Pediatric Neurosurgery - In Multiple-Choice Questions (Mar 25, 2024)_(3031495721)_(Springer).pdf 9783031495724, 9783031495731

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Samer S. Hoz · Abdullah H. Al Ramadan · Ian Pople · Nada Mohammed · Waeel O. Hamouda · Ahmed El Damaty · Mustafa Ismail   Editors

Pediatric Neurosurgery In Multiple-Choice Questions

Pediatric Neurosurgery

Samer S. Hoz  •  Abdullah H. Al Ramadan Ian Pople  •  Nada Mohammed Waeel O. Hamouda  •  Ahmed El Damaty  •  Mustafa Ismail Editors

Pediatric Neurosurgery In Multiple-Choice Questions

Editors

Samer S. Hoz Department of Neurosurgery University of Pittsburgh Pittsburgh, PA, USA Ian Pople Neurosurgery Department Sidra Medicine Doha, Qatar Waeel O. Hamouda Faculty of Medicine, Teaching, & Research Hospitals Cairo University Cairo, Egypt Mustafa Ismail Department of Neurosurgery Neurosurgery Teaching Hospital Al-Risafa, Baghdad, Iraq

Abdullah H. Al Ramadan Dept of Neurosurgery and Spine Surgery Qatif Central Hospital Al Qatif, Saudi Arabia Nada Mohammed Pediatric Neurosurgery Department Bristol Royal Hospital for Children Bristol, UK Ahmed El Damaty Department of Neurosurgery, Faculty of Medicine Heidelberg University Heidelberg, Baden-Württemberg, Germany

ISBN 978-3-031-49572-4    ISBN 978-3-031-49573-1 (eBook) https://doi.org/10.1007/978-3-031-49573-1 © The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 This work is subject to copyright. All rights are solely and exclusively licensed by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors, and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland Paper in this product is recyclable.

V

Samer S. Hoz To my lovely family: Sawsan, Saad, Arwa, Farah, Ward, Khawla, Samhar, Sanaa, Faris, and Luay. To the GYL team, Norberto, Kathi, and Paolo. Abdullah H. Al Ramadan To my Mom Layla (May Allah bless her soul) and my Dad Husain, To My love Rahmah, my daughters Sara and Sama and my supportive family: Ali, Mohammed, Ahmed, Zainab, Sara and Jawad. Ian Pople To my wonderful neurosurgical team and supporting staff at Sidra Medicine, Doha. Nada Mohammed To my parents, brothers and sister. To my friends Osilat, Ghada and Kholood for their unstoppable support. Waeel O. Hamouda To my patients who courageously endure the dismay and hardship of their diseases while trusting me with their lives and dreams. You are all my heroes. Ahmed El Damaty To my family and patients, whom their trust endeavored me on that long tedious way. Mustafa Ismail To my mom Khafia, to my dad Ismail (May Allah bless his soul), to my sister Noor, to brothers Osama and Qutaiba, my family.

Foreword 1 As someone whose postgraduate education started half a century ago and continues to this day, I am envious of the wide range of imaginative educational materials available to the present generation of trainees and consultants. I was fortunate in having some outstanding teachers who illuminated rote learning and dogma with scepticism, and who expected us to start to develop the ability to seek out and marshall evidence, recognise uncertainty and come up with workable solutions to problems for the benefit of our future patients. Dr Hoz and his team are to be applauded for their use of the MCQ format to stimulate understanding of Paediatric Neurosurgery through a step-by-­ step concise overview of each disease from definition, associated anatomy, pathology, clinical features and radiology through to surgical decision-­ making and surgical technique. MCQs have a chequered reputation but have been widely adopted for examinations for their objectivity, ease of grading and validation, and the ability to cover as much material as possible. Much effort is required to construct sound MCQs. Trick questions are to be abhorred. Sadly, MCQs may have a short shelf life when the unscrupulous copy them in order to game the examination and certification process. This book uses MCQs constructively as a way of encouraging the student at all ages to retrieve information from memory (if it ever existed!) and use it to answer clinically relevant questions. Potentially, this process should strengthen understanding and memory. The provision of key references for further reading is very welcome and should stimulate Departmental Chairpersons to ensure that their trainees have access to the necessary electronic library facilities. This book is complimentary to existing international texts such as Youmans and Winn’s Neurological Surgery (4th edition 2016) and the Oxford Textbook of Neurological Surgery (Kirollos, Helmy, Thomson & Hutchinson, 2019). It will certainly fulfil its authors’ aim of helping readers from all countries, from trainee to established consultant and examiner, to identify the strengths and weaknesses in their knowledge of Paediatric Neurosurgery. It deserves a long life with further Editions as this fascinating field advances. John D. Pickard

University of Cambridge,

Cambridge, UK

VII

Foreword 2 Surgery, the most noble art, has been practised for millennia for the improvement of human suffering. Paediatric neurosurgery has the added dimension of treating the child, not just in the present but also the adult that this child may be destined to become. So it becomes paramount that the surgeon, deals with the pathology in a manner that keeps the morbidity of the treatment to an absolute minimum. Science gives us clues as to how we may achieve this, and it remains up to the surgeon to combine this science with the art to do our best for future generations. Learning, seemingly ‘abstract’ facts, is rarely fun especially for the active surgical spirit yet often these ‘abstract’ facts give us vital clues into the pathology and its treatment. Prof Samer Hoz and his team of editors have done the most commendable job of picking out the most relevant facts and presenting them in a fashion that makes the tedious task of learning them more exciting and indeed more productive. The objective is to broaden the readers understanding of paediatric neurosurgery and to stimulate interest and further self-directed research and learning. The format ensures the facts become easily accessible which should serve to help with recall and board assessments. In this manner, entire topics are made more lucid and facts more relevant to the practice of surgery. To the readers, I have little doubt you will find this book useful, I did. Know your facts, learn to use them in an unbiased fashion and answers hitherto unknown will become clear to you. Noor ul Owase Jeelani

Great Ormond Street Hospital for Children,

London, UK

Foreword 3 Basically, one of the main drawbacks a multi-authored book may have is the sense of unevenness in format and approach among the various chapters, that is why the editors of this book propend to offer a rigid scheme for their contributors to adhere to in order to make their book as uniform as possible. The readability of this book is further enhanced by the authors’ and editors’ shared understanding that an attitude of assured non-confusing knowledge must be prevailing. The editors have chosen 20 subjects (chapters) that cover the large spectrum of pediatric neurosurgery, including those dealing with the commonly encountered disorders of cerebrospinal fluid dynamics and congenital malformations, while nevertheless dedicating other chapters to the less famous cases of spinal trauma and peripheral nerves disorders. The questions and answers in each chapter cover almost all the topics attaining to a specific disease; those might include mechanisms of disease, histopathology, medical management, diagnostic techniques, operative indications and contraindications, special points in the anesthetic technique, detailed description of related operations, and postoperative complications. Over the years, controversy has arisen over the advantages and disadvantages of pursuing the specialty of pediatric neurosurgery in comparison with that of general neurosurgery, we come more and more to know that neurosurgical conditions within this vulnerable age group possess their specific characteristics and challenges. As a pediatric neurosurgeon with more than 34 years of experience who has built his carrier in a developing country, I hope that my colleague neurosurgeons would find this reference of value in their pursue of knowledge related to and in their practice of pediatric neurosurgery as I have found it.

Julio S. Brossard Alejo Department of Neurosurgery Dr. Juan Bruno Zayas Alfonso General Hospital Santiago de Cuba, Cuba Southern Children's Hospital Santiago de Cuba, Cuba Hospital Infantil Sur Santiago de Cuba, Cuba

IX

Key Features 55 Pediatric Neurosurgery in Multiple Choice Questions, the first review book to use the multiple-choice question format, is dedicated to neurosurgical pediatric neurosurgery. 55 The mission of the book is to help readers understand the content and maintain the knowledge, rather than merely finding answers for tricky questions. 55 The chapters of this book provide comprehensive coverage of the core concepts in pediatric neurosurgery. 55 This essential review mirrors the multiple-choice format adopted by the majority of shelf and board examinations. 55 This study companion provides more than 500 MCQs in a convenient format that is suitable for self-study. 55 The strategy and the format of the questions provide a step-by-step, thorough explanation of each disease from the definition, associated anatomy, pathology, clinical features, radiology to surgical decision-making, and surgical tricks, providing a comprehensive and concise overview. 55 Answers and definitions appear immediately below the questions to facilitate information retention. 55 This book is an adjunct to the existing texts and does not intend to be the primary source of information; it rather aims to help readers identify their relevant strengths and weaknesses in the area. 55 These questions are structured as a refresher course for both long and short study sessions. 55 Pediatric Neurosurgery in Multiple Choice Questions is an important asset for residents across neurosurgical disciplines as it includes much of the pediatric neurosurgery knowledge that neurosurgical residents need to prepare for their certification tests. It is also useful for those seeking ways to solidify their knowledge or maintain their current certification.

Preface We are delighted to introduce pediatric neurosurgery in Multiple Choice Questions as your study companion. Navigating the complexities of pediatric neurosurgery demands precision, depth of knowledge, and a keen understanding of the delicate nuances that distinguish pediatric cases from adults. The book Pediatric Neurosurgery in Multiple Choice Questions encapsulates the breadth of pediatric neurosurgery. The choice to present in an MCQ format promotes active engagement, facilitating both learning and self-­ assessment. We put this humble book as a guide to the mysterious vascular aspect of pediatric neurosurgery, trying to concentrate on the core, intended to help the reader via a systematic approach to diagnose and manage the common pediatric neurosurgical problems by applying scientific knowledge to practice. It is the first review book to use the multiple-choice question format in pediatric neurosurgery, and it contains over 500 questions, and all are genuine (new). Each MCQ is designed to provide the reader with four correct facts and one false statement, as well as brief explanations to aid in consolidation. These questions are designed to provide a refresher course either in long or briefer study sessions as time permits during a busy day in the neurosurgical practice. As we journey together through these pages, they may not only inform but also inspire excellence in patient care. We sincerely hope you enjoy and benefit from the book. Pittsburgh, PA, USA Al Qatif, Saudi Arabia Doha, Qatar Bristol, UK Cairo, Egypt Heidelberg, Baden-Württemberg, Germany Baghdad, Iraq

Samer S. Hoz Abdullah H. Al Ramadan Ian Pople Nada Mohammed Waeel O. Hamouda Ahmed El Damaty Mustafa Ismail

XI

Contents 1

Neuroanatomy...............................................................................................................   1 Muffaq K. Lashhab, Oday Atallah, Ahmed Muthana, Mohammedbaqer A. Al-Ghuraibawi, Naba G. Husseini, and Samer S. Hoz

2

Development of Central Nervous System....................................................  17 Aras F. Albarazanchi, Oday Atallah, Ahmed Muthana, Tabarek F. Mohammed, Sara A. Mohammad, and Samer S. Hoz

3

Neurological Examination.....................................................................................  27 Nada Mohammed, Leen R. Azzam, and Ian Pople

4

Hydrocephalus...............................................................................................................  39 Eleni Tsianaka, Ahmed Muthana, Fatimah O. Ahmed, and Samer S. Hoz

5

CSF Diversion..................................................................................................................  49 Oday Atallah, Laith T. Al-Ameri, Zinah A. Al-Araji, Zainab A. Alaraji, Huda Abdulrazaq, and Samer S. Hoz

6

Craniosynostosis..........................................................................................................  59 Fatima A. Fakhroo, Mariam H. Allehaibi, Fatimah O. Ahmed, and Abdullah H. Al Ramadan

7

Chiari Malformations.................................................................................................  75 Ahmed Adel Farag, Ahmed Abdelrahman Abdullah, Ali A. Dolachee, and Waeel O. Hamouda

8

Congenital Arachnoid Cysts and Dandy Walker Malformation................................................................................................. 115 Fehid Habalrih, Mashael Almarwani, Mustafa Ismail, and Abdullah H. Al Ramadan

9

Encephalocele................................................................................................................ 129 Amna S. Hussein, Mohamed F. Alsawy, Mustafa Ismail, and Waeel O. Hamouda

XII

10

Contents

Spina Bifida...................................................................................................................... 141 Nada Mohammed, Raghad O. Aljohani, and Ian Pople

11

Pediatric Brain Tumors............................................................................................. 163 Oday Atallah, Abdullah K. Al-Qaraghuli, Noor M. Shaker, Noor M. Akar, Alkawthar M. Abdulsada, and Samer S. Hoz

12

Spinal Tumors................................................................................................................. 177 Honida A. Ibrahim, Nada Mohammed, Maliya Delawan, and Ian Pople

13

Neurocutaneous Syndromes................................................................................ 197 Ahmed M. ElGhamry, Mostafa H. Algabri, Ahmed K. Al-Kishawi, Mustafa Ismail, and Ahmed El Damaty

14

Cranial Trauma............................................................................................................... 213 Ali Eltaj Osman, Nada Mohammed, Sadeem A. Albulaihed, and Ian Pople

15

Spinal Trauma................................................................................................................. 235 Muhammad F. Khan, Abdulrahman R. Nazer, Ameer M. Aynona, and Waeel O. Hamouda

16

Neurovascular Diseases........................................................................................... 251 Osman Elamin, Ahmed Muthana, Rokaya H. Abdalridha, Jaafer AbdulWahid, Sajjad G. Al-Badri, and Samer S. Hoz

17

Brain and Spinal Infections................................................................................... 273 Ruqaya A. Kassim, Muthanna N. Abdulqader, Alkawthar M. Abdulsada, Zahraa A. Alsubaihawi, Abrar A. Khoailed, Mustafa Ismail, and Samer S. Hoz

18

Functional and Epilepsy Surgery...................................................................... 293 Sarah Basindwah, Abdulrahman R. Nazer, Ali A. Basalamah, Fatimh A. Alsaffar, Mahmood F. Alzaidy, and Abdullah H. Al Ramadan

XIII Contents

19

Peripheral Nerves........................................................................................................ 311 Oday Atallah, Sarah F. Hassan, Mahmood F. Alzaidy, Ghazwan Hazem, Osamah M. Al-Shaikhli, Younus M. Al-Khazaali, and Samer S. Hoz

20

Pediatric Neuropathology..................................................................................... 321 Sadeq Wasil Al-Dandan, Mustafa Ismail, and Abdullah H. Al Ramadan

Contributors Rokaya H. Abdalridha  Babylon University, College of Medicine, Babylon, Iraq Ahmed Abdelrahman Abdullah  Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt Muthanna N. Abdulqader  Department of Neurosurgery, Neurosurgery Teaching Hospital, Baghdad, Iraq Huda Abdulrazaq  College of Medicine, Alnahrain University, Baghdad, Iraq Alkawthar M. Abdulsada  Azerbaijan Medical University, Baku, Azerbaijan Jaafer AbdulWahid  University of Al-Nahrain, College of Medicine, Baghdad, Iraq Fatimah O. Ahmed  University of Mustansiriyah, College of Medicine, Baghdad, Iraq Noor M. Akar  College of Medicine, Al-Nahrain University, Baghdad, Iraq Laith T. Al-Ameri  Al-Kindy College of Medicine—University of Baghdad, Baghdad, Iraq Zainab A. Alaraji  University of Al-Nahrain, College of Medicine, Baghdad, Iraq Zinah A. Al-Araji  College of Medicine, Alnahrain University, Baghdad, Iraq Sajjad G. Al-Badri  Babylon University, College of Medicine, Iraq University of Baghdad, College of Medicine, Baghdad, Iraq Aras F. Albarazanchi  Diana Princess of Wales Hospital, Grimsby, UK Sadeem A. Albulaihed  Department of Neurosurgery, Alfaisal University, College of Medicine, Riyadh, Saudi Arabia Sadeq Wasil Al-Dandan  King Fahad Hospital Hofuf, Al-Ahsa, Saudi Arabia

XV Contributors

Mostafa H. Algabri  University of Baghdad, College of Medicine, Baghdad, Iraq Mohammedbaqer A. Al-Ghuraibawi  University of Warith Al-Anbiyaa, College of Medicine, Karbala, Iraq Raghad O. Aljohani  Al-Rayyan Medical Colleges, Madinah, Saudi Arabia Younus  M.  Al-Khazaali  College of Medicine, Al-Nahrain University, Baghdad, Iraq Ahmed K. Al-Kishawi  Al-Mustansiriyah University, College of Medicine, Baghdad, Iraq Mariam H. Allehaibi  Maternity and Children’s Hospital, Makkah, Saudi Arabia Mashael Almarwani  Prince Sultan Military Medical City, Riyadh, Saudi Arabia Abdullah K. Al-Qaraghuli  MedStar Health Research Institute, MedStar Washington Hospital Center, Washington, DC, USA Abdullah H. Al Ramadan  Department of Neurosurgery and Spine Surgery, Qatif Central Hospital, Eastern Health Cluster, Qatif, Saudi Arabia Fatimh A. Alsaffar  Dammam Medical Complex, Dammam, Saudi Arabia Mohamed F. Alsawy  Faculty of Medicine, Cairo University, Cairo, Egypt Osamah M. Al-Shaikhli  Imam Ali General Hospital, Baghdad, Iraq Zahraa  A.  Alsubaihawi  University of Baghdad, College of Medicine, Baghdad, Iraq Mahmood F. Alzaidy  University of Baghdad, College of Medicine, Baghdad, Iraq Oday Atallah  Department of Neurosurgery, Hannover Medical School, Hannover, Germany Ameer M. Aynona  University of Babylon, College of Medicine, Babylon, Iraq Leen R. Azzam  Royal College of Surgeons in Ireland-Bahrain, School of Medicine, Adliya, Kingdom of Bahrain

