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Q74R-296K-RQNS-GY2Y
Pediatric Neurosurgery
Tricks of the Trade

Alan R. Cohen, MD, FACS, FAAP, FAANS


Neurosurgeon-in-Chief
Chairman
Department of Neurosurgery
Boston Children’s Hospital
Franc D. Ingraham Professor of Neurosurgery
Harvard Medical School
Boston, Massachusetts

1,039 illustrations

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For Dody
Contents

Foreword. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xv

Preface. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xvii

Acknowledgments. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xix

Contributors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xxi

Section Iâ•… Introduction. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1


Section Editor: Tae Sung Park

1 Basic Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3


Alan R. Cohen
2 Diagnostic Procedures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15
Chad A. Glenn, Naina L. Gross, and Timothy B. Mapstone
3 Neuroanesthesia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25
Sulpicio G. Soriano and Craig D. McClain
4 Pre- and Postoperative Management of the Neurosurgical Patient. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
Robert C. Tasker
5 Pediatric Neurosurgical Positioning. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51
Jonathan A. Pindrik, Sheng-fu Larry Lo, Edward S. Ahn
6 Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58
Daniel M. Schwartz, Andrew Paul Warrington, Anthony K. Sestokas, and Ann-Christine Duhaime
7 Surgical Safety. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 83
Thomas G. Luerssen

Section IIâ•… Neurology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89
Section Editor: Scott L. Pomeroy

8 Neonatal Neurologic Examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91


Charles C. Duncan and Laura R. Ment
9 Neurologic Examination of the Child and Adolescent. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96
Jessica H. R. Goldstein and Nancy Bass

Section IIIâ•… Congenital Malformations. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 101
Section Editor: Concezio Di Rocco

Section III.Aâ•… Malformations of the Scalp and Skull. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 103


10 Congenital Defects of the Scalp and Skull. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 105
Daniel James Guillaume
11 Deformational Plagiocephaly. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109
Mark R. Proctor
12 Nonsyndromic Synostosis: Overview. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113
David H. Harter and David A. Staffenberg
13 Sagittal Synostosis Repair Surgery. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 119
D. Douglas Cochrane and Peter Albert Woerdeman
14 Operative Techniques in Cranial Vault Reconstruction: Nonsyndromic Coronal Craniosynostosis . . . . . . . . . . . . . . . . . . . . . . . . . . 125

Christopher C. Chang, Derek M. Steinbacher, Charles C. Duncan, and John A. Persing
15 The Surgical Repair of Unilateral Coronal Synostosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132
Jodi L. Smith, Laurie L. Ackerman, and Robert J. Havlik
16 The Surgical Repair of Metopic Synostosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 138
Philipp R. Aldana and Nathan J. Ranalli
17 Syndromic Craniosynostosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143
Concezio Di Rocco, Paolo Frassanito, Sandro Pelo, and Gianpiero Tamburrini

vii
viii Contents
18 Minimally Invasive Craniosynostosis Surgery. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151
David F. Jimenez and Constance M. Barone
19 External Distraction for Frontofacial Advancement. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 158
Richard D. Hayward and David J. Dunaway
20 The Surgical Management of Craniopagus Twins. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 171
James Tait Goodrich and David A. Staffenberg

Section III.Bâ•… Malformations of the Brain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 191
21 Malformations of the Cerebral Hemispheres. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 193
Michael D. Partington and Debbie K. Song
22 Occipital Encephalocele. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 197
James Ayokunle Balogun and James M. Drake
23 Surgical Approach to Sphenoethmoidal Encephaloceles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 203
Robert F. Keating and Derek A. Bruce
24 The Chiari I Malformation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 210
Zachary L. Hickman and Neil Feldstein
25 The Chiari II Malformation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 222
Hugh J. L. Garton

Section III.C Malformations of the Spine. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231
26 Craniocervical Junction Abnormalities in Children. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 233
Arnold H. Menezes
27 Disorders of the Vertebral Column. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 240
Luigi Bassani and Douglas Brockmeyer
28 Spinal Deformity/Kyphosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 246
Steven W. Hwang
29 Scoliosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 252
Kaine C. Onwuzulike and George H. Thompson

Section III.Dâ•… Malformations of the Spinal Cord . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 267
30 Myelomeningocele. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 269
Benjamin C. Warf
31 Tight Filum Terminale . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 274
Thomas J. Wilson and Karin Muraszko
32 Spinal Tethering Tracts. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 280
Casey Madura and Bermans J. Iskandar
33 Spinal Lipomas. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 287
Tarik Ibrahim, Robin M. Bowman, and David G. McLone
34 Split Cord Malformation: From Gastrulation to Operation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 293
Dachling Pang
35 Congenital Spinal Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 306
Elias Boulos Rizk, R. Shane Tubbs, and W. Jerry Oakes

Section IVâ•… Hydrocephalus and Disorders of Cerebrospinal Fluid Circulation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 311

Section Editor: John Kestle

36 The Pathophysiology and Classification of Hydrocephalus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 313


David M. Frim and Ashley Ralston
37 Ventricular Shunting for Hydrocephalus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 316
Walavan Sivakumar, Jay Riva-Cambrin, Vijay M. Ravindra, and John Kestle
38 Endoscopic Treatment of Hydrocephalus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 321
Alexandra D. Beier and Abhaya V. Kulkarni
39 Congenital Intracranial Cysts. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325
Spyridon Sgouros and Vassilios Tsitouras
40 The Dandy-Walker Malformation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 330
Conor Mallucci and Christopher Parks
41 Idiopathic Intracranial Hypertension. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 335
Sarah J. Gaskill and Arthur E. Marlin
Contents ix
Section Vâ•… Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 339
Section Editor: George I. Jallo

42 Management of Pediatric Scalp Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 341


Arthur Wang, Jordan M. S. Jacobs, and Avinash Mohan
43 Skull Fractures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 348
Elizabeth C. Tyler-Kabara
44 Traumatic Brain Injury. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 355
Brian T. Farrell and Nathan R. Selden
45 Penetrating Head Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 363
Kyle G. Halvorson and Gerald A. Grant
46 Vascular Injuries. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 374
Jeffrey C. Mai, Kyle M. Fargen, Spiros Blackburn, and David W. Pincus
47 Abusive Head Injuries. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379
Shenandoah Robinson
48 Cranioplasty . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 385
Jordan P. Steinberg and Arun K. Gosain
49 Neurointensive Care of Head Injuries. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 395
Ash Singhal and Alexander Ross Hengel
50 Pediatric Vertebral Column and Spinal Cord Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 399
Dachling Pang and Sui-To Wong
51 Brachial Plexus Birth Injuries. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 412
Nathan J. Ranalli and T. S. Park

Section VIâ•… Neoplasms. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 421


Section Editor: Frederick A. Boop

52 Molecular and Genetic Advances in the Treatment of Brain Tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 423


Vijay Ramaswamy, Marc Remke, and Michael D. Taylor

Section VI.Aâ•… Supratentorial Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 429


53 Craniopharyngioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 431
Jeffrey H. Wisoff
54 Pineal Region Tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 437
J. Gordon McComb and Laurence Davidson
55 Cerebral Hemispheric Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 450
Robert P. Naftel, Elizabeth C. Tyler-Kabara, and Ian F. Pollack
56 Intraventricular Tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 457
Renee M. Reynolds and Richard G. Ellenbogen
57 Tumors of the Optic Pathway and Hypothalamus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 466
Liliana C. Goumnerova
58 Pituitary Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 473
Gautam U. Mehta and John A. Jane Jr.

Section VI.Bâ•… Infratentorial Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 479
59 Cerebellar Astrocytoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 481
Stephanie L. Da Silva and Mark D. Krieger
60 Medulloblastoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 488
Lauren Ostling and Corey Raffel
61 Infratentorial Ependymomas. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 496
Michael DeCuypere and Frederick A. Boop
62 Pediatric Brainstem Gliomas. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 502
Jonathan Roth and Shlomi Constantini
63 Intracranial Epidermoids. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 509
Henry W. S. Schroeder

Section VI.Câ•… Scalp, Skull, and Skull Base Neoplasms. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 521
64 Tumors of the Scalp and Skull . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 523
Nalin Gupta and William Y. Hoffman
65 Tumors of the Skull Base and Orbit. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 529
Kaisorn L. Chaichana, Ignacio Jusue-Torres, and George I. Jallo

x Contents
Section VI.Dâ•… Neoplasms of the Spine, Spinal Cord, and Peripheral Nerves. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 535
66 Tumors of the Vertebral Column. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 537
Sudhakar Vadivelu and Andrew Jea
67 Extramedullary Spinal Cord Tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 547
Timothy W. Vogel and Jeffrey R. Leonard
68 Intramedullary Spinal Cord Tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 556
Michael Weicker and Rick Abbott
69 The Surgical Management of Pediatric Brachial Plexus Tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 563
Elias Boulos Rizk and John “Jay” C. Wellons III

Section VI.Eâ•… Other. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 571
70 The Neurocutaneous Syndromes. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 573
Herbert E. Fuchs
71 Adjuvant Chemotherapy and the Role of Neurosurgery for Pediatric Central Nervous System Tumors. . . . . . . . . . . . . . . . . . . . . . 584
Mark W. Kieran
72 Adjuvant Radiation Therapy for Pediatric Tumors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 589

Thomas E. Merchant

Section VIIâ•… Infections. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 595

Section Editor: A. Graham Fieggen

Section VII.A Cranial. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 597


73 Meningitis and Encephalitis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 599
Dhruve Jeevan and Michael E. Tobias
74 Cranial Epidural Abscess and Subdural Empyema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 605
William E. Whitehead
75 Cerebral Abscess. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 612
Justin Davis and Thomas A. Pittman
76 Tuberculous, Fungal, and Parasitic Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 616
A. Graham Fieggen and Anthony A. Figaji
77 Cysticercosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 630
Tenoch Herrada-Pineda, Juan Antonio Ponce-Gomez, Salvador Manrique-Guzman, and Francisco Revilla-Pacheco

Section VII.Bâ•… Spinal. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 641


78 Evaluation and Management of Pediatric Spinal Infections. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 643
Jonathan Yun, Brian J. A. Gill, and Richard C. E. Anderson

Section VIIIâ•… Epilepsy and Functional Disorders. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 649
Section Editor: Matthew D. Smyth

79 Epilepsy Classification, Evaluation, and Imaging. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 651


Iván Sánchez Fernández and Tobias Loddenkemper
80 The Surgical Treatment of Epilepsy: Overview . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 662
Hai Sun, Sergey Abeshaus, and Jeffrey G. Ojemann
81 Invasive Monitoring in Pediatric Neurosurgery. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 665
Jean-Pierre Farmer and Jeffrey Atkinson
82 The Surgical Treatment of Temporal Lobe Epilepsy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 674
Benjamin A. Rubin and Howard L. Weiner
83 The Surgical Treatment of Extratemporal Epilepsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 684
Alexander G. Weil and Sanjiv Bhatia
84 The Surgical Treatment of Rolandic Epilepsy in Children. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 695
Christian J. Cantillano Malone and James T. Rutka
85 Hemispherotomy and Hemispherectomy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 703
Michael H. Handler and Brent O’Neill
86 Palliative Surgical Procedures for Epilepsy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 712
Matthew D. Smyth and Aimen S. Kasasbeh
87 The Evaluation and Treatment of Spasticity. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 723
Shenandoah Robinson
88 Intrathecal Therapy for Movement Disorders. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 731
Bruce A. Kaufman
Contents xi
89 Microelectrode-Guided Deep Brain Stimulation in Children. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 737
Ron L. Alterman and Irene P. Osborn
90 Interventions for Acute and Chronic Pain in Children. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 742
Charles Berde

Section IXâ•… Vascular Disorders. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 753
Section Editor: R. Michael Scott

91 Stroke in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 755


Cormac O. Maher
92 Pediatric Aneurysms. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 759
Allyson Alexander and Michael S. B. Edwards
93 Pediatric Arteriovenous Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 768
Graciela Zuccaro and Javier González Ramos
94 Cavernous Malformations and Venous Malformations. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 779
Christopher David Kelly and Raphael Guzman
95 Vein of Galen Aneurysmal Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 788
Alejandro Berenstein and Srinivasan Paramasivam
96 Moyamoya Syndrome/Pial Synangiosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 799
Edward R. Smith and R. Michael Scott
97 Surgical Management of Spinal Arteriovenous Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 805
Shakeel A. Chowdhry and Robert F. Spetzler

Section Xâ•… New and Emerging Techniques . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 815
Section Editor: James M. Drake

98 Advances in Neuroimaging. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 817


Edward Yang and Caroline D. Robson
99 Intraoperative Imaging. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 827
Paul Klimo Jr., David J. Daniels, and Asim F. Choudhri
100 Interventional Neuroradiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 834
Bradley A. Gross, Michael J. Ellis, and Darren Orbach
101 Image-Guided Surgery. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 841
Yair M. Gozal and Timothy W. Vogel
102 Advances in Neuroendoscopy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 846
Robert P. Naftel and John “Jay” C. Wellons III
103 Endoscope-Assisted Microsurgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 853
Henry W. S. Schroeder
104 Laser Ablation of Deep Lesions. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 860
Joseph R. Madsen
105 Techniques for Limiting Blood Loss and Blood Transfusions in Pediatric Neurosurgery. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 867
Paul Steinbok

Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 875
Video Table of Contents

Video Editor: Timothy W. Vogel

1 Basic Surgical Technique



Alan R. Cohen
9 Neurological Examination of the Child and Adolescent

Jessica H. R. Goldstein and Nancy Bass
13 Sagittal Synostosis Repair Surgery
Limited Vertex Craniectomy and Postoperative Helmetting
Sagittal Synostosis: Open Technique

D. Douglas Cochrane and Peter Albert Woerdeman
15 The Surgical Repair of Coronal Synostosis

Jodi L. Smith
16 Metopic Synostosis: Bifrontal Craniotomy and Supraorbital Advancement

Philipp R. Aldana and Nathan J. Ranalli
18 Minimally Invasive Endoscopic Treatment of Sagittal Synostosis

David F. Jimenez and Constance M. Barone
24 The Chiari I Malformation

Neil Feldstein
27 Anterior Cervical Discectomy and Fusion

Douglas Brockmeyer
28 Spinal Osteotomies

Steven W. Hwang
31 Tight Filum Terminale

Karin Muraszko
33 Surgical Management of Spinal Lipomas

Tarik Ibrahim, Robin M. Bowman, and David G. McLone
37 Ventriculoperitoneal Shunt Placement

Jay Riva-Cambrin, John Kestle, Vijay M. Ravindra, and Walavan Sivakumar
38 Endoscopic Third Ventriculostomy: Tectal Glioma with Hydrocephalus

Abhaya V. Kulkarni
39 Suprasellar Arachnoid Cyst

Spyridon Sgouros
41 Idiopathic Intracranial Hypertension

Sarah J. Gaskill
44 External Ventricular Drain Insertion in an Infant

Lissa C. Baird and Nathan R. Selden
51 Brachial Plexus Repair Surgery

T. S. Park
55 Cerebral Hemispheric Tumors

Ian F. Pollack and Robert P. Naftel
59 Cerebellar Astrocyotoma

Mark D. Krieger
60 Medulloblastoma
Lauren Ostling and Corey Raffel
61 Posterior Fossa Ependymoma
Frederick A. Boop
63 Intracranial Epidermoids

Henry W. S. Schroeder
65 Tumors of the Skull Base and Orbit

George I. Jallo
77 Neurocysticercosis: Endoscopic Treatment

Tenoch Herrada-Pineda
82 The Surgical Treatment of Temporal Lobe Epilepsy

Benjamin A. Rubin and Howard L. Weiner

xii
Video Table of Contents xiii
86 Palliative Surgical Procedures for Epilepsy

Matthew D. Smyth
89 Deep Brain Stimulation for Movement Disorders

Ron L. Alterman
93 Arteriovenous Malformations

Graciela Zuccaro
94 Cavernous Malformations and Venous Malformations

Raphael Guzman
95 Vein of Galen Malformations

Alejandro Berenstein and Srinivasan Paramasivam
97 Compact Intramedullary Spinal Arteriovenous Malformation

Shakeel A. Chowdhry and Robert F. Spetzler
102 Advances in Neuroendoscopy

Robert P. Naftel and John “Jay” C. Wellons III
103 Endoscope-Assisted Microsurgery

Henry W. S. Schroeder
Foreword

Most neurosurgeons are familiar with the quote Pediatric Neurosurgery: Tricks of the Trade is also
Ars longa, vita brevis—the art is long, life is short. replete with superior video content that demon-
Although written in Latin, this quote is ascribed to the strates, step-by-step, critical aspects of the various
ancient Greek physician Hippocrates, from his work neurosurgical procedures, from patient positioning,
entitled Aphorismi. Perhaps unlike any other neuro- to the skin incision, to the craniotomy, to the intra-
surgical subspecialty, pediatric neurosurgery is a dis- dural phase of the operation. Tricks of the trade are
cipline of nuances in which the subtleties of practice liberally provided throughout the voice-over com-
are brought to bear over the course of one’s profes- ments from the videos. You will see and learn how to
sional career. Even then, a lifetime is insufficient to bevel your scissors while opening the dura in order to
learn everything. Of course, I speak of both the cog- avoid cortical venous lacerations; to open the blades
nitive and the procedural sides of pediatric neurosur- of an endoscopic forceps in the coronal plane when
gery. Our practice of pediatric neurosurgery is, after performing an endoscopic third ventriculostomy
all, an amalgam of time-honored methods that have to avoid injuring the basilar artery; and to preserve
been passed on to us from our mentors, who learned the ependyma beneath the corpus callosum to avoid
in a similar manner from their mentors, in a cycle dat- a cerebrospinal fluid disturbance postoperatively
ing back to the origins of pediatric neurosurgery with during corpus callosotomy, among many, many other
Franc Ingraham at the Children’s Hospital in Boston, pearls and tricks. The videos are a major strength of
who himself was a disciple of the founding father of the textbook and are edited at a perfect length for
organized neurosurgery, Harvey Cushing. What bet- learners, being set at 3 to 5 minutes to capture the
ter way is there for us to appreciate and learn the neurosurgical essence of each procedure.
wisdom of the ages in pediatric neurosurgery than Benjamin Franklin once wrote: “Tell me and I for-
to adapt the teachings of experts who have imparted get; teach me, and I may remember; involve me, and
their knowledge to us in this unique and accessible I learn.” This quote is particularly applicable to the
textbook. present textbook, as the charge to the authors for
In Pediatric Neurosurgery: Tricks of the Trade, res- each chapter was to write the definitive work on the
idents, fellows, and neurosurgeons alike will find an subject area and to provide caveats and experiential
up-to-date compendium of Pearls and Pitfalls involv- knowledge so that readers could not only have the
ing approaches to pediatric neurosurgical conditions best academic resource at their fingertips on a given
that will help improve the delivery of patient care pediatric neurosurgical condition, but could also
and satisfy best practices. From patient positioning, practice complication avoidance by carefully observ-
to neuroanesthetic considerations, to optimum post- ing the advice that has been handed down to us by
operative management, it is clear that this textbook our sage predecessors. To return to Franklin’s quote
transcends the typical multiauthored textbook on in closing, Pediatric Neurosurgery: Tricks of the Trade
the technical aspects of pediatric neurosurgery. I was is an example of the ultimate method of teaching as
thus pleased to see the attention paid to neurosur- it involves all of us in an exercise in learning that is
gical judgment throughout all chapters, as it is just unlikely to be duplicated for years to come. We owe
as important to know when to operate as to know Alan Cohen, editor, and the authors of each chapter
when not to. In addition, care was taken to ensure a big debt of gratitude for providing us with a tome
that the content of the textbook was comprehen- that will help all of us better look after our patients
sive, innovative, and timely. As examples, chapters and achieve the best outcomes possible, though the
on the management of scoliosis, external distraction art is long.
for frontofacial advancement, intrathecal therapy for
movement disorders, and laser ablation for deep brain James T. Rutka, MD, PhD, FRCS(C), FACS, FAAP, FAANS
lesions would not have existed in textbooks written R. S. McLaughlin Professor and Chair
for pediatric neurosurgeons a decade ago. Department of Surgery
Division of Neurosurgery
The Hospital for Sick Children
University of Toronto
Toronto, Ontario, Canada

xiv
Preface
“Simplicity is the ultimate sophistication.”
Leonardo da Vinci

The practice of pediatric neurosurgery is one of the formers to a state of seasoned wisdom. Just as there
most remarkable and rewarding endeavors in all of are many ways to play the same tune, there are many
medicine. The ability to help alleviate suffering and ways to carry out the same operation. And some clear-
restore health to an ailing, sometimes gravely stri- ly work out better than others. Why is that? What is
cken child, is an extraordinary privilege granted to a it that makes certain surgeons exceptional and others
limited few. It is this special privilege that makes the only good or average at best? What are the factors
many arduous years of training in pediatric neurosur- necessary to deliver a virtuoso performance? What
gery bearable. measures can we take to improve our proficiency in
By and large, pediatric neurosurgery is a difficult the operating room?
field. Surgical disorders of the developing nervous Surely, nature plays an important role, as some
system are often complex and present exceptional surgeons seem to operate with innate, effortless dex-
challenges to the pediatric neurosurgeon, who must terity right from the get go. But inborn ability is only
learn to master a broad field in great depth over a part of the picture, and it’s not worth focusing on this
limited time. It is essential for the pediatric neuro- too much since there’s little we can do to alter our
surgeon to acquire a detailed understanding of a vast genetic heritage. Fortunately, there’s much that can
amount of specialized anatomy, pathology, and pa- be done to perfect our technique, enhance our perfor-
thophysiology that is constantly changing throughout mance, and improve our outcomes.
childhood. The explosion of knowledge in the last de- We can learn a lot from the experience of others.
cade about the molecular and genetic bases for neu- One major key to success, I believe, is using the ac-
rosurgical disorders is a bit overwhelming and will tions of others, both good and bad, to improve our
form the basis for more effective, tailored treatments own abilities. Surgical judgment develops over time.
for our patients. At the same time, advances in neuro- Astute practitioners learn much from their own mis-
diagnostic imaging and improvements in surgical in- takes and from the mistakes of others. Elite perfor-
strumentation are occurring at a rapid pace. The field mance is the result of patience, practice, and perseve-
is becoming so complex that it is sometimes difficult rance. Elite performers demonstrate an economy of
to comprehend. movement in the OR. They are deeply focused on the
This book is an attempt to simplify some of these problem at hand and the paramount goal of getting
complexities. Its purpose is twofold: (1) to present the patient safely through the procedure, whether it
a concise overview of the current state-of-the-art of is the removal of a small dermoid cyst from the skull
pediatric neurosurgery as elaborated by expert colle- or a large cavernous malformation from the medulla.
agues from around the world; and (2) to provide the They come to the OR well prepared. They are orga-
reader with a blueprint for enhancing performance nized. They use checklists. They have learned to re-
and achieving excellence in the operating room. main methodical and calm, even in the presence of
Great surgeons, like great musicians, possess an crisis. They respect their colleagues and inspire the
elusive skill set that is easy to recognize but harder to OR staff to work together as a team.
define. The consistent features appear to be a unique My hope is that this book will provide pediatric
combination of inherent technical ability that has neurosurgeons with a strategy for becoming elite
been refined by meticulous, painstaking practice—a performers. This is a book about the art of pediatric
combination that has ultimately brought these per- neurosurgery. It is intended to fill a void. There are

xv
xvi Preface

numerous publications about the details of pediatric tric neurosurgical operations written by masters from
neurosurgical disorders, including journal articles, around the world who share their personal surgical
didactic texts, and atlases of anatomy and surgery. pearls, operative nuances, procedural refinements, and
What is missing is a personal account of the journey, techniques for avoiding pitfalls and for dealing with
a compilation of the wisdom of the masters, a tricks them should they occur. I hope that these nuances
of the trade of pediatric neurosurgery in which experts from the masters will inspire readers to improve their
share what they have learned from past experiences, skill sets and to set higher expectations for themselves.
both their successes and their mistakes. I hope this will lead them to pursue intense, focused,
Much of what separates a great neurosurgeon from deliberate practice in an effort to achieve excellence in
a good neurosurgeon is nuance. Many times a subtle the operating room.
operative maneuver here or there can simplify a dif-
ficult procedure or prevent a dangerous or catastro- Alan R. Cohen, MD
phic event later on. This book is a collection of pedia- Boston, Massachusetts
Acknowledgments
He who studies medicine without books sails an uncharted sea,
but he who studies medicine without patients does not go to sea at all.
Sir William Osler

I wish to extend my heartfelt thanks to the parents of to be guided by an expert editorial and production team
my patients for entrusting me with the care of those who brought a passion to the project that I cannot repay.
whom they hold most dear in the world. It is a great In particular, I would like to thank Kay Conerly, Judith
privilege to be a pediatric neurosurgeon and to share Tomat, Sara D’Emic, Liz Palumbo, Nikole Connors, Kenny
the lives and dreams of these courageous young resil� Chumbley, and Tim Hiscock.
ient heroes, who have taught me more about neuro- Finally, I thank Jennifer Pryll, whose beautiful art-
surgery than I could have ever imagined. work graces the book’s cover.
I am also most grateful to Thieme Publishers for Alan R. Cohen, MD
helping me bring this book to fruition. I was fortu�nate

xvii
Contributors

Rick Abbott, MD Richard C. E. Anderson, MD, FACS, FAAP


Professor, Clinical Neurosurgery Associate Professor, Division of Pediatric
Department of Neurosurgery Neurosurgery
Albert Einstein College of Medicine Department of Neurological Surgery
Bronx, New York Columbia University
Morgan Stanley Children’s Hospital of
Sergey Abeshaus,€MD New York Presbyterian
Attending Neurosurgeon New York, New York
Department of Neurological Surgery
Pediatric Neurosurgery Unit Jeffrey Atkinson, MD, FRCS(c)
Rambam Health Care Campus Paediatric Neurosurgeon€
Haifa, Israel McGill University Health Centre
Assistant Professor
Laurie L. Ackerman, MD, FAANS Departments of Neurology, Neurosurgery, and
Assistant Professor Paediatric Surgery
Department of Neurosurgery McGill University
Riley Hospital for Children at Indiana University Montreal, Quebec, Canada€
Health
Goodman Campbell Brain and Spine Lissa C. Baird, MD
Indianapolis, Indiana Pediatric Neurosurgeon
Medical Director of Pediatric Neuro-oncology
Edward S. Ahn, MD Oregon Health & Science University
Associate Professor of Neurosurgery, Pediatrics, and Portland, Oregon
Plastic Surgery
Division of Pediatric Neurosurgery James Ayokunle Balogun, MBBS, FWACS
The Johns Hopkins University School of Medicine Division of Pediatric Neurosurgery
Baltimore, Maryland The Hospital for Sick Children
Toronto, Ontario, Canada
Philipp R. Aldana, MD
Associate Professor and Chief€€€€€ Constance M. Barone, MD, FACS
Department of Neurosurgery Professor of Neurosurgery
University of Florida College Department of Neurosurgery
of Medicine–Jacksonville€€€€€€€€€€€ University of Texas Health Science Center
Jacksonville, Florida San Antonio, Texas

Allyson Alexander, MD, PhD Nancy Bass, MD


Neurosurgery Associate Professor
Division of Pediatric Neurosurgery Department of Pediatrics and Neurology
Lucile Packard Children’s Hospital Case Western Reserve University School of Medicine
Stanford University School of Medicine Rainbow Babies and Children’s Hospital
Stanford, California Case Western Reserve University
Cleveland, Ohio
Ron L. Alterman, MD
Professor of Neurosurgery
Harvard Medical School
Chief, Division of Neurosurgery
Beth Israel Deaconess Medical Center
Boston, Massachusetts

xviii
Contributors╅ xix
Luigi Bassani, MD Robin M. Bowman, MD
Assistant Professor Associate Professor
Director of Pediatric Neurosurgery Department of Neurological Surgery
Rutgers New Jersey Medical School Northwestern University Feinberg School
Newark, New Jersey of Medicine
Evanston, Illinois
Alexandra D. Beier, DO, FACOS
Assistant Professor of Neurosurgery and Pediatrics Douglas Brockmeyer, MD
Division of Pediatric Neurosurgery Professor of Neurosurgery
University of Florida Health–Jacksonville Chief of Pediatric Neurosurgery
Jacksonville, Florida Department of Neurosurgery
University of Utah
Charles Berde, MD, PhD Salt Lake City, Utah
Sara Page Mayo Chair
Chief, Division of Pain Medicine Derek A. Bruce, MB, ChB
Department of Anesthesiology, Perioperative, Professor of Neurosurgery and Pediatrics
and Pain Medicine Center for Neuroscience and Behavioral Medicine
Boston Children’s Hospital Children’s National Medical Center
Professor Washington, DC
Department of Anaesthesia (Pediatrics)
Harvard Medical School Christopher C. Chang, MD
Boston, Massachusetts Craniofacial Surgery
Department of Plastic Surgery
Alejandro Berenstein, MD The Johns Hopkins Hospital
Site Chair University of Maryland Shock Trauma Center
Senior Faculty Neurosurgery, Radiology, Pediatrics Baltimore, Maryland
Institute of Neurology and Neurosurgery
Mount Sinai Roosevelt Kaisorn L. Chaichana, MD
Mount Sinai St. Luke’s Assistant Professor of Neurosurgery, Oncology, and
New York, New York Otolaryngology
Department of Neurosurgery
Sanjiv Bhatia, MD, FACS Johns Hopkins Hospital€
Pediatric Neurosurgery Baltimore, Maryland
Miami Children’s Hospital
Associate Professor Asim F. Choudhri, MD
Department of Neurosurgery Associate Chair–Research Affairs
University of Miami Miller School of Medicine Department of Radiology
Miami, Florida Associate Professor of Radiology, Ophthalmology,
and Neurosurgery
Spiros Blackburn, MD University of Tennessee Health Science Center
Assistant Professor Director of Neuroradiology
Department of Neurosurgery Le Bonheur Neuroscience Institute
University of Florida Le Bonheur Children’s Hospital
Gainesville, Florida Memphis, Tennessee

Frederick A. Boop, MD Shakeel A. Chowdhry, MD


Professor and Chairman Department of Neurosurgery
Department of Neurosurgery Northshore University Health System
University of Tennessee Health Sciences Center Assistant Professor
Semmes-Murphey Clinic University of Chicago Pritzker School of Medicine
Memphis, Tennessee Chicago, Illinois
xx Contributors
D. Douglas Cochrane, MD, FRCS(C) Concezio Di Rocco, MD
Head, Division of Neurosurgery Professor
Professor of Surgery Director of Pediatric Neurosurgery
Department of Pediatric Surgery International Neuroscience Institute
Department of €Surgery Hannover, Germany
British Columbia Children’s Hospital
University€of€British Columbia James M. Drake, BSE, MBBCh, MSc, FRCS(C), FACS
Vancouver, British Columbia, Canada Division Head, Pediatric Neurosurgery
Professor of Surgery
Alan R. Cohen, MD, FACS, FAAP, FAANS University of Toronto
Neurosurgeon-in-Chief Shoppers Drug Mart
Chairman Harold Hoffman Chair Pediatric Neurosurgery
Department of Neurosurgery Director, Centre for Image Guided Innovation and
Boston Children’s Hospital Therapeutic Intervention
Franc D. Ingraham Professor of Neurosurgery The Hospital for Sick Children
Harvard Medical School Toronto, Ontario, Canada
Boston, Massachusetts€
Ann-Christine Duhaime, MD
Shlomi Constantini, MD, MSc Director, Pediatric Neurosurgery
Professor and Director Massachusetts General Hospital
Department of Pediatric Neurosurgery Professor
The Israeli Neurofibromatosis Center Department of Neurosurgery
Dana Children’s Hospital Harvard Medical School
Tel-Aviv Medical Center Boston, Massachusetts
Tel Aviv University
Tel Aviv, Israel David J. Dunaway, FDSRCS, FRCS
Specialty Lead for Craniofacial Surgery
David J. Daniels, MD, PhD Great Ormond Street Hospital
Assistant Professor of Neurosurgery London, England
Pediatric Neurosurgery
Mayo Clinic Charles C. Duncan, MD
Rochester, Minnesota Professor of Neurosurgery and Pediatrics
Department of Neurosurgery
Stephanie L. Da Silva, BA Yale School of Medicine
Clinical Research Coordinator€ New Haven, Connecticut
Division of Neurosurgery
Children’s Hospital Los Angeles Michael S. B. Edwards, MD, FAANS, FACS, FAAP
Los Angeles, California Lucile Packard Endowed Professor of Neurosurgery
and Pediatrics
Laurence Davidson, MD, FAANS Co-Director, Center for Children’s Brain Tumors at
Director, Pediatric Neurosurgery Stanford
Division of Neurosurgery Department of Neurosurgery
Walter Reed National Military Medical Center Stanford University
Assistant Professor of Surgery and Pediatrics Lucile Packard Children’s Hospital Stanford
Uniformed Services University of the Health Sciences Stanford, California
Bethesda, Maryland
Richard G. Ellenbogen, MD, FACS
Justin Davis, MD Professor and Chairman
Department of Neurological Surgery Department of Neurological Surgery
Neuroscience Associates of Kansas City Theodore S. Roberts Endowed Chair
Overland Park, Kansas Director, Neurosciences Institute
University of Washington School of Medicine
Michael DeCuypere, MD, PhD Seattle Children’s Hospital
Department of Neurosurgery Seattle, Washington
University of Tennessee Health Science Center
Memphis, Tennessee
Contributors xxi
Michael J. Ellis, MD, FRCS(C) € €€ Anthony A. Figaji, MMed, PhD, FC Neurosurg(SA)
Medical Director SARChI Professor of Clinical Neuroscience
Pan Am Concussion Program Division of Neurosurgery
Department of Surgery and Pediatrics Section University of Cape Town
of Neurosurgery Cape Town, South Africa
University of Manitoba €
Co-Director, Canada North Concussion Network Paolo Frassanito, MD
Scientist, Children’s Hospital Research Institute of Consultant
Manitoba€ Pediatric Neurosurgery
Winnipeg, Manitoba, Canada Catholic University Medical School
Rome, Italy
Kyle M. Fargen, MD, MPH
Faculty David M. Frim, MD, PhD
Department of Neurosurgery Ralph Cannon Professor and Chief
University of Florida Section of Neurosurgery
Gainesville, Florida University of Chicago
Chicago, Illinois
Jean-Pierre Farmer, MD, CM, FRCS(C)
Dorothy Williams Professor and Chair Herbert E. Fuchs, MD, PhD, FAANS, FAAP, FACS
Department of Pediatric Surgery Associate Professor of Surgery
Professor of Neurosurgery, Pediatric Surgery, Division of Neurosurgery
Oncology, and Surgery€ Chief of Pediatric Neurosurgical Services
McGill University Department of Surgery
Surgeon-in-Chief and Director Duke University Medical Center
Neurosurgery Division Durham, North Carolina
The Montreal Children’s Hospital
McGill University Health Centre Hugh J. L. Garton, MD, MHSc
Montreal, Quebec, Canada Associate Professor
Department of Neurosurgery
Brian T. Farrell, MD, PhD University of Michigan
Department of €Neurological Surgery Ann Arbor, Michigan
Oregon Health€& Science University€
Portland, Oregon Sarah J. Gaskill, MD
Professor
Neil Feldstein, MD, FACS Department of Neurosurgery and Brain Repair
Associate Professor Neurological Surgery Division of Pediatric Neurosurgery
Department of Neurological Surgery University of South Florida
New York Presbyterian Hospital Tampa, Florida
Columbia University Medical Center
Director, Pediatric Neurological€Surgery Brian J. A. Gill, MD
Morgan Stanley Children’s Hospital€ Department of Neurological Surgery
New York, New York Columbia-New York Presbyterian Hospital€
New York, New York
Iván Sánchez Fernández, MD
Division of Epilepsy and Clinical Neurophysiology Chad A. Glenn, MD
Department of Neurology Department of Neurosurgery
Boston Children’s Hospital University of Oklahoma Health Sciences Center
Harvard Medical School Oklahoma City, Oklahoma
Boston, Massachusetts
Jessica H. R. Goldstein, MD
A. Graham Fieggen, MSc, MD, FC Neurosurg(SA) Assistant Professor of Pediatrics
Helen and Morris Mauerberger Professor and Head Department of Pediatric Neurology
Division of Neurosurgery Case Western Reserve University School of Medicine
University of Cape Town Rainbow Babies and Children’s Hospital
Cape Town, South Africa Cleveland, Ohio
xxii Contributors
James Tait Goodrich, MD, PhD, DSci (Hon) Nalin Gupta, MD, PhD
Director, Division of Pediatric Neurosurgery Benioff UCSF Professor in Children’s Health
Children’s Hospital at Montefiore Departments of Neurological Surgery and Pediatrics
Professor of Clinical Neurosurgery, Pediatrics, University of California–San Francisco
Surgery (Plastic and Reconstructive Surgery) Chief, Division of Pediatric Neurosurgery
Leo Davidoff Department of Neurological Surgery UCSF Benioff Children’s Hospital
Albert Einstein College of Medicine San Francisco, California
Bronx, New York
Raphael Guzman, MD
Arun K. Gosain, MD, FACS, FAAP Professor of Neurosurgery
Professor and Chief Vice Chair and Chief, Pediatric Neurosurgery
Division of Pediatric Plastic Surgery Department€of Neurosurgery
Lurie Children’s Hospital Division of Pediatric Neurosurgery
Northwestern University Feinberg School of University Children’s Hospital of Basel
Medicine Basel, Switzerland
Chicago, Illinois
Kyle G. Halvorson, MD
Yair M. Gozal, MD, PhD Department of Surgery
Department of Neurosurgery Duke University Medical Center
University of Cincinnati College of Medicine Durham, North Carolina
Cincinnati, Ohio
Michael H. Handler, MD, FACS, FAAP
Liliana C. Goumnerova, MD, FRCS(C), FAANS McMurry Seebaum Chair and Chief of Pediatric
Director, Pediatric Neurosurgical Oncology Neurosurgery
Boston Children’s Hospital/Dana Farber Cancer Professor
Institute University of Colorado School of Medicine
Associate Professor of Neurosurgery Denver, Colorado
Harvard Medical School
Department of Neurosurgery David H. Harter, MD
Boston Children’s Hospital Assistant Professor of Neurosurgery
Boston, Massachusetts Division of Pediatric Neurosurgery
New York University
Gerald A. Grant, MD, FACS€ New York, New York
Associate Professor
Department of Neurosurgery€ Robert J. Havlik, MD
Chief of Pediatric Neurosurgery Chairman
Lucile Packard Children’s Hospital Department of Plastic Surgery
Stanford University Medical College of Wisconsin
Stanford, California Milwaukee, Wisconsin

Bradley A. Gross, MD Richard D. Hayward, MBBS, FRCS(Eng)
Department of Neurosurgery Professor
Brigham and Women’s Hospital and Harvard Department€of Pediatric Neurosurgery
Medical School Great Ormond Street Hospital for Children NHS Trust
Boston, Massachusetts London, England

Naina L. Gross, MD Alexander Ross Hengel, BSc


Assistant Professor Clinical Research Coordinator
Department of Neurosurgery Department of Surgery
University of Oklahoma Division of Pediatric Neurosurgery
Oklahoma City, Oklahoma University of British Columbia
Vancouver, British Columbia, Canada
Daniel James Guillaume, MD
Associate Professor Tenoch Herrada-Pineda, MD
Department of Neurosurgery Department of Pediatric Neurosurgery
University of Minnesota ABC Medical Center
Minneapolis, Minnesota Mexico City, Mexico
Contributors xxiii
Zachary L. Hickman, MD John A. Jane Jr., MD
Department of Neurological Surgery Professor of Neurosurgery and Pediatrics
New York-Presbyterian Hospital Department of Neurosurgery
Columbia University Medical Center University of Virginia Health System
Department of Neurological Surgery Charlottesville, Virginia
Morgan Stanley Children’s Hospital
Columbia University Medical Center Andrew Jea, MD
New York, New York Associate Professor
Department of Neurosurgery
William Y. Hoffman, MD, FAAP Baylor College of Medicine
Professor and Chief Staff Neurosurgeon
University of California–San Francisco Plastic Surgery Director, Neuro-Spine Program
Stephen J. Mathes Endowed Chair Director, Educational Programs
Vice Chair, Department of Surgery Texas Children’s Hospital
University of California–San Francisco Houston, Texas€
San Francisco, California
Dhruve Jeevan, MD, MA
Steven W. Hwang, MD Department of Neurosurgery
Assistant Professor€ The Hospital for Sick Children
Department of Neurosurgey University of Toronto
Tufts Medical Center Toronto, Ontario, Canada
Chief of Pediatric Neurosurgery
Floating Hospital for Children David F. Jimenez, MD, FACS
Boston, Massachusetts Professor and Chairman
Department of Neurosurgery
Tarik Ibrahim, MD University of Texas Health Science Center
Department of Neurological Surgery San Antonio, Texas
Loyola University
Chicago, Illinois Ignacio Jusue-Torres, MD
Salisbury Fellow
Bermans J. Iskandar, MD Department of Neurosurgery
Professor of Neurosurgery and Pediatrics The Johns Hopkins University School of Medicine
Director, Pediatric Neurosurgery Baltimore, Maryland
Department of Neurological Surgery
University of Wisconsin Hospital and Clinics Aimen S. Kasasbeh, MD, PhD
Madison, Wisconsin Neural Engineering Laboratory
Mayo Clinic
Jordan M. S. Jacobs, MD Rochester, Minnesota
Assistant Professor of Plastic Surgery
Department of Surgery Bruce A. Kaufman, MD
Mount Sinai Health System Professor
Director Department of Neurosurgery
Westchester Cleft and Craniofacial Teams Medical College of Wisconsin
New York, New York Chief, Pediatric Neurosurgery
Children’s Hospital of Wisconsin
George I. Jallo, MD Milwaukee, Wisconsin
Professor of Neurosurgery, Pediatrics and Oncology
Director, Pediatric Neurosurgery Robert F. Keating, MD
Department of Neurosurgery Professor and Chief
The Johns Hopkins University Division of Neurosurgery
Baltimore, Maryland Children’s National Medical Center
George Washington University School of Medicine
Washington, DC€
xxiv Contributors
Christopher David Kelly, MD Tobias Loddenkemper, MD
Department of Neurosurgery Director of Clinical Epilepsy Research
University Hospital Basel Division of Epilepsy and Clinical Neurophysiology
Basel, Switzerland Associate Professor
Department of Neurology
John Kestle, MD Boston Children’s Hospital
Professor and Vice Chair, Clinical Research Harvard Medical School
Department of Neurosurgery Boston, Massachusetts
University of Utah
Salt Lake City, Utah Thomas G. Luerssen, MD, FACS, FAAP
Chief, Pediatric Neurological Surgery
Mark W. Kieran, MD, PhD€€€€€€€€€€ Chief Quality Officer–Surgery
Director, Pediatric Medical Neuro-Oncology€€€€€€ Texas Children’s Hospital
Department of Pediatric Hematology/Oncology Professor of Neurological Surgery
Dana-Farber Cancer Institute Department of Neurological Surgery
Boston Children’s Hospital Baylor College of Medicine
Harvard Medical School Houston, Texas
Boston, Massachusetts
Joseph R. Madsen, MD
Paul Klimo Jr., MD, MPH Director, Epilepsy Surgery Program
Chief, Pediatric Neurosurgery Associate Professor of Neurosurgery
Le Bonheur Children’s Hospital Department of Neurosurgery
Department of Neurosurgery Boston Children’s Hospital
Semmes-Murphey Neurologic and Spine Institute Harvard Medical School
Memphis, Tennessee Boston, Massachusetts

Mark D. Krieger, MD Casey Madura, MD


Billy and Audrey Wilder Chair Department of Neurosurgery
Division of Neurosurgery University of Wisconsin Hospital and Clinics
Children’s Hospital Los Angeles Madison, Wisconsin
Professor, Department of Neurological Surgery
Keck School of Medicine Cormac O. Maher, MD
University of Southern California Associate Professor
Los Angeles, California Department of Neurosurgery
University of Michigan
Abhaya V. Kulkarni, MD, PhD, FRCS(C) Ann Arbor, Michigan
Professor and Neurosurgeon
Division of Neurosurgery Jeffrey C. Mai, MD, PhD
The Hospital for Sick Children Neurosurgeon
University of Toronto Inova Medical Group Neurosurgery
Toronto, Ontario, Canada Fairfax, Virginia

Jeffrey R. Leonard, MD, FAANS Conor Mallucci, MBBS, FRCS


Neurosurgeon-in Chief Consultant Paediatric Neurosurgeon€
Department of Pediatric Neurosurgery Department of Neurosurgery
Nationwide Children’s Hospital Alder Hey Children’s NHS Foundation Trust€
Professor Liverpool, England
Department of Neurological Surgery
The Ohio State Medical School Christian J. Cantillano Malone, MD
Columbus, Ohio Paediatric Neurosurgery and Epilepsy
Departamento de Neurocirugía€
Sheng-fu Larry Lo, MD, MHS Pontificia Universidad Católica de Chile
Department of Neurosurgery Hospital Sotero del Rio
The Johns Hopkins University Santiago, Chile€
Baltimore, Maryland
Contributors xxv
Salvador Manrique-Guzman, MD,€MSc Laura R. Ment, MD
Neurosurgeon Associate Dean and Professor
Department of€Neurosurgery€ Departments of Pediatrics and Neurology
ABC Medical Center Yale School of Medicine
Mexico City, Mexico New Haven, Connecticut

Timothy B. Mapstone, MD Thomas E. Merchant, DO, PhD


Wilkins Professor and Chairman Member and Chairman
Department of Neurological Surgery Department of Radiation Oncology
The University of Oklahoma Baddia J. Rashid Endowed Chair in Radiation
Health Sciences Center Oncology
Oklahoma City, Oklahoma St. Jude Children’s Research Hospital
Memphis, Tennessee
Arthur E. Marlin, MD, MHA
Professor of Neurosurgery Avinash Mohan, MD
Division of Pediatric Neurosurgery Assistant Professor of Neurosurgery and Pediatrics
University of South Florida Department of Neurosurgery
Tampa, Florida New York Medical College
Valhalla, New York
Craig D. McClain, MD, MPH
Senior Associate in Perioperative Anesthesia Karin Muraszko, MD
Assistant Professor of Anaesthesia Chair and Julian T. Hoff, MD, Professor, Neurological
Boston Children’s Hospital Surgery
Harvard Medical School Professor, Pediatrics and Communicable Diseases
Boston, Massachusetts Professor, Plastic Surgery
University of Michigan
J. Gordon McComb, MD Ann Arbor, Michigan
Chief Emeritus
Division of Neurosurgery Robert P. Naftel, MD
Children’s Hospital of Los Angeles Assistant Professor
Professor Department of Neurosurgery
Department of Neurological Surgery Vanderbilt University
University of Southern California Keck School Nashville, Tennessee
of Medicine
Los Angeles, California W. Jerry Oakes, MD€€€€€€€€€€€€€€€€€€€€€€€€€€
Professor of Neurosurgery and Pediatrics
David G. McLone, MD, PhD Surgeon-in-Chief
Chief Emeritus of Pediatric Neurosurgery Department of Neurosurgery
Children’s Memorial Hospital Children’s of Alabama
Professor Birmingham, Alabama
Northwestern University Feinberg School
of Medicine Jeffrey G. Ojemann, MD
Ann and Robert H. Lurie Children’s Hospital Professor of Neurological Surgery
of Chicago Richard G. Ellenbogen Chair in Pediatric
Chicago, Illinois Neurosurgery
Seattle Children’s Hospital
Gautam U. Mehta, MD Seattle, Washington
Department of Neurosurgery
University of Virginia Health System Brent O’Neill, MD
Charlottesville, Virginia Assistant Professor
Department of Neurosurgery
Arnold H. Menezes, MD University of Colorado School of Medicine
Professor and Vice Chairman Children’s Hospital Colorado
Department of Neurosurgery Aurora, Colorado
University of Iowa Hospitals and Clinics
Iowa City, Iowa
xxvi Contributors
Kaine C. Onwuzulike, MD, PhD Sandro Pelo, MD, PhD
Department of Neurosurgery Professor
University of Utah School of Medicine Chief, Department of Maxillo-Facial Surgery
Salt Lake City, Utah Catholic University Medical School
Rome, Italy
Darren Orbach, MD, PhD
Division Chief John A. Persing, MD€€€
Interventional and Neurointerventional Radiology Professor of Plastic Surgery
Boston Children’s Hospital Professor of Neurosurgery
Boston, Massachusetts Chief, Section€of Plastic Surgery
Department of Surgery
Irene P. Osborn, MD Yale University School of Medicine€
Associate Professor of Anesthesiology New Haven, Connecticut
Albert Einstein College of Medicine
Director, Division of Neuroanesthesia David W. Pincus, MD, PhD
Mountefiore Medical Center L. D. Hupp Professor of Pediatric Neurosurgery
Bronx, New York Department of Neurosurgery
University of Florida
Lauren Ostling, MD Gainesville, Florida
Clinical Instructor
Department of Neurological Surgery Jonathan A. Pindrik, MD
University of California–San Francisco School Assistant Professor
of Medicine Department of Neurosurgery
San Francisco, California Nationwide Children’s Hospital
The Ohio State University
Dachling Pang, MD, FRCS(C), FRCS(Eng), FACS Columbus, Ohio
Professor of Paediatric Neurosurgery
University€of€California–Davis Thomas A. Pittman, MD
Chief, Regional Centre for Paediatric Neurosurgery Professor
Kaiser Permanente Hospitals Department of Neurosurgery
Northern California University of Kentucky
Oakland, California Lexington, Kentucky

