation to 161 with inability to follow commands, progressive lethargy, and worsening midline shift on head CT (Figure 24.2). After extensive discussion with the family, the decision was made to proceed with emergent hemicraniectomy (Figure 24.3).

A

B

Figure 24.2 Axial (A) and coronal (B) head CT without contrast 36 hours after admission demonstrates increased brain edema with 1.2 cm of midline shift. The patient was subsequently taken to the operating room for a left hemicraniectomy.

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A

B

Figure 24.3 Axial (A) and coronal (B) postoperative head CT without contrast demonstrates left sided hemicraniectomy with significant subtemporal decompression, resultant complete resolution of midline shift, and maximal decompression of the mesial temporal brain.

LEARNING POINTS

• Patients with large ischemic strokes require intensive care and maximal medical management led by the neurology or the neurocritical care team.

• Brain edema is expected to peak over the first 48–96 hours after the stroke, and thus vigilant observation for neurological decline is necessary.6

• Hemicraniectomy is often used as a life-saving measure for patients with malignant strokes. Whether or not to pursue this procedure depends largely on the patient’s age, location of the stroke, baseline functional status, and most importantly, on the patient’s values and wishes.1 • There have been a number of randomized controlled trials examining the efficacy of hemicraniectomy in the context of MCA or hemispheric stroke, including DESTINY, DESTINY II, DECIMAL, and HAMLET.3-5,8 All demonstrate that hemicraniectomy is able to decrease mortality rates from 46–75% to 12–27%, with an absolute mortality risk reduction of a half to a third. • It is important to keep in mind that those receiving a hemicraniectomy may survive but with significant morbidity. Results from these studies suggest that those under 60 years of age with strokes on the nondominant side, and those with better pre-stroke functional status have the best outcomes. Vahedi et al. performed a meta-analysis of patients in the DESTINY, DECIMAL, and HAMLET trials, and found that those who underwent surgery within 48 hours from stroke had better functional outcomes.7 • Ultimately, this procedure is offered on a case-by-case basis and depends greatly on the patient’s goals of care. • Uncal herniation results in direct brainstem compression and is a neurosurgical emergency. A key step during a hemicraniectomy is a wide subtemporal decompression to alleviate uncal herniation. Early mobilization and aggressive physical rehabilitation is associated with improved • neurological outcomes in patients after ischemic stroke.2 In post-hemicraniectomy patients, a customized helmet is important for safe mobilization. Eventually, bone flap replacement or a cranioplasty will be necessary.

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References 1. Alexander P, Heels-Ansdell D, Siemieniuk R, et al. Hemicraniectomy versus medical treatment with large MCA infarct: a review and meta-analysis. BMJ Open. 2016;6(11). 2016 Nov 24. e014390. 2. Cumming TB, Thrift AG, Collier JM, et al. Very early mobilization after stroke fast-tracks return to walking: further results from the phase II AVERT randomized controlled trial. Stroke. 2011;42(1):153– 158. 3. Hofmeijer J, Kappelle LJ, Algra A, et al. Surgical decompression for space-occupying cerebral infarction (the Hemicraniectomy after Middle Cerebral Artery infarction with Life-threatening Edema Trial [HAMLET]): a multicentre, open, randomised trial. Lancet Neurol. 2009;8(4):326–333. 4. Jüttler E, Schwab S, Schmiedek P, et al. Decompressive surgery for the treatment of malignant infarction of the middle cerebral artery (DESTINY ): a randomized, controlled trial. Stroke. 2007;38(9):2518–2525. 5. Jüttler E, Unterberg A, Woitzik J, et al. Hemicraniectomy in older patients with extensive middlecerebralartery stroke. N Engl J Med. 2014;370(12):1091–1100. 6. Robertson FC, Dasenbrock HH, Gormley, WB. Decompressive hemicraniectomy for stroke in older Adults: a review. J Neurol Neuromedicine. 2017;2(1):1–7. 7. Vahedi K, Hofmeijer J, Juettler E, et al. Early decompressive surgery in malignant infarction of the middle cerebral artery: a pooled analysis of three randomised controlled trials. Lancet Neurol. 2007;6(3):215– 222. 8. Vahedi K, Vicaut E, Mateo J, et al. Sequential-design, multicenter, randomized, controlled trial of early decompressive craniectomy in malignant middle cerebral artery infarction (DECIMAL Trial). Stroke. 2007;38(9):2506–2517.

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25

Acute Aphasia And Right-Sided Weakness Andrew Luksik, MD n

Risheng Xu, MD/PhD

Consult Page 89F with a left MCA syndrome and perfusion mismatch, consult for thrombectomy

Initial Imaging

Figure 25.1 An axial head CT without contrast shows a hyperdense left MCA sign (arrow) and hypodensity in the left lentiform nucleus (arrow heads), concerning for acute left MCA occlusion. Vessels on head CT without contrast are typically isodense, and a hyperdensity within a vessel indicates a hyperacute clot or a calcification. The right insular hypodensity seen is likely sequela from an old stroke.

Walking Thoughts Have the ABC’s been appropriately addressed? n When was the patient last seen normal? n Has the stroke team evaluated the patient? n Was tissue plasminogen activator (tPA) given? n

122

123

ss n n n

n n

What is the patient’s NIH stroke scale score? Is the patient on any antiplatelet or anticoagulant medications? What imaging has been performed? Is there a large vessel occlusion? Does perfusion imaging need to be obtained? Does the patient need a mechanical thrombectomy? Does the patient have an advanced directive in place? Who is her healthcare agent?

History of Present Illness An 89 year old female with a history of prior strokes, hypertension, and atrial fibrillation, notably not on any anticoagulants or antiplatelets, presents to the emergency department (ED) after being found down in her assisted living facility. She was last seen well eating dinner approximately 10 hours prior. At baseline per her daughter, she is able to independently carry out the majority of her activities of daily living. She was initially triaged in the ED as a trauma patient due a presumed fall. However, on examination, she was noted to have a left gaze deviation and hemiparesis of the right side with aphasia. The stroke team was activated, and she was taken immediately to the CT scanner. Head CT without contrast showed a hyperdense left middle cerebral artery (MCA) sign and subtle hypodensity in the left MCA territory (Figure 25.1). Given concern for a large vessel occlusion, a CT angiogram (CTA) of the head and neck was performed, which showed an abrupt cut-off at the left proximal MCA (M1 or horizontal segment). CT perfusion (CTP) demonstrated marked perfusion deficit with relatively preserved cerebral blood volume (Figure 25.2). Given the large penumbra and relatively small core infarct, neurosurgery was consulted for possible thrombectomy. The stroke neurology team assessed the patient, giving her a NIH stroke scale score of 20. Her Alberta stroke program early CT (ASPECTS) score based on initial head CT was 9.

Vital Signs T 37.2oC, HR 72, BP 145/74, RR 27, SpO2 100% on 4 L nasal cannula

A

B

CBF

C

CBV

Figure 25.2 Coronal head CTA with maximal intensity projections (A) demonstrates occlusion of the left M1 segment of the MCA. CT perfusion shows a significantly larger area of decreased perfusion (dark blue) on cerebral blood flow imaging (B) compared to the smaller area of core infarct (dark blue) seen on cerebral blood volume imaging (C) concerning for ischemic penumbra.

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Pertinent Labs Na 140, Plt 144, Cr 1.3, INR 1.1, aPTT 29.0

Glasgow Coma Scale Motor: 5 Verbal: 2 Eye opening: 4 GCS Total: 11

Physical Exam Alert, eyes open Not following commands Markedly aphasic, makes incoherent noises Pupils 3 mm and briskly reactive Left gaze preference Right facial droop Left upper and lower extremities moving spontaneously, at least antigravity Right upper and lower extremities minimal movement to pain

Triage Management This is a patient with an acute left MCA syndrome with significant perfusion mismatch on imaging, requiring mechanical thrombectomy for optimal outcome. The ABC’s must first be addressed given her altered mental status and tachypnea. She is intubated for airway protection. Because the patient was last seen normal more than 4.5 hours prior, she is not a candidate for IV tPA. From a neurosurgical perspective, our involvement depends on whether the patient is a candidate for mechanical thrombectomy. The patient has a clinical syndrome concerning for large vessel occlusion, which is confirmed on CTA (Figure 25.2A). Because the time the patient was last known normal was 12 hours ago, she needed imaging to demonstrate that there is salvageable brain tissue. Thus, a CT perfusion scan was obtained to determine if there is an ischemic penumbra that can be saved with reperfusion. In our case, although there is some completed infarct (Figure 25.2C), there is still a significant area at risk (Figure 25.2B). Therefore, in this case the patient would benefit from revascularization of the large vessel occlusion. Once the decision to pursue thrombectomy is made, ensuring the patient is transported to the neurointerventional radiology (NIR) suite expeditiously is critical, as time is brain. The patient’s critical condition and need for mechanical thrombectomy will be discussed with her daughter. The stroke neurology team will continue guiding overall stroke care, including blood pressure management, and further stroke workup. The patient will be admitted to the neurocritical care unit under the neurology service postoperatively. This is an emergent, operative consultation.

Assessment This is an 89 year old female with a history significant for atrial fibrillation off anticoagulation, hypertension, and prior strokes, who presents to the ED with a left MCA syndrome. Imaging shows a left M1 occlusion with perfusion mismatch. She is outside of the tPA window with a NIH stroke scale score of 20 and an ASPECTS score of 9. She will need an emergent mechanical thrombectomy.

ss

A

125

B

Figure 25.3 A. Anterior-posterior projection of a pre-thrombectomy left internal carotid artery injection demonstrates a left M1 occlusion. B. Post-thrombectomy injection shows TICI 3 or complete reperfusion of the left MCA territory.

Plan n

n

n

Emergent activation of the NIR stroke and anesthesia teams for mechanical thrombectomy SBP target >160 mmHg to maximize collateral perfusion of ischemic penumbra before revascularization; SBP 3 or aspect ratio > 1.6

1

Location (single)

Basilar artery bifurcation

5

Vertebral/basilar artery

4

AcomA or PcomA

2

Maximum diameter (single)

Other (multiple)

Aneurysm sizerelated risk (single)

⎕

3

Contralateral stenoocclusive vessel 1 disease Age-related risk (single)

⎕

Aneurysm growth on serial imaging 4 Aneurysm de novo formation on serial imaging

Treatment

⎕

< 40 years

0

41–60 years

1

61–70 years

3

71–80 years

4

> 80 years

5

< 6.0 mm

0

6.0–10.0 mm

1

10.1–20.0 mm

3

> 20 mm

5

Aneurysm complexityrelated risk

High

3

Low

0

Intervention-related risk

Constant

⎕

⎕

⎕

5

TOTAL ­ Favors conservative management   

  

References 1. Etminan N, Brown Jr RD, Beseoglu K, et al. The unruptured intracranial aneurysm treatment score: a multidisciplinary consensus. Neurology. 2015;85(10):881–889. 2. Greving JP, Wermer MJ, Brown Jr RD, et al. Development of the PHASES score for prediction of risk of rupture of intracranial aneurysms: a pooled analysis of six prospective cohort studies. Lancet Neurol. 2014;13(1):59–66.

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3. Juvela S, Poussa K, Porras M. Factors affecting formation and growth of intracranial aneurysms: a long- term follow-up study. Stroke. 2001;32(2):485–491. 4. Olafsson E, Hauser WA, Gudmundsson G. A population-based study of prognosis of ruptured cerebral aneurysm: mortality and recurrence of subarachnoid hemorrhage. Neurology. 1997;48(5):1191– 1195. 5. Sonobe M, Yamazaki T, Yonekura M, Kikuchi H. Small unruptured intracranial aneurysm verification study: SUAVe study, Japan. Stroke. 2010;41(9):1969–1977. 6. UCAS Japan Investigators, Morita A, Kirino T, et al. The natural course of unruptured cerebral aneurysms in a Japanese cohort. N Engl J Med. 2012;366(26):2474–2482. 7. Wiebers DO, Whisnant JP, Huston 3rd J, et al. Unruptured intracranial aneurysms: natural history, clinical outcome, and risks of surgical and endovascular treatment. Lancet. 2003;362(9378):103–110.

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Worst Headache Of Life Jennifer E. Kim, MD n Risheng Xu, MD/PhD

Consult Page 65F with worst headache of life, nausea, vomiting, confusion. CT + subarachnoid hemorrhage

Initial Imaging

A

B

C

Figure 27.1 An axial head CT without contrast shows diffuse, thick subarachnoid hemorrhage with blood in the lateral and third ventricles (A) the basal cisterns and left greater than right Sylvian fissures (B) and the fourth ventricle (C) There is early hydrocephalus with prominent temporal horns, third ventricles, and lateral ventricles.

Walking Thoughts n n

n n n n

n

What is the current GCS of the patient? Is she able to protect her airway? Is the patient on any anticoagulant or antiplatelet medications? If so, have reversal agents been given? Are coagulation labs available or pending? What medical comorbidities does the patient have? What is the age and baseline function of the patient? Does this patient need acute cerebrospinal fluid (CSF) diversion with an external ventricular drain (EVD)? Does the patient have a CT angiogram (CTA) available? Does the patient need a cerebral angiogram?

History of Present Illness A 65 year old female with a history of hypothyroidism and hypertension presents to the emergency department (ED) with thunderclap headache. Per family, the patient was resting at home 135

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B

Figure 27.2 Coronal (A) and sagittal (B) CTA head with contrast shows a 4 mm posteroinferiorly-projecting posterior communicating artery aneurysm (arrows).

when she suddenly developed an excruciating, worst headache of life with unrelenting nausea and vomiting. She became progressively lethargic and confused. The family called emergency medical services, and the patient was brought to the ED. The patient’s son denies any history of anticoagulant or antiplatelet medications, loss of consciousness, or seizure-like activity. A head CT without contrast shows thick, diffuse subarachnoid hemorrhage (SAH) in the basal cisterns extending into the left greater than right sylvian fissures, along with intraventricular hemorrhage (IVH) without casted ventricles. There is mild hydrocephalus with dilated temporal horns but without transependymal edema. CTA of the head and neck demonstrates a 4 mm posteroinferiorly-projecting, saccular posterior communicating artery aneurysm (Figure 27.2). Of note, the patient does have a 50 pack-year smoking history but does not have a familial history of cerebral aneurysms.

Vital Signs T 36.7oC, HR 96, BP 175/69, SpO2 92% on room air

Pertinent Labs Na 140, Hgb 13.3, Plt 292, INR 1.0, PT 10.3, aPTT 0.9

Glasgow Coma Scale Motor: 6 Verbal: 4 Eye opening: 3 GCS total: 13

Physical Exam Sleepy Eyes closed but opens to voice Oriented to self only No ptosis Pupils 3 mm equal, round, and reactive to light

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Extraocular movements intact Face symmetric, tongue midline No pronator drift Moving all extremities to command, symmetrically and with good strength

Triage Management The patient has a ruptured intracranial aneurysm and needs admission to the neurocritical care unit. She is lethargic but able to follow commands; her Hunt and Hess grade is 3 with a mortality rate of 37% (Table 27.1). On head CT, the patient has diffuse SAH with IVH; her modified Fisher grade is 4 which corresponds to a 40% chance of developing vasospasm (Table 27.2). There are signs of developing hydrocephalus but the patient is still able to follow commands. Given that she has a neurological exam that may be trended over time, the patient does not yet require an EVD. Nonetheless, she will need close monitoring for acute hydrocephalus and/or increased intracranial pressure (ICP), at which time an EVD would be warranted. Her blood pressure is elevated and will be lowered to a systolic blood pressure (SBP) of 85 mmHg, antibiotics Postoperative admission to the neurocritical care unit Early mobilization and physical therapy evaluation

B

Fig. 37.2 Sagittal (A) and axial (B) views of a thoracic spine CT without contrast demonstrate significant disc calcification without ossified posterior longitudinal ligament [OPLL] or ossified ligamentum flavum [OLF].

37—Leg WeAkNess ANd NumbNess

187

LEARNING POINTS

• Compression of the thoracic spinal cord can be due to degenerative disc disease, spondylotic disease, trauma, infection, or tumor.

• Clinical presentation depends on the location and degree of compression. Signs and symptoms of thoracic myelopathy include gait disturbance, balance difficulties, abnormal sensation, weakness, hyperreflexia, clonus, increased tone, and bowel, bladder, or sexual dysfunction. Signs and symptoms of thoracic radiculopathy include pain (classically in a band-like distribution) that can be misinterpreted as cardiac pain, costochondritis, pleural pain, or gastrointestinal difficulties.4 • Thoracic spinal stenosis is rare, estimated to be less than 1% of all spinal stenosis cases.2 • Average age of presentation is in the 50s and is more common in men and people of East Asian descent.2 • Ossified posterior longitudinal ligament (OPLL) and ossified ligamentum flavum (OLF) are two of the most common causes.2 OPLL tends to occur in the mid- to upper thoracic spine while OLF tends to occur in the lower thoracic spine.2 • Thoracic disc herniations account for less than 1% of all symptomatic disc herniations. This rarity is thought to be due to the rigidity of the thoracic spine compared to the more mobile cervical and lumbar spine.3 • The majority of thoracic disc herniations are midline in location and occur below T8.3 • Thoracic disc herniations are more likely to be calcified, occurring in up to 70% of cases.3 Once calcified, they can become adherent to the dura, and can also be associated with an intradural component.3 • In a review of 15 studies with a total of 1,036 patients undergoing surgery for thoracic disc herniation, mortality was minimal, but morbidity was as high as 29% with medical, surgical site, cerebrospinal fluid-related, and neurological complications.1 Posterolateral approaches had a lower risk for medical and surgical complications as compared to anterior and lateral approaches.1 • CT of the spine is used to assess for disc calcification, OPLL, or OLF, as well as instrumentation planning as needed.

References 1. Brotis AG, Tasiou A, Paterakis K, Tzerefos C, Fountas KN. Complications associated with surgery for thoracic disc herniation: a systematic review and network meta-analysis. World Neurosurg. 2019;132:334–342. 2. Buchanan IA, Wang JC, Hsieh PC. Thoracic spinal stenosis. In: Baaj A, Kakaria U, Kim H, eds. Surgery of the Thoracic Spine: Principles and Techniques. Thieme. 1st ed. 2019. https://doi.org/10.1055/b-006-163751. 3. Gadhi, S. V., Januszewski, J., & Uribe, J. S. Midline disc herniations of the thoracic spine. In: Baaj A, Kakaria U, Kim H, ed. Surgery of the Thoracic Spine: Principles and Techniques. Thieme. 1st ed. https://doi. org/10.1055/b-006-163751. 4. Shirzadi A, Drazin D, Jeswani S, Lovely L, Liu J. Atypical presentation of thoracic disc herniation: case series and review of the literature. Case Rep Orthop. 2013:621476. 2013.

C H A P T E R

38

Leg Pain, Weakness, And Saddle Anesthesia Ann Liu, MD

n Yike Jin, MD

Consult Page 31F pregnant with leg weakness and pain. MRI with severe compression.

Initial Imaging

A

B

Fig. 38.1 Sagittal (A) and axial (B) T2-weighted MRI of the lumbar spine without contrast demonstrates a large L4-L5 disc herniation (dotted line) with severe compression and complete effacement of the thecal sac.

Walking Thoughts What is the patient’s neurological exam? What symptoms does the patient have? What is the timeline of her symptoms? n Does the patient have any history of recent trauma? n Is there concern for cauda equina syndrome? n What is the patient’s postvoid residual or bladder scan? n Does the patient need urgent or emergent surgery? n n

188

189

a n n

n n

What is the patient’s gestational age and pregnancy history? What comorbidities or medical conditions would preclude surgery or need to be optimized before surgery? Does the patient need any additional imaging? Is the patient on any anticoagulant or antiplatelet medication?

History of Present Illness A 31 year old female, who is 22 weeks gestation with a history of obesity and chronic low back pain, presents to the emergency department with right leg pain and weakness. She has a history of chronic low back pain for many years that has been managed with pain injections and physical therapy. Five weeks ago, she had a fall and began having pain and numbness down the back of her right leg. The pain has progressively worsened to the point where she began limping one week ago; however, earlier today, her leg pain was so severe that she was unable to walk. She denies any bowel or bladder dysfunction, but on further questioning, she has noticed some numbness around her genitals, which she thought was due to her pregnancy. This is her third pregnancy, and thus far, it has been uneventful. She is not on any anticoagulant or antiplatelet medications.

Vital Signs T 36.8°C, BP 138/56, HR 89, SpO2 94% on room air

Pertinent Labs Hgb 11.3, WBC 8.3, Plt 262, INR 1.0, aPTT 24.8

Physical Exam Alert, oriented to self, place, and year Distressed, in significant pain Cranial nerves intact Bilateral upper extremities 5/5 strength Hip Flexion

Knee Extension

Knee Flexion

Dorsiflexion

Plantar Flexion

Toe Extension

RLE

3/5

3/5

3/5

3/5

3/5

3/5

LLE

4/5

4/5

4/5

4/5

4/5

4/5

*Lower extremity strength exam was effort-dependent due to pain

Pain and numbness in a right S1 dermatomal distribution Perianal and perineal numbness No Hoffman’s sign No clonus Reflexes 1+ throughout Rectal tone present Postvoid residual: 494 ccs

Triage Management The patient presents with signs and symptoms concerning for cauda equina syndrome with imaging demonstrating complete effacement of the thecal sac at L4-L5 secondary to a disc herniation. She will need emergent surgery for decompression. The goal of surgery will be to preserve neurological

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function, and it is unclear what deficits will remain after surgery. The risks and benefits must be emphasized when discussing surgery with the patient. Given her urinary retention, a foley will be placed to decompress her bladder. Because this is a neurosurgical emergency, her pregnancy does not preclude surgery. The obstetrics team will be consulted urgently to help evaluate the patient, her fetus, and manage her pregnancy perioperatively. This is an emergent and operative consult.

Assessment A 31 year old female at 22 weeks gestation with obesity and chronic low back pain presents with right leg pain and numbness, bilateral leg weakness, saddle anesthesia, and urinary retention concerning for cauda equina syndrome. She will need emergent surgery for decompression.

Plan n

n

Consult to the obstetrics team to discuss intraoperative and perioperative precautions and monitoring of the fetus Emergent operative planning for L4-L5 laminectomy and discectomy n Posting highlights: n Jackson table n Consider intraoperative neuromonitoring n High speed drill and/or ultrasonic bone scalpel n Anesthesia: discussion with obstetrics anesthesia team and perioperative antibiotics

LEARNING POINTS

• Cauda equina syndrome (CES) is a serious condition caused by compression of the cauda equina nerve roots resulting in motor, sensory, bowel, bladder, and or/sexual dysfunction, which can be permanent if untreated. It is a neurosurgical emergency, and the treatment is surgical decompression. • Despite CES being an emergency, it is relatively rare. In a systematic review of 26 studies, 0.27% of patients presenting with low back pain had a final diagnosis of radiological and clinical CES. In patients with signs and symptoms concerning for CES, 18.9% had a final diagnosis of radiological and clinical CES.3 • The diagnosis of CES can be difficult due to the nonspecificity and low predictive value of clinical signs and symptoms.4,8 Due to the potential permanent neurological deficits that can occur with CES, evaluation and work-up must be expeditious and depends on careful consideration of history, physical exam, and imaging. • It is specifically important to assess the nature, severity, and duration of each symptom and attempt to distinguish between the different types of urinary incontinence and bowel incontinence. • With regards to urinary incontinence, it is important to differentiate between incontinence from CES (which typically presents with overflow due to urinary retention) and other nonCES causes: • Urge incontinence: the patient feels the urge to urinate, but does not always make it to the bathroom in time • Stress incontinence: the patient leaks a small amount of urine with increased intraabdominal pressure/straining (e.g. laughing or coughing) • Urinary urgency and frequency can be seen in more common situations such as urinary tract infection. • In terms of bowel incontinence, it is important to differentiate between true incontinence (the inability to feel when one needs to have a bowel movement with spontaneous stooling) and diarrhea secondary to another etiology. • Common non-CES causes of urinary retention include medications (e.g. opioids), urinary tract infection, and even pain itself.

a

191

• Important physical exam maneuvers include the assessment of saddle sensation and rectal tone, as well as measurement of a postvoid residual or bladder volume if the patient is unable to urinate. The lack of rectal tone, saddle anesthesia, and a high postvoid residual or bladder volume are all red flag signs for CES but must be personally confirmed. • In adults, a normal PVR is generally considered to be less than 50 mL. In the elderly, between 50 and 100 mL is considered normal.1,9 • The threshold for an abnormal postvoid residual is poorly defined. Recent studies have suggested that a PVR of greater than 200 mL greatly increases the suspicion of CES.4,9 • In some cases where a foley catheter is placed before a postvoid residual can be obtained, one useful test is gentle pulling of an inflated foley while the patient is unaware. This assesses for trigone sensitivity and should produce the urge to micturate. • Initial imaging evaluation consists of an MRI of the lumbar spine without contrast. If a patient has preexisting spinal hardware, MRI with a hardware suppression protocol or a CT myelogram can be obtained. • Because of the wide heterogeneity of patients presenting with CES, several recent studies have attempted to define subcategories of CES including:5-7 • CES incomplete (CESI): the patient has objective evidence of CES (typically with both altered sensation and bladder function) but retains voluntary control of micturition. • CES retention (CESR): the patient has neurogenic retention of urine with a paralyzed, insensate bladder, and ultimately overflow urinary incontinence. • Several studies have shown that patients with CESR have worse outcomes than those with CESI. • Surgical intervention consists of urgent lumbar decompression to maximize the chance of functional recovery. Family members and patients are counseled that the goal of surgery is to prevent further or permanent neurological deficit, but there is no guarantee of functional improvement. • The timing of decompressive surgery is controversial and there is no strong evidence in the literature to support a specific time cutoff for surgery. Overall, the earlier the intervention, the greater the chance for preventing neurological decline while maximizing possible recovery.2



References 1. Ballstaedt L, Woodbury B. Bladder Post Void Residual Volume. [Updated 2020 Aug 27]. in: StatPearls [Internet]. Treasure Island (FL). StatPearls Publishing; 2020 Jan; 2020. Available from: https://www.ncbi. nlm.nih.gov/books/NBK539839/. 2. Chau AM, Xu LL, Pelzer NR, Gragnaniello C. Timing of surgical intervention in cauda equina syndrome: a systematic critical review. World Neurosurgery. 2014;81(3–4):640–650. 3. Hoeritzauer I, Wood M, Copley PC, Demetriades AK, Woodfield J. What is the incidence of cauda equina syndrome? A systematic review [published online ahead of print, 2020 Feb 14]. J Neurosurg Spine. 2020:1–10. 4. Katzouraki G, Zubairi AJ, Hershkovich O, Grevitt MP. A prospective study of the role of bladder scanning and post-void residual volume measurement in improving diagnostic accuracy of cauda equina syndrome. Bone Joint J. 2020;102-B(6):677–682. 5. Sun JC, Xu T, Chen KF, et al. Assessment of cauda equina syndrome progression pattern to improve diagnosis. Spine (Phila Pa 1976). 2014;39(7):596–602. 6. Todd NV, Dickson RA. Standards of care in cauda equina syndrome. Br J Neurosurg. 2016;30(5):518– 522. 7. Todd NV. Early cauda equina syndrome (CESE). Br J Neurosurg. 2017;31(4):400. 8. Todd, N. V. Guidelines for cauda equina syndrome. Red flags and white flags. Systematic review and implications for triage. Br J Neurosurg. 31(3):336–339. 9. Venkatesan M, Nasto L, Tsegaye M, Grevitt M. Bladder scans and postvoid residual volume measurement improve diagnostic accuracy of cauda equina syndrome. Spine (Phila Pa 1976). 2019;44(18):1303–1308.

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39

Right Leg Pain And Weakness With Prior Lumbar Fusion Yike Jin, MD

Consult Page 73M with history of prior lumbar surgery with right leg pain and thigh weakness. MRI shows synovial cyst. Please evaluate.

Initial Imaging

A

B

Fig. 39.1 Sagittal (A) and axial (B) views of a T2-weighted MRI of the lumbar spine demonstrate bilateral facet hypertrophy contributing to severe L3-L4 canal stenosis and a right-sided synovial cyst (arrow) at this level with associated lateral recess and foraminal stenosis. There is artifact seen from the patient’s prior L4-L5 fusion hardware. These imaging findings are consistent with adjacent segment degeneration.

Walking Thoughts n n

n n

n n n

192

What is the patient’s neurological exam? What other symptoms does the patient have (e.g. mechanical back pain, bowel or bladder symptoms, saddle anesthesia)? How long have the symptoms been bothering him? What was the patient’s prior lumbar surgery? When was the surgery? What were the patient’s preoperative symptoms, and did they resolve after surgery? Are his current symptoms similar to or different from those that he had before surgery? What nonoperative measures, if any, has he tried? What are the patient’s medical comorbidities?

193

39—Right Leg PAin And WeAkness With PRioR LumbAR Fusion

History of Present Illness A 73 year old male with hypertension, hyperlipidemia, coronary artery disease with cardiac bypass surgery, prostatectomy for prostate cancer, and prior L4-L5 decompression and fusion presents to the emergency department (ED) with 6 months of right knee and leg pain and acute worsening in the past few days. About 5 years ago, the patient began having difficulty walking due to left foot weakness. This slowly progressed for about a year before he ultimately underwent an L4-L5 decompression and fusion at an outside hospital. Postoperatively, he had worsening function of his legs bilaterally, with a complete left foot drop and a partial right foot drop. He underwent many weeks of intensive physical therapy after his surgery with no improvement in his function. In the past several months, he started having excruciating right-sided back pain that radiates to his right knee down to his ankle. At times it seems to radiate from his ankle back up to his right thigh. The most severe pain is the radiating pain from his lower back to his right knee. This pain is sharp and stabbing; he rates it 9 out of 10. He also has some low back pain that is less bothersome. His leg and back pain worsen with standing and walking; the pain is alleviated when lying down. He has not had pain like this before. He also reports some increased difficulty with gait but denies bowel or bladder symptoms. He denies any numbness, tingling, or saddle anesthesia. He denies any trauma or other inciting event. He underwent a right L3-L4 epidural steroid injection approximately 3 weeks ago which gave him some temporary benefit for 1 week, but the pain has now returned and worsened in the past few days. He also previously tried physical therapy with minimal benefit. He does not have any recent imaging of his lumbar spine and has not seen his original surgeon. He takes aspirin 81 mg daily for his coronary artery disease but does not take any other antiplatelet or anticoagulant medications.

Vital Signs T 36.5°C, HR 66, BP 145/68, SpO2 96% on room air

Pertinent Labs Hgb 14.7, WBC 4.2, Plts 112

Physical Exam Alert and oriented to person, place, and year Bilateral upper extremities 5/5 strength throughout Hip Flexion

Knee Extension

Knee Flexion

Dorsiflexion

Plantar Flexion

Toe Extension

RLE

5/5

4/5

5/5

4+/5*

4+/5*

4+/5*

LLE

5/5

5/5

5/5

5/5

4/5*

4/5*

*Patient reports baseline weakness after his first surgery

Sensation intact to light touch No Hoffman’s sign No clonus

194

SECTION IV—SPINE

Reflexes 2+ throughout No saddle anesthesia Rectal tone present

Triage Management The patient is presenting with several months of back and right leg pain without any inciting event. On imaging, he has a right sided L3-L4 synovial cyst, causing foraminal and lateral recess stenosis. One of the common long-term risks of having an instrumented fixation or fusion procedure is increased stress on the facet joints of the adjacent levels. In this specific case, our patient’s prior L4-L5 fusion has placed increased stress on the L3-L4 facet joints as well as the L5-S1 joints. This increased stress can lead to accelerated degeneration with facet or ligament hypertrophy or, as in this case, synovial cyst formation. Patients with spine pathologies can present with a multitude of symptoms (e.g. leg pain, back pain, weakness, sensory deficits), and it is important to determine the chronicity and severity of each symptom. It is also imperative to evaluate if the patient’s presentation correlates with imaging findings. Although our patient reports both back and leg pain, his most debilitating symptom is radicular right leg pain radiating to his right knee. On examination, he also has new right quadriceps weakness. His pain and weakness are consistent with an L3 or L4 radiculopathy and is likely being caused by the right-sided L3-L4 synovial cyst seen on imaging. The patient has already tried nonoperative measures with pain injections and physical therapy without improvement. Because he continues to have worsening symptoms, he is a candidate for surgical decompression. Prior to surgery, he will undergo flexion-extension x-rays of the lumbar spine to better assess for dynamic instability at L3-L4, which may indicate the need for fusion in addition to decompression. Because our patient’s symptoms have been progressive over months, if his pain can be controlled in the ED, he can be discharged, and surgery can be scheduled on a nonemergent basis. Despite a trial of pain medication in the ED, his pain is uncontrolled and the patient is interested in expedited surgical treatment. He will be admitted to the floor and evaluated by the medicine team for preoperative optimization. This is a nonemergent, operative consult.

Assessment This is a 73 year old male with a previous L4-L5 decompression and fusion with residual foot weakness who presents with 6 months of right knee and leg pain and new right quadriceps weakness. MRI of his lumbar spine demonstrates adjacent segment degeneration with a right sided L3-L4 synovial cyst causing lateral recess and foraminal stenosis. Given that the patient has tried and failed nonoperative management, he will undergo surgical decompression with possible fusion.

Plan n n n

n n

Admit to the neurosurgical floor XR flexion-extension films of the lumbar spine to assess for dynamic instability Consult to medicine for preoperative risk stratification given his prior coronary artery disease and cardiac bypass surgery Hold aspirin 81 mg Operative planning for a posterior L3-L4 decompression with synovial cyst resection with possible extension of fusion to L3

39—Right Leg PAin And WeAkness With PRioR LumbAR Fusion n

195

Posting highlights: Jackson table n Intraoperative neuromonitoring: SSEP/EMG n High-speed drill n Be mindful of increased risk of dural violation in repeat spine surgeries n Possible spinal fusion instrumentation n

LEARNING POINTS

• Adjacent segment pathology is a concern after spinal fusion surgery due to reduced mobility at the operated segment. The term now encompasses two concepts: adjacent segment degeneration and adjacent segment disease. Adjacent segment degeneration refers to radiographic changes at levels adjacent to a fusion construct, while adjacent segment disease refers to clinical symptoms from the radiographic findings.1 • It is still unclear whether adjacent segment pathology occurs due to increased stress after fusion procedures or as a natural progression of spondylosis. • In an updated meta-analysis, the overall incidence of adjacent segment degeneration is 27.8% as compared to 7.6% for adjacent segment disease. The reoperation rate for adjacent segment pathology in this meta-analysis was 4.6%.1 • Increased stress on adjacent joints can manifest as worsening joint degeneration, facet hypertrophy, ligamentous hypertrophy, or synovial cyst formation. • Synovial cysts are benign extradural fluid-filled sacs that form from the facet joint capsule. The pathogenesis is unclear, but they are thought to be markers of spinal instability because of their disruption of the facet joints.2 A recent meta-analysis demonstrated that synovial cysts are likely to present with facet joint osteoarthritis, with a pooled prevalence rate of 89.3%.2 • Nonoperative measures for adjacent segment pathology includes anti-inflammatory medications, physical therapy, pain injections, and pain management. For synovial cysts, CT-guided cyst aspiration can also be trialed. If nonoperative management fails and/or the patient’s symptoms worsen, surgical • intervention may be warranted. Surgical management involves either a decompression alone or decompression with fusion.