XVI

Contributors

Ali A. Basalamah  King Saud University Medical City, Riyadh, Saudi Arabia Sarah Basindwah  King Saud University Medical City, Riyadh, Saudi Arabia Maliya  Delawan  Gulf Medical University, College of Medicine, Ajman, United Arab Emirates Ali A. Dolachee  Department of Surgery, Al-Kindy College of Medicine, University of Baghdad, Baghdad, Iraq Osman Elamin  Department of Neurosurgery, Jordan Hospital and Medical Center, Amman, Jordan Ahmed  El Damaty  Pediatric Neurosurgery Division, Heidelberg University, Heidelberg, Germany Ahmed M. ElGhamry  Department of Neurosurgery, St George’s University Hospital, London, UK Fatima  A.  Fakhroo  Bahrain Defense Force Hospital—Royal Medical Services, Riffa, Kingdom of Bahrain Ahmed Adel Farag  King Abdullah Medical City Makkah, Makkah, Saudi Arabia Fehid Habalrih  Prince Sultan Military Medical City, Riyadh, Saudi Arabia Waeel O. Hamouda  Neurological & Spinal Surgery Department, Cairo University Faculty of Medicine, Teaching & Research Hospitals, Cairo, Egypt Sarah F. Hassan  University of Baghdad, College of Medicine, Baghdad, Iraq Ghazwan Hazem  Al-Wasitey Teaching Hospital, Baghdad, Iraq Samer S. Hoz  Department of Neurosurgery, University of Pittsburgh Medical Center (UPMC), Pittsburgh, PA, USA Amna S. Hussein  Banner University Medical Center, University of Arizona, Phoenix, AZ, USA Naba G. Husseini  College of Medicine, University of Mosul, Mosul, Iraq

XVII Contributors

Honida  A.  Ibrahim  Department of Neurosurgery, Ribat University Hospital, Khartoum, Sudan Mustafa  Ismail  Department of Neurosurgery, Neurosurgery Teaching Hospital, Baghdad, Iraq Ruqaya A. Kassim  University of Baghdad, College of Medicine, Baghdad, Iraq Muhammad F. Khan  Security Forces Hospital—Dammam, Dammam, Saudi Arabia Abrar A. Khoailed  University of Baghdad, College of Medicine, Baghdad, Iraq Muffaq  K.  Lashhab  Ali Omar Askar Neurosurgery Hospital, Tripoli University, Tripoli, Libya Sara A. Mohammad  University of Baghdad, College of Medicine, Baghdad, Iraq Nada  Mohammed  Senior Clinical Fellow, Pediatric Neurosurgey, Bristol Royal Hospital for Children, Bristol, UK Tabarek  F.  Mohammed  University of Baghdad, College of Medicine, Baghdad, Iraq Ahmed Muthana  University of Baghdad, College of Medicine, Baghdad, Iraq Abdulrahman R. Nazer  Prince Sultan Military Medical City, Riyadh, Saudi Arabia Ali Eltaj Osman  Armed Forces Hospital, Wadi Aldawasir, Saudi Arabia Military School Dist., Wadi Aldawasir, KSA Ian Pople  Division Chief of Neurosurgery, Department of Surgery, Sidra Medical & Research Center, Doha, Qatar Noor M. Shaker  University of AL-Ameed, College of Medicine, Karbala, Iraq Eleni  Tsianaka  Neurosurgery Department, Kuwait Hospital, Sabah Al Salem, Kuwait

Abbreviations ACA

Anterior cerebral arteries

AChE Acetylcholinesterase ADC

Apparent diffusion coefficient AFP Alpha-fetoprotein AKA Also known as AVM Arteriovenous malformations BBB Blood–brain barrier bSSFP MRI Balanced steady-state free precession MRI CBC Complete blood count cGy Centigray CN Cranial nerve CNS Central nervous system CRP C-Reactive protein CRPS Complex regional pain syndrome CSF Cerebrospinal fluid CT Computed tomography DSA Digital subtraction angiography DWI Diffusion weighted imaging EEG Electroencephalogram ESR Erythrocyte sedimentation rate ETV Endoscopic third ventriculostomy FGFR Fibroblast growth factor receptor FLAIR Fluid-attenuated inversion recovery GCS Glasgow Coma Scale GMFCS Gross motor function classification system GRE Gradient echo HGB Hemangioblastoma HHT Hereditary hemorrhagic telangiectasia ICH Intracerebral hemorrhage ICP Intracranial pressure

XIX Abbreviations

ICU

Intensive care unit IV Intravenous IVH Intraventricular hemorrhage LOC Level of consciousness LP Lumbar puncture MAP Mean arterial pressure MB Medulloblastoma MCA Middle cerebral arteries MMD Moyamoya disease MRI Magnetic resonance imaging MRV Magnetic resonance venography NAI Non-accidental injury NCD Neurocutaneous disorders NF1 Neurofibromatosis type 1 NF2 Neurofibromatosis type 2 NPO Nothing by mouth NTD Neural tube defects OAD Occipital atlantal dislocation PCA Pilocytic astrocytoma PET Positron emission tomography PFD Posterior fossa decompression PTS Posttraumatic seizures RARE MRI Rapid acceleration with relaxation enhancement MRI RCC Renal-cell carcinoma SAH Subarachnoid hemorrhage SBP Systolic blood pressure SDE Subdural empyema SEGA Subependymal giant cell astrocytoma SWS Sturge–Weber syndrome TBI Traumatic brain injury TIA Transient ischemic attack TP53 Tumor protein 53

XX

VGAM

Abbreviations

Vein of Galen aneurysmal malformation VHL Von Hippel-Lindau disease VP Ventriculoperitoneal WHO World Health Organization WI Weighted imaging yrs Years

1

Neuroanatomy Muffaq K. Lashhab, Oday Atallah, Ahmed Muthana, Mohammedbaqer A. Al-Ghuraibawi, Naba G. Husseini, and Samer S. Hoz

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 S. S. Hoz et al. (eds.), Pediatric Neurosurgery, https://doi. org/10.1007/978-3-031-49573-1_1

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M. K. Lashhab et al.

1. Circumventricular organs. The FALSE answer is:

A. The subfornical organ is a circumventricular organ. B. The subcommisural organ lacks a blood–brain barrier. C. The area postrema is the only paired circumventricular organ. D. The pineal gland has a role in maintaining the circadian rhythm. E. Organum vasculosum is an outlet for hypothalamic peptides. vvAnswer B

55 The subcommisural organ is the only circumventricular organ with an intact blood–brain barrier. 2. The climbing fibers of the cerebellum. The FALSE answer is:

A. They are excitatory fibers. B. They travel through the inferior cerebellar peduncle. C. They arise from the contralateral inferior olivary nucleus. D. They secrete aspartate. E. They synapse with both purkinje and granule cells. vvAnswer D

55 They secrete glutamate. 3. The hypothalamic nuclei. The FALSE answer is:

A. The posterior nucleus is responsible for arousal. B. The medial nucleus is concerned with satiety. C. The anterior and medial nuclei have a role in the parasympathetic response. D. The posterior and lateral nuclei are important in the sympathetic response. E. The anterior nucleus increases body temperature. vvAnswer E

55 The anterior nucleus decreases body temperature, while the posterior nucleus increases body temperature. 4. Visual field defects. The FALSE answer is:

A. Left nasal hemianopia is due to a lesion involving the left perichiasmal area. B. Left homonymous hemianopia is due to a lesion involving the right optic tract.

3 Neuroanatomy

1

C. Left homonymous superior quadranopia is due to a lesion involving the lower right optic radiation. D. Left homonymous superior quadranopia is due to a lesion involving the upper right optic radiation. E. Left homonymous hemianopia is due to a lesion involving the right occipital lobe. vvAnswer C

55 Left homonymous inferior quadranopia is due to a lesion involving the lower right optic radiation. 5. The deep peroneal nerve, innervation. The FALSE answer is:

A. Extensor digitorum brevis muscle. B. Extensor hallucis longus muscle. C. Extensor digitorum longus muscle. D. Peroneus longus muscle. E. Tibialis anterior muscle. vvAnswer D

55 The superficial peroneal nerve innervates the peroneus longus. 6. The corticospinal tract. The FALSE answer is:

A. Ninety percent of the descending fibers travel in the lateral corticospinal tracts. B. Uncrossed fibers travel in the anterior corticospinal tracts. C. Divides into three tracts at the spinomedullary junction. D. Passes through the anterior limb of the internal capsule. E. The sacral fibers occupy the most lateral aspect. vvAnswer D

55 The corticospinal tract fibers pass through the posterior limb of the internal capsule. 7. Papez circuit. The FALSE answer is:

A. It’s known as the medial limbic circuit. B. The amygdala is part of the circuit. C. The anterior thalamic nuclei constitute a key component of the hippocampal system for episodic memory. D. The circuit includes the mammillary bodies. E. The fornix is the major output tract of the hippocampus.

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M. K. Lashhab et al.

vvAnswer B

55 The amygdala is not part of the circuit. 8. Benedikt’s syndrome. The FALSE answer is:

A. It’s known as the paramedian part of midbrain syndrome. B. It includes cranial nerve (CN) III palsy. C. It involves the red nucleus. D. It is associated with hemiataxia. E. It can result from a superior cerebellar artery occlusion. vvAnswer E

55 It can result from a posterior cerebral artery occlusion. 9. Cerebral venous system. The FALSE answer is:

A. Internal cerebral veins are formed by the union of thalamostriate, choroidal, septal, epithalamic, and lateral ventricular veins. B. The basal vein of Rosenthal drains the anterior and medial temporal lobes. C. The vein of Galen merges with the inferior sagittal sinus to form the straight sinus. D. The vein of Trolard drains into the sigmoid sinus. E. The vein of Labbe drains into the transverse sinus. vvAnswer D

55 The vein of Trolard drains into the superior sagittal sinus. 10. The innervation of the reflexes. The FALSE answer is:

A. CN V2 mediates the sneeze reflex. B. CN 7 mediates the pectoral reflex. C. CN 10 mediates the cough reflex. D. CN 7 mediates the jaw-jerk reflex. E. CN 9 mediates the gag reflex. vvAnswer D

55 CN V mediates the jaw-jerk reflex. 11. The contents of the tarsal tunnel. The FALSE answer is:

A. Tibial nerve. B. Flexor hallicus longus tendon. C. Tibialis posterior tendon.

5 Neuroanatomy

1

D. Flexor digitorum brevis tendon. E. Flexor digitorum longus tendon. vvAnswer D

55 The flexor digitorm brevis is a broad muscle found deep in the sole of the foot, and its tendon is not part of the tarsal tunnel. 12. The musculocutaneous nerve, innervation. The FALSE answer is:

A. Coracobrachialis muscle. B. Biceps brachii muscle. C. Brachioradialis muscle. D. Brachialis muscle. E. Lateral forearm muscle. vvAnswer C

55 The brachioradialis muscle is innervated by c6 through the radial nerve. 13. The axillary nerve, innervation. The FALSE answer is:

A. Teres minor muscle. B. Teres major muscle. C. Deltoid muscle. D. The long head of the triceps muscle. E. Skin over the shoulder. vvAnswer B

55 The teres major muscle is supplied by the subscapular nerve from the C 5 to C 6 roots. 14. The nucleus pulposus of the intervertebral disc. The FALSE answer is:

A. It’s composed of collagen and a proteoglycan matrix. B. It’s abneural and avascular. C. It acts as a shock absorber. D. It is derived from the neural tube. E. It is made of 66% to 86% water. vvAnswer D

55 It is an embryological remnant of the notochord.

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15. The contiguous ligaments of the spine. The FALSE answer is:

A. Supraspinous ligament. B. Ligamentum flavum. C. Posterior longitudinal ligament. D. Anterior longitudinal ligament. E. Interspinous ligament. vvAnswer B

55 The ligamentum flavum is noncontiguous and connects laminae between segments. 16. The collagen fibers of the intervertebral disc. The FALSE answer is:

A. Type I is found in the normal anulus fibrosus. B. Type II is found in the nucleus pulposus. C. Type III is increased in areas of minor to advanced degeneration. D. Type VI is increased in areas of minor to advanced degeneration. E. Type III staining is enhanced in the degenerated endplate. vvAnswer E

55 Type II staining is enhanced in the degenerated endplate. 17. The contributors of spinal cord blood supply. The FALSE answer is:

A. Anterior radicular arteries. B. Posterior radicular arteries. C. Ascending cervical arteries. D. Carotid arteries. E. Deep cervical arteries. vvAnswer E

55 The carotid arteries are responsible for the blood supply to the brain, neck, and face but not the spinal cord. 18. Artery of Adamkiewicz. The FALSE answer is:

A. It supplies the anterior spinal cord. B. It is a large lower thoracic and upper lumber region blood supplier. C. It originates as a branch of the left posterior intercostal artery. D. It originates as a branch of the right posterior intercostal artery. E. It is known as a great anterior radicular artery.

7 Neuroanatomy

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vvAnswer D

55 It typically arises from a left posterior intercostal artery at the level of the ninth to 12th intercostal artery. 19. Spinal cord tracts, functions. The FALSE answer is:

A. Anterior spinothalamic tract: crude touch and pressure. B. Lateral spinothalamic tract: pain and temperature. C. Dorsal spinocerebellar tract: muscle and joints unconscious information. D. Lateral corticospinal tract: voluntary movements. E. Rubrospinal tract: reflex head turning. vvAnswer E

55 The tectospinal tract is responsible for controlling the movement of the head in response to auditory and visual stimuli. 20. Clarke’s nucleus. The FALSE answer is:

A. It is mainly located from C7 to 13–14 levels. B. It is found in the medial part of lamina VII. C. It is an important structure for motor function. D. It is located in the base of the posterior gray column. E. It’s known as nucleus dorsalis. vvAnswer C

55 The Clarke’s nucleus is involved in unconscious proprioception. 21. Rexed lamina, correspondence. The FALSE answer is:

A. Lamina II: substantia gelatinosa. B. Lamina IV: nucleus proprius. C. Lamina VII: dorsal nucleus of Clarke. D. Lamina I: marginal zone. E. Lamina V: Renshaw cells. vvAnswer E

55 Renshaw cells are mainly located in rexed laminae VII and VIII. 22. Spinal ligaments, extensions. The FALSE answer is:

A. The apical ligament extends from the tip of the dens to the basion. B. The posterior longitudinal ligament extends from C1 to S1.

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C. The transverse atlantal ligament extends between the tubercles of the lateral masses of the atlas. D. The anterior longitudinal ligament extends from the basioocciput to S1. E. Alar ligaments extend from the dens to the lateral margins of C1. vvAnswer E

55 Alar ligaments extend from the dens to the lateral margins of the foramen magnum. 23. Muscles that are innervated by the recurrent branch of the laryngeal nerve. The FALSE answer is:

A. Transverse arytenoid muscle. B. Thyroepiglottic muscle. C. Posterior cricoarytenoid muscle. D. Cricothyroid muscle. E. Anterior cricoarytenoid muscle. vvAnswer D

55 The recurrent laryngeal nerve innervates all intrinsic laryngeal muscles except the cricothyroid, which is supplied by the external branch of the superior laryngeal nerve. 24. The inferior division of the oculomotor nerve. The FALSE answer is:

A. It innervates the levator palpebrae muscle. B. It innervates the inferior oblique muscle. C. It innervates the medial rectus muscle. D. It innervates the inferior rectus muscle. E. It passes through the anuulus of Zinn. vvAnswer A

55 The levator palpebrae muscle is innervated by the superior division of the oculomotor nerve. 25. The hypoglossal nerve, innervation. The FALSE answer is:

A. Palatoglossus muscle. B. Styloglossus muscle. C. Genioglossus muscle. D. Hypoglossus muscle. E. Intrinsic muscles of the tongue.

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vvAnswer A

55 The vagus nerve innervates the palatoglossus muscle. 26. The CN contains special visceral afferent fibers. The FALSE answer is:

A. B. C. D. E.

Facial nerve. Glossopharyngeal nerve. Olfactory nerve. Trigeminal nerve. Vagus nerve.

vvAnswer D

55 The trigeminal nerve doesn’t contain special visceral afferent fibers. 27. Skull base triangles and borders. The FALSE answer is:

A. Glasscock’s triangle: third division of trigeminal + grater petrosal nerve + foramen spinosum/arcuate eminence. B. Kawase’s triangle: third division of trigeminal nerve + GSPN + arcuate eminent + superficial petrosal sinus. C. Parkinson’s triangle: oculomotor nerve + second division of trigeminal nerve + tentorial edge. D. Clinoidal triangle: optic nerve + oculomotor nerve + tentorial edge. E. Supratrochlear triangle: oculomotor nerve + trochlear nerve + dura of petrosal edge. vvAnswer C

55 Parkinson’s triangle is bound by the trochlear nerve, the first division of the trigeminal nerve, and the tentorial edge. 28. Structures pass through the annulus of Zinn. The FALSE answer is:

A. Frontal nerve. B. Oculomotor Nerve. C. Roots of the ciliary ganglion. D. Nasociliary nerve. E. Abducens nerve. vvAnswer A

55 The frontal nerve passes outside the annulus of Zinn.

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29. Ventricular system portions that contain choroid plexus. The FALSE answer is:

A. Third ventricle posterior roof. B. Fourth ventricle roof. C. Lateral ventricle posterior roof. D. Cerebral aqueduct. E. Interventricular foramen of luschka. vvAnswer D

55 The only portion of the ventricular system that does not contain choroid plexus is the cerebral aqueduct. 30. Brain stem nuclei, functions. The FALSE answer is:

A. Interstitial nucleus of cajal: horizontal eye movements. B. Darkshevich’s nucleus: nucleus of posterior commissure. C. Pretectal nucleus: controls the direct and consensual pupillary light reflex. D. Locus ceruleus: cortical activation and REM sleep. E. Raphe nuclei: deep sleep mood. vvAnswer A

55 Interstitial nucleus of cajal controls vertical eye movements. 31. The floor of the fourth ventricle, structures. The FALSE answer is:

A. Facial colliculi: located above the laterally projecting fibers of the stria medullari. B. Vagal trigon: located lateral to the hypoglossal trigon. C. Hypoglossal trigon: is closest to midline. D. Hypoglossal trigon: overlies the hypoglossal nucleus. E. Hypoglossal trigon: located below the stria. vvAnswer E

55 The hypoglossal trigon is located above the stria. 32. The anatomy of the cerebellum. The FALSE answer is.

A. Anterior lobe: belong to the paleocerebellum. B. Flocculus and nodulus: belong to the archicerebellum. C. Neocerebellum: belong to the posterior lobe. D. Uvula: belong to the neocerebellum. E. Lingula: belong to the paleocerebellum.