Srinivasan Paramasivam, MD, MRCS Ed Ian F. Pollack, MD, FACS, FAAP€


Assistant Professor Chief, Pediatric Neurosurgery€
Department of Neurosurgery–Cerebrovascular Program Children’s Hospital of Pittsburgh€
Mount Sinai Health System Leland Albright Professor of Neurological Surgery€
New York, New York Vice Chairman for Academic Affairs
Department of Neurological Surgery€
T. S. Park, MD Co-Director, UPCI Brain Tumor Program€
Neurosurgeon-in-Chief University of Pittsburgh School of Medicine€
St. Louis Children’s Hospital Pittsburgh, Pennsylvania
Shi H, Huang Professor or Neurological Surgery
Washington University Scott L. Pomeroy, MD, PhD
St. Louis, Missouri Chair, Department of Neurology
Neurologist-in-Chief
Christopher Parks, BSc, MBBS, FRCS(SN) Boston Children’s Hospital
Consultant Paediatric Neurosurgeon Bronson Crothers Professor of Neurology
Department of Paediatric Neurosurgery Director, Intellectual and Developmental Disabilities
Alder Hey Children’s NHS Foundation Trust Research Center
Liverpool, England Harvard Medical School
Boston, Massachusetts
Michael D. Partington, MD
Pediatric Neurosurgeon
Gillette Children’s Specialty Healthcare
St. Paul, Minnesota
Contributors xxvii
Juan Antonio Ponce-Gómez, MD Marc Remke, MD
Department of Neurosurgery Department of Pediatric Neuro-Oncogenomics
Lic. Adolfo López Mateos Hospital Department of Pediatric Oncology, Hematology, and
Institute of Security and Social Services of State Clinical Immunology
Workers University Children’s’ Clinic, and Department of
Mexico City, Mexico Neuropathology
Medical Faculty
Mark R. Proctor, MD, FAAP, FAANS Heinrich-Heine-University
Associate Professor of Neurosurgery Düsseldorf, Germany
Director of Craniofacial Surgery German Cancer Consortium and German Cancer
Boston Children’s Hospital Research Center
Harvard Medical School Heidelberg, Germany
Boston, Massachusetts
Francisco Revilla-Pacheco, MD, MBA, FACS
Corey Raffel, MD, PhD Neurosurgeon
Professor of Clinical Neurosurgery and Pediatrics Department of Neurosurgery€
Department of Neurological Surgery The American British Cowdray Medical Center
University of California–San Francisco Mexico City, Mexico
San Francisco, California
Renee M. Reynolds, MD
Ashley Ralston, MD Assistant Professor of Neurosurgery
Department of Neurosurgery University at Buffalo Neurosurgery
University of Chicago Pritzker School of Medicine Pediatric Neurosurgery
Chicago, Illinois Women and Children’s Hospital of Buffalo
Buffalo, New York
Vijay Ramaswamy, MD, PhD, FRCP(C)
Attending Neuro-Oncologist Jay Riva-Cambrin, MD, MSc
Division of Hematology/Oncology Associate Professor
The Hospital for Sick Children Department of Neurosurgery
Toronto, Ontario, Canada University of Utah
Salt Lake City, Utah
Javier González Ramos, MD
Neurosurgeon Elias Boulos Rizk, MD, MSc
Department of Neurosurgery Assistant Professor of Pediatric Neurosurgery
Hospital de Pediatría Prof. Dr. Juan P. Garrahan Department of Neurosurgery
Buenos Aires, Argentina Penn State University Hershey Medical Center
Hershey, Pennsylvania
Nathan J. Ranalli, MD
Assistant Professor of Neurosurgery and Pediatrics Shenandoah Robinson, MD, FACS, FAAP
Division of Pediatric Neurological Surgery Director of Functional Neurosurgery
University of Florida Health Science Center– Associate Professor of Neurosurgery and Neurology
Jacksonville Department of Neurosurgery
Wolfson Children’s Hospital Boston Children’s Hospital
Jacksonville, Florida Harvard Medical School
Boston, Massachusetts
Vijay M. Ravindra, MD
Department of Neurosurgery Caroline D. Robson, MB, ChB
Clinical Neurosciences Center Operations Vice Chair, Radiology
University of Utah Division Chief, Neuroradiology
Salt Lake City, Utah Department of€Radiology
Boston Children’s Hospital
Boston, Massachusetts
xxviii Contributors
Jonathan Roth, MD Spyridon Sgouros, MD, FRCS(SN)
Pediatric Neurosurgeon Head of Department
Department of Pediatric Neurosurgery Mitera Children’s Hospital
Dana Children’s Hospital Professor
Tel Aviv Medical Center University of Athens Medical School
Tel Aviv, Israel Athens, Greece

Benjamin A. Rubin, MD Ash Singhal, MD, FRCS(C)


Department of Neurosurgery Clinical Assistant Professor
New York University Langone Medical Center Pediatric Neurosurgeon
New York, New York Division of Neurosurgery
British Columbia Children’s Hospital
James T. Rutka, MD, PhD, FRCS(C), FACS, FAAP, Vancouver, British Columbia, Canada
FAANS
R. S. McLaughlin Professor and Chair Walavan Sivakumar, MD
Department of Surgery Department of Neurosurgery
Division of Neurosurgery Clinical Neurosciences Center
The Hospital for Sick Children University of Utah
University of Toronto Salt Lake City, Utah
Toronto, Ontario, Canada
Edward R. Smith, MD
Henry W. S. Schroeder, MD, PhD Co-Director
Professor and Chairman Cerebrovascular Surgery and Interventions Center
Department of Neurosurgery Director
University Medicine Greifswald Pediatric Cerebrovascular Surgery
Greifswald, Germany Department of Neurosurgery, Vascular Biology
Program
Daniel M. Schwartz, PhD Boston Children’s Hospital
Teaneck, New Jersey Harvard Medical School
Boston, Massachusetts
R. Michael Scott, MD
Professor of Neurosurgery Jodi L. Smith, PhD, MD, FAANS
Harvard Medical School John E. Kalsbeck Professor and Director of Pediatric
Fellows Family Chair in Pediatric Neurosurgery Neurosurgery
Neurosurgeon-in-Chief, Emeritus Riley Hospital for Children at Indiana
Department of Neurosurgery University Health
Boston Children’s Hospital Goodman Campbell Brain and Spine
Boston, Massachusetts€ Associate Professor of Neurological Surgery
Indiana University School of Medicine
Nathan R. Selden, MD, PhD Indianapolis, Indiana€
Campagna Chair of Pediatric Neurological Surgery
Director, OHSU Neurological Surgery Residency Matthew D. Smyth, MD, FAANS, FACS, FAAP
Program Professor of Neurosurgery and Pediatrics
Department of Neurological Surgery Director, Pediatric Epilepsy Surgery Program
Oregon Health & Science University Department of Neurosurgery
President, Congress of Neurological Surgeons Washington University
Chair, Committee on Resident Education St. Louis Children’s Hospital
Society of Neurological Surgeons St. Louis, Missouri
Portland, Oregon
Debbie K. Song, MD
Anthony K. Sestokas, PhD, DABNM, FASNM Pediatric Neurosurgeon€
Chief Clinical Officer Department of Neurosurgery
Department of Intraoperative Neuromonitoring Gillette Children’s Specialty Healthcare
SpecialtyCare St. Paul, Minnesota
Nashville, Tennessee
Contributors xxix
Sulpicio G. Soriano, MD, FAAP Gianpiero Tamburrini, MD
BCH Endowed Chair in Pediatric Neuroanesthesia Professor
Professor of Anaesthesia Department of Pediatric Neurosurgery
Departments of Anesthesiology, Perioperative and Institute of Neurosurgery
Pain Medicine€ Catholic University Medical School
Boston Children’s Hospital Rome, Italy
Harvard Medical School
Boston, Massachusetts Robert C. Tasker, MA, MD (Cantab); MBBS (Lond);
DCH, FRCPCH,€FRCP,€FHEA (UK); AM (Harvard)
Robert F. Spetzler, MD Professor of Neurology
Director, Barrow Neurological Institute Professor of Anaesthesia (Pediatrics)
J. N. Harber Chairman and Professor of Neurological Chair in Neurocritical Care
Surgery Boston Children’s Hospital
Division of Neurological Surgery Senior Associate Staff Physician
Barrow Neurological Institute Department of Neurology
Phoenix, Arizona Department of Anesthesiology, Perioperative and
Pain Medicine
David A. Staffenberg, MD, DSci (Hon), FACS Division of Critical Care Medicine
Vice Chair, Department of Plastic Surgery Harvard Medical School
Chief, Division of Pediatric Plastic Surgery Boston, Massachusetts
Professor of Plastic Surgery, Neurosurgery, and
Pediatrics Michael D. Taylor, MD, PhD, FRCS(C)
Department of Plastic Surgery Garron Family Chair in Childhood Cancer Research
New York University Langone Medical Center The Hospital for Sick Children
New York, New York Professor of Surgery
Division of Neurosurgery
Derek M. Steinbacher, DMD, MD, FACS, FAAP University of Toronto School of Medicine
Associate Professor Toronto, Ontario, Canada
Director of Craniofacial Program
Plastic and Maxillofacial Surgery George H. Thompson, MD
Yale University School of Medicine Director, Pediatric Orthopaedic Surgery
New Haven, Connecticut Rainbow Babies and Children’s Hospital
University Hospitals Case Medical Center
Jordan P. Steinberg, MD, PhD Professor, Orthopaedic Surgery and Pediatrics
Pediatric Craniofacial Surgery Case Western Reserve University
Children’s Healthcare of Atlanta Cleveland, Ohio
Atlanta, Georgia
Michael E. Tobias, MD
Paul Steinbok, MBBS, FRCS(C) Co-Chief of Pediatric Neurosurgery
Professor Maria Fareri Children’s Hospital
Department of Surgery Valhalla, New York
British Columbia Children’s Hospital Assistant Professor of Neurosurgery
University of British Columbia New York Medical Center
Vancouver, British Columbia, Canada Hawthorne, New York

Hai Sun, MD, PhD Vassilios Tsitouras, MD


Assistant Professor Neurosurgeon
Department of Neurological Surgery Department of Pediatric Neurosurgery€
Louisiana State University Health Sciences Mitera Children’s Hospital€
Center–Shreveport Athens, Greece
Shreveport, Louisiana
R. Shane Tubbs, PhD
Professor
Division of Pediatric Neurosurgery
Children’s Hospital of Alabama
Birmingham, Alabama
xxx Contributors
Elizabeth C. Tyler-Kabara, MD, PhD John “Jay” C. Wellons III, MD, MSPH
Associate Professor Chief of Pediatric Neurosurgery
Department of Neurological Surgery Professor of Neurosurgery and Pediatrics
University of Pittsburgh Department of Neurosurgery
Pittsburgh, Pennsylvania Vanderbilt University Medical Center
Monroe Carell Jr. Children’s Hospital at Vanderbilt
Sudhakar Vadivelu, DO Nashville, Tennessee
Co-Director, Cerebrovascular Program
Assistant Professor of Neurosurgery and Radiology William E. Whitehead, MD
Division of Pediatric Neurosurgery Associate Professor
Cincinnati Children’s Hospital Medical Center Department of Neurosurgery
Cincinnati, Ohio Baylor College of Medicine
Texas Children’s Hospital
Timothy W. Vogel, MD Houston, Texas
Assistant Professor
Division of Pediatric Neurosurgery€ Thomas J. Wilson, MD
Division€of Developmental Biology Department of Neurosurgery
Cincinnati Children’s Hospital€ University of Michigan
University of Cincinnati Ann Arbor, Michigan
Cincinnati, Ohio
Jeffrey H. Wisoff, MD
Arthur Wang, MD Professor of Neurosurgery and Pediatrics
Department of Neurosurgery Director, Division of Pediatric Neurosurgery
New York Medical College New York University Langone Medical Center
Westchester, New York New York, New York

Benjamin C. Warf, MD Peter Albert Woerdeman, MD, PhD


Hydrocephalus and Spina Bifida Chair Pediatric Neurosurgeon
Boston Children’s Hospital Department of Neurosurgery
Associate Professor of Neurosurgery University Medical Center Utrecht
Harvard Medical School Wilhelmina Children’s Hospital
Boston, Massachusetts Utrecht, The Netherlands€

Andrew Paul Warrington, ECNE Sui-To Wong, MBBS, MMedSc, FHKAM, FRCSEd
Senior International Clinical Specialist Consultant Neurosurgeon
Intraoperative Neurophysiology Department of Neurosurgery
Medtronic Tuen Mun Hospital
Rochester, New York Hong Kong, China

Michael Weicker, MD Edward Yang, MD, PhD


Four Corners Spine and Pain Staff Neuroradiologist
Farmington, New Mexico Department of Radiology
Boston Children’s Hospital
Alexander G. Weil, MD, FRCS(C) Boston, Massachusetts
Assistant Professor
Department of Surgery Jonathan Yun, MD
Division of Pediatric Neurosurgery Department of Neurological Surgery
Sainte-Justine University Hospital Center Columbia-New York Presbyterian Hospital
University of Montreal New York, New York
Montreal, Quebec, Canada
Graciela Zuccaro, MD, PhD
Howard L. Weiner, MD Head
Professor of Neurosurgery and Pediatrics Department of Neurosurgery
Division of Pediatric Neurosurgery Children’s Hospital Juan P. Garrahan
Department of Neurosurgery Professor of Neurosurgery
New York University Langone Medical Center Buenos Aires University
New York, New York Buenos Aires, Argentina
Section I
Introduction
Section Editor: Tae Sung Park

This section covers a wide range of fundamental issues erative period, such as causes of delayed emergence
in pediatric neurosurgery and contains seven chapters. from anesthesia, choice of intravenous fluids for dif-
Chapter 1, “Basic Surgical Technique,” provides ferent age groups, and management of hyponatremia
steps of preoperative and operative planning and and hypernatremia.
execution of surgery in the operating room. Dr. Chapter 5 is devoted to the positioning of chil-
Cohen, the editor of this textbook, emphasizes the dren during surgery. It includes descriptions of spe-
importance of sound surgical judgment, setting the cial cautions in rigid cranial immobilization, supine
tone in the operating room, and the goal of getting positioning for specific common operative proce-
the patient safely and expeditiously through the sur- dures, prone positioning for tumor surgery, and
gery and out of the operating room. Other operative lateral positioning for lumboperitoneal shunt and
details of neurosurgery are also provided. baclofen pump insertion.
Chapter 2 addresses common diagnostic and ther- Chapter 6 addresses intraoperative neurophysi-
apeutic procedures: assessment of shunts, lumbar ological monitoring in children. It not only provides
puncture, external ventricular catheter placement, details of various monitoring techniques, but also
and subdural taps. In addition to operative detail discusses tailored monitoring for specific procedures.
and preoperative planning, the authors describe the Chapter 7 addresses surgical safety. The chapter
equipment needed and provide expert suggestions. focuses on three major and related strategies derived
Chapter 3 covers important areas of pediatric from highly reliable organizations, which have gained
neuroanesthesia, such as special equipment, vascular widespread acceptance: the development of a culture
access and positioning, management of fluids, and of safety, the creation of effective surgical teams, and
blood loss. The authors also address the anesthetic the use of communication support tools—specifically,
considerations for specific neurosurgical procedures. checklists and handoff scripts in patient care.
Chapter 4, “Pre- and Postoperative Management This section, then, provides the reader with a
of the Neurosurgical Patient,” starts with a review of comprehensive overview of basic principles that
cerebrovascular and CSF physiology. It ends with a should be followed for the practice of safe and suc-
review of common clinical problems in the periop- cessful pediatric neurosurgery.
1 Basic Surgical Technique
Alan R. Cohen

If an operation is difficult, you are not doing it properly.


Robert E. Gross, MD (1905–1988)
Surgeon-in-Chief, Boston Children’s Hospital

1.1╇ Introduction and Background 1.1.2╇ Setting the Tone

1.1.1╇Overview The operating room (OR) is a theater, and the stage


should be set before the patient enters the room. The
Attention to basic surgical technique is of paramount patient is the focus of all activity, and the OR staff and
importance to the success of the simplest of proce- equipment should be positioned to maximize efficiency
dures as well as the most complex. A cavalier sur- and flow. Once the patient has been anesthetized,
geon can turn a beautiful operation into a disastrous meticulous attention is paid to positioning. Pressure
misadventure in a split second. The proper use of points are padded, and the region of interest is gener-
instruments and the careful, meticulous handling of ally placed at the highest point in the field. Overhead
tissue during dissection are essential skills that must lighting is adjusted, and the operator will often put on a
be mastered by all successful surgeons. But the real headlight and loupes. The operating microscope is bal-
key to successful surgery is preoperative planning, anced, and ancillary equipment is readied for use.
which, by definition, begins well before the patient The tension is often high in the operating room,
enters the operating room. and the surgeon should attempt to set the tone and
Preoperative planning mandates sound surgi- relax the room. The surgical team will function bet-
cal judgment, which can be an elusive skill. Simply ter when the members are calm and know what is
because an operation is feasible doesn’t necessarily expected of them. The attitude of the surgeon will
mean it should be done. According to an old Hebrew set the tone for the entire team.
proverb, the art of surgery is the ability to use supe- The more a surgical procedure can be standard-
rior judgment to avoid having to use superior skills. ized, the better. Standardization improves efficiency
Is surgery necessary in the first place? What is the and reduces the chance for error. Immediately before
purpose of the operation? Are there alternative incision is made, a “time-out” is performed by the
measures that could avoid surgery and its attendant entire operative team according to predetermined
risks? These are all issues of surgical judgment. checklists (Fig. 1.1). Checklists help the surgeon
The surgeon planning an operation is like the gen- ensure the safety of a procedure, much as they help
eral preparing for combat. Sometimes the greatest the airline pilot ensure the safety of a flight.
warrior is one who can manage a conflict without A preprocedure timeout entails correct identifi-
fighting. cation of the patient, the procedure, and the opera-
This chapter focuses on the basic surgical tech- tive site. For lateralized procedures, the operative
niques involved in carrying out a craniotomy in a site is marked unambiguously before the patient
child. Some technical considerations are unique, enters the OR. If possible, the patient or family
such as the rich vascularity of the scalp. Other prin- should participate in the preoperative site marking.
ciples can be generalized to other neurosurgical pro- A standardized checklist is used and should include
cedures, such as those performed on the spine, spinal information about relevant history, physical find-
cord, and peripheral nerves. ings, lab results, and imaging studies. Confirmation
is made that there is a signed consent. Medication
allergies should be noted. Anticipated risks are dis-
cussed, along with whether blood is available if a
transfusion is expected. Special equipment for the
procedure should be reviewed, and medications,

3
4 Section Iâ•… Introduction
out the entire case. Placing the head in a dependent
position, for example, can increase venous pressure
and bleeding. Awkward twisting of the neck can lead
to jugular vein compression and increase the risk of
bleeding.
The head is supported on a doughnut or padded
horseshoe or carefully fixed in a pinion head holder.
Positioning also applies to the surgical team. The
assistant surgeon and scrub nurse should be placed
where they can be of maximal benefit. There should
be adequate space for equipment, such as the oper-
ating microscope, image guidance systems, and
neurophysiologic monitoring. If endoscopy is used,
the video screen should be placed such that the key
members of the team can see it without straining
Fig. 1.1â•… The entire operative team participates in the pre- their necks. The overhead lighting is brought in at
procedural timeout, which is performed according to a stan- different angles to maximize brightness on the oper-
dard checklist protocol. ative field. The surgeon will often wear a headlight
and loupes.

such as antibiotics, antiepileptics, and steroids,


should be discussed.
Flow
The names of individuals on the procedure team
A great operation is like a carefully orchestrated bal-
are recited, including the surgeon, assistant surgeon,
let. The surgeon choreographs the procedure and
anesthesiologist and assistant, scrub nurse, circulat-
must pay scrupulous attention to the flow of the case.
ing nurse, OR technicians, and others who will be
Smooth flow is a hallmark of a successful opera-
participating in the case. The names of those partici-
tion. The surgeon should always be thinking two or
pating in the case are also written on a board view-
three steps ahead, anticipating which instruments
able to all in the room. The act of having individuals
will be needed. The best operations are not ones in
recite their names out loud may seem childlike, but
which the surgeon acts in a rushed fashion, but ones
it actually enables team members to identify one
that flow smoothly from one step to the next. Mem-
another and develop a rapport. In a time of crisis, it
bers of a team who have worked together for some
is much easier for members of the team to speak up
time get to know each other’s style. A good surgeon
if they know one another’s names. A second timeout
will always have an instrument in each hand and will
is performed before final closure of the wound.
rarely take his eyes off the operative field. When ask-
ing for an instrument, the surgeon holds up a hand
1.1.3╇Goals without looking away (Fig. 1.3). A good assistant will

From the moment the patient is wheeled into the OR,


every action of every individual on the team should be
directed toward getting the patient safely and expedi-
tiously through the procedure and out of the OR.

1.2╇ Operative Detail and


Preparation
1.2.1╇ Preoperative Planning
Positioning
Fig. 1.2â•… The “military tuck” position for a posterior fossa cra-
The importance of patient positioning cannot be over- niotomy. The head of the bed is elevated, and the child is care-
stated (Fig. 1.2). Improper positioning can cause the fully fixed in the pinion head holder such that the neck is flexed
surgeon and the surgical team to struggle through- but lifted somewhat so that it is parallel to the floor.
1â•… Basic Surgical Technique 5
facilitate lining up the incision properly for closure.
The incision is then walled off with cloth towels
and covered by an iodoform-impregnated adherent
drape. The scalp is then infiltrated with a dilute solu-
tion of bupivacaine or xylocaine with epinephrine
(1:200,000 dilution) to help minimize blood loss.

1.2.2╇ Key Steps and Operative Nuances


Scalp Incision

The incision should be designed to avoid interrupt-


ing the major arterial supply to the scalp. If future
surgery is considered, the incision should be planned
so that it could be extended to form a new incision
if necessary (e.g., conversion of a burr hole to a cra-
niotomy). Whenever possible, the incision should be
designed so as not to sit directly over subcutaneous
hardware, such as shunt valves and reservoirs.
The rich vascularity of a child’s scalp facilitates
wound healing but can also lead to significant blood
loss during surgery. The operator takes advantage of
the fact that the scalp sits directly over the firm skull.
The operator’s nondominant hand is placed firmly
on one side of the incision with the fingers spread.
The assistant’s hand is placed on the opposite side.
Fig. 1.3â•… Economy of movement. The surgeon’s eyes are fixed Because the scalp overlies the firm skull, this pres-
on the operative field as the scrub nurse passes instruments to
sure serves to control bleeding. This pressure mini-
the surgeon’s raised hands.
mizes bleeding from the scalp during incision.
The operator holds the knife as if it were a pen,
with the index finger on top to permit graduated
downward pressure on the blade (Fig. 1.5). The inci-
anticipate the surgeon’s needs and have comple-
mentary instruments in the field. A good scrub nurse
can foresee which instrument the surgeon will need
next and will often have it ready without ever having
been asked.
The finest surgeons practice economy of move-
ment. One action gently blends into the next, and
nothing appears hurried. Every action has a purpose
that was thought out in advance, and there are no
unnecessary maneuvers. With each step, the surgeon
anticipates the worst-case scenario. What could go
wrong? What pitfalls might appear? What operative
catastrophes must be avoided? This frame of mind
allows the surgeon to stay on course but also to act
calmly and confidently should a crisis occur.

Preparation and Draping

There are various methods for preparing the skin


prior to incision. I prefer to clean the skin with iso-
propyl alcohol and prep with povidone-iodine scrub
and solution. I mark the incision with a sterile felt Fig. 1.4â•… The incision is marked after the skin is prepped but
pen after the field is dry but before the drapes are before the drapes are placed so that anatomical landmarks can
placed (Fig. 1.4). This allows me to see available ana- be seen clearly. The area is surrounded by towels and covered
tomical landmarks. Cross-hatch marks are drawn to with an iodoform-impregnated band.
6 Section Iâ•… Introduction
It is a good practice to leave the pericranium
attached to the skull to minimize blood loss. For
frontal craniotomies that may traverse the frontal
sinuses, a vascularized pericranial flap can be har-
vested for later mobilization (Fig. 1.7). This flap is
based on the supraorbital arteries. Alternatively, a
vascularized temporalis fascial flap can be mobilized.
For closure, the frontal sinus is exenterated and filled
with a graft of abdominal fat and fibrin glue before
being covered by the vascularized pericranial graft.
For a midline posterior fossa exposure, it is essen-
tial to identify the avascular ligamentum nuchae.
Veering off to one side can lead to significant blood
loss from the vascular strap muscles of the neck.
One trick to keep on the midline is to use a hemo-
stat forceps to spread the tissue, looking for the slight
angulation of the muscle fibers on either side of the
midline. Dissection through the ligamentum nuchae
Fig. 1.5â•… The scalp is incised with a no. 15 blade knife. The can be performed with virtually no blood loss.
knife is held like a pen in the dominant hand. The incision is
made smoothly with the belly of the knife. To minimize bleed-
ing, pressure is applied to either side of the proposed incision Craniotomy
by the nondominant hand of the surgeon on one side and the
assistant on the other.
In the modern era of power tools, some may not be
familiar with the old-fashioned manual technique for
performing a craniotomy. Hand-driven tools still have
sion is made smoothly with the belly of the blade. a role in neurosurgery, particularly for making burr
The scalp is opened only as far as the region that can
be compressed by the fingers of the operator and
assistant. Once the subcutaneous tissue is mobi-
lized slightly, it is grasped with toothed forceps and
everted for application of spring clips (Fig. 1.6). I pre-
fer low-profile clips, such as baby Raney clips. I apply
the Raney clips such that the superficial (external) lip
of the clip is placed first, and then the deep (internal)
lip is rolled into place to incorporate the full thick-
ness of the scalp in the clip. Placing the superficial
lip of the clip first prevents the clip from causing the
iodoform-impregnated drape to slide off the scalp.
Such spring clips are used sparingly in young infants
because they can exert significant pressure on the
scalp. Alternatively, a monopolar coagulator can be
used to divide the subcutaneous tissue.
Excessive suctioning of the soft tissues should be
avoided as it can stir up more bleeding. Scalp bleed-
ing is controlled by bipolar or monopolar coagulation.
The bleeding site can often be identified by applying
and removing a gauze sponge with the nondominant
hand. Suction can be used if excess bleeding pools up
in the wound. Suction is essential to keep the field
dry when using the monopolar coagulator, as this
device will not work in a wet field.
For a pterional flap, I generally divide the tempo-
ralis fascia sharply with a knife and use monopolar
Fig. 1.6â•… Scalp clips can be applied to control bleeding.
cautery to go through the muscle. Excessive cauter- The scalp edge is held up with toothed forceps, and the clip
ization of the temporalis muscle can lead to atrophy, is applied from the outside first to prevent dislodging the
but in a young child the paramount focus is preven- iodoform-impregnated drape from the skin. In young infants,
tion of blood loss. A myocutaneous flap can be held Raney clips should be used judiciously and protected by a
under tension with fish hooks. gauze sponge to prevent pressure necrosis of the scalp.
1â•… Basic Surgical Technique 7

a b

Fig. 1.7â•… The scalp can be mobilized in the supra-pericranial plane to minimize bleeding from the skull. In selected cases, a vascular-
ized pericranial flap can be mobilized and covered with a moistened gauze sponge for later use in covering an exposed frontal sinus.
(a) Before mobilization. (b) After mobilization.

holes in the young child. The skull of a young child or brace, and the operator applies pressure on the brace
infant can be quite thin, and use of a power perforator handle while turning the perforator in a clockwise
must be done with care to avoid the risk of plunging. fashion. Care must be taken not to plunge. When the
A hand-driven McKenzie perforator can be used tip of the perforator penetrates the inner table of the
to make burr holes in a young child, and it is an skull, it generates a resistance, causing the perforator
acceptable way to make burr holes in an older child to “catch.” The perforator is removed and a round or
if power instruments are not available (Fig. 1.8a). pineapple-shaped burr is fixed in the Hudson brace
The McKenzie perforator is attached to a Hudson in order to widen the burr hole (Fig. 1.8b). Unlike

a b

Fig. 1.8â•… Drilling the burr hole with hand instruments. (a) The initial hole is made with a McKenzie perforator attached to a Hudson
brace. (b) The hole is widened with a burr attached to a Hudson brace.
8 Section Iâ•… Introduction
the perforator, this burr does not catch. To prevent dissector, such as a dental tool, Penfield no. 3 dis-
plunging, the operator should lift the instrument sector, or Gigli saw guide. To prevent excess dural
after several turns to assess the progress of the open- bleeding, the operator should strip sparingly, and
ing. The site is irrigated by the assistant. Bone bleed- only in areas overlying proposed cuts. In many cases,
ing during the burr hole creation can be controlled dural stripping is not necessary. If the dura is pen-
by administering bone wax and drilling through it. etrated during stripping at one burr hole, the bone
Bone wax can be used sparingly to stop bleeding cut should be made from another burr hole toward
after the hole has been made. In young infants, extra the site that was torn.
care must be taken because the McKenzie perforator The bone cuts to connect the burr holes are made
may penetrate the skull during a single turn of the with a power drill and footplate (e.g., Midas Rex B-1
brace. A small opening made by the perforator can be bit). The bone cuts are beveled slightly outward to
expanded using curettes and a Kerrison punch. help prevent sinking of the flap when it is replaced,
More commonly, the burr hole is made with power though this is less of a concern with the current
instruments. An acorn bit can be used with a Midas use of plates and screws to resecure the bone. Cuts
Rex drill (Medtronic, Minneapolis, MN, USA). Care should be made from the outer edge of one burr hole
must be exercised to prevent laceration of the dura, to the outer edge of the next in order to maximize
because the acorn can sometimes jump due to “chat- the size of the craniotomy.
ter” created by the power drilling process. A power The drill is held with two hands, and a gen-
perforator is effective in creating a nice round burr tle forward pressure is applied to cut the bone
hole. The power perforator has a clutch that should (Fig.€1.10). Depressing the dura a millimeter or so
stop the drill from spinning once the inner table has helps engage the bit properly to make a smooth
been violated. This safety measure cannot be guaran- cut. If the drill gets stuck, the operator can gen-
teed, so the operator uses the nondominant hand as tly angle the instrument forward and backward
a brace to prevent plunging (Fig. 1.9). Smaller power to help get back on track. It is important to keep
perforators are available for use in infants. the drill properly aligned to prevent it from get-
Once the holes are made, the dura can be gently ting stuck. The assistant irrigates the field, and the
stripped from the undersurface of the bone with a bone dust is collected and placed in a moistened

Fig. 1.9â•… The burr hole is commonly made using a power Fig. 1.10â•… The craniotomy is elevated using a high-speed drill
perforator that has a clutch to help prevent plunging. The and footplate. Depressing the footplate about a millimeter helps
surgeon’s nondominant hand is used to provide tactile feed- make the cut smoother. Rocking the drill forward and backward
back and serve as a counterbrace in the event the instrument can help prevent it from getting stuck. The cuts are made to
plunges. The bone dust is collected in a moist cup for replace- allow maximal exposure of the craniotomy. The cuts can be bev-
ment during closure. eled to prevent sinking of the bone flap, though this is less of
a problem when the bone is resecured with plates and screws.
1â•… Basic Surgical Technique 9
is raised, centered on the pterion (Fig. 1.12). If more
frontal exposure is indicated, the bone flap can be
tailored and extended more anteriorly. The inferior
cut is made last, as the thickened bone over the lat-
eral sphenoid wing can be difficult to cut across and
sometimes needs to be cracked. Epidural bleeding
is controlled with the bipolar forceps. Bone bleed-
ing is controlled with wax. The lateral edge of the
sphenoid wing can be removed with rongeurs and
shaved down with a drill to enhance the exposure. In
selected cases, the orbital roof can be removed or an
orbitozygomatic approach can be fashioned to gain
lower exposure. Such exposures can be particularly
helpful for approaching lesions that extend superi-
orly, such as a craniopharyngioma going up high to
the third ventricle. The lower bone removal allows
a b the operator a more direct approach to high lesions
with less retraction of brain.

Interhemispheric Transcallosal Craniotomy


The interhemispheric approach to the anterior ven-
tricular system can be performed through a variety
Fig. 1.11â•… Hand instruments can be used to elevate the cra- of scalp incisions. I prefer a bicoronal incision and
niotomy. (a) A Gigli saw guide is carefully passed beneath the use a quadrilateral bone flap whose medial extent is
bone in the epidural space from one burr hole to another. (b) directly on the midline (Fig. 1.13). For such a midline
The Gigli saw is attached to the guide, and the operator sweeps approach, I place the most posterior midline burr
the saw back and forth at a shallow angle of about 30 degrees hole about 1 cm behind the bregma and the most
above the skull on each side until each cut is completed. anterior hole about 5 or 6 cm in front of the bregma.
Lateral holes are made 4 to 5 cm off the midline. A
useful anatomical guide is that a line drawn from the
cup for replacement during closure. Examples of
several craniotomies are listed below.
Bone cuts can also be made with hand-held tools.
A Gigli saw guide is passed gently under the bone
from one burr hole to another. The Gigli saw is a
twisted wire with an eyelet that can be attached to
a hook on the guide, facilitating passage of the saw
under the bone. The saw is then held with handles or
clamps in the surgeon’s two hands, and the bone is
cut from inside out with back-and-forth movements
while the guide is left in place to protect the dura
(Fig. 1.11a,b).

Pterional Craniotomy
The pterional craniotomy is fashioned using a curvi-
linear scalp incision beginning in front of the tragus
and extending to the midline, just behind the hair-
line. A myocutaneous flap is reflected forward and
held in place with fish hooks. The bone flap is made
with two burr holes. The posterior hole is positioned
over the low squamous temporal bone just above the Fig. 1.12â•… Pterional craniotomy. The bone flap is fashioned
posterior margin of the zygoma. The anterior hole is with two burr holes: one at the squamous temporal bone above
placed in the keyhole region. An elliptical craniotomy the posterior zygoma and the other in the keyhole region.
10 Section Iâ•… Introduction

Midline Posterior Fossa Craniotomy


The midline posterior fossa craniotomy is a stan-
dard workhorse in pediatric neurosurgery. The
occipital bone is exposed through a vertical mid-
line incision that is kept open with automatic
retractors. The foramen magnum is identified
and cleared of soft tissue with curettes. For low
approaches that require removal of the posterior
arch of C1, I expose the rostral posterior arch of C2
and work toward the region of the foramen mag-
num and C1 going from above downward and from
below upward, trying to prevent premature entry
into the atlanto-occipital membrane and dura. One
must exercise caution working laterally at C1 to
avoid injuring the vertebral arteries and the para-
vertebral venous plexus.
Fig. 1.13â•… Craniotomy for a midline interhemispheric trans- The craniotomy is fashioned using two burr
callosal approach. The medial burr holes are placed last, holes. The burr holes are placed just inferior to the
directly on the midline. Bone cuts are made going away from transverse sinus, with each hole placed 1 to 2 cm on
the midline. The midline is carefully stripped, and midline cuts either side of the midline (Fig. 1.14). The lateral cuts
are made last. The dura is reflected medially to protect the are made first and extend inferiorly into the fora-
sinus. men magnum. The bone is thick at the lateral mar-
gins of the foramen magnum, so the drill is brought
more medially to enter the foramen. Exposure at
bregma to the external auditory meatus will bisect the foramen magnum can be widened with ron-
the foramen of Monro. geurs and Kerrison punches after the bone flap has
For the transcallosal approach, the midline burr
holes over the superior sagittal sinus are placed last,
and the midline is stripped last. The thinking behind
this is that the operator must always anticipate the
worst-case scenario (e.g., sagittal sinus tear) and pre-
pare for it. Drilling is always performed in a direc-
tion away from the venous sinuses. If the sinus is
injured, the bone flap can be elevated rapidly if the
other cuts have already been made. Sinus injury can
result in torrential bleeding. The bleeding site must
be properly exposed. The head of the bed should be
elevated. Sometimes bleeding can be controlled with
thrombin-soaked gelfoam and pressure. Larger tears
may need to be covered with muscle and oversewn
with a dural reflection.
Some surgeons expose the superior sagittal sinus
by placing burr holes on either side of it. I place burr
holes directly over the sinus and strip gently with
a dental tool, followed by a Penfield no. 3, followed
by a Gigli saw guide. If stripping is not easy, I will
add more burr holes over the midline. Once the bone
flap is off, visualization of midline structures can be Fig. 1.14â•… Midline posterior fossa craniotomy. The foramen
facilitated by using an acorn bit to bevel the native magnum is cleared with curettes. Two burr holes are made just
bone along the midline and to permit a more medial inferior to the transverse sinus, each just lateral to the midline.
reflection of the dura. In reflecting the dura medially, Bone cuts are made from the burr holes extending laterally
care must be taken to avoid occluding the superior and inferiorly to the foramen magnum. The midline cut con-
sagittal sinus. necting the two burr holes is made last. Rostral to the left.
1â•… Basic Surgical Technique 11
been removed. If the operator has difficulty reach- bleeding site could be identified and controlled once
ing the foramen magnum from above, the drill can the dura is opened. Dural tenting sutures should be
be removed and cuts can be made starting from the used sparingly because they can shrink the dural
foramen magnum going upward. surface available for closure. In some cases, such as
The final cut across the midline is made last. The a craniotomy for epidural hematoma, multiple dural
dura is stripped from the undersurface of the bone tenting sutures are a very effective way of obtain-
with dissectors. The operator uses the drill to go part- ing hemostasis and obliterating the epidural space.
way from one side and then completes the cut going In selected cases, such as an epidural hematoma, a
from the other side. The bone over the midline has a central dural tack-up suture can be used to fasten the
keel that can be quite thick, and frequently there are dura to drill holes placed in the center of the bone
underlying emissary veins that need to be controlled. flap, helping to obliterate the epidural space.
Bone bleeding is controlled with wax. The craniotomy Several steps are taken before the dura is incised.
can be widened as necessary with rongeurs. Appropriate instruments are organized. Microsuc-
If superior exposure is necessary (e.g., for an tion devices are hooked up. If a retraction system is
infratentorial supracerebellar approach), the opera- to be used, it is set in place. For a vascular case, such
tor uses an acorn drill bit to bevel the bone overly- as an aneurysm or arteriovenous malformation, clips
ing the transverse sinuses. This maneuver permits a are brought out and loaded.
little more rostral reflection of the rostral dural leaf- The dura is opened with a no. 15 blade knife on
lets, which can aid significantly in the exposure over a long handle, using gentle, repetitive cuts about a
the top of the cerebellum. centimeter in length to deepen the durotomy until
the arachnoid is visible. The dura can be held up with
a fine-toothed forceps or a fine hook. The dural open-
Dural Opening ing is extended by cutting along the groove of a den-
tal instrument placed underneath the dura by the
Epidural bleeding can be controlled by tenting the assistant surgeon. Alternatively, the dural opening is
dura to drill holes placed around the margins of extended using Metzenbaum scissors (Fig. 1.16). The
the craniotomy with 4–0 Nurolon sutures (Ethi- operator looks underneath the dura to ensure that
con, Somerville, NJ, USA; Fig. 1.15). Tenting sutures there are no bridging veins in the way. If the brain
should be applied prior to durotomy, because if a
cortical vessel were to be injured by the needle, the

Fig. 1.16â•… The dura is opened initially with a no. 15 blade


knife. The dural incision can be extended by using the blade
to cut over a dental instrument or with Metzenbaum scis-
Fig. 1.15â•… The dura is tented to the margins of the craniotomy sors. The scissors are beveled to prevent excess pressure on
with 4–0 Nurolon sutures. Tenting is done prior to durotomy. the brain. The dural flap can be held under tension with 4–0
The tenting sutures are applied close to the bone to minimize Nurolon sutures and covered with a moist collagen sponge to
shrinkage of the dura. minimize shrinkage.
12 Section Iâ•… Introduction
is tight, the scissors are held at a flat angle, nearly microfibrillar collagen film, such as Bicol (Codman
parallel to the dura, in order to prevent the internal Neuro, Raynham, MA, USA), or a wet gauze sponge.
blade of the scissors from injuring the brain.
The durotomy is planned to maximize exposure
and minimize bleeding. In the posterior fossa mid- Brain Manipulation and Dissection
line, the dura is opened in a Y shape to help control
bleeding from the occipital sinus (Fig. 1.17). Such If the brain is to be retracted, it is retracted gently.
bleeding can be troublesome, particularly in young Automatic brain retractors should be placed in a low
children, who may have large venous lakes in the profile so that the operator’s hands can be as close to
central portion of the posterior fossa dura. Bleeding the wound as possible. If the brain is to be entered,
can be effectively controlled using silver clips, with it is also done gently. For a sulcal approach, the pia
care taken to include both the inner and outer layers mater is opened sharply and gentle dissection is car-
of dura in the clips (Fig. 1.17). Occipital sinus bleed- ried out under microsurgical guidance. For a gyral
ing can also be sealed with bipolar coagulation. approach, the corticectomy can be made in a lin-
Other dural openings are tailored to the specific ear or circular fashion. The surgeon tries to find the
site. U-shaped openings can be reflected toward the shortest route to the lesion, but plans may need to
location of venous sinuses. This reduces the chance be modified to avoid traversing eloquent structures.
of lacerating bridging veins during durotomy and The portion of exposed brain that is not part of the
can also help to tamponade sinus bleeding should operative trajectory is covered with a moist collagen
it occur. The dural incision should be at least a cen- sponge and kept moist with irrigation throughout
timeter away from the adjacent bone edge to allow the procedure.
the operator enough room for dural closure. I make Microsurgery is best performed with the surgeon
the turns in the U at right angles to help me find the sitting in a comfortable position. Arm rests help pre-
appropriate landmarks for closing the dura. Linear or vent fatigue and facilitate fine microsurgical manip-
cruciate openings are used in selected cases. ulation. When available, a mouthpiece attached to
The dura is held open with retraction sutures of the microscope enables the surgeon to make fine
4–0 Nurolon. The dural flap is covered with a moist adjustments during the procedure without slowing
the flow.
The operative field is kept dry with suction and
bipolar coagulation. The suction tip is used gently to
clear the field and identify bleeding sites, which are
coagulated with the bipolar forceps. Some bleeding
can be controlled with pressure from a Cottonoid
(Codman Neuro, Raynham, MA, USA) patty placed
over the torn vessel with the bipolar forceps. The
patty is gently slid off the bleeder by the bipolar for-
ceps, and the suction tip exposes the bleeder, which
is then coagulated using the bipolar forceps. Persis-
tent oozing from an edematous brain can be covered
with a piece of thrombin-soaked Gelfoam (Pfizer,
New York, NY). Use of warmed irrigating solution
helps to promote hemostasis.

Managing the Lesion

Fig. 1.17â•… The posterior fossa dura is opened in a Y shape with Strategies for removing a tumor differ depending on
silver clips utilized to control bleeding from the occipital sinus. its location, its consistency, and its vascularity. Nev-
It is important to include both the inner and outer dural leaf- ertheless, some general principles apply. Whenever
lets in the clips. Note ependymoma in the vallecula. Rostral at possible, the tumor should be devascularized early
the top. in the procedure. Access to feeding vessels, such as
1â•… Basic Surgical Technique 13
the anterior choroidal artery supply to a choroid bleeding from the occipital sinus, they are removed.
plexus papilloma, should be part of the preopera- Often this can be done without stirring up more
tive planning. Deep feeders, such as posterior cho- bleeding. If bleeding occurs, the exposed dural edges
roidal vessels, can sometimes be embolized through can be sealed with bipolar coagulation or oversewn
an endovascular approach prior to surgery. Some with a running suture of 4–0 Nurolon. A pericranial
tumors are best debulked in a piecemeal fashion to autograft can be sutured in place to facilitate a water-
prevent excessive retraction of the brain. Others, tight dural closure, or an allograft (e.g., cadaveric
such as hypervascular hemangioblastomas, should AlloDerm) can be used. The operative bed is filled
be circumferentially stripped of their blood supply with irrigation prior to placement of the final suture.
and removed as a single specimen to prevent major After the dura has been closed, the anesthesiologist
blood loss. In separating a tumor from normal brain, performs a Valsalva maneuver to test the integrity of
it is preferable to retract the tumor, when possible, the closure.
rather than the brain. Usually, both the tumor and
the brain require some manipulation. Bone
Some very vascular tumors, such as primitive
neuroectodermal tumors or malignant gliomas, can The bone flap is replaced with titanium plates and
bleed profusely. These tumors are best handled by screws (Fig. 1.18). In young infants, absorbable plates
rapid gutting. Dividing the mass into quadrants and and screws or suture material is preferable to pre-
moving from one to another can be helpful. After vent migration of metallic hardware deep through
debulking of the tumor in one quadrant, hemostasis the dura or superficially though the scalp. Sutures
is achieved with bipolar coagulation or packing with or wire can also be used to secure the bone in older
Gelfoam and a Cottonoid, while attention is directed children. The bone dust collected from the opening
toward another quadrant. is applied to cover gaps between the craniotomy and
skull.

Closing

Brain
A closing timeout is performed to ensure that all
sponges and Cottonoid patties are accounted for. The
surgeon must be fastidious in achieving hemostasis
before closing the dura. The operative cavity is filled
with warmed Ringer solution, and bleeding sites are
identified and coagulated with the bipolar. If Cotto-
noid patties have been used, they are irrigated and
gently removed.