References 1.

Donnally 3rd CJ, Patel PD, Canseco JA, et al. Current incidence of adjacent segment pathology following lumbar fusion versus motion-preserving procedures: a systematic review and meta-analysis of recent projections. Spine J. 2020;20(10): 1554–156. 2. Ramhmdani S, Ishida W, Perdomo-Pantoja A, Witham TF, Lo SL, Bydon A. Synovial cyst as a marker for lumbar instability: a systematic review and meta-analysis. World Neurosurg. 2019;122:e1059– e1068.

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40

Arm And Leg Weakness And Numbness After MVC Ann Liu, MD

n Yike Jin, MD

Consult Page 55M hx of C4-6 fusion presenting with loss of sensation and weakness in right arm after MVC

Initial Imaging

A

B

Fig. 40.1 A. Sagittal cervical spine CT without contrast demonstrates the patient’s known anterior fusion from C4 to C6 as well as sclerotic endplate changes at C3-C4. There is also grade I retrolisthesis of C3 on C4. B. Axial view at C3-C4 demonstrates severe stenosis at this level. There are no obvious fractures.

A

B

Fig. 40.2 Sagittal (A) and axial (B) STIR sequences of a cervical spine MRI without contrast demonstrates severe C3-C4 stenosis with increased STIR signal change of the spinal cord at this level. There is no obvious ligamentous injury or disruption.

196

197

40—ArM ANd Leg WeAkNess ANd NuMbNess After MVC

Walking Thoughts n n

n

n n n

What are the patient’s current ABCs? Has the trauma team evaluated him yet? What is the severity of the patient’s motor and sensation deficits? Is he weak in the right arm only or also in other extremities? What is the patient’s prior surgical history? When was the surgery and why did he have surgery? Does the patient have any other injuries? What is his other medical history? Is he taking any antiplatelet or anticoagulant medications?

History of Present Illness A 55 year old male with atrial fibrillation presents to the emergency department after a motor vehicle crash (MVC) with severe neck pain, and bilateral arm and leg weakness and paresthesias. Twenty-five years prior, he was in an MVC and underwent a C4-C6 anterior cervical discectomy and fusion. After this initial fusion, he reports he had 90% strength in his left arm but otherwise had no deficits. He has an MRI of his cervical spine from five years prior that demonstrates known adjacent level disease with severe cervical stenosis at C3-C4. This morning, he was rear-ended and his head hit the steering wheel. He currently reports severe neck pain, “a thousand pins” in his arms and legs, and patchy numbness throughout. He also has difficulty moving his arms and legs. He is taking aspirin 81 mg daily, last taken this morning. He was previously on rivaroxaban for his atrial fibrillation but has not taken it for 6 months after undergoing a cardiac ablation. In the emergency department, he underwent an urgent CT scan of his cervical spine demonstrating no acute fractures but retrolisthesis of C3 on C4 above his prior fusion causing severe stenosis. Subsequent MRI of his cervical spine demonstrates severe stenosis at C3-C4 with cord signal change. On trauma evaluation, he has no other injuries.

Vital Signs T 36.6°C, HR 61, BP 115/76, SpO2 97% on room air

Pertinent Labs None

Physical Exam ASIA C Alert, awake, oriented to self, place, and year Hard cervical collar in place, endorsing extreme neck pain Deltoid

Biceps

Triceps

Wrist Flexion

Wrist Extension

Grip

RUE

4+/5

5/5

1/5

2/5

1/5

1/5

LUE

4+/5

5/5

1/5

2/5

1/5

1/5

Hip Flexion

Knee Extension

Knee Flexion

Dorsiflexion

Plantar Flexion

RLE

2/5

1/5

1/5

3/5

3/5

LLE

2/5

1/5

1/5

3/5

3/5

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SECTION IV—SPINE

Sensation to light touch decreased in hands and legs bilaterally No clonus, no Hoffman’s sign No hyperreflexia Rectal tone intact

Triage Management Given the mechanism of injury, a full trauma assessment must be completed. Currently, the patient is hemodynamically stable with a mean arterial pressure (MAP) of 89, and he is breathing well on room air. The patient’s significant weakness, paresthesias, and imaging are concerning for central cord syndrome, which likely occurred from a hyperextension injury during his MVC in combination with his known cervical stenosis at C3-C4. With any cervical spine injury, immobilization with a hard cervical collar is essential. Good spinal cord perfusion is ensured with close blood pressure monitoring and an elevated MAP goal. He will need surgery for decompression and stabilization. In the interim, he will be admitted to the intensive care unit to maintain an elevated MAP. This is an urgent and operative consult.

Assessment This is a 55 year old male with a prior C4-C6 anterior cervical discectomy and fusion who presents to the ED after a motor vehicle crash with severe neck pain, paresthesias, and weakness in all four extremities. CT of his cervical spine demonstrates C3-C4 adjacent level disease and MRI demonstrates severe stenosis at this level with cord signal change concerning for central cord syndrome.

Plan Full trauma evaluation (if not already obtained) Admit to the intensive care unit with hourly neurological checks n Hard cervical collar on at all times n Admission labs: CBC, BMP, coagulation panel, type and screen, reserve 2 units of blood and 1 unit of platelets for surgery n Arterial line and foley placement n MAP >85 mmHg for spinal cord perfusion, vasopressor medications as needed n Consult to cardiology for preoperative risk assessment given his history of atrial fibrillation n No corticosteroids n NPO with IV fluids n Operative planning for posterior cervical decompression and fusion n Posting highlights: n Skull clamp n Intraoperative neuromonitoring: MEP/SSEP/EMG n Consider preflip MEP/SSEP n Fluoroscopy, consider intraoperative CT n High-speed drill n Spinal fusion instrumentation n Bone allograft n For anesthesia: mean arterial pressure >85 mmHg for spinal cord perfusion (particularly during induction), antibiotics n Postoperative care in the intensive care unit n Early physical therapy and occupational therapy evaluation with mobilization and splinting as needed n n

40—ArM ANd Leg WeAkNess ANd NuMbNess After MVC

199

LEARNING POINTS

• Central cord syndrome is an incomplete spinal cord injury and is a clinical diagnosis. Classically, motor symptoms affect the arms (particularly the hands) more so than the legs, and sensory deficits can occur in a “cape-like” distribution across the back and arms. The nature and severity of presenting symptoms are varied based on the extent of injury. Predisposing factors include cervical stenosis or the presence of underlying osteophytes and/or spondylosis. • Central cord syndrome classically occurs from hyperextension injuries in combination with spinal stenosis. However, central cord syndrome can also result from fracture-dislocation injuries, acute disc herniations, and rarely without radiographic abnormality.2 • Initial trauma evaluation is important: securing airway, maintaining hemodynamic stability, and evaluation of other injuries. Up to 30% of patients with cervical spine injuries have noncontiguous injuries elsewhere in the spine.2 • The prognosis of neurological recovery is quite variable due to the heterogenous patient population. Recovery of function is thought to start first in the lower extremities, followed by return of bladder function, and finally return in arm function. Hand and finger function improve last, if at all.2 • In patients with central cord syndrome from stenosis without bony injury, there is a variable degree of spontaneous recovery of neurological function without intervention. This has limited the study of surgical versus medical management treatments for central cord syndrome, and the role of decompressive surgery in this patient population remains controversial.2 • The 2013 Congress of Neurological Surgeons (CNS) Trauma Guidelines with Level 3 recommendations2 for acute traumatic central cord syndrome are: • Intensive care is recommended, particularly for patients with severe neurological deficits. • Maintain a MAP of 85–90 mmHg for the first week after injury. • Early reduction of fracture-dislocation injuries is recommended. • Surgical decompression, particularly if there is focal and anterior compression, is recommended. • The timing of surgery for central cord syndrome is controversial, but it is generally preferable to operate during the first hospital admission.3 The Surgical Timing in Acute Spinal Cord Injury Study in 2012 was a prospective, multicenter cohort study evaluating the timing of surgery.6 It involved six institutions in adults with cervical spine trauma (not exclusive to central cord syndrome). Early decompression occurred within 24 hours while delayed decompression occurred beyond 24 hours. The study concluded that decompression within 24 hours of injury is associated with improved neurological outcome. This was defined as an increase of at least 2 grades on the ASIA Impairment scale at 6 months follow-up. Although there was improved neurological outcome with early decompression, there was no difference in complication rate or mortality between early and delayed decompression.6 • The use of steroids for central cord syndrome is controversial. While some studies4,5 have found that administration of methylprednisolone within 8 hours of spinal cord injury has efficacy in improving neurological function, the 2013 CNS Trauma Guidelines7 do not recommend the use of methylprednisolone. There is no Class I or Class II medical evidence supporting its clinical benefit; however, Class I, II, and III evidence exists that high-dose steroids have significant morbidity and mortality. • Predictors of neurological and functional outcome after acute traumatic central cord syndrome due to spinal stenosis include admission ASIA motor score, midsagittal diameter of the spinal cord at the point of maximal compression, maximum canal compromise, length of parenchymal damage on T2-weighted MRI, and age.1

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References 1. Aarabi B, Alexander M, Mirvis SE, et al. Predictors of outcome in acute traumatic central cord syndrome due to spinal stenosis. J Neurosurg Spine. 2011;14(1):122–130. 2. Aarabi B, Hadley MN, Dhall SS, et al. Management of acute traumatic central cord syndrome (ATCCS). Neurosurgery. 2013;72(suppl 2):195–204. 3. Anderson KK, Tetreault L, Shamji MF, et al. Optimal timing of surgical decompression for acute traumatic central cord syndrome: a systematic review of the literature. Neurosurgery. 2015;77(suppl 4):S15–S32. 4. Bracken MB. Steroids for acute spinal cord injury. Cochrane Database Syst Rev. 2012;1(1):CD001046. Published 2012 Jan 18. 5. Bracken MB, Shepard MJ, Collins Jr WF, et al. Methylprednisolone or naloxone treatment after acute spinal cord injury: 1-year follow-up data. Results of the second National Acute Spinal Cord Injury Study. J Neurosurg. 1992;76(1):23–31. 6. Fehlings MG, Vaccaro A, Wilson JR, et al. Early versus delayed decompression for traumatic cervical spinal cord injury: results of the Surgical Timing in Acute Spinal Cord Injury Study (STASCIS). PLoS One. 2012;7(2):e32037. 7. Hurlbert RJ, Hadley MN, Walters BC, et al. Pharmacological therapy for acute spinal cord injury. Neurosurgery. 2013;72(suppl 2):93–105.

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41

Worsening Back Pain And Leg Weakness James Feghali, MD n

Daniel Lubelski, MD n Yike Jin, MD

Consult Page 73M PMH diastolic heart failure, here with back pain, LE weakness, and recurrent falls

Initial Imaging

A

B

Fig. 41.1 A. Sagittal T2-weighted MRI of the thoracolumbar spine without contrast demonstrates a round, well-circumscribed, hypointense intradural mass at T11-T12 with a broad base along the dural margin (arrow). B. An axial sequence demonstrates the intradural mass within the right lateral spinal canal (arrow) causing moderate narrowing of the spinal canal with leftward displacement and compression of distal thoracic cord/ conus medullaris. There is minimal contrast enhancement of the mass (not pictured here), which can be seen with dense calcification.

Walking Thoughts n n

n n n

What is the patient’s neurological exam? What other symptoms is he having? Are there any red-flag symptoms or concern for cauda equina syndrome? What is the timeline of his symptoms (e.g. acute, chronic, progressive)? Do his symptoms correlate with the imaging findings? Does the patient have a cancer history? 201

202 n

n

SECTION IV—SPINE

What are the patient’s medical comorbidities? Is he on any anticoagulant or antiplatelet medications? Does this patient need surgery and if so, when?

History of Present Illness A 73 year old male with obstructive sleep apnea, chronic obstructive pulmonary disease, morbid obesity, colon cancer with a colectomy 30 years prior, and chronic diastolic heart failure presents to the emergency department with 2 days of uncontrollable back pain. Six months ago, he began having some lower back pain without any trauma or inciting event. Since then, his pain has progressively worsened, and he developed lower extremity weakness with recurrent falls. For the past 3 years, he has been using a cane due to gait instability, but he now requires a walker due to weakness. He also notes some new bowel and bladder incontinence. He denies any numbness of his legs, radicular pain, or saddle anesthesia. He previously smoked a pack of cigarettes per day but quit 40 years prior. He is on aspirin 81 mg daily but not on any other anticoagulant medications.

Vital Signs T 35.7°C, HR 63, BP 144/67, SpO2 95% on room air

Pertinent Labs Pending

Physical Exam Alert, oriented to person, place, and year Cranial nerves intact Deltoid

Biceps

Triceps

Wrist Flexion

Wrist Extension

Grip

RUE

5/5

5/5

5/5

5/5

5/5

5/5

LUE

5/5

5/5

5/5

5/5

5/5

5/5

Hip Flexion

 

 

 

 

RLE

4+/5

4+/5

4+/5

4+/5

4+/5

LLE

4+/5

4+/5

4+/5

4+/5

4+/5

Sensation intact to light touch throughout No Hoffman’s sign Significantly increased tone in bilateral lower extremities 3+ patellar reflexes 3 beats of clonus in the right ankle and 1 beat of clonus in the left ankle Upgoing toe on the right; downgoing toe on the left No saddle anesthesia Rectal tone intact No tenderness on palpation or percussion over the spine Bladder scan: 600 ccs

41—Worsening BAck PAin And Leg WeAkness

203

Triage Management The patient is presenting with back pain, bilateral leg weakness, falls, and is found to have long-tract signs on examination with hyperreflexia, increased tone, and clonus. This constellation of signs and symptoms are consistent with thoracic myelopathy secondary to the newly identified, compressive T11-T12 intradural, extramedullary mass seen on MRI, suggestive of a meningioma. The marked T1- and T2- hypointensity with minimal contrast enhancement correspond to dense calcification of the mass. Due to his progressive symptoms, the patient will be admitted to the neurosurgical floor for possible surgical planning. Because the patient presents with worsening pain but his symptoms have been largely stable for several months without an acute decline, he does not need emergent surgery. He would best benefit from medical optimization first given his significant medical comorbidities, and the medicine team will be consulted for preoperative risk assessment. His aspirin will be held. He also appears to have urinary retention, so a urinary catheter will be placed. While admitted, physical therapy and fall precautions will be important to prevent falls. If cleared for surgery, the main goal of surgery is to prevent further neurological decline. It is difficult to predict how much function he will regain after surgery, and surgical risks are considerable and high given his medical comorbidities. This should be emphasized when discussing the risks and benefits of surgery with the patient. This is a nonemergent and possibly operative consult.

Assessment This is a 73 year old male with multiple medical comorbidities on aspirin 81 mg daily who presents with acutely worsening back pain in the setting of 6 months of lower back pain, lower extremity weakness, and gait instability. Imaging reveals a T11-T12 compressive intradural mass suggestive of a meningioma. Given his complex medical history, he will undergo preoperative optimization and risk assessment prior to deciding upon surgery.

Plan n n n n n n n

n n

Admission to the neurosurgical floor Consult to medicine for preoperative risk assessment Placement of foley catheter Oral pain medications Fall precautions Consider CT to evaluate for calcification within the mass (Figure 41.2) If cleared by the medicine team and the patient wishes to proceed with surgery, operative planning for a T12 laminoplasty for intradural tumor resection n Posting highlights: n Jackson table n Intraoperative fluoroscopy for localization n Intraoperative neuromonitoring: MEP/SSEP/EMG n High-speed drill and/or ultrasonic bone scalpel n Microinstruments n Microscope n Ultrasonic aspirator n Bipolar cautery n Intraoperative ultrasound n Plan for dural closure: direct suturing, fibrin glue, and/or fibrin sealant patch n For anesthesia: avoid hypotension, mean arterial pressure >85 mmHg, antibiotics Postoperatively, the patient will be admitted to the neurocritical care unit Early mobilization and physical therapy evaluation

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Fig. 41.2 Sagittal CT of the thoracolumbar spine without contrast demonstrates significant hyperdensity throughout the mass, indicating heavy calcification.

LEARNING POINTS

• Intradural spinal tumors are classified as either intramedullary or extramedullary based on their relationship to the dura mater and spinal cord parenchyma. The majority of intradural tumors are benign, low-grade lesions. • Intradural, extramedullary tumors are more common in adults and are most frequently meningiomas, nerve sheath tumors (e.g. schwannoma, neurofibroma), or paragangliomas.2 Intramedullary tumors include ependymomas, astrocytomas, and hemangioblastomas. • The presentation of intradural spinal tumors depends on the location, size, and growth rate. • Spinal meningiomas are solid, well-circumscribed, smooth, round, frequently calcified tumors with marginal dural thickening (dural tail sign). They are more common in females and are usually found in the dorsolateral thoracic region.1,3,5 • Because spinal meningiomas typically grow slowly, significant spinal cord compression can occur before a patient begins to exhibit neurological deficits. Back and radicular pain typically precedes weakness and sensory deficits, with bowel and bladder dysfunction usually as a late finding.3 • Meningiomas display mild hyperintensity on T2-weighted imaging but are iso- to hypointense on T1-weighted imaging. Gadolinium administration leads to homogenous enhancement, with the exception of calcified areas.1,5 • When safe and possible, complete (gross-total) resection of a spinal meningioma is the optimal treatment modality. Partial debulking can be considered for adherent tumors in order to maximize neurological outcomes while minimize risk complication.3 • Often, partially debulked tumors can be followed in the outpatient setting to monitor for signs of growth and recurrence. Adjuvant radiotherapy can be considered in cases of subtotal resections and higher-grade lesions.3,4 Functional and neurological outcomes for spinal meningiomas are favorable with several • studies demonstrating a functional improvement in the majority of patients. Risk factors for surgical morbidity includes extensive tumor calcification and elderly age.3

41—Worsening BAck PAin And Leg WeAkness

205

References 1. Abul-Kasim K, Thurnher MM, McKeever P, Sundgren PC. Intradural spinal tumors: current classification and MRI features. Neuroradiology. 2008;50:301–314. 2. Parsa AT, Lee J, Parney IF, Weinstein P, McCormick PC, Ames C. Spinal cord and intraduralextraparenchymal spinal tumors: current best care practices and strategies. J Neurooncol. 2004;69:291–318. 3. Ravindra VM, Schmidt MH. Management of spinal meningiomas. Neurosurg Clin N Am. 2016; 27(2):195–205. 4. Setzer M, Vatter H, Marquardt G, Seifert V, Vrionis FD. Management of spinal meningiomas: surgical results and a review of the literature. Neurosurg Focus. 2007;23:E14. 5. Van Goethem JWM, van den Hauwe L, Ozsarlak O, De Schepper AMA, Parizel PM. Spinal tumors. Eur J Radiol. 2004;50:159–176.

C H A P T E R

42

Acute Inability To Walk Ann Liu, MD

n Yike Jin, MD

Consult Page 45M patient with hx metastatic colorectal cancer with inability to walk

Initial Imaging

A

B

Fig. 42.1 A. Sagittal thoracolumbar CT without contrast demonstrates lytic lesions of the T12 vertebral body with normal alignment. There is no evidence of vertebral body collapse. There are diffuse intraosseous lesions throughout the spinal column (not pictured). B. Axial view demonstrates extraosseous extension of the T12 lesion into the ventral aspect of the spinal canal with involvement of the posterior elements (right worse than left).

206

207

42—AcuTe InAbIlITy To WAlk

A

I

B

Fig. 42.2 A. Sagittal T2-weighted MRI of the thoracolumbar spine without contrast demonstrates an expansile lesion at T12 with posterior epidural extension. There is cord signal change at T11-T12 and multiple intraosseous lesions throughout the thoracolumbar spine. B. An axial sequence at T11-T12 demonstrates circumferential epidural cord compression with no evidence of CSF surrounding the cord.

Walking Thoughts n n n n n

n

n n

What is the patient’s neurological exam—what are his deficits? Why is the patient unable to walk? Is it secondary to pain or to weakness? Does he have any bowel or bladder dysfunction? How long has he had symptoms? What is the patient’s oncologic history? Does he have known metastases to the brain or spine? What is his baseline functional status? What is the patient’s prognosis from his colorectal cancer? Does he need surgical intervention urgently or emergently? Is he on any anticoagulant or antiplatelet medications?

History of Present Illness A 45 year old male with metastatic colorectal cancer treated with surgical resection and chemotherapy presents to the emergency department (ED) with three days of bilateral leg weakness and inability to walk. He was diagnosed with colorectal cancer one year prior but initially refused surgery and opted for chemotherapy alone for 7 months. During that time, he was found to have a metastatic lesion within the T12 vertebral body. He only had a moderate response to the chemotherapy and ultimately underwent surgical resection of his colorectal cancer with colostomy creation. Due to recent progression of his colorectal cancer, he was scheduled to start palliative radiation. The patient reports that three days prior, he was able to walk to a doctor’s appointment, but since then, he has had gradual weakness in his legs. He has not been able to walk since that doctor’s appointment. Prior to this, he was able to complete all of his activities of daily

208

SECTION IV—SPINE

living on his own. He endorses numbness and tingling in his legs but denies any difficulty with bowel or bladder function. He notes that he has had several months of low back pain that radiates into his legs and worsens with movement. Occasionally, the pain awakens him from his sleep. He does not take any pain medication. He denies any antiplatelet or anticoagulant use. Additional imaging in the ED demonstrates bilateral diffuse deep venous thromboses (DVTs), nonocclusive pulmonary emboli (PEs), a right liver lesion, metastatic lymphadenopathy, a right clavicular fracture, and a left greater trochanter lytic lesion.

Vital Signs T 37.2°C, HR 107, BP 181/110, SpO2 99% on room air

Pertinent Labs Pending

Physical Exam Alert, oriented to person, place, and year Cachectic Cranial nerves intact Deltoid

Biceps

Triceps

Wrist Flexion

Wrist Extension

Grip

RUE

5/5

5/5

5/5

5/5

5/5

5/5

LUE

5/5

5/5

5/5

5/5

5/5

5/5

Hip Flexion

Knee Extension

Knee Flexion

Dorsiflexion

Plantar Flexion

RLE

1-2/5

1-2/5

1-2/5

3/5

4/5

LLE

1-2/5

1-2/5

1-2/5

3/5

4/5

Minimal sensation in bilateral legs No clonus, no Hoffman’s sign No hyperreflexia Upgoing toes Rectal tone intact No saddle anesthesia

Spinal Instability Neoplastic Score (SINS) Location: 3 (junctional) Mechanical pain: 3 (pain with movement) Bone lesion: 2 (lytic) Spinal alignment: 0 (normal) Vertebral body collapse: 1 (no collapse with >50% body involved) Posterolateral involvement: 3 (bilateral) Total: 12

209

42—AcuTe InAbIlITy To WAlk

Triage Management This patient has metastatic colon cancer and presents with the acute inability to walk due to progressive leg weakness secondary to a T12 lesion causing grade 3 epidural spinal cord compression (ESCC). While a presentation of acute weakness from spinal cord compression would typically be considered a neurosurgical emergency necessitating surgical decompression, the patient’s complex cancer and medical history, and overall prognosis must be considered before deciding upon treatment. Even prior to his current presentation, the patient’s cancer had progressed despite systemic chemotherapy and surgery. On updated imaging in the ED, he has multiple new sites of metastases, and in discussion with his primary oncologist, his overall survival prognosis is less than 3 months. Additionally, he has new DVTs and PEs, which would increase the risk of surgical decompression. Given that he has been non-ambulatory for more than 48 hours, it is unclear how much strength he would regain even with surgery. A frank discussion was had with the patient and his wife discussing all these points, and they ultimately declined surgery. He will be admitted to the hospital by the medicine team for management of his new DVTs and PEs, and the radiation oncology team will be urgently consulted for palliative radiation to his spinal lesion. This is an emergent but ultimately nonoperative consult.

Assessment This is a 45 year old male with progressive metastatic colorectal cancer who presents with three days of weakness and inability to walk. Imaging of his spine demonstrates significant circumferential cord compression at T12. Given his progressive disease and overall poor prognosis with a survival of less than 3 months, the patient has declined surgical decompression and opted for radiation treatment instead.

Plan n n

n n n

n n

Admit to the medicine floor High dose corticosteroids for cord compression (dexamethasone 10 mg IV now and then 4 mg every 6 hours) Proton pump inhibitor while on corticosteroids Consult to radiation oncology for emergent radiation Consult to medical oncology for overall management of his metastatic colorectal cancer and his corticosteroids No restrictions on anticoagulation for his DVTs and PEs from a neurosurgical standpoint No activity restrictions

LEARNING POINTS

• The clinical signs and symptoms of an extradural spinal tumor depend on the location in the spine, the rate of tumor growth, the degree of bony involvement, and the amount of neural compression. Patients may present with cervical myelopathy (Chapter 35), neck pain (Chapter 36), thoracic myelopathy (Chapter 37), or cauda equina (Chapter 38). The differential diagnosis for extradural spinal cord tumors includes: • Metastasis

Osteolytic: lymphoma, lung, breast, prostate, renal cell Osteoblastic: prostate, breast

Primary spinal tumor

Chordomas, osteoid osteoma, osteoblastoma, aneurysmal bone cyst, chondrosarcoma, osteochondroma, hemangioma, giant cell tumors

Miscellaneous

Plasmacytoma, multiple myeloma, Ewing’s sarcoma, neurofibroma, schwannoma

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SECTION IV—SPINE

• Management of these tumors and surgical decompression often involves multiple teams (e.g. medical oncology, radiation oncology) depending on the lesion type, location, and responsiveness to systemic treatment. • The spine is the most common osseous site for metastatic disease, which can spread to the vertebral column via hematogenous seeding, direct extension, or by seeding of the cerebrospinal fluid (CSF). Metastatic epidural spinal cord compression (MESCC) most commonly originates from breast, lung, or prostate tumors.5,8 • The epidural spinal cord compression scale is used to grade the amount of compression based on MRI.2 • Grade 0: bone-only disease • Grade 1: • 1a: epidural impingement without deformation of the thecal sac • 1b: deformation of the thecal sac without spinal cord abutment • 1c: deformation of the thecal sac with spinal cord abutment without cord compression • Grade 2: spinal cord compression with CSF visible around the cord • Grade 3: spinal cord compression, no CSF visible around the cord • Treatment for MESCC is rarely curative and requires careful consideration of life expectancy, quality of life, and functional status. Goals of treatment include preservation of neurological function, pain relief, and mechanical stabilization. Surgery ranges from decompression alone and “separation surgery” (which involves minimal tumor resection to separate the tumor margin from the spinal cord) to an attempt at complete tumor resection and complex spinal reconstruction. Separation surgery creates a space between the spinal cord and tumor to minimize radiation toxicity to the spinal cord. • In the absence of mechanical instability, for ESCC grades 0, 1a, and 1b, radiation is considered to be the initial treatment. For grade 1c, the role of surgery vs. radiosurgery is controversial. For grades 2 and 3, surgical decompression is recommended prior to radiation therapy, unless the tumor is highly radiosensitive.6 • The Spinal Instability Neoplastic Score (SINS) was devised to aid in the diagnosis of neoplastic instability and combines six parameters: location, mechanical pain, alignment, osteolysis, vertebral body collapse, and posterior element involvement (Table 42.1).4 A low SINS score of 0 to 6 is considered stable and does not require surgical stabilization. A high SINS score of 13 to 18 is considered unstable and reliably predicts the need for surgical stabilization. • The neurologic, oncologic, mechanical, and systemic (NOMS) decision framework incorporates four assessments (neurologic, oncologic, mechanical instability, and systemic disease) to determine the use of radiation, surgery, and/or systemic therapy for spinal metastases (Table 42.2).6 • Neurologic considerations include the degree of MESCC, myelopathy, and/or functional radiculopathy. • Oncologic considerations include the expected tumoral response and the durability of response to available treatments. • Mechanical instability is an independent indication for surgical stabilization or percutaneous cement augmentation (kyphoplasty/vertebroplasty), regardless of the neurologic or oncologic assessment. • Systemic disease considerations include whether the patient can tolerate the proposed intervention and is based on the extent of tumor dissemination, medical comorbidities, and tumor histology. • In a randomized study of patients undergoing surgery with radiotherapy or radiotherapy alone, surgery and radiotherapy yielded statistically significant improvement in survival, overall ambulation, maintenance of ambulation, recovery of ambulation, narcotic and corticosteroid requirement, and bowel/bladder continence.7 • Highly radiosensitive tumors include multiple myeloma, lymphoma, and small cell lung carcinoma. Radiosensitive tumors include breast, prostate, and thyroid cancer. Radioresistant tumors include colorectal, renal cell, and melanoma. • The timing of surgery has not been well studied, but in general, it is thought that neurological outcomes may improve if decompression surgery is performed within 48 hours of symptom onset.1 • A general historical prerequisite for surgery is an expected survival of greater than 3 months as patients with a short life expectancy should not be subjected to the invasiveness of surgery

211

42—AcuTe InAbIlITy To WAlk

and its recovery time. However, a recent study showed that when controlled for baseline performance status, quality of life 6 weeks after surgery was independent of survival. Thus, a patient’s baseline performance status may be more important than his or her predicted life expectancy in the decision-making for surgery.3 These findings may be even more pertinent as continued advances in medical oncology allow for improved survival and prognosis. • A systematic review of the use of steroid therapy in MESCC found that:5 • Immediate steroid therapy following a diagnosis of MESCC followed by definitive treatment may increase the proportion of patients who remain ambulatory 1 year after therapy. • Steroid therapy is most effective when administered within 12 hours of symptom onset with an initial bolus of IV dexamethasone 10 mg with subsequent 4 mg every 6 hours (either oral or IV). After definitive therapy, steroids can be rapidly weaned to minimize side effects.

TABLE 42.1 n SINS score.4 Location Junctional (occiput-C2, C7-T2, T11-L1, L5-S1)

3

Mobile (C3-C6, L2-L4)

2

Semirigid (T3-T10)

1

Rigid (S2-S5)

0

Mechanical Pain Yes

3

Occasional pain but not mechanical

1

Pain-free lesion

0

Bone Lesion Lytic

2

Mixed (lytic/blastic)

1

Blastic

0

Spinal Alignment Subluxation/translation present

4

De novo kyphosis/scoliosis

2

Normal alignment

0

Vertebral Body Collapse > 50% collapse

3

< 50% collapse

2

No collapse but > 50% body involved

1

None of the above

0

Posterolateral Spinal Elements* Involvement Bilateral

3

Unilateral

1

None of the above

0

Total Score Stable

0–6

Indeterminate

7–12

Unstable

13–18

  

*Posterolateral spinal elements include the facet, pedicles, or costovertebral joints.

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TABLE 42.2 n Current NOMS decision framework.6 Neurologic

Oncologic

Mechanical

Low-grade ESCC + no myelopathy

Radiosensitive

Stable

cEBRT

Unstable

Stabilization then cEBRT

Stable

SRS

Radioresistant

Systemic

Decision

Unstable

Stabilization then SRS

High-grade ESCC Radiosensitive +/- myelopathy

Stable

cEBRT

Unstable

Stabilization then cEBRT

Radioresistant

Stable

Able to tolerate open surgery

Decompression/ stabilization then SRS

Unable to tolerate open cEBRT surgery Unstable

Able to tolerate open surgery

Decompression/ stabilization then SRS

Unable to tolerate open Stabilization* then surgery cEBRT   

  

References 1. Bakar D, Tanenbaum JE, Phan K, et al. Decompression surgery for spinal metastases: a systematic review. Neurosurg Focus. 2016;41(2):E2. 2. Bilsky MH, Laufer I, Fourney DR, et al. Reliability analysis of the epidural spinal cord compression scale. J Neurosurg Spine. 2010;13(3):324–328. 3. Dea N, Versteeg AL, Sahgal A, et al. Metastatic spine disease: should patients with short life expectancy be denied surgical care? An International Retrospective Cohort study. Neurosurgery. 2020;87(2):303–311. 4. Fisher CG, DiPaola CP, Ryken TC, et al. A novel classification system for spinal instability in neoplastic disease: an evidence-based approach and expert consensus from the Spine Oncology Study Group. Spine. 2010;35:E1221–E1229. 5. Kumar A, Weber MH, Gokaslan Z, et al. Metastatic spinal cord compression and steroid treatment: a systematic review. Clin Spine Surg. 2017;30(4):156–163. 6. Laufer I, Rubin DG, Lis E, et al. The NOMS framework: approach to the treatment of spinal metastatic tumors. Oncol. 2013;18(6):744–751. 7. Patchell RA, Tibbs PA, Regine WF, et al. Direct decompressive surgical resection in the treatment of spinal cord compression caused by metastatic cancer: a randomised trial. Lancet. 2005;366:643–648. 8. Sciubba DM, Petteys RJ, Dekutoski MB, et al. Diagnosis and management of metastatic spine disease. A review. J Neurosurg Spine. 2010;13(1):94–108.

C H A P T E R

43

Subacute Leg Pain, Numbness, And Weakness Ryan P. Lee, MD n

Yike Jin, MD

Consult Page 61F with bilateral lower extremity pain and weakness, MRI showing possible fistula

Initial Imaging

Fig. 43.1 T2-weighted sagittal MRI of the thoracic spine shows dorsal perimedullary flow voids (blue arrow) concerning for a dural arteriovenous fistula. There is also T2 hyperintensity in the cord likely from associated edema (yellow arrow). Incidental hemangiomas are seen within multiple vertebral bodies.

Walking Thoughts n n

What is the patient’s neurological exam? Is the patient weak, or is her weakness pain limited? 213

214 n n

n

n n n

SECTION IV—SPINE

What is the timeline of her symptoms? Where is the fistula on MRI? Do the patient’s symptoms localize to the site of the lesion seen on MRI? Is there any acute decompensation suggestive of acute hemorrhage, or are her symptoms more gradual due to spinal cord dysfunction from alteration in vascular flow dynamics? Does the patient need further imaging? Does the patient need surgery? What is the timing of surgery? Does the patient take any antiplatelet or anticoagulant medications?