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vvAnswer E

55 The lingual is considered a part of the vermis belonging to the archicerebellum. 33. The deep cerebellar nuclei. The FALSE answer is:

A. B. C. D. E.

Globose. Fastigial. Emboliform. Vestibular. Dentate.

vvAnswer D

55 The vestibular nuclei are located in the medulla and pons of the hindbrain. 34. Skull base foraminae, contain. The FALSE answer is:

A. Foremen cecum: middle meningeal artery. B. Foramen lacerum: vidian nerve. C. Foremen ovale: CN V3. D. Foramen rotundum: CN V2. E. Jugular foramen: Jacobson nerve. vvAnswer A

55 The middle meningeal artery passes through the foramen spinosum. 35. Cerebral vein of Galen, direct drainage. The FALSE answer is:

A. Precentral cerebellar vein. B. Basal vein of Rosenthal. C. Internal cerebral vein. D. Thalamostriate vein. E. It drains into the straight sinus. vvAnswer D

55 The thalamostriate vein drains into the internal cerebral vein.

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36. Associations of thalamic nuclei and their corresponding cortical ­projections. The FALSE answer is:

A. Pulvinar: cingulate gyrus. B. Anterior nuclei: cingulate cortex. C. Mediodorsal nuclei: orbital frontal cortex. D. Ventral posterolateral nuclei: somatosensory cortex. E. Medial and lateral geniculate bodies: frontal cortex. vvAnswer A

55 The pulvinar projects efferent fibers to the visual cortex. 37. Brodmann’s area locations. The FALSE answer is:

A. Brodmann’s area 4: postcentral gyrus. B. Brodmann’s area 5: superior parietal lobule. C. Brodmann’s area 17: calcarine fissure. D. Brodmann’s area 39: angular gyrus. E. Brodmann’s area 44: frontal operculum. vvAnswer A

55 Brodmann’s area 4: precentral gyrus. 38. Areas involved in the posterior cerebral artery infarction. The FALSE answer is:

A. B. C. D. E.

Thalamus. Occipital lobe. Choroid plexus. Temporal lobe. Medulla.

vvAnswer E

55 The medulla is supplied by anterior and posterior spinal arteries, PICA, and vertebral arteries. 39. Upper limb myotomes. The FALSE answer is:

A. C2: neck flexion. B. C3: neck flexion. C. C4: shoulder elevation. D. C5: shoulder elevation. E. C6: elbow flexion.

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vvAnswer B

55 C3 is responsible for neck lateral flexion. 40. Lower limbs myotomes. The FALSE answer is:

A. L1/L2: hip adduction. B. L3/L4: knee extension. C. L5/S1: knee flexion. D. L5: great toe extension. E. S1: great toe flexion. vvAnswer A

55 L1/2 is responsible for hip abduction.

Bibliography 1. Ganong WF. Circumventricular organs: definition and role in the regulation of endocrine and autonomic function. Clin Exp Pharmacol Physiol. 2000;27(5–6):422–7. 2. Watanabe M, Kano M. Climbing fiber synapse elimination in cerebellar Purkinje cells. Eur J Neurosci. 2011;34(10):1697–710. 3. Dampney RA, Horiuchi J, Killinger S, Sheriff MJ, Tan PS, McDowall LM. Long-term regulation of arterial blood pressure by hypothalamic nuclei: some critical questions. Clin Exp Pharmacol Physiol. 2005;32(5–6):419–25. 4. Pollock A, Hazelton C, Rowe FJ, Jonuscheit S, Kernohan A, Angilley J, Henderson CA, Langhorne P, Campbell P.  Interventions for visual field defects in people with stroke. Cochrane Database Syst Rev. 2019;5:CD008388. 5. Tzika M, Paraskevas GK, Kitsoulis P. The accessory deep peroneal nerve: a review of the literature. Foot. 2012;22(3):232–4. 6. Welniarz Q, Dusart I, Roze E.  The corticospinal tract: evolution, development, and human disorders. Dev Neurobiol. 2017;77(7):810–29. 7. Forno G, Lladó A, Hornberger M.  Going round in circles—the Papez circuit in Alzheimer’s disease. Eur J Neurosci. 2021;54(10):7668–87. 8. Paidakakos NA, Rokas E, Theodoropoulos S, Dimogerontas G, Konstantinidis E. Posttraumatic Benedikt’s syndrome: a rare entity with unclear anatomopathological correlations. World Neurosurg. 2012;78(6):715–e13. 9. Uddin MA, Haq TU, Rafique MZ. Cerebral venous system anatomy. J Pak Med Assoc. 2006;56(11):516. 10. Jääskeläinen SK. Differential diagnosis of chronic neuropathic orofacial pain: role of clinical neurophysiology. J Clin Neurophysiol. 2019;36(6):422–9. 11. McSweeney SC, Cichero M.  Tarsal tunnel syndrome—a narrative literature review. Foot. 2015;25(4):244–50. 12. Guerri-Guttenberg RA, Ingolotti M.  Classifying musculocutaneous nerve variations. Clin Anat. 2009;22(6):671–83.

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13. Leechavengvongs S, Teerawutthichaikit T, Witoonchart K, Uerpairojkit C, Malungpaishrope K, Suppauksorn S, Chareonwat B. Surgical anatomy of the axillary nerve branches to the deltoid muscle. Clin Anat. 2015;28(1):118–22. 14. Nomura T, Mochida J, Okuma M, Nishimura K, Sakabe K. Nucleus pulposus allograft retards intervertebral disc degeneration. Clin Orthop Relat Res. 2001;389:94–101. 15. Choi SJ, Shin MJ, Kim SM, Bae SJ.  Non-contiguous spinal injury in cervical spinal trauma: evaluation with cervical spine MRI. Korean J Radiol. 2004;5(4):219–24. 16. Sharabi M, Wade K, Haj-Ali R. The mechanical role of collagen fibers in the intervertebral disc. In: Biomechanics of the spine. Academic Press; 2018. p. 105–23. 17. Colman MW, Hornicek FJ, Schwab JH. Spinal cord blood supply and its surgical implications. JAAOS J Am Acad Orthop Surg. 2015;23(10):581–91. 18. Murthy NS, Maus TP, Behrns CL. Intraforaminal location of the great anterior radiculomedullary artery (artery of Adamkiewicz): a retrospective review. Pain Med. 2010;11(12):1756–64. 19. Honeycutt CF, Kharouta M, Perreault EJ. Evidence for reticulospinal contributions to coordinated finger movements in humans. J Neurophysiol. 2013;110(7):1476–83. 20. Boehme CC. The neural structure of Clarke’s nucleus of the spinal cord. J Comp Neurol. 1968;132(3):445–61. 21. Coulon P, Bras H, Vinay L.  Characterization of last-order premotor interneurons by transneuronal tracing with rabies virus in the neonatal mouse spinal cord. J Comp Neurol. 2011;519(17):3470–87. 22. Gillespie KA, Dickey JP. Biomechanical role of lumbar spine ligaments in flexion and extension: determination using a parallel linkage robot and a porcine model. Spine. 2004;29(11):1208–16. 23. Maranillo E, Leon X, Orus C, Quer M, Sanudo JR. Variability in nerve patterns of the adductor muscle group supplied by the recurrent laryngeal nerve. Laryngoscope. 2005;115(2):358–62. 24. Sadagopan KA, Wasserman BN.  Managing the patient with oculomotor nerve palsy. Curr Opin Ophthalmol. 2013;24(5):438–47. 25. Lin HC, Barkhaus PE.  Cranial nerve XII: the hypoglossal nerve. Semin Neurol. 2009;29(1):45–52. 26. Bailey TW, Hermes SM, Andresen MC, Aicher SA. Cranial visceral afferent pathways through the nucleus of the solitary tract to caudal ventrolateral medulla or paraventricular hypothalamus: target-specific synaptic reliability and convergence patterns. J Neurosci. 2006;26(46):11893–902. 27. Hendricks BK, Benet A, Lawrence PM, Benner D, Preul MC, Lawton MT. Anatomical triangles for use in skull base surgery: a comprehensive review. World Neurosurg. 2022;164:79–92. 28. Lacey H, Oliphant H, Smith C, Koenig M, Rajak S.  Topographical anatomy of the annulus of zinn. Sci Rep. 2022;12(1):1064. 29. Greiner T, Manzhula K, Baumann L, Kaddatz H, Runge J, Keiler J, Kipp M, Joost S. Morphology of the murine choroid plexus: attachment regions and spatial relation to the subarachnoid space. Front Neuroanat. 2022;16:1046017. 30. Biacabe B, Chevallier JM, Avan P, Bonfils P. Functional anatomy of auditory brainstem nuclei: application to the anatomical basis of brainstem auditory evoked potentials. Auris Nasus Larynx. 2001;28(1):85–94.

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31. Moon RD, Walsh P, Singleton WG, Upex A, Edwards RJ, Carter MR, Fellows GA. Intra-operative neurophysiological mapping to identify distorted functional anatomy of the 4th ventricle in a 5-month-old infant. Childs Nerv Syst. 2022;38:1371. 32. Lehman VT, Black DF, DeLone DR, Blezek DJ, Kaufmann TJ, Brinjikji W, Welker KM. Current concepts of cross-sectional and functional anatomy of the cerebellum: a pictorial review and atlas. Br J Radiol. 2020;93(1106):20190467. 33. Habas C.  Functional imaging of the deep cerebellar nuclei: a review. Cerebellum. 2010;9:22–8. 34. Ginat DT, Ellika SK, Corrigan J. Multi–detector-row computed tomography imaging of variant skull base foramina. J Comput Assist Tomogr. 2013;37(4):481–5. 35. Levrier O, Gailloud PH, Souei M, Manera L, Brunel H, Raybaud C. Normal galenic drainage of the deep cerebral venous system in two cases of vein of Galen aneurysmal malformation. Childs Nerv Syst. 2004;20:91–7. 36. Tamietto M, Pullens P, de Gelder B, Weiskrantz L, Goebel R. Subcortical connections to human amygdala and changes following destruction of the visual cortex. Curr Biol. 2012;22(15):1449–55. 37. Ardila A, Bernal B, Rosselli M. How localized are language brain areas? A review of Brodmann areas involvement in oral language. Arch Clin Neuropsychol. 2016;31(1):112– 22. 38. Capitani E, Laiacona M, Pagani R, Capasso R, Zampetti P, Miceli G. Posterior cerebral artery infarcts and semantic category dissociations: a study of 28 patients. Brain. 2009;132(4):965–81. 39. Bell SW, Brown MJ, Hems TJ. Refinement of myotome values in the upper limb: evidence from brachial plexus injuries. Surgeon. 2017;15(1):1–6. 40. Hashimoto S, Murohashi T, Yamada S, Iesato N, Ogon I, Chiba M, Tsukamoto A, Hitrota R, Yoshimoto M.  Broad and asymmetric lower extremity myotomes: results from intraoperative direct electrical stimulation of the lumbosacral spinal roots. Spine. 2023:10–97. https://doi.org/10.1097/BRS.0000000000004737.

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Development of Central Nervous System Aras F. Albarazanchi, Oday Atallah, Ahmed Muthana, Tabarek F. Mohammed, Sara A. Mohammad, and Samer S. Hoz

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 S. S. Hoz et al. (eds.), Pediatric Neurosurgery, https://doi. org/10.1007/978-3-031-49573-1_2

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1. Nervous system develops from ectoderm. The FALSE answer is:

2

A. By the third week, the neural plate becomes a groove with neural folds along each side. B. By the fourth week, the neural fold join to form the neural tube. C. By the fourth week, the neural tube exhibits three anterior dilations. D. The central canal of the spinal cord and the brain’s ventricles are formed by the neural tube’s lumen. E. Cells along the margin of the neural groove are called the neural fold. vvAnswer E

55 Cells along the margin of the neural groove are called the neural crest. 2. Central nervous system (CNS) development. The FALSE answer is:

A. Two days after conception, the neural tube, which will develop into the brain and spinal cord, is formed. B. Folate is essential for embryonic CNS development. C. The prevention of hypoglycemia and its resultant effects on CNS development are critically important in the newborn period. D. Neuropathologic alterations likely result from copper deficiency, as copper is crucial during the embryonic stages of CNS development. E. During neural development, excess neural cells are eliminated by programmed cell death (PCD) in both the CNS and peripheral nervous system. vvAnswer A

55 Neural development begins 2 weeks after conception with the formation of the neural tube, which will become the brain and spinal cord. 3. Glial cells. The FALSE answer is:

A. These cells are primitive supporting cells. B. They are composed of neuroepithelial cells. C. They migrate from the neuroepithelial layer to the mantle and marginal layers. D. They are mainly found in the marginal layer. E. They serve both structural and metabolic purposes.

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vvAnswer D

55 These cells are situated between blood vessels and neurons where they provide support and serve metabolic functions. 4. Neural tube development. The FALSE answer is:

A. The notochord plays a crucial role in development patterning but does not directly contribute to the mature nervous system. B. The ectoderm consists primarily of the columnar and cuboidal regions. C. The columnar ectoderm is the lateral surface ectoderm that forms the epidermis of skin, hair, glands, the anterior pituitary, teeth enamel, and sensory placodes. D. The neural plate develops from the buccopharyngeal membrane to the primitive node, sitting atop the notochord and paraxial mesoderm. E. There are two neural bending processes occurring in the formation of the neural groove and neural tube: initial bending leads to the formation of the neural groove, while later bending leads to the formation of the neural tube. vvAnswer C

55 The columnar ectoderm is a midline neural plate that gives rise to the neural tube and crest. 5. Brain development. The FALSE answer is:

A. In week 4, the neural tube separates into the three primary brain vesicles that will eventually give rise to the forebrain, midbrain, and hindbrain. B. The telencephalon, which consists of secondary vesicles, matures into the structure of the cerebral hemispheres. C. Anencephaly occurs when the anterior neuropore does not close. D. The metencephalon, the secondary vesicle, develops into the adult’s midbrain. E. The medulla arises from the hindbrain. vvAnswer D

55 The hindbrain vesicle gives rise to the secondary vesicle metencephalon, which forms the adult structure of the pons and cerebellum, while the midbrain is formed by the midbrain vesicle.

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6. Cerebral hemispheres. The FALSE answer is:

2

A. Appear in the early stages of the fifth week of development as bilateral evaginations of the prosencephalon’s lateral wall. B. By the middle of the second month, the basal portion of the cerebral hemispheres begins to grow and bulge, acquiring the appearance of corpus striatum. C. Near the diencephalon, the choroid plexus is composed of a single layer of ependymal cells covered by vascular mesenchyme. D. The amygdala is a thickening of the hemisphere’s wall that occurs just above the choroidal fissure. E. Laterally, the diencephalon, mesencephalon, and cephalic portions of the metencephalon are all encompassed by the expanding hemispheres. vvAnswer D

55 The hippocampus develops from a thickening of the hemisphere’s wall above the choroidal fissure. 7. Commissures. The FALSE answer is:

A. In the lamina terminalis, the anterior commissure is the first of the crossing bundles to become visible. B. The hippocampal commissure (fornix) is the second commissure to appear. C. By the third week of development, the corpus callosum is present. D. The anterior commissure is made up of nerve fibers that reach from one olfactory bulb in each hemisphere to the other. E. The corpus callosum joins the nonolfactory regions of the right and left cerebral cortices. vvAnswer C

55 By the tenth week of development, the corpus callosum has formed. 8. Development of the spinal cord. The FALSE answer is:

A. The neural tube has three cellular layers: the ventricular zone, the intermediate zone, and the marginal zone. B. Neuroblasts, precursors to neurons, are created in the ventricular zone. C. Glioblasts originate in the ventricular zone and divide into the alar and basal plates in the intermediate zone.

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D. The posterior horn of the spinal cord is composed of afferent neurons that develop from basal plate cells. E. The neural tube’s lumen transforms into the central canal of the spinal cord. vvAnswer D

55 The cells in the alar plate differentiate into afferent (sensory) neurons and make up the spinal cord’s posterior horn, while those in the basal plate give rise to efferent (motor) neurons that make up the cord’s anterior horn. 9. Development of meninges. The FALSE answer is:

A. Cells of the neural crest and mesenchyme (mesoderm) migrate to surround the developing CNS between 20 and 35 days of gestation, giving rise to the meninges. B. The origin of the dura mater is in the paraxial mesoderm. C. The pia and arachnoid maters originate from neural crest cells. D. Spina bifida with myeloschisis occurs when the caudal neuropore does not close by the end of the fourth week of development. E. At the end of the second trimester, the meninges’ overall structure is complete. vvAnswer E

55 The meninges formation is complete by the end of the first trimester. 10. Development of the spinal cord. The FALSE answer is:

A. At week 8 of development, the spinal cord fills the entire vertebral canal. B. The conus medullaris may reach the L3–L4 ­vertebra in preterm infants. C. The mantle is the outermost segment of the spinal cord. D. Two ventral horns of the developing spinal cord protrude ventrally to form a ventral median fissure. E. The dorsal median septum is formed when the dorsal horns of the developing spinal cord fuse with one another. vvAnswer C

55 The outer cellular layer of the spinal cord is called the marginal zone, while the middle layer is called the mantle.

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11. Cerebral palsy. The following are direct causes that induce injury to the developing brain which results in cerebral palsy. The FALSE answer is:

2

A. B. C. D. E.

Bilirubin encephalopathy. Encephalitis. Intracranial hemorrhage. Hypoglycemia. Hypokalemia.

vvAnswer E

55 Hypokalemia is not a cause of cerebral palsy, but hypoglycemia is a significant one. 12. A 25-year-old primigravida woman undergoes amniocentesis during the 25th week of gestation, which reveals an increased level of acetylcholinesterase (AChE) in the amniotic fluid. The FALSE answer is:

A. There is a significant risk of neural tube defect. B. The results indicate the possibility of a fetal ­abnormality due to a failed fusion. C. AChE levels above normal indicate failed apoptosis. D. Neural tube defect would develop if the neural tube failed to fuse around either the anterior or posterior neuropores. E. Prenatal diagnosis of neural tube defect is made possible by the leakage of alpha-fetoprotein and AChE. vvAnswer C

55 Neural tube defect develops when the neural tube fails to fuse where the anterior or posterior neuropores are located. As a result, the spinal canal and amniotic cavity continue to communicate. Failed fusion is the fetal abnormality in this case. 13. Cerebrospinal fluid (CSF). The FALSE answer is:

A. Produced by the choroid plexuses of the cerebral ventricles. B. The ependymal layer of the plexuses secretes between 100 and 1000 mL of CSF daily. C. CSF also travels into the subarachnoid space, which surrounds the CNS, and the spinal canal. D. From the subarachnoid space, CSF is absorbed into the veins. E. The CSF “floats” the brain and acts as a cushion for it.