Dura
The dura is closed primarily with interrupted or
running sutures of 4–0 Nurolon. Smaller sutures are
used in young infants. If the dura is tense, it is best
approximated beginning in a region of lesser tension Fig. 1.18â•… The bone flap is secured using titanium plates and
and working toward the tighter area. Often this tech- screws. In young infants, titanium should be avoided because
nique will permit successful closure of even recalci- the plates can sit “proud” and penetrate the scalp, or the
trant dura. screws can go deep and penetrate the dura and brain. In young
The posterior fossa dura usually requires place- infants, the bone flap can be replaced using absorbable plates
ment of a graft. If silver clips were used to control and screws or suture material. Rostral at the bottom.
14 Section Iâ•… Introduction

Scalp throws. The skin is closed with a simple running 4–0


or 5–0 absorbable suture.
The scalp is closed in layers with absorbable sutures.
If scalp clips have been used, only a few are removed
at a time in order to prevent bleeding while the
scalp is reapproximated. An inverted suture is used 1.3╇ Outcomes and Postoperative
to close the galea. When working in tight areas, it is Course
helpful to apply the inverted suture at a slight angle
on one side and a mirror image angle on the other
(Fig. 1.19). The suture is tied parallel to the incision,
1.3.1╇ Postoperative Considerations
and the lower arm of the suture is pulled preferen- The importance of basic technique in pediatric
tially on the first throw in order to effectively bury neurosurgery cannot be overemphasized. Detailed
the knot and evert the scalp edges for final closure attention must be paid to both the nontechnical and
of the skin. A square knot is tied with a total of 4 the technical aspects of an operation. Procedures are
often carried out to treat life-threatening conditions,
and even a small technical error can lead to cata-
strophic consequences.

1.3.2╇Complications
Careful attention to basic surgical technique will
help the operator minimize the risk of complications
as well as provide guidelines for managing compli-
cations, should they occur. A major pitfall associated
with pediatric neurosurgical procedures is blood
loss. It is my hope that the techniques described in
this chapter will offer the surgeon strategies to mini-
mize bleeding and to handle it should it occur. Other
complications to be avoided are wrong-side sur-
gery, infection, and cerebrospinal fluid (CSF) fistulas.
Wrong-side surgery can be prevented by paying duti-
ful attention to the checklist during a timeout. The
risk of infection can be reduced by gentle handling
of tissue. CSF fistulas can be avoided by performing
a watertight dural closure whenever possible and
Fig. 1.19â•… The galea is reapproximated with inverted absorb-
able sutures. If the working area available is tight, the sutures using measures to divert CSF, such as ventricular
can be placed in an angled fashion. The suture is tied parallel drainage, when necessary.
to the incision, and the lower arm of the suture is pulled pref- The wise surgeon will remember that in any
erentially on the first throw in order to effectively bury the knot operative procedure, very small mistakes can lead to
and evert the scalp edges. very big problems.
2 Diagnostic Procedures
Chad A. Glenn, Naina L. Gross, and Timothy B. Mapstone

2.1╇ Bedside Functional 2.1.5╇Contraindications


Assessment of Ventricular Shunt: 1. Local infection near puncture site, or systemic
Introduction and Background infection
2. Exposed ventricular shunt tubing, reservoir,
2.1.1╇Indications or valve
3. Need for subarachnoid CSF (e.g., to diagnose
1. Need for functional assessment of ventricular meningitis)
catheter
2. Need for evaluation of intracranial pressure
(ICP)
3. Need for emergent drainage of cerebrospinal 2.2╇ Operative Detail and
fluid (CSF) Preparation
4. Need for injection of pharmacologic agents for
therapeutic or diagnostic purposes 2.2.1╇ Preoperative Planning
and Special Equipment
2.1.2╇Goals The location of the CSF reservoir may be palpated
directly. Review of skull X-ray images aids in further
1. Determination of ventricular catheter
localization. The reservoir usually consists of a pro-
function
truding convex bulb. It may be located over a burr
2. Measurement of ICP hole or in line with the ventricular catheter (Fig. 2.1).
3. Drainage of CSF
4. Administration of pharmacologic agents
Equipment Needed

2.1.3╇ Alternate Procedures 1. Hair clippers


2. Standard surgical site preparation materials
1. Evaluation of ventricular shunt in the and drapes
operating room 3. Sterile gloves, surgical hat, and facemask
4. Small-bore or winged needles, 25 gauge
5. 3- to 5-milliliter (mL) syringes
2.1.4╇Advantages
6. Sterile saline-filled syringes
1. Safety and ease of procedure 7. 3-way stopcock and manometer
2. Performed at bedside for emergent CSF 8. Sterile specimen containers
drainage
3. Rapid interpretation of findings

15
16 Section Iâ•… Introduction

Fig. 2.1â•… Bedside functional assessment of ventricular shunt. A winged needle is inserted into the cerebrospinal fluid (CSF) reser-
voir at a perpendicular angle to assess for spontaneous flow of CSF. The reservoir should always be proximal to the valve mechanism
with respect to the ventricular catheter.

2.2.2╇ Key Steps of the Procedure mating opening pressure (Fig. 2.1). Because the tub-
and Operative Nuances ing is typically not marked and the location of the
reservoir may be above or below the external audi-
After the reservoir is palpated, hair clippers may be tory meatus, only gross observations can be made.
used to trim a small patch of hair. The site is prepped
and draped in the standard and sterile fashion. The
Absence of Spontaneous Flow
needle is inserted perpendicular to the reservoir.
Immediately after, spontaneous flow of CSF may be
Absence of spontaneous flow may be due to low
observed. A syringe should not be attached during
ICP, an air lock, or an occluded ventricular catheter.
initial needle placement, as this may create an air
Attachment of a syringe with gentle negative pressure
lock, blocking CSF flow. If spontaneous flow is noted,
may facilitate CSF flow. If this is ineffective, 1 to 2 mL
a manometer is attached to measure opening pres-
of sterile saline may be flushed into the reservoir to
sure and the pressure response to a Valsalva maneu-
try to flush out any occlusion. Do not flush additional
ver if the patient is able. The opening pressure is
saline if there is no return. If only the volume of fluid
most accurate when the patient is calm and the base
flushed returns, it may indicate that the ventricu-
of the manometer is level with the external auditory
lar system is collapsed or that the catheter tip is not
meatus. If spontaneous flow is absent, a syringe may
located in the ventricular system. If a larger volume
be attached and gentle suction applied to initiate
returns, it may indicate that the ventricular catheter
flow. A syringe may also be used to withdraw CSF for
occlusion was dislodged. If flushing does not generate
diagnostic studies or to administer pharmacologic or
CSF flow, either the proximal catheter is not function-
radiographic agents. The needle is then removed at
ing properly or the reservoir was not accessed.
the same angle at which it was inserted, minimizing
reservoir damage and skin bleeding.
Volume of CSF to Remove
2.2.3╇ Expert Suggestions The volume of CSF that is appropriate to remove
depends upon the clinical scenario. A sample of 3 to 4
Winged Needles mL of CSF is typically sent for routine studies. If there
is concern about elevated ICP, a larger volume is com-
Winged needles are preferred because low-resis-
monly removed. In the case of a firm fontanelle or con-
tance tubing is attached to the needle base with a
cerning radiographic findings, 20–30 mL of CSF may
threaded attachment at the opposite end, allowing
be drained. Indications that enough fluid has been
attachment of a manometer or syringe as well as
drained include neurologic improvement, fontanelle
easy visualization of CSF column height when esti-
2â•… Diagnostic Procedures 17
softening, and lowering of ICP measurement. Excessive 2.3╇ Outcomes and Postoperative
drainage may result in catheter occlusion or neurologic
decline secondary to hematoma development. Clinical Course
considerations as well as diagnostic imaging serve to
aid in determining how much CSF to drain. 2.3.1╇ Postoperative Considerations
1. Proper technique and interpretation of
Assessing the Distal Catheter ventricular shunt puncture
2. Laboratory evaluation of CSF
If the reservoir is not located over a burr hole, one
may consider applying local pressure to the ven-
tricular catheter proximally after a CSF column has 2.3.2╇Complications
been obtained to evaluate for decreasing column
height, serving as an indicator of distal catheter and 1. Irreparable damage to ventricular shunt system
valve function. However, applying pressure does not 2. Infection
ensure that the proximal portion of the catheter has
3. Proximal catheter occlusion from repeated
been entirely occluded, limiting interpretation.
attempts to withdraw CSF
4. Introduction of air into the vascular system in
2.2.4╇ How to Avoid Pitfalls the patient with a ventriculoatrial shunt

Locate the Reservoir

Do not attempt puncture until the location of the res- 2.4╇ Lumbar Puncture:
ervoir is known with relative certainty. If the valve has Introduction and Background
an integrated reservoir, the reservoir will be proximal
to the valve mechanism. Improper needle introduc- 2.4.1╇Indications
tion may result in catheter laceration or damage to the
valve mechanism, requiring replacement. 1. Need for subarachnoid cerebrospinal fluid
(CSF) for diagnostic or therapeutic purposes
2. Need for administration of intrathecal
Do Not Use Large-Volume Syringes or
therapeutic or radiographic agents
Large-Gauge Needles
3. Need to evaluate intracranial pressure (ICP)
Large-volume syringes are capable of generating
excessive negative pressure at the catheter tip, which
may damage adjacent neurovascular structures or 2.4.2╇Goals
pull choroid plexus into the catheter lumen. Large-
gauge needles may increase the risk of developing a 1. Laboratory evaluation of CSF
persistent CSF leak from the reservoir or damaging 2. Relief of elevated ICP through therapeutic
the adjacent catheter or valve mechanism. drainage of CSF
3. Administration of intrathecal therapeutic or
radiographic agents
2.2.5╇ Salvage and Rescue
Development of CSF Collection under the Scalp 2.4.3╇ Alternate Procedures
Reservoir puncture may result in CSF leakage despite 1. Cervical spinal puncture
proper technique, though this is uncommon. This
may occur when larger-gauge needles are used or
the subgaleal pocket created for valve placement has 2.4.4╇Advantages
not yet sealed. CSF may be drained by needle aspira-
tion with careful avoidance of underlying shunt com- 1. Absence of spinal cord
ponents. An occlusive dressing can then be applied. 2. Familiar technique
Persistent or enlarging CSF collections require fur-
ther investigation in the operating room.
18 Section Iâ•… Introduction

2.4.5╇Contraindications
1. Spinal defect or associated mass
2. Obstructive hydrocephalus
3. Nonfunctioning ventricular shunt
4. Local skin infection
5. Bleeding diathesis

2.5╇ Operative Detail and


Preparation
2.5.1╇ Preoperative Planning
and Special Equipment
Equipment Needed

1. Standard surgical site preparation materials


and drapes
2. Sterile gloves, surgical hat, and facemask
3. Lumbar puncture kit to include local
anesthetic, specimen collection tubes, spinal
needle (21–22 gauge), manometer

2.5.2╇ Key Steps of the Procedure


and Operative Nuances
With the help of an assistant, the patient is placed in
the left lateral decubitus position (for a right-handed Fig. 2.2â•… Lumbar puncture. The spinal needle is introduced
individual) and curled into the fetal position. The into the L3–L4 or L4–L5 interspace. Resistance will be met as
back should be perpendicular to the ground. The the needle advances through the supraspinous and interspi-
iliac crest is palpated to localize the L4–L5 interspi- nous ligaments until a “pop” or sudden decrease in resistance
nous space. The site is then marked, prepped, and occurs as the needle pierces the thecal sac.
draped. A small amount of local anesthetic is used. It
is important to ensure that all needed equipment is
readily available prior to attempting puncture.
The spinal needle is introduced through the L3–L4
or L4–L5 interspace, with the bevel facing upward at 2.5.3╇ Expert Suggestions
an angle that is aiming toward the umbilicus and par-
allel to the ground (Fig. 2.2). Resistance will be met Proper Positioning
as the needle passes through the supraspinous and
interspinous ligaments. A “pop” or sudden decrease Proper positioning is vital. An assistant should be avail-
in resistance may be felt as the needle pierces the the- able to assist with maintaining the desired position.
cal sac. Next, the bevel is rotated toward the patient’s
head, and the stylet is removed to check for spontane-
ous CSF flow. Opening pressure may be measured in Measuring Opening Pressure
the calm patient with legs straightened.
CSF is then drained into the collection tubes to Be sure to use local anesthetic. Opening pressure
send for desired studies. The stylet is replaced, and measurements are useless in the agitated patient.
the needle is withdrawn. If no leakage of CSF is noted The legs should also be straightened, as fetal posi-
at puncture site, a small dressing is applied. tioning may increase intraabdominal or intratho-
2â•… Diagnostic Procedures 19
racic pressure, which may translate to an artificially 2.6╇ Outcomes and Postoperative
elevated measurement.
Course
Obtaining CSF 2.6.1╇ Postoperative Considerations
Always use a spinal needle that contains a stylet. 1. Flat positioning for a few hours after a large
Use of a needle without a stylet may introduce epi- volume of CSF is drained
thelial tissue into the spinal canal and result in an 2. Laboratory evaluation of CSF
epidermoid.
Never attempt to instill or aspirate the spinal nee-
dle until CSF flow has been established, as this may 2.6.2╇Complications
result in subarachnoid hemorrhage and epidural or
subdural hematoma. 1. Herniation syndrome or hematoma (may
develop acutely or in a delayed fashion
secondary to sudden decompression of CSF in
2.5.4╇ How to Avoid Pitfalls the setting of elevated ICP)
2. Infection secondary to poor technique or
Avoid Overpositioning systemic infection
Though the fetal position opens the spinal inter- 3. Development of parasthesias secondary to
spaces, facilitating needle passage, excessive knee- nerve root injury
to-chest pressure may result in hypoxia in the infant. 4. Spinal headache
5. Subcutaneous CSF leak

Do Not Overdrain

Avoid free flow of CSF between collections. Always 2.7╇ External Ventricular
replace the stylet when not obtaining specimen.
Overdrainage may precipitate herniation or hema-
Catheter Placement:
toma development. Introduction and Background
2.7.1╇Indications
Check Puncture Site
• Therapeutic need for ventricular
Do not initiate lumbar puncture above L2, as this decompression through cerebrospinal fluid
may result in puncture of the spinal cord. (CSF) drainage
• Therapeutic need for administration of che-
motherapeutic or other pharmacologic agents
2.5.5╇ Salvage and Rescue • Diagnostic need for ventricular CSF for
laboratory analysis
Neurologic Changes
• Diagnostic need for injection of radiographic
If the patient develops altered mental status, agents to facilitate ventricular imaging
weakness, or severe parasthesias, the spinal nee- • Diagnostic need for intracranial pressure (ICP)
dle should be carefully removed and the procedure measurement
aborted. Additional imaging may be warranted
if the cause of the symptoms cannot readily be
explained. 2.7.2╇Goals
1. Measurement of ICP
Leakage of CSF 2. Therapeutic ventricular decompression
3. Collection of ventricular CSF for laboratory
Repeated puncture of the dura mater and arachnoid
analysis
may result in persistent CSF leakage. Leakage may
improve with flat positioning for 24 to 48 hours but, 4. Injection of radiographic contrast agents for
if persistent, may indicate the need for a blood patch. ventricular imaging
5. Administration of chemotherapeutic or other
pharmacologic agents
20 Section Iâ•… Introduction

2.7.3╇ Alternate Procedures 6. External CSF collection system with pressure


transducer
1. Placement of ICP monitor 7. Cardiac and apnea monitors
2. Lumbar puncture

2.8.2╇ Key Steps of the Procedure and


2.7.4╇Advantages Operative Nuances
1. Rapid assessment of ICP Cranial imaging is reviewed to choose the appro-
2. Potential for therapeutic drainage of CSF to priate side and approach for ventricular catheter
lower ICP placement. The patient is positioned in a recumbent
3. Repeated ventricular CSF sampling as needed position with the head of bed elevated at 20 to 30°.
An assistant is preferred for maintenance of proper
4. Administration of chemotherapeutic,
patient positioning.
pharmacologic, or radiographic agents into
A coronal site on the nondominant hemisphere
the ventricular space
is preferred. The coronal suture is palpated, and the
midpupillary line is marked. A marking pen is then
used to plan an incision that is approximately 1 cm
2.7.5╇Contraindications anterior to the coronal suture. The hair surrounding
the planned incision site as well as the tunneling site
1. Bleeding diathesis
is trimmed. The area is prepped and draped in ster-
2. Need for subarachnoid CSF (e.g., to diagnose ile fashion. Local anesthetic is injected at the incision
meningitis) and tunneling site.
The incision is carried to the cranium with the
scalpel blade used to scrape away adjacent pericra-
nium. The twist drill is used to make a hole 1 cm
2.8╇ Operative Detail and anterior to the coronal suture in the midpupillary
Preparation line without penetrating the dura mater. Forceps are
used to remove any bone dust, and this process is
2.8.1╇ Preoperative Planning enhanced with gentle irrigation of saline.
and Special Equipment A spinal needle or scalpel is used to fenestrate the
dura mater. The premeasured catheter is aimed in
Prior to consideration of ventricular catheter place- the coronal plane perpendicular to the skull surface
ment, intracranial imaging should be obtained to and toward the ipsilateral medial canthus (Fig. 2.3).
evaluate the ventricular anatomy and for surgical A gentle “pop” is often felt as the ventricular space is
planning. Several cranial sites have been described entered. The stylet should be removed once the ven-
for ventricular puncture. The approaches most com- tricular space is entered before the catheter is advanced
monly utilized at our institution are coronal (the further. It is at this point that the opening pressure may
Kocher point), posterior parietal (the Keen point), be determined, as drainage of even a small amount of
and occipital. A method employed at our institu- CSF may result in an artificially low measurement.
tion to identify the coronal entry site in the pediatric After the opening pressure check, the catheter
population is described here. may be advanced an additional centimeter after
the stylet is removed. The ending depth of the cath-
eter will vary depending upon ventricular anatomy,
Equipment Needed skull thickness, and patient age, but with standard
approaches in the pediatric population this depth
1. Hair clippers should not exceed 4.5 to 5 cm at the skull.
2. Standard surgical site preparation materials The trocar is attached to the catheter and tun-
and drapes neled under the scalp 3 to 5 cm away from the inci-
sion while catheter depth is maintained. CSF flow is
3. Sterile gloves, surgical gown, surgical hat, and
confirmed after the trocar is removed. The threaded
facemask
cap is attached to the end of the catheter to prevent
4. Cranial access kit to include local anesthetic, excess CSF drainage as the catheter is secured to the
marking pen, scalpel with handle, twist drill, scalp. Nylon suture is employed to close the scalp
spinal needle, 10 mL of sterile saline, trocar, incision and to secure the catheter to the scalp. The
basic instruments, collection tubes, and nylon catheter is laid in a curvilinear or circular line and
suture sutured to the scalp at multiple points. A U-shaped
5. Ventricular catheter with stylet stay stitch may also be placed where the catheter
2â•… Diagnostic Procedures 21

a b

Fig. 2.3â•… (a,b) External ventricular catheter placement. An incision is planned, preferably on the nondominant hemisphere, that
allows a twist drill hole to be made approximately 1 cm anterior to the coronal suture along the midpupillary line. The ventricular
catheter is advanced in a perpendicular fashion to enter the ventricular space.

exits the scalp so that the site may be easily closed 2.8.4╇ How to Avoid Pitfalls
after the drain is removed.
The catheter is then attached to the collection Check Blood Clotting Parameters
system, which is set to the desired height relative to
the external auditory meatus and is attached to the Ensure that basic coagulation parameters and plate-
pressure transducer. Once the catheter is attached, let counts are normal. The pediatric population
the ICP and waveform may be assessed. is less commonly taking anticoagulants or anti-
platelet agents, but this simple step should not be
disregarded.
2.8.3╇ Expert Suggestions
Sedation Place Catheters at the Correct Depth and
Within CSF Pathways
Sedatives may be employed to ensure that patients
are calm, as rapid head movements in an agitated The ventricular catheter depth should never be
patient may result in damage to neurovascular struc- greater than 6 cm at the skull surface using stan-
tures or anomalous catheter placement. dard approaches. Imaging should be obtained to
ensure that all catheter fenestrations are located
within CSF pathways if radiographic or other phar-
Skull Thickness macologic agents are to be given. Administration of
these agents intraparenchymally may result in neu-
Skull thickness will vary with patient age. Extra care rologic injury.
must be taken to avoid plunging through the dura
mater when using the twist drill on the younger
patient with a softer and thinner cranium.
22 Section Iâ•… Introduction

Prevent Pressure Damage in Posterior Fossa 2.10╇ Percutaneous Subdural Tap:


Mass and Hydrocephalus
Introduction and Background
In patients with obstructive hydrocephalus second-
ary to a posterior fossa mass lesion, rapid drainage 2.10.1╇Indications
of CSF, and the concomitant decrease in supratento-
rial pressure, may be sufficient to widen the pres- 1. Need for physical or laboratory evaluation of
sure differential between the infratentorial and extra-axial fluid collection
supratentorial compartments, facilitating upward 2. Signs or symptoms of elevated intracranial
herniation of the cerebellum through the incisura. pressure (ICP) or mass effect
Instead, the ICP should be allowed to decrease
gradually to the upper limits of normal through CSF
drainage. The ICP is approximated by the height of 2.10.2╇Goals
the CSF column on the vertically raised premea-
sured ventricular catheter. 1. Physical and laboratory assessment of extra-
axial collection, including culture or cytology
when indicated
2.8.5╇ Salvage and Rescue 2. Therapeutic drainage of fluid to reduce
elevated intracranial pressure or mass effect
Catheter Debris

Catheter debris from choroid plexus, blood clot, 2.10.3╇ Alternate Procedures
or infection may result in a nonfunctioning ven-
tricular catheter. Gentle flushing, first distally and 1. Burr hole craniotomy
then proximally, with sterile saline may dislodge 2. Subdural catheter placement
any debris. If resistance is met or bright red blood
is noted in the catheter, imaging should rapidly be
obtained.
2.10.4╇Advantages
1. Bedside procedure
2.9╇ Outcomes and Postoperative 2. Rapid physical and laboratory analysis of
extra-axial fluid collection
Course 3. Therapeutic drainage of extra-axial fluid
collection when associated with elevated ICP
2.9.1╇ Postoperative Considerations
1. Postoperative imaging to ensure proper
catheter placement (should absolutely be
2.10.5╇Contraindications
obtained if pharmacologic or radiographic
1. Local scalp infection
agents are to be administered)
2. Minimal extra-axial collections in an
asymptomatic patient
2.9.2╇Complications 3. Bleeding diathesis

1. CSF infection
2. Intracranial hemorrhage secondary to direct
vascular damage or rapid ventricular drainage 2.11╇ Operative Detail and
3. Seizure Preparation
4. Development of catheter track from the
ventricular system to the scalp 2.11.1╇ Preoperative Planning and
5. Upward herniation Special Equipment
This procedure is most often performed in infants
with an open or fibrosed fontanelle, but it may
also be performed in patients with splayed coronal
sutures. In addition, it may be performed bilaterally
when clinically indicated.
2â•… Diagnostic Procedures 23

Equipment Needed

1. Hair clippers
2. Standard surgical site preparation materials
and drape
3. Sterile gloves, surgical hat, and facemask
4. Local anesthetic
5. Subdural or winged needles, 23 to 25 gauge
6. Sterile 10-mL threaded syringes
7. Sterile specimen containers
8. Hemostat (optional)
9. Small dressing
10. Cardiac and apnea monitors

2.11.2╇ Key Steps of the Procedure and


Operative Nuances
Cranial imaging is reviewed to identify the optimal
location of puncture. With the help of an assistant,
the patient is positioned supine with his or her head
slightly elevated. The anterior fontanelle is palpated,
identifying the lateral, anterior, and posterior bound-
aries (Fig. 2.4). The lateralmost aspect of the anterior
fontanelle is prepped and draped in sterile fashion
after the hair is trimmed. Sedation or local anesthetic
may be utilized in an agitated patient.
The boundary of the skull and the fontanelle is
identified. The skin overlying the skull at the bound-
ary is pushed forward and held in position. The nee- Fig. 2.4â•… Percutaneous subdural tap. After the lateral boundary
dle is introduced at a 15° from perpendicular angle of the anterior fontanelle is palpated, the overlying scalp is gen-
just beyond the edge of the skull into the subdural tly pushed forward. The spinal needle is introduced at an angle
space (Fig. 2.4). The depth of needle placement is 15° from perpendicular and advanced into the subdural space.
closely monitored. A rapid decrease in resistance
may be felt as the subdural space is entered. Fluid
may flow spontaneously in those with elevated ICP
or may require gentle aspiration. Once the flow is
established, a hemostat may be placed onto the nee- Imaging
dle at the scalp edge to maintain depth.
Once the desired volume of fluid is removed It is advisable to obtain cranial imaging prior to sub-
and collected into specimen containers, the needle dural puncture to discern whether the procedure
is carefully removed. If the skin has been pulled will likely be beneficial and, if so, to localize the opti-
as described above, there should be only minimal mal site for needle placement.
drainage. Brief, simple pressure is applied if needed.
A sterile bandage may be placed at the needle punc-
ture site. Fluid Drainage

Depending upon the clinical scenario, varying vol-


2.11.3╇ Expert Suggestions umes of drainage are appropriate. In the patient
with large collections thought to be contributing to
Scalp Manipulation elevated ICP, drainage may continue until neurologic
status improves or the fontanelle softens. Initially,
Pulling the scalp at the skull edge over the fonta- this volume should be just enough to relieve sig-
nelle for needle placement enables an occluded tract nificant pressure, minimizing the risk for iatrogenic
once the needle is removed and the adjacent skin is injury and decline secondary to a rapid decrease in
allowed to return to its resting position. This greatly ICP. Patients requiring repeated tap may be drained
reduces the likelihood of postprocedural drainage. more aggressively on subsequent taps.
24 Section Iâ•… Introduction

2.11.4╇ How to Avoid Pitfalls urgent cranial imaging should be obtained for fur-
ther evaluation.
Use Correct Needle Depth

In most situations, the needle depth should not exceed


1.5 to 2 cm. Advancing the needle farther increases 2.12╇ Outcomes and Postoperative
the likelihood of damaging cortical structures. Course
2.12.1╇ Postoperative Considerations
Use a Lateral Approach If Possible
1. Infectious versus hemorrhagic nature of fluid
If acceptable, lateralizing the puncture site as much to direct further work-up
as possible minimizes the risk of venous sinus dam-
2. Improvement in neurologic exam (may
age. The midpupil line marks the optimal medial
indicate symptomatic elevated ICP)
boundary.

2.11.5╇ Salvage and Rescue 2.12.2╇Complications

When to Seek Additional Imaging 1. Hematoma development from improper


technique or excessive drainage
If the fluid being drained changes as volume is 2. Infection
removed to resemble bright red blood, or if there
is a neurologic change noted after the procedure,
3 Neuroanesthesia
Sulpicio G. Soriano and Craig D. McClain

3.1╇ Introduction and Background cardiac murmur, low room-air oxygen saturation,
cyanosis, or respiratory distress), it is necessary to
obtain echocardiography and an assessment by a
3.1.1╇Goals pediatric cardiologist in order to optimize cardiac
function prior to surgery. There are also specific peri-
The anesthetic management of infants and chil-
operative concerns in pediatric patients (Table 3.1).
dren undergoing neurosurgical procedures should
Preoperative fasting guidelines have evolved
be based on the developmental stage of the patient.
and are frequently dictated by regional practices
The evolving maturational changes of the various
(Table 3.2). The purpose of limiting oral intake is to
organ systems have a significant impact on the selec-
minimize the risk of aspiration of gastric contents
tion of drugs and techniques for the safe conduct
on induction. However, prolonged fasting periods
of anesthesia. Age-dependent differences in cranial
and vomiting may induce hypovolemia and hypo-
bone development, cerebrovascular physiology, and
glycemia, which can exacerbate hemodynamic and
neurologic lesions distinguish neonates, infants, and
metabolic instability under anesthesia.
children from their adult counterparts. In particu-
lar, the central nervous system (CNS) undergoes a
tremendous amount of structural and physiological 3.2.2╇ Key Steps of the Procedure and
change during the first 2 years of life. The goal of this
chapter is to highlight these age-dependent differ- Operative Nuances
ences and their effect on the anesthetic management
of the pediatric neurosurgical patient. Induction of Anesthesia

A smooth transition into the operating suite depends


on the level of anxiety and the cognitive development
3.2╇ Operative Detail and and age of the child. Children between the ages of 9 to
12 months and 6 years may have separation anxiety.
Preparation Midazolam, administered orally or intravenously, is
effective in relieving anxiety and producing amnesia.
3.2.1╇ Preoperative Planning and Parental involvement during induction of anesthesia
Special Equipment is common in pediatric operating rooms and requires
full engagement of the surgical team. Obtunded and
Neonates and infants have the highest risk of any age lethargic patients do not require premedication with
group for perioperative respiratory and cardiovas- sedatives and should have an anesthetic induction
cular morbidity and mortality. The systemic effects performed in an expeditious manner.
of general anesthesia and the physiological stress of Induction of anesthesia is dictated by the patient’s
surgery have an important impact on this vulnerable comorbidities and neurologic status. If the patient
group. Therefore, a thorough review of the patient’s does not have intravenous access, anesthesia can be
history can reveal conditions that may increase the induced with sevoflurane, nitrous oxide, and oxygen
risk of adverse reactions to anesthesia and identify by mask. Intracranial hypertension may be exacer-
patients who require more extensive evaluation or bated by hypercarbia and hypoxia, which may occur
whose medical condition needs to be optimized if the airway becomes obstructed during induction,
before surgery. If a cardiac defect is suspected (loud and vigilant maintenance of a patent airway with

25
26 Section Iâ•… Introduction
Table 3.1â•… Coexisting conditions that affect anesthetic management

Condition Anesthetic implications

Congenital heart disease Hypoxia


Arrhythmias
Cardiovascular instability
Paradoxical air emboli

Prematurity Postoperative apnea

Gastrointestinal reflux Aspiration pneumonia

Upper respiratory tract infection Laryngospasm, bronchospasm, hypoxia, pneumonia

Craniofacial abnormality Difficult tracheal intubation

Denervation injuries Hyperkalemia after succinylcholine


Resistance to nondepolarizing muscle relaxants
Abnormal response to nerve stimulation

Epilepsy Hepatic and hematological abnormalities


Increased metabolism of anesthetic agents
Ketogenic diet

Arteriovenous malformation Congestive heart failure

Neuromuscular disease Malignant hyperthermia


Respiratory failure
Sudden cardiac death

Chiari malformation Apnea


Aspiration pneumonia

Hypothalamic/pituitary lesions Diabetes insipidus


Hypothyroidism
Adrenal insufficiency

mild hyperventilation will alleviate this problem. Table 3.2â•… Preoperative fasting guidelines
In patients with intravenous access, anesthesia can
be induced with propofol. Some patients presenting Substance Fasting period
for neurosurgical procedures may be at particular Clear liquids 2 hours
risk for aspiration of gastric contents, and a rapid-
sequence induction of anesthesia with succinylcho- Breast milk 4 hours
line is required to intubate the trachea expeditiously.
Contraindications to the use of succinylcholine Formula or fortified breast milk 6 hours
include malignant hyperthermia susceptibility, mus- Solid food Midnight
cular dystrophies, and recent denervation injuries.

Vascular Access and Positioning

Limited access to the patient during neurosurgical gist. Cannulation of the radial artery provides direct
procedures requires secure intravenous access prior blood pressure monitoring and sampling for blood
to the start of surgery. Large peripheral venous can- gas analysis. Other useful arterial sites in infants and
nulae are sufficient for most craniotomies. Should children include the dorsalis pedis and posterior tib-
initial attempts fail, central venous cannulation may ial artery.
be necessary. Femoral vein catheterization avoids the Patient positioning requires careful preoperative
risk of pneumothorax and does not interfere with planning to allow adequate access to the patient for
cerebral venous return. Furthermore, femoral cath- both the neurosurgeon and the anesthesiologist. Fur-
eters are more easily accessible to the anesthesiolo- thermore, various surgical positions affect the physi-
3â•…Neuroanesthesia 27
Table 3.3â•… Physiologic effects of patient positioning

Position Physiological effect

Head-up/sitting Increased cerebral venous drainage


Decreased cerebral blood flow
Increased venous pooling in lower extremities
Postural hypotension

Head-down Increased cerebral venous and intracranial pressure


Decreased functional residual capacity (lung function)
Decreased lung compliance

Prone Venous congestion of face, tongue, and neck


Decreased lung compliance
Venocaval compression

Lateral decubitus Decreased compliance of downside lung

ologic status of the patient (Table 3.3). The prone Management of Fluids and Blood Loss
position can increase intraabdominal pressure and
lead to impaired ventilation, venocaval compres- Meticulous fluid and blood administration is essen-
sion, and bleeding due to increased epidural venous tial in order to minimize hemodynamic instability,
pressure. Soft rolls are generally used to elevate and especially in the pediatric patient. Stroke volume
support the lateral chest wall and hips in order to is relatively fixed in the neonate and infant, so the
minimize abdominal and thoracic pressure. Perform- patient should be kept euvolemic. Normal saline is
ing neurosurgical procedures with the patient’s head generally chosen because it is mildly hyperosmolar
slightly elevated facilitates venous and CSF drainage and should minimize cerebral edema, but rapid infu-
from the surgical site. However, this increases the like- sion of more than 60 mL/kg of normal saline may
lihood of venous air emboli (VAE). Significant rotation cause hyperchloremic acidosis. The routine admin-
of the head can also impede venous return via com- istration of glucose-containing solutions is generally
pression of the jugular veins and can lead to impaired avoided during neurosurgical procedures, except in
cerebral perfusion, increased ICP, and venous bleed- patients who are at risk for hypoglycemia. Patients
ing. Obese patients may be difficult to ventilate in the with diabetes mellitus or total parenteral alimenta-
prone position and may benefit from the sitting posi- tion, as well as premature and small newborn infants,
tion. In addition to the physiological sequelae of the may require glucose-containing intravenous fluids.
sitting position, a whole spectrum of neurovascular Premature neonates have a circulating blood vol-
compression and stretch injuries can occur. ume of approximately 100 mL/kg total body weight;
full-term newborns have a volume of 90 mL/kg; and
infants have a blood volume of 80 mL/kg. Maximal
Maintenance of Anesthesia allowable blood loss (MABL) can be estimated using a
simple formula: MABL = Estimated circulating blood
The most frequently used technique during neuro- volume × (Starting hematocrit – Minimum accept-
surgery consists of an opioid (fentanyl, sufentanil, able hematocrit)/Starting hematocrit.
or remifentanil) and low-dose isoflurane or sevoflu- Transfusion of 10 mL/kg of packed red blood cells
rane. Dexmedetomidine can be used as an adjunct; increases hemoglobin concentration by 2 g/dL. Pedi-
it does not significantly affect most intraoperative atric patients are susceptible to dilutional thrombo-
neurophysiologic monitoring and reduces opioid cytopenia in the setting of massive blood loss and
requirements. Patients on chronic anticonvulsant multiple red blood cell transfusions. Administration
therapy usually require a larger dose of neuromus- of 5 to 10 mL/kg of platelets increases the platelet
cular blocking agents and opioids because of induced count by 50,000 to 100,000/mm3. The routine use
enzymatic metabolism of these agents. The use of of the antifibrinolytic tranexemic acid in surgical
neuromuscular blocking agents should be discussed procedures with excessive blood loss, such as pos-
with the surgical and monitoring teams if assess- terior spine fusions, cardiac surgery, and craniofa-
ment of motor function during seizure and spinal cial reconstructive procedures, has been shown to
cord surgery is planned. decrease blood loss in pediatric patients.
28 Section Iâ•… Introduction

Anesthetic Management of Specific be uncertain. Perioperative diabetes insipidus (DI)


Neurosurgical Procedures can lead to electrolyte and hemodynamic derange-
ments. Laboratory studies should therefore include
Congenital Disorders serum electrolytes and osmolality, urine specific
gravity, and urine output. DI is marked by sudden
Myelomeningocele/Encephalocele polyuria (> 4 mL/kg/hr), hypernatremia, and hyper-
osmolarity. Initial management consists of infusion
Tracheal intubation of a neonate with a myelomenin-
of aqueous vasopressin (1 to 10 mU/kg/hr) and judi-
gocele or encephalocele can be challenging depend-
cious fluid administration that matches urine output
ing on the size and location of the defect. The supine
and estimated insensible losses.
patient may be elevated on rolled-up towels in order
to minimize direct pressure on the lesion. Blood and
fluid loss depends upon the size of the lesion and the
Epilepsy
amount of tissue dissection required to repair the Epilepsy surgery poses several anesthetic manage-
defect. ment issues. General anesthetics can compromise
the effectiveness of intraoperative neurophysiologic
Hydrocephalus monitors that guide the resection of the epilepto-
The anesthetic technique depends on the patient’s genic focus. High levels of volatile anesthetics and
symptoms. In a patient with intact mental status or neuromuscular blockade may also suppress cortical
one in whom intravenous access cannot be estab- stimulation. Nitrous oxide can precipitate pneumo-
lished, an inhalation induction with sevoflurane and cephalus after a recent craniotomy (3 weeks) and
gentle cricoid pressure may be used. If, however, the should be avoided until after the dura is opened.
patient is obtunded, is at risk for herniation, or has A variety of techniques have been advocated
a full stomach, intravenous access should be estab- to facilitate intraoperative assessment of motor-
lished in order to perform a rapid-sequence induction sensory function and speech. In the “sleep-awake-
followed by tracheal intubation. VAE may occur dur- asleep” technique, the patient undergoes general
ing placement of the distal end of a ventriculoatrial anesthesia for the surgical exposure. The patient is
shunt if the operative site is above the heart. Acute then awakened for functional testing, and general
obstruction of a ventricular shunt requires urgent anesthesia is reinstituted when patient cooperation
treatment because an acute increase in intracranial is no longer needed. Most cooperative patients will
pressure in the relatively small cranial vault of the tolerate sedation with propofol or dexmedetomi-
infant or child can have devastating consequences. dine. Propofol does not interfere with electrocor-
ticography (ECoG) if it is discontinued 20 minutes
before monitoring in children undergoing an awake
Neoplasms craniotomy. Supplemental opioids are administered
to provide analgesia. It is, however, imperative that
Posterior Fossa Tumors candidates for craniotomy under local anesthesia or
Posterior tumors may impinge upon brainstem sedation be mature and psychologically prepared to
structures vital to the control of respiration, heart participate in this procedure.
rate, and blood pressure, complicating the intraop-
erative management of these patients. Respiratory Cerebrovascular Disease
control centers can be damaged during surgical dis-
section. Stimulation of the nucleus of cranial nerve The primary goal of the anesthesiologist during cere-
V can cause hypertension and tachycardia. Irrita- brovascular surgery is to optimize cerebral perfusion
tion of the nucleus of the vagus nerve may result in while minimizing the risk of bleeding. A large arte-
bradycardia or postoperative vocal cord paralysis. riovenous malformation (AVM) in neonates may be
Continuous observation of the blood pressure and associated with high-output congestive heart failure
electrocardiography (ECG) are essential to detect requiring vasoactive support. Hypertensive crisis
encroachment upon these vital structures. Inadver- after embolization or surgical resection of the AVM
tent entry into the straight and transverse sinus can should be rapidly treated with vasodilators.
precipitate massive VAEs. The goal of anesthetic management of patients
with moyamoya disease is to optimize cerebral per-
Supratentorial Tumors fusion with aggressive preoperative hydration and to
maintain normotension or mild hypertension during
Craniopharyngiomas may be associated with surgery and the postoperative period. Intraoperative
hypothalamic and pituitary dysfunction. Steroid normocapnia is essential, because both hyper- and
replacement therapy with either dexamethasone or hypocapnia can lead to steal phenomenon from the
hydrocortisone may be required because the integ- ischemic region. Intraoperative EEG monitoring may
rity of the hypothalamic-pituitary-adrenal axis may be utilized during surgery to detect cerebral isch-
3â•…Neuroanesthesia 29
emia. Optimization of cerebral perfusion should be 3.3╇ Outcomes and Postoperative
extended into the postoperative period to maintain
euvolemia and, using sedatives and opioids, to pre- Course
vent hyperventilation induced by pain and crying.
3.3.1╇ Postoperative Care
Neuroendoscopy
Hemodynamic and Respiratory Support
Technological advances in endoscopic surgery have
provided less-invasive approaches to the surgical The severity of the intraoperative course dictates the
management of CNS lesions. Despite the relative safety need for admission to an intensive care unit. Factors
of this procedure, hypertension, arrhythmias, and like massive blood loss, hemodynamic instability,
neurogenic pulmonary edema have been reported in neurological deficits, seizures, and prolonged surgi-
conjunction with acute intracranial hypertension due cal time necessitate close observation and immediate
to lack of egress of irrigation fluids and/or manipula- treatment. The acuity of the intensive care setting is
tion of the floor of the third ventricle. essential for early detection and treatment of evolv-
ing postoperative events. These include bleeding,
neurological deficits, electrolyte abnormalities,
3.2.3╇ Salvage and Rescue respiratory distress, and fluid shifts. The immediate
availability of magnetic resonance imaging (MRI) or
Hemodynamic collapse due to massive blood loss or computed tomography (CT) is mandatory in order to
venous air embolism looms as a catastrophic compli- assess evolving neurological deficits.
cation for any major craniotomy. Large-bore intrave-
nous access and arterial blood pressure monitoring
are therefore essential for these procedures. Mas- Sedation and Pain Management
sive blood loss should be aggressively treated with
crystalloid and blood replacement and vasopressor Postoperative neurosurgical patients should be com-
therapy (e.g., dopamine, epinephrine, norepineph- fortable, awake, and cooperative to complete serial
rine). Venous air embolism commonly occurs during neurological examinations. In pediatrics, these goals
the surgery. Maintaining normovolemia minimizes can be difficult to maintain because of the cognitive
this risk. Early detection of a VAE with continuous level of the patient. The mainstay of sedation in the
precordial Doppler ultrasound may allow treat- pediatric intensive care unit remains a combina-
ment to be instituted before large amounts of air tion of an opioid and a benzodiazepine. Opioids like
are entrained. Should a VAE produce hemodynamic morphine and fentanyl should be carefully titrated
instability, the operating table must be placed in the to minimize postcraniotomy pain while maintaining
Trendelenburg position in order to improve cerebral consciousness. Propofol is a potent, ultrashort-act-
perfusion and prevent further entrainment of intra- ing sedative-hypnotic but has only limited utility in
vascular air. Special risks exist in neonates and young pediatrics because it is associated with a fatal syn-
infants because right-to-left cardiac mixing lesions drome of bradycardia, rhabdomyolysis, metabolic
can result in paradoxical emboli. In the case of severe acidosis, and multiple organ failure when used over
cardiovascular collapse, some pediatric centers have extended periods in small children. Dexmedetomi-
rapid-response extracorporeal membrane oxygen- dine has analgesic properties and is a useful agent for
ation (ECMO) teams, which can provide cardiopul- reversible sedation.
monary support when the crisis is refractory to
standard cardiopulmonary resuscitation algorithms.
4 Pre- and Postoperative Management
of the Neurosurgical Patient
Robert C. Tasker

4.1╇ Introduction and Background erative transition of care. The neurosurgeon and anes-
thesiologist need to convey succinctly what happened
Perioperative management of pediatric neuro- in the operating room and what they want to happen
surgical patients presents many challenges. Many in the immediate postoperative period. The postoper-
conditions and complications are unique to small ative attendants need to be cognizant of any concerns
children, and a basic understanding of age-related about bleeding, compromised tissue, derangements
physiology and pharmacology is essential in mini- in homeostasis, and requirements for postoperative
mizing perioperative morbidity. The “pediatric” age antibiotics, steroids, etc. We find that this process is
range is from newborn through to adult; thought of best served by attending-to-attending signout. Also, it
in another way, this is a mass range of 1 to 100 kg—a is my view that, if the postoperative attendant is not
difference in mass of two orders of magnitude. In familiar with the procedures that have occurred in the
terms of the changing brain, the pediatric range operating room, then that attendant should take the
spans a sixfold increase in brain weight from birth time to observe what actually happens (e.g., crani-
to maturity, with most of the change occurring by otomy for resection of tumor, craniosynostosis repair,
the age of 2 years. Chiari decompression, craniotomy and resection for
epilepsy, bypass procedure for vascular insufficiency,
and resection of arteriovenous malformation would
4.1.1╇ Goal of Perioperative Care be a good start).
This chapter is a review of basic information
The overall goal of perioperative care is to prepare the and understanding that are required by those
patient for the right surgery, at the right time, under involved in perioperative care. The format of this
the right circumstances of physiology and pharma- chapter differs from others in this book in that it
cology, all with the best expected outcomes for the is about “what are you thinking,” “what informa-
child’s underlying condition. In order to achieve these tion needs to be conveyed between attendants,”
objectives, there must be clear documentation in the and why. Please note that, at a number of points in
patient’s medical record and good communication this chapter, recommendations are made concern-
between all team members. All hospitals will have ing drugs and drug doses. Many of these drugs are
their own policy and procedures manuals that cover not licensed for use in children or for the indica-
the details of what is expected of medical practitio- tions described. Readers should check usage with
ners and what is considered best care practices in pre- their own national drug regulatory, hospital phar-
paring a child for a neurosurgical procedure. Table€4.1 maceutical, and professional bodies.
outlines the checklist for the general preoperative case
review. After the operation, those caring for the child
will also need information in a systematic manner. We
use the formula one hand-off technique. The basis of Clinical Pearl 1
this technique is that there is limited time to transfer
information and the right details need to be conveyed Have a checklist of information that should be
to the next team caring for the child, be it in the neu- signed out at the transitions of patient care (pre-
rosurgical ward or in the surgical intensive care unit operative to operative, operative to postoperative,
(ICU). Table 4.2 outlines the checklist for the postop- postoperative to discharge).

30
4â•… Pre- and Postoperative Management of the Neurosurgical Patient 31

Table 4.1â•… Elective neurosurgical admissions


Activity Details Action

Clerking • Presenting complaint • Documented


• Previous medical history
• Developmental level
• Previous anesthetics
• Medications
• Allergies
• Seizures

Examination •C entral and peripheral nervous systems • Documentation of neurologic deficits
• Raised ICP • May need preoperative resuscitation and
• Cardiopulmonary treatment
• General level of hydration

Comorbidities • Intercurrent illness • Discuss with anesthesiologist


• Any cardiopulmonary disease
• Any gastroesophageal reflux
• Endocrinopathy

Blood testing • Hemoglobin


• Urea and electrolytes
• Blood cross-matched

Fasting period • Solid food • 6 hours


• Formula • 6 hours
• Breast milk • 4 hours
• Clear fluids • 2 hours

Preoperative anesthesia review • IV access


• Premedication

Special preoperative • Cerebrovascular cases • Intravascular euvolemia


interventions required
• Endocrine-risk cases • Stress hormone and other HPA-axis
assessment

Medications • All medications, including route • Discussion of route of administration and


whether medications can be withheld

Seizure plan • Current AEDs, if any • Review AEDs with pediatric epilepsy/
neurology

Abbreviations: AEDs, antiepileptic drugs; HPA, hypothalamic-pituitary-adrenal; ICP, intracranial pressure.