History of Present Illness A 61 year old female with a history of hypertension and diabetes presents with several weeks of progressive bilateral lower extremity weakness and difficulty walking. The patient recalls that her symptoms started about nine months ago, where she noticed bilateral lower extremity pain, numbness, and weakness. There was no inciting event. The pain is mostly in her legs, and she does not have significant back pain. The pain and numbness started in her thighs and progressed to involve her entire legs. The pain is constant, burning in nature, and rated 7 out of 10. She saw her primary care physician for evaluation and had a negative workup for myositis and myopathy. She was started on gabapentin and is scheduled for electromyography and nerve conduction studies with consideration of muscle biopsy. The gabapentin has somewhat helped the leg pain but her numbness is unchanged. Over the last few weeks, however, the patient has had trouble walking because she cannot feel the ground. She denies saddle anesthesia, and bowel or bladder dysfunction. She denies any known spinal pathology, prior spinal surgery, and a personal or familial history of vascular lesions. She presented today to the emergency department because she “has trouble knowing where her legs are.” She does not take any antiplatelet or anticoagulant medications.

Vital Signs T 37.2°C, BP 160/77, HR 72, SpO2 99% on room air

Physical Exam Awake, alert, no apparent distress Oriented to self, place, and year Average body habitus, no obvious deformity or congenital malformation Pupils equal, round, and reactive to light Extraocular movements intact bilaterally, tongue midline, face symmetric Deltoid

Biceps

Triceps

Wrist Flexion

Wrist Extension

Grip

RUE

5/5

5/5

5/5

5/5

5/5

5/5

LUE

5/5

5/5

5/5

5/5

5/5

5/5

Hip Flexion

Knee Extension

Knee Flexion

Dorsiflexion

Plantar Flexion

RLE

5/5

5/5

5/5

5/5

5/5

LLE

5/5

5/5

5/5

5/5

5/5

ss

215

Patchy areas of diminished sensation to light touch and pinprick in bilateral lower extremities not consistent with a particular spinal level or dermatomal distribution Severely diminished proprioception in toes Patient imbalanced when standing with eyes closed (Romberg test positive) No pathologic ankle clonus or hyperreflexia

Triage Management This case does not represent an emergency as there has been no acute neurological deterioration. The patient’s symptoms have been present for nine months, with worsening of numbness over the past few weeks. Although she complains of weakness, there is no objective weakness on exam, and her inability to walk is likely due to decreased sensation and proprioception. She also does not have any red flag symptoms such as bowel or bladder dysfunction. Spinal MRI demonstrates thoracic perimedullary flow voids that likely represent a spinal dural arteriovenous fistula (dAVF) or some other type of vascular malformation. Although there is cord edema, there is no apparent intramedullary lesion, extrinsic mass causing cord compression, or evidence of acute hemorrhage. It is likely that this vascular lesion is causing myelopathy through alteration of flow dynamics via steal phenomenon (where blood is preferentially shunted from low flow capillaries to high flow fistula) and/or venous congestion. The patient is stable, and additional imaging is needed with catheter angiography to characterize the angioarchitecture of the lesion for further treatment planning. An MRI of the panneuroaxis with and without gadolinium may be obtained to determine if there are any other concomitant lesions, and to characterize the extent of the spinal cord edema. Given the chronicity of the patient’s symptoms, she may be scheduled for outpatient workup. However, because the imaging suggests a dAVF, the patient will likely require surgical treatment once her workup is complete. This is a non-emergent and likely operative consult.

Assessment This is a 61 year old female with hypertension and diabetes who presents with subacute lower extremity numbness, pain, and difficulty walking. MRI is concerning for a spinal dAVF. However, a definitive diagnosis cannot be made without traditional catheter angiography.

Plan n n n n n

No emergent neurosurgical intervention indicated Check a post-void residual to ensure that the patient is not retaining urine Outpatient procedural planning for spinal catheter angiography Outpatient MRI of the brain and spine with and without contrast If the patient has red flag symptoms (e.g. weakness, bowel or bladder dysfunction), consider inpatient admission for expedited angiography and treatment

Follow-up The patient underwent spinal angiography the following week which confirmed a dAVF fed primarily by small arterial feeders arising from the right T6 intersegmental artery. There is early venous drainage into dilated and tortuous perimedullary veins (Figure 43.2A). One week following angiography, she underwent a T7-T8 laminoplasty for surgical clipping of the dAVF. Intraoperative spinal angiography demonstrated complete obliteration of the dAVF with good filling of the anterior spinal artery without early venous drainage (Figure 43.2B). The patient did well postoperatively and was discharged to home on postoperative day three.

216

A

SECTION IV—SPINE

B

Fig. 43.2 A. Preoperative catheter spinal angiography demonstrates small arterial feeders arising from the right T6 intersegmental artery (arrow) supplying a dural arteriovenous fistula with early venous drainage into dilated and tortuous perimedullary veins (dotted line). There is a prominent anterior radiculomedullary artery supplying the anterior spinal artery at the same level. B. Intraoperative spinal angiography after surgical clipping demonstrates filling of the anterior spinal artery from a right T6 injection without early venous drainage to suggest residual fistula. The previously seen dilated and tortuous perimedullary veins are no longer seen (dotted line).

LEARNING POINTS

• Spinal vascular malformations are most commonly divided into four classical subtypes.1,2 • Type I lesions are spinal dural AV fistulas, where one or more radicular arteries directly connect to the venous system at the nerve root sleeve, causing arterialization of the perimedullary venous plexus.3,8 • Type II lesions are intramedullary glomus arteriovenous malformations (AVMs), which are analogous to intracranial AVMs and have a nidus in the spinal cord parenchyma. • Type III lesions are juvenile (metameric) AVMs. They are typically extensive (intra- and extradural) and can invade much of the surrounding anatomy. • Type IV lesions are pial (intradural perimedullary) AV fistulas, which are typically fed by the anterior spinal artery and are thus concentrated ventrally. • Conus medullaris AVMs and extradural spinal AV fistulas are more recently described additional separate subtypes.3 • The presence of intra- or extra-medullary hemorrhage on imaging in a patient with acute back pain or neurological deficits should raise immediate suspicion for a spinal vascular lesion or hemorrhagic tumor. • Similarly, a spinal vascular malformation should be considered in the differential for all patients with cerebral angiogram-negative intracranial subarachnoid hemorrhage. • Type I spinal dural AV fistulas are low flow lesions that most commonly present insidiously with progressive neurological deterioration from venous hypertension and altered flow dynamics causing ischemia; in these patients, hemorrhage and associated aneurysms are uncommon.4

ss

217

• Because of a typically nonspecific manifestation of symptoms, spinal dAVFs are usually diagnosed late and after previous misdiagnoses.5,6 • Patients may have been previously treated for peripheral neuropathy, radiculopathy, degenerative spondylosis causing central canal stenosis, or autoimmune disorders such as multiple sclerosis. • The most common symptoms are leg weakness, sensory disturbance, and bowel/ bladder dysfunction. • Importantly, patients may present with upper or lower motor neuron findings.6 • Patients typically present in the fifth to seventh decades of life, with a strong male predominance.8 • MRI is the initial diagnostic test of choice, typically displaying serpiginous perimedullary flow voids on T2-weighted sequences. There may or may not be T2 hyperintensity within the spinal cord from edema. • Of note, flow voids may be subtle and can be easily missed. • Oftentimes, a single segment of the spine is imaged; however, spinal dAVF can cause edema and related symptoms distant to the actual level of the fistulous connection, and thus pan-neuroaxis imaging may be critical.6 • Conventional catheter angiography is the gold standard for diagnosis and specific characterization of spinal vascular lesions. For spinal dAVFs, there may be one or more feeding vessels, and they typically originate in the thoracolumbar spine.3,4 • Treatment options include surgical ligation and endovascular embolization. • Most patients with spinal dAVFs will experience improvement postoperatively; however, neurological improvement may occur over months to years.7

References 1. Di Chiro G, Doppman J, Ommaya AK. Selective arteriography of arteriovenous aneurysms of spinal cord. Radiology. 1967;88(6):1065–1077. 2. Heros RC, Debrun GM, Ojemann RG, Lasjaunias PL, Naessens PJ. Direct spinal arteriovenous fistula: a new type of spinal AVM. Case report. J Neurosurg. 1986;64(1):134–139. 3. Huffmann BC, Gilsbach JM, Thron A. Spinal dural arteriovenous fistulas: a plea for neurosurgical treatment. Acta Neurochirurgia (Wien). 1995;135(1–2):44–51. 4. Koenig E, Thron A, Schrader V, Dichgans J. Spinal arteriovenous malformations and fistulae: clinical, neuroradiological and neurophysiological findings. J Neurol. 1989;236(5):260. 5. Kim LJ, Spetzler RF. Classification and surgical management of spinal arteriovenous lesions: arteriovenous fistulae and arteriovenous malformations. Neurosurgery. 2006;59(5 suppl 3):S195–S201; discussion S3-13. 6. Rosenblum B, Oldfield EH, Doppman JL, Di Chiro G. Spinal arteriovenous malformations: a comparison of dural arteriovenous fistulas and intradural AVM’s in 81 patients. J Neurosurg. 1987;67(6):795–802. 7. Safaee MM, Clark AJ, Burkhardt JK, Winkler EA, Lawton MT. Timing, severity of deficits, and clinical improvement after surgery for spinal dural arteriovenous fistulas. J Neurosurg Spine. 2018;29(1): 85–91. 8. Symon L, Kuyama H, Kendall B. Dural arteriovenous malformations of the spine. Clinical features and surgical results in 55 cases. J Neurosurg. 1984;60(2):238–247.

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44

Sudden Bilateral Lower Extremity Weakness With Hemodynamic Instability Kurt Lehner, MD n

Risheng Xu, MD/PhD

Consult Page 60M h/o metastatic prostate cancer presenting with leg weakness, MRI with abnormal T2 signal and enhancement, thoracic compression fracture

Initial Imaging

A

B

Fig. 44.1 A. A T2-weighted MRI of the cervical and thoracic spine without contrast demonstrates a T2hyperintense signal extending from approximately T1-T7 (arrow). B. A sagittal pan-spine MRI shows multifocal bony metastatic disease, most notably at T5 and T7 without significant canal compromise.

Walking Thoughts n n

n

218

What is the patient’s neurological exam? What is the onset and progression of the patient’s weakness (acute versus chronic)? Is the patient having any other symptoms (e.g. numbness, bowel/bladder dysfunction, saddle anesthesia)? What is the patient’s prostate cancer history? Where are the known sites of metastasis?

44—SuddEn BILatEraL LoWEr ExtrEmIty WEaknESS WItH HEmodynamIc InStaBILIty 219

n n

n

n

n

What treatment has the patient undergone? Has the patient received spinal radiation? What other medical history does the patient have? Do these alter the differential diagnosis? The spine MRI does not show obvious spinal cord compression, but rather abnormal signal within the intrinsic spinal cord. What can cause this? Does this explain the patient’s physical exam and presentation? What additional studies will be necessary to determine whether or not surgery is necessary? Does the patient take any antiplatelet or anticoagulant medications?

History of Present Illness A 63 year old male with a history of metastatic prostate cancer and hypertension presents to the emergency department (ED) with acute bilateral lower extremity weakness. The patient states that this morning he began experiencing severe burning chest pain located across the front of his chest. He then had an episode of nausea and vomiting followed by immediate lower extremity weakness and loss of sensation below his waist without significant back pain. This occurred over a period of 2–3 minutes. He denies bowel and bladder incontinence. He reports no history of trauma. The patient was brought to the hospital by ambulance where he was found to be bradycardic and hypotensive. In the ED, he was given 4 liters of normal saline but has remained hypotensive despite resuscitation and initiation of vasopressors. An MRI of the pan-spine was obtained and is demonstrated in Figure 44.1. In terms of his oncological history, the patient is currently on a third-line chemotherapy agent. At the initial time of his prostate cancer diagnosis, the patient already had metastatic disease with spinal lesions. He has not undergone prior radiation or surgical intervention. His spinal metastases have been monitored on serial imaging and are currently stable. He is not on any antiplatelet or anticoagulant medications.

Vital Signs T 37.8°C, BP 60/37 on norepinephrine, HR 60, RR 15, SpO2 99% on room air

Pertinent Labs Na 140, K 3.4, Cr 1.0, INR 1.1, aPTT 27.9

Glasgow Coma Scale Motor: 6 Verbal: 5 Eye opening: 4 GCS Total: 15

Physical Exam Alert and oriented to person, place, and date Speech fluent, appropriate Extraocular movements intact bilaterally, tongue midline, face symmetric Bilateral upper extremities 5/5 strength in all muscle groups

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RLE

0/5

0/5

0/5

0/5

0/5

LLE

0/5

0/5

0/5

0/5

0/5

No sensation below the T4 level including light touch, pain, temperature, or vibration No hyperreflexia Toes mute No clonus Rectal tone intact, perianal sensation intact Foley in place

Triage Management This is a patient with a history of metastatic prostate cancer with multiple known and unchanged spinal metastases who presents with acute onset symmetric bilateral lower extremity weakness with a sensory level. His initial bradycardia and persistent hypotension despite resuscitation and vasopressors is concerning for neurogenic shock. His physical exam localizes to a thoracic lesion. This presentation could be consistent with acute spinal cord compression amenable to surgical resection, and it is imperative that spinal imaging is expedited to rule out cord compression secondary to epidural metastatic disease or a pathologic fracture. However, the patient had a pan-spine MRI which demonstrates a widely patent spinal canal, and thus his neurological deficits are not from spinal cord compression. There is a longitudinal T2 hyperintensity in the thoracic spinal cord without evidence of an intrinsic mass, which could be due to a variety of inflammatory or vascular causes (Table 44.1). Given the imaging findings and his sudden onset of symptoms, a diagnosis of intramedullary spinal cord tumor is unlikely. There are no obvious flow voids to suggest a spinal dural arteriovenous fistula (dAVF), or a slow insidious onset of symptoms characteristic of venous hypertension. His MRI does not show subarachnoid hemorrhage to suggest rupture of a vascular lesion. His acuity of symptoms is most concerning for a spinal cord infarct or an inflammatory process. He will need to be admitted to the neurocritical care unit for hemodynamic support of his neurogenic shock and further workup. This is an urgent but likely nonoperative consult.

Assessment This is a 63 year old male with a history of metastatic prostate cancer who presents with acute onset lower extremity weakness with a T4 sensory level and neurogenic shock concerning for a spinal cord infarct or an acute inflammatory insult.

Plan n n n n

n

Admit to the neurocritical care unit for hemodynamic stabilization and support Arterial line placement Recommend neurology consult for further work-up of spinal cord hyperintensity Patient would benefit from repeat spinal MRI including diffusion weighted sequences (Figure 44.2) Plan for diagnostic spinal angiogram to evaluate for vascular etiologies (Figure 44.3)

44—SuddEn BILatEraL LoWEr ExtrEmIty WEaknESS WItH HEmodynamIc InStaBILIty 221

TABLE 44.1 n Common longitudinal T2-hyperintense spinal cord lesions1,2 Category

Pathology

MRI Characteristics

Comments

Neoplastic

Ependymoma

Homogenous enhancement with discrete borders

“Cap sign” - low intensity signal at margins of tumor related to hemosiderin

Astrocytoma

Eccentrically located within the Cystic components related cord with heterogeneous to the tumor, and a syrinx enhancement are common

Hemangioblastoma

Contrast-enhancing cystic lesion with a mural nodule

Usually solitary; if multiple, consider Von HippelLindau disease

Metastasis

Extensive vasogenic edema; presents with variable contrast enhancement of lesion

Lung, breast, renal cell, melanoma, and lymphoma are the most common primaries

Multiple sclerosis/ transverse myelitis

Disseminated T2-hyperintense lesions in space and time

Active lesions may contrast enhance

Subacute combined degeneration

Non-enhancing, posterolateral lesions with the cord becoming atrophic late in the course

Related to vitamin B12 deficiency

Cord infarction

Longitudinal lesion, usually in the thoracolumbar (Artery of Adamkiewicz) or cervical (artery of Lazorthes) cord; enhancement is uncommon

Typically a result of atheromatous plaque disruption, aortic dissection, or hypotension

AV fistulas/ malformations

T2-flow voids can frequently be seen near the cord; may present with hemorrhage

T2-signal is due to venous hypertension, angiography is necessary for diagnosis

AIDS-related myelopathy

Symmetrical high signal on T2 Diagnosis of exclusion commonly in thoracic region associated with latewith a predilection for the stage HIV infection; dorsal columns and lateral signal may also be corticospinal tracts, usually caused by related does not enhance opportunistic infections

Neurosarcoidosis

 

Syringo/hydromyelia

Expansile, cystic lesion which Variety of etiologies, may be central (hydromyelia) should also evaluate for or eccentric (syringomyelia) the presence of Chiari malformation

Diastematomyelia

Hyperintense signal resulting from volume averaging in sagittal imaging, diagnosis apparent on axial/coronals

Demyelinating

Vascular

Infectious/ Inflammatory

Other

  

Very rare, may have leptomeningeal involvement, at times can be difficult to distinguish from multiple sclerosis

Usually diagnosed as infant

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SECTION IV—SPINE

Fig. 44.2 Diffusion-weighted imaging of the cervical and thoracic spine demonstrates restricted diffusion of the anterior portion of the thoracic spinal cord (arrow) consistent with infarction of the artery of Adamkiewicz.

*

Fig. 44.3 Anteroposterior view of a spinal angiogram in the lumbar region demonstrates occlusion of the right L2 intersegmental artery (arrow) approximately 1.8 cm distal to its origin. The left intersegmental artery (asterisk) is intact. There is non-visualization of the artery of Adamkiewicz, which may have arisen from the right L2 intersegmental artery.

44—SuddEn BILatEraL LoWEr ExtrEmIty WEaknESS WItH HEmodynamIc InStaBILIty 223 LEARNING POINTS

• Hyperintense spinal cord signal on T2-weighted images is seen in a wide-ranging variety of spinal cord processes including simple MR artifact, congenital anomalies, tumor, vascular, infectious/inflammatory, and other disease categories.1,2 Not all abnormal spinal cord lesions require neurosurgical management. • The differential for T2-hyperintense longitudinal lesions of the spinal cord is broad (Table 44.1). Characterization of the abnormal areas of T2 signal as well as their appearance on other MR imaging sequences, when combined with clinical context and laboratory investigations, will often allow for narrowing of the differential diagnosis. • Spinal arteriovenous fistulas (AVF) can cause lesions similar to an intrinsic spinal cord tumor. Angiography is the gold standard for diagnosis and is critical in guiding endovascular versus open treatment. Spinal AVFs are discussed separately in Chapter 43. • Many causes of T2-hyperintensity can cause cord swelling secondary to inflammation; a work-up for alternative autoimmune or infectious etiologies is frequently necessary in select patients. Spinal cord biopsy may be necessary to make the diagnosis in patients with nonspecific • imaging and an otherwise negative neurological work-up. In a retrospective series of patients undergoing spinal cord biopsy for cryptogenic cord signal change and expansion, common findings included demyelination, neurosarcoidosis, nonspecific inflammation, eosinophilic vasculitis, vascular malformation, tuberculosis, and schistosomiasis.3,4

References 1. Bou-Haidar P, Peduto AJ, Karunaratne N. Differential diagnosis of T2 hyperintense spinal cord lesions: part A. J Med Imaging Radiat Oncol. 2008;52(6):535–543. 2. Bou-Haidar P, Peduto AJ, Karunaratne N. Differential diagnosis of T2 hyperintense spinal cord lesions: part B. J Med Imaging Radiat Oncol. 2009;53(2):152–159. 3. Cohen-Gadol AA, Zikel OM, Miller GM, Aksamit AJ, Scheithauer BW, Krauss WE. Spinal cord biopsy: a review of 38 cases. Neurosurgery. 2003;52(4):806–816. 4. Lee M, Epstein FJ, Rezai AR, Zagzag D. Nonneoplastic intramedullary spinal cord lesions mimicking tumors. Neurosurgery. 1998;43(4):788–795.

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45

Right Leg Numbness And Foot Drop James Feghali, MD n

Daniel Lubelski, MD n Risheng Xu, MD/PhD

Consult Page 36M skateboarding accident, can’t feel or move below right knee

Initial Imaging

Fig. 45.1 Anteroposterior view of a right knee x-ray demonstrates a comminuted avulsion fracture of the fibular head with fracture fragments displaced proximally approximately 2 cm near the lateral knee joint space.

Walking Thoughts What was the mechanism of the trauma? Are there any fractures (e.g. fibular head/neck)? Are there other associated vascular (e.g. popliteal artery), meniscal, or ligamentous injuries? n Are the neurological findings consistent with injury to a particular nerve? If so, what is the mechanism of nerve injury (e.g. laceration, stretch, compression)? n Is only a single nerve involved, or does the injury affect several nerves? Are there multiple sensory and/or motor distributions affected? n Is there imaging available for the entire course of the suspected affected nerve or is the imaging limited to the injured site? n Are there features of nerve injury on imaging (e.g. increased size of the nerve, increased size and signal of the individual fascicles, increased signal intensity of the nerve, deviation from the normal anatomical course)? n Based on the mechanism, preliminary clinical exam, and imaging findings, what is the expected severity of injury (e.g. neuropraxia versus axonotmesis)? n What is the likelihood of spontaneous recovery? n What is the need and timing for nerve conduction studies and/or surgical exploration and repair? 224 n

n

225

45—Right Leg NumbNess AND Foot DRop

History of Present Illness A 36 year old healthy male presents with loss of sensation distal to the right knee and foot drop following injury after a skateboarding accident. He reports crashing his right knee into a tree and recalls feeling a twisting sensation. He landed on his side and experienced significant pain in the right knee with inability to bear weight. He noted a right foot drop immediately after the accident, which prompted him to come to the emergency department. He also reports numbness on the right side of his shin and the top of his right foot. He experienced no head trauma or loss of consciousness and denies any back pain or significant knee swelling. He is not on any anticoagulant or antiplatelet medications.

Vital Signs T 37.1°C, HR 113, BP 107/54, SpO2 98% on room air

Pertinent Labs None

Physical Exam Alert and oriented to self, place, and year Pupils equal, round, and reactive to light Extraocular movements intact bilaterally, tongue midline, face symmetric Bilateral upper extremities 5/5 strength in all muscle groups Hip Flexion

Knee Extension

Knee Flexion

Dorsiflexion

Plantar Flexion

Foot Eversion

Foot Inversion

RLE

5/5

5/5

5/5

0/5

5/5

0/5

5/5

LLE

5/5

5/5

5/5

5/5

5/5

5/5

5/5

Diminished sensation over the right lateral tibial region and dorsum of the right foot including the web space between the first and second toes as well as the nail beds Intact sensation over the right calf, medial aspects of the right ankle and foot, as well as over the sole and heel of the right foot 2+ bilateral patellar and Achilles tendon reflexes Point tenderness over the right fibular head 3+ right dorsalis pedis and right popliteal artery pulses with normal color and temperature No open lacerations or exposed bone

Triage Management The patient presents with weakness in his right dorsiflexion and right foot eversion, and has decreased sensation on the right lateral tibial surface and dorsum of the right foot. This is consistent with a right common peroneal nerve (CPN) injury associated with comminuted avulsion of the right fibular head. The right CPN injury is confirmed by signs of direct nerve injury on MRI (not shown). The CPN injury may have been caused by laceration, stretch, or contusion at the level of the bony fracture, and may also be caused by a proximal stretch injury. In the acute setting, it is difficult to determine exactly where along the nerve the injury begins and ends. It is also difficult to know what portions of the nerve are viable versus permanently injured. In this situation, surgery is not indicated acutely. Waiting approximately 2–3 months would allow sufficient time to determine the extent of nerve injury. If the nerve is in continuity, simply giving time for regeneration may be all that

226

SECTION IV—SPINE

is needed. However, if there is evidence of little to no nerve regeneration after 2–3 months, surgery may be recommended for exploration and possible nerve grafting. Electromyography (EMG) and nerve conduction studies (NCS) will be useful beginning approximately 2–3 weeks after the injury. This will serve as a baseline and give a sense of the severity of the injury. Consistent with our initial vascular exam, the assessment of the vascular surgery team confirms the absence of vascular injury. The orthopedic service evaluated the patient and recommended nonoperative intervention with an ankle-foot orthotic with plans for repeat imaging and assessment of the right knee in 2 weeks. This is an urgent and possibly operative consult.

Assessment This is a 36 year old male who presents with a closed traumatic right CPN injury. He does not have any acutely operative vascular or orthopedic injuries. He may be managed conservatively for the time being with EMG and nerve conduction studies approximately 2–3 weeks after injury and close follow up with neurosurgery and orthopedic surgery. If the orthopedic surgery team takes him for surgical exploration of the knee, the neurosurgery team will be available for intraoperative inspection and tagging of the CPN to facilitate possible future repair.

Plan n n n n

Maintain ankle-foot orthotic per orthopedic surgery recommendations Passive range of motion exercises with stretching of the Achilles tendon EMG and nerve conduction studies in 2–3 weeks Possible future surgical repair depending on the extent of neurological recovery with possible nerve grafting in 2–3 months

Follow-up Approximately two weeks later, the patient had an EMG and nerve conduction study that showed no motor units in the peroneal nerve distribution distal to the short head of the biceps femoris. He underwent an outpatient knee MRI that showed an anterior cruciate ligament rupture and partial tear of the posterior cruciate ligament. Orthopedic surgery reevaluated the patient, and he underwent a lateral fibula open reduction and internal fixation with lateral reconstruction three weeks after the initial injury. Intraoperative neurosurgical exploration at that time revealed the nerve to be crushed at the fracture site at the level of the CPN bifurcation into the deep and superficial peroneal branches. The nerve was found to be firm and swollen at least 13 cm proximal to the crush site. The nerve was dissected free from scar tissue, decompressed, and mobilized to a more anatomical position. In addition, the nerve was tagged with suture at the start of the crush injury, the fibular head region, as well as the distal portion of the crush injury. Because the nerve was found to be in continuity, the decision was made to give the nerve a chance to regenerate spontaneously. The patient was followed with serial physical exams and EMGs every few months. At his last follow up 16 months after the initial injury, the patient had partial recovery of his CPN: he now exhibited eversion at 3/5 strength, and tibialis anterior activation at 2/5 strength. Consistent with this, his EMG and nerve conduction studies demonstrated motor units in both the peroneus longus as well as the tibialis anterior. The plan was made for continued nonoperative care with physical therapy. LEARNING POINTS

• Nerve injuries may be divided via the Sunderland classification system. This has implications on when and how to intervene after nerve injury (Table 45.1).2

On average, human peripheral nerves regenerate at a rate of approximately 1 inch per • month, or about 1 millimeter per day.13-14

45—Right Leg NumbNess AND Foot DRop

227

• Traumatic mononeuropathy is primarily a clinical diagnosis that is based on sensorimotor deficits that correspond to a nerve in the setting of trauma.

• Nerve conduction studies and electromyograms are noninvasive tests that have a diagnostic role in the delayed setting but not immediately after injury. NCS combined with EMG can determine whether a nerve injury is complete or incomplete, and thus guide prognosis and the likely course of recovery.4,12 • NCS measures how fast the nerves can send electrical signals. • An EMG test records the electrical signals that muscles generate when they are at rest or activated. • In cases where a nerve injury is confirmed intraoperatively, delayed EMG may be used to document recovery. • L5 radiculopathy and peroneal neuropathy can both present with weakness of the foot dorsiflexors and toe extensors. However, L5 radiculopathy may present with foot inversion weakness, as compared to a peroneal neuropathy which presents with foot eversion weakness.11 • If the patient has subtle peroneal nerve injury, strengthening via physical therapy may help with functional recovery. If the patient has complete loss of strength, passive range of motion exercises and an ankle-foot orthosis are imperative to maintain proper ankle range of motion so that residual heel cord contracture will not preclude the ability to walk.1,7,16 • Electrical stimulation of the nerve and/or target muscles is controversial. There have been limited reports of applying electrical fields/gradients across a repaired peripheral nerve to speed up axonal regeneration, mainly in animal studies.5,6 • Animal studies demonstrate that as little as one hour of direct nerve electrical stimulation immediately after repair of a transected femoral nerve in the rat promotes a dramatic increase in target muscle reinnervation. • In a clinical pilot study, one hour of electrical stimulation was applied after median nerve decompression at the wrist for 21 patients with carpal tunnel syndrome and thenar atrophy. The electrical stimulation group showed evidence of accelerated axonal regeneration and target reinnervation.5 • Surgery and timing for nerve repair depends on the type of injury. A helpful rule for the timing of repair in nerve injury is the rule of 3’s.12 • Early surgery (1 cm • Fracture fragments with sharp edges that can pierce the dura • Clinical or radiographic evidence of dural penetration • Gross wound contamination • Underlying hematoma • Gross pneumocephalus • Depression over the motor area2 • The location of the fracture also affects the treatment strategy: • While the parietal bone is the most commonly fractured, injuries in this region are generally covered by hair and have the best chance of being partially remodeled without intervention. • Patients with frontal bone fractures, particularly older children with aerated frontal sinuses, are more likely to require surgical intervention as their fractures may involve the frontal sinus, skull base, and orbit, and have an increased chance of causing a CSF leak, ocular complications, and cosmetic deformities.1 Ping-pong fractures

• In newborns and infants, depressed skull fractures can appear as a “ping-pong ball” or “pond” fracture, with inward buckling of the bone surface without loss of bone continuity.

• Smaller deformities can be managed non-surgically in the absence of underlying brain injury, as these deformities will generally correct over time.

• Operative management or other interventions such as vacuum extraction should be considered if the defect is larger (>5 mm in depth, >2 cm in length) or if there are concerning features (associated neurological deficit, signs of CSF leak, increased ICP).3 Growing skull fractures

• A growing skull fracture is also known as a posttraumatic leptomeningeal cyst. They are an extremely rare complication of pediatric skull fractures, where the brain progressively herniates through torn dura as a persistent swelling or pulsatile mass. This can result in neurological deficits or seizures. • Growing skull fractures occur almost exclusively in children under three years of age, and can present days to months after the initial head injury. • On imaging, a progressive widening of the fracture with scalloped edges may be seen. Doppler ultrasound or MRI studies can help diagnose a growing skull fracture. • Surgical intervention with dural closure is mandatory.4

References 1. Bonfield CM, Naran S, Adetayo OA, Pollack IF, Losee JE. Pediatric skull fractures: the need for surgical intervention, characteristics, complications, and outcomes. J Neurosurg Pediatr. 2014;14:205–211. 2. Bullock MR,PD, Chesnut R, Gordon D. Chapter 7: surgical management of depressed cranial fractures. Neurosurgery. 2006;58(3). https://doi.org/10.1227/01.NEU.0000210367.14043.0E. 3. Hung KL, Liao HT, Huang JS. Rational management of simple depressed skull fractures in infants. J Neurosurg Pediatr. 2005;103(Pediatrics 1):69–72. 4. Liu XS, You C, Lu M, Liu JG. Growing skull fracture stages and treatment strategy. J Neurosurg Pediatr. 2012;9:670–675.

C H A P T E R

52

Loss Of Consciousness After Bike Accident Lydia Ju-mi Bernhardt, MD n

Ann Liu, MD

Consult Page 11 yo healthy F, fall from bike with skull fracture

Initial Imaging

B

A

C

D

Fig. 52.1 Initial head CT without contrast. A. Axial view, bone window demonstrating a left temporal bone fracture extending to the skull base. B. Axial view, parenchymal window demonstrating a 6 mm epidural hematoma overlying the left temporoparietal convexity with a focus of pneumocephalus. C. Axial view, parenchymal window demonstrating a small contrecoup right parietal convexity hemorrhagic contusion measuring 4 mm. D. CT 3D reconstruction of the skull highlighting the left parietal and temporal fractures.

254

52—LOss Of COnsCiOusness After Bike ACCident

255

Walking Thoughts n n n

n n n n n

What is the patient’s GCS and neurological status? What is the mechanism of injury? Does the patient have any other injuries? Has the patient been evaluated by the trauma team? What was the patient’s condition immediately after the injury? How large is the fracture? Is it open or closed? Are there any associated intracranial injuries? Is there concomitant cervical spine injury that must be considered? Is there any concern for nonaccidental trauma?

History of Present Illness An 11 year old girl with no significant past medical history presents to the emergency department after a bike accident and hitting her head on the curb with loss of consciousness. Her mom reports that her daughter was riding her bike down a driveway when she slipped on leaves, fell off the bike, and hit the left side of her head on the curb. She was not wearing a helmet. She immediately lost consciousness, but regained consciousness a few minutes later. She was brought to her local hospital, where she was initially confused with multiple bouts of emesis. Her mother reports that she is at her neurological baseline. She endorses pain at the nape of her neck but denies headaches, weakness, numbness, tingling, vision changes, hearing loss, or leakage of fluid from her ears or nose. Head CT demonstrated a nondisplaced left temporoparietal skull fracture extending to the skull base with an underlying 6 mm extra-axial hematoma and a 4 mm contrecoup right parietal hemorrhagic contusion. X-rays of the cervical spine did not reveal any fracture or subluxation. She was transferred to our hospital for further management. Trauma evaluation revealed a fracture of the 1st metacarpal bone of the right hand, but was otherwise negative for other injuries.

Vital Signs T 36.8°C, HR 105, BP 105/52, SpO2 99% on room air

Pertinent Labs Na 141, Plt 269, INR 1.0, APTT 23.0

Glasgow Coma Scale Motor: 6 Verbal: 5 Eye opening: 4 GCS Total: 15

Physical Exam Awake, alert, oriented to self, place, and year Hard cervical collar in place Pupils equal, round, and reactive to light Extraocular movements intact, face symmetric, tongue midline No pronator drift Bilateral upper extremities 5/5 except for right grip 4/5 due to pain and swelling of thumb

256

SECTION V—PEDIATRIC

Bilateral lower extremities 5/5 Sensation intact to light touch throughout No leakage of fluid from ears or nose

Triage Management The patient has a nondisplaced left temporoparietal fracture with an associated 6 mm left epidural hematoma without mass effect, and a small contrecoup right parietal hemorrhagic contusion. Although the patient had some initial confusion, she is now at her neurological baseline. The patient has a concomitant right hand injury and will be admitted to the pediatric trauma service. While no acute operative intervention is needed, she requires frequent neurological checks given the degree of intracranial injuries with potential for progression of bleeding and clinical deterioration. On imaging, effusions in the left sphenoid sinus and mastoid air cells, as well as a focus of pneumocephalus along the medial aspect of the left middle cranial fossa raise concern for additional occult temporal and/or sphenoid bone fractures—the patient should also be monitored for signs of CSF leak, including otorrhea and rhinorrhea. Although plain films did not reveal any gross bony abnormalities in the patient’s cervical spine, given the severity of her fracture, an MRI of her cervical spine is warranted to rule out ligamentous injury. This is an urgent but nonoperative consult.

