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Principles and Practice of Pediatric Neurosugery3rd EditionA. Leland Albright, MDClinical ProfessorDepartment of NeurosurgerySchool of Medicine and Public HealthUniversity of Wisconsin–MadisonMadison, WisconsinConsultantPediatric NeurosurgeonKijabe HospitalKijabe, KenyaIan F. Pollack, MD, FAANS, FACS, FAAPChief, Pediatric NeurosurgeryChildren’s Hospital of PittsburghWalter Dandy Professor of Neurological Surgery, 2001–2013Leland Albright Professor of Neurological Surgery, 2013–presentVice Chairman for Academic AffairsDepartment of Neurological SurgeryCo-Director, UPCI Brain Tumor ProgramUniversity of Pittsburgh School of MedicineCo-Chair, National Cancer Institute Brain Malignancies Steering CommitteePittsburgh, PennsylvaniaP. David Adelson, MD, FACS, FAAPDirectorBarrow Neurological Institute at Phoenix Children’s HospitalDiane and Bruce Halle Endowed Chair for Pediatric NeurosciencesChief, Pediatric Neurosurgery/Children’s NeurosciencesClinical ProfessorUniversity of Arizona College of Medicine–PhoenixAdjunct ProfessorSchool of Biological and Health Systems EngineeringArizona State UniversityPhoenix, ArizonaThiemeNew York Stuttgart Delhi Rio de Janeiro

Thieme Medical Publishers, Inc.333 Seventh Ave.New York, NY 10001Executive Editor: Kay ConerlyManaging Editor: Judith TomatSenior Vice President, Editorial and Electronic Product Development:Cornelia SchulzeProduction Editor: Sean WoznickiInternational Production Director: Andreas SchabertInternational Marketing Director: Fiona HendersonDirector of Sales, North America: Mike RosemanInternational Sales Director: Louisa TurrellSenior Vice President and Chief Operating Officer: Sarah VanderbiltPresident: Brian D. ScanlanPrinter: Everbest Printing Co., Ltd.Library of Congress Cataloging-in-Publication DataPrinciples and practice of pediatric neurosurgery / [edited by]A. Leland Albright, Ian F. Pollack, P. David Adelson. – Third edition.p. ; cm.Includes bibliographical references and index.ISBN 978-1-60406-799-6 (alk. paper) – ISBN 978-1-60406-801-6(eISBN)I. Albright, A. Leland, editor of compilation. II. Pollack, Ian F., editorof compilation. III. Adelson, P. David, editor of compilation.[DNLM: 1. Neurosurgical Procedures. 2. Adolescent. 3. Child.4. Infant. WS 340]RD593617.4’80083–dc232013040460Important note: Medicine is an ever-changing science undergoingcontinual development. Research and clinical experience are continually expanding our knowledge, in particular our knowledge ofproper treatment and drug therapy. Insofar as this book mentionsany dosage or application, readers may rest assured that theauthors, editors, and publishers have made every effort to ensurethat such references are in accordance with the state of knowledgeat the time of production of the book.Nevertheless, this does not involve, imply, or express any guarantee or responsibility on the part of the publishers in respect to anydosage instructions and forms of applications stated in the book.Every user is requested to examine carefully the manufacturers’leaflets accompanying each drug and to check, if necessary in consultation with a physician or specialist, whether the dosage schedulesmentioned therein or the contraindications stated by the manufacturers differ from the statements made in the present book. Suchexamination is particularly important with drugs that are eitherrarely used or have been newly released on the market. Every dosageschedule or every form of application used is entirely at the user’sown risk and responsibility. The authors and publishers request everyuser to report to the publishers any discrepancies or inaccuraciesnoticed. If errors in this work are found after publication, errata willbe posted at www.thieme.com on the product description page.Some of the product names, patents, and registered designsreferred to in this book are in fact registered trademarks or proprietary names even though specific reference to this fact is not alwaysmade in the text. Therefore, the appearance of a name withoutdesignation as proprietary is not to be construed as a representationby the publisher that it is in the public domain. 2015 Thieme Medical Publishers, Inc.Thieme Publishers New York333 Seventh Avenue, New York, NY 10001 USA 1 800 782 3488, customerservice@thieme.comThieme Publishers StuttgartRüdigerstrasse 14, 70469 Stuttgart, Germany 49 [0]711 8931 421, customerservice@thieme.deThieme Publishers DelhiA-12, Second Floor, Sector-2, Noida-201301Uttar Pradesh, India 91 120 45 566 00, customerservice@thieme.inThieme Publishers Rio, Thieme Publicações Ltda.Argentina Building 16th floor, Ala A, 228 Praia do BotafogoRio de Janeiro 22250-040 Brazil 55 21 3736-3631Printed in China54321ISBN 978-1-60406-799-6also available as e-bookeISBN 978-1-60406-801-6This book, including all parts thereof, is legally protected by copyright. Any use, exploitation, or commercialization outside the narrowlimits set by copyright legislation without the publisher’s consent isillegal and liable to prosecution. This applies in particular to photostat reproduction, copying, mimeographing or duplication of anykind, translating, preparation of microfilms, and electronic dataprocessing and storage.