4.2╇ Brain Hydrodynamic 4.2.1╇ Cerebrospinal Fluid


Physiology
Eighty percent of cerebrospinal fluid (CSF) is pro-
There are general principles of brain hydrodynamic duced in the choroid plexus of the lateral and fourth
physiology that will be known to all practitioners of ventricles. The remainder is produced in the intersti-
neurosurgery. However, to the novice in pediatric tial space and ependymal lining. In the adult, normal
neurosurgery, there are nuances that are also worth CSF volume is 150 mL (50% intracranial and the rest
knowing, particularly as they relate to some of the intraspinal). In the neonate, CSF volume is 50 mL.
problems that may become evident postoperatively, The rate of CSF production across all ages is 0.15 to
and these are discussed in later sections. 0.30 mL/min (up to ≈ 450 mL/day).
32 Section Iâ•… Introduction
Table 4.2â•… Postoperative signout using “formula one”

Item Details

Operation • Procedure
•C  omplicated or uncomplicated
• Concerns
•W  ound drains
• E xternal ventricular drain (e.g., where and what head of pressure being used)

Anesthesia •P  remedication used


• L aryngoscopy and whether the airway is difficult
• Anesthesia
• Emergence
•P  ostoperative pain management
•W  hether sedation is required (e.g., neurovascular child needing dexmedetomidine for deep
sedation to prevent early postoperative agitation, crying, and hypocapnia)

Intraoperative fluids • Intravenous fluids used


• Estimated blood loss and use of blood products; final hematocrit
• Urine output
• Any intraoperative laboratory tests

Surgical plan • Head-of-bed positioning required


• Blood pressure targets
• Perioperative antibiotics
• Use of antiemetics and steroids
• Laboratory tests
• In those who return with an endotracheal tube in situ, the plan for extubation
• Frequency of neurologic observations and special instructions on signs to watch for
• Seizure plan
• Postoperative imaging plan
• What has been discussed with the family and any other consultants required (e.g., infectious
disease, neuro-oncology)

Postoperative fluids • Volume required (maintenance or more)


• Serum sodium target
• Strategy for cerebral edema, if required

Organ system review • Special concerns, particularly in regard to cormorbidities and when other therapies are required
(e.g., asthma treatment)
• When feeding can be restarted

There are two pathways for CSF circulation: a


major, adult, pathway with CSF absorption through
Clinical Pearl 2
arachnoid villi (arachnoid granulation) into the
venous sinuses, and a minor, infantile, pathway with Large CSF losses in young children with an exter-
CSF drainage through the ventricular ependyma, nal ventricular drain in situ may represent a
the interstitial and perivascular space, and perineu- significant wasting of sodium. Pay attention to
ral lymphatics. The need for CSF circulation begins perioperative CSF drainage volume and serum
early during intrauterine development because the sodium concentration.
choroid plexus is formed during the first trimester.
Because arachnoid granulations do not appear until
just before birth, it is unlikely that CSF reabsorption
via the adult route of circulation is the major path- 4.2.2╇ Intracranial Pressure
way during infancy. In fact, there is some evidence
that arachnoid granulations continue to develop well Intracranial pressure (ICP) is the pressure of CSF
into the second decade, and so the “infantile” route inside the cerebral ventricles, which is often approx-
of circulation may be significant in childhood as well. imated with cerebral intraparenchymal pressure
4â•… Pre- and Postoperative Management of the Neurosurgical Patient 33

measured using a fiber-tipped microsensor. In


health, ICP is determined by cerebral blood flow
Clinical Pearl 3
(CBF) and CSF circulation. The Davson equation
describes this relationship and states that ICP is the The zero reference point for ICP is the level of
sum of sagittal sinus pressure and the product of CSF the third ventricle, using the external auditory
formation rate and resistance to CSF outflow. Normal meatus (EOM) as the marker. However, there are
values for sagittal sinus pressure, CSF formation rate, circumstances when this reference point may be
and resistance to CSF outflow are 5 to 8 mm Hg, 0.3 important to consider, for example:
mL/min, and 6 to 10 mm Hg/mL/min, respectively. In • When calculating the cerebral perfusion pres-
most clinical situations, sagittal sinus pressure stays sure (i.e., difference between mean blood pres-
constant or is coupled to central venous pressure. In sure and mean ICP), remember that blood
practice, measured ICP is often greater than the value pressure is calibrated to the level of the right
calculated using the equation. The difference is due atrium (RA).
to a vascular component, which is probably a result • When the head of the patient’s bed is elevated,
of pulsation in the arterial bed and is determined by the EOM and RA are not at the same level, and
the interaction between pulsatile arterial inflow and the cerebral perfusion number reported to you
venous outflow curves, cardiac function, and cere- as a simple automated calculation will have a
bral vasomotor tone. systematic error.
Under usual conditions, ICP remains less than
15 mm Hg and reflects the volume of three com- • In the operating room, you will be used to hav-
partments: brain parenchyma (1,200–1,600 mL in ing both the arterial pressure and the ICP cali-
the adult human), extracellular or CSF (100–150 brated to the same level. However, in the ICU,
mL), and cerebral blood volume (CBV) (100–150 you will not be able to convince colleagues to
mL). Since the intracranial vault is fixed in volume recalibrate the arterial line to the EOM.
in the developed cranium, increases in the size of
one component of the intracranial contents must be
compensated by removal of an equivalent amount
of another intracranial component, or ICP will A 70-kg adult consumes about 250 mL O2/min in the
increase. The point at which perfusion-compro- basal resting state. Therefore the brain, which repre-
mising ICP elevation occurs is dependent on brain sents only about 2% of total body weight, accounts
elastance and potential displacement of intracranial for 20% of the resting total body O2 consumption.
contents. Normal values of ICP are less in newborns The functions of nervous tissues are mainly
(2–6 mm Hg) and children (< 15 mm Hg) than in excitation and conduction of nerve impulses, and
adults. The impact of the cranial vault on intracra- these are reflected in the increasing activity of
nial hydrodynamics is also different in children. the brain. Electrical energy, ultimately, is derived
The infant’s cranium has the potential for growth, from chemical processes, and it is likely that most
with open fontanelles and sutures, so that there is of the energy consumption of the brain is used
greater total compliance of the system. For example, for active transport of ions. Oxygen is used in the
a slow-growing tumor will not have an acute mass brain almost entirely for the oxidation of carbohy-
effect because compensatory increase in intracra- drate. The energy equivalent of the total cerebral
nial volume occurs with expansion (widening of metabolic rate is, therefore, approximately 20 W,
sutures and fontanelles) and growth. As a result, or 0.25 kcal/min. Let us assume that this energy is
infants and young children may have advanced used mainly for the synthesis of high-energy phos-
intracranial pathology at the time of presentation, phate bonds, that the efficiency of the energy con-
with little reserve. servation is approximately 20%, and that the free
energy from hydrolysis of the terminal phosphate
of adenosine triphosphate (ATP) is 7 kcal/mol. This
4.2.3╇ Cerebral Metabolism energy expenditure supports the steady turnover of
close to 7 millimoles, or approximately 4 × 1021 mol-
The brain is one of the most metabolically active ecules, of ATP per minute in the entire brain.
organs of the body, and it requires a constant sup- In the normal, in vivo state, glucose is the only
ply of oxygen (O2) and nutrient, mainly glucose. In significant substrate for energy metabolism in the
the adult, the brain consumes about one-fifth of brain. The stoichiometry of glucose utilization
total body O2 utilization. Cerebral metabolic rate for and CMRO2 is as follows. The normal, conscious
oxygen (CMRO2) in normal, conscious young men is human brain produces carbon dioxide (CO2) at
approximately 3.5 mL O2 per 100 g brain mass per about the same rate of CMRO2 of 156 µmol/100 g
minute. The CMRO2 of an entire adult brain of average tissue/min, leading to a respiratory exchange ratio
weight (i.e., 1.4 kg) is therefore about 50 mL O2/min. of 1:1. CMRO 2 and CO2 production are equivalent
34 Section Iâ•… Introduction
to a rate of glucose utilization of 26 µmol/100 g
tissue/min, assuming 6 µmol of O 2 consumed and
Cerebral Oxygen Equations
CO2 produced for each micromole of glucose com-
pletely oxidized to CO 2 and water. (The actual glu- Equations describing the pathway for blood and
cose utilization is, however, 31 µmol/100g/min. oxygen in the brain (see text for details):
For complete oxidation of glucose, the theoreti- CMRO2 = (CBF × CaO2) – (CBF × CvO2) – CiO2
cal ratio of O 2:glucose utilization is 6; the excess
Q = ∆P/R
glucose utilization is responsible for a measured
ratio of only 5.5 µmol O 2/µmol glucose. The fate of Q = (Π × r4 × ∆P)/(8 Ŋ × L)
the excess glucose is unknown, but it is probably Q = CBV/t`
distributed in lactate, pyruvate, and other inter-
CBV = 1.09 × CBV0.29
mediary metabolites.)
OEF = (SaO2 – SjvO2)/SaO2
OEF = CMRO2/(CBF × 1.34 × [Hb] × SaO2
Development and Cerebral Glucose Metabolism
where CaO2 is the oxygen content of arterial blood;
Cerebral glucose utilization in 5-week-old infants is CBF is the cerebral blood flow; CBV is the cere-
three-quarters that of the adult brain. The developing bral blood volume; CiO2 is the oxygen content of
brain also metabolizes lactate, ketone bodies, amino brain tissue; CMRO2 is the cerebral metabolic rate
acids, and free fatty acids. Adult rates of glucose uti- for oxygen; CvO2 is the oxygen content of venous
lization are first achieved by the age of 2 years; after blood; [Hb] is the hemoglobin concentration; Ŋ is
this age, there is a further increase through to the blood viscosity; L is vessel length; OEF is oxygen
age of 8 years, followed by a decline in metabolic rate extraction fraction of brain; ∆P is the pressure
through to the age of 20 years. This crescendo-decre- gradient between inflow and outflow; Q is flow;
scendo pattern of change likely represents the con- R is resistance to flow; r is vessel radius; SaO2 is
sequence of brain development and the subsequent oxyhemoglobin saturation of arterial blood; SjvO2
“pruning” of neurons, synapses, and pathways that is oxyhemoglobin saturation of jugular venous
occurs with maturation. blood; and t` is mean transit time of blood.

Cerebral Oxygen Kinetics

Human brain maturation is incomplete at birth and 4.2.4╇ Cerebral Blood Flow
continues to progress during the first years of life.
As already discussed, brain development is associ- CBF is coupled to CMRO2, and both increase pro-
ated with regional changes in glucose metabolism. portionately after birth. Whole-brain CBF at birth
Given that O2 needs to be delivered to the tissue at a is, on average, 50 mL/100 g/min. CBF increases
rate that is biochemically proportionate to metabolic after birth, peaks between the ages of 5 and 8 years
needs, it should come as no surprise that regional (≈€70 mL/100 g/min), and then declines to the adult
cerebral blood flow (CBF) also changes with develop- level (≈€50 mL/100 g/min) in early teenage. Despite
ment. CMRO2 must be equal to the total amount of O2 these changes, oxygen extraction fraction (OEF) is
delivered to cerebral tissue per unit time minus the constant even in early childhood. CBF responsive-
amount left in the venous circulation per unit time ness or reactivity to changes in CO2, O2, and cerebral
and the amount that accumulates in the cerebral tis- perfusion pressure (CPP, as defined by difference
sue per unit time (see box Cerebral Oxygen Equa- in mean blood pressure [BP] and mean ICP) clearly
tions). The extraction of O2 from cerebral tissue is so occurs in the developing brain. Under anesthesia,
closely matched to the brain’s metabolic needs that CBF decreases by about 20%; under deep general
O2 content of brain tissue is small. The vast majority anesthesia, it decreases by up to 50%. In white mat-
of the arteriovenous O2 content difference (AVDO2) is ter, blood flow is about 25% of that of the cortex in
made up of O2 offloaded from hemoglobin, and the the awake state, but it is not markedly influenced
amount of O2 offloaded from hemoglobin in the cere- by anesthesia; differences between white and
bral circulation is tightly regulated by many physi- gray matter diminish when subjects are deeply
ologic factors, including brain pH, brain temperature, anesthetized.
concentration of cerebral metabolites, and amount The physical laws that describe steady laminar
of adult hemoglobin. flow of uniform fluids through nondistensible tubes
are helpful in understanding in vivo cerebrovascular
4â•… Pre- and Postoperative Management of the Neurosurgical Patient 35

Cerebral Blood Flow and Vasospasm

Vasospasm is extremely rare in children; whether


this is because we do not look for it or because we
do not have good age-specific normative data from
trans�
cranial Doppler (TCD) is unknown. However,
from the preceding discussion we can see how a
reduction in vessel radius will lead to risk of isch-
emia. In clinical practice, the highest risk of pediat-
Fig. 4.1â•… Mean blood pressure by age, showing the mean and ric aneurysmal subarachnoid hemorrhage is during
5th and 95th percentiles for average-sized children at each postoperative days 4 to 14.
age.

4.2.5╇ Cerebral Blood Volume


hemodynamics. Ohm’s law predicts that flow is pro-
portional to the pressure gradient between inflow and CBV is determined by two factors: CBF and capaci-
outflow divided by the resistance to flow (see box Cere- tance vessel diameter (i.e., small veins and venules).
bral Oxygen Equations). In brain, CPP is taken as the CBV increases with vasodilation and decreases with
driving pressure for CBF. Critical questions like “How vasoconstriction. Although CBF frequently changes in
low can perfusion pressure go?” are very difficult to the same direction as CBV, these variables are inversely
address. The answer is different in adults; but when related under normal situations (e.g., autoregulation)
should we be concerned? Fig. 4.1 summarizes normal or in pathological situations. Further, blood volume is
mean BP in children aged 1 to 17 years (boys and girls), not equally distributed throughout the brain; blood
and this, at least, is a starting point for tight control of volume per unit weight is greater in gray matter than
the driving pressure to CBF. In the perioperative patient, in white, with further variation among the various
we often use both intra-arterial lines and noninvasive nuclei. Average CBV in humans is 3 to 4 mL/100 g tis-
cuff measurements, and it is important to know what sue. Pathology, which affects either CBF or cerebral
to do with these measurements. For example, what venous capacitance, may modulate CBV with subse-
number is the “real” number when there is a difference quent effects on ICP. More quantitatively, the central
in the two measurements? (See Clinical Pearl 4.) volume principle relates the volume that intravascu-
Vascular resistance is determined principally by lar blood occupies within brain (CBV in mL) and the
vessel radius (see box Cerebral Oxygen Equations), volume of blood that moves through the brain per
and an estimate of cerebrovascular resistance (CVR) can unit time (CBF in mL/min) (see box Cerebral Oxygen
be made for any vascular segment of interest in which Equations). For example, although CBV is increased
flow and upstream and downstream pressure gradi- during vasodilation, CBF may not change if blood flow
ents are known. Poiseuille’s law shows that the major velocity is correspondingly reduced. Surplus blood
determinants of CBF are perfusion pressure, blood volume accumulates primarily within cerebral veins,
viscosity, and vessel radius (see box Cerebral Oxygen known to receive sympathetic innervation and to
Equations). Vessel length is an unchanging parameter. respond to sympathetic stimulation, and within capil-
laries to a smaller degree. Normally, increases in CBV
can be physiologically controlled by two maneuvers:
Clinical Pearl 4 increased blood outflow to the extracranial venous
circulation and restricted inflow via constriction of
Invasive arterial line BP is usually measured the major feeding arteries.
from the radial artery. Noninvasive BP is mea-
sured with a pressure-sensitive cuff around the
upper limb (i.e., brachial artery BP). When there 4.2.6╇ Cerebrovascular Autoregulation
is a difference in the measurements, consider:
Cerebral resistance arteries dilate during reductions in
• Brachial pressure may be a better measure of CPP and constrict during increases in pressure (Fig.€4.2).
central pressure when monitoring indicates As a result, CBF remains relatively constant over a fairly
apparent hypotension. broad range of arterial pressure defined as the autoreg-
• In young, healthy individuals with good “elas- ulatory plateau. The lower and upper limits of the auto-
tic” tissue, pulse pressure amplification occurs regulatory plateau in adults have been determined as
in peripheral arteries and may cause radial ar- approximately 50 to 60 mm Hg and 150 to 160 mm Hg,
tery BP (particularly systolic pressures) to be respectively. The lower limit of autoregulation refers
higher than more central pressures. to the point at which CBF starts to decrease and not to
the point at which cerebral resistance arteries are fully
36 Section Iâ•… Introduction

Clinical Pearl 5
In regard to cerebral autoregulation and devel-
opment, be conservative and assume that there is
a very narrow autoregulatory range around base-
line blood pressure.

4.2.7╇ Cerebrovascular Response to


Carbon Dioxide
Under steady-state conditions, over a period of 10
minutes, inhalation of 5 to 7% CO2 results in a 75%
increase in CBF. Inhalation of gas containing only
10% O2 increases CBF by 35%. Both hypercapnia
Fig. 4.2â•… Cerebral blood flow as a function of perfusion pres- and hypoxia lead to a decrease in cerebral vascu-
sure: the autoregulatory curve can be shifted to the right by lar resistance (CVR), indicating that the increase in
increased sympathetic nervous system (SNS) stimulation or CBF is a consequence of vasodilation. In 1948, Kety
chronic hypertension (HTN). The circles denote the change in and Schmidt described a curvilinear relationship
vessel diameter and, therefore, change in vascular resistance. between arterial partial pressure of CO2 (PaCO2)
and CBF. A reduction of PaCO2 from 40 to 20 mm
Hg decreased CBF, but not to the same extent as the
vasodilated. Cerebral resistance arteries may continue increase in CBF when PaCO2 is increased from 40 to
to dilate to some degree even after the lower limit of 60 mm Hg. In these studies, CMRO2 did not change
CBF autoregulation is exceeded. Passive reductions in during this degree of hypercapnia or hypoxia. The
diameter occur only at very low levels of CPP. Thus, the increase in CBF without any increase in CMRO2
lower limit of cerebral vasodilation does not match pre- resulted in a decrease in AVDO2 (i.e., reduced O2
cisely the lower limit of CBF autoregulation. extraction). The hypercapnia-induced increase in
Reductions of CPP below the lower limit of auto- CBF is ≈ 6% per mm Hg change in PaCO2, and hypo-
regulation result in hypoperfusion in the brain. In capnia decreases CBF by ≈ 3% per mm Hg change
an attempt to compensate for reductions in CBF, the in PaCO2 (Fig. 4.3). The relation between CBF and
extraction coefficient of O2 from the blood increases. CBV (including arterial, capillary, and venous blood
No clinical symptoms are observed until reductions volume) during changes in PaCO2 has also been
in CPP exceed the ability of increased O2 extraction investigated in humans. The increase in CBV during
to satisfy metabolic demands of cerebral tissues. The
mechanisms responsible for CBF autoregulation are
not yet clearly understood. Possibilities that have
been considered include neurogenic, myogenic, met-
abolic, and endothelial factors.

Cerebrovascular Regulation in Development

In normal term infants, several days are required


for the maturation of vascular responses. Studies in
preterm babies have shown that CBF increases over
the first 3 postnatal days. The lower limit of cerebral
autoregulation of 29 mm Hg has been described in
nonanesthetized preterm infants less than 30 weeks
gestational age. There are no available data on which
to determine whether the cerebral autoregulation
curve for ex-premature infants mimics that of term
infants. There is also evidence that autoregulatory
reserve is less in older infants than in children and
adults and that the physical state of the patient mat- Fig. 4.3â•… Cerebral blood flow as a function of partial pressure
ters. For example, in cases of craniosynostosis, very of carbon dioxide or oxygen. See how the oxygen curve shifts
low CPPs have been recorded. to the right in the presence of anemia ([Hb] low). PaCO2, par-
tial pressure of carbon dioxide in arterial blood; PaO2, partial
pressure of oxygen in arterial blood.
4â•… Pre- and Postoperative Management of the Neurosurgical Patient 37
hypercapnia is less than that in CBF, and the degree 4.2.8╇ Cerebrovascular Response to
of decrease in CBV during hypocapnia is less than
that in CBF (see box Cerebral Oxygen Equations).
Oxygen
According to Poiseuille’s law, CBV increases propor- Hypoxia elevates CBF. CBF does not change in response
tionally to the square of the diameter, yielding the to small deviations of the PaO2 around normal levels.
relation CBV = c × CBF0.5, which is in good agreement Rather, when PaO2 falls to ≈ 50 mm Hg, regional CBF
with the relation during changes in PaCO2. begins to rise. As PaO2 is further reduced below this
The question of whether alteration in PaCO2 threshold, CBF increases exponentially. It may reach
changes CBF equally in all brain regions is some- over 400% of basal flow at lowered PaO2 in an attempt
what controversial, as is the question of whether to maintain O2 delivery. There is no significant change
both gray and white matter behave in the same way. in CMRO2 over the range of PaO2 from 23 to 100 mm Hg.
There appears to be a developmental difference The responses of the cerebral circulation to
in CBF response to CO2, although in all age groups, hypoxia relate to hemoglobin oxygen saturation
CBF increases with increasing PaCO2. In both the (SaO2). At a PaO2 > 70 mm Hg, the SaO2 is 100%. How-
fetus and the newborn, gray matter CBF increases ever, when the PaO2 reaches about 50 mm Hg, the
at PaCO2 greater than 40 mm Hg but changes little SaO2 is 85%. Under conditions of reduced availabil-
at lower PaCO2 levels. Also, the change in CBF per ity of O2 (e.g., anemia), the regional CBF/PaO2 curve
mm Hg change in PaCO2 is higher in the newborn is shifted to the right (Fig. 4.3). Hypoxic elevations
than in the fetus, which suggests that the cerebro- in regional CBF are not associated with changes in
vascular response to CO2 is not complete at birth. metabolic rates. However, the vasodilation is addi-
This depressed CO2 response in the fetus may be tive with that produced by metabolic signals, par-
correlated to a difference in CMRO2 (i.e., when CBF ticularly acidosis and hypercapnia.
responses are normalized for CMRO2, the increase in
CBF is greatest in newborns, smaller in adults, and
even smaller in fetuses).
Hypocapnia does not alter the lower limit of cere- 4.3╇ Anesthetics Influencing
bral autoregulation in mature animals but does lead to
lower CBF per unit change in CPP. At lower BP, below
Postoperative Care
the level of cerebral autoregulation, there is an atten-
One should not manage a child in the postoperative
uation of the slope of the CBF/CPP graph, suggesting
period without knowing what has gone on before.
that the cerebral vascular response is attenuated with
Considering the pathway from preoperative treat-
hypotension. CBF in preterm infants is dependent on
ments to induction of anesthesia and the surgical
PaCO2. The cerebral vascular response to PaCO2 is less
procedure to recovery room care, and then admis-
in the first day after birth and increases with gesta-
sion to the ICU, as a continuum is absolutely essen-
tional age. It is also attenuated, but not eliminated, in
tial. Postoperative neurosurgical children will have
hypotensive infants. This reactivity is present even
been exposed to a number of therapies before and
in preterms; it is estimated to be about 4% per mm
during surgery. As discussed already, at the time
Hg PCO2. Intraoperatively, it is also preserved in chil-
of handoff, a formal assessment should be made of
dren (18 months to 7 years of age) anesthetized with
pre-, intra-, and postoperative issues (Table 4.2).
sevoflurane (see later section on drug effects on CBF).
An essential component of the handoff assessment
Hypocapnia may, therefore, result in cerebrovascular
is medicines, anesthetics, and fluid therapy. For
vasoconstriction and reduction of CBF.
example, the patient may have been hyperhydrated
in preparation for a cerebrovascular procedure (as
Clinical Pearl 6
Acute change in PaCO2 has an acute effect on CBF: Clinical Pearl 7
• Hypercapnia increases CBF by 6% per mm Hg
Acute change on PaO2 has an acute effect on CBF:
increase in PaCO2 above 40 mm Hg. Be con-
cerned about this possibility in the patient • Hypoxia (PaO2 ≤ 50 mm Hg) increases CBF.
with obstructed breathing or sleep apnea. • SaO2 < 85% increases CBF.
• Hypocapnia decreases CBF by 3% per mm Hg • The effect of SaO2 and PaO2 on CBF is exacer-
decrease in PaCO2 below 40 mm Hg. Be con- bated by the presence of anemia. That is, the
cerned about this possibility in the patient who homeostatic threshold for hypoxic drive to
is in pain and crying or is hyperventilating. increase CBF is raised. The hypotensive or hy-
pocapnic infant will not be able to mount this
protective response; thus, they are at risk.
38 Section Iâ•… Introduction
described later). They will have received anesthet- or remifentanyl) along with nitrous oxide (70%) and
ics that have been specifically used because they low-dose (0.2–0.5%) isoflurane is a frequently used
depress cerebral metabolism. However, it is the other technique, and when it comes to postoperative con-
unwanted cerebrovascular effects that may be of siderations, those caring for such patients should be
concern postoperatively. aware of cumulative dosing and timings. For exam-
ple, nitrous oxide may contribute to postoperative
nausea and vomiting and dose-dependent increase
4.3.1╇ Intraoperative Anesthetic Drugs in CBF. Clearly, we do not want a postoperative infant,
and Agents following a procedure on an arteriovenous malfor-
mation, to be vomiting such that episodic rises in
Volatile anesthetics act as potent vasodilators in the intrathoracic pressure are transmitted to intracranial
cerebral circulation. They are capable of uncoupling vessels (see Clinical Pearl 8).
the usual relationship between CBF and CMRO2
(described previously). Such uncoupling increases
CBV and, in consequence, raises ICP or worsens Clinical Pearl 8
intracranial hypertension. Desflurane and isoflu-
rane blunt the autoregulatory response of main- The cerebral circulation can be modeled as a Star-
taining CBF with change in CPP. Nitrous oxide has ling resistor, where the important “downstream”
vasodilatory effects. Both isoflurane and sevoflu- pressure is ICP or cerebral venous pressure, which-
rane decrease CMRO2, but flow-metabolism cou- ever is higher. Cerebral venous pressure may be
pling is maintained (Table 4.3). raised acutely by several causes of obstruction to
Intravenous anesthetics, sedative/hypnotic drugs, venous outflow from the cranium:
and opioids also have effects, but they do not cause
cerebrovascular vasodilatation. For example, barbi- • Kinking of neck vessels by head position, or neck
turates and propofol maintain autoregulation and vessels obstructed by central venous catheter
flow-metabolism coupling while reducing absolute • Neck collar that is too tight, or tight circumfer-
level in CBF, CBV, and CMRO2. Propofol maintains ential neck ties for securing tracheostomy
cerebrovascular reactivity to CO2 when the latter is • Periodic rise in intrathoracic pressure—cough-
above 30 mm Hg. However, propofol at high doses ing, crying, Valsalva maneuver, mechanical ven-
induces hypotension. Maintenance of anesthesia tilation with high mean airway pressure or high
during neurosurgery with an opioid (fentanyl or end-expiratory pressure (typically > 8 cm H2O)
other related synthetic opioids, such as sufentanil

Table 4.3â•… Effects of anesthetics, benzodiazepines, and opioids on cerebral metabolism, circulation, and intracranial
pressure

Pressure
Agent CMRO2 CBF autoregulation ICP

Inhaled anesthetic ↓↓ Â�↑ Absent Â�Â�↑


• Sevoflurane
• Isoflurane
• Desflurane

Inhaled nitrous oxide ↑ or no change Â�Â�↑ Present Â�↑Â�

Intravenous anesthetic ↓↓ ↓↓ Present ↓↓


• Propofol
• Thiopental

Dissociative anesthesia No change Â�Â�Â�↑Â�Â�↑Â� ? Â�Â�Â� ↑Â�


↑Â�Â�
• Ketamine

Sedative benzodiazepines ↓↓ ↓ Present ↓

Analgesic opioids No change No change Present No change

The direction of arrows indicates increase or decrease, and the number of arrows indicates the strength of the derangement above “no
change” qualitatively. Abbreviations: CBF, cerebral blood flow; CMRO2, cerebral metabolic rate for oxygen; ICP, intracranial pressure.
4â•… Pre- and Postoperative Management of the Neurosurgical Patient 39

4.3.2╇ Emergence from Anesthesia approved in your institution—the authors’ choice is


labetalol since it is readily titratable. Dexmedetomi-
At the end of the operative procedure, in the neuro- dine infusion is being used increasingly in pediatric
surgical patient, careful planning is required to bal- neurosurgery; there are no data, but its use is gaining
ance the need for early endotracheal extubation and popularity because it acts as a sedative, sympatho-
awakening so as to obtain a clinical examination ver- lytic, and analgesic.
sus the risk of profound hemodynamic instability on
cerebral hydrodynamics. There are, potentially, two
interlocking vicious cycles in pathophysiology that Emerging from Anesthesia
ultimately lead to hemodynamic instability, cerebral
edema, and intracranial hemorrhage (Fig.€4.4), and Cases for Consideration of Delaying Extubation
these should be considered in the context of what and Emergence from Anesthesia
is required in the postoperative period. Hence, in
• Preoperative altered level of consciousness
some high-risk children there might be a plan for
postoperative mechanical ventilation or deep seda- • Surgery lasting > 6 hours
tion (see box Emerging from Anesthesia). Emer- • Large tumor resection with preoperative mid-
gence agitation may be due to pain, a full bladder, line shift
dysnatremia (described later), drug reaction (e.g., • Injury to cranial nerves IX, X, XI
paradoxical reaction to midazolam or diphenhydr-
amine), or emergence delirium (e.g., reaction to • Complications during surgery (see Table 4.5)
sevoflurane). The treatable causes should be identi- • Intraoperative brain swelling
fied and dealt with or even anticipated. For example, • Hypothermia
pain and agitation can be anticipated and treated • Coagulopathy
with bolus doses of opioids, propofol, clonidine, or
dexmedetomidine. The stress response with emer- • Acid-base abnormality
gence hypertension can be treated with low-dose • Dysnatremia (see text for details)
fentanyl infusion (1.5 µg/kg/hr) and an antihyperten-
sive. In regard to the antihypertensive, the choice is
between a beta-blocker (labetalol or esmolol) and a
calcium channel antagonist (nicardipine). There is no 4.3.3╇ Failure to Awaken
ideal drug: beta-blockers have the risk of bradycardia
and cardiac conduction delays, whereas the calcium Occasionally, a patient may unexpectedly fail to
channel antagonists cause cerebral vasodilation, awaken at the end of surgery. A number of factors
impaired autoregulation, and risk of hypotension. should be considered and corrected (Table 4.4) and,
Use what you are familiar with and what has been if necessary, emergency imaging should be done.

Fig. 4.4â•… Stress-induced pathophysiology during emergence from anesthesia. CBF, cerebral blood flow; CMRO2, cerebral metabolic
rate for oxygen; ICP, intracranial pressure; mBP, mean blood pressure.
40 Section Iâ•… Introduction
Table 4.4â•… Factors to consider in the patient failing to worsening intraoperative cerebral hydrodynam-
wake up at the end of surgery ics (as described previously) and may lead to brain
injury. Seizures, hyperpyrexia, and hypoglycemia
Factor Intervention may also require management. The anesthesiologist
will attend to these and notify the operating neu-
Hypothermia Warm patient
rosurgeon of the occurrence. Table 4.5 provides an
Hypoglycemia Check blood glucose and treat overview of specific problems that may also occur
accordingly during and after surgery. (Hemorrhage, “brain relax-
ation,” and cerebral edema are considered in later
Hypercapnia Check blood gas and supportive sections.)
ventilation

Hypo- Check serum electrolytes and treat


osmolality accordingly
4.5╇ Perioperative Intravenous
Hypothyroid Thyroid hormone required for
benzodiazepine metabolism; support
Fluids
patient until patient awakens and
The prescription of intravenous maintenance flu-
thyroid function tests available
ids for children unable to tolerate oral therapy is
Neuromuscular Impaired hepatic and/or renal fundamental in perioperative care. What we want
blockade metabolism may prolong the effect to know is how much and what type of fluid we
should give postoperatively. The main aims of fluid
Altered drug See thyroid above; also consider therapy are, first, to have a hemodynamically stable
metabolism drug dosing and drug clearance of patient; second, to avoid electrolyte abnormalities;
continuous infusions used
and third, to ensure adequate glucose control. The
Seizure Assess and treat accordingly first aim requires careful maintenance of intravas-
cular volume. Preoperative fluid restriction or use
Intracranial Imaging required and surgery of mannitol, hypertonic saline, or diuretics may lead
bleeding to BP instability and even cardiovascular collapse
Intracranial Imaging required
intraoperatively, with problems carrying over into
pressure/ the postoperative phase. It is therefore important to
ischemia know what has happened intraoperatively, the esti-
mated blood loss as a proportion of blood volume, as
well as fluid inputs and outputs before ward or ICU
admission.
Normal saline is the preferred intravenous fluid
for neurosurgery cases because its osmolality (308
mOsm/L) should minimize occurrence of hypona-
4.4╇ Surgery-Related Perioperative tremia (serum sodium < 135 mmol/L) and cerebral
Crisis Management edema. In pediatric practice, we calculate rate of
maintenance fluid administration scaled to weight of
In common with many forms of surgery, in neuro- the patient using the classic Holliday and Segar for-
surgery there is the general risk of infection, bleed- mula for daily fluids: 100 mL/kg for the first 10 kg in
ing, and deep vein thrombosis. In infants and small body weight; add 50 mL/kg to 1,000 mL for the next
children there is also the risk of postoperative hypo- 10 kg in body weight (i.e., weight 10 to 20 kg); add
thermia, which may affect the postoperative course 20 mL/kg to 1,500 mL for weight above 20 kg. These
if there is significant vasoconstriction. For example, rates are based on normal conditions in health and
shivering related to hypothermia or volatile anes- they may not reflect what is needed in the operating
thetics can double the body’s oxygen consumption room. The following are examples of the issues that
and increase mean arterial blood pressure by 35%. need to be considered. How long was the procedure?
Both of these responses may be counterproductive How much maintenance fluid has been given? It is
in the child undergoing arterial bypass surgery for not unusual for long procedures to necessitate main-
moyamoya and should therefore be avoided. tenance fluid up to 10 mL/kg/hr. Were any saline
In regard to the general principles for brain resus- boluses given? When more than 60 mL/kg has been
citation that may be required, children can be con- used, there is the risk of hyperchloremic metabolic
sidered the same as adults. That is, hypotension, acidosis, which may not be appreciated unless serum
hypoxia, and hypercapnia all have the potential for chloride is measured.
4â•… Pre- and Postoperative Management of the Neurosurgical Patient 41
Table 4.5â•… Surgery-related perioperative complications and their management
Complication Risk Treatment

Aspiration of gastric contents •P


 atients with full stomach At the time of endotracheal intubation:
•P
 atients with raised ICP and vomiting in the •S uction airway before mechanical
presence of altered level of consciousness ventilation (MV)
•M V with 100% oxygen (FiO2 1.0) and
positive end expiratory pressure (PEEP)
•P ostoperative MV should be anticipated

Intrathoracic trauma •H
 eart, lung, and great vessel injury from Evaluate for signs of cardiorespiratory-
procedure to tunnel sheath for VPS abdominal instability and treat accordingly

Latex allergy •P
 atients with myelomeningocele A type 1 IgE-mediated reaction with
•P
 atients with sensitization to latex urticaria, angioedema, bronchospasm, and
anaphylactic shock:
•P revent further exposure by removing all
triggering agents
•A ssess airway, breathing, and circulation
(ABC) and treat with full cardiorespiratory
support if needed
•S urvey of the skin for urticaria
•R educe or discontinue volatile anesthetics
•T reat, if needed, with airway, oxygen,
fluid bolus, epinephrine, steroids, and
antihistamines
•S end off serum tryptase levels once the
patient is stable

SDH or upward herniation Rapid lowering of open EVD or Evaluate neurologic status for signs of raised
decompression of hydrocephalus: ICP:
•H emorrhage: rupture of cortical bridging •A  BCs and support if required
veins • Imaging
•U pward herniation: brain stem signs with •S  urgical intervention as required
bradycardia, EKG changes, and irregular
breathing

Venous air embolism (VAE)  uring placement of ventriculoatrial shunt


•D VAE is recognized by an abrupt fall in EtCO2,
•D
 uring craniosynostosis repair hypoxia, hypotension, bradyarrhythmia,
•S
 urgical site higher than heart and “mill wheel” murmur. Avoid the use of
 ir in intravenous tubing
•A nitrous oxide because of its low blood-gas
•P
 ersistence of patent foramen ovale, which partition coefficient and its ability to increase
increases the potential for paradoxical air the size of any air embolus that occurs.
embolism Treatment of VAE should be immediate:
•C onsider compressing jugular veins
•S urgeon to flood the operative field or pack
wound/bone; lower surgical site to below
heart level
•M V with FiO2 1.0 and zero PEEP;
discontinue volatile anesthetic, Valsalva
maneuver and prevent negative ITP
•A spirate air in central line in situ
•A BCs and full cardiorespiratory support if
needed

Hemodynamic instability •S
 timulation of floor of third ventricle with General evaluation for causes of
during ETV arrhythmia, bradycardia, asystole; or, hemodynamic instability, such as hypoxia,
hypertension from catecholamine release hypercapnia, distended bladder, awareness,
•R
 aised ICP and raised ICP. In addition:
•A  BCs and support if required
• In case of procedure-related stimulus, treat
if it does not resolve spontaneously when
stimulation has stopped

(Continued on page 42)


42 Section Iâ•… Introduction
Table 4.5 (Continued)â•… Surgery-related perioperative complications and their management
Complication Risk Treatment

Postoperative aSAH Rare in children but can occur postoperative Evaluate with serial TCD and ad hoc use of
vasospasm day 4 to 14 adult therapies:
•T riple-H therapy, including: hydration,
hemodilution, and hypertension
•N imodipine, angioplasty

Normal perfusion pressure High-flow AVM with postoperative Should be anticipated in high-flow AVM
breakthrough parenchymal hemorrhage or cerebral edema cases:
•S taged embolization to surgical procedure
•M aintenance of normal to slightly low BP
postoperatively

AV fistulas and vein of Galen High-output cardiac failure May need hemodynamic support and MV
malformation

Moyamoya Bypass surgery with risk of stroke related to Postoperative risk of stroke should be
perioperative events or natural history anticipated and limited:
•A void dehydration, hyperventilation, and
hypotension
•A ggressive analgesia to minimize BP
fluctuations and hyperventilation

Abbreviations: aSAH, aneurysmal subarachnoid hemorrhage; AV, arteriovenous; AVM, arteriovenous malformation; BP, blood pressure;
BPS, ventriculoperitoneal shunt; EKG, electrocardiogram; EtCO2, end-tidal carbon dioxide; ETV, endoscopic third ventriculostomy; EVD,
external ventricular drain; FiO2, fraction of inspired oxygen; ICP, intracranial pressure; IgE, immunoglobulin E; ITP, intrathoracic pres-
sure; SDH, subdural hematoma; TCD, transcranial Doppler; VPS, ventriculoperitoneal shunt.

Before embarking on detailed analysis of salt


Clinical Pearl 9
and water balance, when an abnormally low serum
sodium level is received from the laboratory, first
Calculation of weight-based maintenance fluid
make some assessment of salt losses, for example,
requirement in a child: Administer
salt loss via CSF drainage. Second, consider whether
• 100 mL/kg for the first 10 kg (0–10 kg) the hyponatremia is actually pseudohyponatremia
• 50 mL/kg for the next 10 kg (11–20 kg) due to the use of perioperative radiologic nonionic
hyperosmolar contrast medium.
• 20 mL/kg for the next 10 kg (21–30 kg)
• 10 mL/kg for the next 10 kg (≥ 31 kg)

A 35-kg child should therefore receive 1,000


mL + 500 mL + 200 mL + 50 mL = 1,750 mL/day Clinical Pearl 10
or 73 mL/hr as maintenance therapy. In general, it
is good to limit the maximum calculated mainte- Salt loss via CSF drainage. For a child weighing 35
nance at an adult level of 2,500 mL/day. If, however, kg, we estimate the following:
the intention is to give 1.5 times maintenance, then
the volumes would be 2,625 mL/day or 109 mL/hr. • Usual sodium maintenance is 2 to 4 mmol/kg/
day (70 to 140 mMol/day)
• CSF drainage up to 400 mL/day is equivalent to
60 mmol sodium/day
4.6╇ Postoperative Dysnatremia
• In this instance, the child could require up to
(Hyponatremia and Hypernatremia) 200 mmol sodium/day (≈ 6 mmol/kg/day), and
we have not yet accounted for urinary losses.
Disorders of salt and water homeostasis are common
in postoperative neurosurgical patients. The four main Therefore, making an assumption that hypo-
renal salt and water-handling problems that occur in natremia is due to SIADH and therefore volume-
such children are inappropriate intravenous fluid admin- restricting the child, without first checking that
istration, euvolemic state of antidiuretic hormone excess adequate sodium replacement has occurred, is
(i.e., syndrome of inappropriate ADH excess [SIADH]), the wrong course of action.
cerebral salt wasting (CSW), and diabetes insipidus (DI).
4â•… Pre- and Postoperative Management of the Neurosurgical Patient 43

4.6.1╇ Inappropriate Intravenous Fluids pump are removed from the membranes of individ-
ual proximal tubular cells, leading to reduced ability
Postoperatively, there is significant risk of hospital- to reabsorb filtered sodium ions.)
acquired hyponatremia (serum sodium concentra- Studies from the 1950s to the 1990s in healthy
tion < 135 mmol/L). In this setting, postoperative adults looked at transitioning from low to high salt
pain, stress, nausea, vomiting, narcotics, and vol- intake (0.6 g/day to 20.5 g/day) and the reverse. Simi-
ume depletion may all have the potential to stimu- lar studies have been carried out using saline solu-
late vasopressin production and to induce a state of tions. A change to a high salt intake leads to a new
euvolemic hyponatremia not dissimilar to SIADH. steady state in hormone secretion, with high ADH,
However, what is also clear is that hypotonic fluids low renin, and low aldosterone. If there is a decrease
or restricted fluids may all result in hyponatremia. in rate of infusion of saline, the patient continues
There have been few trials of intravenous fluids in to excrete sodium because of secondary renal salt
postoperative neurosurgical patients in the pediatric wasting due to the prevailing endocrine environ-
ICU, but those carried out to date indicate that urine ment—high ADH, low renin, low aldosterone. There
tonicity is almost always fixed in the postoperative is at least a 2-day delay in restoring hormone physi-
period (e.g., in those receiving normal saline at main- ology back to baseline when salt intake is reduced
tenance, urine tonicity is ~ 200 mOsm/L, whereas in back to normal levels; the body is generally much
those receiving any other prescription, urine tonicity better at “switching on” rather than “switching off”
is lower, ~ 160 mOsm/L), and urine output is constant mechanisms.
at ~ 1 mL/kg/hr. What this indicates is that isotonic
fluids prevent postoperative falls in serum sodium
concentration, but hypotonic fluids may result in Clinical Pearl 11
falls in serum sodium concentration because of renal
desalination. In none of these studies was fluid over- Perioperative care with intravenous fluids is an
load or significant hypernatremia a complication. assault to homeostasis, even when using isotonic
Another important consideration when assess- fluids. Consider the following when assessing salt
ing salt and water balance is to review what has and water balance:
happened to the child throughout the perioperative • Always assess intake and output and measure
period. By nature, our physiology is geared toward the tonicity balance.
surviving a salt-poor dietary environment. Humans
are not very good at dealing with salt excess. So, for • Patients are at risk of hyponatremia when
example, take an average 4- to 8-year-old child who there are abrupt decreases in intake of normal
weighs between 16 and 26 kg. The recommended saline since we have induced a high-ADH, low-
salt intake per day in normal 4- to 8-year-olds is 1.2 renin, and low-aldosterone state with the high
g/day (20 mMol/day). Actual upper limit of intake by salt intake.
4- to 8-year-olds from observational studies in 2004 • Not all hyponatremia is SIADH; it may be “ap-
is ~ 2.6 g/day (44 mMol/day). In perioperative neuro- propriate” for the patient.
surgical practice, we commonly place patients on 1.5 • So called “salt wasting” in patients receiving
times fluid maintenance using normal saline, which high volumes of saline is not necessarily “cere-
in a 26-kg child is equivalent to a salt intake of 21.8 bral salt wasting,” as it may represent the new
g/day (374 mMol/day). We then often reduce intake, steady state of output matching input.
abruptly, to maintenance fluids of 14.5 g/day (249 • In the patient with hypothalamic-pituitary ab-
mMol/day). So, we have taken a child with a normal normality, remember two things and act upon
salt intake—albeit likely higher than what public the laboratory results when available: thyroid
health would recommend—and increased this intake hormone is needed to excrete a water load, and
by a factor of 8 perioperatively and then reduced this cortisol is also needed.
intake to a sixfold increase from baseline. During
these interventions we sometimes see unexpected
hypernatremia or hyponatremia.
Let us consider the “threat” to homeostasis that 4.6.2╇ Syndrome of Inappropriate
has occurred in the perioperative period and the Antidiuretic Hormone Secretion
physiologic attempts to rectify this change. High salt
intake is a powerful physiologic stimulus and results Postoperative hyponatremia, euvolemia, and ADH
in renal and hormonal changes to excrete sodium. excess defines the SIADH. As a screening approach,
The stimulus to sodium excretion is an expanded we often use the following parameters: serum sodium
arterial volume. The mechanism of renal loss of the concentration <€135 mmol/L, minimal or normal
salt load occurs in the proximal tubule. (The luminal urine output < 2 mL/kg/hour, variable urinary sodium
sodium transport system and the basolateral sodium concentration (spot urine sodium > 20 mmol/L), and
44 Section Iâ•… Introduction
variable urine osmolarity. Therefore, regular monitor- often >€130 mmol/L. Taking 0.6 L/kg body weight as the
ing of intravascular volume, urine output and tonicity, apparent volume of distribution for sodium, one should
and serum electrolytes is needed during the period of anticipate an immediate increase of 3 to 5 mmol/L in
administering intravenous fluids. The other causes of serum sodium concentration with a rapid intravenous
hyponatremia that need to be excluded are volume bolus of 4 to 6 mL/kg body weight of 3% saline.
depletion, edematous states (congestive heart failure,
cirrhosis, and nephrosis), renal dysfunction, adrenal
insufficiency, and hypothyroidism. If the cause is peri- Clinical Pearl 12
operative SIADH, it has occurred because of free water
retention at the same time as natriuresis, which main- Serum uric acid is a useful corroborating test in
tains fluid balance at the expense of serum osmolality. diagnosing SIADH:
The ADH excess leads to increased water permeability
• Serum uric acid is < 4.0 mg/dL.
in the collecting duct, water retention, and subclini-
cal volume expansion, with an increase in total body • Increased urate clearance with fractional ex-
water of 7 to 10%. Volume expansion also triggers cretion of urate > 12% (normal < 10%) at time of
hemodynamic regulatory mechanisms to maintain hyponatremia, which improves on correction
plasma volume at the expense of sodium, which is of hyponatremia.
in part due to a pressure natriuresis and a secondary
release of natriuretic peptides.
Because of the risk of hyponatremia in the periop- 4.6.3╇ Cerebral Salt Wasting
erative period, many clinicians choose to avoid using
hypotonic solutions altogether. It should be noted CSW is a diagnosis of exclusion based on clinical cri-
that Ringer’s lactate sodium (130 mmol/L) might also teria, and it is overdiagnosed in my experience. The
result in a fall in serum sodium (see preceding dis- essential features of the syndrome are renal sodium
cussion). This fluid is often used intraoperatively, as and chloride wasting in a patient with a contracted
it is a balanced solution with a physiologic amount effective arterial blood volume, where other causes
of base, calcium, and potassium and will limit the of excess sodium excretion have been excluded. Vol-
hyperchloremic acidosis that occurs with large vol- ume contraction is likely to be present when there is a
umes of normal saline. deficit of sodium that exceeds 2 mmol/kg. Hyponatre-
The treatment of SIADH is to reduce free water mia is a nonspecific clue. The literature suggests that
excess by fluid restriction and diuretics. If a hypona- this condition is common in children after all types
tremic seizure occurs, then hypertonic saline should of neurosurgical procedures and that it results from
be used to correct serum sodium; the level to be tar- excessively high atrial or brain natriuretic peptide lev-
geted is that at which the seizure comes under control, els. The box Cerebral Salt Wasting lists some of the