Assessment This is a healthy 11 year old female who presents with transient loss of consciousness, headaches, and emesis after head trauma following a bicycle accident. She is found to have a nondisplaced left temporal fracture extending through the skull base. Her exam is reassuring; however, given the presence of a small left epidural hematoma and right parietal hemorrhagic contusion, close monitoring is warranted.

Plan n

n

n n n n

Admission to the trauma service in the pediatric intensive care unit with neurochecks every hour Interval imaging of her head at 6 hours (at our institution, we have an “ultrafast” MRI protocol to minimize radiation from CT scan for pediatric patients) MRI of the cervical spine; maintain hard collar NPO with dextrose containing IV fluids; if repeat imaging is stable, then can start diet If there is concern for seizures, can consider anti-epileptics Monitor for leakage from ears and nose

LEARNING POINTS

• Based on several large series, pediatric patients with simple skull fractures without any other associated intracranial pathology, including hemorrhage or pneumocephalus, can be safely discharged home from the emergency department if asymptomatic, nonaccidental trauma is not suspected, and parents are aware of return precautions.1-4 • Many patients with skull fractures sustain injuries to other areas of the body besides the skull. Most commonly, in order of frequency, these include intracranial hemorrhage (epidural, subdural, subarachnoid, or intraparenchymal), orthopedic injuries, facial fractures, and ophthalmological injuries.2 Skull base fractures with possible vessel involvement warrant additional vascular imaging. • Nonaccidental trauma should be considered in the differential based on the history (e.g. delays in seeking treatment, inconsistent stories) and physical exam (e.g. retinal hemorrhage, encephalopathy, irritability, vomiting, developmental delay) particularly in patients less than 18 months old. See Figure 52.2 for algorithm of care for pediatric isolated skull fractures adapted from • Donaldson et al.3

52—LOss Of COnsCiOusness After Bike ACCident

Pediatric patient presenting to ED with isolated skull fracture on imaging. ED evaluation and consult to neurosurgery

Is there concern for nonaccidental trauma (NAT) based on history and physical exam?

Yes

Recommend admission for NAT workup and social services

No Yes Are neurological deficits present? Is GCS < 15?

Further neurological workup as necessary

No Is the patient tolerating PO/feeds and otherwise at baseline?

No

Observe until emesis remits. If emesis persists, provide IV fluids for rehydration and consider admission.

Yes Following neurosurgical consult, may discharge patient to parents/guardians capable of monitoring the child for any changes in neurological status. Provide appropriate follow-up. Fig. 52.2 Algorithm for the care of pediatric isolated skull fractures (adapted from Donaldson et al.3).

References 1. Arrey EN, Kerr ML, Fletcher Jr S, C.S.C., Sandberg DI. Linear nondisplaced skull fractures in children: who should be observed or admitted? J Neurosurg Pediatr. 2015;16:703–708. https://doi.org/ 10.3171/201 5.4.PEDS1545. 2. Bonfield, CM, Naran S, Adetayo, OA, Pollack IF, Losee, JE. Pediatric skull fractures: the need for surgical intervention, characteristics, complications, and outcomes. J Neurosurg Pediatr. 14:205–211. 3. Donaldson K, Li X, Sartorelli KH, Weimersheimer P, Durham SR. Management of isolated skull fractures in pediatric patients a systematic review. Pediatr Emerg Care. 2019;35(4):301–308. 4. Kommaraju K, Haynes H, Ritter AM. Evaluating the role of a neurosurgery consultation in management of pediatric isolated linear skull fractures. Pediatr Neurosurg. 2019:21–27. https://doi.org/10.1159/000495792.

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53

Right Sided Jerking And Decreased Appetite Jose Luis Porras, MD n

Ann Liu, MD

Consult Page 2 month old with right sided jerking and decreased appetite

Initial Imaging

RP

A

B

Sp Til in: t: - 55 6

C

D

Fig. 53.1 Head CT without contrast demonstrates a left acute convexity subdural hematoma (A) extending to the falx and left temporal fossa (B), a right sided small falcine subdural hematoma (B), and a linear nondisplaced left parietal skull fracture (C, D).

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259

Walking Thoughts n

n n n n n n

Has the trauma team performed an initial assessment with attention given to the ABCs (airway, breathing, circulation)? What is the mechanism of injury? What is the child’s GCS and neurological status? Is there concern for elevated intracranial pressure? What is this child’s past medical history? Are there any risk factors for nonabusive causes or risk factors for intracranial bleeding? Is there concern for nonaccidental trauma?

History of Present Illness A 2 month old male (born 35 weeks gestational age via an uncomplicated vaginal delivery) presents to the emergency department with right sided jerking and decreased feeds. The child lives at home with his mother; his parents are separated. He was in his usual state of health when three days prior to presentation, his mother observed him having jerking movements of his right arm and leg. She also felt that he had been breast-feeding less. The weekend prior, he was in his father’s care who denied any behavioral changes or trauma. The father did notice that the patient’s left head was swollen, which he attributed to his son “growing.” While under his grandmother’s care yesterday, he was observed to also have drooling and additional episodes of right sided jerking, during which he made no eye contact with his grandmother. Due to persistent right sided jerking, his mother brought him to a local hospital today. In the emergency room, he had intermittent 2-minute episodes of right arm and leg jerking. A lorazepam and fosphenytoin load were administered, leading to resolution of these episodes. Head CT was obtained and demonstrated a left 5 cm nondisplaced, linear parietal skull fracture, an acute 6 mm left convexity subdural hematoma, and an acute 3 mm right parietal subdural hematoma without significant midline shift. He was transferred to our emergency department for further management.

Vital Signs T 36.8oC, HR 131 (normal 120–180), BP 89/42 (normal SBP 65–85), SpO2 100% on room air

Pertinent Labs Hgb 6.7, WBC 7.1, Plt 500, Na 143, Cr 0.2 INR 1.0, PT 10.6

Glasgow Coma Scale Motor: 6 (moving spontaneously, purposefully) Verbal: 3 (cries in response to pain) Eye opening: 2 (open to stimulation) GCS total: 11

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Physical Exam Left parietal scalp swelling and bruising Anterior fontanelle full, not tense Eyes closed but open to stimulation No gaze deviation Pupils equal, round, and reactive to light Face symmetric Moving bilateral upper and lower extremities symmetrically with good strength Multiple bruises of various ages on arms and legs

Triage Management In a 2 month old, who is not expected to be highly mobile, the patient has multiple signs and symptoms, and a history concerning for nonaccidental trauma (NAT). The mother provides no clear mechanism of injury without falls or accidents and sought out care in a delayed fashion. Currently, his seizures have been controlled with antiepileptics, and his neurological exam is reassuring. Given the small size of his bilateral subdural hematomas, surgery is not indicated at this time. However, he will require close monitoring in the pediatric intensive care unit given his recent seizures and the possibility of subdural hematoma expansion. His care will require consultation with neurology for his seizures, ophthalmology to evaluate for retinal hemorrhages, and social work for NAT workup. This is an urgent and nonoperative consult.

Assessment This is a 2 month old boy presenting with seizures and imaging findings concerning for nonaccidental trauma, including a 5 cm left nondisplaced, linear parietal skull fracture and left greater than right subdural hematomas, with no clear mechanism of injury.

Plan n n

n

n n n n n n

Admission to pediatric intensive care unit with frequent neurological assessments Implement institutional nonaccidental trauma protocol n Hard cervical collar until cervical spine injury can be ruled out n Skeletal survey Complete coagulation profile to evaluate for coagulopathy and other causes of the intracranial bleed Brain and cervical spine MRI without contrast (Figure 53.2) Interval brain imaging to assess for subdural hematoma stability Consultation to neurology for seizure management Consultation to ophthalmology to evaluate for retinal hemorrhages Consultation to social work Maintain NPO status and maintenance fluids

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Fig. 53.2 MRI brain without contrast diffusion sequence demonstrates extensive diffusion restriction of the left frontal, temporal, parietal, and occipital lobes, compatible with acute infarction. When taken together with the patient’s bilateral subdural hematomas, this is concerning for nonaccidental trauma.

LEARNING POINTS

• Shaken baby syndrome, now referred to as abusive head trauma, occurs when the baby’s head experiences angular accelerations and decelerations in a whiplash-like fashion.1

• Approximately 10% of children less than 10 years of age who present to the ED after an alleged accident are in fact victims of child abuse.1 Abusive head trauma most commonly occurs in infants younger than two years old with the highest incidence in the first several months of life.5 • Risk factors for abusive head trauma include perinatal illness and prematurity. Risk factors for perpetrating abuse include parental age, parents having experienced childhood abuse, psychiatric disorders, and unstable family dynamics.5 • Suspicion for child abuse may be raised when findings include retinal hemorrhage, bilateral chronic subdural hematomas, multiple skull fractures (or those associated with intracranial injury), and significant neurological injury with minimal external trauma. • Victims of abusive head trauma often present with seizures, respiratory compromise including apnea, lethargy, irritability, emesis, and poor feeding.2 • Skull fractures in the context of child abuse involve the parietal bone in up to 90% of cases.3 • Characteristics of skull fractures concerning for NAT include multiple fractures, bilateral fractures, and those crossing sutures. • Retinal hemorrhage is considered to be near pathognomonic of battering in the traumatized child with multiple injuries and without a consistent history. It may also be seen in benign subdural effusion in infants, acute high-altitude sickness, acute intracranial pressure increases, or Purtscher’s retinopathy (loss of vision after major trauma).2

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• Consideration should also be given to other causes or risk factors for intracranial hemorrhage including coagulopathies (hemophilia, vitamin K deficiency), osteogenesis imperfecta, birth trauma, congenital vascular malformations, and rare metabolic deficiencies.2 • Initial management of a suspected victim of abusive head trauma includes establishing and closely following GCS while also assessing for signs of increased ICP. Medical and surgical treatment is identical to the treatment of any other severe traumatic brain injury. • Reported mortality rates of abusive head trauma are as high as 35%. Clinical features associated with fatal child abuse include initial GCS of 3–5, retinal hemorrhage, intraparenchymal hemorrhage, or cerebral edema. Morbidity is significant with many patients experiencing long-term deficit in neurocognitive functioning.4 Death is most often due to intracranial hypertension.

References 1. Duhaie AC, Gennarelli TA, Thibault LE, Bruce DA, et al. The shaken baby syndrome: a clinical, pathological, and biomechanical study. J Neurosurg. 1987;66:409–415. #2. 2. Eisenbrey AB. Retinal hemorrhage in the battered child. Childs Brain. 1979;5:40–44. #3. 3. Meservy CJ, Towbin R, McLaurin RL, et al. Radiographic characteristics of skull fractures resulting from child abuse. AJR. 1987;149:173–175. 4. Shein SL, Bell MJ, Kochanek PM, et al. Risk factors for mortality in children with abusive head trauma. J Pediatr. 2012;161(4):716–722. 5. Vinchon M, Defoort-Dhellemmes S, Desurmont M, Dhellemmes P. Accidental and nonaccidental head injuries in infants: a prospective study. J Neurosurg. 2005;102:380–384. 12.

C H A P T E R

54

Numbness And Weakness After Head Collision Ann Liu, MD

Consult Page Young male football accident. Heard a pop in neck. Now with decreased strength/sensation on right side.

Initial Imaging

A

B

C

Fig. 54.1 Midline (A), right side (B), and left side (C) sagittal CT of the cervical spine without contrast was completed at the time of initial consultation and demonstrated mild reversal of normal cervical lordosis but without fracture (T-spine and L-spine are not shown but were negative for fracture or abnormality).

Walking Thoughts What is the patient’s neurological exam? What was the mechanism of injury? n Does the patient have any other injuries? Has a trauma assessment been completed? n Is he wearing a cervical collar? n What imaging has been completed? n n

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History of Present Illness A 15 year old male with no significant past medical history presents to the emergency room after being hit in the head and neck while playing football. The patient and his parents report that he was playing as the quarterback when two of his colleagues hit him on the right side of his head and neck while going full speed. His head whipped to the left side, and he immediately felt neck pain and some back pain. He also subsequently had a sensation of weakness in his upper extremities. He fell to the ground and had head strike without loss of consciousness. He was initially able to stand and took a few steps but then fell back to the ground. Currently he reports some numbness in his right arm and shooting pains down to his right elbow. He also has numbness in his legs but has not felt pain or weakness in his lower extremities. He has had no bowel or bladder incontinence and no saddle anesthesia. He is not on aspirin or anticoagulation. CT scans of his cervical, thoracic, and lumbar spine were unremarkable. Trauma assessment was negative for other injuries.

Vital Signs T 37.2oC, HR 87, BP 114/55, SpO2 96% on room air

Physical Exam Alert, awake Oriented to self, hospital, and date and year Hard cervical collar in place, endorsing neck pain Deltoid

Biceps

Triceps

Wrist Flexion

Wrist Extension

Grip

RUE

4/5

4/5

4/5

4-/5

4-/5

3/5

LUE

4/5

4/5

4/5

4-/5

4-/5

3/5

Hip Flexion

Knee Extension

Knee Flexion

Dorsiflexion

Plantar Flexion

RLE

4+/5

4/5

4/5

3/5

3/5

LLE

4+/5

4/5

4/5

3/5

3/5

Right arm and chest: decreased sensation in C5 to T5 distribution Right leg: decreased sensation throughout (in a nondermatomal distribution) compared to the left leg Rectal tone intact No clonus, no Hoffman’s sign No hyperreflexia

Triage Management The patient appears to have signs and symptoms concerning for spinal cord injury with pain, numbness, and weakness in his arms and legs. He is hemodynamically stable, and his assessment by the pediatric trauma team is otherwise negative. He has a hard collar in place, and he should continue to have his neck immobilized. He will need further imaging to evaluate for spinal cord injury and admission for close monitoring. This is an urgent but nonoperative consult.

n

265

Assessment This is a 15 year old male with no significant past medical history who presents with extremity weakness and numbness after being hit in the neck while playing football. He has some weakness in all extremities but is at least antigravity in all muscle groups; his sensation is decreased on the right side. The CT scan of his entire spine was negative for any fractures or abnormalities. His clinical picture is concerning for spinal cord injury without radiographic abnormality (SCIWORA) secondary to cervical hyperextension injury.

Plan n n n n n

Admit to neurosurgical intensive care unit for observation and neurological checks Monitor for bowel/bladder dysfunction Maintain hard cervical collar and strict spinal precautions at all times MRI pan-spine without contrast XR flexion-extension films of the cervical spine

Follow-Up The patient underwent pan-spine MRI, which was negative for ligamentous injury, hematoma, fracture, or cord signal change (Fig. 54.2). The flexion-extension x-rays of his cervical spine did not demonstrate instability or abnormal mobility (Fig. 54.3). Within 24 hours, his strength slowly improved, and he was downgraded to the floor. He underwent physical therapy evaluation and was discharged with outpatient follow-up in one week and repeat flexion-extension x-rays of the cervical spine. He had strict instructions to maintain his hard cervical collar with no return to play.

A

B

Fig. 54.2 Sagittal T2-weighted (A) and STIR (B) MRI of the cervical and upper thoracic spine without contrast demonstrated no ligamentous injury, hematoma, fracture, or cord signal change.

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A

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B

C

Fig. 54.3 Neutral (A), flexion (B), and extension (C) lateral x-rays of the cervical spine demonstrated no dynamic instability.

LEARNING POINTS

• Spinal cord injury without radiographic abnormality (SCIWORA) was first defined by Pang and Wilberger in 1982 as “objective signs of myelopathy as a result of trauma, whose plain films of the spine, tomography, and occasionally myelography carried out at the time of admission showed no evidence of skeletal injury or subluxation.”1 • SCIWORA has a higher incidence in children, particularly those less than 9 years of age, which is thought to be due to increased ligamentous elasticity and hypermobility of the juvenile vertebral column.1 • 2013 Congress of Neurological Surgeons Trauma Guidelines with Level 3 recommendations for evaluation and treatment:3 • MRI is recommended in the region of suspected neurological injury. • Flexion-extension radiographs are recommended in the acute setting and at late followup (even if MRI is negative) to assess for spinal stability. • Spinal angiography and myelography are not recommended. • External immobilization is recommended for up to 12 weeks. • Noncontact and contact sports (“high-risk activities”) should be avoided for up to six months. • Delayed neurological deterioration can occur several days after trauma, and thus, children should be monitored closely, and parents counseled appropriately. There are no reports of children who have developed spinal instability following the diagnosis of SCIWORA. • SCIWORA was defined in an era where MRI was not as readily available for evaluation and diagnosis. In modern times, MRI findings in children with SCIWORA can range from normal to complete cord disruption.2 • Prognosis appears to be strongly related to the presenting neurological examination and MRI findings. Overall, patients with normal MRI have been found to have an excellent prognosis.2

References 1. Pang D, et al. Spinal cord injury without radiographic abnormalities in children. J Neurosurg. 1982;57:114–129. 2. Pang D. Spinal cord injury without radiographic abnormalities in children, 2 decades later. Neurosurg. 2004;55:1325–1343. 3. Rozzelle CJ, et al. Spinal cord injury without radiographic abnormality (SCIWORA). Neurosurg. 2013;72:227–233.

C H A P T E R

55

Worsening Leg Pain And Weakness Ryan P. Lee, MD n

Ann Liu, MD

Consult Page 9 year old male history of tethered cord with worsening leg weakness/pain

Initial Imaging

Fig. 55.1 Sagittal T2-weighted lumbar spine MRI without contrast shows post-surgical changes of his prior lumbar laminectomy, partial sacral agenesis, low lying tethered cord, and syringohydromyelia. These findings are unchanged from a year prior when the patient was asymptomatic.

Walking Thoughts What is the patient’s medical history? What is his tethered cord history? Has he had prior detethering or related surgeries? n What are the patient’s symptoms (weakness, pain, bowel/bladder dysfunction)? n How long has the patient been having symptoms? n What type of imaging is available? What prior imaging is there to compare to? n n

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History of Present Illness This is a 9 year old boy with a history of lumbosacral lipoma, tethered cord, and prior lumbar laminectomy for detethering as an infant who presents with progressive right lower extremity weakness and left lower extremity pain. He is normally active in sports but has been developing right leg weakness for several months. More recently, he has had left leg pain with activity. He now has an abnormal gait when running. His parents have noticed that his right foot is turning inward as well. He denies any changes in sensation, bowel function, or bladder function. Born at 37 weeks gestation, he had an uncomplicated birth but was found to have hypospadias, a sacral dimple, and sacral Mongolian spots. He eventually underwent MRI of the lumbar spine, which showed a low conus medullaris at L5-S1, a small lipoma in that area, hydrosyringomyelia, posterior bony segmentation anomalies, and a dorsal dermal sinus tract terminating at the sacral dimple. At 7 months of age, he underwent lumbosacral laminectomy for cord release, lipoma debulking, and dermal sinus tract excision. He did quite well afterward, becoming very active in sports until his current presentation.

Vital Signs T 36.7°C, HR 80, BP:113/64, SpO2 99% on room air

Physical Exam Alert and oriented, interactive, appropriate for age Normocephalic Pupils equal, round, and reactive to light Bilateral upper extremities full strength throughout

RLE LLE

Hip Flexion 5/5 5/5

Knee Extension 5/5 5/5

Knee Flexion 5/5 5/5

Dorsiflexion 4+/5 5/5

Plantar Flexion 4+/5 5/5

Eversion 4+/5 5/5

Right foot turning inward during gait and on tip-toes Sensation to light touch intact throughout Patellar reflexes 2+ bilaterally No clonus bilaterally No upgoing toes Prior posterior lumbar incisions well-healed without concern for infection

Triage Management This is not an emergent presentation. The patient has been developing symptoms over the course of months, and surgical intervention is not acutely necessary. However, there is enough evidence to favor a diagnosis of tethered cord syndrome and to begin to plan for surgical exploration and cord release in the timeframe of weeks to months. Earlier intervention, when symptoms are mild, is associated with increased chance of reversibility of deficits.1 While the patient does have a syrinx in the lumbosacral cord, it is stable and would have been expected to increase in size if this alone were causal for his symptoms. If his syrinx had increased in

55—Worsening Leg PAin And WeAkness

269

size and was thought to be contributing to his symptoms, then surgical treatment of the syrinx (e.g. syringo-subarachnoid shunting) may need to be considered. This is a nonurgent but operative consult.

Assessment This is a 9 year old male with a history of lumbosacral lipoma resection, cord detethering, and lumbar hydrosyringomyelia, who presents with progressive right lower extremity weakness and left lower extremity pain concerning for tethered cord syndrome. MRI of the lumbar spine shows similar findings to an MRI from a year and a half prior when the patient was asymptomatic. However, tethered cord syndrome is a clinical diagnosis, and his worsening symptoms warrant surgical consideration.

Plan No acute neurosurgical intervention indicated Check a post-void bladder residual to ensure that the patient is not retaining urine n Outpatient urodynamic studies n Pain management by the emergency room n Outpatient physical and occupational therapy n Follow-up as an outpatient to plan surgery at next available opportunity n n

LEARNING POINTS

• Tethered cord, an abnormal attachment of the spinal cord to surrounding tissues, is an anatomical and structural finding, usually associated with spinal dysraphism. When clinically symptomatic, it is called tethered cord syndrome. • The symptomatology of tethered cord syndrome is thought to be stretch-induced6 and its presentation is age-dependent:4 • Children under 10 years of age typically present with gait difficulties, foot deformities, or incontinence. • Teenagers more typically develop scoliosis or incontinence. • Adults typically present with pain, often perineal in location. • MRI is the radiographic modality of choice for diagnosis and follow-up. Typical findings on MRI include low-lying conus, thickened (>2 mm) or fatty filum terminale, posterior filum location, distal cord syrinx, occult posterior element bony defects, and any classic dysraphic anomaly.2 • The imaging may not necessarily change at onset or progression of symptoms, as MRI cannot necessarily show the degree of stretch. • Tethered cord syndrome should be suspected in myelomeningocele patients if there is increasing spasticity, increasing scoliosis, worsening gait, or deteriorating urodynamics.3 • If there is history of myelomeningocele, there is likely to be radiographic evidence of tethering, whether or not the patient is symptomatic. • In myelomeningocele patients with a shunt and/or Chiari II malformation, shunt malfunction and/or brainstem compression should be considered first when symptomatic. • Symptoms thought to be from tethering-induced stretch (e.g. from tight filum terminale or caudal lipomyelomeningocele) are most likely to improve with detethering, as opposed to symptoms thought to be from structural cord damage such as local compression or ischemia, which may persist despite detethering.5

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References 1. Hoving E. Pathophysiology and management of tethered cord (including myelomeningocele). In: Jallo G, Kothbauer K, Recinos V, eds. Handbook of Pediatric Neurosurgery. New York, NY: Thieme; 2018. 2. Hinshaw DB, Jacobson JP, Hwang J, Kido DK. Imaging of tethered spinal cord. In: Yamada S, ed. Tethered Cord Syndrome in Children and Adults. 2nd ed. New York, NY: Thieme; 2010:51–64. 3. Park TS, Cail WS, Maggio WM, et al. Progressive spasticity and scoliosis in children with myelomeningocele: radiological investigation and surgical treatment. J Neurosurg. 1985;62:367–375. 4. Schneider S. Neurological assessment of tethered spinal cord. In: Yamada S, ed. Tethered Cord Syndrome in Children and Adults. 2nd ed. New York, NY: Thieme; 2010:43–50. 5. Yamada S, Won DJ. What is the true tethered cord syndrome? Childs Nerv Syst. 2007;23(4):371–375. 6. Yamada S, Zinke DE, Sanders D. Pathophysiology of “tethered cord syndrome.” J Neurosurg. 1981;54:494–503.

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56

Persistent Headaches Lydia Ju-mi Bernhardt, MD n

Ann Liu, MD

Consult Page 13 y/o male, history of “brain cysts”, here with headache, nausea, vomiting

Initial Imaging None obtained prior to consult

Walking Thoughts n n n

n n n

What is the patient’s GCS and neurological exam? What is his prior history of “brain cysts”? Does the patient have any signs or symptoms of increased intracranial pressure (ICP) or mass effect from the cyst? Where in the brain is the cyst? How big is the cyst? What prior imaging is available? What is the chronicity of the cyst? Has the cyst ruptured or hemorrhaged? If so, does he need any surgical intervention?

History of Present Illness A 13 year old boy, with a history of “brain cysts” diagnosed in Romania, presents to the emergency department with 3 days of persistent holocephalic headaches with one episode of nausea and vomiting the night prior to presentation. His current headaches are similar to his chronic baseline headaches, which are not positional, and are associated with photophobia and nausea. He denies numbness, weakness, additional episodes of nausea or vomiting, vision changes, diplopia, seizurelike activity, or loss of consciousness. A prior MRI of his brain obtained at another hospital over one year ago demonstrated a 4 × 3 cm left middle cranial fossa arachnoid cyst with mild mass effect but no midline shift, hydrocephalus, or vasogenic edema.

Vital Signs T 37.1 °C, HR 82, BP 126/73, SpO2 99% on room air

Glasgow Coma Scale Motor: 6 Verbal: 5 Eye opening: 4 GCS Total: 15 271

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Physical Exam Alert, awake, oriented to self, hospital, and year Pupils equal, round, and reactive to light No papilledema Visual fields intact to confrontation Extraocular movements intact, face symmetric, tongue midline No pronator drift Bilateral upper extremities 5/5 Bilateral lower extremities 5/5 Sensation intact to light touch throughout

Triage Management The patient presents with persistent headaches in the setting of a known arachnoid cyst that was previously imaged over a year ago. His current headaches are similar to his baseline headaches, and it is unclear whether they are related to the arachnoid cyst. He does not have other signs or symptoms of increased ICP. Updated imaging will allow for evaluation of interval changes and provide a better understanding of whether his symptoms are related to the cyst. Depending on the imaging findings and whether his symptoms are felt to be secondary to those changes, a discussion about the risks and benefits of surgical intervention will need to occur with the family, as arachnoid cyst surgery for headaches alone does not always relieve symptoms.

Assessment This is a 13 year old boy with a known left temporal arachnoid cyst who presents with three days of persistent holocephalic headaches with one episode of nausea and vomiting. He is back to his baseline, and his exam is reassuring. His headaches are concerning for possible migraines; however, without further imaging, we cannot rule out that his symptoms may be secondary to changes in the arachnoid cyst. This is a nonurgent consult.

Plan n n n n

No acute neurosurgical intervention at this time MRI brain with and without contrast If MRI shows stable arachnoid cyst, then patient can follow-up in clinic Headache management

Follow-Up Repeat MRI brain with and without contrast (Figure 56.1) in the ED demonstrates stable size and appearance of the patient’s known arachnoid cyst without hemorrhage or increased mass effect on adjacent parenchyma. He received pain medications with resolution of his headache. He was discharged from the ED and his parents were counseled on return precautions.

273

56—Persistent HeadacHes

A

B

Fig. 56.1 MRI brain with and without contrast. A. Axial T2-weighted image demonstrates a 4 × 3 cm extraaxial cystic lesion within the anterior left middle cranial fossa, isointense to CSF. There is no mass effect, parenchymal vasogenic edema, hydrocephalus, or midline shift. B. Sagittal T1-weighted image with contrast demonstrates no contrast enhancement.

LEARNING POINTS

• Primary intracranial arachnoid cysts (IACs) arise during development from the splitting of the arachnoid membrane. IACs are typically asymptomatic and are generally incidental findings. They are identified in 2.6% of children, compared to 1.4% of adults. In a large pediatric series, 6.8% of cysts were symptomatic.1 Conservative versus surgical management of arachnoid cysts is controversial, and depends on a number of factors, including whether a patient is symptomatic and the size of the cyst.3 • Signs and symptoms are dependent on location but do not always correlate with size. 50–60% of arachnoid cysts are found in the middle cranial fossa and are classified according to the Galassi system:3 • Type I: small, spindle-shaped cyst limited to the anterior portion of the middle cranial fossa with no mass effect; free communication with subarachnoid space on CT cisternogram • Type II: involves the proximal and intermediate segments of the Sylvian fissure, displaces the temporal lobe; partial communication with subarachnoid space on CT cisternogram • Type III: large cyst that fills the middle cranial fossa, displacing the temporal, frontal, and parietal lobes; little communication with subarachnoid space on CT cisternogram Other common locations include the posterior fossa and suprasellar region. • IACs may present with symptoms of elevated ICP (such as headaches, nausea, vomiting, and lethargy), seizures, focal signs or symptoms of a space occupying lesion, progressive macrocephaly, or a focal enlargement of the skull. Cysts in the suprasellar region may additionally present with hydrocephalus, visual impairment, and endocrinopathies including precocious puberty. • Occasionally, hemorrhage into the cyst or rupture of the cyst itself resulting in subdural CSF hygromas or overt subdural hemorrhage can occur, leading to sudden clinical deterioration. • Surgical intervention is recommended for clearly symptomatic cysts, for example those causing hydrocephalus, neurological deficit, focal seizures, or cosmetic deformity.

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Surgery is controversial for cysts that are 1) questionably symptomatic, for example in those patients with headaches or nonlocalized seizures; or 2) large and asymptomatic, as there is a theoretically increased risk of perioperative hemorrhage into large IACs. Small, asymptomatic cysts can be observed. • Surgical treatment options for IACs include 1) drainage by needle aspiration or burr hole evacuation, 2) excision and fenestration of the cyst into basal cisterns via craniotomy, 3) endoscopic cyst fenestration through a burr hole, or 4) placement of a shunt to drain and close the cyst.2-4 Despite surgery, cyst recurrence is common. Shunt placement has high rates of cyst elimination but are limited by shunt-related complications.2 Of note, headaches experienced by patients with arachnoid cysts often persist even after adequate surgical treatment of the cyst.1

References 1. Al-Holou W, Yew A, Boomsaad Z, Garton H, Muraszko K, Maher C. Prevalence and natural history of arachnoid cysts in children. J Neurosurg Pediatr. 2010;5:578–585. https://doi.org/10.3171/2010.2.P EDS09464. 2. Ali ZS, Lang S, Bakar D, Storm PB, Stein SC. Pediatric intracranial arachnoid cysts: comparative effectiveness of surgical treatment options. Childs Nerv Syst. 2014:461–469. https://doi.org/10.1007/ s00381-013-2306-2. 3. Jafrani R, Raskin JS, Kaufman A, Lam S. Intracranial arachnoid cysts: pediatric neurosurgery update. Surg Neurol Int. 2019. https://doi.org/10.4103/sni.sni. 4. Mustansir F., Bashir S., & Darbar A. (20158). Management of arachnoid cysts: a comprehensive review. Cureus. 2018;10;10(4):e2458. https://doi.org/10.7759/cureus.2458.

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57

Fever And Forehead Swelling Jose Luis Porras, MD n

Ann Liu, MD

Consult Page 7 y/o male with 2 weeks of fever and 1 day of acute midline forehead swelling

Initial Imaging

R

Fig. 57.1 Axial head CT without contrast demonstrates left paranasal sinusitis marked by opacification of the frontal sinus, ethmoid air cells, and sphenoid sinus with extension of infection/inflammation into the frontal extracranial soft tissues.

Walking Thoughts n n n n

n n

What is the patient’s GCS and neurological status? What are the patient’s vitals? Does the patient have any risk factors for sinus infection? Does the patient have any signs or symptoms of orbital involvement, intracranial involvement, meningitis, or encephalitis? Has the patient been on any antibiotics? What other services (otolaryngology, ophthalmology) have seen the patient?

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A

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B

C

Fig. 57.2 Axial MRI of the brain. A. T2-weighted sequence without contrast demonstrates opacification of the frontal sinus and ethmoid air cells. Of note, the patient has a left temporal arachnoid cyst. B. T2-weighted sequence without contrast demonstrates a small right frontal epidural fluid collection. C. T1-weighted sequence with contrast demonstrates the right frontal epidural fluid collection with reactive pachymeningeal enhancement of the frontal lobes.

History of Present Illness A 7 year old male with no significant past medical history presents to the emergency department (ED) with two weeks of fever, fatigue, headache, and one day of sudden forehead swelling. The patient’s mother states that approximately two weeks ago, he developed nausea, vomiting, fevers, and headache, which were managed conservatively under the assumption that he had the flu. His nausea and vomiting improved, but the fevers and headache persisted. After a week of no improvement, he was seen at a local ED where he was prescribed azithromycin for sinusitis after monospot, influenza, and rapid strep testing were negative. Yesterday, he awoke from a nap with new midline forehead swelling prompting a repeat visit to a local ED, where head CT demonstrated severe left sinusitis with soft tissue swelling of the forehead and epidural abscess. Ceftriaxone, vancomycin, and metronidazole were initiated, and he was transferred to our institution for further management. Currently, the patient has a headache but denies nausea, vomiting, difficulty swallowing, neck stiffness, vision changes, or nasal drainage.

Vital Signs T 36.5°C, HR 108 (normal 70–110), RR 22 (normal 18–30), BP 97/51 (normal SBP 90s–110s), SpO2 98% on room air

Pertinent Labs Na 140, Cr 0.3, Hgb 9.6, WBC 15.66, Plt 332, INR 1.1, aPTT 24.5 ESR 79, CRP 5.5

Glasgow Coma Scale Motor: 6 Verbal: 5 Eye opening: 4 GCS total: 15

57—Fever And ForeheAd Swelling

277

Physical Exam Boggy midline forehead swelling Alert, oriented to self, hospital, and year Pupils equal, round, and reactive to light Extraocular movements intact, symmetric smile, tongue midline No pronator drift Bilateral upper extremities 5/5 Bilateral lower extremities 5/5 Sensation intact to light touch Negative Kernig’s and Brudzinski’s signs

Triage Management The patient presents with progressive sinus infection for the past two weeks that now has intracranial extension despite prior antibiotic treatment for his sinusitis. Fortunately, he is neurologically intact, without severe symptoms of meningitis or encephalitis. His epidural abscess is small and therefore he will not require emergent operative intervention but should undergo surgery soon. In the meantime, he will need to be closely monitored for possible expansion of the epidural abscess, seizures and/or sepsis, and continuation of broad-spectrum antibiotics. Otolaryngology and/or plastic surgery can be consulted for multidisciplinary operative planning, and infectious disease will be contacted for antibiotic recommendations. This is an urgent and operative consult.