This book is dedicated toSusan Ferson, Julie and Todd AlbrightConnie, Benjamin, and Andrew PollackBarbara, Samuel, and Richard Adelson; Casey, Brittany, and David Biederman

ContentsPreface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xContributors . . . . . . . . . . . . . . . . . . . . . . . . . . . xiGeneral Topics1.The History of Pediatric Neurosurgery . . . . 3R. Michael Scott2.Normal and Abnormal Development of theNervous System . . . . . . . . . . . . . . . . . . . 10Timothy M. George and David Cory Adamson3.Neurologic Examination of the Newborn,Infant, and Child . . . . . . . . . . . . . . . . . . . 27Michele Yang and Michael Painter4.Caring for the Pediatric NeurosurgicalPatient . . . . . . . . . . . . . . . . . . . . . . . . . . 38Stephanie Greene5.Pediatric Neurosurgery in DevelopingCountries . . . . . . . . . . . . . . . . . . . . . . . . 5112. Intraventricular Hemorrhage andPost-Hemorrhagic Hydrocephalus . . . . . 137Jeffrey R. Leonard and David D. Limbrick Jr.13. The Dandy-Walker Complex andArachnoid Cysts . . . . . . . . . . . . . . . . . . 145Hugh J. L. Garton14. Extracerebral Fluid Collections in Infants . . 162Sandi Lam and David M. Frim15. Congenital Intracranial Malformations . . 171Jonathan D. Bui and Tien T. Nguyen16. Chiari Malformations . . . . . . . . . . . . . . . 192R. Shane Tubbs, Christoph Johannes Griessenauer, andW. Jerry Oakes17. Encephaloceles, Meningoceles, andDermal Sinuses . . . . . . . . . . . . . . . . . . . 205David F. Jimenez and Constance M. Barone18. Congenital Lesions of the Scalp and Skull. . .230Suresh N. Magge, Gary F. Rogers, and Robert F. KeatingA. Leland Albright and Benjamin C. Warf6.Ethical Issues in Pediatric Neurosurgery . . 6019. Craniosynostosis . . . . . . . . . . . . . . . . . . 237Lissa C. Baird and Mark R. ProctorPatrick J. McDonald and Nalin Gupta7.Applications of Cellular Therapy inPediatric Neurosurgery . . . . . . . . . . . . . . 67Eric Thompson and Nathan R. Selden20. Craniofacial Syndromes . . . . . . . . . . . . . 249Richard Hayward21. Craniopagus Twins . . . . . . . . . . . . . . . . 267Samuel R. Browd, James Tait Goodrich, and Marion L. WalkerCongenital and Developmental CerebralDisorders8.Neurogenetic Basis of PediatricNeurosurgical Conditions. . . . . . . . . . . . . 80Claudia C. Faria, Yuzo Terakawa, and James T. Rutka9.Hydrocephalus . . . . . . . . . . . . . . . . . . . . 89Amal Abou-Hamden and James M. Drake10. Treatment of Hydrocephalus with Shunts . . 100Ricky R. S. Kalra and John Kestle11. Neuroendoscopy . . . . . . . . . . . . . . . . . . 119Alan Cohen and Timothy W. VogelCongenital and Developmental Spine Disorders22. Open Fetal Surgery forMyelomeningocele . . . . . . . . . . . . . . . . 278Nalin Gupta23. Spinal Meningoceles . . . . . . . . . . . . . . . 286Jennifer Strahle and Karin Muraszko24. Myelomeningocele . . . . . . . . . . . . . . . . 294Mark S. Dias and Elias Boulos Rizk25. Occult Spinal Dysraphism . . . . . . . . . . . 308D. D. Cochranevii

Contents26. Surgical Management of ComplexSpinal Cord Lipomas . . . . . . . . . . . . . . . 32542. Brainstem Gliomas . . . . . . . . . . . . . . . . 553Heather J. McCrea and Mark M. SouweidaneDachling Pang43. Cerebellar Astrocytomas . . . . . . . . . . . . 56327. Craniovertebral Junction . . . . . . . . . . . . 342Arnold H. Menezes and Raheel Ahmed28. Congenital Intraspinal Cysts. . . . . . . . . . 357Andrew B. Foy and Bruce A. Kaufman29. Congenital Vertebral Anomalies. . . . . . . 364Douglas Brockmeyer30. Skeletal Syndromes . . . . . . . . . . . . . . . . 381Mari L. Groves, Robin Yang, and Benjamin S. Carson Sr.31. Syringomyelia and Hydromyelia . . . . . . . 392Bermans J. Iskandar, Brandon G. Rocque, and Joshua E. MedowNeoplasms32. Scalp and Skull Neoplasms . . . . . . . . . . 