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vvAnswer B

55 About 400–500 mL of CSF is produced daily by the ependymal layer of the plexuses. 14. CN. The FALSE answer is:

A. All 12 CN nuclei are established by week 2 of development. B. Only the CN I and CN II do not originate in the brainstem. C. Out of all the brain regions, only the CN III develops outside the hindbrain. D. Cranial nuclei’s motor neurons reside in the brainstem. E. Not all CN are composed of both motor and sensory fibers. vvAnswer A

55 All 12 CN nuclei are present by the fourth week of development. 15. Origins of CN. The FALSE answer is:

A. Telencephalon is where the CN I originates. B. The CN II originates from the diencephalon. C. The CN III originates from the metencephalon. D. The CN IV originates from the metencephalon. E. The CN V originates from the metencephalon. vvAnswer C

55 The CN III originates from the mesencephalon. 16. Autonomic nervous system. The FALSE answer is:

A. It contains both sympathetic and parasympathetic parts. B. It is a two-neuron system composed of preganglionic and postganglionic axons. C. The intermediate (lateral) horns are home to the preganglionic neurons of the sympathetic nervous system. D. Sympathetic trunks and paravertebral (preaortic) ganglia containing postganglionic neurons are located along the aorta. E. The brainstem is where the nuclei of all CN that are parasympathetic preganglionic neurons are located. vvAnswer D

55 Preganglionic neurons are located in the paravertebral (preaortic) ganglia and sympathetic trunks along the aorta.

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17. Autonomic nervous system. The FALSE answer is:

2

A. It is composed of motor (efferent) fibers that innervate smooth and cardiac muscle and secretory glands. B. It is also known as the visceral motor system. C. The brain’s gray matter is home to the preganglionic neurons’ cell bodies. D. The brain’s white matter is home to the postganglionic neurons’ cellular bodies. E. The sympathetic nervous system uses the neurotransmitter norepinephrine, while the parasympathetic nervous system relies on acetylcholine. vvAnswer D

55 Postganglionic neurons’ cell bodies are located outside of the CNS in autonomic ganglia, and their fibers terminate on target organs. 18. Holoprosencephaly. The FALSE answer is:

A. Holoprosencephaly occurs in 1/15,000 live births but in 1/250 pregnancies that result in a stillbirth. B. In severe cases, a single telencephalic vesicle is formed from the lateral ventricles. C. The prosencephalon sometimes splits into two cerebral hemispheres in less severe cases. D. Sometimes the presence of a single central incisor is the only sign of holoprosencephaly in extremely mild cases. E. Usually, the corpus callosum and olfactory bulbs/tracts are hypoplastic or absent. vvAnswer A

55 While holoprosencephaly affects 1  in 15,000 live births, it is present in 1/250 of all pregnancies that end prematurely. 19. Ossification defects in bones of the skull. The FALSE answer is:

A. It occurs in 1 in every 12,000 births. B. Meningocele, meningoencephalocele, and meningohydroencephalocele are all possible outcomes. C. The basilar and lateral aspects of the occipital bone are the most commonly affected bones. D. If the hole in the occipital bone is narrow, only the meninges will protrude.

25 Development of Central Nervous System

2

E. Depending on the size of the hole, brain tissue or even the ventricle could squeeze through and enter the meningeal sac below. vvAnswer C

55 The squamous portion of the occipital bone is the most common site of involvement. 20. Cranial defects. The FALSE answer is:

A. Exencephaly is characterized by failure of the cephalic part of the neural tube to close. B. Anencephalic fetuses have renal agenesis, which results in oligohydramnios early in the pregnancy. C. A prenatal insult like an infection or drug exposure could cause microcephaly, but it could also be inherited. D. Large clefts between the cerebral hemispheres are characteristic of the extremely rare disorder known as schizencephaly. E. Spina bifida and anencephaly can be caused by hyperthermia, which can be caused by maternal infection or by taking sauna or hot tub baths. vvAnswer B

55 Due to the absence of a swallowing reflex, anencephalic fetuses tend to develop polyhydramnios in the final 2 months of pregnancy.

Bibliography 1. Arendt D, Tosches MA, Marlow H.  From nerve net to nerve ring, nerve cord and brain—evolution of the nervous system. Nat Rev Neurosci. 2016;17(1):61–72. 2. Holland LZ, Carvalho JE, Escriva H, Laudet V, Schubert M, Shimeld SM, Yu JK. Evolution of bilaterian central nervous systems: a single origin? EvoDevo. 2013;4:1– 20. 3. Parpura V, Heneka MT, Montana V, Oliet SH, Schousboe A, Haydon PG, Stout RF Jr, Spray DC, Reichenbach A, Pannicke T, Pekny M.  Glial cells in (patho) physiology. J Neurochem. 2012;121(1):4–27. 4. Avagliano L, Massa V, George TM, Qureshy S, Bulfamante GP, Finnell RH. Overview on neural tube defects: from development to physical characteristics. Birth Defects Res. 2019;111(19):1455–67. 5. Stiles J, Jernigan TL.  The basics of brain development. Neuropsychol Rev. 2010;20(4):327–48. 6. McGilchrist I.  Reciprocal organization of the cerebral hemispheres. Dialogues Clin Neurosci. 2010;12(4):503–15.

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7. Comer JD, Alvarez S, Butler SJ, Kaltschmidt JA.  Commissural axon guidance in the developing spinal cord: from Cajal to the present day. Neural Dev. 2019;14(1):1–6. 8. Alaynick WA, Jessell TM, Pfaff SL.  SnapShot: spinal cord development. Cell. 2011;146(1):178. 9. Dasgupta K, Jeong J.  Developmental biology of the meninges. Genesis. 2019;57(5):e23288. 10. Lu DC, Niu T, Alaynick WA. Molecular and cellular development of spinal cord locomotor circuitry. Front Mol Neurosci. 2015;8:25. 11. Vitrikas K, Dalton H, Breish D.  Cerebral palsy: an overview. Am Fam Physician. 2020;101(4):213–20. 12. Zaganjor I, Sekkarie A, Tsang BL, Williams J, Razzaghi H, Mulinare J, Sniezek JE, Cannon MJ, Rosenthal J. Describing the prevalence of neural tube defects worldwide: a systematic literature review. PLoS One. 2016;11(4):e0151586. 13. Tumani H, Huss A, Bachhuber F. The cerebrospinal fluid and barriers–anatomic and physiologic considerations. Handb Clin Neurol. 2018;146:21–32. 14. Tubbs RS, Rizk EB, Shoja MM, Loukas M, Barbaro N, Spinner RJ, editors. Nerves and nerve injuries, History, embryology, anatomy, imaging, and diagnostics, vol. 1. Academic Press; 2015. 15. Joyce C, Le PH, Peterson DC.  Neuroanatomy, cranial nerve 3 (oculomotor). In: StatPearls. StatPearls Publishing; 2022. 16. Gibbons CH.  Basics of autonomic nervous system function. Handb Clin Neurol. 2019;160:407–18. 17. Freeman R, Chapleau MW. Testing the autonomic nervous system. Handb Clin Neurol. 2013;115:115–36. 18. Roessler E, Muenke M. The molecular genetics of holoprosencephaly. Am J Med Genet C Semin Med Genet. 2010;154(1):52–61. 19. Morice A, Paternoster G, Ostertag A, James S, Cohen-Solal M, Khonsari RH, Arnaud E.  Anterior skull base and pericranial flap ossification after frontofacial monobloc advancement. Plast Reconstr Surg. 2018;141(2):437–45. 20. Sandlin AT, Chauhan SP, Magann EF. Clinical relevance of sonographically estimated amniotic fluid volume: polyhydramnios. J Ultrasound Med. 2013;32(5):851–63.

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Neurological Examination Nada Mohammed, Leen R. Azzam, and Ian Pople

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 S. S. Hoz et al. (eds.), Pediatric Neurosurgery, https://doi. org/10.1007/978-3-031-49573-1_3

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1. The important components of newborn history. The FALSE answer is:

3

A. Parents’ medical and genetic history. B. Past pregnancies. C. Prenatal screening test. D. Social history. E. Risk factors of sepsis are not important. vvAnswer E

55 It is important to ask about risk factor of sepsis, such as: preterm delivery, and maternal high temperature. 2. In Newborn history. The FALSE answer is:

A. Gestational age has nothing to do with the newborn neurological examination. B. A complete pregnancy history is important. C. History of labor and delivery will give a clue to the timing of neurological injury. D. The child’s Apgar scores is of importance. E. Family history is essential. It may give a clue to varieties of neurological disorders. vvAnswer A

55 Gestational age is very important in the interpretation of the newborn neurological examination. 3. Components of newborn neurological examination. The FALSE answer is:

A. Level of alertness. B. CN. C. Motor function. D. Examination of sensory system is not applicable. E. Primitive reflexes. vvAnswer D

55 Sensation can be assessed, but limited to touch and pain. 4. Observation of the neonate. The FALSE answer is:

A. Observation of a resting infant is the most important factor in neonatal examination. B. Observation should include: level of alertness, respiratory pattern, tone, and spontaneous movement.

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C. Pre term neonate at 28 weeks typical posture is: flexed arms and fully extend legs. D. Full term infants will be in a full flexion posture. E. Premature neonate at 24 weeks will be lying in lateral position. vvAnswer C

55 The typical posture for preterm neonates 28  weeks is lying with fully extended arms and legs. 5. Observation of the neonate. The FALSE answer is:

A. A neonate who has brachial plexus injury will show a spastic upper limb. B. Seizures can be evident as a stereotypic, repetitive rhythmic movement in a limb. C. Episodes of apnea and irregular respiration may indicate posterior fossa lesions or seizures. D. A sleeping neonate will have normal sleep pattern with no spontaneous movement. E. Hyper alert and abnormal posture may indicate encephalopathy. vvAnswer A

55 In plexus injury the limb will be flaccid. 6. Examination of the newborn’s head and face. The FALSE answer is:

A. The anterior fontanel is diamond shape and is 1–4 cm. B. The triangular posterior fontanel, at birth can just admit the tip of the finger. C. At birth the normal splaying-width of suture can reach 2.5 cm. D. Head molding is normal after vaginal delivery. E. Small anterior fontanel seen in hyperthyroidism, while wide fontanel indicates hypothyroidism. vvAnswer C

55 The normal suture split (width) is 1 cm. 7. Examination of the newborn’s head. The FALSE answer is:

A. Caput succedaneum is a collection of fluid above the periosteum and disappears in few days. B. Cephalhematoma is a collection of blood underneath the periosteum and can cross the suture lines.

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C. Cephalhematoma usually is due to rupture of blood vessels and disappears in a few weeks. D. Subgaleal hemorrhage is usually due to rupture of emissary veins post-traumatic delivery. E. Sutures ridging may indicate craniosynostosis.

3

vvAnswer B

55 Cephalhematoma does not cross the suture lines. 8. Primitive reflexes. The FALSE answer is:

A. Are gestational age-dependent. B. Sucking, snout, and rooting reflexes counts as primitive oral reflexes. C. Moro reflex appear at 28 weeks gestational age and disappear at 6 months of age. D. Abnormal primitive reflexes is a sign of neurological dysfunction. E. Grasping reflex persist until 18 months of age. vvAnswer E

55 Grasping reflex disappears at 4–6 months of age. 9. Primitive reflexes. The FALSE answer is:

A. Checking primitive reflexes is not important in newborn neurological examination. B. Absence or asymmetry of primitive reflexes before 4 months of age may indicate cerebral palsy. C. Each primitive reflex has specific age to appear in and disappear at. D. Presence of 5 or more abnormal reflexes is a clue of cerebral palsy or mental delays. E. Oral primitive reflexes are essential for neonates feeding. vvAnswer A

55 Primitive reflexes are very important in newborn neurological examination. 10. Neonate physical examination. The FALSE answer is:

A. Skin birthmarks may indicate spinal dysraphism. B. Abnormal hair whorl may indicate abnormal brain growth. C. Skin abnormalities may indicate neuro-cutaneous syndromes. D. Head circumference at birth is 33 ± 2 cm for a normal full-term baby. E. Sacral dimples can be a normal finding.

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vvAnswer D

55 Normal range of head circumference at birth is 35 ± 2 cm. 11. Neurological examination in the neonate. The FALSE answer is:

A. Mental status cannot be assessed during sleep. B. Duration of sleep and degree of activity during wakefulness is the same in the pre-term infant regardless of age. C. In a full-term neonate eyes can blink to bright light stimulus. D. At the age of 34 weeks or older, the neonate may be able to visually fix and follow. E. Lethargic neonates will respond poorly to stimulation. vvAnswer B

55 The duration of sleep and degree of activity during wakefulness differ depending on age. 12. Regarding CN examination in the neonate. The FALSE answer is:

A. Olfactory nerve assessment can be achieved. B. Normal visual acuity can be confirmed if the newborn can fix and follow. C. Disconjugate eye gaze cannot be seen in a normal neonate. D. Sucking is a good indicator for the status of the muscles of mastication. E. CN VII can be assessed at rest, during crying and sucking. vvAnswer C

55 If a normal neonate is not fixing on an object, a disconjugate eye gaze can normally be elicited. 13. In the CN examination in the neonate. The FALSE answer is:

A. Normal sucking indicates normal CN IX, X, XII. B. Blinking or startle, after a sharp sound can indicate intact hearing. C. CN IX, X can be tested by the presence of the gag reflex. D. Shoulder drop can indicate CN XI palsy. E. The CN XII is difficult to be assessed in neonates. vvAnswer E

55 Tongue atrophy and deviation can indicate hypoglossal nerve injury.

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14. Neonate motor and sensory examination.

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A. Normal motor function in a normal newborn is age dependent. B. Motor examination includes: posture, tone, and spontaneous motor activity, all by observation. C. Hypertonia can by indicated in full term neonate by the presence of extension in the lower limbs. D. Flexion of upper limbs in a preterm neonate is normal. E. Sensory examination is difficult to be conducted in neonates. vvAnswer D

55 The flexion of upper limbs in a premature neonate indicates hypertonia. 15. Neurological examination of the infant. The FALSE answer is:

A. Stranger anxiety may indicate good cognitive development. B. Unlike in neonates, observation has no role in examining the infant’s mental status. C. Language can be evaluated by observing body signals like head turning to words and smiling. D. Primitive reflexes should be evaluated. E. Detailed history is important step in the neurological assessment. vvAnswer B

55 Inspection plays an important role in an infant’s mental status e­ valuation. 16. CN examination in infants. The FALSE answer is:

A. Ammonia can be used to test the olfactory nerve. B. Pupillary reaction and optic discs are important, and should be assessed when examining the optic nerve. C. Eye movement examination will give a clue about the function of CN III, IV, and VI. D. Infants older than 3 months can localize the sounds. E. Inability to turn head from side to side may indicate CN XI ­dysfunction. vvAnswer A

55 The strong irritant such as alcohol and ammonia will test the trigeminal nerve, instead of testing the olfactory nerve.

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17. CN examinations in infants. The FALSE answer is:

A. Caloric test can be used to test CN VIII. B. No need to check pupillary reflex if optic disc examined. C. CN V can be examined by checking facial sensation and muscles of mastication. D. Similar to neonates, gag reflex tests the function of CN IX, X. E. Hypoglossal nerve can be examined by assessment of the tongue. vvAnswer B

55 Examining pupillary reflex is important to check the function of both CN II, III. 18. Motor examination in infants. The FALSE answer is:

A. Tone can be examined by measuring the popliteal angle and scarf sign. B. Muscle atrophy indicates lower motor neuron lesions, and can be seen in both pyramidal and extrapyramidal lesions. C. Infants can usually sit unsupported at 7 months. D. Spasticity usually indicates extrapyramidal lesion, while rigidity indicates a pyramidal problem. E. Pincer grasp is a good indicator for the development of fine motor skill. vvAnswer D

55 Spasticity indicates pyramidal lesions, while rigidity is a sign for ­extrapyramidal lesions. 19. Infants’ neurological examination. The FALSE answer is:

A. Gait usually widens progressively with age. B. Cerebellar abnormalities can be detected by observation such as truncal swing. C. Abnormal gait and posture are good indicator for abnormal proprioception. D. Examination of the pain sensation should be done last. E. Withdrawing the limb from the stimulus is a clue to intact touch sensation. vvAnswer A

55 Gait usually narrows with age.

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20. Child neurological examination. The FALSE answer is:

3

A. Lesions in the Wernicke area will result in Receptive aphasia. B. Expressive aphasia is a result of a lesion in the inferior frontal gyrus. C. In conduction aphasia, the patient cannot name objects but can repeat words or phrases. D. Dysarthria can result from lesions affecting some lower CN. E. Abnormalities in arcuate fasciculus will cause conduction aphasia. vvAnswer C

55 In conduction aphasia child cannot repeat words or phrases but may be able to name objects. 21. Neurological examination in children. The FALSE answer is:

A. Children above 7 years can calculate, which indicates a non-dominant hemisphere function. B. Visual spatial function is a function of the non-­dominant hemisphere. C. The CN I can be tested at the age 6 years and above. D. CN IX, X can be assessed by the gag reflex. E. In upper motor neuron lesions of the CN XII, the tongue deviates toward the opposite side. vvAnswer A

55 Calculation is a function of the dominant hemisphere. 22. Child neurological examination. The FALSE answer is:

A. The power can be assessed by Medical Research Council system in older children. B. By examining different types of activities such as standing, walking, and turning, different parts can be examined simultaneously: central and peripheral. C. Inability to identify objects indicates parietal lobe dysfunction. D. Clonus indicates grade 4 hyper reflexia. E. Dysmetria is a good indicator for an extrapyramidal lesion. vvAnswer E

55 Dysmetria indicates cerebellar problems.

35 Neurological Examination

23. Regarding pediatric neurological examination. The FALSE answer is:

A. Abnormal asymmetric tonic neck reflex indicates abnormalities in both cerebral hemispheres and the brain-stem. B. Infant cannot sit with the persistence of the asymmetric tonic neck reflex. C. Child cannot walk if he did not develop a parachute reflex. D. Presence of ankle clonus in a neonate indicates a brain-stem lesion. E. Knee jerk mediated by L4 nerve root. vvAnswer D

55 Ankle clonus can be found in a normal newborn baby. 24. Regarding pediatric neurological examination. Causes of macrocephaly include. The FALSE answer is:

A. B. C. D. E.

Megalencephaly. Subdural hematoma. Hydrocephalus. Rickets. Hypothyroidism.

vvAnswer E

55 Hypothyroidism will cause microcephaly. 25. Regarding pediatrics examination. Causes of microcephaly include. The FALSE answer is:

A. B. C. D. E.

Craniosynostosis. Osteopetrosis. Toxoplasmosis. Anemia. Hypopituitarism.

vvAnswer B

55 Osteopetrosis will cause macrocephaly by causing a thick skull vault. 26. Regarding pediatric neurological examination. The FALSE answer is:

A. Truncal ataxia can be caused by a lesion in the cerebellar vermis. B. Tandem gait can be caused by cerebellar hemisphere abnormalities. C. Romberg sign is negative in sensory ataxia. D. Athetosis is more obvious during voluntary movement. E. Limbs, face, and axial muscles will be affected in chorea.