Assessment This is a 7 year old male with no significant past medical history presenting with two weeks of fever, fatigue, headache, and one day of sudden forehead swelling with imaging findings concerning for sinusitis, soft tissue swelling, and epidural abscess, all suggestive of Pott’s puffy tumor.

Plan n n

n n n n n n

Admission to the pediatric intensive care unit for close neurological monitoring Obtain MRI of the brain with and without contrast to better assess the extent of intracranial infection Consultation to otolaryngology and/or plastic surgery Consultation to infectious disease Continue broad-spectrum IV antibiotics Keep NPO with maintenance fluids in anticipation of operative intervention Pre-operative labs, including type and screen; reserve 2 units of red blood cells for surgery Urgent surgery for abscess evacuation, sinus exenteration, irrigation, and debridement n Posting highlights: n Intraoperative culture swabs n Antibiotic irrigation n For anesthesia: broad-spectrum IV antibiotics

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LEARNING POINTS

• Pott’s puffy tumor is a subperiosteal abscess of the anterior wall of the frontal sinus with underlying frontal bone osteomyelitis.2 Infection can spread through direct erosion from the frontal sinus or by migration of septic emboli through the diploic veins that drain the mucosa of the frontal sinuses.3 • Mild symptoms include headache, fever, periorbital swelling, and nasal discharge (which may or may not be purulent).1,3 • Complications may include meningitis; epidural, subdural, and/or intracerebral abscess; and venous sinus thrombosis. Symptoms of lethargy, altered mental status, seizures, nausea, and vomiting are concerning for intracranial involvement,1 which occurs in roughly 72% of cases.2 • CT is the initial imaging modality of choice and may demonstrate bony resorption, periosteal reaction, and contrast enhancement. • The most common organisms are Streptococci species followed by Staphylococci species.1,3 Negative intraoperative cultures are likely due to preoperative antibiotic use.3 • The goals of surgery include draining the abscess, debridement of osteomyelitic bone, and removal of granulation tissue from the dura to prevent the spread of infection.3 If a craniotomy is required, cranioplasty with titanium mesh may be performed at time of initial debridement or deferred.1 With the advent of antibiotics, mortality has decreased from 60% to 3.7%.3 •

References 1. Bambakidis NC, Cohen AR. Intracranial complications of frontal sinusitis in children: Pott’s puffy tumor revisited. Pediatr Neurosurg. 2001;35:82–89. 2. Flamm ES. Percivall Pott: an 18th-century neurosurgeon. J Neurosurg. 1992;76:319–326. 3. Koltsidopoulos P, Papageorgiou E, Skoulakis C. Pott’s puffy tumor in children: a review of the literature. Laryngoscope. 2018;2018:20.

C H A P T E R

58

Gait Difficulty And Falls Ann Liu, MD

n Dimitrios Mathios, MD

Consult Page 73F with NPH, unable to ambulate.

Initial Imaging

P Fig. 58.1 An axial head CT without contrast demonstrates mildly enlarged ventricles with a right frontal approach ventricular catheter terminating in the body of the left lateral ventricle. In comparison with prior imaging, this is stable (not shown).

Walking Thoughts What is the patient’s neurological exam? Why is she unable to ambulate? n Does she have any other medical comorbidities? n Is the patient having any other symptoms? n What is the timeline of her symptoms? n What is her normal pressure hydrocephalus (NPH) history? n

280

58—GAit DiFFiculty AnD FAlls

281

Fig. 58.2 XR shunt series demonstrates a Certas valve set to 5. Abdominal XR demonstrates no kinks or disconnections of the distal shunt tubing (not shown).

n

n

n n n

Does the patient have a shunt? If so, when was the shunt placed and what type of shunt does the patient have? Has the shunt ever been revised? If so, when was it last revised, and what was the indication for revision? Is the patient having a shunt malfunction? Does the patient need surgery, and if so, what is the timing of surgery? Is the patient taking any antiplatelet or anticoagulant medications?

History of Present Illness A 73 year old female with a history of normal pressure hydrocephalus with a right ventriculoperitoneal shunt placed one year prior, diabetes, and hypertension presents to the emergency department (ED) with worsening gait and increased falls. She had initially presented to neurology clinic two years prior for evaluation of NPH after experiencing a year of progressive gait difficulties, imbalance, urinary incontinence, and memory difficulties. She underwent a large volume lumbar puncture (LP) and underwent pre- and post-drainage tests including: Montreal Cognitive Assessment, digit symbol substitution test, 10 meter walk time, Timed Up and Go (TUG) test, dual TUG test, six minute walk distance, Modified Clinical Test of Sensory Interaction in Balance, and Mini-Balance Evaluation Systems Test (BEST). She had significant improvement in 7 out of 8 tests, and she and her family wished to proceed with shunting. She underwent a right ventriculoperitoneal shunt placement one year prior with a Certas valve set at 5. At her 6 month follow-up after her initial shunt placement, she had improvement of her gait and memory. One week prior to her current ED admission, she noted difficulty walking. Earlier today, she had two falls without head strike. She denies any urinary incontinence, new confusion, headaches, weakness, numbness, or tingling. Her family, who is at bedside, states that she is otherwise at her neurological baseline. She has never had a shunt malfunction or revision surgery before. She is not on any anticoagulant or antiplatelet medications.

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Vital Signs T 37.2°C, HR 67, BP 125/53, SpO2 100% on room air

Pertinent Labs WBC 5.3, urinalysis negative for urinary tract infection

Physical Exam Awake, alert Oriented to self, hospital, and year with choices (baseline) Pupils equal and reactive to light Extraocular movements intact Face symmetric, tongue midline No pronator drift Full strength in all extremities Scalp and abdomen incisions clean, dry, and intact, without erythema or underlying swelling Shunt interrogated at bedside, confirming a Certas setting of 5

Triage Management This patient has a shunt for NPH and presents with symptoms that could be related to her shunt. Her white count is normal, and she does not have a urinary tract infection. On examination, she is at her cognitive baseline without focal deficits. Her head CT demonstrates stable ventricular size, and X-rays of her shunt confirm a Certas setting of 5 without kinks or disconnections. Her symptoms could be due to a shunt malfunction or inadequate drainage. In order to rule out a shunt malfunction, a shunt tap (see Chapter 77 for procedural details) was performed at bedside with spontaneous flow, an opening pressure of 15, and natural pulsations. This indicates that her shunt is working, and her symptoms may be due to inadequate drainage. This is a nonurgent, nonoperative consult.

Assessment This is a 73 year old female with a history of NPH with a right ventriculoperitoneal shunt without revision who presents with worsening gait and increased falls. Head CT shows stable ventricular size compared to prior imaging. A shunt tap was performed at bedside, ruling out a shunt malfunction as the cause of her symptoms. Her shunt was adjusted from a setting of 5 down to 4.

Plan n n n

Certas valve adjustment from a setting of 5 to 4 Discharge home with family who have been counseled on return precautions Follow-up in clinic in 1–2 weeks

Follow-Up The patient was seen in clinic in 2 weeks with marked improvement in her gait. She will be followed closely to monitor for overdrainage.

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LEARNING POINTS



References 1. Factora R, et al. NPH: diagnosis and new approaches to treatment. Clin Geriatr Med. 2006;22(3):645– 657. 2. Hakim S, et al. The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure: observations on cerebrospinal fluid hydrodynamics. J Neurol Sci. 1965;2:307–327. 3. Halperin JJ, et al. Practice guideline: idiopathic NPH: response to shunting and predictors of response: report of the guideline development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2015;85(23):2063–2071. 4. Marmarou A, et al. The value of supplemental prognostic tests for the preoperative assessment of idiopathic normal-pressure hydrocephalus. Neurosurgery. 2005;57(3):S17–S28. 5. Rocque BG, et al. Ventricular shunt tap as a predictor of proximal shunt malfunction in children: a prospective study. J Neurosurg Pediat. 2008;1(6):439–443.

C H A P T E R

59

Emesis And Lethargy In A Shunted Patient Tej D. Azad, MD/MS n

Dimitrios Mathios, MD

Consult Page 13yo M hx VP shunt with fall, now with emesis and lethargy

Initial Imaging

A

B

Fig. 59.1 X-ray shunt series. A. Lateral view of the skull demonstrates the right proximal shunt catheter and a Strata valve. The distal catheter is not present. B. Anteroposterior view of the abdomen shows the distal shunt tubing coiled in the pelvis. Of note, the patient’s G-tube is seen in the upper left quadrant.

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A

285

B

Fig. 59.2 Axial T2-weighted brain MRI without contrast (ultrafast sequences). A. Imaging from one year prior demonstrates small ventricles. B. Current imaging in the ED demonstrates significantly enlarged ventricles compared to prior.

Walking Thoughts n n n n n

n

n n

n n

What is the patient’s neurological exam and current vitals? What are the patient’s symptoms? What is the timeline of symptoms? Why does the patient have a shunt? What type of shunt is currently in place? What is the patient’s shunt history? n When was the shunt placed? n Has the patient ever required a shunt revision and if so, when was the last one? n Has the patient ever had a shunt malfunction? If so, what symptoms did the patient have at that time? n Was the shunt setting recently changed? Is there prior imaging of the patient’s ventricles? If so, does the patient’s ventricles change in size with prior shunt malfunctions? Does the patient need an urgent bedside procedure (e.g. shunt tap)? Does this patient need to go to the operating room for a shunt revision? If so, what is the timing of surgery? Does the patient have other medical comorbidities? Is the patient on any antiplatelet or anticoagulant medications?

History of Present Illness A 13 year old male with Joubert syndrome, developmental delay, Dandy-Walker malformation, and congenital hydrocephalus with a right parietal ventriculoperitoneal shunt (VPS) presents

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to the emergency department after a fall from his bed with subsequent emesis and progressive lethargy. The fall was unwitnessed, and he was found on the floor next to his home hospital bed by his parents. His mother reports that he tolerated feeds through his gastrostomy tube earlier in the day but has had nothing by mouth for the past 10 hours. His mother denies any focal deficits, seizure-like activity, or fevers. At baseline, he is vocal but nonverbal, nonambulatory, and has a dysconjugate gaze. He initially underwent shunt placement at 2 years of age due to increasing head circumference secondary to congenital communicating hydrocephalus. He has had three distal shunt revisions due to malfunctions; his most recent revision was one year prior due to a disconnect in the distal catheter. His prior shunt failures have been characterized by nausea, emesis, headache, and lethargy, as well as increased ventricular size on imaging. His current valve is a Strata set at 1.5, and he has not had any recent adjustments. He is not on any antiplatelet or anticoagulant medications.

Vital Signs T 36.8°C, BP 106/59, HR 50s to 70s, SpO2 97% on room air

Pertinent Labs Na 138, WBC 6.6, Plt 183, INR 1.1, aPTT 25.0

Physical Exam Baseline nonverbal and does not follow commands due to developmental delay Lethargic but arousable Baseline dysconjugate gaze; no forced downgaze deviation Patient refusing examination of pupils Moving all extremities spontaneously Incisions clean, dry, and intact Shunt valve and proximal catheter palpable, without any swelling or fluid collection Shunt catheter not palpable distal to the valve Abdomen soft and nontender

Triage Management This patient has a right VPS for congenital hydrocephalus and a history of distal shunt disconnections. Currently, he is presenting with progressive lethargy, emesis, and intermittent bradycardia, which is consistent with his prior shunt malfunctions. Urgent brain imaging and shunt series demonstrate enlarged ventricles and a disconnection of the shunt catheter just distal to the valve, with coiling of the catheter in the abdomen. He has a distal shunt malfunction and will require urgent surgery for revision. Because the patient has enlarged ventricles with symptoms concerning for elevated intracranial pressure (e.g. lethargy and intermittent bradycardia), his shunt will be accessed at bedside under sterile conditions to drain cerebrospinal fluid (CSF) as a temporizing measure while surgery is being coordinated. The pediatric surgery team was involved in his previous surgeries for distal catheter placement in the abdomen, and they will be consulted again for assistance in this surgery. He will be admitted to the pediatric intensive care unit for close neurological checks and monitoring of his vitals (particularly bradycardia) while same-day or next-day surgery is planned. This is an urgent and operative consult.

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Assessment This is a 13 year old male with Joubert syndrome, developmental delay, Dandy-Walker malformation, and congenital hydrocephalus with a right VPS who presents with a distal shunt malfunction.

Plan n

n

n

n

n

Because an MRI was used instead of a head CT to evaluate the patient’s ventricular size, his Strata valve must be checked and adjusted as needed to make sure the valve setting has not changed Bedside shunt tap in the ED to evaluate for proximal shunt patency n If his ICP is elevated, removal of CSF to temporarily lower his ICP Consult to the pediatric surgery team for intraoperative assistance of distal catheter placement Surgical planning for same-day or next-day right VPS revision with open or laparoscopic placement of a new distal catheter and retrieval of the old distal catheter n Posting highlights: n Distal shunt catheter n Prepare for possible proximal catheter or valve revision n Manometer n For anesthesia: antibiotics While planning for surgery, admit to the pediatric intensive care unit for close monitoring of vitals and neurological checks n If there is concern for neurological deterioration, there is a low threshold to re-tap his shunt or proceed to the operating room emergently for revision.

LEARNING POINTS

• Shunt malfunction is a common complication in patient with shunts for hydrocephalus. The rates of malfunction vary greatly in the literature; however, one meta-analysis described the following annual failure rates:6 Pediatric

First-year 31.3%

Beyond the f irst year 4.5%

Adult

16.2%

5.2%

• Noninfectious causes of shunt malfunction include obstruction, disconnection, catheter fracture, catheter migration, and mechanical failure of the valve. Shunt infection is discussed in Chapter 64. • In the evaluation of a shunt malfunction, it is critical to understand the patient’s hydrocephalus and shunt history. In particular, the following questions should be obtained from the history: • Why does the patient have a shunt? • What type of shunt does the patient have (e.g. valve type [programmable vs. nonprogrammable], location)? • When was the patient’s last revision and why? • If the patient has had a prior malfunction, what were the presenting symptoms? • If the patient has had a prior malfunction, did the ventricles enlarge on imaging? • The presentation of a shunt malfunction can be quite varied, depending on the age of the patient and the type of malfunction. Because an acute malfunction can have potentially Continued

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devastating consequences including neurological injury, herniation, and death, any shunted patient who presents with a potential neurological complaint should be evaluated as a shunt malfunction until proven otherwise.1 • Common symptoms include headaches, nausea, vomiting, and decreased level of consciousness.2,4 Children can present with irritability, bulging fontanelle, increased head circumference, and loss of developmental milestones.2 Other symptoms can include increased seizure frequency, vision changes, and gait abnormality.1 • In a patient with suspected shunt malfunction, understanding prior presentations with shunt failure is critical as patients can often present with the same symptoms. • Family members (e.g. parents of pediatric patients) are particularly attuned to the symptoms of a shunt malfunction.1 • Important aspects of the physical examination include:1 • Vital signs—bradycardia, abnormalities in blood pressure and respiratory rate are concerning for elevated intracranial pressure (ICP) • Palpation of the shunt along its entire course—evaluate for skin erythema or fluid collections along the shunt • Palpation of fontanelle (when applicable)—estimates ICP; a bulging fontanelle is concerning • Fundoscopic examination—assess for papilledema as a measure of chronically elevated ICP • Cranial nerve evaluation—can detect abnormalities such as abducens nerve paresis or upgaze palsy • Radiographic studies are often essential in the diagnosis of shunt malfunction: • Head CT or MRI evaluates the size of the ventricular system. • It is important to compare the images to prior studies at a time when the patient was not having a malfunction. • If the patient’s ventricles are enlarged compared to prior, there is a high suspicion of malfunction. • It is also important to know if the patient’s ventricles increased in size with prior malfunction. • If the patient’s ventricles do not change in size with malfunction and currently the ventricles are the same size, this does not rule out a shunt malfunction. • If the patient’s ventricles do change in size with malfunction and currently the ventricles are the same size, shunt malfunction is less likely. • It is important to know if the patient has had a recent valve setting adjustment as a decrease in the amount of drainage from the valve may cause enlarged ventricles with or without symptoms of malfunction. • A plain radiograph shunt series evaluates for disconnections, breaks, or kinks in the shunt system. • When a distal malfunction is suspected, abdominal CT or ultrasound may demonstrate a pseudocyst, ascites, visceral perforation, or malposition of the catheter. • Radionuclide or shunt patency studies can be performed to assess for an occlusion but are rarely used in the acute setting. • A shunt tap is a bedside procedure that can assist in the diagnosis and evaluation of shunt malfunction. The steps of the procedure are described in Chapter 77. • Imaging is typically done before the tap to assess the size of the ventricles. When the ventricles are small or slit-like, a shunt tap can result in a proximal malfunction by causing occlusion of the catheter with brain tissue or collapsing of the ventricular walls. In these cases, a shunt tap may be risky. • During a shunt tap, a distal malfunction can sometimes be diagnosed by manually occluding the inlet portion of the valve and then allowing fluid in the needle tubing to flow distally. Slow or absent distal flow can indicate distal malfunction. • If the proximal catheter is patent, a shunt tap with drainage of CSF to a normal pressure can acutely treat elevated ICPs and temporize the hydrocephalus.

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289

• If a patient is unstable or if there is concern for herniation, ABC’s should be prioritized with subsequent head imaging and possible emergent shunt tap after stabilization of the patient. • Prediction of shunt failure, distal or proximal, remains challenging,5 but risk factors include prior failure, congenital hydrocephalus, cardiac risk factors, seizure history, or history of neuromuscular disease.3 • Distal malfunctions occur at the level of the valve and the distal catheter. They are typically not as emergent as proximal malfunctions as they can be temporized with repeat shunt taps while planning surgical revision. • Abdominal access in patients with multiple distal revisions may benefit from the general surgery team’s assistance.

References 1. Awad AJ, Iyer RR, Jallo GI. Acute shunt malfunction. In: Loftus C, ed. Neurosurgical Emergencies. 3rd ed. Thieme; 2017. 2. Garton HJ, Kestle JR, Drake JM. Predicting shunt failure on the basis of clinical symptoms and signs in children. J Neurosurg. 2001;94(2):202–210. 3. Gonzalez DO, Mahida JB, Asti L, et al. Predictors of ventriculoperitoneal shunt failure in children undergoing initial placement or revision. Pediatr Neurosurg. 2017;52(1):6–12. 4. Khan F, Rehman A, Shamim MS, Bari ME. Factors affecting ventriculoperitoneal shunt survival in adult patients. Surg Neurol Int. 2015;6:25. 5. Rossi NB, Khan NR, Jones TL, Lepard J, McAbee JH, Klimo Jr P. Predicting shunt failure in children: should the global shunt revision rate be a quality measure? J Neurosurg Pediatr. 2016;17(3):249– 259. 6. Stein SC, Guo W. Have we made progress in preventing shunt failure? A critical analysis. J Neurosurg Pediatr. 2008;1(1):40–47.

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Nausea And Lethargy In A Shunted Patient Tej D. Azad, MD/MS n

Dimitrios Mathios, MD

Consult Page 25 yo M hx VP shunt p/w lethargy and nausea. Recs?

Initial Imaging

A

B

C

Fig. 60.1 X-ray shunt series. Lateral view of the skull (A) and anteroposterior view of the chest (B) and abdomen (C) demonstrates the patient’s left proximal catheter, the Certas valve, the ProSa device, and the left distal catheter without any obvious kinks, breaks, or discontinuities. There is also evidence of abandoned shunt hardware in the skull, chest, and abdomen and the patient’s scoliosis spinal hardware.

Fig. 60.2 Axial head CT without contrast demonstrates moderately-sized ventricles which are stable compared to prior imaging. The patient’s left proximal catheter as well as an abandoned right catheter are seen.

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291

Walking Thoughts n n n n n

n

n n

n n

What is the patient’s neurological exam and current vitals? What are the patient’s symptoms? What is the timeline of symptoms? Why does the patient have a shunt? What type of shunt is currently in place? What is the patient’s shunt history? n When was the shunt placed? n Has the patient ever required a shunt revision and if so, when was the last one? n Has the patient ever had a shunt malfunction? If so, what symptoms did the patient have at that time? n Was the shunt setting recently changed? Is there prior imaging of the patient’s ventricles? If so, does the patient’s ventricles change in size with prior shunt malfunctions? Does the patient need an urgent bedside procedure (e.g. shunt tap)? Does this patient need to go to the operating room for a shunt revision? If so, what is the timing of surgery? Does the patient have other medical comorbidities? Is the patient on any antiplatelet or anticoagulant medications?

History of Present Illness A 25 year old male with a history of mild developmental delay, seizures, hypothyroidism, scoliosis with surgical correction, and congenital hydrocephalus with a left frontal ventriculoperitoneal shunt (VPS) presents to the emergency department with three days of progressive lethargy and nausea. His mother reports that three days ago, he began having a headache and mild fatigue, but no emesis. Since then, he has had worsening headaches and lethargy, as well as nausea. His mother denies any focal deficits, seizure-like activity, or fevers. He initially underwent shunt placement as a baby due to congenital hydrocephalus. He has experienced multiple shunt failures requiring revision, and his last shunt malfunction was 8 months prior. At that time, he presented with similar symptoms of nausea, headache, and lethargy, but his ventricles were unchanged in size. X-rays of his shunt demonstrated a distal disconnection. During surgery, his opening pressure was noted to be over 30 cm H2O. Postoperatively, his symptoms resolved, and repeat head imaging demonstrated stable ventricular size. His current valve setting is a Certas 6 with a ProSa 22. He is not on any antiplatelet or anticoagulant medications.

Vital Signs T 36.8°C, BP 118/78, HR 52, RR 16, SpO2 99% on room air

Pertinent Labs WBC 9.2, Plt 188, INR 1.2, aPTT 27.1

Physical Exam Eyes open to voice Sleepy but arousable Oriented to self, hospital, and year Eyes dysconjugate (baseline strabismus), but pupils reactive and symmetric

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Extraocular movements intact, face symmetric, tongue midline Bilateral upper and lower extremities full strength Incisions clean, dry, and intact Shunt reservoir refills very slowly after being depressed

Triage Management This patient has a left VPS for congenital hydrocephalus and is presenting with symptoms concerning for shunt failure. Although his head imaging does not show a change in his ventricular size, a shunt malfunction cannot be ruled out since his ventricles have previously remained unchanged with prior known malfunctions. He has some mild bradycardia with a heart rate intermittently in the 50s, when normally his heart rate has been in the 80s during prior clinic visits. On examination, he is sleepy but still following commands. The valve reservoir does not refill when pressed, which is concerning for proximal shunt failure. An emergent shunt tap is planned to be done at bedside for further evaluation. This is an emergent and likely operative consult.

Shunt Tap A sterile shunt tap is performed at bedside. Upon insertion of a 25-gauge butterfly needle into the valve reservoir, no flow of fluid is seen spontaneously or with dropping the needle tubing below the level of the valve.

Assessment This is a 25 year old male with congenital hydrocephalus and a left VPS presenting with concern for a proximal shunt malfunction. Shunt tap confirms the concern for proximal obstruction given no CSF flow. Emergent surgery is planned for immediate shunt revision.

Plan n

Emergent planning for left ventriculoperitoneal shunt revision Posting highlights: Proximal shunt catheter n Prepare for possible distal catheter or valve revision n Manometer n For anesthesia: antibiotics Postoperatively, the patient can be admitted to the floor

n

n

n

LEARNING POINTS

• The general workup of a shunt malfunction is discussed in Chapter 59. • Proximal malfunctions are more common in children. Obstruction of the proximal catheter is a common cause of acute malfunction and can be due to occlusion with ventricular debris or brain tissue (e.g. choroid plexus).1 In patients who are shunt-dependent, a proximal shunt malfunction is a neurosurgical • emergency as there is no way to temporize symptoms via shunt tap, and the untreated hydrocephalus can then cause increased intracranial pressure and herniation. Evaluation and workup must be expeditious.

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293

• If a patient is unstable or if there is concern for herniation, ABC’s should be prioritized with subsequent head imaging and possible emergent shunt tap after stabilization of the patient.

• An important aspect of the physical examination for proximal shunt malfunction is pumping the valve reservoir—this maneuver is controversial1,2 and should be cautiously done if the ventricles are small. Slow or no refill of the valve reservoir in a symptomatic patient is concerning for proximal malfunction. • A shunt tap is a bedside procedure that can assist in the diagnosis and evaluation of shunt malfunction. The steps of the procedure are described in Chapter 77. In a proximal shunt malfunction, no or very slow spontaneous CSF flow can be found. • If there is concern for a partial obstruction of the proximal catheter, in carefully selected instances, adjustment of the valve setting to increase the amount of drainage may be a temporizing measure while surgery is being planned. • In the operating room, because the proximal catheter may be difficult to remove, be prepared to place an entirely new catheter. • In complex patients who may have abandoned hardware, ensure that the functioning system is evaluated.

References 1. Awad AJ, Iyer RR, Jallo GI. Acute shunt malfunction. In: Loftus C, ed. Neurosurgical Emergencies. 3rd ed. Thieme; 2017. 2. Browd SR, Ragel BT, Gottfried ON, Kestle JR. Failure of cerebrospinal fluid shunts: part I: obstruction and mechanical failure. Pediatr Neurol. 2006;34(2):83–92.

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61

Worsening Vision In Pseudotumor Cerebri Jennifer E. Kim, MD n Dimitrios Mathios, MD

Consult Page 37F h/o pseudotumor with worsening vision, consult for shunt placement

Initial Imaging

A

C

B

D

Fig. 61.1 Preoperative brain MRI without contrast. An axial T2-weighted sequence demonstrates normalsized ventricles (A) with bilateral optic nerve atrophy, right greater than left (B). An empty sella is visualized on a sagittal T1-weighted sequence (C). There is no evidence of a mass or lesion. Coronal time-of-flight MRV shows a hypoplastic but patent left transverse sinus (D).

294

i

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Walking Thoughts What is the patient’s neurological exam? What is the patient’s vision now and how quickly did it deteriorate? n Was the patient evaluated by ophthalmology for papilledema? n What other symptoms is she having? n What is the patient’s pseudotumor cerebri (PTC) history? How and when was she diagnosed? n What treatments has she tried for her PTC (e.g. acetazolamide, serial lumbar punctures)? n Does the patient have venous sinus stenosis? n What are the patient’s medical comorbidities? n If she needs a shunt, does the patient have any contraindications for ventriculoperitoneal, lumboperitoneal, or ventriculoatrial shunting (e.g. multiple abdominal surgeries, Chiari malformation, bacteremia, abnormal venous anatomy, prior jugular or subclavian thrombosis, cardiac irritability)? n n

History of Present Illness A 37 year old female with known pseudotumor cerebri, diabetes mellitus, hypertension, hyperlipidemia, end stage renal disease (ESRD) secondary to IgA nephropathy and focal segmental glomerulosclerosis, and morbid obesity presents to the emergency department with headaches and acutely worsening vision. The patient was diagnosed with PTC two years ago on work-up of headaches and worsening left vision. At that time, her opening pressure on lumbar puncture (LP) was >30 cm H2O, and she was recommended to undergo a ventriculoperitoneal shunt for her PTC; however, she declined shunt placement. Ultimately, she underwent a left optic nerve sheath fenestration (ONSF) with improved vision. She was started on acetazolamide, but this was quickly discontinued due to slow clearance secondary to her ESRD. Although she had outpatient therapeutic LPs scheduled, she missed several of these appointments. Two days prior to presentation, she developed pressure-like headaches and acute blurred “tunnel vision.” She otherwise denies weakness, numbness, tingling, nausea, or vomiting. Ophthalmological evaluation in the ED demonstrated bilateral pale optic discs with end-stage edema on fundus exam, and severe peripheral vision loss that was worse in the right eye. LP was performed at bedside in lateral decubitus position and legs extended with an opening pressure of 37 cm H2O. She had drainage of 20 mL of cerebrospinal fluid to a closing pressure of 25 cm H2O in adults and >28 cm H2O in children) in a properly performed lumbar puncture • Secondary causes of pseudotumor cerebri include cerebral venous sinus thrombosis or stenosis and medications (e.g. vitamin A/retinoids, antibiotics such as tetracycline). Pseudotumor cerebri with an unclear etiology can be described as “idiopathic intracranial hypertension.”2 • Imaging is notable for normal to “slit-like” ventricles without evidence of an obstructive or mass lesion. While a head CT scan can be obtained as a primary survey, a contrasted MRI is recommended to rule out other pathologies that could cause elevated ICP. • MRI may also show other signs of elevated ICP such as an empty sella or optic nerve head flattening or protrusion. • CISS (Constructive Interference Steady State) and CINE sequences may be useful to assess for CSF flow obstruction and the safety of LP. • MR or CT venography can also show venous sinus stenosis or thrombosis, which has been implicated as a cause of intracranial hypertension in the majority of patients with secondary PTC.4

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• The most common presenting symptom is headache, which is usually bifrontal and pressure-like. It may mimic a headache caused by a brain tumor with increased intracranial pressure (thus, the name “pseudotumor”). The second most common presenting symptom is vision loss, which can manifest as transient vision loss, diplopia, limited peripheral vision, blurred or dark spots, and in chronic cases, tunnel vision.2 • Nearly all PTC patients are found to have some degree of papilledema. Severe papilledema and acute or significant vision loss warrants urgent treatment.2 • Conservative therapy with weight loss, ICP-reducing medications such as acetazolamide, and serial LPs is appropriate in patients with headaches and preserved or stable vision. • Surgical intervention is warranted in patients with vision loss and intractable headaches. The goal of treatment is to preserve vision and alleviate symptoms (typically headache). • Optic nerve sheath fenestration can be employed to urgently decompress the optic nerves; however, CSF diversion with a ventriculoperitoneal, ventriculoatrial, or lumboperitoneal shunt is also effective in preserving vision while also treating headaches.1 • Venous sinus stenting may also be effective in both the acute and chronic setting for PTC patients found to have transverse sinus stenosis with a significant pressure gradient across the stenotic segment.4 • There is limited data on the optimal surgical management for medically refractory PTC. In one recent systematic review, pooled analysis demonstrated an overall similar improvement in visual outcomes across treatment modalities of optic nerve sheath fenestration, shunting, and venous sinus stenting; however, there is insufficient evidence to recommend or reject any treatment modality for PTC.3 • Lumbar CSF pressure is most accurately measured with:2 • The patient in lateral decubitus position and legs extended • The base of the manometer must be level with the right atrium (or otherwise with a height correction applied) • Minimal sedation as hypercapnia can artificially raise the CSF pressure measurement • Avoidance of Valsalva maneuvers (which can artificially elevate ICP) Contraindications for the following types of shunts include: • • Ventriculoperitoneal shunt: peritoneal infection, peritoneal adhesions, extensive abdominal operations • Ventriculoatrial shunt: bacteremia or infection, congestive heart failure, pulmonary hypertension, abnormal venous anatomy, cardiac malformation, cardiac irritability, prior jugular or subclavian venous thrombosis • Lumboperitoneal shunt: existing Chiari malformation, obstructive hydrocephalus, subcutaneous infection of the back, history of lumbar fusion, peritoneal infection, peritoneal adhesions, extensive abdominal operations

References 1. Fonseca PL, Rigamonti D, Miller NR, Subramanian PS. Visual outcomes of surgical intervention for pseudotumour cerebri: optic nerve sheath fenestration versus cerebrospinal fluid diversion. Br J Ophthalmol. 2014;98(10):1360–1363. 2. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 2013;81(13):1159–1165. 3. Lai LT, Danesh-Meyer HV, Kaye AH. Visual outcomes and headache following interventions for idiopathic intracranial hypertension. J Clin Neurosci. 2014;21(10):1670–1678. 4. Radvany MG, Solomon D, Nijjar S, et al. Visual and neurological outcomes following endovascular stenting for pseudotumor cerebri associated with transverse sinus stenosis. J Neuro Ophthalmol. 2013;33(2):117– 122. 5. Thambisetty M, Lavin PJ, Newman NJ, Biousse V. Fulminant idiopathic intracranial hypertension. Neurology. 2007;68(3):229–232.

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Headache, Confusion, And Chronic Rhinorrhea Dimitrios Mathios, MD

Consult Page 14F with chronic sinusitis, prior meningitis pw headache, confusion, leakage from nose. Head CT unrevealing. Imaging recs?

Initial Imaging

Fig. 62.1

Axial head CT without contrast demonstrates no acute abnormality or pneumocephalus.

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Walking Thoughts □ □ □ □ □ □ □

What is the patient’s current neurological exam? What are her current vitals? What symptoms has she been having and what is the timeline? What is leaking from her nose? Is it clear? How long has she had this leakage? When did she have meningitis? What was the cause and how was it treated? Is there concern for meningitis or a cerebrospinal fluid leak? With a reportedly negative head CT, what additional imaging does she need?

History of Present Illness A 14 year old female with a history of prior meningitis and chronic sinusitis presents to the emergency department (ED) with several days of headaches and body aches. Earlier today, she vomited several times after breakfast and seemed confused. Her parents deny any sick contacts at home and report that she has otherwise been healthy. They deny any focal neurological deficits or seizure-like activity. Currently, the patient reports headache, neck stiffness, and light sensitivity. Her parents report that about one year prior, she had a similar presentation to the ED and was ultimately found to have Streptococcus pneumonia sepsis and meningitis. She had a prolonged hospital course with IV antibiotic treatment but was ultimately discharged home without any residual deficits. Workup for the cause of her meningitis was negative, and thus was presumed to be community-acquired. Of note, she has had several years of sinusitis and intermittent clear drainage from her nose. Her parents note that every time she leans forward (e.g. brushing her teeth), she complains of nose stuffiness and a weird taste in her mouth.

Vitals T 39.6°C, BP 128/82, HR 60, SpO2 99% on room air

Pertinent Labs Na 136, WBC 27.3, ESR 105, CRP 26.4

Glasgow Coma Scale Motor: 6 Verbal: 4 Eye Opening: 3 GCS Total: 13

Physical Exam Sleepy but opens eyes to voice Requests for room lights to be turned off Oriented to self and year, but confused Pupils equal, round, and reactive to light Extraocular movements intact, face symmetric, tongue midline Moves all extremities weakly to command No active drainage from nose Flexing her neck induces hip and knee flexion

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Triage Management The patient has a history of prior meningitis and is presenting with altered mental status, fever, and elevated inflammatory markers, concerning for recurrent meningitis. Although she is hemodynamically stable, she will need urgent sampling of her cerebrospinal fluid (CSF) to assess for meningitis and admission to the pediatric intensive care unit (PICU) for close monitoring. Once her CSF and blood have been obtained for cultures, broad-spectrum antibiotics can be initiated. Her presentation of recurrent meningitis in an otherwise healthy young girl is worrisome, and her history of sinusitis and clear rhinorrhea is concerning for a CSF leak that could be the cause of her meningitis. If any rhinorrhea is noted during her admission, the fluid can be collected and sent for beta-2 transferrin. Her meningitis treatment takes precedence and after stabilization in the PICU, she will undergo additional head imaging to assess for skull base defects and/or CSF leak. This is an emergent and potentially operative consult.