Cerebral Salt Wasting


Diagnosis of cerebral salt wasting in a patient • Presence of an inhibitor of renal reabsorption of
with excretion of sodium and chloride without sodium, such as osmotic agents or high concen-
obvious cause tration of ligands for the calcium receptor in the
loop of Henle (e.g., hypercalcemia, gentamicin)
Exclude: • Obligatory excretion of sodium by the excretion
of anions other than chloride
• A physiologic cause for the excretion of sodium
chloride (e.g., an expanded extracellular fluid • High-output renal failure (renal tubular damage,
volume from hyperhydration with 1.5 times such as obstructive uropathy, interstitial nephri-
maintenance followed by abrupt decreases in tis, and acute tubular necrosis)
intake) • Sodium wasting from CSF or drainage
• A noncerebral cause for natriuresis: Other explanations for salt wasting:
• Diuretics
• Natriuretic agents of cerebral origin
• States with low aldosterone (a stimulator for
reabsorption of sodium) • Downregulation of renal sodium transport by
chronic hypervolemia
• Adrenocortical insufficiency
• Pressure natriuresis from adrenergic agents
• Congenital salt-losing renal tubular disorders
(Bartter or Gitelman syndrome) • Suppression of the release of aldosterone
4â•… Pre- and Postoperative Management of the Neurosurgical Patient 45
diagnoses that should be excluded before concluding Knowledge of the patterns of DI that can occur
that the patient has CSW. (It should be noted that atrial following surgery in the hypothalamic-pituitary area
natriuretic peptide level is high in the patient who has is important. The most common is that associated
been managed with hypervolemic fluid management. with local edema as a result of traction or manipula-
In this instance, natriuresis as a response to volume tion of the pituitary stalk. This lesion usually results
control is part of homeostasis, and not CSW.) in transient polyuria that begins 2 to 6 hours after
The incidence of CSW is apparently on the order surgery and resolves as edema diminishes in 1 to 7
of 1 to 5% of neurosurgical procedures, and it has days. A “triphasic pattern” has also been described
been reported in association with calvarial remodel- (see Clinical Pearl 13). Transection of the pituitary
ing, tumor resection, and hydrocephalus. A screen- stalk or destruction of the hypothalamic median
ing approach, when there is concern about possible eminence will result in permanent DI. Frequently,
CSW, is hyponatremia (<€135 mmol/L) with brisk permanent DI, either partial or complete, develops
diuresis (>€3 mL/kg/hr) and elevated urine sodium without interphase changes.
(>€120 mmol/L) when available, or elevated urinary
osmolarity (>€300 mOsm/L water). The physiol-
ogy involves inappropriate and excessive release of Clinical Pearl 13
natriuretic peptides that leads to a primary natri-
uresis and volume depletion. A secondary hormonal The triphasic pattern in postoperative electrolyte
response occurs with an increase in the renin-angio- disturbance and DI, with the following phases:
tensin system and arginine vasopressin production. • Initial: polyuria in the first postoperative days
The median onset of CSW is on postoperative day 3,
lasting a median of 3 days. In contrast to hyponatre- • Second: normal urinary output or SIADH re-
mia due to SIADH, the fractional excretion of urate sulting from release of previously stored ADH
is elevated but does not improve when hyponatremia from damaged neurons
is corrected (see Clinical Pearl 12). Patients with • Depletion of vasopressin stores with polyuria
CSW are more likely to have suffered postoperative
stroke, to have chiasmatic or hypothalamic tumors,
and to be younger than patients with normal post- There are a variety of successful approaches to
operative sodium concentration. Almost half of the treating DI. It is useful to have a neuroendocrine
patients with CSW have postoperative hyponatremic assessment preoperatively, with a perioperative care
seizures (serum sodium < 130 mmol/L). The treat- plan, since deficiencies of thyroid and/or adrenocorti-
ment of CSW involves sodium administration to cal hormones can coexist. In the child with known DI,
match urinary losses and correction of intravascular preoperatively, some endocrinologists prefer not to
volume contraction. In some instances, more rapid replace vasopressin and to restrict total fluid intake to
resolution of hyponatremia after volume expansion approximately twice-normal maintenance (scaling to
has been achieved with fludrocortisone. body surface area rather than weight, i.e., 3 L/m2/day),
recognizing that this can result in mild hypernatremia
and thirst but minimizing the more dangerous risk of
4.6.4╇ Diabetes Insipidus water intoxication with vasopressin administration.
Others prefer to withhold long-acting DDAVP (des-
DI results from a deficiency of vasopressin, and it mopressin acetate, the synthetic analog of ADH) in
is an expected complication of surgical procedures the perioperative period and instead manage DI with
near the pituitary or hypothalamus. It is most fre- intermittent injections of intramuscular vasopressin.
quently seen in association with craniopharyngi- Administration of excessive fluids in this setting, as
oma, where it can be a presenting symptom in 40% with the perioperative maintenance of DDAVP, can
of cases. In most patients, DI is transient, but ~ 6% result in hyponatremic seizures.
develop permanent DI. The diagnosis should be sus- When new-onset, postoperative DI is recognized,
pected when serum sodium rises above 145 mmol/L the following strategy should lead to serum sodium
in association with urine output above 2.5 mL/kg/ concentrations between 130 and 150 mmol/L. These
hr for 3 consecutive hours, or more than 4 mL/kg/ patients respond to an infusion of aqueous vasopres-
hr in any one hour. The urine osmolality should be sin (20 units/500 mL). Aqueous vasopressin is used
hypotonic (<€300 mOsm/L) in the face of increased because of its rapid onset of action and brief dura-
plasma osmolality (>€300 mOsm/L) in the absence of tion of effect. However, its potential vascular effects
glycosuria, mannitol use, and renal failure. Important (i.e., hypertension) mean that close observation in a
consequences of this condition are severe dehydra- monitored setting is required. The infusion is started
tion and hypovolemia, since urine output is driven by at 0.5 mΥ/kg/hr and titrated upward in 0.5 mU/kg/
lack of vasopressin. hr increments every 5 to 10 minutes until urine out-
46 Section Iâ•… Introduction
put decreases to less than 2 mL/kg/hr. It is rare to and adolescents can tolerate 18 to 24 hours of fasting.
require more than 10 mU/kg/hr. Once urine output is One risk of giving exogenous glucose is that, with the
less than 2 mL/kg/hr, the vasopressin infusion is not stress of critical illness (and resulting insulin resis-
adjusted downward. Neither is fluid administration tance), hyperglycemia may be induced and, in turn,
adjusted according to urine output. Antidiuresis with may be associated with neurologic injury and poor
vasopressin is essentially an “all-or-none” phenom- outcome. However, while it is known that hypergly-
enon, and the aqueous infusion is used to produce cemia may worsen ischemia, it remains unclear that
a “functional SIADH” state. This strategy recognizes the opposite—tight glycemic control—offers signifi-
that renal blood flow remains normal in the normo- cant benefits to children. Therefore we follow a con-
volemic, but maximally antidiuresed, child. Because servative approach that maintains the random serum
urine output is then minimal (0.5 mL/kg/hr), other glucose level in the normal range, below 180 mg/dL.
clinical markers of volume status must be followed
closely. For example, anuria together with increased
heart rate or decreased BP may be evidence of hypo-
volemia. Vasopressin infusion does not induce acute 4.7╇ Brain Relaxation and
tubular necrosis, and severe oliguria or anuria is an Treatment of Cerebral Edema
indication for additional fluid and not for decreasing
or discontinuing the infusion. When vasopressin is Cerebral edema may occur intraoperatively in the
used, infusion fluids should be carefully restricted patient with open dura and visible brain swelling.
because in the presence of full antidiuresis, exces- It may also occur postoperatively. Table 4.6 summa-
sive fluids (oral or intravenous) can lead to intravas- rizes the treatments used to prevent and treat cere-
cular volume overload. In addition, administration bral edema. There is no evidence base for using these
of hypotonic fluids (oral or intravenous) can result therapies in general neurosurgery, and it should be
in dangerous hyponatremia. Restricting fluids to noted that Table 4.6 is merely guidance on what
replacement of insensible losses, which is generally people report that they use, often borrowed from
considered to be about two-thirds usual mainte- traumatic brain injury (TBI) care. For the most up-
nance rates, may prevent this complication. to-date guidance on the management of raised ICP
In children at risk of developing permanent DI, in the context of severe TBI in infants, children, and
in whom adequate oral intake has been established, adolescents, the reader should look at the searchable
intravenous fluids and the vasopressin infusion can guidelines on the Brain Trauma Foundation website
be discontinued while free oral intake is permitted. (see http://braintrauma.org/coma-guidelines/). The
Subsequent treatment of DI is withheld until the child second edition of the pediatric guidelines was pub-
demonstrates polyuria. At this time, treatment with lished in 2012 and is available as open access.
DDAVP, rather than restarting a vasopressin infusion,
is recommended. DDAVP is a synthetic vasopressin
with duration of action of 12 to 24 hours. It is usually 4.7.1╇ Rescue Hyperosmolar Therapy in
administered intranasally at a dose of 5 to 10 micro- Suspected Cerebral Edema
grams. Oral DDAVP can be used at 10 to 20 times the
nasal dose. Antidiuresis generally begins within 1 Two intravenous hyperosmolar agents are used for
hour. In children with known DI, DDAVP treatment the treatment of cerebral edema: mannitol and 3%
can be resumed once an intact thirst mechanism has hypertonic saline (3% HS). Increasingly, 3% HS is the
returned and oral intake occurs without vomiting. preferred osmotherapy agent rather than mannitol.
There are no randomized controlled trials support-
ing this practice, so it is important to understand the
4.6.5╇ Glucose or No Glucose in advantages and disadvantages of these therapies.
Perioperative Intravenous Fluids
Intraoperatively, the stress response is generally Mannitol
able to maintain normal serum glucose levels with-
out exogenous glucose administration. However, in Mannitol has the potential to fail in treating cere-
postoperative infants and small children, particularly bral edema. An intravenous bolus dose is distributed
if there has been an effective fast of 6 to 12 hours, throughout the extracellular fluid within 3 minutes,
there is the risk of perioperative hypoglycemia. It is except in areas protected by the intact blood-brain
therefore advisable to use glucose-containing fluids barrier (BBB). There is an acute rise in extracellular
to meet baseline demands. Infants require continu- osmolality in body tissues because mannitol (molec-
ous infusions of glucose at 5 to 6 mg/kg/min in order ular weight 182) causes an influx of water from the
to maintain serum levels. Hence, normal saline in 2.5 intracellular compartment; this restores osmotic
to 5% dextrose is often used. In general, older children equilibrium between the intracellular and extracel-
4â•… Pre- and Postoperative Management of the Neurosurgical Patient 47
Table 4.6â•… Interventions for limiting and treating cerebral edema

Factor or agent Intervention

Cerebral venous drainage • Head elevation: increased risk of venous air embolism if craniotomy is underway (see Table 4.5)
• Head midline
• Minimize intrathoracic pressure

CPP optimization • No guidelines as to level (see text for details)


• Target normal mean arterial blood pressure (see Fig. 4.1)

PaCO2 optimization • Target the normal range of 35 to 40 mm Hg


• Mild hyperventilation (30 to 35 mm Hg) can be used for short periods

Intravenous hyperosmolar • Mannitol: 0.25 to 1 g/kg raises serum osmolarity by 10 to 20 mOsm/L


therapy (see text for details)

Anesthetic agents • Barbiturate bolus: induced cerebral vasoconstriction and reduces CMRO2
(see Table 4.3) • Propofol bolus (see Table 4.3)

Dexamethasone • Used in vasogenic edema around tumor or abscess


• Complications include stress hyperglycemia, hypertension, and septic shock

Hypothermia • Reduces CMRO2, but of no proven value in TBI


• Avoidance of hyperthermia may be just as effective

CSF drainage • May be used if there is access to the lateral ventricles

Abbreviations: CMRO2, cerebral metabolic rate for oxygen; CPP, cerebral perfusion pressure; CSF, cerebrospinal fluid; ICP, intracranial
pressure; PaCO2, partial pressure of carbon dioxide in arterial blood; TBI, traumatic brain injury.

lular compartments, at a higher volume than before 3% Hypertonic Saline


the drug was given. This water shift dilutes and low-
ers serum sodium concentration. Subsequent renal As noted already in the discussion of SIADH, taking
clearance of mannitol from the circulation produces 0.6 L/kg body weight as the apparent volume of dis-
an osmotic diuresis and elimination of free water, tribution for sodium, one should anticipate an imme-
further raising total body osmolality and serum diate increase of 3 to 5 mmol/L in serum sodium
sodium concentration. The net effect of the initial, concentration with a rapid intravenous bolus of 4 to
single dose of mannitol is no change or a small net 6 mL/kg body weight of 3% HS. Table 4.7 summarizes
rise in total body osmolality. However, repeated dos- the data that better explain the effect of changes in
ing of mannitol over 48 hours, with coadministra- serum sodium concentration during the course of
tion of isotonic saline, will lead to a consistent rise in treatment for cerebral edema. What is important is
serum osmolality and serum sodium concentration. the state in serum–BBB–extracellular space kinetics.
For example, the half-life for equilibration of sodium
across the intact BBB is 1 hour, but less if the bar-
Clinical Pearl 14 rier is disrupted. This means that change in serum
sodium concentration as a strategy for treating cere-
Nonresponse to intravenous mannitol is well bral edema will, theoretically, become less effec-
recognized after a single bolus dose: tive the longer one adopts this strategy—unless, of
course, one is prepared to continue to escalate serum
• Hyponatremia is well described in the neurol- level to very high values. We should also note from
ogy/neurosurgery intensive care literature. Table 4.7 that the choroid plexus behaves more like
• “Nonresponse” with failure to elevate serum the peripheral circulation, since it has a reflection
sodium concentration by ≥ 1 mmol/L over 48 coefficient to sodium of zero. Hence, changing serum
hours of treatment is seen in at least 22% of neu- sodium concentration will have no effect on limiting
rocritical care adult cases receiving mannitol for water flow across the endothelium within the cho-
cerebral edema and raised intracranial pressure. roid plexus, but mannitol would have such an effect.
• In practice, it makes sense to use 3% hypertonic Similarly, as vasogenic cerebral edema worsens, one
saline first, and then mannitol. would expect a fall in the reflection coefficient for
sodium (decrease from 1 to 0.93 to zero). Therefore,
48 Section Iâ•… Introduction
Table 4.7â•… Blood–brain barrier (BBB) physiologic function based on the Starling equation
Reflection coefficient Permeability-
Intravenous Effective surface product
solution mOsm/L BBB CP (mL [g × min]–1) Half times (t1/2)

Saline 0.9% 285 1.00 1.00 2.4 × 10–4 1 hour

Mannitol 20% 1,100 0.90 > 0.53 1.0 × 10–3 2.3 hours

3%-HS 1,026 1.00 1.00 2.4 × 10–4 1 hour

Albumin — 1.00 — 1.5 × 10–6 —

According to the Starling equation:


Jcap = Lcap [(Pplasma – Ptissue) – σprotein(πprotein,plasma – πprotein,tissue) – σsalt(πsalt,plasma – πsalt,tissue)]
Driving pressure = (Pplasma – Ptissue) – σprotein(πprotein,plasma – πprotein,tissue) – σsalt(πsalt,plasma – πsalt,tissue)
where Pplasma – Ptissue is the hydrostatic pressure difference between plasma and tissue; πprotein,plasma – πprotein,tissue is the dif-
ference in protein osmotic pressure between plasma and tissue; Lcap is capillary hydraulic conductivity; Jcap is capillary water flow; σ is
the osmotic reflection coefficient; R is the universal gas constant (0.082 L×atm/mol×K); T is the absolute temperature (kelvins); Csolute
is the concentration of impermeant solute, and πsolute,plasma is the solute osmotic pressure in plasma; BBB, blood–brain barrier; CP,
choroid plexus; permeability-surface product the capillary hydraulic conductivity; half times, approximate exchange half times of the
intact BBB for various substances; 3%-HS, 3% hypertonic saline. See text for details.

prolonged and repeated dosing of intravenous 3%-HS coagulation is likely. At this level, serum prothrom-
is not without risk—it will have limited effect and bin and partial thromboplastin times should be
may even worsen the edema as the BBB fails. obtained and fresh frozen plasma given if necessary.
Taken together, in patients with acute onset of The derangements in the balance between coagula-
cerebral edema, it seems reasonable to start with tion and anticoagulation may be complex (e.g., some
3%-HS to gain acute control, but mannitol should degree of hypercoagulability, which may be related
also be introduced (molecular weight 182, reflection to hemodilution, or as hemorrhage approaches 100%
coefficient of 0.9 at BBB). of blood volume, coagulopathy due to factor deple-
tion) and advice from the blood bank and transfusion
service is often required. Your hospital should have a
Massive Transfusion Protocol, and for certain proce-
4.8╇ Blood Loss dures blood loss should be anticipated and a special
protocol used. This strategy may include anticipatory
Intraoperatively, neurosurgical trauma may lead to use of tranexamic acid, fresh frozen plasma, plate-
uncontrollable bleeding because of the technique lets, cryoprecipitate, and calcium gluconate. In the
being used. For example, during endoscopic ven- postoperative period, blood loss via drains should be
triculostomy, there is risk of trauma to the basilar followed closely, and signs of significant blood loss
artery or its branches, which will result in bleed- (tachycardia, hypotension, hypocapnia, and respira-
ing and hemodynamic instability. In this instance, tory variation in systolic blood pressure) should be
emergency craniotomy is likely to be needed as well recognized and acted upon.
as other rescue therapies (e.g., intravenous fluids,
vasopressors, blood and blood products, mechanical
ventilation with 100% oxygen, and ICP-directed ther-
apies). In surgeries involving craniotomy, there may 4.9╇ Postoperative Pain and
be some degree of accumulated blood loss during the Sedation Management
procedure, and blood should be replaced.
Ideally, postoperative neurosurgical patients are
comfortable, awake, and sufficiently cooperative so
4.8.1╇ Massive Blood Loss as to complete serial neurological examinations and
not be at risk of hyperventilation and its consequent
Massive blood loss may occur in young infants under-
effects on cerebral hydrodynamics. In pediatrics,
going intracranial tumor surgery or in infants under-
these goals can be difficult to maintain because of
going craniosynostosis repair, for example. When
differences in development and understanding of the
blood loss reaches 50 to 75% of the preoperative blood
patient.
volume (or 40 to 60 mL/kg), some derangement in
4â•… Pre- and Postoperative Management of the Neurosurgical Patient 49

4.9.1╇Analgesia 4.9.2╇ Nausea and Vomiting


Each patient should have an age-appropriate assess- Postoperative nausea and vomiting are horrible and
ment of pain. Various scales are used, and you should can lead to changes in cerebral hydrodynamics that
be familiar with the protocol in your own institution, are counterproductive to good recovery and heal-
such as the Modified Infant Pain Scale in infants or ing after operations requiring craniotomy. They
the Visual Analog Scale in older children. Even the are frequent in infants and children and should be
most minimal scalp incision can cause postopera- prevented with prophylactic antiemetic drugs. It is
tive pain. Surgery around the neck may result in sig- common practice to start administering these agents
nificant nuchal spasm, and regular muscle relaxants intraoperatively (Table 4.8). Again, use the agent
are required. The postoperative strategy for muscle your hospital prefers for infants and children. Some
relaxation and analgesia should be discussed during agents may be better at preventing vomiting but
the formula one signoff (Table 4.2), since what has have no effect on nausea.
occurred intraoperatively influences what is optimal
postoperatively. For example, short-acting intrave-
nous opioids (e.g., fentanyl, remifentanil) may have 4.9.3╇ Intensive Care Unit Sedation and
been used along with longer-acting agents (e.g., mor- Analgesia
phine). Prior remifentanil usage is associated with
subsequent tolerance to opiates and a high postop- For some patients on extended management in the
erative opiate requirement. All hospitals will have a ICU, a low level of sedation with analgesia is required
pain service, and a planned postoperative protocol using a combination of opioid and benzodiazepine
will be in place (which includes use of patient- or via continuous infusion. The ideal sedation includes
nurse-controlled analgesia, monitoring for respira- short-acting or reversible agents that can be with-
tory depression and somnolence, and indications drawn intermittently to permit assessment. Some
for narcotic reversal with naloxone), which should agents suitable for adults are unsuitable in chil-
be followed. Although intravenous opiates are fre- dren, and some agents used widely in pediatrics are
quently used, other agents, such as acetaminophen less useful in adults. In the mechanically ventilated
and nonsteroidal anti-inflammatory drugs (NSAIDs), child, the most commonly used sedative agent is
may be a useful addition to lessen the total dose of midazolam. Titration to a validated sedation score is
opiate and to reduce the risk of respiratory depres- recommended, and regular “drug holidays” help pre-
sion and compromised neurologic examination. vent excessive sedation and tolerance. Infants and
Table 4.8 lists pediatric dosages that may be used in children receiving sedative infusion for more than
the postoperative period. 5 days are at risk of withdrawal when infusions are

Table 4.8â•… Pediatric dosages of analgesics and antiemetics used in the postoperative period
Agent Dosage Effect

Acetaminophen •O
 ral: 10–15 mg/kg q4h Analgesia
•R
 ectal in children: initial dose 30 mg/kg, then •M aximum dose in children is 100 mg/kg or
subsequent dosage 20 mg/kg q6h 4 g/day, whichever is lower
•R
 ectal in neonates: initial dose 30 mg/kg, then •M aximum dose in neonates and infants is
subsequent dosage 20 mg/kg q12h 75 mg/kg/day

Oxycodone Oral: 0.05–0.15 mg/kg Analgesia

Morphine • Intravenous in infants (> 2 months) and children: Analgesia


incremental dosages of 0.025–0.1 mg/kg •D osing interval will be affected by half-life
•P  CA: 10–20 µg/kg/dose, lockout 8 to 15 min, (2 hours after 2 months)
basal 0–30 µg/kg/hour • PCA 4-hour limit of 250–400 µg/kg

Ondansetron • Intravenous: 0.15 mg/kg Antiemetic

Dexamethasone • Intravenous: 0.0625–1.0 mg/kg Antiemetic

Abbreviations: PCA, patient-controlled analgesia; q4h, every 4 hours; q6h, every 6 hours; q12h, every 12 hours.
50 Section Iâ•… Introduction
discontinued abruptly. In regard to analgesia, opi- 4.11╇ Brain Death
oids like morphine and fentanyl should be carefully
titrated to minimize postcraniotomy pain yet main- The determination of brain death in infants and chil-
tain consciousness. Patient-controlled analgesia may dren is a clinical diagnosis based on the absence of
be helpful. neurologic function with a known reversible cause
Propofol is a potent, ultrashort-acting sedative-hyp- of coma. In the United States, the American Academy
notic that is extremely useful in adult neurocritical care of Pediatrics recently updated the 1987 Task Force
but has only limited utility in pediatrics. This is because Recommendation for Guidelines for the Determination
of its association with the propofol infusion syndrome— of Brain Death in Infants and Children. These guide-
a fatal syndrome of bradycardia, rhabdomyolysis, meta- lines are open access and available from the U.S.
bolic acidosis, and multiple organ failure—when used Department of Health and Human Services Agency
over extended periods. While the mechanism of the for Healthcare Research and Quality website (see
syndrome remains unclear, it appears related to both http://www.guideline.gov/content.aspx?id=34605).
the duration of therapy and the cumulative dose. These In this latest document, after establishing a known
difficulties are much less common in adults. Some cen- mechanism and cause of coma, the diagnosis
ters have advocated propofol use in children under strict requires normothermia, normotension, normoxia,
controls, but propofol is generally limited to operative and the absence of confounding toxins or medica-
anesthesia, procedural sedation, and continuous infu- tions. The examination seeks to establish the com-
sions of limited duration (<€24 hours). plete absence of cortical and brainstem function. An
Dexmedetomidine (an intravenous alpha-2 ago- apnea test (documenting the absence of respiratory
nist), a newer agent, is an ultrashort-acting, single- effort despite PaCO2 > 60 mm Hg, from a baseline
agent sedative sometimes used in the postoperative of 40 mm Hg) is conducted last. In order to estab-
period. Studies involving children are preliminary, lish irreversibility, age-related observation periods
with centers reporting case series. The drug appears to are necessary. An observation period of 24 hours is
be effective when used for periods of 24 hours or less. required for infants up to the age of 30 days of age,
Opioid cross-tolerance makes it a useful agent for treat- and 12 hours for infants and children older than 30
ment of fentanyl or morphine withdrawal. Transient days and younger than 18 years. The first exami-
increases in BP can be seen with boluses, followed by nation determines whether the child has met the
hypotension and bradycardia as sedation deepens. accepted neurologic examination criteria for brain
death. The second examination confirms brain death
based on an unchanged and irreversible condition. It
is recommended that assessment of neurologic func-
4.10╇ Seizures and Status tion after cardiopulmonary arrest or other severe
Epilepticus acute brain injuries should be deferred for 24 hours,
or longer if there are concerns or inconsistency in the
Postoperative seizures are uncommon, but they do examination.
have the potential for devastating effect. In known Ancillary studies, such as electroencephalogra-
epileptics there should be a postoperative plan for phy and radionuclide cerebral blood flow studies,
seizure control. Phenytoin is commonly used for pro- are not required to establish the diagnosis of brain
phylaxis, but maintaining therapeutic serum levels death, nor are they a substitute for the clinical exam-
can be a challenge. Levetiracetam is being used more ination. These studies are used when components
frequently, possibly because of the ease of use. Both of the clinical examination or apnea testing cannot
drugs can be given intravenously, but, in contrast be completed safely because of the patient’s medical
to phenytoin, administration of levetiracetam does instability, if there is uncertainty about the results
not require electrocardiographic and hemodynamic of the neurologic examination, or if a medication or
monitoring. Neither is serum drug level monitoring metabolic effect is present.
required to avoid toxicity. The other antiepileptic Attendants who are familiar with practice in
drugs frequently used in pediatrics include pheno- adults will recognize an immediate difference
barbital, carbamazepine, and valproic acid. between the pediatric and adult criteria for brain
Status epilepticus can be treated with lorazepam death: in children, we continue to use two examina-
(0.1 mg/kg) as an intravenous push over 2 minutes. tions, whereas in adults the guidelines indicate that
Lorazepam may be repeated after 10 minutes and a single examination suffices. You should be familiar
may be accompanied by intravenous fosphenytoin with the protocol for determination of brain death in
(20 mg/kg) if the initial doses are ineffective. Phe- your own institution—all centers in the United States
nobarbital (20 mg/kg) is also an effective first-line have their own checklists and policy documents.
antiepileptic drug. Again, most hospitals will have an
agreed protocol for emergency seizure care and drug
management—be familiar with yours.
5 Pediatric Neurosurgical Positioning
Jonathan A. Pindrik, Sheng-fu Larry Lo, and Edward S. Ahn

5.1╇ Introduction and Background centers (including the authors’ institution) imple-
ment rigid pin fixation in children aged 3 years and
Positioning represents a significant component of above with incremented force starting at 30 to 40 lb
operative planning in all neurosurgical subspecial- (Fig. 5.1).1,3 The pin force may be increased toward
ties, including pediatric neurosurgery. Appropriate adult levels (60 lb, using adult pins) in older children
alignment of the head, neck, and spine for specific and adolescents with normal cranial development.1
operative approaches requires careful deliberation to Although skull thickness generally increases with
optimize target exposure, patient safety, and surgeon age, a high degree of variability exists among dif-
comfort. Nuances in the anatomy of infants, children, ferent age groups and patients.1 The calvaria within
and adolescents require special consideration to infants below age 1 year often ranges between 2
optimize patient safety. Key factors include anterior and 3 mm in thickness. Additional factors, such as
fontanelle patency, fusion of skeletal sutures, cal- chronic hydrocephalus, cause cranial vault thinning
varial vault thickness, strength and bulk of cervical in younger children.3,4 Therefore, rigid cranial fixation
paraspinal musculature, and overall torso or extrem- requires a carefully planned and tailored approach
ity size. Careful consideration of these factors, com- for individual patients.
bined with open communication among the surgical, To decrease the risks of cranial fixation in children,
nursing, and anesthetic teams, allows safe position- alternative strategies have been suggested to achieve
ing and optimal operative exposure in pediatric
neurosurgery.

5.2╇ Positioning Detail and


Preparation
5.2.1╇ Rigid Cranial Immobilization
Attaining rigid cranial fixation in infants and young
children presents challenges due to immaturity of
the cranial vault. Standardized recommendations
regarding cranial fixation in pediatric patients do not
exist, despite its common usage and frequent reports
of related complications. Skull thickness variability
within different age groups complicates the adop-
tion of uniform guidelines for cranial immobilization.
Some pediatric neurosurgical centers implement
rigid pin fixation in children aged 1 to 2 years, with
forces below 30 lb (often 20 lb or lower for young Fig. 5.1â•… Rigid cranial fixation. For midline prone positioning,
infants).1 However, forces below 30 lb carry risks of rigid cranial fixation with the Mayfield skull clamp at a pin force
pin slippage as a result of inadequate seating of the of 40 lb (in younger children) provides adequate stability and
pin upon the calvaria.2 Most pediatric neurosurgical support of the head.

51
52 Section Iâ•… Introduction
safe cranial immobilization. Assessing the skull thick- the potential risks and complications associated with
ness on preoperative head computed tomography (CT) other positioning styles (prone, lateral, sitting).
verifies the feasibility of applying rigid pin fixation.3
Furthermore, avoidance of the temporal squamosa
(if possible) during pinning may decrease the risks Ventricular Shunt Insertion or Revision
of skull fracture.3 Pediatric pins offer shorter, duller,
and blunter tips to help prevent skull perforation. Frontal, parietal, and occipital approaches to ventric-
Below age 3, other strategies include three-point skull ular shunt insertion or revision can be accomplished
clamps reinforced with rubber plugs or disks over the safely and effectively with supine positioning. While
cranial pins, combinations of the standard horseshoe frontal shunt manipulation favors neutral or near-
headrest and cranial fixation systems, or joint usage neutral head positioning, parietal or occipital shunt
of the horseshoe headrest and adhesive U-drapes to placement generally requires generous head rota-
achieve cranial immobilization.1–3,5 Incorporating a tion contralaterally. The supple nature of most pedi-
horseshoe headrest or suctioned beanbag to support atric necks (barring torticollis, contractures, or prior
the weight of the head allows the cranial pins or adhe- occipitocervical fusion) typically allows adequate
sive drapes to solely address immobilization.1,3,5 head rotation. A small to moderate-sized bump (sheet
or gel roll) placed under the ipsilateral torso may aid
surgical site exposure in addition to head turning.
5.2.2╇ Supine Position The patient’s torso can remain in a neutral position
with the upper extremities tucked and padded by the
Supine positioning involves the patient lying with his side. This supine, neutral position of the torso accom-
or her back and torso dependently on the operating modates distal shunt insertion into multiple termini
table. This straightforward style of positioning accom- (ventriculoatrial, ventriculopleural, and ventriculo-
modates various neurosurgical procedures (Table 5.1). peritoneal shunts). Proximal shunt revision or inser-
Transmitting the patient’s mass to a proportionately tion into noncomplicated, dilated ventricles typically
large surface area with adequate soft tissue and mus- can be performed without cranial fixation. In this con-
culature, supine positioning generally avoids many of text, the head may rest on circular foam padding or

Table 5.1â•… Examples of neurosurgical operations for different positions

Supine Prone Lateral Sitting/Concorde

Ventricular shunt insertion or Chiari malformation (I, II) Lumboperitoneal shunting Posterior fossa tumor or
revision decompression Intrathecal baclofen pump lesion resection
ETV Infratentorial tumor or lesion insertion or revision Pineal region tumor
Endoscopic supratentorial resection Retrosigmoid approach resection
arachnoid cyst Occipital tumor or lesion for lesions within lateral Occipital region tumor or
fenestration resection posterior fossa or lesion resection
Supratentorial tumor biopsy Myelomeningocele repair cerebellopontine angle
or resection Suboccipital decompression cistern
Endoscopic endonasal for cerebellar hemorrhage
approaches to skull base or edema (infarct, etc.)
lesions Posterior cervical, thoracic,
Seizure focus phase II or lumbar surgery
monitoring or resection Extended posterior cranial
Vagal nerve stimulator vault reconstructions
insertion or revision (prone with head in seal
Brachial plexus exploration position)
and repair
Supratentorial SDH or EDH
evacuation
Frontal, temporal, or parietal
skull fracture correction
Hemicraniectomy
Open craniosynostosis repair
Cranial reduction or
expansion

Abbreviations: EDH, epidural hematoma; ETV, endoscopic third ventriculostomy; SDH, subdural hematoma.
5â•… Pediatric Neurosurgical Positioning 53

Fig. 5.2â•… Supine positioning for ventricular shunt insertion. Standard supine positioning with the head turned contralaterally and
resting upon a horseshoe headrest allows access to the cranial ventricular shunt and distal peritoneal shunt insertion sites.

a cerebellar (horseshoe) headrest reinforced with soft


cotton wrapping (Fig. 5.2). Complex hydrocephalus
or diminutive ventricular caliber (slit ventricle syn-
drome) may require cranial fixation to allow intraop-
erative frameless stereotactic navigation.

Tumor Resection in the Frontal, Temporal,


Parietal, or Skull Base Regions

Depending on location, most lesions within the fron-


tal, temporal, and parietal regions or arising from the
skull base can be approached with supine position-
ing. While bicoronal approaches for midline pathol-
ogy typically employ neutral head positioning with
slight neck flexion, unilateral lesions usually require
head rotation contralaterally. Placement of a small Fig. 5.3â•… Cranial fixation and frameless stereotactic navigation.
to moderate-sized bump under the ipsilateral scap- Rigid cranial fixation allows reference registration of an intraop-
ula or torso may aid head rotation. Slight head tilt- erative navigation system and provides immobility necessary for
ing and neck extension allow the ipsilateral frontal critical steps in intracranial, neuro-oncologic surgery.
lobe to fall away from the anterior cranial fossa, as
needed for skull base lesions. Neck manipulation
(including rotation, flexion, and extension) should be Endoscopic Approaches to the Lateral and Third
performed carefully given the weak cervical paraspi- Ventricles
nal musculature. Excessive flexion or “kinking” of the
neck may result in internal jugular vein (IJV) occlu- Endoscopic approaches to the third and lateral ventri-
sion, impairing intracranial venous outflow and rais- cles via a precoronal burr hole typically employ supine
ing intracranial pressure (ICP). Rigid cranial fixation positioning with neutral head location (Fig.€5.4).
allows intraoperative neuronavigation and prevents Endoscopic third ventriculostomy (ETV) can be per-
slight head movement during key portions of the formed usually without cranial fixation and with the
procedure (craniotomy, dural opening, tumor resec- head resting upon a cerebellar (horseshoe) headrest
tion) (Fig. 5.3). reinforced with soft cotton wrapping. Endoscopic
54 Section Iâ•… Introduction

Fig. 5.5â•… Positioning for brachial plexus exploration and repair.


Brachial plexus exploration and repair often require supracla-
vicular and brachial approaches depending on the extent of
Fig. 5.4â•… Supine position for endoscopic third ventriculos- injury. The outstretched, sterilely prepared extremity may be
tomy (ETV). Standard positioning for stereotactic-assisted ETV placed on a draped arm rest.
via a precoronal burr hole involves supine neutral orientation
with slight neck flexion and attachment of the neuronaviga-
tion reference marker.
5.2.3╇ Prone Position
fenestration of arachnoid cysts may require differ- Prone positioning involves dependency of the face,
ent head positioning and angling depending on cyst ventral torso, and extremities on the operating table.
location. For instance, temporal or middle fossa arach- Entailing more risks than standard supine position-
noid cysts might employ contralateral head rotation ing, prone positioning requires careful deliberation
to optimize cyst entry through minimal cortex and to minimize pressure on dependent structures.
to foster cyst fenestration into the nearest cistern or Anatomic regions at risk include the face and ocu-
ventricle. lar globes, breasts in adolescent girls, abdomen in
overweight or obese patients, hips, male external
genitalia, and other structures under pressure or
Brachial Plexus Exploration and Repair compression. Padding helps minimize these risks by
cushioning firm regions (chest, hips) and allowing
Exploration and/or repair of the brachial plexus softer structures (breasts, abdomen, male external
introduce additional variables to consider for posi- genitalia) to hang freely. Rolled-up sheets or surgi-
tioning. Nerve stimulation to test for upper-extrem- cal towels aligned transversely under the chest and
ity movement usually requires sterile preparation of hips accommodate prone positioning in neonates
the involved arm, forearm, and hand into the surgi- and infants. Gel rolls (reinforced with soft cotton
cal field, or usage of partially transparent surgical wrapping) aligned longitudinally along the bilateral
drapes. Furthermore, harvest of unilateral or bilat- aspects of the patient’s torso can be used for older
eral sural nerve grafts for autologous graft recon- patients (children, adolescents). Upper extremities
struction will require sterile preparation of one or should be tucked at the sides (thumbs down) and
both lower extremities and an apparatus to raise cushioned with foam padding for cranial and cervi-
the lower extremity in a fixed position for patients cal operations or directed superiorly on arm rests
beyond infancy (alternatively, sural nerve grafts can (90° abduction at the shoulder and 90° flexion at
be obtained with the patient in prone position as the the elbow) for thoracic and lumbar operations. If not
first stage of the operation). Supine positioning with secured with rigid cranial fixation, the cranial vault
contralateral head rotation upon a circular foam should be placed face down on a cerebellar (horse-
headrest offers optimal exposure of the brachial shoe) headrest or special foam padding with clear-
plexus. Supra- and infraclavicular approaches to ings for the eyes, nose, mouth, and endotracheal
the brachial plexus incorporate similar positioning. tube. Both surgeons and anesthesiologists should
Distal exploration of the peripheral nerve branches check pressure around the orbit to minimize pres-
in the arm require elbow extension and shoulder sure transmitted to the ocular globes. Members of
abduction of the sterilely prepared upper extremity the surgical and nursing team also should check all
lying upon an arm rest (Fig. 5.5). dependent regions for appropriate padding and all
5â•… Pediatric Neurosurgical Positioning 55
freely hanging soft tissue structures (breasts, abdo- positions apply to lumboperitoneal shunting, inser-
men, external genitalia). Potential disadvantages tion or revision of an intrathecal baclofen pump, and
of prone positioning include decreased drainage of retrosigmoid approach for lesions within the lateral
cerebrospinal fluid (CSF) and blood and increased posterior fossa or cerebellopontine angle.
airway pressures.6 Several guiding principles can simplify the chal-
lenging nature of lateral positioning. Extension of
the dependent lower extremity and flexion of the
Infratentorial Operations above lower extremity (this orientation may vary
depending on surgeon preference) with an inter-
Positioning for infratentorial or craniocervical opera- posed pillow helps stabilize the lower body and
tions (Chiari decompression, posterior fossa lesion relax the ipsilateral iliopsoas muscle. Suction-
resection, suboccipital craniectomy) generally fol- assisted molding of a beanbag conformed to the
lows standard principles. Patients below the age body contour helps stabilize the torso. However,
of 3 years may be positioned without rigid cranial the beanbag should be contorted appropriately to
fixation, as described previously. However, rigid cra- maintain access to the lumbar posterior midline
nial fixation should be considered for older patients and lateral abdominal region for lumboperitoneal
(children and adolescents) (Fig. 5.6). Bringing the shunting and intrathecal baclofen pump insertion.
torso superiorly along the operating table with the An axillary roll or gel cushion inserted below the
shoulders lying just beyond the table’s edge allows dependent axilla provides cushion and support for
appropriate head positioning and guards against this region.
chin compression. Generous neck flexion and pos- The park bench position additionally employs
terior translation of the head place the suboccipi- rigid cranial fixation with head rotation contra-
tal region into a prominent and accessible location. laterally toward the ground to expose the suboc-
Furthermore, these maneuvers enhance exposure of cipital retrosigmoid region (Fig. 5.7). Placing the
the craniocervical junction and upper cervical spine. patient’s torso and surrounding beanbag superiorly
Excessive neck flexion and obstructed venous outflow on the operating table, with the level of the shoul-
can be avoided by checking for two-fingerbreadth der beyond the upper edge of the table, allows the
spacing between the chin and neck or sternal notch. dependent upper extremity to rest comfortably on
Positioning for posteriorly located (occipital) lesions foam padding and sheets supported by the Mayfield
above the tentorium can proceed similarly, without attachment (Integra, Plainsboro, NJ, USA). The non-
the need for substantial neck flexion. dependent upper extremity should be cushioned
with a pillow and secured with foam padding and
tape wrapped circumferentially around the torso
5.2.4╇ Lateral Position and beanbag (after suction-assisted molding). As
with other positions, all pressure points and ana-
Lateral positioning places the sides of the patient’s tomic regions abutting rigid structures should be
torso and lower extremities dependently to support checked to avoid excessive compression.
body weight. The lateral decubitus and park bench

Fig. 5.7â•… Park bench positioning. Park bench positioning


Fig. 5.6â•… Midline prone positioning. Rigid cranial fixation with employs rigid cranial fixation, beanbag stabilization, copious
midline prone positioning and cervical flexion provides ade- padding, and generous amounts of cloth tape to achieve safe
quate exposure of the suboccipital region and craniocervical immobilization and adequate exposure of the lateral suboc-
junction. cipital, retrosigmoid region.
56 Section Iâ•… Introduction

5.2.5╇ Sitting Position Intraoperative techniques to detect VAE include


transesophageal echocardiography (TEE) or precor-
More frequently used abroad, the sitting position dial Doppler ultrasound (pcDUS), measurement of
entails perioperative risks that limit its usage in most end-tidal pressures of carbon dioxide (PECO2), and
American centers. This orientation involves upper arterial line placement with continuous monitoring
torso elevation to 90 to 100°, with gentle cervical flex- of mean arterial pressure (MAP).6–9 Preoperative TEE
ion to tilt the head forward approximately 20 to 30° can help diagnose patent foramen ovale (PFO), a rec-
(Fig. 5.8).7,8 As in prone positioning, excessive cervical ognized contraindication to the sitting position due
flexion should be avoided by checking for two-finger- to elevated risk of PAE.8,9 Precipitous decline in PECO2
breadth spacing between the chin and neck or ster- and/or MAP (with elevated heart rate), along with
nal notch.7,8 Advantages of the sitting position include pcDUS or TEE positive findings, suggest clinically
natural anatomic orientation and enhanced surgi- relevant VAE.6,8,9 Additional monitoring techniques
cal exposure, improved drainage of CSF and blood, to optimize patient safety for the seated position
decreased intracranial pressure as a result of enhanced include somatosensory and motor evoked potentials
venous drainage, decreased airway pressures and (SSEPs, MEPs).
improved ventilation, and the ability to observe facial Methods to reduce the risk of VAE include fluid
reflections of cranial nerve stimulation.6–9 Significant administration, antigravity or antishock suits in
disadvantages include risks of venous air embolism older patients, elastic banding of the lower extremi-
(VAE), paradoxical air embolus (PAE) with cardiac ties in younger children, and lower-extremity eleva-
or end organ ischemia, hemodynamic instability or tion.6–9 These measures help maintain adequate
hypotension, pneumocephalus or tension pneumo- total and cerebral venous pressure while preventing
cephalus, postoperative infratentorial hemorrhage, venous pooling in the lower extremities. Mechanical
upper airway and tongue edema, spinal cord injury, ventilation with normocapnia and normal positive
and compression or traction peripheral neuropathies end-expiratory pressure (PEEP; 5 to 10 cm H2O) and
(including brachial plexus injuries).6–9 Furthermore, central venous line insertion represent other tech-
awkward or strained surgeon hand orientation con- niques to help prevent or prepare for VAE.6–9 Addition-
tributes to the undesirable nature of this position. ally, craniotomy edges should be waxed or covered
with hemostatic agents to occlude diploic vein sites.7–9
In the event of confirmed VAE, methods to prevent
adverse sequelae include covering exposed sinuses
with saline-soaked cotton material or gelatin foam
while flooding the surgical field with saline.7–9 The
operating table may be tilted to lower the patient’s
head.7,8 Jugular venous compression may help identify
sites of dural venous sinus entry that can be repaired
with suturing, muscle or fascia plugging, or occlusion
with hemostatic clips.7–9 In the event of hemodynamic
instability or collapse, the operating table may be
tilted to bring the right side of the heart up while the
anesthetic team may attempt to aspirate the VAE from
the right atrium via the central venous catheter.7,8
Constant communication between the neurosurgical
and anesthetic teams helps reduce the risks and pre-
vent adverse sequelae of VAE.