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vvAnswer C

55 Romberg sign is positive in sensory ataxia, which is usually due to spinal cord or peripheral nerve diseases.

3

27. Regarding the screening methods for developmental delay. The FALSE answer is:

A. Were created to facilitate and help identify children with developmental delays. B. Bayley infant neurodevelopmental screen and Denver-II are indirectly administered scales. C. Parents-based questionnaires better in identifying mild developmental delay. D. Screening tests cannot identify learning disabilities which will appear at school age. E. The Intelligence Quotient testing can predict attention, social skills, intelligence, and maturational level. vvAnswer B

55 Those two tests works as directly administered scales. 28. Regarding screening methods. The FALSE answer is:

A. The Wechsler intelligence scale of children III is the most commonly used for cognitive test. B. The Wechsler intelligence scale of children III is suitable between 6 and 16 years. C. Subtle developmental abnormalities are usually eliminated by normal screening. D. Developmental screen assesses the patient’s current developmental level. E. Screening tests may not be able to identify children with mild delay. vvAnswer C

55 Normal screen does not eliminate subtle developmental abnormalities.

Bibliography 1. Mukhopadhyay S, Puopolo KM. Risk assessment in neonatal early onset sepsis. Semin Perinatol. 2012;36(6):408–15. 2. Evans C.  History taking and the newborn examination: an evolving perspective. In: Examination of the newborn: an evidence-based guide. Wiley-Blackwell; 2011. p. 13–46.

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3. Mercuri E, Ricci D, Pane M, Baranello G. The neurological examination of the newborn baby. Early Hum Dev. 2005;81(12):947–56. https://doi.org/10.1016/j.earlhumdev.2005.10.007. Epub 2005 Nov 7. 4. Wusthoff CJ. How to use: the neonatal neurological examination. Arch Dis Child Educ Pract Ed. 2013;98:148–53. 5. Volpe JL. Neurological examination: normal and abnormal features. In: Volpe JL, Inder TE, Barra BT, et al., editors. Volpe’s neurology of the newborn. 6th ed. Philadelphia, PA: Elsevier; 2018. p. 191–205. 6. Fuloria M, Kreiter S.  The newborn examination: part I.  Emergencies and common abnormalities involving the skin, head, neck, chest, and respiratory and cardiovascular systems. Am Fam Physician. 2002;65(1):61–8. 7. Yoon SD, Cho BM, Oh SM, Park SH. Spontaneous resorption of calcified cephalhematoma in a 9-month-old child: case report. Childs Nerv Syst. 2013;29:517–9. 8. Mestre T, Lang AE.  The grasp reflex: a symptom in need of treatment. Mov Disord. 2010;25(15):2479–85. 9. Modrell AK, Tadi P. Primitive reflexes. In: StatPearls. StatPearls Publishing; 2022. 10. Kurtoğlu S, Hatipoğlu N, Mazıcıoğlu MM, Akın MA, Çoban D, Gökoğlu S, Baştuğ O.  Body weight, length and head circumference at birth in a cohort of Turkish newborns. J Clin Res Pediatr Endocrinol. 2012;4(3):132. 11. Wusthoff CJ. How to use: the neonatal neurological examination. Arch Dis Childhood Educ Pract. 2013;98(4):148–53. 12. Hawes J, Bernardo S, Wilson D.  The neonatal neurological examination: improving understanding and performance. Neonatal Netw. 2020;39(3):116–28. 13. Boban M, Brinar VV, Habek M, Rados M. Isolated hypoglossal nerve palsy: a diagnostic challenge. Eur Neurol. 2007;58(3):177. 14. Khan OA, Garcia-Sosa R, Hageman JR, et  al. Core concepts: neonatal neurological examination. Neoreviews. 2014;15:e316. 15. Khan OA, Garcia-Sosa R, Hageman JR, Msall M, Kelley KR. Core concepts: neonatal neurological examination. Neoreviews. 2014;15(8):e316–24. 16. Doty RL, Cometto-Muňiz JE. Trigeminal chemosensation. In: Handbook of olfaction and gustation. Marcel Dekker; 2003. p. 1673–704. 17. Swaiman KF. Neurological examination of the term and preterm infant. In: Swaiman KF, editor. Pediatric neurology principles & practice. 4th ed. Philadelphia, PA: Mosby Elsevier; 2006. p. 47–6. 18. Salandy S, Rai R, et al. Neurological examination of the infant: a comprehensive review. Clin Anat. 2019;32(6):770–7. 19. Ashby AT, Beier AD. Review of pediatric neurologic history and age-appropriate neurologic examination in the office. Pediatr Clin. 2021;68(4):707–14. 20. Ardila A. A review of conduction aphasia. Curr Neurol Neurosci Rep. 2010;10:499–503. 21. Vlaev I, Chater N, Stewart N, Brown GD. Does the brain calculate value? Trends Cogn Sci. 2011;15(11):546–54. 22. Manto M. Mechanisms of human cerebellar dysmetria: experimental evidence and current conceptual bases. J Neuroeng Rehabil. 2009;6:1–8. 23. Boyraz I, Uysal H, Koc B, Sarman H. Clonus: definition, mechanism, treatment. Med Glas (Zenica). 2015;12(1):19–26.

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24. Kurian MA, Jungbluth H. Genetic disorders of thyroid metabolism and brain development. Dev Med Child Neurol. 2014;56(7):627–34. https://doi.org/10.1111/dmcn.12445. Epub 2014 Mar 26. 25. Stark Z, Savarirayan R.  Osteopetrosis. Orphanet J Rare Dis. 2009;4:5. https://doi. org/10.1186/1750-­1172-­4-­5. 26. Forbes J, Cronovich H. Romberg test. In: StatPearls. StatPearls Publishing; 2022. 27. Aly Z, Taj F, Ibrahim S. Missed opportunities in surveillance and screening systems to detect developmental delay: a developing country perspective. Brain Dev. 2010;32(2):90–7.

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Hydrocephalus Eleni Tsianaka, Ahmed Muthana, Fatimah O. Ahmed, and Samer S. Hoz

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 S. S. Hoz et al. (eds.), Pediatric Neurosurgery, https://doi. org/10.1007/978-3-031-49573-1_4

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1. Hydrocephalus definition. The FALSE answer is:

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A. Hydrocephalus is an active process. B. The term “hydrocephalus” does not include CSF increase in spaces outside the ventricles. C. The term “hydrocephalus” excludes pathological conditions that do not involve the ventricles. D. Benign familial macrocephaly is not a case of hydrocephalus. E. Hydrocephalus is always related to overproduction of CSF. vvAnswer E

55 Hydrocephalus is “an active distension of the ventricular system of the brain related to the inadequate passage of CSF from its point of production within the ventricular system to its point of absorption into the systemic circulation”. 2. Hydrocephalus-related functional anatomy. The FALSE answer is:

A. Most of CSF is produced by the choroid plexus. B. Arachnoid villi are absent before birth. C. CSF is absorbed mainly by cranial arachnoid granulations. D. Spinal arachnoid granulations do not have any role in CSF absorption. E. Arachnoid granulations are piriform structures. vvAnswer D

55 Spinal arachnoid granulations are structures which absorb CSF. 3. Infantile hydrocephalus etiology. The FALSE answer is:

A. Secondary hydrocephalus can be a complication of hemorrhage, infection or neoplasm. B. Intrauterine infections can not cause hydrocephalus. C. Hemorrhage is the most common cause. D. About 1/3 of cases are related to aqueductal stenosis. E. Is associated with more than 100 different syndromes. vvAnswer B

55 Intrauterine infections (e.g. enterovirus, lymphocytic choriomeningitis and toxoplasmosis) can cause hydrocephalus.

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4. Newborn examination in hydrocephalus. The FALSE answer is:

A. Retinopathy can be related to Dandy-Walker malformation. B. Thumb flexion deformity can be related to X-­linked aqueductal stenosis. C. Rate of head growth is an important factor in assessing the severity of hydrocephalus. D. The size of the ventricles can be determined by ultrasound. E. Brain Magnetic Resonance Imaging (MRI) is important for ­identification of any related anomalies. vvAnswer A

55 Retinopathy is not characteristic of Dandy-Walker malformation. It can be strongly related to an intrauterine infection or a retina and brain ­dysplastic process. 5. Infant clinical examination in hydrocephalus. The FALSE answer is:

A. Vomiting and weight loss can be present. B. Hydrocephalus can be presented with unilateral or bilateral sixth nerve palsy. C. Roving eye movements are associated with good visual outcomes. D. Patient can have motor developmental delay. E. Neck retraction can be a sign of an acute increase in Intracranial Pressure (ICP). vvAnswer C

55 Roving eye movements are associated with unfavorable visual outcome. 6. Diagnosis of Prenatal hydrocephalus. The FALSE answer is:

A. Atrial width is used to diagnose fetal ventriculomegaly. B. Atrial width is stabilized after the second trimester of pregnancy. C. Atrial width above 12 mm is diagnostic for ventriculomegaly. D. The degree of ventriculomegaly changes during pregnancy. E. Fetal ventriculomegaly is always diagnostic for prenatal ­hydrocephalus. vvAnswer E

55 Fetal ventriculomegaly increases suspicions of prenatal hydrocephalus but is not enough for a definitive diagnosis.

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7. Hydrocephalus in children older than 2 years of age. The FALSE answer is:

A. Can present with signs of increased ICP. B. Symptoms get aggressively worse during the day. C. Gait and mental disorders are the most common features. D. Abnormal hypothalamic function is a common feature. E. Motor disorder can evolve to gross ataxia and severe spasticity.

4

vvAnswer B

55 Symptoms are worse during early morning hours, because of the hypoventilation and the lying position of the body during sleeping. 8. Growth of children with hydrocephalus. The FALSE answer is:

A. Premature birth increases the possibilities of short stature. B. Shunt malfunction can reduce the growth hormone secretion. C. Shunt malfunction can cause sleep disorders. D. Hydrocephalus can cause Gonadotropin-­independent precocious puberty. E. Gonadotropin-releasing hormone analogs are indicated for the treatment of central precocious puberty. vvAnswer D

55 Hydrocephalus can cause Gonadotropin-dependent (central) precocious puberty due to early maturation of the hypothalamic-pituitary-gonadal axis. 9. Imaging of hydrocephalus in children. The FALSE answer is:

A. May be manifested with compression of sulci. B. Compression of the basal cisterns can be seen. C. The floor of the third ventricle can be upwards displaced. D. Corpus callosum can be upwards displaced. E. Periventricular interstitial edema can be a finding of hydrocephalus. vvAnswer C

55 The floor of the third ventricle can be downwards displaced. 10. Hydrocephalus in Chiari malformation. The FALSE answer is:

A. Most of Chiari I cases present with hydrocephalus. B. Hydrocephalus must be treated before Chiari malformation. C. In most of the cases shunt needs revision within the first 10 years of its placement.

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D. Endoscopic third ventriculostomy (ETV) is not the preferable option for hydrocephalus in Chiari II cases. E. Properly treated hydrocephalus can improve the scoliosis in patients with Chiari II and myelomeningocele. vvAnswer A

55 Most of Chiari I cases do not present with hydrocephalus, even those with syringomyelia. The vast majority of Chiari II cases are associated with hydrocephalus. 11. Postnatal management of prenatal hydrocephalus. The FALSE answer is:

A. Body weight and maturation of the pulmonary system are basic criteria for the management decisions. B. Body weight more than 2500 g is indicative for ventriculo-subgaleal shunt. C. CSF reservoir can be an option, when body weight is less than 2500 g. D. Prenatal hydrocephalus due to cystic lesion is indication for endoscopic fenestration. E. Hydranencephaly is an indication for choroid plexus coagulation. vvAnswer B

55 Ventriculoperitoneal (VP) shunt can be placed safely and is preferable when the body weight is at least 2500 g. 12. VP shunt surgical technique. The FALSE answer is:

A. The access to the frontal horn is achieved by Kocher’s point. B. The occipital horn can be reached by either Frazier’s or Dandy’s point. C. The lateral ventricle can be reached by Keen’s point. D. Localization of Frazier’s point is up to 2 cm lateral to the midline and 6 cm above the inion. E. Ultrasound can be useful for insertion of the ventricular catheter, in children with open fontanels. vvAnswer D

55 Frazier’s burr hole point is localized 3–4  cm lateral to the midline and 6 cm above the inion.

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13. Ventricular extrathecal shunt surgical techniques. The FALSE answer is:

4

A. The distal part of the catheter is preferred to be placed into the ­peritoneum. B. Atrium, pleura and gallbladder can be used for the placement of the distal part of the catheter. C. Lumbo-peritoneal shunt can be an option. D. Right internal jugular vein is the best option for ventriculo-atrial shunt. E. During VP shunt placement, the distal part of the catheter should be fixed to the peritoneal wall. vvAnswer E

55 During VP shunt shunt placement, the distal part of the catheter should not be fixed to the peritoneal wall to avoid catheter related complications and disfunction. 14. Acute shunt failure imaging. The FALSE answer is:

A. Comparison with prior imaging is necessary to determine the change. B. Is always manifested with acute ventricular enlargement. C. Transependymal flow of CSF can be seen. D. Subgaleal fluid collection can be seen. E. Intraparenchymal edema can be a finding of acute shunt failure. vvAnswer B

55 Acute shunt failure can be manifested with acute ventricular enlargement, but this is not absolutely necessary. In this case secondary imaging findings (transependymal flow of CSF, subgaleal fluid collection, intraparenchymal edema) can be useful for the diagnosis. 15. Complications of CSF shunt. The FALSE answer is:

A. Improper shunt placement, tension pneumocephalus and intracranial hemorrhage can be early complications. B. Migration of the distal part of the shunt into the bowel can cause intracranial abscess. C. Infections, trapped ventricles and subdural hygroma can be possible complications. D. CSF shunt obstruction can lead to increased ICP. E. Scar tissue around the catheter is necessary for securing catheter’s position.

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vvAnswer E

55 Scar tissue can tether the catheter causing tension and shunt disconnection as child grows. 16. CSF shunt complications. The FALSE answer is:

A. Migration of the catheter into the brain parenchyma can be a complication. B. Over-drainage can cause slit ventricle syndrome. C. Siphoning is the result of catheter migration into a solid organ. D. Migration of the catheter into the colon can lead to anal extrusion. E. Patent processus vaginalis is a pathway through which the catheter can migrate into scrotum. vvAnswer C

55 Siphoning is an effect resulting from postural changes. Upright position can lead to temporarily decreased intraventricular pressure causing increased CSF shunt flow. 17. Aqueductal stenosis. The FALSE answer is:

A. In the majority of patients, the etiology is not known. B. Bacterial meningitis can be a cause of aqueductal stenosis. C. A mutation in the L1-CAM gene is responsible for the most common genetic form of congenital hydrocephalus. D. Bickers-Adams-Edwards syndrome suggests an autosomal congenital hydrocephalus condition. E. Aneurysms of the vein of Galen can cause aqueductal stenosis. vvAnswer D

55 Bickers-Adams-Edwards syndrome suggests an X-linked congenital hydrocephalus condition. 18. Aqueductal stenosis related hydrocephalus. The FALSE answer is:

A. Endoscopic aqueductoplasty is the treatment of choice. B. Low pre-operative Intelligence Quotient assessment indicates poor mental neurodevelopment. C. ETV is assessed post-operatively by phase contrast MRI. D. Parinaud syndrome can be a complication of aqueductoplasty. E. X-linked congenital hydrocephalus has the worse Intelligence Quotient outcome result.

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vvAnswer A

55 ETV is preferable. Endoscopic aqueductoplasty is more risky (increased risk of neurologic deficit) and is reserved only for selected cases (membranous occlusion or short stenosis of the aqueduct). 19. ETV. The FALSE answer is:

4

A. ETV is indicated in cases of purely obstructive hydrocephalus. B. In neonates the access can be achieved through bregmatic fontanel. C. After entering the ventricle, identification of the anatomical structures is mandatory. D. The floor of the third ventricle is identified ­between the mammillary bodies and the infundibular recess. E. The perforation of the floor of the third ventricle should be performed behind the mammillary bodies. vvAnswer E

55 The perforation of the floor of the third ventricle should be performed at the anterior midpoint of the anatomical triangle formed by the mammillary bodies and the infundibular recess. Perforation of the floor behind the mammillary bodies can lead to vessels injury (e.g. basilar artery). 20. ETV intraoperative complications. The FALSE answer is:

A. Bradycardia can occur during manipulation of the third ventricle. B. Bradycardia usually resolves by removing the endoscope. C. The first reaction after hemorrhage due to breached ependyma, is irrigation. D. Any injury to the floor of the third ventricle can result to ipsilateral fornix injury. E. Diabetes insipidus, hyponatremia and hypokalemia can be complications due to hypothalamic iatrogenic injury. vvAnswer D

55 Any injury to the floor of the third ventricle can result to injury of hypothalamus, as it is an anatomical component of the floor. Ipsilateral fornix injury can occur when the endoscope enters from the third ventricle through the lateral ventricle.