Assessment This is a 14 year old female who presents with altered mental status, fever, and recurrent meningitis. Her history of chronic sinusitis and clear rhinorrhea requires a work-up for a skull base defect causing a CSF leak.

Plan Admission to the PICU for hourly neurological checks Early lumbar puncture (LP) for CSF profile (cell count, glucose, protein) and cultures n Blood cultures n Broad-spectrum antibiotics once CSF and blood cultures are obtained n Consult to the infectious disease team for management of antibiotics n Collection of nasal leakage to send for beta-2 transferrin n Maxillofacial CT without contrast to assess for a skull base defect and/or encephalocele n n

Follow-Up The patient was admitted to the PICU, underwent a successful LP, and was started on broadspectrum antibiotics. Her CSF cultures grew Streptococcus pneumonia, and her antibiotics were narrowed based on the infectious disease team’s recommendations. High-resolution maxillofacial CT without contrast demonstrated a suspected bony defect involving the anterior left cribriform plate with concern for a large encephalocele (Figure 62.2). Brain MRI without contrast confirmed the encephalocele. She subsequently underwent a cisternogram, which confirmed a CSF leak from the left cribriform plate defect with contrast extending into the left olfactory fossa and nasal cavity (Figure 62.3). Once she was stable from her meningitis after several days of IV antibiotic treatment, she underwent surgery for an extended endonasal approach for CSF leak repair. A lumbar drain was placed at the beginning of the case and was maintained for 7 days postoperatively to protect the skull base reconstruction and repair. During the weaning trial of the lumbar drain, the patient was observed to have high output from the lumbar drain at a popoff of 20 cm H2O, raising the concern for idiopathic intracranial hypertension. The patient underwent a CT venogram to assess for venous sinus stenosis, which was negative. The patient was maintained on a high dose of acetazolamide during weaning of her lumbar drain and even after the lumbar drain was removed. She did well with no additional nasal drainage and was ultimately discharged home after completion of her IV antibiotic course.

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Fig. 62.2 Coronal maxillofacial CT without contrast shows a small cribiform plate defect on the left (arrow) with subadjacent fluid attenuation concerning for an encephalocele (dotted line).

Fig. 62.3 Sagittal CT cisternogram demonstrates the small cribiform plate defect (yellow arrow) with contrast extending into the nasal cavity (green arrow).

LEARNING POINTS

• Cerebrospinal fluid (CSF) leaks of the skull base occur as a result of bony, dural, meningeal, and arachnoid disruption. There are multiple classification schemes, but CSF leaks can generally be classified based on anatomic site or etiology. CSF leaks can be due to trauma (accidental or iatrogenic), tumor, infection, or congenital defects.3-5 When located in the anterior cranial fossa, CSF leaks can present as rhinorrhea (which can • mimic nasal drainage caused by allergies, sinusitis, and other inflammatory conditions). When located in the middle cranial fossa, CSF leaks can present as middle ear effusions, ear fullness, or rhinorrhea via the eustachian tubes.4 Drainage can be exacerbated with changes in position and activities that increase intracranial pressure (e.g. coughing, sneezing, or other Valsalva maneuvers). Given the connection to the intracranial space, recurrent meningitis of unclear etiology may also be indicative of a CSF leak.

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• Beta-2 transferrin is a protein found only in CSF, aqueous humor, and perilymph. It is often used as a marker of CSF leakage. Any leakage from the nose or ear can be sent for beta-2 transferrin to differentiate CSF from other bodily fluids. Beta-2 transferrin may take days to weeks to result, and treatment for meningitis should not be delayed while awaiting this lab result. • A variety of imaging options exist to aid in the diagnosis of CSF leak. • High-resolution computed tomography can identify bony defects of the skull base. • A brain MRI without contrast can identify herniation of the brain (encephalocele) or meninges (meningocele) through the defect. • A CT cisternogram involves injection of contrast into the thecal sac with subsequent CT imaging to identify the flow of contrast and if any leaks are present. • A pledget study is a nuclear medicine study that requires the placement of cotton pledgets in the nasal cavity by the otolaryngology team to confirm the presence of a CSF leak. A radiotracer is injected intrathecally via lumbar puncture, and the pledgets are then analyzed for radioactivity.1 • Intrathecal fluorescein injection involves the placement of a lumbar drain with the slow injection of fluorescein for intraoperative detection of CSF leak. • Identification and repair of CSF leak is commonly a joint endeavor between the neurosurgery and otolaryngology teams, and surgery can be done via an endonasal approach or an open approach. • Spontaneous CSF leaks are those without an identifiable cause; however, a significant proportion of these are now known to be due to elevated intracranial pressure (ICP). Compared to other causes of CSF leaks (e.g. trauma, tumor), spontaneous CSF leaks have the highest rate of recurrence after surgical repair. • Spontaneous CSF leaks are associated with female gender, obesity, increased ICP, and obstructive sleep apnea.3 • The workup for elevated ICP or pseudotumor cerebri is discussed in Chapter 61. • In patients with a spontaneous CSF leak, lumbar drain placement and continuous measurement of ICP may be helpful to optimize the chances of a successful repair.2,4 • ICP-reducing medications such as acetazolamide can temporize a leak. In severe cases of persistent or recurrent CSF leak due to increased ICP, CSF diversion with a lumbar drain or even permanent shunting may be necessary as an adjuvant to surgical repair.

References 1. Grantham VV, Blakley B, Winn J. Technical review and considerations for a cerebrospinal fluid leakage study. J Nucl Med Technol. 2006;34(1):48–51. 2. Iyer RR, Solomon D, Moghekar A, et al. Venous sinus stenting in the management of patients with intracranial hypertension manifesting with skull base cerebrospinal fluid leaks. World Neurosurgery. 2017;106:103–112. 3. Lobo BC, Baumanis MM, Nelson RF. Surgical repair of spontaneous cerebrospinal fluid (CSF) leaks: a systematic review. Laryngoscope Investig Otolaryngol. 2017;2(5):215–224. 4. Reh DD, Gallia GL, Ramanathan M, et al. Perioperative continuous cerebrospinal fluid pressure monitoring in patients with spontaneous cerebrospinal fluid leaks: presentation of a novel technique. Am J Rhinol Allergy. 2010;24(3):238–243. 5. Woodworth BA, Prince A, Chiu AG, et al. Spontaneous CSF leaks: a paradigm for definitive repair and management of intracranial hypertension. Otolaryngol Head Neck Surg. 2008;138(6):715–720.

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Falls, Memory Loss, And Fevers Dimitrios Mathios, MD

Consult Page 57F here with fevers and history of falls and memory loss. Head CT shows hydrocephalus. Safety of LP?

Initial Imaging

A

C

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D

Fig. 63.1 Sagittal (A) and axial (B-D) views of a head CT without contrast demonstrates enlarged lateral (B) and third ventricles (C) with a normal fourth ventricle (D), as well as a hyperdensity near the cerebral aqueduct (C).

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Walking Thoughts n n n n n n n n n n

What is the patient’s current neurological exam? What are the patient’s vitals? Is there concern for acutely increased intracranial pressure or herniation? What other symptoms does the patient have and what is the timeline? Is the diagnosis of hydrocephalus known or new? What type of hydrocephalus does the patient have (e.g. communicating, obstructive)? Is there concern for infection? If so, what bloodwork has been sent? Why is a lumbar puncture being considered? What additional imaging does the patient need? Is the patient on any antiplatelet or anticoagulant medication?

History of Present Illness A 57 year old female with a history of a renal transplant and rheumatoid arthritis presents to the emergency department (ED) with fevers and abdominal pain. Her symptoms began a few days ago with intermittent fevers, chills, abdominal pain, decreased appetite, and fatigue. She has also had about a year of headaches, memory difficulties, and imbalance. Currently, she has a headache that feels like her usual headaches, but denies any nausea, vomiting, photophobia, phonophobia, focal weakness, numbness, tingling, or vision changes. She is on long-term immunosuppressive medications for her renal transplant but is not on any anticoagulant or antiplatelet medications.

Vital Signs T 38.9°F, BP 115/59, HR 75, SpO2 97% on room air

Pertinent Labs Na 135, WBC 9.4, Plt 213, INR 1.04, aPTT 23.7 Urinalysis concerning for infection Blood gram stain positive for gram-negative bacilli

Glasgow Coma Scale Motor: 6 Verbal: 5 Eye Opening: 4 GCS Total: 15

Physical Exam Awakens easily, oriented to self, year, and place Pupils equal, round, and reactive to light Extraocular movements intact, face symmetric, tongue midline Moves all extremities to command No signs of meningismus (e.g. able to flex neck and bring chin to chest)

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Triage Assessment The patient is immunocompromised secondary to her immunosuppressive medications for her renal transplant and presents with bacteremia that may be secondary to a urinary tract infection (UTI). The ED team is concerned for meningitis given her headaches and concomitant fever and would like to perform a lumbar puncture (LP) for cerebrospinal fluid (CSF) sampling. Her head CT demonstrates asymmetrically enlarged lateral and third ventricles compared to the fourth ventricle, which is normal in size. Additionally, there is a hyperdensity near the cerebral aqueduct. These imaging findings are concerning for obstructive hydrocephalus, which would preclude an LP. Her headaches are not new, and her year-long symptoms of headaches, memory difficulties, and imbalance may be explained by chronic hydrocephalus. She will need a brain MRI with CSF flow sequences to evaluate for an obstruction. Currently, she is neurologically intact and she is hemodynamically stable. Her workup thus far suggests that the likely cause of her bacteremia is a UTI. She also does not have any signs or symptoms of meningitis. With a known source of her infection as well as a low suspicion for meningitis, an LP is not needed at this time. Treatment of her bacteremia and UTI can be initiated while awaiting further brain imaging. This is a nonemergent and possibly operative consult.

Assessment This is a 57 year old female with a prior renal transplant and rheumatoid arthritis on immunosuppressive medications who presents with acute fevers, and chronic headaches, imbalance, and memory difficulties. She is found to be bacteremic secondary to a UTI. On workup of her chronic headaches, head imaging shows concern for obstructive hydrocephalus.

Plan n n n

n

Admission to the medicine service for treatment of bacteremia and UTI No lumbar puncture at this time Brain MRI without contrast, constructive interference in steady state (CISS) and CINE sequences (initially without contrast due to her renal transplant) If obstructive hydrocephalus is confirmed on MRI, the risk of lumbar puncture is high. Elective surgery for CSF diversion (e.g. endoscopic third ventriculostomy) can be planned as an outpatient after the patient’s infection is appropriately treated.

Follow-Up The patient is admitted to the medicine service and started on broad-spectrum antibiotics. Brain MRI with CSF flow sequences confirmed aqueductal stenosis (Figure 63.2) with reduced CSF flow in the cerebral aqueduct and fourth ventricle. LP was not performed. The patient was ultimately discharged home with a prolonged course of antibiotics. One month after discharge, she underwent successful elective endoscopic third ventriculostomy with improvement of her headaches, imbalance, and memory difficulties.

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Fig. 63.2 Midline sagittal brain MRI CISS sequence confirms an obstruction at the distal cerebral aqueduct (arrow). CINE sequences (not shown here) demonstrate reduced CSF flow in the cerebral aqueduct and fourth ventricle.

LEARNING POINTS

• When a patient presents with hydrocephalus, it is important to identify if the hydrocephalus is communicating or noncommunicating.

• Aqueductal stenosis is narrowing of the cerebral aqueduct, which can lead to obstructive hydrocephalus. Because it is the narrowest part of the ventricular system, it is a common site of blockage.1 • Causes of aqueductal stenosis include congenital abnormalities, infection/inflammation, hemorrhage, and tumor. Aqueductal stenosis can also be seen in rare genetic syndromes, such as X-linked hydrocephalus or neurofibromatosis.1 • Aqueductal stenosis is a common cause of congenital hydrocephalus and can be diagnosed in utero. Early diagnosis is important as it can be associated with moderate to severe developmental delay, even with optimal surgical treatment.1 • The clinical presentation can vary depending on the age and severity of the resultant hydrocephalus, but oftentimes, the onset is insidious. Children can present with rapidly enlarging head circumference, headache, delayed psychomotor development, school difficulties, and endocrinology abnormalities. Adults can present with headaches, visual changes, and a presentation similar to that of normal pressure hydrocephalus (memory changes, gait difficulties, and urinary incontinence).1 • Diagnostic imaging includes the following:1 • Ultrasonography can be used in fetal cases or newborns. • CT without contrast showing asymmetry of ventricular dilation is suggestive of obstruction. • High-resolution MRI is important to confirm the patency of the aqueduct. CSF flow sequences are a helpful adjunct.

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• The indication for surgery is oftentimes CSF diversion to treat the hydrocephalus, rather than to open the aqueduct.1 CSF diversion is usually treated with endoscopic third ventriculostomy (ETV); however, the placement of a shunt may be more appropriate in very advanced cases of hydrocephalus where normal brain landmarks are distorted and the brainstem is pushed forward to the clivus. The success of ETV varies depending on age, etiology, and history of prior shunt (the ETV success score).2 In aqueductal stenosis, the reported success rates of ETV range between 23% to 94%.3 • Lumbar puncture in patients with obstructive hydrocephalus can be risky due to concern for downward or transtentorial herniation.

References 1. Cinalli G, Spennato P, Nastro A, et al. Hydrocephalus in aqueductal stenosis. Childs Nerv Syst. 2011;27(10):1621–1642. 2. Kulkarni AV, Drake JM, Kestle JR, et al. Predicting who will benefit from endoscopic third ventriculostomy compared with shunt insertion in childhood hydrocephalus using the ETV Success Score. J Neurosurg Pediatr. 2010;6(4):310–315. 3. Spennato P, Tazi S, Bekaert O, Cinalli G, Decq P. Endoscopic third ventriculostomy for idiopathic aqueductal stenosis. World Neurosurgery, 79 suppl 2: S21.e13–S21.e20.

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Abdominal Pain And Fevers In A Shunted Patient Nancy Abu-Bonsrah, MD n

Dimitrios Mathios, MD

Consult Page 48F here with abdominal pain, low grade fevers. CT abdomen shows multiple abscesses

Initial Imaging

Fig. 64.1 Axial head CT without contrast demonstrates the patient’s known right parietal ventriculoperitoneal shunt with stable ventricular size compared to prior imaging. Of note, the patient’s left cochlear implant, an abandoned left frontal shunt catheter, and areas of parenchymal calcification are also seen.

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Fig. 64.2 Sagittal (A) and coronal (B) abdominal CT with contrast demonstrates multiple rim-enhancing fluid collections (dotted line) within the lower abdomen and pelvis concerning for abscesses with the distal ventriculoperitoneal catheter seen in proximity (arrow).

Walking Thoughts n n

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n n n n n

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What is the patient’s neurological exam and current vitals? Does the patient have any neurological symptoms? Is she exhibiting any signs or symptoms concerning for sepsis or meningitis? What is the timeline of symptoms? Why does the patient have a shunt? What is the patient’s shunt history? When was the shunt placed? What type of shunt is in place? Has the patient ever required a shunt revision and if so, when was the last one? Has the patient ever had a shunt malfunction? If so, what symptoms did the patient have at that time? Has the patient had a shunt-associated infection before? Has the patient had a recent abdominal procedure? What bloodwork has been sent? What other imaging does the patient need? Does the patient need an urgent bedside procedure (e.g. shunt tap or externalization)? Does this patient need to go to the operating room for a shunt revision? If so, what is the timing of surgery? Does the patient have other medical comorbidities? Is the patient on any antiplatelet or anticoagulant medications?

History of Present Illness A 48 year old female with hypertension, hyperlipidemia, and a complex neurosurgical history presents to the emergency department (ED) with abdominal pain and low-grade fevers. She was initially diagnosed at the age of 9 years with a thalamic pilocytic astrocytoma that was treated with surgery and radiation. As a result of her treatment, she developed hydrocephalus that was shunted when she was 10 years old and has had multiple shunt revisions since. Her last

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shunt malfunction was 7 years prior when she presented with lethargy, altered mental status, and slurred speech. At that time, she was found to have a shunt obstruction on a shunt patency study without an increase in the size of her ventricles on brain imaging. She underwent revision and her shunt has been working well since. Currently she has a right ventriculoperitoneal shunt (VPS) with a Strata valve set at 1.5. She has a seizure disorder for which she is on lacosamide and clobazam. At baseline, she has left hearing loss with a cochlear implant and is mildly developmentally delayed, living at home with her siblings. She also has had a gastrostomy tube (g-tube) in place for many years. Currently, she presents to the ED with several days of right lower quadrant abdominal pain and decreased appetite. She has not had any nausea or vomiting, but because her pain worsened today with a low-grade fever, her family brought her into the ED. She has a history of recurrent urinary tract infections (UTIs); she has not had any recent abdominal procedures or issues with her g-tube. Her family notes that she is a little sleepier (which is common for her in the setting of infection) and headaches, but otherwise has not had slurred speech, weakness, or other new focal neurological changes. She is not on any anticoagulant or antiplatelet medications. CT of the abdomen in the ED demonstrates multiple rim-enhancing fluid collections within the lower abdomen and pelvis, concerning for abscesses, with the ventriculoperitoneal catheter seen in close proximity. Head CT shows stable ventricular size compared to prior imaging in our system, and x-rays of her shunt do not show any disconnections, kinks, or breaks in her shunt system.

Vital Signs T 38.2oC, BP 95/56, HR 69, RR 16, SpO2 97% on room air

Pertinent Labs Na 140, Hgb 12, WBC 21, Plt 190, INR 1.1, aPTT 25 ESR 114, CRP 12.9 Urinalysis concerning for a UTI

Glasgow Coma Scale Motor: 6 Verbal: 5 Eye opening: 3 GCS Total: 14

Physical Exam Eyes open to voice Oriented to self, place, and year Pupils equal, round, and reactive to light Extraocular movements intact, face symmetric, tongue midline Follows commands briskly in all extremities Scalp and abdominal incisions clean, dry, intact without underlying swelling Abdominal pain on palpation

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Triage Management This is a patient with shunt-dependent hydrocephalus who is presenting with multiple abdominal abscesses and concern for a shunt infection. Although she does not seem to have signs or symptoms of meningitis, she is at high risk of meningitis given the direct connection between her ventricular system and her abdomen through her shunt. She will need to undergo cerebrospinal fluid (CSF) sampling and diversion of her shunt away from her abdomen while her abdominal abscesses are being treated. Because she has had a known neurological decline with shunt obstruction, she is shunt-dependent, and it is important to ensure continued CSF diversion while her infection is being treated. The options for CSF diversion in the setting of infection include shunt externalization (either at bedside or in the operating room) or complete removal of her shunt system with placement of an external ventricular drain (EVD) in the operating room. With either option, she will need to be monitored with a higher level of care. If she is hemodynamically unstable, she can be started on broad-spectrum antibiotics; otherwise, antibiotics can be started after her CSF is sampled. The general surgery team will be consulted to assist in the management of her abdominal abscesses, and the infectious disease team will manage her antibiotic course. This is an emergent and operative consult.

Assessment This is a 48 year old female with shunt-dependent hydrocephalus and a right VPS who presents with abdominal pain, decreased appetite, and low-grade fevers. She is found to have multiple abdominal abscesses and there is concern for shunt infection.

Plan n

n

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n n n

Admit to the neurosurgical intermediate care unit or neurocritical care unit for close neurological checks and monitoring of vital signs Plan for bedside shunt externalization in the neurocritical care unit and CSF sampling n Procedural highlights: n Surgical prepping solution n Sterile drapes and towels n Local anesthetic n Shodded mosquito clamps n Curved clamps n Silk sutures n Straight connector n Distal catheter n External drainage system (can level at the patient’s abdomen) n CSF tubes for basic labs (cell count, glucose, protein, cultures) n Consider sending the distal catheter tip for microbiological analysis n Close coordination with neurocritical care team for pain medication Blood cultures After CSF sampling and blood cultures, can start broad-spectrum antibiotics with meningitis coverage Consult to the general surgery team for management of abdominal abscesses consult to the infectious disease team for antibiotic management and treatment of UTI Continue home antiseizure medications

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LEARNING POINTS

• Shunt infections are a common complication that can be difficult to treat depending on the nature of the infection. There is a wide range of infection rates reported in the literature; however, the associated morbidity and mortality can be significant.2,5 • Shunt infections can occur via four mechanisms:5 • Colonization at time of surgery • Retrograde infection from the distal end of the shunt • Through the skin (e.g. via shunt tap or erosion of the catheter through the skin) • Hematogenous seeding • Risk factors for shunt infections include factors related to the patient and the operation itself:5 • Patient factors include premature birth, younger age, prior shunt infection, cause of hydrocephalus (infection is more likely with a cause of purulent meningitis, hemorrhage, and myelomeningocele). • Factors related to surgery include a higher number of people within the operating room, exposure to perforated surgical gloves, intraoperative use of the neuroendoscope, longer length of surgery, improper patient skin preparation, skin shaving, and exposure of a large area of skin during the procedure. • Shunt infections may be reduced by ensuring proper sterile technique during surgery, providing perioperative antibiotic prophylaxis, and implanting antibiotic-impregnated shunt materials. • Symptoms of shunt infection are quite variable depending upon the pathogenesis of infection, the organism, and the type of shunt. Clinical presentation can include fever, headache, lethargy, nausea/vomiting, erythema along the shunt tract, and/or possible seizures. Patients with distal abdominal catheters, especially in the setting of an abdominal pseudocyst, may present with abdominal pain, food intolerance, and peritonitic symptoms.5 • When evaluating patients with concern for shunt infection, standard neuroimaging (noncontrast head CT scan and shunt series) for evaluation of the shunt is frequently obtained to rule out other causes of the patient’s presentation. MRI of the brain with contrast may detect ventriculitis. Abdominal imaging with ultrasound or CT may show the presence of abscesses or a pseudocyst.5 • An abdominal pseudocyst associated with a ventriculoperitoneal shunt is a fibrous, nonepithelialized cyst wall surrounding the distal catheter, preventing CSF resorption. Although it can be a sterile collection caused by mechanical irritation or peritonitis, many are associated with concurrent infection of the pseudocyst and/or CSF. • If a pseudocyst is seen in a patient with a ventriculoperitoneal shunt, the suspicion for shunt infection is high until proven otherwise.1 • CSF cultures are the most important test to establish a diagnosis of infection. CSF analysis showing an elevated white blood cell count, elevated protein levels, and decreased glucose levels point to likely bacterial infection.5 • If initial CSF cultures are negative, they should be held for at least 10 days in an attempt to isolate more indolent organisms such as Cutibacterium acnes (formerly known as Propionibacterium acnes). • Negative cultures do not exclude infection and can be repeated if the suspicion for infection is high. • CSF can be obtained via a shunt tap, lumbar puncture, or direct sampling if drain externalization or removal is performed. CSF sampling may show contamination, colonization, or infection.5 • Contamination: an isolated positive culture, but with a normal CSF profile and lack of clinical symptoms. The most common contaminant organisms are coagulase-negative Staphylococci. Other clues include light growth, growth only in enrichment broth, and growth of the organism in a minority of cultures.

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• Colonization: multiple positive CSF cultures but with a normal CSF profile and lack of clinical symptoms

• Infection: positive CSF culture(s) with an abnormal CSF profile and/or clinical symptoms • The most common organisms associated with shunt infections include coagulase-negative Staphylococci (particularly Staphylococcus epidermidis), Staphylococcus aureus, C. acnes, and gram-negative bacteria. Depending on the virulence of the organism, infection can present within a few weeks to months of shunt insertion or revision. Fungal infections are rare, with Candida species as the most likely pathogen in the postoperative setting. If there is concern for fungal infection, CSF β-d-glucan can be sent.5 • Treatment options include antibiotics alone or supplementation of antibiotic treatment with partial or complete shunt hardware removal.5 • Antibiotic treatment alone is not recommended but can be used in patients who cannot tolerate a procedure. • When possible, the patient’s shunt must be externalized, replaced with an external ventricular drain (EVD), or removed.3,4 There is insufficient evidence in the literature to recommend one option over the other. • Removal may be necessary in the setting of a proximal shunt infection to control the source of infection. If patients are not shunt-dependent (e.g. with normal pressure hydrocephalus), removal of the hardware without replacement is ideal. If the patient is shunt-dependent, shunt externalization or replacement with an EVD allows for continued CSF diversion while the infection is treated. • The efficacy of treatment is monitored clinically as well as with repeat CSF sampling for clearance of cultures and improvement in CSF profile.5 • For patients with shunt infections, it is important to ensure they are hemodynamically stable and do not need immediate initiation of broad-spectrum antibiotics. Ideally, CSF sampling and blood cultures should be obtained before the initiation of antibiotics. • The infectious disease team should be involved to assist with antibiotic choice, duration, and the timing of reinternalization. The duration of antibiotics varies depending on the isolated microorganism, the extent of infection, and CSF findings. The timing of reinternalization also varies depending on the isolated microorganism, the severity of infection, and the improvement/sterilization of CSF on repeat sampling. • Other ancillary services such as the general surgery or interventional radiology team may be involved to treat abdominal abscesses or assist in the operating room for shunt reinternalization.



References 1. Mobley 3rd LW, Doran SE, Hellbusch LC. Abdominal pseudocyst: predisposing factors and treatment algorithm. Pediatr Neurosurg. 2005;41(2):77–83. 2. Prusseit J, Simon M, von der Brelie C et al. Epidemiology, prevention and management of ventriculoperitoneal shunt infections in children. Pediatr Neurosurg. 45(5): 325–336. 3. Schreffler RT, Schreffler AJ, Wittler RR. Treatment of cerebrospinal fluid shunt infections: a decision analysis. Pediatr Infect Dis J. 2002;21(7):632–636. 4. Tamber MS, Klimo Jr P, Mazzola CA, Flannery AM, Pediatric Hydrocephalus Systematic Review and Evidence-Based Guidelines Task Force. Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 8: management of cerebrospinal fluid shunt infection. J Neurosurg Pediatr. 2014;14(suppl 1):60–71. 5. Tunkel AR, Hasbun R, Bhimraj A, et al. 2017 Infectious Diseases Society of America’s clinical practice guidelines for healthcare-associated ventriculitis and meningitis. Clin Infect Dis. 2017;64(6):e34– e65.

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65

Increased Spasticity And Beeping Baclofen Pump Nancy Abu-Bonsrah, MD n

Ann Liu, MD

Consult Page 18Y M presents with baclofen pump beeping

Initial Imaging

*

A

B Fig. 65.1 XR abdomen anteroposterior view demonstrates (A) normal baclofen pump position one year prior and (B) rotation of the baclofen pump at present. Note the directionality of the catheter access port (arrow), indicating that the baclofen pump has flipped, preventing access to the reservoir port (asterisk).

316

p

317

Walking Thoughts n n n n n n n

n

What are the patient’s vital signs? Is he hemodynamically stable? What is the patient’s neurological exam? Why does the patient have a baclofen pump? How long has his pump been beeping? When was it last refilled or interrogated? Has he had prior issues with his baclofen pump? Does he have any symptoms of baclofen withdrawal? If so, how long has he been symptomatic? Has he taken any medication for his symptoms? What additional imaging studies (e.g. X-rays, CT) need to be ordered?

History of Present Illness An 18 year old male with a prior history of a motor vehicle collision two years prior with resultant spastic paraplegia and intrathetcal (IT) baclofen pump presents to the emergency department (ED) after his baclofen pump began beeping. Two weeks ago, he presented to clinic for a scheduled pump refill, but the refill was unable to be completed in clinic. He was instructed to go to the ED at that time for refill, but he did not comply with these instructions. A few days ago, his refill alarm started to beep, and he was instructed by his neurologist to begin taking oral baclofen 100 mg four times a day. He currently has increased spasticity and tone in his legs compared to his baseline. He denies any sweating, fevers, confusion, or other symptoms concerning for baclofen withdrawal or overdose. At baseline, he has no voluntary movement in his legs. He has not had any prior malfunctions with his baclofen pump.

Vital Signs T 36.9°C, HR 103, RR 18, BP 101/55, SpO2 96% on room air

Pertinent Labs WBC 8.4 Urinalysis and urine toxicology negative

Physical Exam Alert and oriented to self, place, and year Pupils equal, round, and reactive to light Extraocular movements intact bilaterally Tongue midline, face symmetric Bilateral upper extremities 5/5 strength in all muscle groups Bilateral lower extremities no movement with increased tone on passive range of motion and increased spasticity Abdominal and back incisions clean dry and intact without swelling or erythema No pruritus or piloerection

Baclofen Pump Interrogation Baclofen: 2000 mcg/ml concentration Infusion mode: simple continuous

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Infusion rate: NA Daily dose: 1199.2 mcg/day Reservoir volume: 0 Estimated elective replacement indicator: 70 months

Triage Management This patient has an empty intrathecal baclofen pump and must be urgently evaluated for any signs or symptoms of baclofen withdrawal, which can be life-threatening. The patient is mildly tachycardic and only has a slight increase in his tone and spasticity compared to his baseline. Fortunately, his mental status is intact, he is hemodynamically stable, and thus he does not appear to be in acute baclofen withdrawal. On imaging, his pump has flipped with the access port facing internally. He will need urgent operative management to revise and refill his pump. He will be admitted to the neurocritical care unit for close monitoring of acute baclofen withdrawal while surgery is planned. This is an urgent and operative consult.

Assessment This is an 18 year old male with an intrathecal baclofen pump for spastic paraplegia who presents with increased tone and an empty baclofen pump. Imaging demonstrates a flipped pump, preventing access to refill the pump.

Plan n n

n

n n n n

Urgent (same-day or next-day) surgery for baclofen pump revision and filling If the patient is admitted for next-day surgery, he will need frequent evaluation of vitals and neurological checks in the neurocritical care unit to monitor for signs of baclofen withdrawal. Oral or IV baclofen; if the patient develops worsening withdrawal symptoms, benzodiazepines can also be given. NPO with IV fluids Coagulation studies in preparation for surgery Consider CT L-spine without contrast to evaluate catheter position prior to surgery Postoperatively, the patient can be admitted to the floor with close monitoring of signs and symptoms of baclofen overdose. If there are concerns for overdose, the patient will need a higher level of care.

LEARNING POINTS

• Baclofen is a medication used to treat spasticity and can be given orally, intravenously, or intrathecally. Intrathecal pumps are often preferred to oral baclofen in patients with spasticity in order to directly deliver baclofen to the cerebrospinal fluid (CSF). • The etiologies of IT baclofen pump malfunctions may be varied and include pump disconnection, displacement from the intrathecal space, fibrosis at the tip, breaks/kinks/ holes in catheter, disconnection between the proximal and distal catheter, battery failure, pump failure, and programmable chip malfunction.1,2 Symptoms of baclofen overdose include lightheadedness, drowsiness, confusion, • muscle atony, and ataxia. Treatment includes standard life support measures, the use of parasympathomimetics, reversible cholinesterase inhibitors, physostigmine 2 mg intravenously, and CSF drainage via lumbar drain.1

p

319

• Baclofen withdrawal occurs due to central nervous system hyperexcitability. • Mild symptoms can be nonspecific and include sudden increase in spasticity, pruritus without a rash, fever, seizures, tachycardia, and piloerection.1

• More severe symptoms include hyperthermia, hemodynamic instability, and altered mental status. This can then progress to rhabdomyolysis, disseminated intravascular coagulation, multiorgan failure, cardiac arrest, coma, and death.1 • High doses of benzodiazepines and oral/IV baclofen can be used to treat withdrawal while diagnosis of IT pump system failure is pursued.1 • In the case of life-threatening symptoms, supportive treatment is important with an emphasis on maintaining airway, ventilation, and circulatory support in a critical care setting.2 • Definitive treatment of withdrawal due to pump complications consists of restoration of a functioning IT delivery system. • The baseline functional status of patients with an IT baclofen pump can vary greatly, and it is important to understand the indication for baclofen (e.g. cerebral palsy, traumatic paresis). • The symptoms of acute baclofen withdrawal and overdose are nonspecific and can overlap with other conditions such as infection/sepsis, medication or drug use, metabolic abnormalities, and post-anesthesia recovery. It is important to simultaneously evaluate for these treatable and reversible etiologies. • It is important to compare new imaging to prior baseline imaging of the patient’s baclofen pump and catheter to assess for any changes, which can be subtle. • To determine the site of IT drug delivery failure:1 I. Interrogate the pump. Check the programming and filling status of the pump with the system telemetry to rule out programming errors and/or an empty pump reservoir. II. Perform AP and lateral X-rays, looking for disconnections, kinks, and dislodgment of the catheter. III. If the X-rays are nondiagnostic, real-time fluoroscopy can be used to check the pump rotor function after programming a 90-degree pump rotor rotation and visual radiographic observation. IV. If the pump is functional, one can attempt side port aspiration of 2 to 3 mL of CSF (necessary to remove baclofen from the catheter and the possibility of baclofen overdose during subsequent tests). If successful, an IT baclofen bolus can be programmed through the pump–catheter system as a therapeutic trial. V. Fluoroscopy with iodinated contrast injection of 3 mL through the accessory port may be used to analyze catheter continuity, connectedness, and IT position (again, 2–3 mL of CSF should be aspirated to avoid IT baclofen bolus). Disconnection of the catheter from the pump, leaks, or perforations, as well as catheter tip dislodgments or migration, can sometimes be visualized after contrast injection. CT imaging of the entire pump and catheter system can reveal several potential abnormalities with more accuracy than plain films or fluoroscopy.

References 1. Chapter 37 Recognition and Management of Intrathecal Baclofen and Narcotic Withdrawal Syndromes. In: Loftus C, ed. Neurosurgical Emergencies. 3rd ed. Thieme; 2017. 2. Saulino M, Anderson DJ, Doble J, et al. Best practices for intrathecal baclofen therapy: troubleshooting. Neuromodulation. 2016;19(6):632–641.