5.3╇ Outcomes and Postoperative


Course
Successful positioning for pediatric neurosurgery
involves establishing an orientation comfortable
for both the patient and the surgeon that allows
successful completion of the operation without
Fig. 5.8â•… Sitting position. The sitting position aids surgical position-related complications. Although infre-
exposure of posterior fossa, occipital, and pineal region lesions quent, adverse events related to positioning
but entails multiple significant risks that limit its usage in most require careful review and scrutiny to prevent
American pediatric neurosurgical centers.
5â•… Pediatric Neurosurgical Positioning 57
their recurrence. Contact between hard or rigid References
surfaces and the skin, especially over bony sur-
faces or those with minimal subcutaneous soft ╇1. Berry C, Sandberg DI, Hoh DJ, Krieger MD, McComb
JG. Use of cranial fixation pins in pediatric neurosur-
tissue, may lead to pressure ulcers and skin irrita-
gery. Neurosurgery 2008;62(4):913–918, discussion
tion or breakdown.10 Many of these complications 918–919
can be prevented with adequate padding and vigi-
╇2. Agrawal D, Steinbok P. Simple technique of head fixa-
lant attention to compressed surfaces. Dependent tion for image-guided neurosurgery in infants. Childs
body surfaces supporting moderate weight may Nerv Syst 2006;22(11):1473–1474
exhibit myonecrosis, especially during prolonged ╇3. Vitali AM, Steinbok P. Depressed skull fracture and
surgery.10 Cranial pin fixation carries risks of scalp epidural hematoma from head fixation with pins for
laceration, depressed skull fracture, epidural craniotomy in children. Childs Nerv Syst 2008;24(8):
hematoma, dural laceration, subdural hematoma, 917–923, discussion 925
cortical lacerations or contusions, CSF leak, and ╇4. Wong WB, Haynes RJ. Osteology of the pediatric
perforation of shunt hardware.1,3 Depending on skull. Considerations of halo pin placement. Spine
their extent, depressed skull fractures or epidural 1994;19(13):1451–1454
hematomas may require emergent operative inter- ╇5. Sgouros S, Grainger MC, McCallin S. Adaptation of
vention. Despite their proposed safety, pediatric skull clamp for use in image-guided surgery of chil-
pins carry risks of local skin pressure or necrosis, dren in the first 2 years of life. Childs Nerv Syst
especially during prolonged operations.3 2005;21(2):148–149
Patient orientation and body arrangement con- ╇6. Orliaguet GA, Hanafi M, Meyer PG, et al. Is the sit-
tribute substantially to position-related compli- ting or the prone position best for surgery for pos-
cations. Excessive head rotation combined with terior fossa tumours in children? Paediatr Anaesth
flexion or extension may lead to postoperative neck 2001;11(5):541–547
discomfort or infrequent cases of upper cervical ╇7. Lindroos AC, Niiya T, Randell T, Romani R, Hernesniemi
destabilization.11 If identified early, upper cervical J, Niemi T. Sitting position for removal of pineal region
rotary subluxation can be treated conservatively lesions: the Helsinki experience. World Neurosurg
2010;74(4-5):505–513
with reduction and external immobilization.11 Sig-
nificant neck flexion may also cause intraoperative ╇8. Jadik S, Wissing H, Friedrich K, Beck J, Seifert V, Raabe A.
A standardized protocol for the prevention of clinically
intracranial hypertension due to venous outflow
relevant venous air embolism during neurosurgical in-
obstruction. Prone positioning with the head lying terventions in the semisitting position. Neurosurgery
on a horseshoe headrest requires careful attention 2009;64(3):533–538, discussion 538–539
to orbital pressure to avoid ocular complications ╇9. Dilmen OK, Akcil EF, Tureci E, et al. Neurosurgery in the
(ischemic optic neuropathy).1,10 Traction or com- sitting position: retrospective analysis of 692 adult and
pression of the extremities during prolonged cases pediatric cases. Turk Neurosurg 2011;21(4):634–640
may cause temporary or permanent postoperative 10. Harrop JS. Patient positioning: is it really a big deal?
peripheral nerve deficits (brachial plexus injuries, World Neurosurg 2012;78(5):440–441
ulnar nerve compressive neuropathy, radial nerve 11. Heary RF, Reid P, Carmel PW. Atlantoaxial rotatory
palsy).10 Additional position-related complications fixation after ventriculoperitoneal shunting. Neurope-
include facial or bodily burns from pooling of prepa- diatrics 2011;42(5):197–199
ratory solutions on the headrest or operating table.
6 Intraoperative Neurophysiological
Monitoring During Pediatric
Neurosurgical Procedures
Daniel M. Schwartz, Andrew Paul Warrington, Anthony K. Sestokas,
and Ann-Christine Duhaime

6.1╇ Introduction and Background surgical perspective. These observations have arisen
from a great deal of pediatric intraoperative moni-
The goal of pediatric neurosurgery is to cure or melio- toring experience and study, but it should be kept
rate disease of the nervous system using surgical meth- in mind that different practitioners may approach
ods that maximize benefit to the child while minimizing these goals in different ways, and that the field con-
risk. Intraoperative neurophysiological monitoring is tinues to grow and to benefit from careful study and
one of the most important modalities used to achieve technologic advancement.
these goals. The history of what can be done, how it
can be done, and its utility for the surgeon has evolved
along with other technical and conceptual advances in
the procedural specialties. Intraoperative monitoring is 6.2╇ Overview and Goals of
a relatively young field, and neurosurgeons in general, Neuromonitoring
and pediatric neurosurgeons in particular, may have
varying experiences with different sorts of monitoring Multimodality (evoked potentials, electromyography,
modalities and practitioners. electroencephalography, cerebral blood flow) intraop-
One of the earliest uses of intraoperative moni- erative neurophysiology (ION) has become common-
toring, which had as its aim minimizing potentially place in pediatric neurosurgery. The underlying premise
preventable damage to neural structures, was cra- of ION in pediatric neurosurgery is multifaceted:
nial nerve monitoring during cerebellopontine angle
and otologic surgery. The professionals involved in • To assess continuously the functional integrity
this aspect of the emerging field often came from of central and peripheral nervous system
backgrounds in audiology. Another early use of anatomical structures, pathways, and vascular
monitoring in the general sense was intraoperative supplies at risk for iatrogenic injury
corticography to assess epileptogenic tissue during • To identify, verify presence of, and determine
seizure surgery, most often performed by neurolo- proximity of specific neural structures and
gists or neurophysiologists specializing in epilepsy. landmarks that either are obscure from, or
As somatosensory and later motor evoked poten- outside of, the surgeon’s immediate visual field or
tials came into use in scoliosis and spine tumor sur- have abnormal or distorted anatomical features
gery, the field of intraoperative monitoring began to • To help guide placement of indwelling
expand, becoming an independent specialty, arising therapeutic neurostimulators or recording
from these various lineages. As its use in children may electrodes used to treat neurologic disease
encompass differences in maturational physiology, (e.g., epilepsy, dystonia)
disease processes, and anesthetic considerations, • To provide an intraoperative diagnostic tool
additional experience has been gained and further for lesion identification prior to surgical
subspecialization has occurred. At present, the prac- intervention (e.g., neurolysis)
tice models, training, credentialing processes, and
oversight of intraoperative monitoring vary among These different goals can be met by techniques
organizations, specialties, and institutions. This that monitor function as well as those that locate,
chapter provides an overview of the modalities most or map, functional areas or structures. This chap-
commonly utilized in children, anesthetic impli- ter focuses on the application of multimodality ION
cations, typical pediatric neurosurgical scenarios across the broad spectrum of pediatric neurosurgical
encountered, role and limitations of monitoring, and procedures.
58
6â•… Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures 59
myelination, result in paradoxical SSEP latencies at
6.3╇Intraoperative subcortical and cortical levels. Subcortical responses
Neurophysiological Monitoring recorded from an electrode positioned over the
Modalities in Children proximal cervical spine, for example, have shorter
latencies, whereas responses from electrodes placed
6.3.1╇ Somatosensory Evoked over the somatosensory cortex are prolonged rela-
tive to those of a normal adult. By 6 to 8 years of age,
Potentials (SSEPs) neuromaturation of the somatosensory pathway has
Since the seminal work of Nash and coworkers1 progressed such that central conduction velocity is
describing the continuous recording of lower- comparable to that of a normal adult.
extremity somatosensory evoked potentials to assess As a result of neural immaturity, cortical SSEPs to
spinal cord function during surgical correction of lower-extremity (posterior tibial nerve) stimulation
spinal deformity in children, SSEPs have become a are often markedly labile and of inadequate ampli-
mainstay neuromonitoring modality used to detect tude to permit reliable and valid neuromonitoring in
disruption of transmission along a peripheral sen- infants under the age of 6 months. Conversely, cortical
sory nerve, nerve plexus, and the entire neuraxis SSEPs to upper-extremity (median and ulnar nerve)
from the spinal cord to the sensory cortex. stimulation are usually present and more reliable.
Briefly, SSEPs are elicited by stimulating a periph- Decreasing the stimulus repetition rate can
eral nerve, typically the posterior tibial or pero- help overcome the compromising effect of central
neal nerve in the lower extremities or the ulnar myelin immaturity when ulnar nerve SSEPs need to
or median nerve in the upper extremities. Lower- be recorded in infants less than 6 months of age, as
extremity SSEPs permit assessment of dorsal col- depicted in Fig. 6.1a–c. Observe that, had a standard
umn–medial lemniscal system (DC-MLS) function stimulus repetition rate of 4.1 to 5.1 Hz been used to
along the entire length of the neuraxis (i.e., spinal monitor cortical SSEPs in this patient, the neuromoni-
cord, brainstem, and cerebral hemispheres), whereas toring clinician would have been led to conclude that
upper-extremity SSEPs provide functional DC-MLS responses simply were too labile and attenuated to
assessment from the cervical segment of the spinal permit reliable and valid detection of emerging injury.
cord and cephalad. The application of recording electrodes for SSEP
The ascending sensory volley triggered by poste- monitoring in children is the same as that for adults;
rior tibial or peroneal nerve stimulation enters the however, the neuromonitoring clinician must be cog-
spinal cord through dorsal sacral and lumbar nerve nizant of the cranial suture lines and fontanelles that
roots at several segmental levels (S1–L4) and may may be open. Hence, careful attention should be paid to
ascend the spinal cord via multiple pathways. The directing subdermal needle electrodes away from these
general consensus is that the dorsal or posterior col- areas so as not to cause a transdural puncture wound.
umn spinal pathways are the primary sites of media-
tion for the SSEP.2–4 Other pathways, such as the dorsal
spinocerebellar and anterolateral tracts, may also con-
6.3.2╇ Transcranial Electrical Motor
tribute to the early somatosensory evoked responses Evoked Potentials (tceMEPs)
that are used for monitoring spinal cord function.5–7
Upon ascending the spinal cord, the neural sig- TceMEPs are neuroelectrical events recorded from
nal enters the medullary nuclei in the brainstem. descending motor pathways, including the cortico-
Because there are no afferent pathway synapses prior spinal tract (CST), spinal cord interneurons, anterior
to this subcortical entry point, SSEPs recorded up to horn cells, spinal nerve roots, and skeletal muscles
the level of the lower brainstem reflect the integrity after transcranial application of a high-voltage anodal
predominantly of peripheral nerve fibers and spi- electrical stimulus.
nal cord white matter. Beyond the medullary nuclei, CST axons project from cortex through the inter-
the neural pathway mediating the SSEP continues as nal capsule to the caudal medulla. Here, the fibers
the internal arcuate fiber system, which crosses the decussate and descend into the lateral and anterior
brainstem and ascends as the medial lemniscal path- funiculi of the spinal cord. In contrast with ascending
way, projecting to the thalamus; there is another spinal cord axons, which mediate SSEPs, descending
synaptic junction in the thalamus that in turn proj- CST axons that mediate tceMEPs enter the spinal cord
ects to the sensorimotor cortex, where additional gray matter, where they interact with spinal interneu-
synaptic interaction may occur. rons. There are both direct and indirect axonal projec-
Neural maturation plays a dominant role in deter- tions to alpha motor neurons, which in turn innervate
mining success of SSEP recordings in infants, particu- peripheral muscle.8 Lateral CST fibers that synapse in
larly those less than 3 months of age. Compared to the cervical segment of the spinal cord are arranged
adults, shorter limb length and smaller-diameter medially, followed laterally by fibers that synapse in
peripheral nerve fibers, as well as incomplete central the thoracic, lumbar, and sacral regions, respectively.
60 Section Iâ•… Introduction

Fig. 6.1â•… Subcortical and cortical somatosensory evoked potentials to interleaved stimulation of the left (columns 1 and 2, respec-
tively) and right (columns 3 and 4, respectively) ulnar nerves in a 5-month-old infant at three stimulation rates: (A) 4.1 Hz, (B) 3.1 Hz, (C)
2.1Hz. Subcortical responses were recorded between conventional electrode position Fpz and the upper cervical spine (Cs2). Cortical
responses were recorded between Fpz and Cp4 (left ulnar nerve stimulation) and between Fpz and Cp3 (right ulnar nerve stimulation).

TceMEPs mediated by the CST can be recorded


from the spinal epidural or subdural space via a
catheter-type electrode or from peripheral muscu-
lature using subdermal needle electrodes. Responses
recorded from the epidural space are led by a D-wave
triggered by depolarization of motor neurons that
project directly into the CST. In awake or lightly
anesthetized patients, the D-wave is followed by a
series of I-waves elicited indirectly via cortical syn-
apses.9 Descending cortical volleys then summate
temporally and spatially to excite spinal motor neu-
rons that project to skeletal muscles, triggering com-
pound muscle action potentials.
The intraoperative monitoring of D-waves has par-
ticular value during excision of intramedullary spinal
cord tumors10; however, it requires a recording elec-
trode to be placed directly over dura, either percutane-
ously or through a laminotomy, precluding routine use
of this technique in most corrective spine surgeries. It
is both easier and preferable to record myogenic motor
responses from upper- and lower-extremity periph-
eral muscle for most spine procedures. Unlike D-waves,
which provide information only about the spinal cord,
myogenic motor evoked potentials can also assess and
identify functionally significant changes in individual
spinal nerve roots or peripheral nerves.11–13
Anodal current stimulation is more effective at
activating the corticomotor neurons than cathodal
stimulation; the active electrode is positive.14 Opti- Fig. 6.2â•… Schematic showing typical sites for anodal transcra-
mal stimulation sites for motor cortex activation are nial electrical stimulation (M1-4, Mz) in relation to conven-
interpolated from standard scalp electrode positions tional electrode positions Cp1–Cp4, Cpz, Pz, Fpz (International
defined by the International 10/20 system, as illus- 10-20 System of Electrode Placement).
trated in Fig. 6.2.
6â•… Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures 61
In the pediatric population, prebent stainless steel minimal response emergence at select myotomes.
beveled needle electrodes are preferable to corkscrew- At the longest ISI (3.3 msec), however, large-ampli-
type electrodes, especially in infants, where unclosed tude myotomal tceMEPs were readily apparent at all
fontanelles and sutures can be at risk for invasive recording sites, suitable for consistent neuromonitor-
electrode placement. Routinely, anodal stimulation is ing of corticospinal tract integrity (Fig. 6.3d).
applied to the electrode site of M1 and M2 for activa- Triggering of tceMEPs can be challenging in some
tion of right and left contralateral limbs, respectively; pediatric patients, particularly those with congenital
unilateral activation is facilitated by placing the cath- abnormalities or pre-existing motor deficits. Facili-
ode at Cz. Lower-extremity tceMEPs are elicited more tation of tceMEPs using preconditioning techniques
prominently with anodal stimulation at Mz and the has been shown to enhance responses that would
cathode placed a few centimeters anterior. otherwise not be monitorable. Journée et al reported
Transcranial electrical stimulation for myotomal that delivery of two stimulus trains closely spaced
motor evoked potentials usually consists of constant- in time increased motor evoked potential ampli-
voltage rectangular pulse trains (N = 3 to 9 stimuli) tudes by a mean factor of over 15 times in a group
having durations (pulse width) of 50 to 75 µsec and of patients whose responses to a single train were
interstimulus intervals (ISIs) that range from 1 to smaller than 100 microvolts.15
5 msec. Use of longer pulse durations and ISIs can In general, facilitation is most beneficial in chil-
improve the acquisition of reliable tceMEPs in infants dren with pre-existing motor deficits who present
under 12 months, as shown in Fig. 6.3a–d. Note that with unreliable, labile, small-amplitude responses
with stimulation using 5 pulses at a pulse width (PW) at baseline and who otherwise would be deemed
of 50 µsec and ISI equal to 1 msec, no tceMEPs could unmonitorable. On occasion, however, facilitation
be recorded in a 3-month-old infant, thereby render- can help improve amplitude resolution and, hence,
ing the case unmonitorable. Increasing the number of interpretation of meaningful intraoperative tceMEP
pulses to 6, PW to 75 µsec, and ISI to 2 msec produced change in neurologically normal pediatric patients

Fig. 6.3â•… Effect of adjusting stimulus train


parameters on triggering of tceMEPs from left
upper and lower extremities in a 3-month-old
infant.Constant-voltage stimulation set at
300 V. Muscle abbreviations: AS, external anal
sphincter; FD, first dorsal interosseous; GA,
gastrocnemius; IO, iliopsoas; QD, quadriceps;
TA, tibialis anterior. (a)â•… N, 5 stimulus pulses
delivered using an interstimulus interval (ISI)
of 1 msec and pulse width (PW) of 50 µsec.
a
(Continued on page 62)
62 Section Iâ•… Introduction

Fig. 6.3 (Continued)â•… (b) N, 5 pulses; ISI, 1 msec;


PW, 75 µsec. (c) N, 6 pulses; ISI, 2 msec; PW, 75
c
µsec.
6â•… Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures 63

d
Fig. 6.3 (Continued)â•… (d) N, 6 pulses; ISI, 3.3 msec; PW, 75 µsec.

with small responses to single-train stimulation, as surgeons and neuromonitoring providers regard-
exhibited in Fig. 6.4. We have not seen any false-neg- ing potential untoward effects, particularly seizure
ative results even with extremely high-risk children onset, following repetitive transcranial electrical
using tceMEP facilitation (Fig. 6.4). stimulation (RTES) for tceMEP monitoring. Other
Despite the proven value of tceMEP mapping expressed safety concerns include the possibility of
and monitoring in pediatric spine and intracranial tongue bite injury, other movement-induced injury,
surgery, there continues to be concern among some arrhythmia, and adverse interactions with implants.

Fig. 6.4â•… TceMEP facilitation in a 13-year-old boy with a history of quadriplegic cerebral palsy. Upper row of traces shows motor
evoked potentials from right upper- and lower-extremity muscles recorded after presentation of a single stimulus train consisting
of five pulses spaced 1 msec apart. Note that while there are visible potentials from first dorsal interosseous and abductor hallucis
muscles, there are no monitorable responses from quadriceps and tibialis anterior muscles. Lower row of traces shows facilitated
motor evoked potentials to a second identical stimulus train delivered immediately after the first. Responses from quadriceps and
tibialis anterior muscles are now clearly visible after facilitation by the first stimulus train, and there is amplitude enhancement of
responses from first dorsal interosseous and abductor hallucis muscles.
64 Section Iâ•… Introduction
In a large series (N = 18,862) investigating the in approximately 95% of pediatric patients less than
safety of RTES for tceMEP monitoring in pediatric and 12 months old and in 100% more than 1 year old. By
adult patients ranging in age from <€1 year to > 70 comparison, lower-extremity tceMEPs are recordable
years, however, Schwartz et al reported no evidence in about 85% of children less than 12 months old, in
of seizure provocation, neuronal damage, or cardio- 95% of those between 12 and 24 months old, and in
vascular effects.16 The rare complication noted, across 100% of children more than 2 years old. To be sure,
all age categories, was self-limiting tongue bite, which tceMEP recording may be less successful in children
can be mitigated easily using a soft bite block inserted with severe pre-existing neurologic compromise,
by the anesthesiologist following intubation. such as those with spastic cerebral palsy; however,
A follow-up study by Shah and coworkers evalu- even in this population we have been able to record
ated the safety of RTES for tceMEP monitoring in 45 acceptable tceMEPs for valid neuromonitoring inter-
children with severe spastic cerebral palsy and an pretation at least 50% of the time.18
active history of seizure disorder.17 Here again, no
child showed signs of RTES-provoked seizure onset
during or immediately after tceMEP-monitored sur- 6.3.3╇ Stimulated Electromyography
gical correction of neuromuscular scoliosis.
Our experience in neuromonitoring of tceMEPs Stimulated (i.e., evoked, triggered) electromyography
in adult and pediatric spine surgery over more than (stEMG) is used to:
two decades has shown it to be extremely safe and • Confirm functional integrity of a neural
highly sensitive and specific for detection of emerging element (e.g., cranial nerve, spinal nerve root,
spinal cord injury.16–19 We have found that reluctance peripheral nerve)
to attempt tceMEP monitoring in infants and young
• Map the anatomical course and proximity of
children ≤ 1 year of age is often associated with an
specific neural elements in the presence of
incorrect assumption that there will be no monitor-
pathology or other cause of obscurity within
able signals or that it is unsafe. Neuromonitoring per-
the surgeon’s field of view
sonnel who are inexperienced in pediatrics also may
fall back on a standardized approach, failing to alter • Differentiate between neural and nonneural
stimulation and recording parameters (PW, ISI, volt- tissue
age, number of pulses in train, use of facilitation, etc.), Unlike SSEPs and tceMEPs, which are categorized
which can convert an otherwise unmonitorable case as neuromonitoring modalities, stEMG is commonly
into one with well-defined and stable tceMEP ampli- used as a neurophysiological mapping technique.
tudes. In our experience, when there is preoperative Moreover, in contrast to its neuromonitoring coun-
clinical evidence of intact motor function, upper- terparts, the quality, reliability, and validity of stEMG
extremity myotomal tceMEP monitoring is feasible results are not age dependent, as illustrated in Fig. 6.5.

Fig. 6.5â•… Compound action muscle potentials triggered during facial nerve mapping in a 6-day-old infant undergoing resection of
teratoma located in the neck and extending into the skull base. The left, middle, and right columns show triggered responses from
orbicularis oculi, orbicularis oris, and mentalis muscles, respectively. Single-pulse constant-current stimuli (50 µsec duration), rang-
ing in intensity from 1.2 mA (top row) to 0.49 mA (bottom row), were presented at a rate of 2.7 Hz.
6â•… Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures 65
EMG discharge activity. Moreover, perhaps because
the perineural sheath protects peripheral segments
of cranial nerves (e.g., VII, X, XII), spinal nerve roots,
or peripheral nerves of the extremities, spEMG is
Flush tip Monopolar electrode
spreads controlled stimulation not particularly sensitive for detecting mechanical
to a return electrode placed in
nearby tissue.
irritation, compression, or other insult during sur-
gical procedures wherein the nerve is not exposed
a directly. In such situations, tceMEPs may serve as a
more valuable adjunctive neuromonitoring modal-
ity for assessing signal transmission through a
nerve.13,20–22
Inner cathode and outer
anode electrodes minimize
stimulus spread

6.4╇ Optimal Anesthetic Techniques


for ION in the Pediatric Population
b The success of intraoperative neuromonitoring is
highly dependent on anesthetic management. Essen-
Fig. 6.6â•… Examples of (a) disposable monopolar and (b) con- tially, all anesthetic agents depress synaptic function
centric bipolar electrical stimulators commonly used for stEMG in both brain and spinal cord gray matter. As a result,
mapping and testing. neurophysiological signals that traverse or are gen-
erated at synaptic junctions are also depressed. Any
anesthetic that depresses signal amplitude exces-
Because of broad current spread, monopolar sively will produce increased signal variability, inter-
electrical stimulation (Fig. 6.6a) is used to survey pretive ambiguity, and clinical uncertainty.23
proximity of a specific neural element when the The underlying goal for anesthesia, therefore, is to
neurosurgeon’s ability to identify the structure visu- obtain and maintain an anesthetic state that not only
ally is incomplete or unreliable, such as when map- is appropriate for achieving hypnosis, amnesia, aki-
ping the surface of a large acoustic (cerebellopontine nesis, and analgesia, but also allows for continuous,
angle) tumor to search for presence of facial nerve or reliable evaluation of neurophysiological signals for
splayed nerve fascicles. Conversely, concentric bipo- optimal neuromonitoring. Signal variability result-
lar stimulation (Fig. 6.6b) introduces direct focal ing from alterations in anesthetic depth may result
stimulation with minimal current spread; therefore, in interpretive error, as well as unnecessary perfor-
it is better suited to achieve the following goals: mance of a wake-up test, increased surgeon anxiety,
and, ultimately, mistrust of ION data and personnel.
• To differentiate between neural and nonneural
In general, all inhalational agents (isoflurane, des-
tissue
flurane, sevoflurane) produce a dose-related increase
• To differentiate between cranial nerves (e.g., V in latency and reduction in amplitude of the cortical
vs. VII, IX vs. X, etc.) or spinal nerve roots (e.g., SSEP.24 Although the exact sites of action for these
L4 vs. L5 vs. S1, etc.) potent agents remain unclear, the gases appear to dis-
• To assess neural functional integrity solve in the neuronal plasma membrane. The result-
ing effect is inhibition of ion channel function with
significant alteration in synaptic and axonal transmis-
6.3.4╇ Spontaneous Electromyography sion. Neurophysiological signals that rely on synap-
(spEMG) tic function are influenced to a far greater extent by
inhalational agents than are those not dependent on
Intraoperative spontaneous (free-running) electro- synapses. Even at steady-state low end-tidal concen-
myography involves the recording of electrical activ- trations (0.25 to 0.5 minimum alveolar concentra-
ity produced by skeletal muscle to identify acute tion [MAC]) of inhalational agent, neurophysiological
irritation of cranial, spinal, or peripheral nerves sec- response amplitude can become highly unstable and
ondary to mechanical contact, direct tractional pull, variable, causing signals to be either too obscure to
heat dispersion from electrocautery, or other noxious detect significant change or completely obliterated.
stimuli that can excite the nerve to depolarize. While The effect of potent anesthetics is much less pro-
in many cases early awareness of nerve irritation can nounced on the subcortical SSEP recorded over cer-
prevent overmanipulation and subsequent injury, vical spine than on its cortical counterpart and is
it is possible to injure a nerve by sharp transection, minimal on spinal epidural or peripheral responses.
ischemia, or gradual stretch without intraoperative In the past, when the only available neuromonitor-
66 Section Iâ•… Introduction
ing modality was the SSEP, it was possible to avoid the uncertainty introduced to decision making when
the adverse effects of inhalational anesthesia simply using partial NMB can be significant for intraopera-
by recording a subcortical potential in the presence tive neurophysiological monitoring.33
of muscle relaxation.
Given the ascendant role of tceMEPs for intraop-
erative monitoring of corticospinal tract function,
the depressive effects of volatile anesthetics on the 6.5╇ ION Application During
excitability of motor pathways that mediate tceMEPs Pediatric Neurosurgical Procedures
is of particular significance. As was the case histori-
cally with SSEP monitoring, there continues to be 6.5.1╇Intracranial
considerable controversy about the degree to which
volatile agents depress myogenic tceMEP amplitudes. Supratentorial Procedures
In contrast to ongoing controversies about the
effects of volatile anesthetic gases on SSEPs and myo- The primary role for ION in supratentorial procedures
genic tceMEPs, there seems to be general consensus is evaluation of primary motor and somatosensory
that the effect of nitrous oxide on cortical SSEP and cortices and associated subcortical pathways. These
tceMEP amplitudes is sufficiently great to recommend regions are at risk of injury when they are within
avoiding its use entirely in monitored cases.24–29 This is the immediate vicinity of pathology like tumors and
particularly important if it is used in combination with vascular abnormalities. Multimodality ION provides
a volatile agent, because the introduction of nitrous several tools for ongoing assessment of these corti-
oxide lowers the MAC, thereby having an additive cal and subcortical regions. The monitoring clinician
effect on suppression of evoked potential amplitudes. must be able to evaluate the neural risk factors spe-
In light of the difficulties encountered with vola- cific to the surgical procedure and implement appro-
tile agents and nitrous oxide, we have advocated, priate test modalities so as to provide the surgeon
since 1992, use of a total intravenous anesthetic with accurate and timely feedback about the integ-
(TIVA) regimen in both pediatric and adult surgical rity of monitored structures.
procedures to facilitate uncompromised ION signal Motor and somatosensory evoked potentials
amplitudes. If TIVA is precluded or denied for any provide sensitive and specific coverage of pre- and
reason, a team approach involving the surgeon, anes- postcentral cortical gyri, respectively. If the proce-
thesiologist, and neuromonitoring professional is dure poses risk of vascular compromise, the addition
needed to discuss the risks, benefits, and interpretive of multichannel electroencephalography (EEG) can
ambiguity that may result from using inhalational extend monitoring coverage beyond these regions.
agents or nitrous oxide. The EEG can also help guide the surgeon and anes-
Prior to the advent of multimodality monitoring thesiologist during titrated infusion of neural protec-
of spinal motor tract and nerve root function, it was tive agents, such as etomidate, that may be required
commonplace to keep the patient relaxed pharma- to reduce metabolic demand of the brain during
cologically during surgery. Neuromuscular blocking periods of reduced blood flow.
(NMB) agents have no adverse effect on SSEPs and The supratentorial approach to tumor resection
may in fact improve the quality of recordings by often involves an incision site and scalp reflection
eliminating myogenic interference. In contrast, NMB that displace scalp electrodes from their optimal
agents compromise all responses that are recorded locations, more notably in infants. In these situa-
from muscle (tceMEP, spEMG, stEMG, etc.), which tions, it is helpful to obtain baseline tceMEP and SSEP
depend on reliable transmission of neural signals recordings using an undisturbed scalp electrode
across the neuromuscular junction. setup prior to prepping of the surgical site. This
Although there has been some suggestion that allows for documentation of any pre-existing signal
myogenic tceMEP and EMG monitoring can be con- abnormalities that might otherwise be attributed
ducted in the presence of partial NMB, this practice to suboptimal placement of cephalic electrodes.
should be avoided if at all possible.30–32 The effects of Triggering of tceMEPs can be compromised if the
NMB can vary between the upper and lower extrem- stimulating electrodes are moved away from the
ities, between the left and right sides of the body, primary motor cortex. While increasing stimulation
and between muscles within single limbs, thereby intensity to compensate for electrode displacement
making it difficult, if not impossible, to maintain a may ultimately trigger a motor evoked potential,
homogeneous state of partial equilibrated NMB. that strategy can be counterproductive. Increasing
When combined with the difficulty of maintaining stimulation voltage may shunt current below the
a constant state of partial NMB across time, as well region of desired resection and thus increase the
as the interactive effects that volatile and intrave- likelihood of a false-negative outcome, as illustrated
nous anesthetics have on the pharmacodynamics in Fig. 6.7. The increased stimulation intensity can
and pharmacokinetics of nondepolarizing relaxants, also increase patient movement, putting the patient
6â•… Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures 67

Fig. 6.7â•… Coronal section of the brain showing location of


tumor (highlighted) and hypothetical paths of current flow dur-
ing transcranial electrical stimulation at near-threshold (solid
line) and suprathreshold (dashed line) intensities. The former
path traverses the region of tumor, allowing for monitoring of
involved corticospinal tract fibers. In contrast, the latter path
undercuts the lower margins of the lesion and may activate
distal motor tract fibers even in the presence of proximal dam-
age, leading to a false-negative finding. Fig. 6.8â•… Arrow points to sterile 4-contact electrode strip
placed over cortex to facilitate direct stimulation of motor cor-
tex. Diameter of each contact exposure is 5 mm.
at risk for injury from unintended migration of a sur-
gical instrument.
A preferable strategy is to activate corticomotor stimulation aids in mapping the motor homunculus
neurons using a sterile electrode placed directly over (Fig. 6.10a–c). Use of a flexible monopolar stimulat-
motor cortex, as illustrated in Fig. 6.8. This technique ing probe (see Fig. 6.10) facilitates motor mapping
lowers the stimulation intensity needed for primary from within tumor capsule or adjacent to other
motor cortex activation, which in turn reduces the lesions, such as cortical dysplasia, and aids in local-
risk of current shunting below the resection site. It ization of internal capsule fibers when the lesion
also minimizes or eliminates the effect of patient extends subcortically.
movement, thereby limiting interference with surgi- Unlike mapping of motor cortex, which requires
cal resection, and allows near-continuous monitor- anodal monopolar stimulation, cathodal monopo-
ing and mapping during dissection (Fig. 6.8). lar stimulation should be used to map the internal
Functional mapping of eloquent cortex also can capsule. It is recommended that, when the lesion
facilitate safer resection of cortical and subcorti- is in close proximity to primary motor areas, map-
cal lesions in the frontoparietal regions. SSEP phase ping/testing should be performed repeatedly dur-
reversal testing can be performed to identify the cen- ing resection to confirm that stimulation thresholds
tral sulcus (Fig. 6.9), while low-intensity focal anodal remain unchanged (Fig. 6.9 and Fig. 6.10).

Fig. 6.9â•… Intraoperative neurophysiological mapping of the central sulcus in a 17-year-old female patient prior to resection of epi-
leptogenic focus in right frontal cortex. Recording electrode was similar to that shown in Fig. 6.8. Ulnar nerve SSEP phase reversal
localized the central sulcus between electrodes 3 (EL3) and 4 (EL4).
68 Section Iâ•… Introduction

Fig. 6.10â•… Motor cortex mapping in a 17-year-old male patient prior to resection of epileptogenic zones in the left frontotemporal
region. Motor cortex was mapped under total intravenous anesthesia (propofol/opioid) without muscle relaxation by triggering
motor evoked potentials with anodal pulse trains (3 pulses, 1.1-msec interpulse interval, 50-µsec pulse duration). A cathode was
placed in the contralateral scalp. Muscle abbreviations: DLT, deltoid; ECR, extensor carpi radialis; FDI, first dorsal interosseous; TA,
tibialis anterior; TNG, tongue. (a) Upper panel shows stimulation probe over site A, which triggered low-threshold responses from
the right wrist extensor and hand intrinsic muscles, as illustrated by adjacent traces. (b) Middle panel shows stimulation probe over
site B, which triggered low-threshold responses from the tongue, as illustrated. (c) Lower panel shows sites A and B prior to (left) and
following (right) resection of epileptogenic zones. This patient did not show any motor deficits postoperatively.
6â•… Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures 69

Awake Speech Mapping: An Intraoperative cisely timed electrical stimulation for the surgeon
Neurophysiological Approach to survey cortical tissue while the patient performs
language-oriented tasks. Disruption of the patient’s
The application of ION is integral during procedures behavior during performance of these tasks implicates
requiring resection of epileptogenic foci or lesions the stimulated tissue in language function. A comple-
that are proximate to areas involved in language mentary interpretive role for the pediatric surgical
function. These procedures often require the patient neurophysiologist or pediatric neurologist is to iden-
to remain awake, when this is feasible, so that neu- tify EEG after-discharges (ADs) associated with cortical
rologic status can be observed directly during func- stimulation, as noted in Fig. 6.11a,b. These ADs are key
tional neurophysiological testing. to identifying the upper limit of applied stimulation.
The primary role for the pediatric surgical neuro- The most common approach for mapping language
physiologist in these cases is to provide scaled, pre- function is to implement the Penfield stimulation

Fig. 6.11â•… Electrocorticography during and immediately following bipolar train stimulation to establish cortical after-discharge
threshold. (a) 3-mA biphasic pulse stimulation at 50 Hz. No after-discharges noted on any of three EEG channels following stimu-
lation. (b) 4-mA biphasic pulse stimulation at 50 Hz. After-discharges are noted on the second and third EEG channels following
stimulation.
70 Section Iâ•… Introduction
technique.34,35 Our adaptation of the technique involves the child is maintained throughout the resection
delivery of biphasic pulses at 50 Hz for durations of 3 phase in order to monitor language function.36
to 5 seconds. The period of each biphasic pulse is 1
msec. Initially, we stimulate the facial representation
of primary motor cortex, increasing intensity until we Infratentorial Procedures
observe or the patient reports facial twitches. These
stimulation thresholds can range from 1 to 3 mA and Historically, the applied intraoperative neurophysi-
are considered the “floor” threshold. We then stimu- ological monitoring for infratentorial or brainstem
late cortex outside the primary motor representation, operations focused predominantly on identification
increasing current intensity gradually until after-dis- and functional preservation of cranial nerves VII
charge is noted, as represented in Fig. 6.11b. This lat- and VIII. As an outgrowth, multimodality neuro-
ter intensity is deemed the “ceiling” threshold, which monitoring has expanded infratentorial surveillance
defines the upper limit of applied stimulation during to include all motor cranial nerves and select cra-
functional testing. nial nerve nuclei, as well as extended regions of the
Once effective and safe threshold boundary values brainstem that may be at surgical risk. For example,
have been defined, subsequent stimulation of target transcranial electrical motor evoked potentials, such
cortical tissue takes place while the child performs as those displayed in Fig. 6.12a, can be used to assess
various tasks that probe language function. These descending corticospinal motor fibers in the area of
tasks range from simple counting to more complex the pons and medulla, while somatosensory evoked
sentence and image or word recognition. When stim- potentials (Fig. 6.12b,c) survey long sensory tracts
ulation of a cortical region provokes speech arrest ascending through the regions of the medulla, pons,
or hesitation, the eloquent tissue is usually spared. and midbrain to the thalamus. Brainstem auditory
Because absence of hesitation does not rule out pres- evoked potentials (BAEPs), shown in Fig. 6.12e, are
ence of eloquent cortical tissue, communication with used not only for functional assessment of cranial

Fig. 6.12â•… Example of multimodality monitoring during resection of a posterior fossa ependymoma in a 5-year-old boy. (A) Tran-
scranial electrical motor evoked potentials recorded from bilateral first dorsal interosseous (FD) and tibialis anterior (TA) muscles.
(B) Cervical/brainstem and cortical somatosensory evoked potentials to sequential stimulation of the left and right ulnar nerves.
(C) Cortical somatosensory evoked potentials to sequential stimulation of the left and right posterior tibial nerves. (D) Compound
muscle action potentials recorded from bilateral FD muscles to train-of-four stimulation of the left and right ulnar nerves (neuro-
muscular junction testing). (E) Brainstem auditory evoked potentials to sequential click stimulation of the left and right ears. (F)
Single-channel electroencephalography (to facilitate monitoring of anesthetic depth).
6â•… Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures 71

Fig. 6.13â•… Cranial nerve mapping using stimulated electromyography during resection of a posterior fossa ependymoma in a
5-year-old boy. Cranial nerves IX and XI are identified using concentric bipolar stimulation at < 1 mA. LMA, left masseter muscle,
LMN, left mentalis muscle; LOC, left orbicularis oculi muscle; LOR, left orbicularis oris muscle; LTN, left tongue; LTR, left trapezius
muscle; PA, soft palate.

nerve VIII but also for the ipsilateral and contralat- 6.5.2╇ Spine Surgery
eral brainstem pathway between the auditory nuclei
and rostral lateral lemnisci (Fig. 6.12). Spinal Dysraphism
In conjunction with multimodality evoked poten-
tials, both free-running (spontaneous) and stimu- Spinal dysraphism consists of congenital spinal or
lated electromyography (EMG) can be performed to spinal cord defects secondary to failed closure of
monitor motor function of cranial nerves III–VII and the neural tube or fusion of bony elements during
IX–XII, as well as to map the floor of the fourth ven- early development. There are several pathologies
tricle via identification of select cranial nerve nuclei. associated with spinal dysraphism, including but
Precise localization of these cranial nerves and their not limited to various forms of spina bifida, Chiari
respective nuclei is facilitated by use of a sterile malformation, tethered spinal cord, syringomyelia,
hand-held concentric bipolar electrical stimulator lumbosacral lipoma, diastematomyelia, and dermal
that limits current spread (see Fig. 6.6). Fig.€6.13 sinus tracts. Each of these pathologies may require
presents an illustrative example of cranial nerve neurosurgical intervention with application of ION.38
mapping identifying the glossopharyngeal and spi- Neurosurgical intervention for release of the vari-
nal accessory nerves. ous forms of tethered spinal cord usually involves
precise lumbar or lumbosacral laminectomy near
the site of intended release. The general principle
Chiari Malformation is to begin dissection in normal anatomy and move
systematically toward the area of abnormality. In
Chiari malformations consist of caudal displacement cases of simple filum section, the dura is opened
of the cerebellar tonsils, and sometimes medulla, over the desired location of section followed by care-
through the foramen magnum. Types I and II are the ful dissection of lumbosacral spinal nerve roots and
most common and, when indicated, are usually treated subsequent identification and sectioning of the iso-
with surgical intervention that includes a suboccipital lated filum terminale. In cases where there are other
decompressive craniectomy and possible duraplasty. congenital anomalies, such as lipoma or meningo-
Multimodality ION, including cortical SSEP, tceMEP, cele, the risk of iatrogenic neurologic injury can be
BAEP, and EMG, can be used to assess the integrity of increased. Here again, in general, the dura is opened
the spinal cord, brainstem, and lower cranial nerves as over an area of normal anatomy. As dissection con-
well as for identifying preexisting neurophysiological tinues toward the area of abnormality, ION can aid
deficits and for detecting presence of evolving injury in distinguishing neural from nonneural tissue and
during positioning, bony decompression, and intra- provides constant feedback about the functional
dural exposure. This may be particularly useful in integrity of neural structures at risk.
reoperations or cases thought to be accompanied by The primary role of ION during untethering pro-
significant adhesions, anterior compression, instabil- cedures is to facilitate identification and sparing of
ity, or other high-risk factors.37 spinal nerve roots or fascicles that may be adher-
72 Section Iâ•… Introduction
ent to the filum, sinus tract, fatty mass, or scar tis- evoked EMG responses are usually elicited by depo-
sue. Once the dura is opened, it is important for the larization of adherent low sacral nerve fascicles that
neurosurgeon to establish a positive neural control can be freed prior to sectioning of the filum to reduce
by stimulating a clearly visualized nerve root prior to the risk of postoperative bladder or bowel dysfunc-
stimulation of the filum, as exemplified in Fig. 6.14. tion. Restimulation of the filum circumferentially fol-
Here again, a bipolar (preferably concentric) lowing fascicular dissection should result in absence
stimulator is used to probe individual lumbar and of any recordable stimulated EMG responses even at
sacral roots directly, which typically have depolar- high intensities (Fig. 6.14b).
ization thresholds ≤ 3 mA (pulse duration 100 µsec). Minimizing or eliminating current spread is essen-
Higher thresholds may be indicative of current tial to reliable identification of isolated spinal nerve
spread through cerebrospinal fluid, which should roots and nerve fascicles. Accordingly, use of monopo-
be suctioned from the site. If subsequent circumfer- lar stimulation at durations used typically for detecting
ential stimulation of the filum at intensities up to 7 pedicle screw violation of the medial wall is inappro-
mA triggers a compound muscle action potential, priate for intradural nerve root mapping. Again, we
the filum should be examined carefully for presence recommend concentric bipolar stimulation with pulse
of nerve fascicles. Detailed attention should be paid durations of 50 to 100 µsec (usually 100 µsec) deter-
to the presence of small compound muscle action mined by the presence and magnitude of response
potentials with thresholds < 3 mA. These stimulus- spread across multiple myotomes. While there often is

Fig. 6.14â•… Stimulus-triggered EMG testing prior to sectioning of filum terminale during a spinal cord untethering procedure in a
4-month-old boy. Muscle abbreviations: AS, external anal sphincter; GA, gastrocnemius; IO, iliopsoas; QD, quadriceps; TA, tibialis
anterior. (a) Concentric bipolar stimulation at intensities between 0.2 and 1.8 mA (100-µsec pulse duration, 2.1-Hz stimulation rate)
used to identify lower sacral nerve root fascicle.
6â•… Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures 73

Fig. 6.14 (Continued)â•… (b) Stimulation of filum at intensities up to 9.5 mA prior to sectioning reveals no compound muscle action
potentials, consistent with absence of adherent neural elements.

some spread to an adjacent myotome, such as simul- from multiple myotomes. Some spEMG discharges
taneous EMG responses from both the anterior tibialis coincide with tceMEP decrements, while others do
and gastrocnemius muscles on stimulation of the L5 not; therefore, it is important for the pediatric sur-
nerve root, in most instances the depolarizing nerve gical neurophysiologist to pay close attention to the
can be identified readily based on the response ampli- spEMG throughout the procedure and to provide
tude, defined morphology, and temporal resolution. immediate feedback to the surgeon during episodes
An additional practice that should be avoided is of vigorous neurotonic discharge activity.
“muscle linking”; that is, combining two muscles into
one recording channel (e.g., quadriceps and anterior
tibialis). This oft-noted practice among less experi- Diastematomyelia
enced ION personnel defeats the ability to determine
the innervating nerve root and can lead to erroneous Diastematomyelia is a congenital disorder in which
interpretation communicated to the surgeon (Fig. 6.14). the presence of an osseous or fibrous septum in the
In addition to surveillance of the filum terminale, central portion of the spinal canal causes “splitting”
ION contributes to overall patient safety by monitor- of the spinal cord into two hemicords. This usually
ing nerve root function. Spontaneous EMG, lower- occurs between the low thoracic region and the
extremity tceMEPs, and posterior tibial nerve SSEPs sacrum, with the most common locale being in the
are monitored both during the laminectomy/lami- upper lumbar region. Due to the nature of the surgi-
notomy phase as well as during opening of dura and cal exposure and the need for removal of the abnor-
exploration of exposed lumbosacral roots, to identify mal bony septum and/or dural sheath, the spinal
acute nerve root stretch or compression. TceMEPs cord and its component hemicords may be at risk
should be supported with both spEMG and stEMG for injury from retraction, as illustrated in Fig. 6.15.
74 Section Iâ•… Introduction

Fig. 6.15â•… TceMEP monitoring results from a


14-year-old with diastematomyelia during pro-
cedure to remove osseous septum. Posterior
tibial nerve somatosensory evoked potentials
(not shown) were absent at baseline. Muscle
abbreviations: AD, adductor; AS, external anal
sphincter; FD, first dorsal interosseous (upper
extremity control); GA, gastrocnemius; QD,
quadriceps; TA, tibialis anterior. (a) Baseline
left (upper panel) and right (lower panel) tce-
MEPs from multiple myotomes. (b) Loss of
right-side lower-extremity tceMEPs with spar-
ing of the upper-extremity control and external
anal sphincter responses following retraction
of right hemicord, prompting surgical alert,
hemodynamic intervention, and subsequent
b
closure.
6â•… Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures 75

Fig. 6.15 (Continued)â•… (c) No return of right-side lower-extremity tceMEPs following 40 minutes of pharmacologically induced
hypertension. Postoperatively, this patient was noted to have new-onset right lower-extremity paresis, which showed partial recov-
ery after 10 days.

Monitoring the cord and the adjacent roots on each facilitate safe maximal resection of intramedullary
side can help the surgeon proceed with safe dissec- tumor tissue. Whereas sustained intraoperative loss
tion and removal of the midline tether, as well as sec- of tceMEPs is consistent with compromise of cortico-
tioning the distal fila. spinal tract conduction that may manifest as paresis
or paralysis in the immediate postoperative period,
simultaneous preservation of D-wave amplitudes at
Intradural and Extradural Spine Tumors or above 50% of baseline is a positive long-term prog-
nostic indicator (Fig.€6.16a–c). These patients gen-
Glial tumors are the most common intramedullary erally recover preoperative motor strength over the
spinal cord tumors. Children who present with these course of a few hours to a few days.40 This feedback
lesions usually have pre-exisiting neurological com- can be extremely helpful to the surgeon during dis-
promise, exhibiting symptoms of myelopathy and/ section, allowing for necessary tissue manipulation
or radiculopathy.39 Consequently, they are at height- to effect appropriate tumor removal within a likely
ened risk for additional neurologic compromise recoverable range.
during tumor resection and can benefit from mul- SSEPs can serve two distinct neurosurveillance
timodality intraoperative monitoring of spinal cord purposes during intramedullary spinal cord tumor
and spinal nerve root function with D-waves, myo- resection. The primary role is to assess the func-
genic tceMEPs, and SSEPs. tional integrity of the dorsal column tracts. Alterna-
The D-wave is a direct corticospinal tract (CT) vol- tively, SSEPs can also be used to map the location of
ley generated following single-pulse electrical stimu- the dorsal median sulcus prior to myelotomy. One
lation applied to the cortex. This descending volley such mapping technique makes use of SSEP phase
is recorded over the spinal cord either epidurally reversal to identify the physiologic midline. SSEPs
or intradurally using a catheter-type bipolar elec- are recorded from the scalp to bipolar stimulation
trode.10,40 D-waves complement myotomal tceMEP along the longitudinal axis of the dorsal column. The
monitoring and provide valuable information that can site of stimulation is moved systematically from left
76 Section Iâ•… Introduction

Fig. 6.16â•… Left and right myo-


tomal tceMEPs and D-waves
recorded during resection of a
T2 intramedullary tumor in a
16-year-old female patient. Mus-
cle abbreviations: AD, adductor
longus; AH, abductor hallucis;
FD, first dorsal interosseous; GA,
gastrocnemius; QD, quadriceps;
TA, tibialis anterior. (a) Progres-
sive tceMEP attenuation on the
left side (arrow) evolving to com-
plete response loss from the left
adductor, quadriceps, and tibi-
alis anterior muscles and signifi-
cant (>€65%) response amplitude
attenuation from the left gas-
trocnemius and abductor hallucis
muscles. (b) Significant (> 65%)
amplitude depression of right
lower-extremity myotomal tce-
MEPs (arrow) correlative to signal
changes on the left side.
b
6â•… Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures 77

Fig. 6.16 (Continued)â•… (c) Preservation of D-wave recordings in the presence of significant deterioration of myotomal responses.
Slight changes in D-wave latency and amplitude are secondary to repositioning of the recording electrode to midline. This patient
awoke with new-onset left lower-extremity paresis that resolved within 24 hours.

to right until SSEP polarity inverts as the midline is nerve pathology, and it finds application in proce-
crossed. Alternatively, differential antidromic activa- dures like peripheral nerve transposition, explora-
tion of the left and right posterior tibial nerves can tion and resection of neuroma in continuity, and
be used to localize the dorsal median sulcus using intraoperative treatment of traumatic nerve injury,
the same stimulation paradigm, as illustrated in as shown in Fig. 6.18.
Fig. 6.17. A third strategy involves recording SSEPs We have found that the optimal approach to suc-
directly from the spinal cord during stimulation of cessful stimulation of surgically exposed periph-
the left and right posterior tibial nerves and local- eral nerve and recording of CNAPs is to use tripolar
izing the midline to the region of maximal response stimulation and bipolar recording.42,43 The tripolar/
amplitude. bipolar electrodes are bent like a shepherd’s crook
and are used to support or suspend the nerve away
from other conductive elements within the surgical
6.5.3╇ Peripheral Nerve ION field. The tripolar electrode is designed with both
outer electrodes linked together as the anode, with
ION during peripheral nerve surgery has been per- the middle electrode serving as the cathode. This
formed since the 1960s. In 1968, Kline and DeJonge tripolar arrangement limits the spread of stimulus
first reported on the use of direct nerve stimulation artifact and is particularly helpful in cases where dis-
and recording of compound nerve action potentials tances between the stimulation and recording sites
(CNAPs) to evaluate peripheral nerve injuries.41 This are less than 10 cm. The placement of a ground elec-
technique remains the gold standard for establishing trode between stimulating and recording electrodes
evidence of conduction in the presence of peripheral also aids in reduction of stimulus artifact.
78 Section Iâ•… Introduction

Fig. 6.17â•… Differential antidromic activation of the left and right posterior tibial nerves (PTN) during dorsal column stimulation
used to localize dorsal median sulcus prior to myelotomy. Left and right columns show evoked responses from left and right PTNs,
respectively, during stimulation at various sites over the dorsal spinal cord. Evoked responses recorded using paired subdermal
needles at the level of the medial malleolus. Lateralized bipolar stimulation (6 mA pulses, 200 µsec duration) along the longitudinal
axis of the spinal cord differentially activates the left or right PTN. Stimulation over the midline triggers smaller responses bilaterally.