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Bibliography 1. Rekate HL.  A contemporary definition and classification of hydrocephalus. Semin Pediatr Neurol. 2009;16(1):9–15. 2. Dinçer A, Özek MM.  Radiologic evaluation of pediatric hydrocephalus. Childs Nerv Syst. 2011;27:1543–62. 3. Dewan MC, Rattani A, Mekary R, Glancz LJ, Yunusa I, Baticulon RE, Fieggen G, Wellons JC, Park KB, Warf BC. Global hydrocephalus epidemiology and incidence: systematic review and meta-analysis. J Neurosurg. 2018;130(4):1065–79. 4. Zakaria Z, Van Rostenberghe H, Ramli N, Suhaimi MS, Hazlan SNH, Abdullah JM. The key aspects of neonatal and infant neurological examination: the Ballard score, the infant’s head with hydrocephalus and assessment in a clinical setting. Malays J Med Sci. 2023;30(4):193–206. https://doi.org/10.21315/mjms2023.30.4.16. Epub 2023 Aug 24. 5. Kozlova EM, Remizova NV, Khaletskaia OV. [Hydrocephaly in newborns with perinatal brain damage of moderate severity]. Zh Nevrol Psikhiatr Im S S Korsakova. 2009;109(4):9–12. Russian. 6. Yamasaki M, Nonaka M, Bamba Y, Teramoto C, Ban C, Pooh RK. Diagnosis, treatment, and long-term outcomes of fetal hydrocephalus. Semin Fetal Neonatal Med. 2012;17(6):330–5. 7. Kahle KT, Kulkarni AV, Limbrick DD, Warf BC. Hydrocephalus in children. Lancet. 2016;387(10020):788–99. 8. Hochhaus F, Butenandt O, Schwarz HP, Ring-Mrozik E. Auxological and endocrinological evaluation of children with hydrocephalus and/or meningomyelocele. Eur J Pediatr. 1997;156:597–601. 9. Scheel M, Diekhoff T, Sprung C, Hoffmann KT.  Diffusion tensor imaging in hydrocephalus—findings before and after shunt surgery. Acta Neurochir. 2012;154:1699–706. 10. Coll G, El Ouadih Y, Rabbo FA, Jecko V, Sakka L, Di Rocco F.  Hydrocephalus and Chiari malformation pathophysiology in FGFR2-related faciocraniosynostosis: a review. Neurochirurgie. 2019;65(5):264–8. 11. Spennato P, Saccone G, Fratta A, Scala MR, Sarno L, Gragnano E, Zullo F, Cinalli G. Prenatal diagnosis and postnatal management of congenital unilateral hydrocephalus for stenosis of the foramen of Monro. Radiol Case Rep. 2021;16(9):2530–3. https://doi. org/10.1016/j.radcr.2021.06.011. Erratum in: Radiol Case Rep. 2023 Feb 9;18(4):1652. Erratum in: Radiol Case Rep. 2023 Jan 24;18(4):1645–1646. 12. Pillai SV. Techniques and nuances in ventriculoperitoneal shunt surgery. Neurol India. 2021;69(Suppl):S471–5. https://doi.org/10.4103/0028-­3886.332261. 13. Tamburrini G, Frassanito P, Iakovaki K, Pignotti F, Rendeli C, Murolo D, Di Rocco C.  Myelomeningocele: the management of the associated hydrocephalus. Childs Nerv Syst. 2013;29:1569–79. 14. Hasanain AA, Abdullah A, Alsawy MF, Soliman MA, Ghaleb AA, Elwy R, Ezzat AA, Al Menabbawy A, Marei AA, Abd el Razik B, El Hamaky MI. Incidence of and causes for ventriculoperitoneal shunt failure in children younger than 2 years: a systematic review. J Neurol Surg A Cent Eur Neurosurg. 2019;80(1):26–33. 15. Hanak BW, Bonow RH, Harris CA, Browd SR. Cerebrospinal fluid shunting complications in children. Pediatr Neurosurg. 2017;52(6):381–400. 16. Di Rocco C, Turgut M, Jallo G, Martínez-Lage JF, editors. Complications of CSF shunting in hydrocephalus: prevention, identification, and management. Springer; 2014.

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17. Cinalli G, Spennato P, Nastro A, Aliberti F, Trischitta V, Ruggiero C, Mirone G, Cianciulli E. Hydrocephalus in aqueductal stenosis. Childs Nerv Syst. 2011;27:1621–42. 18. Rodis I, Mahr CV, Fehrenbach MK, Meixensberger J, Merkenschlager A, Bernhard MK, Schob S, Thome U, Wachowiak R, Hirsch FW, Nestler U. Hydrocephalus in aqueductal stenosis—a retrospective outcome analysis and proposal of subtype classification. Childs Nerv Syst. 2016;32:617–27. 19. Yadav YR, Parihar V, Pande S, Namdev H, Agarwal M. Endoscopic third ventriculostomy. J Neurosci Rural Pract. 2012;3(2):163–73. 20. Bouras T, Sgouros S.  Complications of endoscopic third ventriculostomy: a review. J Neurosurg Pediatr. 2011;7(6):643–9.

49

CSF Diversion Oday Atallah, Laith T. Al-Ameri, Zinah A. Al-Araji, Zainab A. Alaraji, Huda Abdulrazaq, and Samer S. Hoz

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 S. S. Hoz et al. (eds.), Pediatric Neurosurgery, https://doi. org/10.1007/978-3-031-49573-1_5

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1. VP shunt complications. The FALSE answer is:

A. Shunt failure is most common in the first 6 months after insertion. B. The most frequent condition is infection. C. A late complication is a pseudocyst. D. Overshunting for low-pressure hydrocephalus can cause a subdural hematoma. E. Perforation of the bowel is an unusual complication. vvAnswer B

5

55 The most frequent complication is obstruction, followed by infection. 2. Infection as a complication of VP shunt. The FALSE answer is:

A. Antibiotic-impregnated shunt catheters significantly decrease the risk of infection. B. The most common cause is contamination of the shunt system during the surgical procedure. C. Most of these infections are caused by Staphylococcus aureus. D. Previous infection is a risk factor. E. Double gloving during surgery is associated with lower infection rates. vvAnswer C

55 The most common organism is coagulase-negative staphylococci. 3. Clinical predictors of VP shunt block in pediatric age group include. The FALSE answer is:

A. Drowsiness. B. Altered mental status. C. Nausea and vomiting. D. Fever. E. Increased head circumference. vvAnswer D

55 Fever is less commonly reported as a predictor of VP shunt block, but it may indicate an alternative diagnosis. 4. Regarding VP shunt obstruction. The FALSE answer is:

A. VP shunt obstruction is the most typical complication. B. The most common location is the distal catheter. C. High red blood cells levels in the CSF have no relation to ­obstruction.

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D. The programmable valve only reduces the risk of proximal catheter obstruction. E. Proximal catheter placement in the anterior horn of the lateral ­ventricle is linked to a decreased rate of malfunction. vvAnswer B

55 The proximal catheter is the most typical obstruction location. 5. Rare complications of VP shunt. The FALSE answer is:

A. Bowel perforation. B. Calcification of shunt. C. Distal catheter migration into scrotum. D. Abdominal pseudocyst. E. Proximal catheter clogged with brain parenchyma. vvAnswer E

55 The proximal catheter block is the most typical site for obstruction, ­making obstruction the most frequent complication. 6. The positioning of the ventricular catheter tip is associated with a higher incidence of irreversible shunt malfunction. The FALSE answer is:

A. Septum pellucidum. B. Contacting the ipsilateral ventricle wall. C. In the third ventricle. D. Contacting the contralateral ventricular wall. E. Superior to the foramen of Monro. vvAnswer E

55 Locations of the ventricular catheter tip above the foramen of Monro and in the body of the lateral ventricle are associated with a very low incidence of shunt malfunctions. 7. Overdrainage in VP shunt. The FALSE answer is:

A. One such complication is a subdural hematoma. B. The risk of slit ventricle syndrome rises in cases of acute overdrainage. C. It is possibly linked to the siphoning effect of the distal catheter. D. The risk of overdrainage may be diminished by the use of an ­antisiphon valve device. E. A lumboperitoneal shunt reduces failure in patients with slit ­ventricle syndrome.

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vvAnswer B

55 Slit-ventricle syndrome is believed to result from chronic overdrainage rather than acute overdrainage. 8. Factors that shorten the shunt survival time include. The FALSE answer is:

A. Individuals who previously required an external drainage system. B. Patients undergoing tumor removal surgery. C. Type of hydrocephalus. D. Hydrocephalus following cranial surgery. E. Age greater than 50 years.

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vvAnswer C

55 There is no correlation between hydrocephalus subtype and shunt failure rate. 9. Risk factors for intracerebral hemorrhage associated with external ventricular drainage. The FALSE answer is:

A. Thrombocytopenia. B. INR greater than 1.4. C. Post-operative use of antiplatelet in first 24 h. D. Antithrombotic use prior to implantation. E. Pediatric age group. vvAnswer E

55 A higher risk was found in people over the age of 50 than in younger children. 10. The following preoperative predictors are associated with poor outcome for VP shunt insertion in human immunodeficiency virus. The FALSE answer is:

A. Presence of immunosuppression. B. CN palsies. C. Low preoperative Glasgow Coma Scale (GCS). D. Hypernatremia. E. Anemia. vvAnswer D

55 Severe hyponatremia is associated with poor outcome.

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11. Lumboperitoneal shunt may be indicated in. The FALSE answer is:

A. Normal pressure hydrocephalus. B. Non-communicating hydrocephalus. C. Idiopathic intracranial hypertension. D. Spinal CSF leaks. E. Pseudomeningoceles. vvAnswer B

55 In contrast to the management of communicating hydrocephalus, a lumboperitoneal shunt is ineffective in the treatment of non-­communicating hydrocephalus. 12. Common complications of lumboperitoneal shunt include. The FALSE answer is:

A. Acquired Chiari malformation. B. CSF leaks. C. Overdrainage D. Infection. E. Intracerebral hematoma. vvAnswer E

55 Intracerebral hematoma is a rare complication following lumboperitoneal shunt. 13. Risk factors for developing post-operative hydrocephalus in pediatric age group. The FALSE answer is:

A. B. C. D. E.

Medulloblastoma. Ependymoma. Brainstem compression. Astrocytoma. Age ≤2 years.

vvAnswer D

55 Astrocytoma is a negative predictor for the development of ­hydrocephalus. 14. ETV is indicated in. The FALSE answer is:

A. Normal-pressure hydrocephalus. B. Simplification of septated hydrocephalus.

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C. Colloid cyst removal. D. Hydrocephalus secondary to pineal tumors. E. Prematurity. vvAnswer E

55 Prematurity is considered as a contraindication in ETV. 15. Contraindications for ETV. The FALSE answer is:

5

A. Previous radiotherapy. B. Distorted ventricular anatomy. C. Dandy–Walker malformation. D. Intraventricular hemorrhage. E. Meningeal infection. vvAnswer C

55 It is indicated in hydrocephalus secondary to congenital aqueductal stenosis, posterior third ventricle tumor, or Dandy–Walker malformation. 16. Surgical technique of ETV. The FALSE answer is:

A. The confluence of the thalamo-striate vein, septal vein, and choroid plexuses is a landmark of the foramen of Monro. B. In order to perforate the floor of the third ventricle, an incision is made between the mammillary bodies and the infundibular recess. C. Fenestration should be made immediately posterior to the dorsum sellae. D. The sharp perforation of the floor of the third ventricle is preferred. E. The waterjet dissection technique is useful for thick, opaque floors. vvAnswer D

55 A basilar artery injury could result from a sharp perforation. In order to reduce the risk of vascular injury, blunt perforation is preferred. 17. Medulloblastoma in children. The FALSE answer is:

A. Obstructive hydrocephalus is a common presentation feature. B. Almost all patients will require a permanent shunt. C. Preoperative shunting may be beneficial for patients with moderate to severe preoperative hydrocephalus. D. An upward transtentorial herniation may complicate preoperative ventricular drainage. E. An external ventricular drain may be used preoperatively.

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vvAnswer B

55 Only a minority of patients with hydrocephalus will require a permanent shunt. 18. Ventriculoatrial shunt complications. The FALSE answer is:

A. The rate of shunt obstructions is higher than with VP shunt. B. Overdrainage. C. Autoimmune glomerulonephritis. D. Pulmonary embolism. E. Thrombosis of the superior vena cava. vvAnswer A

55 The ventriculoatrial shunt shows fewer obstructions than the VP shunt. 19. Temporary management of acute hydrocephalus includes. The FALSE answer is:

A. Extraventricular drainage. B. Repeated lumbar drainage. C. Ventriculo-gallbladder shunt. D. Ventriculosubgaleal shunt. E. Ommaya reservoir insertion. vvAnswer C

55 If a VP shunt cannot be performed, a ventriculo-gallbladder shunt can be used as a permanent method of management. 20. Ommaya ventricular reservoir. The FALSE answer is:

A. Used for the temporary tapping of CSF. B. The third ventricle is the optimal location for the catheter. C. It is appropriate for premature infants with intraventricular hemorrhage. D. It carries a low risk of contamination. E. It reduces the possibility of future shunt revisions. vvAnswer B

55 The catheter should be placed in the lateral ventricle. 21. Ventriculo-subgaleal shunt. The FALSE answer is:

A. It’s a permanent CSF diversion method. B. It is recommended for preterm infants.

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C. The incision is made close to the anterior fontanelle. D. The distal end is located between the galea and the periosteum. E. No valve is used. vvAnswer A

55 In premature infants, a ventriculo-subgaleal shunt is a temporary shunt procedure used for repeated CSF tapping until a VP shunt can be performed.

5

22. Ventriculo-gallbladder shunt. The FALSE answer is:

A. Cholecystitis is a contraindication. B. It’s a permanent method of CSF diversion. C. Bile duct diseases are a contraindication. D. It’s the most common alternative to a VP shunt. E. Most bacterial complications are caused by Staphylococcus ­epidermidis. vvAnswer D

55 Rarely is a ventriculo-gallbladder shunt performed. The most frequent alternate is ventriculoatrial.

Bibliography 1. Paff M, Alexandru-Abrams D, Muhonen M, Loudon W.  Ventriculoperitoneal shunt complications: a review. Interdiscip Neurosurg. 2018;13:66–70. 2. Reddy GK, Bollam P, Caldito G.  Ventriculoperitoneal shunt surgery and the risk of shunt infection in patients with hydrocephalus: long-term single institution experience. World Neurosurg. 2012;78(1–2):155–63. 3. Barnes NP, Jones SJ, Hayward RD, Harkness WJ, Thompson D. Ventriculoperitoneal shunt block: what are the best predictive clinical indicators? Arch Dis Child. 2002;87(3):198–201. 4. Wu Y, Green NL, Wrensch MR, Zhao S, Gupta N. Ventriculoperitoneal shunt complications in California: 1990 to 2000. Neurosurgery. 2007;61(3):557–63. 5. Merkler AE, Ch’ang J, Parker WE, Murthy SB, Kamel H.  The rate of complications after ventriculoperitoneal shunt surgery. World Neurosurg. 2017;98:654–8. 6. Ghritlaharey RK, Budhwani KS, Shrivastava DK, Srivastava J.  Ventriculoperitoneal shunt complications needing shunt revision in children: a review of 5 years of experience with 48 revisions. Afr J Paediatr Surg. 2012;9(1):32–9. 7. Meier U, Stengel D, Müller C, Fritsch MJ, Kehler U, Langer N, Kiefer M, Eymann R, Schuhmann MU, Speil A, Weber F. Predictors of subsequent overdrainage and clinical outcomes after ventriculoperitoneal shunting for idiopathic normal pressure hydrocephalus. Neurosurgery. 2013;73(6):1054–60.

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8. Shah SS, Hall M, Slonim AD, Hornig GW, Berry JG, Sharma V. A multicenter study of factors influencing cerebrospinal fluid shunt survival in infants and children. Neurosurgery. 2008;62(5):1095–103. 9. Miller C, Tummala RP. Risk factors for hemorrhage associated with external ventricular drain placement and removal. J Neurosurg. 2017;126(1):289–97. 10. Liliang PC, Liang CL, Chang WN, Chen HJ, Su TM, Lu K, Lu CH. Shunt surgery for hydrocephalus complicating cryptococcal meningitis in human immunodeficiency virus-­ negative patients. Clin Infect Dis. 2003;37(5):673–8. 11. El-Saadany WF, Farhoud A, Zidan I. Lumboperitoneal shunt for idiopathic intracranial hypertension: patients’ selection and outcome. Neurosurg Rev. 2012;35:239–44. 12. Yang TH, Chang CS, Sung WW, Liu JT. Lumboperitoneal shunt: a new modified surgical technique and a comparison of the complications with ventriculoperitoneal shunt in a single center. Medicina. 2019;55(10):643. 13. Won SY, Dubinski D, Behmanesh B, Bernstock JD, Seifert V, Konczalla J, Tritt S, Senft C, Gessler F. Management of hydrocephalus after resection of posterior fossa lesions in pediatric and adult patients—predictors for development of hydrocephalus. Neurosurg Rev. 2020;43:1143–50. 14. Yadav YR, Parihar V, Pande S, Namdev H, Agarwal M. Endoscopic third ventriculostomy. J Neurosci Rural Pract. 2012;3(2):163–73. 15. De Bonis P, Tamburrini G, Mangiola A, Pompucci A, Mattogno PP, Porso M, Anile C.  Post-traumatic hydrocephalus is a contraindication for endoscopic third-­ ventriculostomy: isn’t it? Clin Neurol Neurosurg. 2013;115(1):9–12. 16. Yadav YR, Bajaj J, Ratre S, Yadav N, Parihar V, Swamy N, Kumar A, Hedaoo K, Sinha M. Endoscopic third Ventriculostomy-a review. Neurol India. 2021;69(8):502. 17. Northcott PA, Robinson GW, Kratz CP, Mabbott DJ, Pomeroy SL, Clifford SC, Rutkowski S, Ellison DW, Malkin D, Taylor MD, Gajjar A. Medulloblastoma. Nat Rev Dis Primers. 2019;5(1):11. 18. Hung AL, Vivas-Buitrago T, Adam A, Lu J, Robison J, Elder BD, Goodwin CR, Jusué-­ Torres I, Rigamonti D. Ventriculoatrial versus ventriculoperitoneal shunt complications in idiopathic normal pressure hydrocephalus. Clin Neurol Neurosurg. 2017;157:1–6. 19. Toma AK. Hydrocephalus. Surgery (Oxford). 2015;33(8):384–9. 20. Peretta P, Ragazzi P, Carlino CF, Gaglini P, Cinalli G. The role of Ommaya reservoir and endoscopic third ventriculostomy in the management of post-hemorrhagic hydrocephalus of prematurity. Childs Nerv Syst. 2007;23:765–71. 21. Iratwar S, Patil A, Rathod C, Korde P, Mundhe V, Deshpande H. Ventriculosubgaleal shunt in children with hydrocephalus. J Datta Meghe Inst Med Sci Univ. 2019;14(3):115–8. 22. Girotti ME, Singh RR, Rodgers BM.  The ventriculo-gallbladder shunt in the treatment of refractory hydrocephalus: a review of the current literature. Am Surg. 2009;75(8):734–7.