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66

Baclofen Pump Infection Due To Wound Breakdown Nancy Abu-Bonsrah, MD n

Ann Liu, MD

Consult Page 54F with wound breakdown at the abdominal site of her intrathecal baclofen pump

Initial Imaging None

Walking Thoughts What are the patient’s vital signs? Is she hemodynamically stable? What is the patient’s neurological exam? n Is there concern for sepsis and/or meningitis? Is she on antibiotics or should they be initiated? n Why does the patient have a baclofen pump? n Where is the wound breakdown and how long has she had this? Is there purulence, drainage, and/or erythema? n Is the hardware exposed? n Is there concern for pump malfunction? Is there concern for either baclofen overdose or withdrawal? n What additional studies (e.g. X-ray, CT, lumbar puncture, bloodwork) need to be ordered? n Can this managed by local wound care, or does she require surgery for revision? If so, what should be the timing for surgery? n If she needs complete removal of her intrathecal baclofen pump, can she be transitioned to oral baclofen? n n

History of Present Illness A 54 year old female with a remote C5 spinal cord injury, resultant spastic quadriplegia with an intrathecal baclofen pump, hydronephrosis, and neurogenic bladder with a right nephrostomy is admitted to the medicine service with a dislodged nephrostomy tube and urosepsis. During this current hospitalization, her primary team notes one day of wound breakdown of her abdominal baclofen pump insertion site. One month prior, the patient underwent elective replacement of her baclofen reservoir located in her right abdomen. Two weeks prior, she noticed that her right nephrostomy tube was dislodged, and she was admitted to the hospital for replacement. This was complicated by septic shock from a urinary tract infection (UTI), for which she is being treated with amikacin due to a 320

66—Baclofen PumP InfecTIon Due To WounD BreakDoWn

321

history of multidrug-resistant UTIs. Currently, she denies any changes in spasticity or her baseline quadriplegia, pain at her abdominal incision, fevers, or chills. She is not on any antiplatelet or anticoagulant medications.

Vital Signs T 35.8°C, HR 97, RR 20, BP 107/65, SpO2 100% on room air

Pertinent Labs WBC 8.3, ESR 75, CRP 4.8

Physical Exam Cachectic Alert and oriented to self, place, and year Pupils equal, round, and reactive to light Extraocular movements intact bilaterally Tongue midline, face symmetric Minimal movement with baseline contraction deformities in her arms and legs Right abdominal incision open with exposed hardware and gross purulence Back incision well healed Right flank urostomy in place Baclofen pump interrogation reveals no error messages

Triage Management This is a patient who presents with wound breakdown at the abdominal site of her intrathecal baclofen pump. She is currently hemodynamically stable, but she will need to be closely monitored for signs of systemic infection, sepsis, or meningitis. There does not seem to be any issue with the function of her pump, and she currently does not have any signs or symptoms of baclofen overdose or withdrawal. Given the proximity of her abdominal incision to her urostomy, special care should be taken to ensure that her incision is not contaminated with urine. She will undergo cerebrospinal fluid (CSF) sampling via lumbar puncture (LP) to evaluate for infection; if there is concern for meningitis, her antibiotic regimen will need to be broadened. The infectious disease team will be consulted to guide her antibiotic regimen. Because her abdominal incision had dehisced entirely and is grossly purulent, she will need surgery for washout and baclofen pump removal to allow that area to heal and to fully treat her infection before replacement of her intrathecal pump system. In preparation, her baclofen will be transitioned from intrathecal dosing to IV and oral administration. During this time, she will be closely monitored for baclofen overdose or withdrawal. While currently she can be admitted to the floor or the intermediate care unit, there is a low threshold for upgrading her care to a higher level (e.g. neurocritical care unit), if needed. This an urgent and operative consult.

Assessment This is a 54 year old female with spastic quadriparesis and recent baclofen pump replacement who presents with wound dehiscence and infection at her abdominal incision. Currently, she is clinically stable and will be weaned off her intrathecal baclofen. Once she is stable on oral baclofen, she will undergo surgery for removal of her baclofen pump and washout. She will also need long-term antibiotics for her wound infection.

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SECTION VII—FUNCTIONAL

Plan n n

n

n

n

n

Blood and urine cultures LP with CSF cell count, glucose, protein, and cultures to evaluate for possible concomitant meningitis n Special care must be taken to avoid puncturing or shearing the baclofen pump catheter during LP n Can consider fluoroscopy guided LP Consult to the infectious disease team for antibiotic initiation and management, especially given her history of multidrug-resistant UTI Consult to the physical medicine and rehabilitation or the neurology team for downtitration of her intrathecal baclofen and uptitration of oral or IV baclofen Admission to neurosurgical floor with neurological checks n Low threshold to upgrade to a higher level of care (intermediate care unit or neurocritical care unit) if she develops signs of sepsis or baclofen overdose/withdrawal n If there is concern for baclofen withdrawal, additional oral baclofen, IV baclofen, and/or IV benzodiazepines can be administered. Operative planning for baclofen pump removal and washout: since the patient is relatively stable and there is no emergent concern for pump malfunction, overdose, withdrawal, or sepsis, there is time for preoperative optimization, as the entire pump will be removed.

LEARNING POINTS

• Baclofen pump infections range from superficial to deep tissue infections and can cause meningitis given the connection to the intrathecal space.

• Several reports describe an increased risk of baclofen pump infection in children as compared to adults. This is hypothesized to be due to poor wound healing, a higher percentage of gastrostomy tubes, higher rates of urinary and fecal incontinence, and more frequent revisions in children.2 • Patients should be assessed for signs and symptoms of baclofen withdrawal, baclofen overdose, meningitis, and/or sepsis. Given the complexity and potential severity of these cases, there is a low threshold for admission to an intensive care unit for close monitoring. • Lumbar puncture can be done to assess the CSF profile for meningitis. There are limited reports on the rate of meningitis with pump infections, but these appear rare.3 • Baclofen pump infection is typically treated with antibiotics and immediate explantation. Salvage techniques to avoid removal of the pump have been described and include longterm IV antibiotics, a combination of IV and intrapump antibiotics, and local washout.1 The infectious disease team can help guide the type of antibiotics and the length of treatment. If removing the pump, consideration should be given to the downtitration of the intrathecal • baclofen dose prior to removal. Oral/IV baclofen and/or IV benzodiazepines can be used to minimize withdrawal symptoms. The physical medicine and rehabilitation or neurology teams can help with adjustments in baclofen dosing and route of administration.

References 1. Hester SM, Fisher JF, Lee MR, Macomson S, Vender JR. Evaluation of salvage techniques for infected baclofen pumps in pediatric patients with cerebral palsy. J Neurosurg Pediatr. 2012;10(6):548– 554. 2. Spader HS, Bollo RJ, Bowers CA, Riva-Cambrin J. Risk factors for baclofen pump infection in children: a multivariate analysis. J Neurosurg Pediatr. 2016;17(6):756–762. 3. Wunderlich CA, & Krach LE. Gram-negative meningitis and infections in individuals treated with intrathecal baclofen for spasticity: a retrospective study. Dev Med Child Neurol, 48(6), 450–455.

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67

Abnormal Eye Movement Kurt Lehner, MD n Ann Liu, MD

Consult Page 5Y female presenting with eye movement abnormalities. MRI with Chiari malformation.

Initial Imaging

Fig. 67.1 Sagittal T2-weighted MRI of the brain without contrast demonstrates cerebellar tonsillar ectopia with crowding of the foramen magnum and 11 mm of downward tonsillar herniation. There is no spinal cord or brainstem signal abnormality.

Walking Thoughts n

n n n n

n

n

What type of eye movement abnormality does she have? Does she have any other symptoms (e.g. headache, paresthesias, weakness)? Does the patient have any signs or symptoms of hydrocephalus? Does the patient have any signs or symptoms of brainstem compression? Do these symptoms worsen with Valsalva maneuvers? Does the patient’s history and exam correlate with the imaging findings? Is she symptomatic from her Chiari I malformation or is it an incidental finding? What is her prior medical history? Does she have any congenital malformations (e.g. spina bifida)? Does she need additional imaging (e.g. MRI of the spine)?

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SECTION VII—FUNCTIONAL

History of Present Illness A previously healthy 5 year old female presents to the emergency department (ED) with her right eye intermittently turning inward (esotropia). Her mother first noticed this a few days prior to presentation before the patient went to bed. It lasted for only a few minutes and self-resolved. Her mother called her pediatrician, who recommended outpatient follow-up in one week. However, the esotropia returned earlier this evening, and the mother brought the patient to the ED. The mother reports that the patient has not had any headaches, nausea, vomiting, diplopia, difficulty walking, difficulty with eating, changes in sensation, or changes in her bowel and bladder function. An MRI obtained in the ED demonstrates cerebellar tonsillar ectopia with 11 mm of downward herniation, consistent with a Chiari I malformation.

Vital Signs T 37.8°C, HR 110 (normal 70–115), RR 22, BP 95/50

Pertinent Labs None

Physical Exam Awake, interactive Oriented to self, place, and age Pupils equal, round, and reactive to light Mild right eye esotropia on resting gaze Extraocular movements intact bilaterally Tongue midline, face symmetric Moving all extremities well

Triage Management This patient presents with an incidentally found Chiari I malformation. On examination, she has new right esotropia but is otherwise intact. The transient nature of her esotropia without other warning signs of increased intracranial pressure, as well as the lack of classical Chiari I symptoms, such as a tussive headache, suggests that this radiographic finding is most likely unrelated to the patient’s presenting symptom. Subtle findings related to a syrinx may be difficult to detect by physical exam alone, so she will need an MRI of her cervical and thoracic spine to assess for this. She will also undergo further evaluation by ophthalmology for the esotropia. This is a nonurgent consult. She does not need an operation at this time and further workup of her Chiari I malformation can be done as an outpatient.

Assessment This is a previously healthy 5 year old female who presents with isolated right esotropia. MRI of her brain demonstrates a Chiari I malformation that is unlikely to be related to her presentation.

67—AbnorMAl EyE MovEMEnt

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Plan n n

n n

No acute neurosurgical intervention Consult to ophthalmology to assess her eye movement abnormalities (this can also be done as an outpatient) Outpatient MRI of the cervical and thoracic spine without contrast to evaluate for syrinx Outpatient neurosurgical follow-up

LEARNING POINTS

• Chiari I malformation is thought to be secondary to a developmental defect creating a smaller-than-normal posterior fossa with subsequent cerebellar tonsillar herniation. This is typically defined as cerebellar herniation >5 mm, although patients can be symptomatic with less herniation or asymptomatic with much greater herniation.3 • Common presenting symptoms include occipital headaches that are worsened with Valsalva maneuvers (e.g. coughing, laughing, crying, straining with bowel movements). Other symptoms can be related to compression of the brainstem by the odontoid process with severe herniation, cranial settling, or from the development of a syrinx within the spinal cord. • In the pediatric population, symptoms can be vague and nonspecific, including persistent crying, oropharyngeal dysfunction, failure to thrive, apnea, and developmental delay.2 • Papilledema can develop with the obstruction of cerebrospinal fluid (CSF) flow and hydrocephalus. • A syrinx is a fluid-filled cavity within the spinal cord (syringomyelia) or brainstem (syringobulbia) and is thought to develop from CSF flow obstruction. Syrinx associated with Chiari I malformation is thought to be progressive, and asymptomatic individuals can become symptomatic if the syrinx is untreated. Thus the presence of a large and/or lobulated syrinx can be an indication for surgery, regardless of symptoms.2 • The work-up of a Chiari I malformation, including associated imaging, is typically done as an outpatient over a period of weeks to months. There are few exceptions to this: a patient with progressive neurological symptoms attributable to the Chiari I malformation, hydrocephalus, or the presence of a syringobulbia will likely require expedited work-up and decompression.2 • If a syrinx is present or there is concern for hydrocephalus or abnormal CSF flow at the cervicomedullary junction, MRI sequences evaluating CSF dynamics (such as constructive interference in steady state [CISS] and CINE) may be helpful. • Posterior fossa decompression with or without duraplasty is considered first-line treatment. There is an ongoing randomized clinical trial comparing the efficacy of posterior fossa decompression with and without duraplasty for treatment of Chiari I malformation with syringomyelia.4 • A frequent consult question is the safety of performing a lumbar puncture (LP) in a patient with Chiari I malformation. The concern arises from creating a large pressure gradient between the intracranial space and the lumbar cistern across the foramen magnum. This may theoretically lead to acute cervical spine or medullary compression from further tonsillar herniation. Unfortunately, there is no consensus on the safety of LP in patients with Chiari I malformation, and each case must be evaluated on an individual basis. In general, low-volume LPs in asymptomatic individuals may be safe. In patients with more concerning symptoms or an exam concerning for brainstem/spinal cord compression, LP should only be pursued in extenuating circumstances with extreme caution.1

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References 1. Engelborghs S, Niemantsverdriet E, Struyfs H, et al. Consensus guidelines for lumbar puncture in patients with neurological diseases. Alzheimers Dement (Amst). 2017; 2017:8, 111–126. 2. Entezami P, Gooch MR, Poggi J, Perloff E, Dupin M, Adamo MA. Current management of pediatric Chiari type 1 malformations. Clin Neurol Neurosurg. 2019;176:122–126. 3. Milhorat TH, Chou MW, Trinidad EM, et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44(5):1005–1017. 4. Posterior fossa decompression with or without duraplasty for Chiari type I malformation with syringomyelia. https://clinicaltrials.gov/ct2/show/NCT02669836.

C H A P T E R

68

Right Facial Pain Brendan F. Judy, MD n

Ann Liu, MD

Consult Page 51F with 3d unremitting right facial pain

Initial Imaging None

Walking Thoughts n

n n n n n

What are the characteristics and distribution of the facial pain? Is it shooting and electric-like? Has the patient had any prior episodes of this pain? Does she have any additional symptoms? Does the patient have any imaging? What treatments, if any, has the patient tried? What are the patient’s medical comorbidities?

History of Presenting Illness A 51 year old female with well-controlled hypertension presents to the emergency department with three days of right-sided electric, shooting facial pain. Her pain began four months ago, and she was evaluated by a neurologist, who started her on carbamazepine. Her pain was initially controlled with the medication, but she has had flare-ups which have been treated with uptitration of carbamazepine and the addition of gabapentin. She has been on maximal medical therapy for one month and her pain is unrelenting. She has been unable to speak or eat for the past three days because these actions trigger the pain. She denies headache, vision changes, numbness in her face, hearing changes, and weakness. She has no history of dental procedures or maxillofacial pathology.

Vital Signs T 37.6°C, HR 85, RR 30, BP 170/60, SpO2 98% on room air

Pertinent Labs Na 130, Cr 1.1, Hgb 15, WBC 11, Plt 200, INR 1.0, aPTT 27.4

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Physical Exam Disheveled, in severe pain Awake, alert, nods appropriately to orientation questions (name, location, year) She does not speak or move her face due to pain Right V3 distribution pain which does not cross midline Intact facial sensation bilaterally Face symmetric, tongue midline No pronator drift Full strength in all four extremities

Triage Management This is a middle-aged patient who presents in acute distress with facial pain consistent with trigeminal neuralgia. She has not undergone brain imaging and will need an MRI of her brain to evaluate for structural causes of her pain. Her hyponatremia is likely secondary to her carbamazepine. Despite maximal first-line medical therapy, she continues to have severe pain affecting her quality of life. This warrants surgical intervention pending her MRI results. This is a nonemergent but possibly operative consult.

Assessment This is a 51 year old female with an acute severe flareup of her right V3 distribution trigeminal neuralgia despite maximal first-line medical therapy.

Plan n

n n

n

Admit to neurosurgical floor for pain control, uptitration of medical therapy, and possible surgical intervention Can consider steroids (dexamethasone 4 mg every 6 hours) MRI brain with and without contrast, high-resolution constructive interference in steady state (CISS) sequences (Figure 68.1) Operative planning for percutaneous rhizotomy (e.g. glycerin or radiofrequency ablation) or microvascular decompression pending MRI results

Fig. 68.1 Axial CISS sequence brain MRI without contrast demonstrates compression of the right trigeminal nerve by the right superior cerebellar artery (arrow).

68—Right Facial Pain

329

LEARNING POINTS

• Trigeminal neuralgia (TN), also known as tic douloureux, is a chronic pain syndrome diagnosed clinically. Type I TN, otherwise known as classical TN, is characterized by sharp, shooting, stabbing pain that is commonly exacerbated by cutaneous triggers such as shaving, eating, combing hair, changing temperatures, or speaking. Type II TN, otherwise known as atypical facial pain, is characterized by constant, dull, burning pain. • The mechanisms underlying TN include vascular or tumoral compression of the nerve, or demyelinating plaques of the root entry zone seen in multiple sclerosis. It can also be idiopathic. • Microvascular compression was first hypothesized to be a mechanism of TN in 1932 and later proved and popularized by Dr. Peter Janetta.3 The superior cerebellar artery is classically found to compress the trigeminal nerve in TN. Vascular compression of cranial nerves VII and IX by the anterior inferior cerebellar artery and posterior inferior cerebellar artery, respectively, can result in hemifacial spasm or glossopharyngeal neuralgia. Other brainstem vessels, including veins or persistent primitive vessels, may also be implicated. • First-line treatment for TN is pharmacological therapy with carbamazepine or oxcarbazepine. Baclofen, lamotrigine, and IV phenytoin can also be considered.2 Studies of the long-term efficacy of medical management are limited, but if the pain becomes medically refractory, then several other procedures are available, including stereotactic radiosurgery (SRS), percutaneous rhizotomy, and microvascular decompression (MVD). • MVD for TN is the microsurgical separation of the trigeminal nerve and the offending vessel. Percutaneous rhizotomy intentionally damages the trigeminal nerve via chemical or thermal means to decrease pain sensation. • If there is evidence of structural compression of the trigeminal nerve on MRI, MVD is the only option that treats the problem rather than the symptoms. MVD results in pain relief in 70%–90% of patients.1,4,6 When comparing MVD with SRS, MVD results in superior rates of short- and long-term pain relief, and less recurrence. However, there are higher postoperative complications including cerebrospinal fluid leak, hearing loss, and wound infection.4 • For patients with multiple medical comorbidities who may not be able to tolerate MVD, radiosurgery and percutaneous rhizotomy are safe options. • SRS methods include Gamma Knife, linear accelerator, and Cyberknife. Radiosurgery results in median rates of freedom from pain without medication ranging from 43%–58%. Median time to relief ranges from 8.5 to 90 days. Median recurrence rates ranges from 23%–29%. Complications include hypesthesia, paresthesia, dysesthesia, dry eye, and keratitis.7 • Percutaneous treatments include glycerol rhizotomy (GR), radiofrequency thermocoagulation (RF), and balloon compression. A systematic review comparing these three techniques found that RF is associated with higher odds for pain relief compared to GR.5 • The choice of treatment must be tailored to each patient and reflect the patient’s medical comorbidities, acceptance of procedural risk, and expectations for immediate versus delayed symptom relief. • Recurrent facial pain after MVD, SRS, or percutaneous treatments may be retreated, although the rates of pain control are lower. • In general, type II TN is more recalcitrant to treatment and has lower rates of immediate postoperative pain relief and higher rates of pain recurrence.8 • Anesthesia dolorosa, or deafferentation pain, is a rare but dreaded complication of TN treatment. It consists of constant neuropathic pain and is extremely difficult to treat. Because TN is a chronic pain condition, patients may be on a multitude of pain medications • requiring coordination with their pain management physician or neurologist for downtitration after surgery.

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SECTION VII—FUNCTIONAL

References 1. Barker 2nd FG, Jannetta PJ, Bissonette DJ, Larkins MV, Jho HD. The long-term outcome of microvascular decompression for trigeminal neuralgia. N Engl J Med. 1996;334(17):1077–1083. 2. Cruccu G, Gronseth G, Alksne J, et al. AAN-EFNS guidelines on trigeminal neuralgia management. Eur J Neurol. 2008;15(10):1013–1028. 3. Jannetta PJ. Arterial compression of the trigeminal nerve at the pons in patients with trigeminal neuralgia. J Neurosurg. 1967;26(1):159–162. 4. Lu VM, Duvall JB, Phan K, Jonker BP. First treatment and retreatment of medically refractive trigeminal neuralgia by stereotactic radiosurgery versus microvascular decompression: a systematic review and metaanalysis. Br J Neurosurg. 2018;32(4):355–364. 5. Texakalidis P, Xenos D, Tora MS, Wetzel JS, Boulis NM. Comparative safety and efficacy of percutaneous approaches for the treatment of trigeminal neuralgia: a systematic review and meta-analysis. Clin Neurol Neurosurg. 2019;182:112–122. 6. Theodros D, Goodwin C, Bender MT, et al. Efficacy of primary microvascular decompression versus subsequent microvascular decompression for trigeminal neuralgia. J Neurosurg. 2017;126(5):1691– 1697. 7. Tuleasca C, Régis J, Sahgal A, et al. Stereotactic radiosurgery for trigeminal neuralgia: a systematic review. J Neurosurg. 2018;130(3):733–757. 8. Tyler-Kabara EC, Kassam AB, Horowitz MH, et al. Predictors of outcome in surgically managed patients with typical and atypical trigeminal neuralgia: comparison of results following microvascular decompression. J Neurosurg. 2002;96(3):527–531.

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69

Right Arm Swelling After Surgery Nancy Abu-Bonsrah, MD n

Daniel Lubelski, MD

Consult Page 30F with recent spine surgery, here with right arm swelling

Initial Imaging None

Walking Thoughts n n n n n

n n

n

What surgery did she have? How long ago was her surgery? What was her postoperative course? When and where did she discharge? What is her level of mobility? What are the patient’s current vital signs? Is she hemodynamically stable? What is the mechanism and timeline of her arm swelling? Did she have any trauma? Does she have any risk factors for hypercoagulability? What studies need to be ordered (ultrasound, CT angiogram [CTA] of the chest)? Does she need to be started urgently on anticoagulation? If so, what are the risks and benefits associated with that decision? What is the most appropriate anticoagulation agent to use in the postoperative setting?

History of Present Illness A 30 year old female with recent cervicothoracic spine surgery presents to the emergency department (ED) with one day of right arm swelling. She underwent surgery one week prior after presenting to our hospital with progressive bilateral leg weakness and urinary retention, and was found to have an expansile, intramedullary cervicothoracic lesion concerning for an ependymoma (Figure 69.1). The patient underwent an uncomplicated C5-T4 laminoplasty for resection of the lesion with C4-T5 instrumentation and fusion. Postoperatively, she had bilateral weakness in her arms and legs (right worse than left, legs worse than arms) with the inability to walk unassisted. Her postoperative course was otherwise unremarkable, and she was discharged to inpatient rehabilitation on postoperative day 6. While in rehabilitation, she was noted to have right upper extremity swelling without irritation or pruritus. She denied swelling, pain, irritation, pruritus, or erythema in her other extremities. She also denied any shortness of breath or recent IV infiltration.

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69—Right ARm Swelling AfteR SuRgeRy

Fig. 69.1 Preoperative sagittal T2-weighted cervicothoracic spine MRI demonstrates an expansile, hyperintense intramedullary lesion spanning from C5 to T4. There is hyperintensity within the cord from C2 to C5.

Vital Signs T 36.8°C, HR 88, BP 108/55, SpO2 97% on room air

Pertinent Labs Na 146, Hgb 10.4, WBC 8.63, Plt 233, INR 1.1, aPTT 21.7

Physical Exam Alert and oriented to self, place, and year Pupils equal, round, and reactive to light Extraocular movements intact, face symmetric, tongue midline RUE LUE

Deltoid 4/5 5/5

RLE LLE

Hip Flexion 1/5 2/5

Biceps 4/5 4/5

Triceps 4/5 4/5

Knee Extension 1/5 3/5

Wrist Flexion 4-/5 4/5 Knee Flexion 1/5 4/5

Patchy sensation in all extremities Incision clean, dry, and well healed Right forearm swelling, erythema, and tenderness to palpation

Wrist Extension 4-/5 4/5

Dorsiflexion 1/5 4/5

Grip 4-/5 4/5

Plantar Flexion 1/5 4/5

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SECTION VIII—POSTOPERATIVE COMPLICATIONS

Triage Management The patient underwent recent major surgery for a cervicothoracic intramedullary ependymoma resection. She was discharged to inpatient rehabilitation but returns to the hospital with right arm swelling concerning for a DVT. Her recent surgery, decreased mobility, and her tumor history places her at a higher risk of hypercoagulability. Currently, she is hemodynamically stable without tachycardia or shortness of breath, but she will need imaging to evaluate for possible DVT. Additionally, the threshold to rule out pulmonary emboli (PE) is low. This is an urgent but nonoperative consult.

Follow-up Imaging In the ED, a right upper extremity ultrasound revealed a near-occlusive DVT of the right subclavian, axillary, and brachial veins. Ultrasound of the right lower extremity revealed an extensive acute, occlusive DVT involving the entirety of her right lower extremity veins. Ultrasounds of her left upper extremity and lower extremity were negative.

Assessment This is a 30 year old female who underwent recent surgery for resection of a cervicothoracic intramedullary tumor who now presents one week after surgery with postoperative DVTs. Although her surgery was recent, given the extensive nature of her DVTs, the benefits of anticoagulation outweigh the risks of surgical site hemorrhage. She is currently hemodynamically stable, at her neurological baseline, and will be admitted to the floor for initiation of anticoagulation. The timing and choice of anticoagulation will be discussed with the hematology service.

Plan n n n

Admission to neurosurgical floor with close neurological exams Low threshold to rule out a PE with a CTA of the chest Consultation to hematology for discussion of timing and choice of anticoagulation

Follow-up After consultation with hematology, the patient was started on a heparin infusion with a goal aPTT of 50–65. An inferior vena cava (IVC) filter was not recommended at that time. One day after initiation of the heparin with a subtherapeutic aPTT, she became acutely hypotensive and hypoxic. She was stabilized with oxygen supplementation and vasopressor medications. Urgent CTA of her chest revealed multiple acute, occlusive bilateral pulmonary emboli, and evidence of right heart strain with right ventricular and atrial dilatation. She was urgently transferred to the neurocritical care unit for close monitoring, and continued respiratory and blood pressure support for her cardiogenic shock. The interventional radiology and cardiothoracic surgery teams were consulted for evaluation of thrombectomy, but she was not a candidate given her recent spine surgery. Her heparin infusion was increased to a higher aPTT goal. Her cardiogenic shock resolved, and after a few days she was ultimately transferred to the floor. She was transitioned from a heparin infusion to warfarin, and she discharged back to the rehabilitation facility on hospital admission day 10. Her neurological exam remained stable throughout her hospitalization.

69—Right ARm Swelling AfteR SuRgeRy

335

LEARNING POINTS

• The rate of DVT and PE for patients undergoing spine surgery ranges from 0.3% to 31%. This is even higher in patients who experience an acute spinal cord injury (41% in one study).3 PEs can be life-threatening, and patients can acutely decompensate. It is important to ensure that the patient is hemodynamically stable. • Postoperative patients are at increased risk for DVT and/or PE due to their limited postoperative mobility, hypercoagulable state, and presurgical comorbidities (e.g. cancer, preoperative mobility status, prior history of DVT/PE).3,6 • Strategies to help reduce the risk of perioperative DVT and PE include external compression devices, early postoperative mobilization, and pharmacologic prophylaxis (e.g. unfractionated heparin, low molecular weight heparin). In patients with preoperative DVT and/or PE, the hematology team can assist in deciding the timing and type of anticoagulation as well as in rare cases, the preoperative placement of an inferior vena cava filter.1,2-4 • Historically, hesitancy to use postoperative chemoprophylaxis stems from risk of surgical site hemorrhage. In the spine, epidural hematoma is most concerning, as it can cause significant and permanent deficits. However, studies have shown that the risk of epidural hematoma after spine surgery in the setting of chemoprophylaxis is low (80 mmHg Admission to the neurocritical care unit after surgery Postoperative MAP goal >80 mmHg to avoid spinal cord hypoperfusion Continued dexamethasone taper Early mobilization and physical therapy evaluation

LEARNING POINTS

• Although the incidence of asymptomatic postoperative spinal epidural hematoma is high (60%–90%), symptomatic hematomas are rare, with an incidence of around 0.2%.3

• Risk factors include age > 60 years, preoperative nonsteroidal antiinflammatory drug use, involvement of > 5 operative levels, INR > 2 within the first 48 hours postoperatively, and preoperative coagulopathy.2,4 • Most cases occur within the first few hours after surgery, which emphasizes the importance of careful monitoring and documentation of neurological status within the early postoperative hours.1 MRI with and without contrast is the diagnostic test of choice for spinal epidural fluid •

71—PostoPerAtIve Leg WeAkNess, NUmbNess, ANd UrINAry INcoNtINeNce

343

LEARNING POINTS—cont’d collections. A hematoma can be distinguished from infection, pseudomeningocele, or other etiology because of its high T1-signal intensity, absence of thecal sac communication, and absence of disc involvement.6 In the event of an emergency or if the patient cannot tolerate the MRI, sequences can be obtained without contrast to expedite diagnosis. Because the suspicion for infection was low in the present case, contrast was not given. • Management of symptomatic spinal epidural fluid collections is often surgical, especially when there is a clear clinical picture of spinal cord compression and worsening neurological function.1 Timing matters: urgent and timely decompression leads to improved outcomes.5 •

References 1. Amiri AR, Fouyas IP, Cro S, Casey ATH. Postoperative spinal epidural hematoma (SEH): incidence, risk factors, onset, and management. Spine J. 2013;13:134–140. 2. Awad JN, Kebaish KM, Donigan J, Cohen DB, Kostuik JP. Analysis of the risk factors for the development of post-operative spinal epidural haematoma. J Bone Joint Surg Br. 2005;87:1248–1252. 3. Glotzbecker MP, Bono CM, Wood KB, Harris MB. Postoperative spinal epidural hematoma: a systematic review. Spine (Phila Pa 1976). 2010;35:E413–E420. 4. Kou J, Fischgrund J, Biddinger A, Herkowitz H. Risk factors for spinal epidural hematoma after spinal surgery. Spine (Phila Pa 1976). 2002;27:1670–1673. 5. Lawton MT, Porter RW, Heiserman JE, Jacobowitz R, Sonntag VK, Dickman CA. Surgical management of spinal epidural hematoma: relationship between surgical timing and neurological outcome. J Neurosurg. 1995;83:1–7. 6. Radcliff K, Morrison WB, Kepler C, et al. Distinguishing pseudomeningocele, epidural hematoma, and postoperative infection on postoperative MRI. Clinical Spine Surgery. 2016;29:E471–E474.

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72

Drainage From Cranial Incision Alice L. Hung, MD n Daniel Lubelski, MD

Consult Page 47F with recent brain surgery presenting with drainage from wound

Initial Imaging

Fig. 72.1 A photograph of the scalp demonstrates a small area of wound dehiscence down to the bone with yellowish discharge expressible with gentle pressure.

Walking Thoughts What was the patient’s prior surgery? When was the surgery? How was the patient’s postoperative course? n How long has the patient had drainage from the incision? n Is there wound dehiscence? If so, is it deep or superficial? n What does the drainage look like (e.g. purulent vs. serous vs. bloody)? Is there a CSF leak? n Does the patient have fevers, chills, meningitis, or other systemic signs of infection? n n

344

72—DraInage From CranIal InCIsIon n

n

n

345

What is the patient’s current neurological exam? Are there any deficits to suggest intracranial involvement? Does the patient have any risk factors for poor wound healing or infection (e.g. diabetes, obesity, smoking, cancer history, prior radiation)? Were there any intraoperative risk factors for infection (e.g. prolonged case, use of implants, revision surgery, prior infection)?

History of Present Illness A 47 year old female with a history of migraines and prior left craniotomy for an incidentally found 3 cm parasagittal meningioma presents to the emergency department (ED) after noticing yellowish drainage from her incision. She had surgery one month prior and had an uncomplicated intraoperative and postoperative course with standard perioperative antibiotics. Her postoperative MRI demonstrated no residual tumor. She was discharged home on postoperative day 3. The patient was washing her hair earlier in the day when a scab from her incision peeled off, and she felt some drainage from this area. She initially called the neurosurgery clinic with concerns about this and was advised to come to the ED. She has otherwise been doing well since surgery. She denies any fevers, chills, headaches, neck stiffness, nausea, vomiting, seizures, weakness, numbness, or other focal neurological deficits. She does not have a history of diabetes, obesity, smoking, cancer, or prior radiation. She is not taking antiplatelet or anticoagulant medications and has completed her postoperative steroid taper. A head CT without contrast demonstrates postsurgical changes at her left craniotomy and a small epidural isodense collection without significant mass effect (Figure 72.2).

Fig. 72.2 Coronal view of a head CT without contrast demonstrates postsurgical changes at the left craniotomy and a small epidural isodense collection (arrow) without significant mass effect

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SECTION VIII—POSTOPERATIVE COMPLICATIONS

Vital Signs T 36.6°C, HR 73, BP 115/66, SpO2 97% on room air

Pertinent Labs Hgb 12.0, WBC 5.85, Plt 321, ESR 23, CRP 0.1

Physical Exam Awake, alert, oriented to self, place, and year Pupils equal, round, and reactive to light Extraocular movements intact, face symmetric, tongue midline No pronator drift Bilateral upper extremities 5/5 Bilateral lower extremities 5/5 Sensation intact to light touch throughout Approximately 1 cm area of dehiscence along the incision near the vertex with a depth down to bone, and thick, yellowish-white purulent fluid that can be expressed No evidence of a CSF leak

Triage Management The patient had cranial surgery a month ago and has been doing well without focal neurological deficits or altered mental status to suggest meningitis or an intradural abscess. She is currently hemodynamically stable without concern for sepsis. Her exam, however, is concerning for local wound infection. Given that there is frank pus and full-thickness wound dehiscence, she will need a wound washout. Because her head CT shows an epidural isodense collection, she will undergo MRI urgently to assess for a deeper infection. She will be admitted to the floor for surgical planning. Should there be any concern for bacteremia or meningitis, a higher level of care may be warranted. The plastic surgery service may be consulted to assist with complex wound closure, and the infectious disease service will manage her antibiotic plan. This is a nonemergent but operative consult.

Follow-Up Imaging Brain MRI with and without contrast in the ED demonstrates epidural contrast enhancement at the surgical site (Figure 72.3) without diffusion restriction. On review of the initial surgery’s operative note, a dural onlay and fibrin glue were used. Because her MRI does not show an obvious abscess (due to the lack of diffusion restriction), the epidural collection seen on both CT and MRI likely represents postsurgical changes from the materials used in her original surgery.

Assessment This is a 47 year old otherwise healthy female who presents 1 month after an uncomplicated craniotomy for a parasagittal meningioma with wound breakdown and purulent drainage. Based on her physical exam and head imaging, the infection appears to be superficial without an obvious intracranial abscess. She will be admitted for surgical washout and antibiotic initiation.

72—DraInage From CranIal InCIsIon

347

Fig. 72.3 Coronal view of a T1-weighted brain MRI with contrast demonstrates epidural contrast enhancement at the surgical site (arrow). Diffusion sequences (not shown here) did not demonstrate diffusion restriction.