408Dale M. Swift and David J. Sacco33. Brain Tumors in the First Two Years of Life . . 423Nelson Moussazadeh and Mark M. Souweidane34. Supratentorial Hemispheric Gliomas . . . 445Ian F. Pollack35. Supratentorial Nonglial HemisphericNeoplasms . . . . . . . . . . . . . . . . . . . . . . 460Scott D. Wait, Frederick A. Boop, and Paul Klimo Jr.36. Optic Pathway Gliomas . . . . . . . . . . . . . 473Ben Shofty, Liat Ben-Sira, Anat Kesler, and Shlomi Constantini37. Craniopharyngiomas . . . . . . . . . . . . . . . 483Jeffrey H. Wisoff and Bernadine Donahue38. Pediatric Pituitary Adenomas . . . . . . . . . 503Erin N. Kiehna and John A. Jane Jr.39. Pineal Region Tumors . . . . . . . . . . . . . . 509Tadanori TomitaPierpaolo Peruzzi, Daniel R. Boué, and Corey Raffel44. Skull Base Tumors . . . . . . . . . . . . . . . . . 574Elizabeth C. Tyler-Kabara, Carl H. Snyderman, andPaul Gardner45. Spinal Extradural Neoplasms and IntraduralExtramedullary Neoplasms . . . . . . . . . . 594Nicholas M. Wetjen, Corey Raffel, and Meghan Murphy46. Intramedullary Spinal Cord Tumors . . . . 605Mari L. Groves and George Jallo47. Spine Tumors . . . . . . . . . . . . . . . . . . . . 614David H. Harter and Howard L. Weiner48. Neurofibromatosis 1 and 2 . . . . . . . . . . 626Ian F. Pollack49. The Phakomatoses . . . . . . . . . . . . . . . . 642Robert P. Naftel and Ian F. Pollack50. Chemotherapy and Biologic Therapy forPediatric Brain Tumors. . . . . . . . . . . . . . 657Roger J. Packer and Tobey J. MacDonald51. Radiotherapy of Pediatric Brain Tumors. . . 676Thomas E. Merchant and Erin S. MurphyTrauma52. Nonaccidental Head Injuries . . . . . . . . . 686Ann-Christine Duhaime53. Accidental Head Injuries in Children. . . . 700Brandon G. Gaynor, Garrett K. Zoeller, and John Ragheb54. Penetrating Craniocerebral Injuries . . . . 706Ira E. Bowen, J. Gordon McComb, and Mark D. Krieger55. Intracranial and Extracranial Hematomasin Children . . . . . . . . . . . . . . . . . . . . . . 718Anthony Figaji40. Medulloblastomas . . . . . . . . . . . . . . . . . 527Vijay Ramaswamy and Michael Taylor41. Ependymomas . . . . . . . . . . . . . . . . . . . 535Scott D. Wait, Michael Taylor, and Frederick A. Boopviii56. Traumatic Brain Injury in Children:Critical Care Management . . . . . . . . . . . 729Michael J. Bell, P. David Adelson, Michael L. Forbes, andPatrick M. Kochanek

Contents57. Rehabilitation and Outcome of HeadInjuries . . . . . . . . . . . . . . . . . . . . . . . . . 743Adam T. Schmidt, Sue R. Beers, and Harvey S. Levin58. Cranioplasty . . . . . . . . . . . . . . . . . . . . . 756Functional Disorders71. Evaluation of Intractable Epilepsy inChildren . . . . . . . . . . . . . . . . . . . . . . . . 932M. Scott Perry and Michael DuchownyJames Tait Goodrich72. Temporal Lobe Epilepsy. . . . . . . . . . . . . 94059. Pediatric Brachial Plexus Palsy . . . . . . . . 780Jeffrey OjemannNathan J. Ranalli and Tae Sung Park73. Extratemporal Epilepsy Surgery . . . . . . . 95160. Principles of Pediatric SpinalColumn Trauma. . . . . . . . . . . . . . . . . . . 789Zachary L. Hickman, Michael McDowell, andRichard C. E. AndersonMatthew D. Smyth and Timothy W. Vogel74. Nonresective and NeuromodulatoryTreatments of Refractory Epilepsy . . . . . 969Joseph R. Madsen, Jay D. Turner, and P. David Adelson61. Specific Injury Patterns and Treatment ofPediatric Spinal Column Trauma. . . . . . . 806Zachary L. Hickman, Michael McDowell, andRichard C. E. Anderson75. Hemispherectomy . . . . . . . . . . . . . . . . . 985Alexandra D. Beier and James T. Rutka76. Spasticity and Movement Disorders . . . . 99462. Spinal Cord Injury . . . . . . . . . . . . . . . . . 833A. Leland AlbrightJoseph H. Miller and Curtis J. Rozzelle63. Intervertebral Disk Disease . . . . . . . . . . 