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Craniosynostosis Fatima A. Fakhroo, Mariam H. Allehaibi, Fatimah O. Ahmed, and Abdullah H. Al Ramadan

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 S. S. Hoz et al. (eds.), Pediatric Neurosurgery, https://doi. org/10.1007/978-3-031-49573-1_6

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1. Craniosynostosis. The FALSE answer is:

A. It is the premature fusion of calvarial sutures. B. Skull growth is hampered along a direction that runs perpendicular to the closed suture. C. Most affected is the sagittal suture. D. The second most affected suture is the metopic synostosis. E. The pathology of primary importance in nonsyndromic craniosynostosis is the skull base. vvAnswer E

55 The pathology of primary importance in nonsyndromic craniosynostosis is cranial vault.

6

2. Craniosynostosis. The FALSE answer is:

A. The skull X-ray shows beaten copper calvaria. B. The skull X-ray has normal lucency in the center of the suture. C. CT brain may show thickening or ridging at the site of synostosis. D. CT brain may show expansion of frontal subarachnoid space. E. CT skull with three-dimensional reconstruction demonstrates the skull abnormality better. vvAnswer B

55 The skull X-ray lacks normal lucency in the center of the suture. 3. Sagittal craniosynostosis. The FALSE answer is:

A. The most common single-suture craniosynostosis. B. Sagittal suture will be a palpable ridge. C. Occipitofrontal circumference is far from normal, but the biparietal diameter is markedly increased. D. Dolichocephalic skull shape. E. Scaphocephalic skull shape. vvAnswer C

55 OFC remains close to normal, but the biparietal diameter is markedly reduced. 4. Craniosynostosis. Genetics in Pfeiffer’s syndrome. The FALSE answer is:

A. There are three types of Pfeiffer’s syndrome, Type I, II, and III. B. Type I Pfeiffer’s is mild entity. C. Type II Pfeiffer’s is severe entity.

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D. Type I Pfeiffer’s has autosomal dominant inheritance. E. Types II and III Pfeiffer’s have autosomal dominant inheritance. vvAnswer E

55 Types II and III have sporadic inheritance. 5. Craniosynostosis. The FALSE answer is:

A. Muenke syndrome—Fibroblast growth factor receptor (FGFR) 3. B. Crouzen syndrome—FGFR2. C. Apert syndrome—FGFR2. D. Jackson-Weiss syndrome—FGFR2. E. Saethre-Chotzen syndrome—FGFR1. vvAnswer E

55 Saethre-Chotzen syndrome—TWIST1. 6. Metopic synostosis. The FALSE answer is:

A. Ridging of the fused suture. B. Temporal narrowing. C. Eye hypertelorism. D. Compensatory bulging of parieto-occipital region. E. Posterior displacement of the superolateral orbital rim. vvAnswer C

55 Eye hypotelorism. 7. Craniosynostosis. Treatment. The FALSE answer is:

A. Surgical indications are to prevent deformity and increased ICP. B. Children with single-suture craniosynostosis will not present with increased ICP. C. Neurocognitive functions improve with surgery. D. Children older than 6 months can tolerate blood loss better. E. Endoscopic procedures for sagittal craniosynostosis are performed between ages 1 and 4 months. vvAnswer B

55 Up to 30% of children with single-suture craniosynostosis will present with increased ICP.

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8. Craniosynostosis. Treatment. The FALSE answer is:

A. Strip craniectomy allows growth of the skull by removing the closed suture. B. Sagittal synostectomy extends from anterior fontanelle to posterior fontanelle. C. In metopic craniosynostosis fronto-orbital advancement is performed in most centers. D. In coronal craniosynostosis fronto-orbital advancement is performed in most centers. E. In lambdoid craniosynostosis fronto-orbital advancement is performed in most centers.

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vvAnswer E

55 In metopic and coronal craniosynostosis fronto-orbital advancement is performed in most centers. In lambdoid craniosynostosis an occipital craniotomy is performed with correction of the posterior plagiocephaly. 9. Craniosynostosis. Unilateral coronal craniosynostosis versus deformational plagiocephaly. The FALSE answer is:

A. Posterior deformation more common than anterior. B. Ipsilateral frontal bone is flat in posterior deformational plagiocephaly. C. Ipsilateral ear displaced anterosuperiorly in unilateral coronal craniosynostosis, posterior deformational plagiocephaly, and posteriorly in anterior deformational plagiocephaly. D. Head shape parallelogram in posterior deformational plagiocephaly and trapezoid in synostotic one. E. Anterior fontanel contralateral deviated in unilateral coronal craniosynostosis and normal in deformational one. vvAnswer B

55 Ipsilateral frontal bone bossing in posterior deformational plagiocephaly. 10. Sagittal craniosynostosis. The FALSE answer is:

A. Bitemporal narrowing. B. Ridging of fused sagittal suture. C. Biparietal narrowing. D. Frontal and occipital bossing. E. Premature fusion of the anterior third is the most common subtype.

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vvAnswer E

55 Sphenocephaly is the most common subtype: premature fusion of middle and posterior middle thirds. 11. Craniosynostosis. The FALSE answer is:

A. Scaphocephaly (dolichocephaly) > sagittal. B. Frontal plagiocephaly > unilateral coronal. C. Trigonocephaly > metopic. D. Posterior plagiocephaly > unilateral lambdoid. E. Turribrachycephaly > bilateral lambdoid. vvAnswer E

55 Turribrachycephaly bilateral coronal. 12. Craniosynostosis. Calvarial embryology and suture biology. The FALSE answer is:

A. The skull of the embryo starts developing between days 26 and 29. B. Divided into neurocranium and viscerocranium. C. The neurocranium surrounds the brain and develops from the surrounding mesenchyme. D. Viscerocranium derives from the first three brachial arches and forms the facial skeleton. E. The neurocranium can be subdivided into cranial base and cranial vault. vvAnswer A

55 The skull of the embryo starts developing between days 23 and 26 of gestation. 13. Craniosynostosis. Calvarial embryology and suture biology. The FALSE answer is:

A. Cranial base is chondrocranium and originates from paraxial mesoderm and undergoes endochondral ossification. B. Cranial vault is the membranous cranium. C. Membranous bones of the skull (parietal, frontal, and squamous portions of temporal bone), their intervening sutures, and underlying dura mater are from cephalic neural crest cells. D. Ectomenix ossifies in a fibrous membrane through an endochondral ossification pattern. E. Centers of ossification begin to form in a specific area of ectomenix after the second month of gestation.

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vvAnswer D

55 Ectomenix ossifies in a fibrous membrane through an intramembranous ossification pattern. 14. Craniosynostosis. Calvarial embryology and suture biology. The FALSE answer is:

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A. Ectomenix also develops into an outer periosteum and inner dura. B. Dura mater appears between days 51 and 53 of gestation. C. The site of suture formation corresponds to the location of major dural reflections. D. Dural reflections are bands of dural attachment to the skull base that conform to the early recesses of the brain. E. Reflections ossify intracranially. vvAnswer E

55 Reflections do not ossify intracranially. 15. Craniosynostosis. Calvarial embryology and suture biology. The FALSE answer is:

A. FGFRs are effective by activating tyrosine kinase pathway. B. FGFR-1 promotes osteoblast differentiation in the cranial suture. C. FGFR-2 promotes proliferation. D. FGFR-3 is found in cartilaginous tissues and acts as an inhibitor of proliferation during chondrogenesis. E. FGFR-4 is expressed in craniofacial sutures and brain tissue. vvAnswer E

55 FGFR-4 is not expressed in craniofacial but is expressed in brain tissue. 16. Craniosynostosis. Visual problems. The FALSE answer is:

A. Abnormal ocular movements and asymmetric visual field are more frequent in unilateral coronal craniosynostosis than other singlesuture synostosis. B. Strabismus is almost exclusive to metopic synostosis children. C. The fix-and-follow test gives abnormal responses in 67% of metopic synostosis. D. Hypofunction of the superior oblique muscle is quite common in unilateral coronal craniosynostosis. E. The abnormal oculomotor movement usually disappears after surgery.

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vvAnswer B

55 Strabismus is almost exclusive to unilateral coronal craniosynostosis children, 75%. 17. Craniosynostosis. Metopic synostosis. The FALSE answer is:

A. The metopic suture is located between the two frontal bones. B. The metopic suture is the first suture to close. C. The metopic suture can close as early as 6 months of age. D. The metopic suture typically closes at the age of 9 months. E. Metopic synostosis is characterized by a trigonocephalic head shape with a frontal keel and a narrowed bitemporal width. vvAnswer C

55 The metopic suture can close as early as 3 months of age. 18. Craniosynostosis. Metopic synostosis. The FALSE answer is:

A. Mental retardation and cognitive impairment higher than other simple craniosynostosis. B. Children with metopic synostosis are not at increased risk of developing astigmatism. C. An incidence rate of 30% for Chiari 1. D. The severity of trigonocephaly is a factor that determines the need for surgical treatment. E. No treatment is necessary for mild suture ridging alone. vvAnswer B

55 Children with metopic synostosis are at increased risk of developing astigmatism. 19. Craniosynostosis. Coronal synostosis. The FALSE answer is:

A. Is the premature fusion of the coronal sutures between frontal and parietal bones. B. It represents 20–30% of all cases of craniosynostosis. C. Bilateral involvement is found as part of syndromic forms of craniosynostosis. D. Unilateral involvement is more common in nonsyndromic cases. E. It produces abnormal cranial vault shape without craniofacial deformity.

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vvAnswer E

55 Coronal suture synostosis produces a craniofacial deformity rather than an abnormal cranial vault shape alone. This is attributed to its contribution to a closed system of articulations linking the cranial vault to the skull base, known as the coronal ring. This closed ring may include the sphenofrontal, sphenoethmoid, and sphenozygomatic articulations. 20. Craniosynostosis. Muenke syndrome is due to FGFR3. The FALSE answer is:

6

A. Unicoronal or bicoronal craniosynostosis. B. Brachydactyly and thimble-like middle ­phalanges. C. Coned epiphyses, carpel, and tarsal fusion. D. Conductive hearing loss. E. Developmental delay. vvAnswer D

55 Sensorineural hearing loss not conductive hearing loss. 21. Craniosynostosis. Crouzon syndrome is due to FGFR2. The FALSE answer is:

A. Coronal synostosis, midfacial hypoplasia, and exophthalmos. B. Involvement of other calvarial sutures. C. Brachycephaly, hypertelorism. D. Hydrocephalus, mental retardation. E. Chiari II malformation. vvAnswer E

55 Chiari I malformation. 22. Craniosynostosis. Apert syndrome is due to FGFR2. The FALSE answer is:

A. Sagittal synostosis. B. Severe syndactyly in fingers and toes. C. Symphalangism. D. Radiohumeral fusion. E. Mental retardation. vvAnswer A

55 Apert syndrome causes coronal synostosis.

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23. Craniosynostosis. Saethre-Chotzen syndrome. The FALSE answer is:

A. Coronal craniosynostosis. B. Limb abnormalities. C. Facial abnormalities (facial asymmetry, low frontal hairline, ptosis, and small ears with prominent ear crura). D. Affected gene TWIST1. E. It is an autosomal recessive disease with complete penetrance and variable expressivity. vvAnswer E

55 It is an autosomal dominant disease with complete penetrance and variable expressivity. 24. Craniosynostosis. Bilateral coronal synostosis. The FALSE answer is:

A. Bilateral coronal synostosis may occur in isolation or as a part of a syndrome. B. Brachycephaly (short head) resulting from a decreased anteroposterior diameter with continuous growth superoinferiorly and mediolaterally. C. The occiput and forehead are flattened, the vertex of the skull is displaced anteriorly, and upper part of the face is widened. D. Teleorbitism or hypertelorism may add to the visible abnormalities. E. A unilateral harlequin deformity is evident in radiographs. vvAnswer E

55 A bilateral harlequin deformity is evident in radiographs. 25. Craniosynostosis. Sagittal synostosis. The FALSE answer is:

A. Sagittal suture separates the two parietal bones. B. Premature fusion of the sagittal suture is the most common type of nonsyndromic craniosynostosis and results in a scaphocephalic head shape. C. The skull is elongated anteroposteriorly, associated with frontal and/ or occipital bossing. D. Biparietal and bitemporal narrowing. E. There are significant abnormalities of the cranial base, orbits, or face with isolated sagittal synostosis. vvAnswer E

55 There are no significant abnormalities of the cranial base, orbits, or face with isolated sagittal synostosis.

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26. Craniosynostosis. Lambdoid synostosis. The FALSE answer is:

A. Isolated fusion of the lambdoid suture is rare. B. Lambdoid suture may be fused unilaterally or bilaterally. C. Bilateral lambdoid synostosis may be found in association with syndromic craniosynostosis. D. In true unilateral lambdoid synostosis the head has trapezoid shape. E. In deformational plagiocephaly the head also has trapezoid shape. vvAnswer E

55 In deformational plagiocephaly the head resembles parallelogram. 27. Craniosynostosis. Cloverleaf skull (kleeblattschädel). The FALSE answer is:

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A. Fusion of the coronal, posterior sagittal, and lambdoid sutures. B. Wide diastasis of the squamosal sutures. C. Type 3 Pfeiffer syndrome. D. Type 2 Pfeiffer syndrome. E. Surgical management is one of the most challenging for a craniofacial surgeon. vvAnswer C

55 It is described with Crouzon, Apert, Carpenter, Beare-Stevenson, and type 2 Pfeiffer syndrome. 28. Craniosynostosis. Positional plagiocephaly. The FALSE answer is:

A. Flattening of the side of sleep preference. B. Cannot be present at the time of birth. C. Usually accentuated with back sleeping during first 3 months. D. Ipsilateral ear is displaced anteriorly. E. Ipsilateral forehead is displaced anteriorly, and the degree of displacement is usually much less than posterior flattening. vvAnswer B

55 Can be present at the time of birth due to prenatal intrauterine conditions like oligohydramnios. 29. Craniosynostosis. Hydrocephalus. The FALSE answer is:

A. Active hydrocephalus has been reported in 12.1%–15%. B. Ventriculomegaly and asymmetry may be found in single-suture synostosis.

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C. In Apert syndrome most cases of enlarged ventricles need shunt. D. Ventricular dilatation is a common feature in complex one. E. Crouzon and sever Pfeiffer syndromes frequently need shunting. vvAnswer C

55 Ventricular dilatation is reported in 40–90% of Apert syndrome, and most cases remain stable without a shunt. 30. Craniosynostosis. Preoperative planning. The FALSE answer is:

A. CT skull with three-dimensional reconstruction. B. Ophthalmological assessment to rule out hydrocephalus. C. If there is a need for neck extension, cervical spine X-rays are needed to rule out craniocervical abnormality. D. MRI brain in all types of synostoses. E. Photographs taken pre- and postoperatively to serve as outcome assessments. vvAnswer D

55 MRI brain for cases of syndromic synostosis. 31. Craniosynostosis. ICP in craniosynostosis. The FALSE answer is:

A. Increased ICP is common in nonsyndromic craniosynostosis. B. The mechanism of ICP elevation in single-suture craniosynostosis is thought to be related to a form of CSF circulation alteration caused by distorted subarachnoid spaces. C. Increased ICP is an important factor in surgical decision. D. Increased ICP can affect intelligence and cause developmental delay. E. After surgical correction it was noticed that there is an improvement in learning abilities, behavioral improvement, and resolution of papilledema. vvAnswer A

55 Increased ICP is common in syndromic craniosynostosis. 32. Craniosynostosis. Acute complications. The FALSE answer is:

A. B. C. D. E.

Hemorrhagic shock. Coagulopathy. Dural tear. Air embolism. Bone gap.

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vvAnswer E

55 Bone gap is a delayed complication. 33. Craniosynostosis. Jackson-Weiss syndrome. The FALSE answer is:

A. Gene affected is FGFR2. B. Toes anomalies. C. Midface hypoplasia. D. Hypertelorism and proptosis. E. Mental retardation. vvAnswer E

55 Normal intelligence.

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34. Craniosynostosis. Surgical management in metopic synostosis. The FALSE answer is:

A. Bifrontal craniotomy with extension to the parietal area 3–4 cm. B. Bilateral orbital rim osteotomy performed using bowstring technique. C. Temporal myo-osseous flap has no rule in the procedure. D. Nasofrontal osteotomy with a wedge bone graft can be used to increase intercanthal distance. E. Reoperation is uncommon except for supplemental augmentation of the frontal area. vvAnswer C

55 Temporal myo-osseous flap is used to advance the muscle and the squamous temporal bone to more adequately correct the temporal narrowing. Fixation is typically achieved with long-acting resorbable suture/plates in the fronto-orbital area, depending on the degree of security needs of the reattachment. 35. Craniosynostosis. Endoscopic techniques. The FALSE answer is:

A. The goal is to reduce operative time, blood loss, the length of hospitalization, and consequently the cost. B. Strip craniectomies are performed through 2–3 cm skin incisions placed generally over the synostosed sutures. C. Patients will need helmets for 6–12 months. D. Some studies showed that strip craniectomies alone are as effective as comprehensive cranial vault remodeling techniques. E. Major differences are that endoscopic techniques have smaller scars and fewer osteotomies.