Plan n n n n n n

Admit to the neurosurgical floor with close neurological monitoring Blood cultures Preoperative coagulation bloodwork NPO/IV fluids Consult to infectious disease for timing and choice of antibiotic initiation Surgical planning for wound washout n Posting highlights: n Multiple culture swabs (for each cranial compartment) n Irrigation (with or without antibiotics) n For anesthesia: once intraoperative cultures are obtained, broad-spectrum IV antibiotics can be administered

LEARNING POINTS

• Postoperative infection after craniotomy for tumor occurs between 1% and 25%. • Numerous studies have investigated factors that may be associated with a higher risk of developing postoperative surgical site infection after craniotomy. Based on a meta-analysis by Fang et al3 of 26 studies, postsurgical site infection was associated with factors such as CSF leak from the original operation, long duration of surgery, venous sinus entry, and repeat operations. Other factors such as emergent procedure, antibiotic prophylaxis, steroid use, and comorbidities such as diabetes mellitus and obesity have been shown to be associated with wound infection in individual studies but did not reach statistical significance in this meta-analysis.3 In postoperative patients who present with fever but without surgical site issues, other • causes of postoperative fever must be evaluated, including urinary tract infection, pneumonia, deep venous thrombosis, pulmonary embolus, and drug fever.

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SECTION VIII—POSTOPERATIVE COMPLICATIONS

 

• When consulted for a patient with concerns for postoperative wound breakdown, it is important to first discern whether this is a superficial infection in a stable patient or a deeper infection with neurological changes, or other systemic instability. • Patients with a superficial infection (e.g. cellulitis, stitch abscess) without wound dehiscence or significant purulent discharge may be initially managed conservatively with a trial of oral antibiotics, local wound care (e.g. antibiotic ointment, dressing changes), and close outpatient follow-up. • If there is concern for persistent, deeper, or systemic infection, surgical management and broad-spectrum antibiotics may be necessary. The type of surgery, antibiotic course, level of care, and expected postoperative recovery may differ depending on the extent of infection. Broad antibiotic coverage and source control are essential. • Intraoperatively, each cranial compartment (subgaleal, epidural, and possibly subdural) may be assessed for infection and cultured.2 Depending on the involvement of the bone flap, several intraoperative strategies may be considered: soaking the bone in iodine, drilling of the infected bone, replacement with a titanium cranioplasty, or leaving the bone off altogether. • The most common organisms found in postoperative infections include gram-positive bacteria, such as Staphylococcus aureus, Staphylococcus epidermidis, and Cutibacterium acnes. Gram-negative organisms such as Enterobacter cloacae, Klebsiella pneumoniae, and Pseudomonas aeruginosa, are less commonly found.4 • Postoperative surgical site infections are associated with increased hospital readmissions, prolonged hospital stays, and higher risk of reoperation.1 • Complex wound closure with assistance from plastic surgery may be needed in patients with a history of multiple prior surgeries, radiation, or cosmesis concerns.

References 1. Chiang HY, Kamath AS, Pottinger JM, et al. Risk factors and outcomes associated with surgical site infections after craniotomy or craniectomy. J Neurosurg. 2014;120(2):509–521. https://doi.org/10.3171/2013.9 JNS13843. 2. Dashti SR, Baharvahdat H, Spetzler RF, et al. Operative intracranial infection following craniotomy. Neurosurg Focus. 2008;24(6):E10. https://doi.org/10.3171/FOC/2008/24/6/E10. 3. Fang C, Zhu T, Zhang P, Xia L, Sun C. Risk factors of neurosurgical site infection after craniotomy: a systematic review and meta-analysis. America Journal Infection Control. 2017;45(11):e123–e134. https:// doi.org/10.1016/j.ajic.2017.06.009. 4. Jiménez-Martínez E, Cuervo G, Hornero A, et al. Risk factors for surgical site infection after craniotomy: a prospective cohort study. Antimicrob Resist Infect Control. 2019;8(1):69. https://doi.org/10.1186/ s13756-019-0525-3.

C H A P T E R

73

Incisional Swelling And Drainage After Spine Surgery Jose Luis Porras, MD n

Daniel Lubelski, MD

Consult Page 61F h/o prior lumbar spine surgery here with drainage from incision

Initial Imaging

A

B

Fig. 73.1 Tender, erythematous, and swollen lumbar incision with a small area of active purulent discharge (arrow) at the superior aspect of the incision (A); there is significant drainage on the patient’s home dressing (B).

Walking Thoughts What was the patient’s prior surgery? When was the surgery? Is there hardware present? n How was the patient’s postoperative course? n What does the patient’s incision look like (e.g. erythema, swelling) and is there any drainage? n If there is drainage, what does it look like? n Does the patient have fevers, chills, meningitis, or other systemic signs of infection? n What is the patient’s current neurological exam? n What other symptoms is the patient having? n n

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350 n

n

n

SECTION VIII—POSTOPERATIVE COMPLICATIONS

Are there symptoms of possible cerebrospinal fluid leak such as positional headaches, nausea, or vomiting? Does the patient have any risk factors for poor wound healing or infection (e.g. diabetes, obesity, smoking, cancer history, prior radiation, poor nutritional status)? Is the patient immunocompromised? Were there any intraoperative risk factors for infection (e.g. prolonged case, use of implants, revision surgery, prior infection)?

History of Present Illness A 61 year old female with a history of diabetes, obesity, hepatitis C, hypertension, tobacco use, and prior L3-L4 laminectomy and discectomy presents to the emergency department (ED) with drainage at her incision site. She initially presented to clinic with several months of radiating pain down her bilateral legs, left worse than right, and was found to have severe stenosis at L3-L4. She underwent an L3-L4 laminectomy and discectomy about two weeks prior. There were no intraoperative complications, and her postoperative recovery was uneventful, with resolution of her bilateral leg pain. She was discharged home the same day of surgery. Three days prior, the patient noted that her surgical site was itching and swollen but not leaking. Her son examined the incision and found a small area that was opening up at the top of her incision. She called the office and was recommended to perform local wound care with application of an over-the-counter antibiotic ointment and daily dressing changes. However, today she felt some drainage from her incision and came to the ED. The patient denies fever, chills, nausea, vomiting, positional headaches, weakness, numbness, tingling, or bowel and bladder dysfunction. Her preoperative bilateral leg pain has not returned. She denies recent use of antiplatelet or anticoagulant medications.

Vital Signs T 36.3°C, BP 123/78, HR 81, SpO2 98% on room air

Pertinent Labs Hgb 11.1, WBC 12.6, Plt 421, INR 0.9, aPTT 21.2 Glucose 294 ESR 55, CRP 2.3

Physical Exam Awake, alert, oriented to self, place, and year Pupils equal, round, and reactive to light Extraocular movements intact, face symmetric, tongue midline No pronator drift Bilateral upper extremities 5/5 Bilateral lower extremities 5/5 Sensation intact to light touch throughout No clonus or Hoffman’s sign Downgoing toes No hyperreflexia Tender, erythematous, and swollen incision with a small area of active purulent discharge at the superior aspect of the incision

351

73—IncISIonAl SwellIng AnD DrAInAge After SpIne Surgery

Triage Management The patient has several risk factors for surgical site infection, including diabetes, smoking, and obesity. She presents with a likely superficial wound infection a few weeks after elective spinal surgery. Her general appearance and stable vital signs do not suggest sepsis, meningitis, or a cerebrospinal fluid (CSF) leak. Although her WBC, ESR, and CRP are mildly elevated, this could be due to her recent surgery. These considerations guide the level of care and urgency of surgery. Given that she has frank pus coming from the incision, this patient will require operative exploration with wound washout, and she will be admitted to the floor for surgical planning. Should there be any concern for bacteremia or meningitis, a higher level of care may be warranted. The infectious disease team will be consulted to guide her antibiotic plan. This is a nonemergent but operative consult.

Assessment This is a 61 year old female with obesity, diabetes, hepatitis C, hypertension, and tobacco use who presents 2 weeks after an elective L3-L4 laminectomy and discectomy with a surgical site infection. The patient is afebrile and hemodynamically stable with no new focal neurological deficits on exam.

Plan n n n n

n

n n

Admit to the neurosurgical floor Blood cultures NPO and IV fluids after midnight If there is concern for deep infection, consider an MRI with and without contrast to evaluate the extent of infection Consult to the infectious disease team for timing and choice of antibiotics n Hold antibiotics pending results of operative cultures. Aggressive antibiotic treatment should begin with any signs of systemic infection or positive cultures. Consult to endocrinology for management of diabetes Surgical planning for same-day or next-day wound revision and washout n Posting highlights: n Multiple culture swabs (for each tissue compartment) n Irrigation (with or without antibiotics) n For anesthesia: hold perioperative antibiotics until intraoperative cultures are obtained

LEARNING POINTS

• The incidence of postoperative spinal wound infections or surgical site infections (SSI), are reported to be less than 15%,3 varying based on the type, location, and complexity of the index surgery. Spinal SSI can have significant morbidity in the form of pseudoarthrosis, neurological injury, paralysis, and systemic infection. • Spinal SSI may present as superficial wound dehiscence or drainage, deep wound dehiscence, epidural abscess, discitis, and/or osteomyelitis. Risk factors for spinal SSI include medical comorbidities, indication for surgery, and • procedure-specific factors.3 Continued

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SECTION VIII—POSTOPERATIVE COMPLICATIONS

 

•

Medical comorbidities, such as anemia, diabetes mellitus, coronary artery disease, coagulopathy, neoplasm, malnutrition, higher American Society of Anesthesiologist score, smoking, and obesity, are potential risk factors for SSI. Spine-specific SSI risk factors, independent of body mass index, include skin fold thickness and L4 spinous processskin thickness as a measure of the distribution and depth of adipose tissue. • The indication for surgery may also affect the risk for spinal SSI. Trauma cases have a higher risk for infection compared to elective cases. In elective cases, patients undergoing surgery for degenerative disease may have a lower infection rate as compared to deformity. • Complex procedures involving more extensive tissue dissection, increased blood loss, and longer operative time may have a higher risk for spinal SSI. Case order may also matter: one study found that cases performed later in the day led to a higher incidence of SSI compared to those cases that were the first of the day. • Intraoperative measures to minimize spinal SSI have been studied. The only irrigation agent that has been demonstrated in systematic review to reduce the rate of spinal SSI is a diluted solution of povidone-iodine. The application of topical vancomycin powder in the surgical site may have a protective effect on the incidence of SSI without significant side effects.2 • Commonly isolated pathogens in spinal SSI include Staphylococcus aureus, Staphylococcus epidermidis, methicillin-resistant organisms (which are more common in revision surgeries), and gram-negative organisms.1 • In the setting of possible spinal SSI, bloodwork including ESR, CRP, and blood cultures can be obtained in addition to standard labs. MRI with and without contrast can be ordered to evaluate the extent of the infection. • If the patient is hemodynamically stable and without evidence of systemic infection, empiric IV antibiotics with Staphylococcal coverage can be initiated after sending wound cultures. The duration of therapy is variable and will depend on factors including the presence of hardware, the extent of infection, and culture results. • Treatment options for spinal SSI include wound care, primary debridement and closure, a closed vacuum system, and antibiotics. Management of these patients is dependent on the extent of the infection.3 • Patients presenting with superficial wound infections (above the fascia) can trial local wound care and/or a course of oral antibiotics. If there is concern for purulence or dehiscence, superficial wounds may be debrided and allowed to heal by secondary intention with guidance from the plastic surgery team or the wound care team. • Deep infections (which extend below the fascia) may require intraoperative exploration and washout. Larger defects may require assistance from the plastic surgery team for reconstruction and/or use of a muscle flap.

References 1. Abdul-Jabbar A, Berven SH, Hu SS, et al. Surgical site infections in spine surgery: identification of micro- biologic and surgical characteristics in 239 cases. Spine (Phila Pa 1976). 2013;38(22):E1425– E1431. 2. Kang DG, Holekamp TF, Wagner SC, Lehman Jr RA. Intrasite vancomycin powder for the prevention of surgical site infection in spine surgery: a systematic literature review. Spine J. 2015;15(4):762–770. 3. Radcliff KE, Neusner AD, Millhouse PW, et al. What is new in the diagnosis and prevention of spine surgical site infections. Spine J. 2015;15(2):336–347.

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74

Leakage From Spinal Incision With Headaches Kurt Lehner, MD n Daniel Lubelski, MD

Consult Page 55F s/p spine surgery with leakage from wound and headaches

Initial Imaging

Fig. 74.1 Sagittal MRI of the lumbar spine without contrast demonstrates a large postoperative fluid collection consistent with a pseudomeningocele causing moderate thecal sac compression. The collection extends to the skin. Contrasted sequences (not pictured here) did not show any contrast enhancement. There is no bone, disc, or joint abnormality to suggest active infection.

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SECTION VIII—POSTOPERATIVE COMPLICATIONS

Walking Thoughts n n

n n n n n n n n n

What surgery did the patient have? When was the patient’s surgery? Were there any complications? Was there a durotomy or a cerebrospinal fluid (CSF) leak noted during surgery? What was her postoperative course? When and where did she discharge to? What is the patient’s baseline neurological function, and what was her function after surgery? What is her current neurological exam? How much leakage is coming from the wound? What is the color and consistency? Does the patient have risk factors for poor wound healing? Are the patient’s headaches positional or do they have any other characteristics? Does the patient have any signs of infection? Does this patient need to go to the operating room, and if so, when? Is the patient on any anticoagulant or antiplatelet medications?

History of Present Illness A 55 year old female with a recent L3-L4 laminectomy and discectomy, a remote L4-L5 laminectomy and discectomy, obesity, and diabetes presents to the emergency department (ED) with leakage from her surgical incision and positional headaches. The patient initially presented with 6 months of radiating pain down her right leg and was found to have a disc herniation at L3-L4. She underwent an L3-L4 laminectomy and discectomy one week prior to presentation. There were no intraoperative complications noted, and her postoperative recovery was uneventful, with resolution of her right leg pain. She had a surgical drain that was removed on postoperative day 1, and the patient was discharged home on postoperative day 2. Two days prior, she noted the return of her right radicular leg pain as well as new headaches. Her headaches appear to be positional, as they worsen with standing and are relieved when supine. Earlier today, she noted leakage of fluid from the bottom of her surgical incision after straining on the toilet. She denies any photophobia, phonophobia, fevers, chills, weakness, numbness, tingling, or bowel/bladder dysfunction. She does not know how well-controlled her blood sugars have been recently; her latest hemoglobin A1C prior to surgery was 9.7%. She is not on any anticoagulant or antiplatelet medications.

Vital Signs T 37.8°C, HR 90, BP 125/78, SpO2 99% on room air

Pertinent Labs WBC 12.2, ESR 45, CRP 1.4 Blood glucose 356

Physical Exam Lying flat, appears uncomfortable Alert and oriented to person, place, and year Bilateral upper extremities 5/5 strength

RLE LLE

Hip Flexion

Knee Extension

Knee Flexion

Dorsiflexion

Plantar Flexion

Toe Extension

5/5 5/5

5/5 5/5

5/5 5/5

5/5 5/5

5/5 5/5

5/5 5/5

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Sensation intact to light touch throughout There is active, continuous leakage of clear fluid from the inferior portion of her lumbar incision, with a 2 cm area of superficial dehiscence without erythema or purulence. The site is nontender and there is fluctuance to palpation under the wound.

Triage Management This patient has breakdown of her surgical site with leakage of fluid and underlying fluctuance. She is found to have a fluid collection on MRI concerning for a pseudomeningocele with a persistent CSF fistula. Although there was not a CSF leak or durotomy noted during her most recent surgery, her prior history of lumbar surgery does increase the risk of CSF leak. Her uncontrolled diabetes and obesity are also risk factors for poor wound healing. Currently, her WBC, ESR, and CRP are mildly elevated, but this could be due to her recent surgery. Fortunately, she is hemodynamically stable and does not appear to have any signs or symptoms of sepsis or meningitis. However, she is at high risk for further wound dehiscence and meningitis, and her open wound must be addressed expeditiously. She is also symptomatic from the leak, based on her positional headaches and return of her radicular leg pain. Given that her presentation and active drainage are concerning for a high-flow CSF leak, conservative measures (e.g. oversewing of the incision, acetazolamide) are unlikely to be successful to stop the leak or relieve her symptoms. She will be admitted the neurosurgical floor for surgical planning. Should there be any concern for systemic infection or meningitis, a higher level of care may be warranted, with the initiation of antibiotics. The plastic surgery team may be consulted to assist with complex wound closure, and the infectious disease team will manage her antibiotic plan. Endocrinology will also be consulted to help manage her blood sugars. This is a nonemergent but operative consult.

Assessment This is a 55 year old female with a history of prior lumbar surgery, uncontrolled diabetes, and obesity who presents one week after an L3-L4 laminectomy and discectomy with positional headaches and clear drainage from a small dehiscence of her wound. There is active drainage concerning for a CSF fistula. She will require admission for revision and washout of her wound.

Plan n n n

n n n n

n

n

Admit to the neurosurgical floor Bedrest with head of bed flat to minimize active drainage If surgery is not imminent, consider oversewing the incision using sterile technique while planning for surgery Blood cultures Preoperative coagulation bloodwork NPO/IV fluids Consult to the infectious disease team for timing and choice of antibiotics n Hold antibiotics pending results of operative cultures. Aggressive antibiotic treatment should begin with any signs of systemic infection or positive cultures Consider consult to the plastic surgery team to aid in complex closure of her wound given her risk factors of diabetes and history of multiple spinal operations Surgical planning for same-day or next-day wound revision and washout with possible CSF leak repair

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SECTION VIII—POSTOPERATIVE COMPLICATIONS n

Posting highlights: Multiple culture swabs (for each tissue compartment) n Irrigation (with or without antibiotics) n Plan for dural repair with direct suturing, fibrin glue, or fibrin sealant patch n Possible insertion of lumbar drain if the dural defect is not found or not amenable to watertight repair n For anesthesia: once intraoperative cultures are obtained, broad-spectrum IV antibiotics can be administered n

LEARNING POINTS

• Incidental durotomy can occur in as many as 2% of spinal surgeries and can lead to intraoperative and postoperative CSF leak. Rates of CSF leak and wound dehiscence after spine surgery are much higher in cases requiring intradural exploration.4,5 • It is important to distinguish between intraoperative versus postoperative CSF leak, as not all intraoperative leaks will result in persistent postoperative leaks. Meticulous, watertight dural and wound closure of an intraoperative CSF leak is key for the prevention of postoperative CSF leak. • Postoperative CSF leaks can lead to positional headaches, pseudomeningoceles, CSF fistulas, and/or wound dehiscence or breakdown. A pseudomeningocele is an extradural collection of CSF resulting from a dural breach that extends into the soft tissue without going through the skin. If the extradural fluid communicates with the external environment or with another body cavity, this results in a CSF fistula. • Patients with pseudomeningoceles are often asymptomatic but can have symptoms that worsen with Valsalva maneuvers (e.g. sneezing, coughing). If the pseudomeningocele is compressive, patients can present with signs and symptoms of radiculopathy or myelopathy depending on the affected spinal level. • Patients with active drainage are at risk for meningitis and require admission. The use of prophylactic antibiotics with CSF leaks is of unclear benefit and may serve to increase the incidence of drug-resistant organisms.2 With any signs of systemic infection, however, aggressive antibiotic therapy should be initiated. • MRI is the imaging modality of choice to investigate a fluid collection or pseudomeningocele. Contrast can be used to assess for infection. • The management of postoperative CSF leak varies based on the severity of the leak and the patient’s symptoms. • In a patient without active drainage or open wound, antibiotics are not necessary and bed rest with an abdominal binder may be sufficient to stop the leak and help reduce the pseudomeningocele. • In some cases with only symptomatic headaches and no active drainage, a blood patch may be used.1 • A small defect in the wound can be oversewn at the bedside with sterile technique. • The use of medications which decrease CSF production, such as acetazolamide, may also be helpful. • Caffeine and hydration may provide symptomatic relief of positional headaches. • Direct exploration of the wound and dura with primary repair is necessary in cases where patients are symptomatic with evidence of nerve or spinal cord compression, or with active leakage and large wound defects that cannot be stopped with simple oversewing of the wound. • Patients with large or multiple CSF leaks which are not amenable to direct repair may require CSF diversion with a lumbar drain or even an external ventricular drain. In severe cases, permanent CSF shunting may be required to facilitate wound healing, but this is rare.1,3

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References 1. Couture D, Branch Jr CL. Spinal pseudomeningoceles and cerebrospinal fluid fistulas. Neurosurg Focus. 2003;15(6):E6. 2. Eljamel MS. Antibiotic prophylaxis in unrepaired CSF fistulae. Br J Neurosurg. 1993;7(5):501–505. 3. Kitchel SH, Eismont FJ, Green BA. Closed subarachnoid drainage for management of cerebrospinal fluid leakage after an operation on the spine. J Bone Joint Surg Am. 1989;71(7):984–987. 4. Mehta AI, Adogwa O, Karikari IO, et al. Anatomical location dictating major surgical complications for intradural extramedullary spinal tumors: a 10-year single-institutional experience. J Neurosurg Spine. 2013;19(6):701–707. 5. Williams BJ, Sansur CA, Smith JS, et al. Incidence of unintended durotomy in spine surgery based on 108,478 cases. Neurosurgery. 2011;68(1):117–124.

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75

Hemiparesis With A Previously Treated Arteriovenous Malformation Risheng Xu, MD/PhD n

Daniel Lubelski, MD

Consult Page Pt w known AVM, here with weakness.

Initial Imaging

Fig. 75.1 Axial head CT without contrast demonstrates a round hypodensity of the right thalamus with surrounding edema but without intracranial hemorrhage.

358

75—HeMiPAresis WiTH A Previously TreATed ArTeriovenous MAlforMATion

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Walking Thoughts n

n n

n

How weak is the patient? Where is the patient’s weakness? How long has he been symptomatic? Does the patient have any other deficits on examination? What is the patient’s arteriovenous malformation (AVM) history? Has he had any prior hemorrhages or treatments? Did the AVM hemorrhage?

History of Present Illness An 18 year old male with a history of a right thalamic Spetzler-Martin grade 3 AVM presents to the emergency department (ED) with one week of right frontal headache, nausea, vomiting, and worsening left-sided weakness. His AVM was initially discovered one year prior after he presented with headache and nausea, and he was found to have an intracerebral hemorrhage. Brain MRI and cerebral angiography performed at that time demonstrated a 3 × 2 cm right thalamic AVM with deep venous drainage (Figure 75.2). He was stabilized, his hemorrhage was monitored, and he was discharged home with no neurological deficits. The patient was then seen as an outpatient and scheduled for stereotactic radiosurgery (SRS), which he completed 11 months prior. The procedure was uneventful. He presents today with one week of progressive left arm and leg weakness and one day of headache, nausea, and vomiting. He denies any seizure-like activity. He denies numbness or tingling bilaterally and is full strength on his right side.

A

B

Fig. 75.2 Axial T2-weighted brain MRI without contrast (A) and CT angiogram (B) demonstrate the original Spetzler-Martin grade 3 AVM with a large, deep draining vein.

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SECTION VIII—POSTOPERATIVE COMPLICATIONS

Vital Signs T 37°C, HR 90, BP 136/62, SpO2 99% on room air

Pertinent Labs Na 139, Glu 95

Physical Exam Alert and oriented to self, place, and year Pupils equal, round, and reactive to light Extraocular movements intact, tongue midline, face symmetric Right upper and lower extremity 5/5 strength Left upper and lower extremity 4-/5 strength Sensation intact to light touch throughout

Triage Management The patient has a Spetzler-Martin grade 3 AVM that was treated with radiosurgery 11 months prior. Initial head CT demonstrates no intracranial hemorrhage. A brain MRI with and without contrast (Figure 75.3) and MRA is completed in the ED, demonstrating significant edema with midline shift. His progressive weakness is likely secondary to edema from partial thrombosis of the

Fig. 75.3 Axial T2-weighted brain MRI without contrast demonstrates the right thalamic AVM with significant surrounding edema and midline shift.

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AVM as a result of radiation treatment. He is alert and awake and will be admitted to the neurosurgical floor for close monitoring. His current sodium level is normal; although he does not require hypertonic saline, his sodium will be trended to ensure normonatremia. He will be treated with steroids, given the edema on MRI. If he becomes weaker, altered, or somnolent on examination, he may need a higher level of care for closer monitoring. This is an urgent but nonoperative consult.

Assessment This is an 18 year old male with a right thalamic Spetzler-Martin grade 3 AVM, now 11 months status post SRS, who presents with left hemiparesis, partial thrombosis of the AVM, significant edema, and midline shift without intracranial hemorrhage on MRI. He will be admitted for observation and monitoring with corticosteroid treatment.

Plan Admit to the neurosurgical floor Avoid hyponatremia n Corticosteroids (dexamethasone IV 10 mg once, 4 mg every 6 hours thereafter) n Proton pump inhibitor n Physical therapy and occupational therapy n No anti-seizure medication n n

LEARNING POINTS

• SRS is usually recommended for the management of small to moderate-sized AVMs that are deep or close to highly eloquent cortex.

• Radiation induces narrowing and obliteration of the AVM vessels. Seventy-five to eighty-six percent of AVMs treated with SRS are obliterated by 2–3 years post-radiation.1,2 SRS has an overall obliteration rate of 70%–80% of all treated AVMs.3 • Patients may develop radiosurgery-related thrombosis or flow changes to the AVM around 8–12 months after treatment. • Adverse radiation effects include hemiparesis, headache, seizure, sensory dysfunction, ataxia, short-term memory loss, and vision changes.1 • In a large series of 755 patients with AVM who underwent a single SRS procedure with at least two years of follow-up, cumulative rates of symptomatic adverse radiation effects were 3.2%, 5.8%, 6.7%, and 7.5% at 1, 2, 3, and 5 years, respectively.1 Factors associated with a higher rate of developing symptomatic adverse radiation effects • include larger AVM volume, higher margin dose, and higher Spetzler–Martin grade. The rates of developing symptomatic adverse radiation effects were higher in the brainstem (22%) or thalamus (16%), compared with AVMs located in other brain locations (4%–8%). The 5-year cumulative rates of irreversible symptomatic adverse radiation effects were 9.1% in the thalamus, 12.1% in the brainstem, and 1.4% in other locations.1

References 1. Kano H, et al. Estimating the risks of adverse radiation effects after gamma knife radiosurgery for arteriovenous malformations. Stroke. 2017;48:84–90. 2. Schneider BF, Eberhard DA, Steiner LE. Histopathology of arteriovenous malformations after gamma knife surgery. J Neurosurg. 1997;87(3):325–327. 3. Starke RM, Yen CP, Ding D, Sheehan JP. A practical grading scale for predicting outcome after radiosurgery for arteriovenous malformations: analysis of 1012 treated patients. J Neurosurg. 2013;119(4):981–987.

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76

Change In Exam After Brain Tumor Biopsy Andrew Luksik, MD n

Daniel Lubelski, MD

Consult Page Hi there, for room 21, patient seems sleepier, please come evaluate him.

Initial Imaging None

Walking Thoughts π

π π

π

π

π π π π

π

π π

Why is the patient admitted? Did the patient have surgery and if so, what type of surgery and when did it occur? Where is the patient currently located (e.g. floor vs. neurocritical care unit)? If applicable, what was the patient’s immediate postoperative exam? Are these symptoms new or expected? What is the patient’s current neurological exam? What and when was the patient’s last exam? How sleepy is he? What is the timing of the patient’s symptoms? Was this a sudden change or progressive? What are his ABCs? Is he protecting his airway? What are his vital signs? What imaging does the patient need? Has the patient received any recent medications that could confound the examination (e.g. pain medication)? If applicable, is the patient at risk for any postsurgical complications (e.g. seizures or hemorrhage)? Does he need to be transferred to a monitored bed? Is he on any anticoagulant or antiplatelet medication?

History of Present Illness A healthy 63 year old male is currently admitted to the neurosurgical floor after recently undergoing a right stereotactic biopsy of a brain lesion. He initially presented as a transfer from an outside hospital with several months of progressive left-sided weakness. About 5 months ago, he woke up with left arm numbness and tingling. Since then, he developed worsening left arm and leg weakness. In the week prior to presentation, he could no longer move his left arm and leg and presented to an outside hospital. MRI there demonstrated a 4 cm mass of the right thalamus, basal ganglia, centrum semiovale, and midbrain with mild hydrocephalus. He was started on dexamethasone and levetiracetam and transferred to our institution. 362

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Two days prior, he underwent a right stereotactic biopsy of the lesion with preliminary pathology consistent with a high grade glioma. The procedure was uncomplicated, and the patient awoke from anesthesia at his neurological baseline with stable hemiplegia. An immediate postoperative head CT showed postsurgical changes without new hemorrhage or abnormality. He recovered well in the neurocritical care unit for one day and was then transferred to the floor. On postoperative day 2, the nurse noted that he was sleepier on routine examination and urgently paged the on-call neurosurgery pager.

Vital Signs T 36.5°C, BP 205/92, HR 122, RR 24, SpO2 97% on room air

Pertinent Labs Na 139, Glu 236, Hgb 15, WBC 12, Plt 194, INR 1.2, aPTT 23.2 (morning values)

Glasgow Coma Scale Motor: 6 Verbal: 3 Eye opening: 1 GCS Total: 10

Physical Exam Lethargic, eyes closed Oriented to name with choices Pupils equal and reactive to light Right upper and lower extremity following commands Left upper and lower extremity minimal movement (baseline) Surgical site clean, dry, and intact

Triage Management This is a patient who underwent a stereotactic brain biopsy of a large, deep right basal ganglia mass two days prior. Although he initially did well postoperatively and transferred to the neurosurgical floor, he was noted today by his nurse to be sleepier. Although there could be multiple causes for the change in exam (e.g. seizure, medications, hemorrhage, hydrocephalus), he needs to be seen quickly for evaluation. With a change in mental status in a post-brain biopsy patient, an emergent head CT is warranted to rule out acute pathology such as hemorrhage. The nurse will obtain an updated set of vitals and begin preparing to take the patient to imaging. Upon arrival to the patient’s room, ABCs are the first step. The patient is still following commands and answering orientation questions; he appears to be protecting his airway. His systolic blood pressure is noted to be in the 200s and HR in the 120s: this needs to be treated urgently with IV antihypertensive medications. Once he is hemodynamically stable for imaging, rapid transport to the scanner is needed. Of note, the nurse reports that he has only been receiving acetaminophen and denies administering any sedating pain medications. Postoperatively, he has been taking dexamethasone and levetiracetam; his last dose was 2 hours prior. He received one dose of subcutaneous heparin this morning. The patient does not have a history of hypertension and is not on any other anticoagulant or antiplatelet medications.

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SECTION VIII—POSTOPERATIVE COMPLICATIONS

Follow-Up The patient undergoes an emergent head CT without contrast (Fig. 76.1) demonstrating biopsy site hemorrhage with increased ventricular size compared to his immediate postoperative scan. On return from head CT, the patient is noted to be unresponsive with fixed and dilated pupils. With this exam change, he will need emergent intubation for airway protection, and a rapid response team is called. On ABC reassessment, he has continued elevated blood pressure despite receiving IV antihypertensives, and additional medication is administered. Given the large brain bleed and concern for brain herniation, medical measures of addressing acute intracranial pressure (ICP) elevation are initiated. The head of the bed is raised, and the patient receives a dose of 23% hypertonic saline and 1 g/kg of mannitol. He is also given an extra dose of dexamethasone and antiseizure medication. An updated set of labs are sent. Once intubated, hyperventilation may be considered. Urgent transfer to the neurocritical care unit is imperative. Because of his worsening hydrocephalus on imaging, an external ventricular drain (EVD) may be placed emergently for ICP monitoring and CSF drainage. An attempt to reach family should be made to discuss the patient’s unexpected decline and obtain procedural consent. An EVD is planned unless there is a clear advanced directive against further measures (e.g. do not resuscitate orders) or the family declines. The patient will be transferred to the neurocritical care unit for placement and management of his hemorrhage, EVD, and hypertension. Of note, if the head CT did not show acute findings, further investigation into the cause of his exam change would have been pursued, including evaluation for seizure or stroke. This is an emergent, operative consult.

A

B

Fig. 76.1 Axial head CT without contrast. A. Immediate postoperative scan with postoperative changes and no acute hemorrhage. The mild hydrocephalus and midline shift is similar to preoperatively. B. Scan after his acute change in mental status shows an acute hemorrhage within the biopsy site with worsening hydrocephalus.

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Assessment This is a 63 year old male with a recent stereotactic biopsy of a large right basal ganglia lesion who had an acute change in exam concerning for brain herniation. His head CT shows a new hemorrhage at the biopsy site causing worsening obstructive hydrocephalus.

Plan n n n

n n n n n n n n

n

Emergent intubation Transfer to the neurocritical care unit with hourly neurological exams Medical management of acute intracranial hypertension: hyperventilation, head of bed elevation, hyperosmolar therapy, hypertonic saline Emergent placement of EVD after preprocedural antibiotics Repeat head CT to confirm EVD placement Arterial line and foley catheter placement Maintain systolic blood pressure below 160 mmHg, nicardipine infusion as needed Maintain elevated sodium goal (e.g. >145) Maintain NPO with maintenance (isotonic or hypertonic) IV fluids Continue dexamethasone and levetiracetam Hold subcutaneous heparin; sequential compression devices for deep venous thrombosis prophylaxis Additional aggressive measures, such as hemicraniectomy, must involve a frank discussion of the risks and benefits with family, particularly given that the patient’s pathology is likely a high grade glioma

LEARNING POINTS

• Stereotactic brain biopsy is generally safe, with a relatively low risk of complications. One recent systematic review found that overall morbidity ranges from 3% to 13%, and mortality varies from 0.7% to 4%. Morbidity and mortality are both usually related to a hemorrhagic complication but can also result from acute brain edema.4 • Most complications manifest with symptoms of neurological impairment (either transient or permanent), seizure, and/or unconsciousness. • Risk factors for morbidity after a stereotactic brain biopsy include diabetes, thalamic and basal ganglia lesions, and deep-seated lesions. Mortality is associated with a lesion located in the basal ganglia or frontotemporal lobes as well as with a histology of lymphoma.4 • Hemorrhage is one of the most common complications after stereotactic brain biopsy. The incidence of symptomatic intracerebral hemorrhage (ICH) after stereotactic brain biopsy has been reported to be less than 10%.2 Rates of asymptomatic bleeds are varied but may be even higher.4 • Described risk factors include deep-seated locations and lymphoma. • Post-biopsy hemorrhage is associated with older age, preoperative hydrocephalus, preoperative cerebral edema, platelet count