6.6╇ What the Pediatric • To identify evolving disruption of biochemical,


metabolic, or hemodynamic processes that
Neurosurgeon Should predispose a neural pathway or anatomical
Understand about the Role of structure to injury
Neuromonitoring • To provide functional identification and
mapping of neural structures (e.g., cranial
Despite the increased use of ION in pediatric neuro- nerves, spinal nerve roots, peripheral nerves,
surgery over the past decade, its role sometimes con- eloquent cortical tissue) to help define safe
tinues to be misunderstood. In general, the principal boundaries for entry, dissection, or resection
goals of intraoperative neurophysiological monitor- • To assess functional integrity of anatomical
ing are as follows44: structures and pathways
6â•… Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures 79
Secondarily, ION can also aid in assessing the
effects of rescue intervention (such as elevation of
blood pressure or removal of compressive or dis-
tracting hardware) as well as adequacy of anesthetic
depth.44,45
The underlying basis for ION, therefore, is to min-
imize the overall incidence and severity of iatrogenic
neurologic injury, thereby improving surgical safety
and outcome. Contrary to the belief of some neuro-
surgeons, neuromonitoring should not be viewed in
the perspective solely of injury prediction, but rather
of injury prevention, as illustrated in Fig. 6.19.
The three cardinal rules for neuromonitoring suc-
cess in pediatric surgery are the following:
• View neuromonitoring manifestations of
“emerging” injury as early warning indicators
of impending functional neural compromise if
the situation is left unattended.
• Foster effective communication of ION alerts
to provoke increased surgical vigilance and/
Fig. 6.18â•… Compound nerve action potentials (CNAPs) or rescue interventions depending upon the
recorded from left median nerve in a 10-year-old child fol- type, pattern, and context of ION change.
lowing traumatic injury. Recordings were made using a bipo-
• Act promptly in the face of neuromonitoring
lar hook electrode placed under the nerve distal to the site of
injury. Constant voltage stimulation was delivered proximally change to stem continued evolution of
using a tripolar electrode. CNAPs with a latency of 2 msec were compromising conditions.
triggered at threshold stimulus intensity less than 2 V, confirm- The prevailing question then relates to when a given
ing conduction through the site of injury.
change in neural transmission is deemed clinically sig-
nificant, warranting a surgical alert and intervention.
To understand this better, the surgeon must recognize
that interpretation of intraoperative neuromonitoring

Fig. 6.19â•… Graphic hypothetical illustration to show that the purpose of ION is to identify early changes in neurophysiological con-
duction to enable timely intervention and prevention, not prediction of permanent neurologic deficit.
80 Section Iâ•… Introduction
data most often takes place in the presence of uncer- tive approach to monitoring at-risk structures, as
tainty or ambiguity. The role of the responsible ION well as be able to articulate any limitations imposed
provider, therefore, is to minimize those variables that by the patient’s baseline neurologic status, by tech-
can have a negative effect on interpretive decisions. nical limitations in the ability to interrogate spe-
The first step toward reducing interpretive ambi- cific structures at risk (such as cerebellum or visual
guity for injury prevention is for the ION provider pathways), or by suboptimal selection of monitoring
to have a clear understanding of why he or she is modalities or anesthetic technique.
involved in the surgery. Just as the surgeon devel- Adding complexity to the questions of what to
ops a surgical plan before engaging in surgery, so too monitor and how best to interpret the data is that
must the responsible ION professional. All too often there are few highly educated and experienced neu-
ION is based on a standardized, “one size fits all” romonitoring personnel with specialized knowledge
approach, rather than a risk-analysis algorithm cen- and training, particularly in pediatric neurophysi-
tered on answering a set of patient- and procedure- ological monitoring. Few would argue about the
specific questions, outlined as follows: need for a fellowship-trained pediatric neurosur-
geon and anesthesiologist; however, there has been
• Which neural structures, pathways, or
a general lack of that same appreciation for pediatric
vascular supplies, both within and outside of
neuromonitoring as a subspecialty requiring highly
the immediate surgical field, might be at risk
knowledgeable and skilled professional personnel
for neural injury?
for maximum benefit.
• Which neuromonitoring modalities should be If neuromonitoring is to be effective in general,
combined to provide the broadest surveillance and in pediatric neurosurgery in particular, then the
of those neural structures, pathways, or ION provider must be viewed as an integral member
vascular supplies potentially at risk for neural of the surgical team, and both the surgeon and anes-
injury? thesiologist must trust that the monitoring profes-
• What anesthetic techniques will have the least sional has the following attributes:
compromising effect on clinical interpretation
of significant signal change? • General and specialized fund of knowledge in
neuromonitoring as a specialty
• What nonsurgical, physiological, or
anesthetic factors (e.g., patient positioning, • Competence and experience in formulating an
hypotension, volume depletion, hypocapnia, appropriate monitoring plan
use of inhalational anesthetics, partial • Ability to make instantaneous decisions
neuromuscular blockade) or medical regarding neurophysiological change or for
comorbidities (e.g., diabetes, pre-existing anatomical mapping
neurologic compromise) can account • Ability to communicate changes effectively
for signal changes that can affect clinical in the context of the surgery, anesthesia, and
interpretation and decision making? physiology
• What surgical maneuvers are associated with • Ability to develop a plausible explanation for
risk of neural injury? neuromonitoring change so as to facilitate an
• What rescue intervention strategies (e.g., interventional strategy
elevation of blood pressure, release of
retractors, temporary cessation from surgery,
etc.) will help restore neural homeostasis in
the unlikely event of significant signal change? 6.7╇ Perspective of a Pediatric
Failure to formulate a rational neuromonitoring Neurosurgeon
plan based on answers to the foregoing six questions
It is often assumed that the main goal of intraop-
not only can lead to inappropriate selection or inad-
erative monitoring is to detect changes and thereby
equate number of monitoring modalities but also
avoid injury. In fact, while this is a critical role of
can increase the likelihood of miscommunication
monitoring, it is also true that the absence of change
between neuromonitoring personnel and the sur-
allows the surgeon working with an experienced
geon and anesthesiologist as well as increase poten-
monitoring team to have the courage to continue
tial interpretive error. Successful implementation of
a surgical intervention to the point of completion.
the neuromonitoring plan depends critically on a
This is true in tumor removal, in which the contin-
coordinated team approach that begins with preop-
ued maintenance of signals from the motor or sen-
erative discussion of surgical, anesthesia, and neu-
sory pathways or lack of irritation of cranial nerves,
romonitoring priorities. It is important that the ION
spinal nerve roots, peripheral nerves, and so forth
provider be able to communicate effectively with the
reassures the surgeon that dangerous territory has
surgeon and anesthesiologist about the most effec-
not been traversed. It is also true in resection of epi-
6â•… Intraoperative Neurophysiological Monitoring During Pediatric Neurosurgical Procedures 81
leptogenic tissue, which often looks grossly identi- ╇4. Larson SJ, Sances A Jr, Christenson PC. Evoked somatosen-
cal to adjacent eloquent cortex or subcortical tracts; sory potentials in man. Arch Neurol 1966;15(1):88–93
the steady maintenance of signals during resection ╇5. York DH. Somatosensory evoked potentials in man:
enables more complete elimination of the epilepto- differentiation of spinal pathways responsible for con-
genic zone. It is likewise true in complex tethered duction from the forelimb vs hindlimb. Prog Neurobiol
cord surgery, in which complete untethering often 1985;25(1):1–25
can be accomplished because the surgeon does not ╇6. Powers SK, Bolger CA, Edwards MS. Spinal cord path-
lose heart in a sea of fat and scar tissue for fear of ways mediating somatosensory evoked potentials. J
Neurosurg 1982;57(4):472–482
transecting vital nerves and causing weakness or
incontinence. In the ideal situation, the surgeon ╇7. Simpson RK Jr, Blackburn JG, Martin HF III, Katz S. Pe-
ripheral nerve fiber and spinal cord pathway contri-
relies on teamwork with the anesthesiologist and the
butions to the somatosensory evoked potential. Exp
monitoring professional, working together to accom- Neurol 1981;73(3):700–715
plish the surgical goal safely and completely.
╇8. Amassian VE, Stewart M, Quirk GJ, Rosenthal JL. Physi-
Despite ION’s status as an extraordinarily useful ological basis of motor effects of a transient stimulus to
adjunct, there are still “blind spots” in ION. These cerebral cortex. Neurosurgery 1987;20(1):74–93
include mapping and monitoring of cerebellar path- ╇9. Amassian VE. Animal and human motor system neuro-
ways, including the dentate nuclei; reliable mapping physiology related to intraoperative monitoring. In: De-
and monitoring of language, memory, and other letis V, Shils JL, eds. Neurophysiology in Neurosurgery.
neurocognitive areas in the anesthetized patient; San Diego, CA: Academic Press; 2002: 3–24
and visual pathway mapping and monitoring. Moni- 10. Deletis V. Intraoperative neurophysiology and method-
toring in very young infants is possible but requires ologies used to monitor the functional integrity of the
specialized techniques from an experienced team. motor system. In: Deletis V, Shils JL, eds. Neurophysi-
Pediatric neurosurgeons can, and should, be willing ology in Neurosurgery. San Diego, CA: Academic Press;
to collaborate with monitoring professionals, scien- 2002: 25–51
tists, and engineers to continue to develop the field 11. Schwartz DM, Sestokas AK, Hilibrand AS, et al. Neuro-
to meet the needs of the children we treat. physiological identification of position-induced neuro-
logic injury during anterior cervical spine surgery. J Clin
Monit Comput 2006;20(6):437–444
12. Bhalodia VM, Sestokas AK, Tomak PR, Schwartz DM.
6.8╇Conclusions Transcranial electric motor evoked potential de-
tection of compressional peroneal nerve injury in
Like most elements of modern surgical practice, the lateral decubitus position. J Clin Monit Comput
successful use of intraoperative neuromonitoring 2008;22(4):319–326
requires teamwork, communication, and a combina- 13. Lyon R, Lieberman JA, Feiner J, Burch S. Relative efficacy
tion of science and experience. Advances in moni- of transcranial motor evoked potentials, mechanically-
elicited electromyography, and evoked EMG to assess
toring over the past several decades have led to an
nerve root function during sustained retraction in a
increasingly important role for neuromonitoring porcine model. Spine 2009;34(16):E558–E564
during pediatric neurosurgical interventions. While
14. Amassian VE, Quirk GJ, Stewart M. A comparison of
more work needs to be done to expand the scope of corticospinal activation by magnetic coil and electrical
ION for children and to address some areas of con- stimulation of monkey motor cortex. Electroencepha-
troversy, the present state of the art clearly improves logr Clin Neurophysiol 1990;77(5):390–401
the safety and increases the scope of what surgeons 15. Journée HL, Polak HE, de Kleuver M, Langeloo DD, Postma
can accomplish. ION is a relatively young field, and AA. Improved neuromonitoring during spinal surgery us-
surgeons can promote its evolution by working ing double-train transcranial electrical stimulation. Med
closely with ION professionals to expand its use to Biol Eng Comput 2004;42(1):110–113
optimize the care of children with disorders of the 16. Schwartz DM, Sestokas AK, Dormans JP, et al. Transcranial
nervous system. electric motor evoked potential monitoring duing spine
surgery: is it safe? Spine 2011;36(13):1046–1049
17. Shah SA, Wiggins CR, Schwartz DM, et al. Spinal cord
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19. Schwartz DM, Auerbach JD, Dormans JP, et al. Neu- 32. Minahan RE, Riley LH III, Lukaczyk T, Cohen DB, Kostuik JP.
rophysiological detection of impending spinal cord The effect of neuromuscular blockade on pedicle screw
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2007;89(11):2440–2449 33. Schwartz DM, Bhalodia BM, Vaccaro A. Neurophysi-
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Intraoperative neurophysiologic detection of iatrogenic lumbar and thoracic spine. In: Loftus CM, Traynelis VC,
C5 nerve root injury during laminectomy for cervical eds. Intraoperative Monitoring Techniques in Neurosur-
compression myelopathy. Spine 2002;27(22):2499–2502 gery, 2nd ed. New York, NY: McGraw-Hill Professional,
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1996;21(14):1676–1686
7 Surgical Safety
Thomas G. Luerssen

The same motives always produce the same actions;


the same events follow from the same causes.
David Hume
An Enquiry Concerning Human Understanding (1748)

7.1╇Introduction preventing patient harm resulted in a reduction of


anesthesia-related deaths from 3.7/10,000 anesthetics
Medicine is delivered by human beings in order to to 1 to 2/200,000 in ASA I or II patients.3
try to help other humans. Regardless of intellect, Entire texts have been written about patient safety
motivation, training, and practice, human beings strategies and processes. Opportunities to prevent
make errors. Studies from the airline industry indi- harm exist at every point of a surgical patient’s man-
cate that, even for routine tasks in low-stress situa- agement, from first encounter to admission to dis-
tions, humans will make an error of some sort about charge and beyond. One common element in error
every 30 minutes. For complex tasks at high stress causation is seen throughout the patient care experi-
levels, this time drops to about 30 seconds.1 Fortu- ence and proves to be at the root of the vast majority of
nately, humans have the capability of adapting and adverse events that result in patient harm. This com-
can correct many errors immediately. However, mon element is absent or ineffective communication.4
because humans are fallible, many errors either are This chapter focuses on three major and related strate-
not recognized or cannot be corrected, and therefore gies derived from HROs that have gained widespread
result in harm and sometimes catastrophe. acceptance and have delivered improved outcomes,
The surgical patient is at particularly high risk for mostly by improving communication. These are:
adverse events in a hospital. The World Health Organi-
zation estimates that, in industrialized countries, major • The development of a culture of safety
complications occur in upward of 20% of inpatient sur- • The creation of effective surgical teams
gical patients, with a death rate of roughly 0.5%.2 Half • The use of communication support tools—
of these complications are thought to be preventable. specifically, checklists and handoff scripts—
Even the most straightforward procedures have numer- in patient care
ous complex steps that provide opportunity for pro-
cess failure and attendant patient injury. Consequently,
efforts at improving patient safety must assume major
importance in a health care system. 7.2╇ A Culture of Safety
Safety is a crucial element in what are called high-
reliability organizations (HROs). Examples of HROs Without an institutional commitment to patient
include the airline industry, nuclear power plants, elec- safety, processes that are implemented, even if man-
trical power grid systems, and military systems con- datory, have little chance of being effective or being
trolling operations on submarines or aircraft carriers. sustained. On the other hand, if a culture of safety is
These organizations are routinely exposed to high-risk established and nurtured, safety microsystems arise
situations but reliably achieve exceedingly low rates almost spontaneously, with substantial and measur-
of failure or accidents. These HROs developed system- able improvements in outcomes and reductions in
atic approaches to error prevention many years ago adverse events.
and continuously refine their safety systems in order HROs have several important characteristics
to approach zero defects. The lessons learned from that are diametrically opposite to the historical
HROs are only recently being applied in health care, approaches used in health care. First, HROs have a
and adoption of proven safety strategies in modern preoccupation with failure. Health care organiza-
health care has been agonizingly slow despite ample tions historically focused on successes and positive
evidence of effectiveness. A notable exception to this outcomes, referring failed processes, serious safety
statement comes from our colleagues in anesthesia. events, and adverse outcomes to “risk management.”
That specialty’s focus on detecting adverse events and Organizations preoccupied with failure measure and
83
84 Section Iâ•… Introduction
study all identifiable events, even if they do not result cause of adverse events in health care.4 Effective team-
in harm, and correct processes prior to an adverse work provides a framework for preventing communi-
event occurring. Repeat failures almost never occur. cation errors, by empowering and encouraging each
HROs avoid oversimplification. The delivery of member of the surgical team (surgeons, anesthesi-
health care is one of the most complex of all human ologists, nurses, technicians, etc.) to monitor not only
endeavors. Complex systems are not amenable to themselves in their own roles but also each other and
simplification strategies, which may seem to work clearly communicate concerns, or even stop a process
well for an organization most of the time, but when that is unclear until all members agree that it is safe
these strategies fail, the outcomes can be disastrous. to proceed. Formation of effective teams is not just
HROs constantly survey and improve their opera- naming individuals and assigning roles. It takes train-
tions. The culture promotes thorough understanding ing and practice and continuous improvement, as well
of operations from the frontline staff to the manage- as “after action” analysis of performance, especially in
ment and encourages communication of issues so the case of process failure. Effective team communi-
that problems are corrected as they are discovered cation is horizontal. This means that all members are
and before they reach a patient. considered equally important in their observations
In line with that, HROs listen to the institutional and expressing concerns, while recognizing that each
experts. Most of the time the true experts are not in member of the team has different knowledge, skill,
positions of authority but are right at the point of and expertise. The major characteristics of effec-
contact or point of care. In a culture of safety, these tive teams are team leadership, mutual performance
people are empowered and even encouraged and monitoring, backup behaviors, adaptability, commu-
rewarded to bring problems forward and to partici- nication, team orientation. and, perhaps most impor-
pate in developing effective solutions. tantly, mutual trust.7 The airline industry developed a
Finally, HROs are nimble and resilient. The culture program generally referred to as Crew Resource Man-
allows rapid responses to unanticipated events and agement (CRM), which has now been successfully
rapid recovery using innovative solutions. Managers applied in health care.8 There are now several train-
are empowered to respond with innovative approaches ing programs developed using the CRM methodology
when the standardized procedures are not working. that teach effective teamwork and communication in
Simply put, a culture of safety encourages trans- health care. There is strong evidence that the invest-
parent reporting, uses internal metrics much more ment in time and resources in team training reduces
than external benchmarks, and is focused on con- major adverse events in surgery.9
tinuous improvement in care delivery processes Lastly, regarding team performance in the OR—also
small and large. Potential problems are addressed based on lessons learned from the aviation industry—
prior to real problems occurring. Unexpected events the author strongly believes that the idea of a “sterile
are reported and dealt with immediately. Solutions cockpit” should be applied in the operating theater.
are developed both “top down” and “bottom up,” Although evidence of effectiveness is limited and prob-
and everyone at every level in the organization is ably impossible to prove, I have not allowed music
invested in error prevention as a core value. and have stringently limited idle conversations, phone
interruptions, and pagers in the operating room. Not
only are they distractions, but also the message they
confer to the team (and others) is that the team’s focus
7.3╇ Formation of Effective Teams is not entirely on the patient and the procedure at hand.

From this point on, the focus of this chapter is the


operating room (OR). Gawande and coworkers have
shown that, for surgical patients, most patient harm 7.4╇ Checklists and Structured
events occur in the OR.5 However, the concepts dis- Communication
cussed—team formation and function, communi-
cation, and the use of checklists and structured Checklists are structured communication and
communication—apply universally throughout the recording of processes that are designed to increase
surgical patient’s care continuum, from first contact standardization and to avoid reliance on memory in
with a health care system to well after discharge. order to reduce errors and omissions. Safety checks
Pronovost and Freischlag, in a recent editorial, pro- have been shown to be effective in high-risk areas
vided a succinct description of the culture of the mod- like aviation and nuclear power. In the seminal report
ern-day OR: “Operating rooms are among the most by the Institute of Medicine in 1999, it was recom-
complex, political, social and cultural structures that mended that verification processes like checklists be
exist, full of ritual, drama, hierarchy, and too often implemented in medical practice.10
conflict.”6 This culture provides an ideal environment In response to that suggestion, the Joint Commis-
for miscommunication or failed communication, sion mandated the use of what was called the “Universal
which, as discussed earlier, are the most common Protocol” (UP) in 2004. This was a rudimentary preop-
7â•… Surgical Safety 85
erative checklist. Most reports indicated that the UP had practice as well as measurement and reporting
minimal if any effect on reducing serious harm events in of performance. It took about one year to achieve
the OR. We studied the UP prior to implementing a more our goal of greater than 95% compliance. However,
robust checklist at Texas Children’s Hospital (TCH) and when we studied the effect of the SSCL on patient
found that, although the UP was reported as completed outcomes, we noted a major reduction in patient
for every operation, it was led by the nurses, while other safety events in the OR and improved efficiency
members of the OR personnel were disengaged and fre- in the OR. Even more interesting, as the SSCL was
quently not even in the room. A trained independent adopted, a safety microsystem appeared in which
observer of the UP during that study scored it as com- the users began expanding and improving the TCH
plete and correct less than half the time. SSCL. After regular and multiple iterations, TCH
In 2009, the World Health Organization’s (WHO) now has six SSCLs in five geographic OR environ-
Safe Surgery Saves Lives Study Group published the ments, along with additional service-specific, case-
results of a robust surgery safety checklist (SSCL) specific, and intraoperative emergency procedure
used in eight hospitals worldwide.11 They showed a checklists (Fig. 7.1).
positive beneficial effect for this SSCL. Even prior to WHO-based checklists are tripartite and are
that publication, many institutions, including ours, meant to address the three major steps in a surgi-
were implementing a WHO-based SSCL. Using qual- cal patient’s journey through the OR. The three
ity improvement methodology, we developed an parts include a preoperative “briefing,” which occurs
SSCL for our patient population and implemented just before the patient is transferred from the pre-
it beginning in January 2009. We learned that, very operative holding area (or hospital floor, intensive
much like forming effective teams, the implemen- care unit, or emergency room) to the OR suite. This
tation of an SSCL requires specific training and is meant to be an opportunity for the surgeon and

Fig. 7.1â•… Current Texas Children’s Hospital surgery safety checklist for the main campus operating rooms. Courtesy of Texas Chil-
dren’s Hospital.
86 Section Iâ•… Introduction
anesthesiologist and the OR team to review the plans occur frequently and are the points where critical
and concerns for the upcoming procedure; to iden- information is omitted or misunderstood. Struc-
tify the patient; to confirm the procedure site and tured handoffs are one of several ways being devel-
side; and to confirm that all the necessary equip- oped to transfer important information efficiently
ment, implants, blood products, drugs, and irrigation and accurately. Two common and well-developed
fluids are available. We try to have the family partici- handoff strategies are SBAR (for Situation—Back-
pate in the preoperative briefing. ground—Assessment—Recommendation) and P5 (for
There is also a postoperative “debriefing,” which Patient—Problems—Pertinent History—Plans or Pro-
occurs before the patient leaves the OR suite. This cedure—Precautions or Pitfalls). Whatever structured
is the opportunity to ensure that all specimens and communication process is adopted by an institution,
laboratory tests are checked and rechecked, that the it takes training and practice among users in order to
receiving patient care area is ready for the patient become effective. Most important, it should become
with staff and equipment, and that there is agree- an expectation for the transmitter and the receiver
ment between surgeon and anesthesiologist about of information, with specific ways of clarifying and
the postoperative care issues. It is also the time for confirming understanding of the information.
“after action analysis,” where problems are dis-
cussed and referred for solution, equipment issues
are identified and referred for repair, and opportu-
nities for improvement are brought forward. Highly 7.6╇Conclusion
effective OR teams use the briefing and debriefing
opportunities and structure to their fullest potential, Patient safety, error prevention, and structured pro-
expanding the information transfer far beyond the cesses to prevent adverse outcomes are assuming
minimums requested. a primary position in health care. It is likely that
The cornerstone of an SSCL is the “timeout” or most institutions and health care systems will move
surgical pause. This occurs immediately before inva- toward mandatory training in error prevention. Fur-
sion of the patient. Most of the time this means prior thermore, credentialing and recredentialing of medi-
to the surgical incision, but we have interpreted this cal privileges will include analysis of adverse events
more broadly and do timeouts prior to pin fixation, and participation in institutional safety and error
injections of anesthetic, placement of lumbar drains, prevention programs. Considering that almost half
and so forth. If anesthesia personnel are placing deep of adverse events occurring in a hospital arise in the
lines or placing blocks, they perform a timeout prior OR, surgeons and OR staff should embrace patient
to their procedures. The timeout is a “system reset”— safety and error prevention efforts. Considering the
the last chance to confirm all the important elements impact that safer surgery can have on an institution,
of the procedure. This is a true checklist, meant to be the OR can become the innovation laboratory for
read point by point—not memorized or altered—and safety program development in the future.
listened to by every member of the team. Any con-
cerns are voiced, and all members of the team must
References
confirm agreement that they are ready to proceed.
There is no reason to forgo performing a timeout, ╇1. Müller M. Increasing safety by implementing opti-
including emergencies. A timeout usually takes less mized structures of team communication and the
than 90 seconds, and complications are reduced in mandatory use of checklists. Eur J Cardiothorac Surg
urgent operations when a preprocedure checklist is 2012;41(5):988–992
used.12 ╇2. World Health Organization. WHO Guidelines for Safe
Checklists are living documents. Our SSCLs are Surgery 2009. Geneva, Switzerland: World Health Or-
revised about every 6 months, using input from ganization; 2009. http://whqlibdoc.who.int/publica-
the OR staff and surgeons and from analysis of tions/2009/9789241598552_eng.pdf
safety reports. Each revision has been immediately ╇3. Pierce EC Jr. The 34th Rovenstine Lecture. 40 years
accepted by the users, and compliance remains behind the mask: safety revisited. Anesthesiology
1996;84(4):965–975
extremely high.
╇4. The Joint Commission. The Joint Commission: four
key root causes loom large in sentinel event data. ED
Manag 2012;24(6):S3–S4
7.5╇ Handoffs ╇5. Gawande AA, Thomas EJ, Zinner MJ, Brennan TA. The
incidence and nature of surgical adverse events in Colo-
Clear communication is essential when transfer- rado and Utah in 1992. Surgery 1999;126(1):66–75
ring patient care from location to location and/or ╇6. Pronovost PJ, Freischlag JA. Improving teamwork to re-
from caregiver to caregiver. In a progressively more duce surgical mortality. JAMA 2010;304(15):1721–1722
complex medical environment, these transitions
7â•… Surgical Safety 87
╇7. Baker DP, Day R, Salas E. Teamwork as an essential cine. To Err is Human: Building a Safer Health System.
component of high-reliability organizations. Health Washington, DC: National Academy Press; 2000
Serv Res 2006;41(4 Pt 2):1576–1598 11. Haynes AB, Weiser TG, Berry WR, et al; Safe Surgery
╇8. Dunn EJ, Mills PD, Neily J, Crittenden MD, Carmack AL, Saves Lives Study Group. A surgical safety checklist to
Bagian JP. Medical team training: applying crew resource reduce morbidity and mortality in a global popula-
management in the Veterans Health Administration. Jt tion. N Engl J Med 2009;360(5):491–499
Comm J Qual Patient Saf 2007;33(6):317–325 12. Weiser TG, Haynes AB, Dziekan G, Berry WR, Lipsitz SR,
╇9. Neily J, Mills PD, Young-Xu Y, et al. Association between Gawande AA; Safe Surgery Saves Lives Investigators and
implementation of a medical team training program Study Group. Effect of a 19-item surgical safety check-
and surgical mortality. JAMA 2010;304(15):1693–1700 list during urgent operations in a global patient popula-
10. Kohn LT, Corrigan JM, Donaldson MS, Committee on tion. Ann Surg 2010;251(5):976–980
Quality of Health Care in America, Institute of Medi-
Section II
Neurology
Section Editor: Scott L. Pomeroy

In this era, with an ever-increasing number of of infants, children, and adolescents. The authors
advanced diagnostic modalities, the neurologic describe the need for careful observation to assess
examination remains the essential first step in overall function, followed by a step-by-step, detailed
assessing an infant or child with nervous system examination to determine whether individual com-
disease. Through skilled examination, the locus of ponents of the nervous system are impaired.
disease can be established and, combined with his- Each of the authors of this section has extensive
tory and general examination, the cause of disease experience in neurologic assessment. The content
can often be inferred and priorities set as to whether of each chapter reflects a careful and systematic
urgent intervention is needed. approach. The chapters describe important obser-
The chapters in this section describe the steps vations and examination tips, pointers and “pearls,”
and procedures needed for neurologic examination which are truly the tricks of the trade.
8 Neonatal Neurologic Examination
Charles C. Duncan and Laura R. Ment

8.1╇ Introduction and Background some for neonatal seizures and an underlying focal
lesion, while the infant with irregular respirations or
Despite the development of sophisticated perina- periods of spontaneous apnea may harbor a serious
tal assessment strategies, the examination of the posterior fossa lesion or be experiencing seizures.
newborn infant remains a key step in the neona- Finally, the baby with no spontaneous move-
tal neurologic evaluation. The neonatal neurologic ments but a normal pattern of respiration may be
examination is gestational age- and state-dependent simply sleeping, but those who appear “hyperalert”
and may provide important information about both with darting eye movements and spontaneous clo-
central and peripheral neurologic problems. While nus may be suffering from hypoxic, toxic, or meta-
much has been written on this topic, the funda- bolic encephalopathy.
mentals of the neurologic examination of the neo-
nate are simple and easy to perform and may offer
critical information about urgent neurologic and
neurosurgical problems. They are dependent on
8.3╇ The Infant’s Response to
observation, the infant’s response to external stim- External Stimulation
ulation, evidence of appropriate neuromaturation,
and an assessment of tone and reflexes. Following a quiet period of observation, the infant
should be stimulated with a bright light, bell, and
noxious stimulation. The eyelids are fused in pre-
term neonates until the 26th week of gestation, but
8.2╇ Observation of the Neonate all neurologically intact infants will blink to light,
even through closed eyelids. Failure to blink to light
A hallmark of the neonatal neurologic examination may reflect an altered level of consciousness or a
is observation of the resting infant. This inspection structural lesion.
should include observations about tone and sponta- Similarly, ringing of the bell should result in
neous movements, respiratory pattern, and level of change of state. Sleeping infants will arouse, while
alertness. active babies may quiet. Movements should be sym-
Although tone and posture are related to post- metric and nonstereotypic, as myoclonic jerks may
menstrual age (i.e., weeks from conception) at the be stimulus-, and particularly noise-, dependent. As
time of assessment, it is important to note both with the response to photic stimulation, the failure
the infant’s posture and spontaneous movements. to respond to a bell may reflect encephalopathy, but
Preterm neonates of 28 weeks or less will lie with it may also be indicative of structural or toxic disor-
arms and legs fully extended, and term infants will ders. Neonates with auditory neuropathies or struc-
assume a posture of full flexion, but in infants of tural malformations may be awake and active but
all postmenstrual ages, spontaneous movements unresponsive to sound. Similarly, those with toxic
should be symmetric and nonstereotypic. An infant exposures, such as hyperbilirubinemia and emerg-
with a hemiparesis will have decreased spontane- ing kernicterus, may also not respond to auditory
ous movement of the affected arm and leg, while a stimulation.
neonate with a plexus injury may show a flaccid arm Finally, a gentle sternal rub (i.e., “noxious stimu-
with weak shoulder, wrist, or hand. lation”) should evoke symmetric movement of all
Similarly, the neonate with repetitive, stereotypic, four extremities. Failure of one arm and leg to mir-
rhythmic movements of a single extremity is worri- ror the other strongly suggests a focal lesion, while

91
92 Section IIâ•… Neurology
a flaccid arm may be indicative of a brachial plexus ber a few key facts. First, tone and emerging motor
lesion. Importantly, evidence of myoclonic activity, skills must always be symmetric; second, intercur-
tonic posturing, or apneic respirations are all consis- rent infectious or metabolic etiologies may cause the
tent with significant neurologic impairment requir- infant to appear severely encephalopathic and thus
ing immediate attention. Likewise, infants with little obscure neurologic findings; and finally, a mismatch
or no response to sternal rub should be immediately in tone and motor skills suggests a central disorder.
evaluated for intracranial, cervical, and metabolic In the preterm neonate, the developmental emer-
life-threatening conditions. gence of tone begins in the lower extremities. Thus
Nonsedated infants with no response to photic, the infant of 28 weeks postmenstrual age will inde-
auditory, and tactile stimulation need urgent evalu- pendently lie with legs fully extended, the neonate of
ation for coma, while those with atypical or focal 30 to 32 weeks will begin to flex the hips and knees,
responses should be evaluated for infectious, struc- and a 34-week infant will begin to demonstrate the
tural, or metabolic causes of encephalopathy. “frog-leg” posture of a term neonate. There is simi-
lar progression of tone in the upper extremities such
that the arms are fully extended in the infants of 28
8.3.1╇ Evidence for Neuromaturation weeks gestation, beginning to flex at 30 to 32 weeks
and fully flexed by 34 to 36 weeks of gestation.
The increasing survival of extremely low gesta- Two signs common to the emergence of tone in
tional neonates demands a working knowledge of the preterm neonate are the “scarf sign” and assess-
neuromaturation, and serial examinations of the ment of the popliteal angle. To perform the scarf sign,
prematurely born should optimally demonstrate the examiner gently pulls the infant’s arm across his
progressive acquisition of the neurologic parameters chest and measures the position of the elbow. The
that characterize term neonates. elbow will not reach the midline in the term infant,
while in a hypotonic neonate or one of lower gesta-
tional age the elbow will extend across the midline
Visual System to the opposite thorax.
Similarly, to perform an assessment of the pop-
Eyelids are fused in preterm neonates through the liteal angle, the examiner uses one hand to flex the
26th week of gestation, and the pupils are dilated infant’s thigh upon her abdomen. Putting gentle
and nonreactive to light until gestational week 28. pressure on the back of the thigh with the other
Eye movements should always be full and equal but hand, the examiner extends the leg and assesses
are frequently difficult to assess in the critically ill the angle formed by the thigh and lower leg. In term
neonate. Notably, although one does not expect the infants, this angle is approximately 90°, while those
newborn infant to fix and follow reliably on a bright of younger gestational age or hypotonia will extend
light or multicolored large object, healthy term neo- their legs 120° or greater.
nates whose mothers have not received sedation or Likewise, the emergence of head control (dem-
pain medication will fix and follow on the day of onstrated by holding the upper extremities and rais-
birth. Similarly, healthy preterm neonates (i.e., “feed- ing the infant from the examination table) does not
ers and growers”) will also fix and follow beginning begin until 34 weeks and is not fully present until 36
at 34 weeks of gestation. to 40 weeks postmenstrual age. Notably, the healthy
When assessing visual function, it is important to preterm infant will also begin to bear weight when
remember both that the focal length of the term neo- placed in an upright position at approximately 34
nate is approximately 6 to 8 inches and that neonates weeks, and a final hallmark of typically developing
will make random horizontal eye movements. Thus, infants at postmenstrual age is the emergence of
when one is certain that the baby is fixing on the shoulder strength, best demonstrated by the abil-
target object, it is key to ensure that she will track ity to not “slip through” when held gently under the
not only horizontally but also up and down. Finally, arms in an upright position.
although it is possible to perform a neurologic exam-
ination on a sleeping infant, newborns are unlikely
to “fix and follow” when awoken from a sound sleep Primitive Reflexes
and may require a second assessment.
Finally, several of the primitive reflexes, such as
the Moro, sucking, and swallowing responses, are
Acquisition of Tone and Motor Skills also gestational age–dependent. Thus, although we
have never seen a surviving neonate who lacked a
While much has been written about the develop- Moro response, infants of less than 36 weeks gesta-
mental progression of both passive and active tone tion demonstrate the incomplete Moro, in which the
in the preterm neonate and the conditions that alter arms are extended but there is no secondary flex-
tone in the term infant, it is important to remem-
8â•… Neonatal Neurologic Examination 93
ion to the midline. Similarly, the sucking response entiated by an assessment of tone and reflexes. The
emerges between 30 and 32 weeks postmenstrual infant with a central lesion will exhibit an altered
age, but the neonate cannot reliably suck and swal- level of arousal, focal increased tone, and brisk
low until 33 to 34 weeks of gestation. reflexes, while the neonate with a brachial plexus
injury will be wide awake and eating well but have
a flaccid, areflexic extremity and the possibility of a
8.3.2╇ Assessment of Tone and Reflexes clavicular fracture.

The assessment of tone, reflexes, and motor skills


(also called “active tone” by many observers) can Symmetric Changes in Tone and Reflexes
provide important information not only about neu-
rodevelopment during the third trimester of gesta- Infants with symmetric changes in tone and reflexes
tion but also about the pattern and localization of may harbor neurologic disease at any point in the
injury in critically ill neonates of any gestational age. neuraxis, and the careful neurologic examination
As previously described, the infant’s tone and motor will help to localize this for the clinician.
skills should be both consistent with his gestational Infants with obtundation, peripheral hypertonia,
age and at all times symmetric. poor active tone (i.e., “mismatch” in active and pas-
It is easiest to obtain reflexes at the biceps, sive tone examinations), and marked hyperreflexia
patella, and Achilles tendons in preterm and term should be evaluated for a central lesion, while flaccid
infants. While the biceps and patellar reflexes are infants with obtundation and absent reflexes require
performed in similar fashion for both neonates and immediate evaluation for spinal injury. Notably, the
older children and adults, it may be easier to obtain former group of infants, in whom an intracranial
the Achilles reflex by flexing the infant’s ankle and lesion is suspected, may exhibit two additional signs
tapping on the plantar surface. Neonates are not yet of “hypertonia.” The first is the “hypertonic Moro,” in
well peripherally myelinated, and reflexes should which there is no first phase of abduction of the upper
be at most 1+ symmetrically present in infants of extremities; instead, the infant whose head has been
all postmenstrual ages. If one notes “brisk reflexes,” gently dropped onto the mat shoots his arms forward
such as ankle clonus or the spread of one knee jerk to in response to this stimulus. The second finding is the
the opposite extremity, the examiner should confirm “hypertonic suck” or “chomp,” a well-known hallmark
such hyperreflexia with both crossed-adductor and of the infant with a central lesion.
patellar reflexes. In contrast, neonates with altered levels of
Thus, one expects the extremely low gestational arousal but gestational age appropriate or some-
age infant to be profoundly floppy with no head con- what depressed tone and strength are more likely to
trol or shoulder strength. The 34-week gestational age demonstrate structural, chromosomal, or metabolic
infant should demonstrate emerging head control and lesions. Additional aspects of the physical examina-
tone within both the lower and upper extremities, tion, such as dysmorphic features, cardiac murmurs,
while the term neonate should have good (but not or organomegaly, as well as imaging studies, may
excessive) passive tone, which permits the full range provide evidence of chromosomal disorders.
of movements, as well as appropriate head control, Finally, alert infants with feeding difficulties, poor
shoulder strength, and the ability to bear weight. central and peripheral tone, and absent reflexes should
be evaluated for spinal muscular atrophy, neuropathy,
or congenital myopathy. Notably, the neurologic char-
Focal Neurologic Findings acteristics of the infant with spinal muscular atrophy
include tongue fasciculations, micrognathia, arthro-
Focal lesions include those characterized by hemi- gryposis (i.e., congenital contractures), congenital hip
paresis or monoparesis. Infants with evidence for a dislocation, and spontaneous fine movements of the
hemiparesis—and particularly those with hyperre- fingers and toes consistent with peripheral fascicula-
flexia—should be urgently assessed for a focal lesion. tions. In addition, their mothers also report decreased
Notably, approximately 75% of infants with perinatal fetal movement and polyhydramnios. Infants with
stroke present with hemiparesis and focal seizures myotonic dystrophy will be similarly hypotonic and
in the newborn period, while those with acute sub- areflexic but exhibit none of the signs of anterior horn
dural hemorrhages are obtunded in association with cell dysfunction. Notably, myotonia is not elicitable in
a spastic hemiparesis. The latter group of infants neonates until the end of the first year of life, but it can
will demonstrate split cranial sutures, full fonta- be easily tested by tapping on the thenar eminence of
nelles, and abnormal eye movements on complete affected mothers. Finally, although rare, infants with
examination. congenital myopathies/dystrophies, neuropathies, or
In contrast, monoparesis may reflect either a mitochondrial disorders may present with hypotonia
central or a peripheral lesion. These are best differ- and hyporeflexia.
94 Section IIâ•… Neurology

8.3.3╇ Elements of the Traditional Cranial Nerve Assessment


Neurologic Examination
Although the pupillary response to light is present
Following the observation, stimulation, and assess- in neonates beginning at the 28th week of gestation,
ment of both neuromaturation and tone/reflexes in infants with fixed and dilated pupils one must
portions of the neonatal neurologic assessment, the always check that the pupils have not been pharma-
examiner is encouraged to perform the more tradi- cologically dilated in preparation for a retinal exam.
tional aspects of the pediatric neurologic examina- Although it has often been written that “infants do
tion. These include assessment of growth parameters, not herniate,” exceptions to the rule do occur, and
assessment of cranial nerve function, and the general encephalopathic infants with the new onset of either
physical examination. unilateral or bilateral fixed and dilated pupils should
be emergently evaluated for intracranial hemor-
rhage or the acute onset of hydrocephalus or edema.
Growth Parameters If the imaging study is unremarkable, the fixed and
dilated pupil(s) should raise concern for a postictal
Critical to the neurologic examination of the neonate phenomenon, and an electroencephalogram (EEG)
is assessing not only the infant’s growth status (i.e., should be performed. As a final word, it is important
appropriate for gestational age [AGA], small for ges- to check the infant’s pupillary responses but not to
tational age [SGA], or large for gestational age [LGA]) evoke periorbital ecchymoses and edema; having the
but also the orbitofrontal circumference (OFC). SGA nonobtunded infant of 32 weeks gestation onward
infants may have experienced maternal placen- suck on a pacifier almost always results in the eye-
tal dysfunction, suffered congenital infections, or opening reflex.
harbor genetic abnormalities (and subsequent CNS In contrast to the pupillary response, eye move-
malformations). Further, although LGA status may ments—both horizontal and vertical—are present
be the result of genetic variants, such as Beckwith- in infants of all gestational ages. Since sleeping,
Wiedemann syndrome, it may reflect abnormalities obtunded, or otherwise critically ill neonates will
in maternal glucose. not typically fix and follow on a bright object, it is
As with weight and length, OFC should be plotted important to note that the doll’s head maneuver, in
on gestation-age-appropriate curves to assess rela- which the infant’s head is moved slowly from side to
tive micro- and macrocephaly. Microcephaly may be side, can be used to elicit horizontal eye movements
attributable to congenital infections, chromosomal in infants until the 46th postmenstrual week. This
variants, congenital CNS malformations, or maternal technique should never be employed, however, in an
toxic exposure. infant in whom there is a question of spinal injury. In
In contrast, macrocephaly is found both in addition, if eye movements appear to be absent and
infants with acute disorders and those with con- cold-water caloric stimulation is to be performed,
genital disorders. The emergent causes of macro- one must first check the intactness of the tympanic
cephaly include hydrocephalus, acute intracranial membranes.
hemorrhages (intraparenchymal, subdural, or Corneal responses may be easily obtained in
epidural), and tumor. These neonates have split sleeping infants by gently applying a sterile swab to
sutures, full fontanelles, and intermittent or fixed the corneal membrane; an alternative strategy is the
downgaze with sixth nerve palsies, as well as simple use of a puff of air directed at the cornea. Sim-
emesis and apnea, and should be evaluated with ilarly, spontaneous facial movements are common
emergency magnetic resonance imaging (MRI) of in sleeping infants, and thus the assessment of cra-
the brain. nial nerve VII may be done in either the sleeping or
Macrocephaly is also found in neonates with awake infant. In the encephalopathic neonate, a gen-
the following disorders: congenital hydrocephalus tle sternal rub almost always results in facial move-
(secondary to fetal hemorrhagic stroke, congenital ment, although the eyelids may not necessarily open.
infection, or structural malformation); neurocu- As described previously, hearing can be checked by
taneous disorders, such as neurofibromatosis and auditory stimulation.
tuberous sclerosis; chromosomal abnormalities; Finally, checking the gag reflex can be challeng-
and familial “big head.” Notably, benign enlarge- ing in neonates of all gestational ages; gently strok-
ment of the subarachnoid spaces, or “external ing the infant’s palms will almost always result in
hydrocephalus,” is not commonly diagnosed in the mouth-opening (the palmo-omental reflex), per-
newborn period but rather occurs across the first mitting the gag to be checked with a soft swab. In
postnatal year. critically ill neonates, the gag reflex is occasionally
8â•… Neonatal Neurologic Examination 95
followed by a secondary period of apnea, suggesting five different ways, characterized as follows: multi-
that the examiner should be particularly vigilant. focal tonic-clonic activity (one arm followed by a leg
“waving” in stereotypic fashion across the warming
table); focal clonic or tonic-clonic events (the hall-
Elements of the General Physical Examination mark of an underlying structural lesion); myoclonus,
or repetitive “Moro”-like events; intermittent tonic
The neonatal neurologic examination would not be activity worrisome for herniation; and apnea. Sus-
complete without a check for dysmorphic and atypi- picion for seizure should be followed by immediate
cal features. Colobomata, or keyhole-shaped defects assessment for hypoglycemia and meningitis fol-
in the iris, retina, choroid, or optic disk, may be a lowed by EEG. Since neonates may not reliably per-
component of syndromes like CHARGE (coloboma, form the given activity during a routine 50-minute
heart defects, atresia of the nasal choanae, retarda- tracing, a video-EEG is preferable whenever possible.
tion of growth, genital abnormalities, and ear abnor-
malities or deafness) or may be found in typically
developing neonates. They are generally unilateral. CNS Imaging
Similarly, congenital cataracts accompany a wide
variety of disorders ranging from congenital infec- The advent of cranial ultrasonography and MRI has
tions, including toxoplasmosis and cytomegalovirus greatly improved our understanding of neonatal
(CMV), to genetic and metabolic causes, including neurologic emergencies, and the imaging strategy
Down syndrome and galactosemia. Facial dysmor- of choice depends upon the infant’s gestation and
phisms may be associated with genetic syndromes; symptoms.
sacral dimples and midline spinal hairy patches are Cranial ultrasound (cUS) provides the opportu-
markers of spinal deformities; and peripheral abnor- nity to evaluate neonates rapidly for intraventricu-
malities, such as rocker bottom feet, may be markers lar hemorrhage, ventricular size, and periventricular
of trisomy 13 or 18. Skin lesions (both hyperpig- anomalies at the bedside. Pitfalls include peripheral
mented and hypopigmented) are found in neurocu- and posterior fossa issues. Serial cUS can demon-
taneous disorders, and hepatomegaly accompanies strate important trends in ventricular size in post-
both congenital infections (CMV) and metabolic/ hemorrhagic hydrocephalus in the premature infant,
genetic disorders ranging from Zellweger syndrome for example.
to galactosemia. MRI ordinarily involves infant transport except for
the few very fortunate nurseries with such machines
within them or adjacent. Ordinarily, contrast is not
Examining the Parents needed unless detailed MR angiography is needed or
parenchymal infection or tumor is of concern. Quick-
The final portion of the examination involves not brain MRI (qbMRI) or fast-spin T2 imaging can pro-
only interviewing but on occasion examining the vide more rapid imaging but limits the information
parents. Macrocephaly is commonly familial, atypi- available for parenchymal lesions. Finally, infants
cal features are not always dysmorphic, and neuro- may not show diffusion changes for a week or more.
muscular disorders frequently run in families. Thus Computed tomography (CT) is rarely utilized at
the parent of an infant with megalencephaly may present except in emergency situations with rapid
have multiple café au lait spots, while that of a floppy neurologic progression, as with acute epidural, sub-
and hyporeflexic neonate may exhibit myotonia. dural, or parenchymal hemorrhages. Exceptions can
occur with depressed fractures and nonaccidental
trauma.
8.3.4╇ Strategies for Examining the Short T1 inversion recovery (STIR) MRI of the
Neurologic System of the Neonate spine has been the most useful in the evaluation of
spinal trauma. In infants, the entire spine can ordi-
Electroencephalography narily be imaged within the coil; hence, obtaining
this imaging well repays the effort. In nonaccidental
Seizures occur in up to 5% of infants in newborn trauma, injuries can be found across the entire spine.
intensive care units, and the seizures of neonates do Serial imaging in infants, whether by cUS, MRI, or
not necessarily mirror those of the older infant and a combination, like serial neurologic examinations,
child. Generally, neonatal seizures are manifest in can be critical to their care.
9 Neurologic Examination of the
Child and Adolescent
Jessica H. R. Goldstein and Nancy Bass