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vvAnswer D

55 Some studies showed that strip craniectomies alone are not as effective as comprehensive cranial vault remodeling techniques. 36. Secondary craniosynostosis. The FALSE answer is:

A. Can be due to metabolic, hematologic, or drug-­related disorders. B. The most common metabolic cause is hypophosphatemic rickets. C. Shunt-related craniosynostosis may develop several months to 2–3 years after shunt. D. Shunted hydrocephalus with features of hyperdrainage and slit ventricles are susceptible to craniosynostosis. E. Coronal suture is the most affected in metabolic craniosynostosis. vvAnswer E

55 Sagittal suture is most common.

Bibliography 1. Governale LS. Craniosynostosis. Pediatr Neurol. 2015;53(5):394–401. 2. Nagaraja S, Anslow P, Winter B. Craniosynostosis. Clin Radiol. 2013;68(3):284–92. 3. Massimi L, Caldarelli M, Tamburrini G, Paternoster G, Di Rocco C. Isolated sagittal craniosynostosis: definition, classification, and surgical indications. Childs Nerv Syst. 2012;28:1311–7. 4. Greig AV, Wagner J, Warren SM, Grayson B, McCarthy JG. Pfeiffer syndrome: analysis of a clinical series and development of a classification system. J Craniofac Surg. 2013;24(1):204–15. 5. Johnson D, Wilkie AO. Craniosynostosis. Eur J Hum Genet. 2011;19(4):369–76. 6. van der Meulen J. Metopic synostosis. Childs Nerv Syst. 2012;28(9):1359–67. 7. Arko L, Swanson JW, Fierst TM, Henn RE, Chang D, Storm PB, Bartlett SP, Taylor JA, Heuer GG.  Spring-mediated sagittal craniosynostosis treatment at the Children’s Hospital of Philadelphia: technical notes and literature review. Neurosurg Focus. 2015;38(5):E7. 8. Kajdic N, Spazzapan P, Velnar T. Craniosynostosis-recognition, clinical characteristics, and treatment. Bosn J Basic Med Sci. 2018;18(2):110. 9. Fotouhi AR, Chiang SN, Peterson AM, Doering MM, Skolnick GB, Naidoo SD, Strahle JM, McEvoy SD, Patel KB. Neurodevelopment in unilateral coronal craniosynostosis: a systematic review and meta-analysis. J Neurosurg Pediatr. 2022;31(1):16–23. 10. Lee BS, Hwang LS, Doumit GD, Wooley J, Papay FA, Luciano MG, Recinos VM. Management options of non-syndromic sagittal craniosynostosis. J Clin Neurosci. 2017;39:28–34. 11. Stanton E, Urata M, Chen JF, Chai Y. The clinical manifestations, molecular mechanisms and treatment of craniosynostosis. Dis Model Mech. 2022;15(4):dmm049390. https://doi.org/10.1242/dmm.049390. Epub 2022 Apr 22.

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12. De Coster PJ, Mortier G, Marks LA, Martens LC. Cranial suture biology and dental development: genetic and clinical perspectives. J Oral Pathol Med. 2007;36(8):447–55. 13. Beederman M, Farina EM, Reid RR.  Molecular basis of cranial suture biology and disease: osteoblastic and osteoclastic perspectives. Genes Dis. 2014;1(1):120–5. 14. Ishii M, Sun J, Ting MC, Maxson RE.  The development of the calvarial bones and sutures and the pathophysiology of craniosynostosis. Curr Top Dev Biol. 2015;115:131– 56. 15. Tubbs RS, Bosmia AN, Cohen-Gadol AA. The human calvaria: a review of embryology, anatomy, pathology, and molecular development. Childs Nerv Syst. 2012;28:23–31. 16. Samra F, Paliga JT, Tahiri Y, Whitaker LA, Bartlett SP, Forbes BJ, Taylor JA. The prevalence of strabismus in unilateral coronal synostosis. Childs Nerv Syst. 2015;31:589–96. 17. Kweldam CF, Van Der Vlugt JJ, Van Der Meulen JJ. The incidence of craniosynostosis in The Netherlands, 1997–2007. J Plast Reconstr Aesthet Surg. 2011;64(5):583–8. 18. Beckett JS, Chadha P, Persing JA, Steinbacher DM. Classification of trigonocephaly in metopic synostosis. Plast Reconstr Surg. 2012;130(3):442e–7e. 19. Lajeunie E, Merrer ML, Bonaïti-Pellie C, Marchac D, Renier D. Genetic study of nonsyndromic coronal craniosynostosis. Am J Med Genet. 1995;55(4):500–4. 20. Doherty ES, Lacbawan F, Hadley DW, Brewer C, Zalewski C, Kim HJ, Solomon B, Rosenbaum K, Domingo DL, Hart TC, Brooks BP. Muenke syndrome (FGFR3-related craniosynostosis): expansion of the phenotype and review of the literature. Am J Med Genet A. 2007;143(24):3204–15. 21. Al-Namnam NM, Hariri F, Thong MK, Rahman ZA. Crouzon syndrome: genetic and intervention review. J Oral Biol Craniofac Res. 2019;9(1):37–9. 22. Wenger TL, Hing AV, Evans KN. Apert Syndrome. 2019 May 30. In: Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2023. 23. Gallagher ER, Ratisoontorn C, Cunningham ML. Saethre-Chotzen Syndrome. 2003 May 16 [updated 2019 Jan 24]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2023. 24. Mulliken JB, Steinberger D, Kunze S, Müller U. Molecular diagnosis of bilateral coronal synostosis. Plast Reconstr Surg. 1999;104(6):1603–15. 25. Hunter AG, Rudd NL, Craniosynostosis I. Sagittal synostosis; its genetics and associated clinical findings in 214 patients who lacked involvement of the coronal suture(s). Teratology. 1976;14(2):185–93. 26. Al-Jabri T, Eccles S. Surgical correction for unilateral lambdoid synostosis: a systematic review. J Craniofac Surg. 2014;25(4):1266–72. 27. Jarrahy R, Kawamoto HK, Keagle J, Dickinson BP, Katchikian HV, Bradley JP. Three tenets for staged correction of Kleeblattschädel or cloverleaf skull deformity. Plast Reconstr Surg. 2009;123(1):310–8. 28. Cummings C, Canadian Paediatric Society, Community Paediatrics Committee. Positional plagiocephaly. Paediatr Child Health. 2011;16(8):493–4. 29. Frassanito P, Palombi D, Tamburrini G. Craniosynostosis and hydrocephalus: relevance and treatment modalities. Childs Nerv Syst. 2021;37:3465. 30. Coelho G, Rabelo NN, Vieira E, Mendes K, Zagatto G, de Oliveira RS, Raposo-Amaral CE, Yoshida M, de Souza MR, Fagundes CF, Teixeira MJ. Augmented reality and phys-

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31. 32. 33.

34. 35. 36.

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ical hybrid model simulation for preoperative planning of metopic craniosynostosis surgery. Neurosurg Focus. 2020;48(3):E19. Derderian C, Seaward J. Syndromic craniosynostosis. Semin Plast Surg. 2012;26(2):64– 75. Nguyen C, Hernandez-Boussard T, Khosla RK, Curtin CM. A national study on craniosynostosis surgical repair. Cleft Palate Craniofac J. 2013;50(5):555–60. Celie KB, Yuan M, Cunniff C, Bogue J, Hoffman C, Imahiyerobo T. Rapidly progressive multisutural craniosynostosis in a patient with Jackson-Weiss syndrome and a de novo FGFR2 pathogenic variant. Cleft Palate Craniofac J. 2019;56(10):1386–92. Hormozi AK, Shahverdiani R, Mohammadi HR, Zali A, Mofrad HR. Surgical treatment of metopic synostosis. J Craniofac Surg. 2011;22(1):261–5. Jimenez DF, Barone CM.  Endoscopic techniques for craniosynostosis. Atlas Oral Maxillofac Surg Clin North Am. 2010;18(2):93–107. Higashino T, Hirabayashi S.  A secondary craniosynostosis associated with juvenile hyperthyroidism. J Plast Reconstr Aesthet Surg. 2013;66(10):e284–6.

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Chiari Malformations Ahmed Adel Farag, Ahmed Abdelrahman Abdullah, Ali A. Dolachee, and Waeel O. Hamouda

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023 S. S. Hoz et al. (eds.), Pediatric Neurosurgery, https://doi. org/10.1007/978-3-031-49573-1_7

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1. In the history of Chiari malformations description. The FALSE answer is:

A. Dr. Nicholas Tulp (1641) was the first to describe hindbrain herniation in a myelodysplastic patient. B. Dr. Theodor Langhans (1881) was the first to observe and report the type II malformations. C. Dr. John Cleland (1883) reported a patient with hindbrain herniation and hydrocephalus. D. Dr. Hans Chiari (1891) attempted to delineate and classify posterior fossa abnormalities. E. Dr. Julius Arnold (1894) described hindbrain herniation with myelodysplasia without hydrocephalus. vvAnswer B

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55 In Über Höhlenbildung im Rückenmark in Folge Blutstauung written by the German physician and anatomist Theodor Langhans (1839–1915), reference is made for the first time to a case of hindbrain herniation described as “pyramidal tumors” in the absence of myelodysplasia (Chiari I malformation). 2. The types of Chiari malformations. The FALSE answer is:

A. Chiari 0: Spinal syrinx with cerebellar tonsil descent. B. Chiari I: More than 5 mm descent of cerebellar tonsil tip past foramen magnum line (McRae line). C. Chiari II: Medulla, fourth ventricle, and tonsil tip descent past foramen magnum with spina bifida. D. Chiari III: Cerebellar herniation through a suboccipital or a high cervical encephalocele. E. Chiari IV: Cerebellar hypoplasia with normal posterior fossa and no hindbrain herniation. vvAnswer A

55 Chiari 0 (zero) shows syrinx without tonsillar descent yet improves with posterior fossa decompression. 3. The atypical types of Chiari malformation can include. The FALSE answer is:

A. Chiari 0: Spinal syrinx without cerebellar tonsil descent. B. Chiari 0.5: Ventral tonsillar herniation. C. Chiari 1.5: Tonsillar herniation (as in Chiari I) with brainstem herniation through foramen magnum.

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D. Chiari V: Absence of the cerebellum with occipital lobe herniation through the foramen magnum. E. Chiari VI: Cerebellar hypoplasia with enlarged posterior fossa. vvAnswer E

55 There is no Chiari malformation type VI. 4. General considerations in the Chiari malformations. The FALSE answer is:

A. Hindbrain anomalies range from simple tonsil herniation to complete cerebellar agenesis. B. It is unlikely that a unifying pathophysiology underlies all these malformations. C. Treatment aims to restore normal posterior fossa volume. D. The focus of treatment is on symptomatic patients. E. There is great variability both clinically and radiologically for each type of Chiari. vvAnswer C

55 Treatment aims to restore normal CSF dynamics across the craniovertebral junction. 5. In the pathophysiology of the Chiari malformations. The FALSE answer is:

A. The Caudal traction theory due to cord tethering by Penfield and Coburn in 1938. B. The mesoderm deficiency causing hypoplasia of the posterior fossa by Di Rocco and Rende in 1989. C. The unified theory by McLone and Knepper in 1989. D. The CSF leak—undersized posterior cranial fossa theory by McLone and Naidich in 1992. E. Chiari types I and II share a common patho-­embryologic origin. vvAnswer E 6. In the pathophysiology of Chiari malformation type I, there is cephalocranial disproportion. The FALSE answer is:

A. Can occur in syndromic multi-suture craniosynostoses. B. Crouzon syndrome can directly reduce posterior fossa volume. C. Elevates ICP and promotes herniation of posterior fossa elements. D. Can occur with vitamin E deficiency. E. Can occur with growth hormone deficiency.

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vvAnswer D

55 Vitamin D-resistant rickets causes bony overgrowth of the posterior fossa, thus reducing its volume. 7. In the pathophysiology of Chiari malformation type I, there is ­craniosynostosis. The FALSE answer is:

A. Base deformity and venous hypertension are involved in Chiari ­malformation type I craniosynostosis. B. The pattern of closure of the skull base sutures determines the incidence of Chiari malformation type I. C. Extremely common in Apert but unusual in Crouzon syndromes. D. Is routinely found in Kleeblattschädel (Cloverleaf skull). E. Posterior cranial vault expansion might be the initial procedure for treatment.

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vvAnswer C

55 Extremely common in Crouzon syndrome (70%) and Pfeiffer syndrome (50%) but distinctly unusual in Apert syndrome ( 5 mitoses/10 HPF of 0.23). C. Unlike in gliomas, diffuse brain infiltration is uncommon. D. Corresponds to WHO grade 3. E. Forty percent harbor germline TP53 mutation.

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vvAnswer C

55 Choroid plexus carcinomas commonly infiltrate the brain parenchyma. 23. Angiocentric gliomas. The FALSE answer is:

20

A. Present with chronic intractable partial epilepsy and correspond to histologic WHO grade 1. B. Superficial cerebrocortical location. C. Angiocentric growth pattern with monomorphic bipolar tumor cells arranged around cortical vasculature. D. Likely arise from bipolar radial glia. E. Astrocytic differentiation on ultrastructure (microvilli, intermediate junctions) and immunoprofile (epithelial membrane antigen (EMA) dot-like reactivity). vvAnswer E

55 The microvilli and intermediate junctions on electron microscopy and EMA dot-like immunoreactivity are features of ependymal differentiation in angiocentric glioma. 24. Subependymal giant cell astrocytomas. The FALSE answer is:

A. Frequently associated with tuberous sclerosis. B. Arise from the lateral walls of the lateral ventricles. C. Correspond to histologic WHO grade 1. D. Well-circumscribed tumor comprised of ganglionic astrocytes. E. Ependymal differentiation on morphology, immunoprofile, and ultrastructure. vvAnswer E

55 Glial and neuronal differentiation on morphology, immunoprofile, and ultrastructure. 25. Pilomyxoid astrocytomas. The FALSE answer is:

A. The hypothalamic/chiasmatic region is most commonly involved. B. Characterized by monomorphous bipolar cells with angiocentric arrangement and myxoid background. C. Typically associated with Rosenthal fibers and eosinophilic granular bodies. D. More aggressive behavior than pilocytic astrocytoma. E. No WHO histologic grade is assigned.

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vvAnswer C

55 Strictly defined, pilomyxoid astrocytomas lack Rosenthal fibers and eosinophilic granular bodies. 26. Diffuse hemispheric glioma, H3 G34-mutant. The FALSE answer is:

A. Predilection toward adolescents and young adults (median age:15–19 years). B. Glioblastoma-like growth pattern, high cellularity, and brain infiltration with brisk mitotic activity. C. Microvascular proliferation and necrosis are essential diagnostic features. D. Olig2-negative, ATRX-negative, and p53-positive. E. Poor prognosis. vvAnswer C

55 Microvascular proliferation and necrosis are usually present but not essential diagnostic features. 27. Supratentorial ependymoma, ZFTA fusion-positive. The FALSE answer is:

A. They represent the majority of supratentorial ependymomas and may occur both in children and in adults. B. ZFTA partner gene is mainly RELA. C. Immunoreactivity for p65 (RELA) or L1CAM is desirable for the diagnosis. D. Poor outcome when compared with that of other supratentorial ependymal tumor types. E. Methylation profiling is the only diagnostic test for detecting ZFTA fusions. vvAnswer E

55 ZFTA fusions can be detected by sequencing methods, FISH, and methylation profiling. 28. CNS tumors with angiocentric/perivascular arrangement. The FALSE answer is:

A. B. C. D. E.

Pilomyxoid astrocytoma. Myxopapillary ependymoma. Angiocentric glioma. Ependymoma. Angiomatous meningioma.

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vvAnswer E

55 Angiomatous meningiomas have large, often hyalinized blood vessels and no angiocentric/perivascular arrangement of tumor cells. 29. CNS tumors with fibrous tissue (collagen). The FALSE answer is:

20

A. Gliosarcoma. B. Subependymal giant cell astrocytoma. C. Desmoplastic infantile astrocytoma. D. Pleomorphic xanthoastroctoma. E. Desmoplastic/nodular medulloblastoma. vvAnswer B

55 Subependymal giant cell astrocytomas do not contain fibrous tissue. 30. Metastatic tumors of the CNS in children. The FALSE answer is:

A. Brain metastases are rare in children (occur in 6–10% of children and 30% of adults with cancer). B. The most common metastases are leukemias and lymphomas. C. The most frequent origins of solid tumor metastases are kidney/adrenal and bone/soft tissue. D. Mostly supratentorial locations. E. Metastatic solid tumors rarely demonstrate synchronous and/or metachronous metastases in organs other than the brain. vvAnswer E

55 The majority of metastatic solid tumors (85%) demonstrate synchronous and/or metachronous metastases in organs other than the brain.

Bibliography 1. DeWitt JC, Mock A, Louis DN. The 2016 WHO classification of central nervous system tumors: what neurologists need to know. Curr Opin Neurol. 2017;30(6):643–9. 2. Komori T. The 2016 WHO classification of tumours of the central nervous system: the major points of revision. Neurol Medicochir. 2017;57(7):301–11. 3. Wang L, Li Z, Zhang M, Piao Y, Chen L, Liang H, Wei Y, Hu Z, Zhao L, Teng L, Lu D.  H3 K27M–mutant diffuse midline gliomas in different anatomical locations. Hum Pathol. 2018;78:89–96. 4. Fangusaro J. Pediatric high grade glioma: a review and update on tumor clinical characteristics and biology. Front Oncol. 2012;2:105.

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