844Steven W. Hwang, Andrew Jea, and Thomas G. LuerssenInfectious Disorders77. Shunt Infections . . . . . . . . . . . . . . . . . 1010Dominic N. P. Thompson and John C. HartleyCerebrovascular Disease64. Cavernous and Venous Malformations . . 858Edward R. Smith and R. Michael Scott65. Moyamoya Disease . . . . . . . . . . . . . . . . 866Peter A. Gooderham and Gary Steinberg66. Intracerebral Aneurysms . . . . . . . . . . . . 874Aaron J. Clark, Jason M. Davies, Kurtis Ian Auguste, andMichael T. Lawton67. Pediatric Arteriovenous Malformations. . 886Edward R. Smith68. Radiosurgical Management of CerebrovascularMalformations in Children . . . . . . . . . . . 897Douglas Kondziolka, Hideyuki Kano, and L. Dade Lunsford69. Vein of Galen Aneurysmal Malformation. . 905Alejandro Berenstein and Yasunari Niimi70. Pediatric Spinal Vascular Malformations . . 92178. Cranial Epidural Abscess and SubduralEmpyema . . . . . . . . . . . . . . . . . . . . . . 1023Christine L. Hammer and Joseph H. Piatt Jr.79. Brain Abscess and Encephalitis. . . . . . . 1036Jonathan Pindrik and Edward S. Ahn80. Tuberculosis, Parasitic Infestations, andFungal Infections . . . . . . . . . . . . . . . . . 1051Graham Fieggen and Llewellyn C. Padayachy81. Infections of the Spinal Axis . . . . . . . . 1065Jeffrey P. Blount, Robert P. Naftel, Benjamin J. Ditty, andMichael J. ConklinNeuroanesthesia82. Pediatric Neuroanesthesia . . . . . . . . . . 1076Sulpicio G. Soriano and Mark A. RockoffIndex . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1090A. Jesse Schuette, Daniel B. Case, Mark J. Dannenbaum,C. Michael Cawley, and Daniel L. Barrowix

PrefaceIt was our hope that the first edition of this book would helpimprove the care of children with pediatric neurosurgicaldisorders and would contribute to the education of theircaregivers. We have been gratified by the widespread acceptance and use of both the first edition in 1999 and the secondedition in 2007, and particularly gratified by its increased usethroughout the world.In 2011, we began work on the third edition, with the sameobjectives as for the first edition. Organization of the bookremains the same: general topics, congenital and developmental cerebral disorders, congenital and developmentalspinal disorders, neoplasms, trauma, cerebrovascular disease, functional disorders, infectious disorders, and neuroanesthesia. The third edition, however, differs from the secondedition in several ways. This edition includes seven chaptersnot included in the second edition: caring for the pediatricneurosurgical patient, cellular therapy for pediatric neurosurgical disease, conjoined twins, lipomeningoceles, skeletalsyndromes, radiotherapy of brain tumors, and Moyamoyadisease. The chapter on caring for the pediatric neurosurgicalpatient is likely to be of value daily.We continued to invite acknowledged authorities to contribute chapters and attempted to identify individuals withbalanced judgment and experience. Most of the chaptersachieved that goal. Readers will note that 42 of the 82chapters in this edition were written by different authorsthan the second edition—a fact that represents primarily thematuration of younger pediatric neurosurgeons into established authorities. Only 29 authors in this edition contributedto the first edition. Readers will also note that chapters in this*xedition were written by authors in Europe (Richard Haywardand Dominic Thompson), the Middle East (Schlomi Constantini), and Africa (Anthony Figaji and Graham Fieggen)—a factthat acknowledges the international readership of the book,but more importantly, the international expertise aboutthose topics.The cover illustration—a magnetic resonance scans of apediatric medulloblastoma and a diffuse intrinsic brainstemglioma (DIPG)—is similar to illustrations on the covers of thefirst two editions of the text, but includes genetic markers ofthose tumors that were unknown in 2007 and that have thepotential to further improve the prognosis of children withmedulloblastomas, and to perhaps finally improve—to atleast a measurable extent—the terrible prognosis of childrenwith DIPG.When comparing the content of this third edition withthat of the first, it is clear that pediatric neurosurgeons areable to give better care to children with many neurosurgicaldisorders, and far better care to those with a few disorders. Itis also clear that for several classical pediatric neurosurgicaldisorders, such as myelomeningoceles and encephaloceles,little has improved since the first edition (other than thebenefits of in utero closures) and to note that their incidencehas steadily declined in developed countries, so that evaluation of prevention and treatment is less feasible.Pediatric neurosurgeons, in general, are grateful for theblessing of caring for children with the disorders describedon the following pages. We three editors remain hopefulthat this third edition will help to further improve theircare.*Note about the cover images: Sagittal magnetic resonance images of pediatric medulloblastoma (lower left) and diffuse intrinsicpontine glioma (DIPG, upper right). For the former tumors, recent studies have demonstrated the existence of at least four molecularly defined tumor subgroups, currently referred to as Shh, Wnt, Group C, and Group D, which have provided new insightsregarding risk stratification and treatment planning. For DIPGs, which have proven resistant to conventional chemotherapy andradiotherapy approaches, recent molecular data has demonstrated frequent alterations in histone modification genes andPDGFRα. Time will tell whether these insights will translate into improvements in response and survival for children with thesechallenging tumors.

ContributorsAmal Abou-Hamden, MDDepartment of NeurosurgeryRoyal Adelaide HospitalAdelaide, AustraliaDavid Cory Adamson, MD, PhDAssociate Professor of Neurosurgery and NeurobiologyDepartment of Surgery (Neurosurgery)Duke UniversityDurham, North CarolinaP. David Adelson, MD, FACS, FAAPDirectorBarrow Neurological Institute at Phoenix Children’s HospitalDiane and Bruce Halle Endowed Chair forPediatric NeurosciencesChief, Pediatric Neurosurgery/Children’s NeurosciencesClinical ProfessorUniversity of Arizona College of Medicine–PhoenixAdjunct ProfessorSchool of Biological and Health Systems EngineeringArizona State UniversityPhoenix, ArizonaRaheel Ahmed, MD, PhDDepartment of NeurosurgeryUniversity of Iowa Hospitals and ClinicsIowa City, IowaEdward Ahn, MDAssistant Professor of NeurosurgeryDivision of Pediatric NeurosurgeryJohns Hopkins University School of MedicineBaltimore, MarylandA. Leland Albright, MDClinical ProfessorDepartment of NeurosurgerySchool of Medicine and Public HealthUniversity of Wisconsin–MadisonMadison, WisconsinConsultantPediatric NeurosurgeonKijabe HospitalKijabe, KenyaRichard C. E. Anderson, MD, FACS, FAAPAssistant Professor of Neurological SurgeryDivision of Pediatric NeurosurgeryColumbia UniversityMorgan Stanley Children’s Hospital of New York PresbyterianNew York, New YorkKurtis Ian Auguste, MDAssociate PhysicianDepartment of Neurological SurgeryUniversity of California–San Francisco BenioffChildren’s HospitalSan Francisco, CaliforniaChildren’s Hospital and Research Center Oaklan

Pediatric Neurosurgeon Kijabe Hospital Kijabe, Kenya Ian F. Pollack, MD, FAANS, FACS, FAAP Chief, Pediatric Neurosurgery Children’s Hospital of Pittsburgh Walter Dandy Professor of Neurological Surgery, 2001–2013 Leland Albright Professor of Neurological Surgery, 2013–present Vice Cha