9.1╇Introduction build a rapport before tackling the more challenging


parts of the exam.
The neurologic examination of the child and adoles- The neurologic examination always begins with
cent is very similar to that of the adult patient, with a thorough history and general physical exam. A
one very important exception: it is much harder! comprehensive history should be obtained and
Often, pediatric patients are unable to explain what should include birth history as well as developmental
their problem is or follow the instructions for a neu- history. Sometimes, the only symptom of
rologic exam. hydrocephalus in a school-age child is worsening
The neurologic examination begins from the school performance. Even in the older child, growth
moment you first walk into the room. Observing the parameters, particularly head circumference, should
child informally while taking a history can provide be obtained and compared to previous values to
valuable information and guide the more formal obtain a trajectory of growth.
examination. Is there an asymmetry in movement? The neurologic exam itself can then be broken
How does the child play and interact with family and down into seven broad categories: mental status,
surroundings? Are there dysmorphic features, unusual cranial nerves, motor, reflexes, sensation, coordination,
head shape, or birthmarks? Many important physical and gait.
findings are uncovered before the formal neurologic
exam begins.
The aim of this chapter is to provide a concise 9.2.1╇ Mental Status
and complete guide to the pediatric neurologic
The mental status test of the young child relies on
examination, with emphasis on strategies for
the physician’s ability to engage the child in con-
examining young, uncooperative, or cognitively
versation.1 The age and developmental level of the
impaired children.
patient must be taken into consideration. The com-
plete mental status examination encompasses the
following modalities: level of consciousness, orien-
9.2╇ The Pediatric Neurologic tation, speech/language, memory, fund of knowl-
edge, insight/judgment, abstract thought, and
Examination calculations.
The power of observation allows the clinician
The purpose of the neurologic exam is to localize to assess the structure and function of the child’s
an area of dysfunction based on a chief complaint. language, the quality and content of play, as well as
In the pediatric population, this requires assessing a affect, eye contact, and behavior.2
patient’s neurologic function in the context of his or
her development, which may be normal or abnormal.
A friendly demeanor, keen observation of the child, 9.2.2╇ Cranial Nerves
and flexibility with the order in which the exam is
performed will help maximize the information to be The cranial nerve examination is the most difficult
gained. For example, many children are apprehen- portion of the neurologic exam in an uncoopera-
sive about having their reflexes tested or funduscopic tive or young child. A colorful toy can be your most
examination but enjoy the “less invasive” strength or important tool.
gait exams. Starting with the “least invasive” exam Cranial nerve I: Sense of smell can be difficult to
elements may help the child relax and allow you to test in a young child. Absence of sense of smell can be
96
9â•… Neurologic Examination of the Child and Adolescent 97
seen in patients following a head injury, with frontal In addition to its auditory functions, CN VIII carries
lobe tumors, or with congenital brain malformations. vestibular information, which can in part be assessed
Cranial nerve II (optic nerve): Funduscopic during the Romberg test (described subsequently).
examination begins with identifying the red reflex Vestibular function can more thoroughly be assessed
from afar. This is vital to ensure there is no “white with caloric testing in symptomatic patients.2
pupil,” which could suggest a retinoblastoma or Cranial nerves IX/X: Many children are very
congenital cataract. To check visual fields in a child, resistant to examination of the posterior pharynx.
show the child a toy in central vision and slowly bring Asking the patient to open the mouth and say “Aaah”
in another toy from the periphery. Visual acuity in and observing for palate symmetry can assess cranial
the young child who is not yet able to identify letters nerves IX/X. To assess cranial nerves IX and X fully,
confidently can be assessed with a picture chart. a gag reflex can be elicited with a cotton swab. This
Cranial nerves III, IV, and VI: To begin the assess- maneuver is frequently met with a good deal of
ment of the extraocular muscles, first examine the resistance from the child and is often best saved until
eyes at rest. Look for a “setting-sun” sign, indicating the end of the exam.
increased intracranial pressure, or a tendency for one Cranial nerve XI: To test the strength of the
eye to drift when covered, suggesting strabismus sternocleidomastoid muscle, have the child turn
(Fig. 9.1). Next, check the child’s eye movements in his or her head to each side against your resistance.
all four quadrants. In a young or uncooperative child, Shoulder shrugs against resistance provide an
moving a colorful toy can help with this assessment. assessment of trapezius muscle strength.
Note any limitations in movement or dysconjugate Cranial nerve XII: “Stick out your tongue!”
gaze. The tongue should be examined for atrophy,
Cranial nerve V: In older children, sensation fasciculations, and deviation. Remember that the
can be tested with various modalities (usually light tongue always deviates toward the side of the lesion!
touch and pinprick) in the three distributions of the
trigeminal nerve with a side-to-side comparison.
Cranial nerve VII: Children usually enjoy this 9.2.3╇Motor
portion of the exam because they get to make “funny
faces.” In an apprehensive child, this can be a good The motor examination encompasses four major
way to begin the examination. The key feature to components: bulk, abnormal movements, tone, and
focus on is symmetry: smiling, crying, raising of the strength (with the mnemonic BATS). In the motor
eyebrows. examination, you are looking for patterns suggesting
Cranial nerve VIII: Rubbing the fingers together a specific localization of disease.
next to each ear screens for asymmetries in hearing. While assessing muscle bulk, you should pay
attention to asymmetries, which may suggest a
subtle hemiparesis. A child with increased muscle
bulk in the calves may have muscular dystrophy.
Aligning the thumbs or great toes near each other and
seeing whether one is bigger or smaller can suggest
an asymmetry in bulk and a hemiparesis. Tone
is a measure of the degree of resistance to passive
movement. In the arms and legs, grasping the wrist
or ankle and gently shaking it back and forth can
measure tone. This maneuver can be repeated gently
at each joint. Tone can provide a clue to the timing
of injury in the central nervous system. Hypotonia
usually indicates an acute injury (such as in spinal
cord shock), whereas hypertonia is typically seen
in a chronic insult. An exception to this occurs with
injury to the cerebellum. In this case, the patient may
remain hypotonic long-term.
When assessing muscle strength, the pattern of
weakness can offer clues to etiology. Proximal muscle
weakness suggests a myopathy, whereas distal mus-
cle weakness suggests a neuropathy. Muscle strength,
graded on a scale from 0 to 5, can be difficult to test
Fig. 9.1â•… Sun-setting eyes. Downward deviation of the eyes formally in the uncooperative child (Table 9.1).
(“setting-sun sign”) secondary to an upward gaze paresis, seen Offering a toy and noting the resistance to your
in patients with increased intracranial pressure. pull tests a child’s distal strength. Asking a child to
98 Section IIâ•… Neurology
Table 9.1â•… Grading of muscle strength Table 9.2â•… Grading of reflexes
0 No muscle movement 0 Absent reflex

1 Minimal movement (a flicker) 1 Hyporeflexia

2 Movement with gravity eliminated 2 Normal

3 Movement against gravity 3 Hyperreflexia, no clonus

4 Able to resist with partial strength 4 Clonus present

5 Able to resist with full strength

9.2.5╇Sensation
stand from a supine position on the floor with the
Just as in adults, a thorough sensory examination
arms folded across the chest is a test of proximal
relies heavily on the ability of the patient to report
muscle strength. If the child uses the hands to “walk
differences in various sensory stimuli. In young chil-
up him- or herself,” this is an indication of proximal
dren, the power of tickling (and sometimes pinching!)
muscle weakness (Gowers maneuver). In a lethargic
the feet and hands can provide at least a crude assess-
or comatose patient, a leg that is laterally deviated
ment of sensation. The sensory examination tests the
can suggest weakness or hemiparesis (Fig. 9.2).
primary sensory modalities and cortical sensation.
Throughout the examination, note should be
Two major ascending pathways carry the primary
made of any involuntary or abnormal movements
sensory modalities: the dorsal column–medial
that the patient exhibits. These can range from
lemniscus system (proprioception or joint position
motor tics to sterotypies, such as hand flapping, to
and vibration tested with 128-Hz tuning fork) and the
choreoathetotic movements. The presence of muscle
anterolateral system (pain, temperature, crude touch).
fasciculations is a lower motor neuron sign.
To test each pathway, one sensory component specific
to that pathway may be chosen (i.e., pain and vibration).
9.2.4╇ Reflexes Detection of sensory level using a pin can be important
in suspected spinal cord pathology. Compare the same
Assessment of reflexes often provides the key clue to area side to side (Does this feel the same on both sides?)
localization. Hyperreflexia indicates a process affect- and move from affected to normal regions.
ing the central nervous system, whereas the presence The Romberg test is a test of proprioception.
of hyporeflexia suggests a nerve or muscle prob- The child is first asked to stand with feet together
lem. Hyperreflexia can be accompanied by clonus or and eyes open. When balance is achieved, the child
spread of the reflex (e.g., crossed adductor sign). The is asked to close the eyes and maintain balance.
presence of a mixed hyper/hyporeflexia can suggest a Removing the visual stimuli requires the child to rely
lesion in the spinal cord or a process affecting both the on proprioception (and to some degree vestibular
central and peripheral nervous system. Localization to sensation) to maintain balance.
the spine is also suggested in a child with hyporeflexia Cortical sensation (integrated sensory modalities)
but bilateral upgoing toes (Babinski sign) (Table 9.2). reflects the integration of the primary sensory
modalities, typically a function of the parietal lobe.
Stereognosis (identification of objects placed in
the hand while the eyes are closed), graphesthesia
(identification of letters or numbers drawn on the
palm), and detection of double simultaneous stimuli
are all examples of tests of cortical sensation. Ability
to perform these tests is age dependent; often they
can be done only in the older child or adolescent.

9.2.6╇Coordination
Coordination testing includes finger-to-nose and heel-
to-shin maneuvers as well as tests of rapid alternating
movements. To help engage young children, use a toy
Fig. 9.2â•… Hemiparesis. In an obtunded or comatose patient, or a light and ask the child to reach and touch it. Until
position of the lower extremities with lateral deviation of the preschool age, it is common for children to exhibit
leg can suggest a hemiparesis.
9â•… Neurologic Examination of the Child and Adolescent 99
mirror movements of the opposite hand (synkinesis).
The location of ataxia follows the anatomy of the cer-
Tricks of the Trade: Neurologic Exam
ebellum: truncal and/or gait ataxia suggests a lesion Pearls
involving the cerebellar vermis, whereas appendicular • Observation! Much can be learned with close
ataxia (predominantly involving the limbs) localizes observation from the moment you walk into
to the cerebellar hemispheres. the room!
• Don’t forget the general physical examination.
9.2.7╇Gait This includes head circumference in all your
patients
The gait examination begins with observation of the • The fundus should be examined in all patients.
normal gait, with attention to arm swing, base of • The motor exam is BATS: bulk, abnormal move-
gait, asymmetries in movement, and length of stride. ments, tone, and strength.
The child should be asked to walk on the toes and • A laterally deviated leg in a comatose or lethar-
heels as well as tandem walk (heel-toe)—a maneuver gic patient suggests a hemiparesis.
that cannot be performed well until around 8 years
of age. A wide-based gait suggests a problem with • Handedness develops at around age 18 months.
balance or ataxia. A “waddling” gait with excessive Always ask parents whether they feel their
lumbar lordosis suggests proximal weakness. In the baby is right or left handed. Early onset hand-
reluctant child, strategic positioning of a parent or edness suggests a hemiparesis.
loved toy can encourage the child to walk or run for • All children should be asked to rise from the
the examination. floor to enable observation of proximal muscle
weakness.

9.3╇Conclusion References
╇1. Griesemer D. The neurologic examination. In: Maria BL,
With practice, any clinician can gain comfort with ed. Current Management in Child Neurology. Hamilton,
performing a complete neurologic examination in the Ontario, Canada: BC Decker; 2005: 14–21
pediatric population. Finessing the powers of obser- ╇2. Swaiman KF. Neurologic examination of the older child.
vation, having a friendly demeanor, and approaching In: Swaiman KF, Ashwal S, Ferriero DM, eds. Swaiman’s
the child at the child’s developmental level will help Pediatric Neurology: Principles and Practice. 4th ed.
maximize the information that can be gathered. Philadelphia, PA: Elsevier; 2006: 17
Section III
Congenital Malformations
Section Editor: Concezio Di Rocco

Identification of the most appropriate management more accurate neuroimaging—for example, the Chi-
of congenital malformations of the brain, spinal cord, ari type I malformation, which is still the subject of
and their envelopes has been the main justification scientific debate. At the same time, the management
for the field of pediatric neurosurgery to emerge of known malformations, such as the Chiari type II
from general neurosurgery. Indeed, it was obvious malformation, is currently carried out according to
early on that an understanding of the physiopathoge- objective criteria rather than subjective interpreta-
netic mechanisms underlying these complex malfor- tion. Moreover, specific preventive measures for this
mations, the effects of which may extend throughout malformation have now been developed, namely, the
life, required the expertise and full engagement of prenatal management of myelomeningocele.
dedicated neurosurgeons. This section of the book The progress achieved by general neurosurgery in
demonstrates the impressive progress that has been the surgical correction of spinal and craniocervical
achieved in the field. junction congenital anomalies has been introduced
It took almost a century for the craniosynostoses in pediatric neurosurgery with excellent results, as
to be recognized on the basis of molecular criteria is demonstrated by several chapters in this section.
rather than merely morphological features. Such an Additionally, the descriptions of the congenital mal-
understanding has had a significant impact, not only formations of the spine and spinal cord that have
on the clinical definition of the individual nosologic been used since the early development of pediat-
entities, but also on the timing and method of their ric neurosurgery have now become more focused,
surgical management, as well as on the possibility of which has aided the accurate delineation of different
predicting specific outcomes for the different types. dysraphic states, each of which requires individual-
Further advances in craniosynostosis correction ized understanding and management.
include the recent introduction of techniques devel- In summary, this section, devoted to congenital
oped in other fields of surgery, such as osteodistrac- malformations of the CNS and its coverings, exem-
tion and the use of the endoscope. plifies the impressive improvements, both theoreti-
Nowadays, cerebral malformations and their more cal and surgical, that have resulted from the strong
common clinical manifestations, namely, seizure dedication of pediatric neurosurgeons in the care of
disorders, are dealt with during the first months or congenitally malformed infants and children. The
years of life. An effect of this shift has been a dramatic section is also a vivid demonstration of how the
improvement in functional prognosis. Also, relatively management of children born with these malforma-
“new” neurosurgical problems have emerged due to tions continues to be a stimulating field of research.
Section III.A
Malformations of the Scalp and Skull
10 Congenital Defects of the Scalp
and Skull
Daniel James Guillaume

10.1╇ Introduction and Background include plain radiographs, computed tomography


(CT), magnetic resonance imaging (MRI), and ultra-
sound.1 Many of the lesions can be managed without
10.1.1╇Indications surgery. Some indications for surgical intervention
include: the need for definitive diagnosis, pain, inter-
Congenital malformations of the scalp and skull are
ference with grooming, impending rupture or risk of
encountered regularly in a pediatric neurosurgical
infection, and risk of bleeding or air embolus.2
practice. The differential diagnosis for such lesions is
quite large and includes aplasia cutis congenita (ACC)
(Fig. 10.1a) and dermoid and epidermoid tumors 10.1.2╇Goals
(Fig. 10.1b), the Langerhans cell histiocytosis spec-
trum of disorders, occult meningocele or encepha- The goals of surgery depend on the type of lesion, loca-
locele, hemangioma, fibrous dysplasia, osteoma, tion, and presence of symptoms. In general, goals are:
lipoma (Fig. 10.2a–c), plexiform neurofibroma,
growing skull fracture, and malignant tumors.1 In • To establish diagnosis if this cannot be made
the majority of cases the lesion will be benign. Diag- based on history, exam, and imaging
nostic workup and management include a complete • To repair the cranial defect
and detailed history and exam, and imaging that may

a b c

Fig. 10.1â•… This 4-month-old girl was found to have three scalp lesions. (a) Near the vertex were two 6-mm, waxy, round, flat pap-
ules with translucent cigarette paper–like membranes through which several hairs could be seen (arrows), consistent with aplasia
cutic congenita (ACC). (b) Anterior to these was a 1-cm flesh-colored firm nontender midline nodule (arrow). Both hands and feet
demonstrated digital dysplasia with apparent constrictive bands around fingers and syndactyly of several fingers and toes, presum-
ably from amniotic band syndrome. Workup included cranial ultrasound, magnetic resonance imaging (MRI), X-ray imaging of
hands and feet, and karyotype studies, all of which were normal except (c) MRI, which demonstrated a cystic lesion. To evaluate for
Adams Oliver syndrome, given the cranial and limb abnormalities, a transthoracic echocardiogram was done, which was normal. All
three lesions were removed. The posterior lesions were consistent with ACC, while the anterior lesion was a dermoid cyst (arrow).

105
106 Section III.Aâ•… Malformations of the Scalp and Skull

a b c

Fig. 10.2â•… (a) This 12-month-old girl was noted to have a scalp mass shortly after birth. Exam showed a 4 × 2.5-cm soft tissue mass
slightly eccentric to the right along the vertex of the scalp that was hairless only over the lesion (arrows), moved with the underly-
ing scalp tissue, and did not increase with Valsalva or position changes. (b) The lesion was mildly homogeneously hyperechoic on
ultrasound and identical to fat on MRI, being hyperintense on T1 (arrow) and (c) hypointense on fat-saturation (arrow) MR images,
consistent with lipoma.

• To decrease the risk of injury to underlying 10.1.5╇Contraindications


brain and vascular structures, including the
sagittal sinus Some relative contraindications to surgical manage-
• To decrease the risk of cerebrospinal fluid ment of malformations of the skull and scalp include
(CSF) leak medical comorbidities that can increase risk of infec-
• To improve the cosmetic appearance tion or bleeding or that can lead to other complica-
tions. Selected lesions, such as ACC of the scalp, can
be effectively treated nonoperatively and will often
10.1.3╇ Alternate Procedures heal spontaneously with frequent changes of saline-
and antibiotic-soaked dressings.
Some congenital skull and scalp malformations,
including cephalohematoma and ACC, can be man-
aged nonsurgically.3,4 On rare occasions, certain scalp
lesions are best managed with chemotherapy or 10.2╇ Operative Detail and
radiation. For example, some cases of Langerhans cell Preparation
histiocytosis require chemotherapy and/or low-dose
radiation therapy, and radiation has been utilized for 10.2.1╇ Preoperative Planning and
treatment of surgically inaccessible hemangiomas, Special Equipment
but such cases are infrequent.5
Preoperative planning includes a carefully obtained
history and full physical exam. This usually comprises
10.1.4╇Advantages a full inspection of the scalp, midline back, and skin.
A detailed full-body exam may demonstrate asso-
The advantages of surgical excision of skull and
ciated findings that can require consultation with
scalp lesions include the ability to obtain a definitive
dermatology, genetics, or other providers. Appropri-
diagnosis, improvement of cosmetic deformity, and
ate imaging studies can aid in diagnosis. Plain radio-
no need to continue following the patient in clinic
graphs may demonstrate calvarial lucencies. The
with occasional imaging studies. Indeed, excision
presence of multiple defects (with the exception of
of a lesion can be more cost-effective, efficient, and
venous lakes) is suggestive of malignancy.1 Expan-
definitive than following a patient in clinic over sev-
sion of the diploë with bulging of one or both tables
eral years with serial imaging.
10â•… Congenital Defects of the Scalp and Skull 107
suggests a benign lesion, whereas full-thickness There are operative nuances associated with par-
lesions affecting both tables can raise concern for ticular lesions, as follows:
malignancy. The presence of peripheral sclerosis or Epidermoid and dermoid tumors are typically
peripheral vascular channels suggests benign lesion. treated with excision and curettage of bone margins.
Hemangiomas classically show a honeycomb, tra- One should search for a tract leading to the intra-
becular, or sunburst pattern, while fibrous dysplasia cranial cavity; if found, this must followed to obtain
can show well-defined islands of bone or a grossly excision of the complete lesion. If the lesion is adja-
mottled appearance. Epidermoid lesions typically cent to the sinus, the surgeon should be prepared for
have a sclerotic edge (Fig. 10.1b).1 dural sinus repair, although sinus damage would be
extremely rare.
Eosinophilic granulomas can spontaneously
10.2.2╇ Expert Suggestions and regress, but most are treated with curettage. Mul-
Comments tiple lesions are treated with chemotherapy and/or
low-dose radiation.5 Grossly, these lesions are pink-
Much information can be obtained from the physi- gray to purple, protruding from the bone, involving
cal exam, which should be systematic in a well-lit pericranium and sometimes dura, but dural penetra-
room. Note the overall appearance and color of the tion is rare. Patients presenting with eosinophilic
lesion and presence or absence of hair. Measure each granuloma should be seen by oncology, as up to 31%
lesion. Palpate to note texture and consistency (firm, develop additional lesions.5
rubbery, fluctuant, soft) and feel how far the lesion In the case of an occult cephalocele, the fibro-
extends below the skin surface. Palpate bony changes vascular stalk should be circumferentially dis-
surrounding the lesion, such as raised or scalloped sected and transected flush with the dura.2 The skull
borders. Feel whether the lesion is attached to the defect beneath the lesion is usually small and does
epidermis, dermis, or bone or is freely movable. Note not require cranioplasty; however, scalp tissue can
changes to skin surrounding the lesion. An ultra- occasionally scar to the underlying dura, causing
sound can easily be obtained without sedation on a pain. A rotational pericranial pedicle flap can add
clinic day and can give valuable information, includ- an additional barrier. For larger skull defects, a split
ing fluid within lesion, vascular flow, and sometimes calvarial graft can be harvested from adjacent skull
involvement of underlying intracranial space. It is and placed into the defect.2 Other surgical treatment
important to take an extra few minutes to perform options include split-thickness or full-thickness skin
a full-body exam, including a careful review of the graft, scalp rotation flaps, pericranial flaps, rib grafts,
skin and midline back, giving consideration to pos- latissimus dorsi muscle flap, and tissue expansion.3
sible associated anomalies (see Fig. 10.1).

10.2.4╇ Hazards, Risks, and Avoidance of


10.2.3╇ Key Steps of the Procedure and Pitfalls
Operative Nuances
Risks include infection, CSF leak, and vascular injury.
For lesions in which there is concern for malignancy, These can often be avoided by careful planning, vigi-
a biopsy may be indicated. In general, the goals of lant interpretation of preoperative imaging studies,
surgery are as stated earlier. and meticulous surgical technique. After excision of
The vast majority of lesions can be localized with large eosinophilic granulomas with extensive calvar-
palpation alone, but for small nonpalpable lesions, ial involvement, there can be an extensive inflamma-
image guidance can be used to keep the scalp scar tory reaction. In some cases, the bone defect repair
small. Most lesions can be removed with an elliptical should be delayed following lesion excision to allow
or semicircular incision. The scalp incision should be the inflammatory reaction to settle down.
large enough to expose the entire defect. The adjacent
scalp should be widely undermined in the subgaleal
plane to allow approximation of the edges without 10.2.5╇ Salvage and Rescue
tension following excision of the lesion. The skull
defect can be dissected by incising the pericranium Surgical excision of most skull and scalp lesions
along the bony edges down to bone, with curettage is relatively low-risk. In the rare event that dam-
of the bony edges. For midline lesions, one should be age to the sagittal sinus occurs, the sinus should
cautious about vascular attachments to the sagittal be repaired during the procedure, and the patient
sinus. Larger defects may require a rotation flap to be should undergo appropriate vascular imaging imme-
made with assistance from a plastic surgeon. diately following the procedure.
108 Section III.Aâ•… Malformations of the Scalp and Skull

10.3╇ Outcomes and Postoperative References


Course ╇1. Willatt JMG, Quaghebeur G. Calvarial masses of infants
and children. A radiological approach. Clin Radiol
10.3.1╇ Postoperative Considerations 2004;59(6):474–486
╇2. Piatt JH. Congenital defects of the scalp and skull. In:
Postoperative care is routine, and most patients can Albright AL, Pollack IF, Adelson PD, eds. Principles and
be sent home the same day as surgery. Infants should Practice of Pediatric Neurosurgery, 2nd ed. New York,
NY: Thieme Medical Publishers; 2008: 254–264
avoid lying directly on the incision until adequate
healing has occurred. ╇3. Santos de Oliveira R, Barros Jucá CE, Lopes Lins-Ne-
to A, Aparecida do Carmo Rego M, Farina J, Mach-
ado HR. Aplasia cutis congenita of the scalp: is
there a better treatment strategy? Childs Nerv Syst
2006;22(9):1072–1079
10.4╇Complications ╇4. Burkhead A, Poindexter G, Morrell DS. A case of exten-
sive aplasia cutis congenita with underlying skull defect
Complications that can occur following excision of and central nervous system malformation: discussion
calvarial or scalp lesions include infection, CSF leak, of large skin defects, complications, treatment and out-
and injury to underlying sagittal sinus for midline come. J Perinatol 2009;29(8):582–584
lesions. ╇5. Rawlings CE III, Wilkins RH. Solitary eosinophilic gran-
uloma of the skull. Neurosurgery 1984;15(2):155–161
11 Deformational Plagiocephaly
Mark R. Proctor

11.1╇ Introduction and Background


Intentional skull molding has long been practiced
around the world as a ritual in certain cultures. We
are currently in an era where we have introduced
unintentional skull molding as a consequence of a
program to protect babies. Sudden infant death syn-
drome, or SIDS, is a somewhat poorly understood but
tragic condition in which seemingly healthy infants
die in their sleep. It became recognized that prone
sleeping seems to predispose children to this con-
dition, which led to the introduction of the Back to
Sleep program in 1992 by the American Academy
of Pediatrics (AAP). In exchange for the reduction in
SIDS, we have created an epidemic of occipital skull
flattening.1 Certainly, in balance, exchanging a fatal
condition for a primarily cosmetic condition is an
excellent trade-off, but for pediatric neurosurgeons
and craniofacial surgeons, this has introduced a new
disease process that essentially never before existed.
The goal of this chapter is to explain my method of
diagnosing the condition, establishing when there is
a need for treatment, and reviewing the treatment
options and results for this relatively benign but
Fig. 11.1â•… Classic head shape associated with deformational
high-frequency condition. I would stress that this is plagiocephaly, with ipsilateral advancement of the occiput,
primarily a conservative treatment paradigm, and ear, and forehead.
there is no focus on the surgical correction of the
disorder, which is almost never indicated.

whom the clinical distinction between deformation


and craniosynostosis may be difficult, but routine
11.2╇Diagnosis radiology exams should not be considered. Indeed,
when I am asked by a pediatrician whether or not to
The vast majority of cases of deformational plagio- obtain X-ray images or a computed tomography (CT)
cephaly are easily diagnosed by clinical exam based scan before sending the patient in for evaluation, the
on the ipsilateral advancement of the occiput, ear, answer is unequivocally negative. I think it is more
and forehead when the infant’s head is viewed from economical, and lower risk, for the child to be sent
above (Fig. 11.1). Radiographic confirmation of the directly to a specialist if the pediatrician is unsure,
diagnosis should very rarely be indicated and cer- and most specialists should be able to establish the
tainly should not be part of routine clinical prac- diagnosis correctly at least 90% of the time without
tice. There will be a small percentage of patients in radiographs.

109
110 Section III.Aâ•… Malformations of the Scalp and Skull

11.3╇ Indications for Treatment


The indications for treatment of deformational pla-
giocephaly are one of the more controversial and
poorly understood aspects of the condition. Most
craniofacial experts believe that it is primarily a cos-
metic condition but the relationship to developmen-
tal delay is poorly understood. It is quite clear that
the conditions rose to epidemic proportions within a
few years after the Back to Sleep program was insti-
tuted in 1992. It is quite possible that supine sleeping
in and of itself has altered developmental milestones
to some extent, as babies often prefer supine posi-
tioning and have less reason to move about and
explore their environment when their faces are
pointing upward and they can freely look around.2
It is also fairly clear that children with developmen-
tal delay who move about less spontaneously are at
higher risk for this condition. However, it is far less
clear that the condition causes developmental delay.3 Fig. 11.2â•… Technique for measuring the cranial vault asymme-
I stress that my indications for treatment rely heav- try using calipers.
ily on the cosmetic issues and theoretical concerns
about functional issues related to the head shape
itself, such as difficulty in fitting for protective head- specific nuance here is that younger children have
wear when the children get older. In addition, con- more time to improve, and I would therefore be more
cerns about ear and jaw issues related to malposition tolerant of conservative therapy. For children with
are by and large not being borne out in clinical prac- brachycephaly, which is a symmetric flattening of
tice over time. the back of the head, the generally accepted criteria
One of the major questions to be answered is for helmet therapy would be a cephalic index (ratio
when the condition requires treatment. There is no of the width of the head divided by the length of the
simple answer. The algorithm is truly based on a head) of 0.93 or greater, whereas below 0.93, conser-
combination of age, response to conservative thera- vative therapies will likely be successful.
pies, and severity of disease. Even at a young age, a
child would be a candidate for repositioning therapy,
as well as physical therapy, if there is significant
muscular torticollis. In addition, instituting tummy 11.4╇ Treatment Options
time while the child is awake is always appropri-
ate, although it is not prudent to contradict the rec- There are several tiers of treatment for deformational
ommendation for supine sleeping, which has had a plagiocephaly. These include:
marked effect on the incidence of SIDS. The Back to • Repositioning
Sleep program has cut the rate of SIDS in half, albeit
• Sleep aids
from a low rate of 1 in 1,000 children to 1 in 2,000
children. In my practice, I will not institute helmet • Treatment of underlying muscular torticollis
therapy before 4 months of age. If I see a child before • Helmet therapy
then, I will establish baseline measurements and • Surgery
institute a program of conservative treatment, with
repositioning and possibly physical therapy if tor-
ticollis exists. I will then plan to see the child back 11.4.1╇Repositioning/Education
when he or she is at least 4 months old. I do find that
anthropometric measurements are extremely help- It should be stressed that deformational plagioceph-
ful, because it is impossible to recall the severity of aly is essentially a preventable condition. There is
the head shape subjectively when you are following no controversy regarding the fact that the condition
a large population of patients (Fig. 11.2). Consistent blossomed after the Back to Sleep program. Unfortu-
with data in the literature, for asymmetries less than nately, while primary care providers are well versed
approximately 9 mm, I would generally not institute in the need to reinforce supine treatment, they are
orthotic treatment. If the asymmetry is greater than much less well versed in the need for education on
9 mm, I would consider the use of the helmet. The how to prevent plagiocephaly. Recent data from a
11â•… Deformational Plagiocephaly 111
study in Canada indicate that the incidence of pla- underlying philosophy of that field that reinstitu-
giocephaly is even higher than originally suspected, tion of normal CSF flow will correct bodily ills, the
on the order of 46% of children.4 Although this may therapy will again be similar to the goals of physical
seem extreme, the fact that it is being recognized therapy. In summary, I don’t support the use of chi-
in the pediatric literature as a significant issue will ropractic or craniosacral therapy, but I don’t discour-
likely lead to a higher degree of education, and hope- age it if the families have pursued it.
fully a much lower incidence of the condition in the
future.
There are several easy repositioning techniques. 11.4.4╇ Helmet Therapy
When an infant is very young and still being swad-
dled, the parent can simply elevate one shoulder for There are fairly extensive data to support the fact
one night and the other shoulder for the next night, that helmets, also referred to as cranial orthoses,
or some similar way switching position after each are quite effective in treating deformational plagio-
nap, and so forth. As the child reaches 2 to 3 months cephaly. Few craniofacial experts would refute this,
of age, repositioning becomes more challenging. One but no doubt others would argue whether or not it is
simple trick is to move the things that the child likes more effective than the natural history of conserva-
to play with or look at to one side or the other, so that tive therapy and, frankly, whether or not the condi-
there is always something to keep the child’s interest. tion even requires treatment.5 I do advocate the use
Finally, tummy time while awake is very effective, of helmets for the more severe cases of deformational
both at keeping the child off the back of the head and plagiocephaly that are either not responding to con-
at encouraging strengthening of the neck muscles by servative therapies or occurring in children older
having the child raise the head and look around. than 6 to 7 months.6 Whereas I would never suggest
to a family that the helmet will lead to a more nor-
mal neurocognitive development of the child, it does
11.4.2╇ Sleep Aids quite consistently improve the morphology of the
head. Helmets are well tolerated and have extraordi-
The use of specialized pillows or devices in the crib narily low complication rates. Because of regulations
is generally not recommended by the AAP. The con- imposed by the Food and Drug Administration, the
cern is that anything soft could somehow impede cost of helmeting has skyrocketed; on average, the
the baby’s ability to breathe, thereby increasing the therapy will cost the family or their insurance com-
potential risk of SIDS. There is the possibility that pany $2,000 to $3,000. The average length of treat-
devices currently under development but not widely ment is 3 months. In my practice, family satisfaction
available might be acceptable sleep aids. However, with helmeting is very high, and the results are very
for the time being, repositioning without the use of reliable. We can generally expect a 50 to 90% reduc-
other devices is preferred, and I don’t encourage the tion in the degree of asymmetry (Fig. 11.3).
use of any particular sleep aid.

11.4.3╇ Treatment of Underlying


Muscular Torticollis
A high percentage of children with deformational
plagiocephaly will be found to have difficulty in
turning the head to the opposite side. Tightness of
the sternocleidomastoid muscle will give a classic tilt
to the side of the affected muscle and rotation to the
contralateral side, since the tight muscle is attaching
to the back of the head. A standard part of the evalu-
ation of any child should be assessing the rotation of
the head. If the muscle is clearly tight, then therapy,
either a home stretching exercise program or formal
physical therapy, should be considered. Some fami-
lies have seen, or wish to see, a chiropractor. In my
view this is acceptable, since the chiropractor really
would be simply treating the torticollis similarly to a
physical therapist. Similarly, some families ask about Fig. 11.3â•… Laser scan representation of the effects of helmet
craniosacral therapy. Whereas I do not support the therapy.
112 Section III.Aâ•… Malformations of the Scalp and Skull

11.4.5╇Surgery get older, which hides the deformity. Also, as a child


gets bigger, we are less likely to see him or her from
This particular section is quite brief, because in my a bird’s eye view, which is when the deformation is
view there is no significant indication for surgery in most noticeable. In addition, the head is 25% of the
this condition. As stated previously, there is no estab- baby’s mass, whereas it is only approximately 9% of
lished neurocognitive concern with deformational an adult’s mass, and visually there is far less focus on
plagiocephaly. In my view, there is little indication the head of an adult. Finally, the skull shape probably
for a major operation for a primarily cosmetic condi- does slowly improve over time in many individuals,
tion, and I have not done surgery for this condition. and the thickening of the bone and the thickening of
In some large series of deformational plagiocephaly, the scalp tend to soften any of the deformity. There-
a small percentage of patients have undergone sur- fore, it is visually less apparent, and some authors
gery, although this has never been my recommenda- have even suggested the incidence of deformation is
tion to a family. lower in older children than it is in adults.7
The distinction between deformational plagio-
cephaly and lambdoid synostosis should be dis-
cussed. In general, once you have seen a case of true
lambdoid synostosis, the distinctions are going to be 11.6╇Conclusion
very clear. The synostosis gives a classic windswept
appearance to the skull in the coronal plane, with the Deformational plagiocephaly is an essentially benign
skull shifting away from the side of fusion. There is condition that is an unintended consequence of the
generally significant elongation of the skull along the Back to Sleep program. The concerns are primarily
affected suture, with a prominent mastoid, inferior cosmetic, and I suggest that the condition be evalu-
displacement, and bossing. X-ray images are often ated in this context. Education of primary care phy-
unreliable at establishing the diagnosis, and a CT sicians and parents should go a long way toward
scan would be an essential step before consideration reducing the incidence, and in those children with
of surgery. significant issues, treatment options should include
repositioning, physical therapy, and helmets. Sur-
gery should almost never be considered.

11.5╇Results References
The results for this condition should be looked at from ╇1. Robinson S, Proctor M. Diagnosis and management
both cosmetic as well as functional perspectives. When of deformational plagiocephaly. J Neurosurg Pediatr
the condition first came to light in the 1990s, there was 2009;3(4):284–295
substantial concern that alterations in ear and jaw posi- ╇2. Hutchison BL, Stewart AW, Mitchell EA. Characteristics,
tion would lead to long-term functional consequences. head shape measurements and developmental delay
Largely, this is not been borne out by clinical practice. in 287 consecutive infants attending a plagiocephaly
Similarly, whereas there does seem to be a higher asso- clinic. Acta Paediatr 2009;98(9):1494–1499
ciation of developmental delay issues in children with ╇3. Collett BR, Gray KE, Starr JR, Heike CL, Cunningham
deformational plagiocephaly, no author has suggested ML, Speltz ML. Development at age 36 months in chil-
that the skull deformity is the cause of the neurocog- dren with deformational plagiocephaly. Pediatrics
nitive problems. It is easy to imagine that a child with 2013;131(1):e109–e115
developmental delay, who has delayed gross motor ╇4. Mawji A, Vollman AR, Hatfield J, McNeil DA, Sauvé R.
function, will have a higher incidence of skull defor- The incidence of positional plagiocephaly: a cohort
study. Pediatrics 2013;132(2):298–304
mity from lying supine on the soft infant skull. I never
counsel patients to pursue treatment because of neuro- ╇5. Mortenson P, Steinbok P, Smith D. Deformational plagio-
cephaly and orthotic treatment: indications and limita-
cognitive issues, and though I do discuss the functional
tions. Childs Nerv Syst 2012;28(9):1407–1412
consequences of ear and jaw asymmetry, I also stress
╇6. Kluba S, Kraut W, Reinert S, Krimmel M. What is the opti-
that it does not seem to be a long-term problem.
mal time to start helmet therapy in positional plagioceph-
From a cosmetic perspective, there is no doubt aly? Plast Reconstr Surg 2011;128(2):492–498
that the appearance issues are more prominent in
╇7. Roby BB, Finkelstein M, Tibesar RJ, Sidman JD. Preva-
a baby than in older individuals. Even if there is no lence of positional plagiocephaly in teens born after
objective improvement in the skull shape, four or the “Back to Sleep” campaign. Otolaryngol Head Neck
five factors make it less noticeable over time. One Surg 2012;146(5):823–828
factor is that most people have more hair as they
12 Nonsyndromic Synostosis: Overview
David H. Harter and David A. Staffenberg

12.1╇ Introduction and Background Indications

Surgery is the only intervention indicated for the


12.1.1╇ Definition, Pathophysiology, and correction of craniosynostosis or, in extreme, unusual
Epidemiology cases, of uncorrected deformational plagiocephaly.
The indication for surgical intervention for nonsyn-
Deformities of the cranium, including abnormal dromic craniosynostoses is largely based on projec-
shape and contour, have long attracted the atten- tions of the child’s appearance after completion of
tion of laypeople, shamans, and medical profession- craniofacial growth without treatment. Our under-
als. Premature closure of a cranial suture may cause standing of cranial vault and skull base develop-
a noticeable deformity of the cranial vault. Some of ment has advanced based on clinical experience and
these deformities are characterized by craniofacial on radiologic and basic laboratory investigations.
asymmetry. The estimated incidence of craniosynos- Most frequently, the decision for surgery is based
tosis is 1 in 2,000 births. The contemporary incidence on knowledge of the natural history of nontreated
may in fact be higher, as milder cases are identified individuals, parental preference, and input from the
and evaluated for treatment.1 surgical team, frequently neurosurgeons and plastic/
Nonsyndromic cranial synostoses are unique in craniofacial surgeons. We believe that an important
neurosurgical practice; the rationale for treatment component of the decision for surgery is the specific
often stems from aesthetic and social concerns, less team’s experience, results, and options—local expe-
frequently from the need to normalize intracranial rience, expertise, and expectations matter. Multiple
pressure (ICP) to preserve neurologic function. Sec- approaches (endoscopic, endoscope-assisted, sutu-
ondary synostosis consists of premature closure of rectomy, and more extensive procedures, such as
one or more sutures with an identifiable risk factor— cranial vault remodeling) are currently used, each
iatrogenic, metabolic, hematologic, pharmacologic, with acceptable safety and cosmetic results.
or structural. Genetic syndromes are identified in 5 Nonsyndromic single-suture synostosis has
to 15% of cases. Syndromic synostoses may involve been correlated with either elevated ICP or devel-
single or multiple sutures, including the skull base, opmental disorders in a small proportion of cases,
and are accompanied by identifiable genetic mark- although a considerable range has been reported.
ers and may include developmental central ner- Without unequivocal clinical data demonstrating
vous system (CNS), skeletal, facial, or other systemic elevated ICP—parenchymal or ventricular measure-
abnormalities. ments, lumbar puncture, or papilledema3—we do
In sporadic, nonsyndromic cases, the initial pre- not include it as a rationale for surgery. We do not
mature pathophysiologic suture closure or senes- emphasize a prophylactic role for surgery in the
cence is usually idiopathic. However, some associated “prevention of possible future developmental or
risk factors have been identified; twin and multiple neurological problems.”4 Although developmental
gestation, oligohydramnios, and intrauterine con- delay has been noted to occur in patients with syn-
striction are among the most commonly implicated. ostoses, no causal relationship has been confirmed;
Clomiphene citrate for infertility has also been asso- neither early nor late surgical intervention has been
ciated with an increased risk of synostosis.2 shown to improve these delays.5

113
114 Section III.Aâ•… Malformations of the Scalp and Skull

Clinical Presentation and Diagnosis who have navigated the choice of procedure, prep-
aration, recovery, and expectations is perhaps the
To understand the clinical presentation of nonsyn- most valuable resource of any craniofacial surgical
dromic synostosis, recognizing that cranial growth is team. We advocate for communication and connec-
driven directly by brain growth is helpful. Head shape tion with families who have been through the pro-
resulting from nonsyndromic single-suture synosto- cess as a component of preoperative decision making
sis depends upon the specific suture involved. The and counseling.
classic model for cranial growth, based on Virchow’s
model, is of growth restriction in the axis perpen- Sagittal Synostosis
dicular to the involved suture, with compensatory
growth in other directions. In practice, this model is Premature closure of the sagittal suture results in
often correct; however, variations may occur by the skull growth that is disproportionately in the ante-
addition of other variables, such as an accompanying rior–posterior dimension. Dolichocephaly (“long
deformational change due to intrauterine restriction head,” cephalic index 74.9 or less) or scaphocephaly
or postnatal factors, such as positioning. (“keel-shaped head”) are the traditionally used terms.
Most cases of nonsyndromic synostosis may be Among cases of sagittal synostosis, a spectrum of
diagnosed by historical and clinical findings. The ini- head shapes may be noted. This variability reflects
tial recognition of abnormal head shape may occur at the severity, timing, and extent of suture closure.
the time of birth, with less severe cases often incor- Findings are typically more notable at the occiput
rectly attributed to cranial molding due to delivery because of the closure of the suture from posterior
or intrauterine factors. The natural history of syn- to anterior. The initial shape seen is a “cupping” of
ostosis is typically a progressive deformity as brain the occiput that many refer to as an “occipital bullet.”
growth continues. A palpable ridge over the involved As synostosis progresses anteriorly, brain growth
suture is a common finding on exam, although not drives increasing cranial volume; frontal bossing and
universal. In our experience, the diagnosis of single- bitemporal pinching will be noted. In principle and
suture synostosis can be made accurately solely on practice, frontal bossing and bitemporal pinching are
clinical grounds. compensatory changes. Surgical correction under
1 year of age allows this compensation to correct
itself. Excepting late corrections, we rarely correct
Family Counseling the forehead during surgery, obviating an unneces-
sarily extensive operation. Closure of only the pos-
Consultation usually includes the pediatric neuro- terior portion of the suture usually results in severe
surgeon and plastic surgeon. Frequently, a pediatric narrowing of the parietal region, an occipital “bullet”
ophthalmologist is consulted to evaluate for papill- or “knob” and significant frontal bossing and widen-
edema. In cases with asymmetry (e.g., unilateral cor- ing, called “golf tee” deformity. Closure of the middle
onal or lambdoid), the ophthalmologist assesses and portion of the suture may result in a saddle-shaped
quantifies strabismus, an ocular cause for torticollis. deformity. In these variants, ridging is usually pal-
Undiagnosed strabismus can be a cause of continued pable over the affected portion of the suture.
head tilt. When patients present early, and surgery is feasi-
It is imperative that these cases be evaluated and ble before 3 months of age, we offer endoscopic strip
treated by an experienced craniofacial team work- craniectomy for milder cases and add barrel-staves
ing in the same location. While many craniofacial for moderate cases.6 However, we use this approach
teams believe in the importance of practicing in a only when parents accept compliance with postop-
multidisciplinary team, we emphasize the need for a erative cranial orthoses. In our synostosis patients
true transdisciplinary approach. Members of a trans- and our deformational plagiocephaly patients, we
disciplinary team work together and have a deep find that fewer parents are willing to accept helmets
understanding of each other’s perspective and skill and the social stigma that accompanies long-term
set. This is much more than a semantic difference. orthotic use. These families may favor surgery that
The value of institutional experience, development does not require a helmet postoperatively. For these
of processes, and long-term patient follow-up not patients, we perform a calvarial vault remodeling
only promotes better outcomes but also provides fer- (CVR), which consists of a suture trellis and resorb-
tile ground for education of students, residents, and able fixation (STAR-Fix). This technique has been
fellows, as well as the development of institutional used successfully with good long-term results.7 The
volume and memory. Resources on our craniofacial technique eliminates the occipital bullet and does
team include nurse practitioners, otolaryngologists, not leave any areas of dura unprotected by bone.
dieticians, social workers, speech therapists, psy- For patients who present later in childhood or in
chologists, geneticists, and craniofacial orthodon- their teens, the area of social concern is the forehead,
tists, as well as a full-time medical photographer on account of the bifrontal bossing and the bitempo-
with 2D and 3D capabilities. Support from parents ral pinching. The occipital bullet is more easily hid-
12â•… Nonsyndromic Synostosis: Overview 115
den. Because this dynamic is different from that in Unilateral Coronal Synostosis
the typical infant, we will perform a bilateral fronto-
orbital expansion (FOE). This can be combined with Unilateral coronal synostosis may cause severe facial
a strip craniectomy when additional growth may and calvarial differences. Differentiating coronal
lead to further deformity. In teens, cranial growth is craniosynostosis from deformational plagiocephaly
almost complete, so the suture may be left in place is critical, as the natural histories and treatment
and ridging can be simply burred flush. The FOE is options are opposite.
performed laterally in order to avoid the developing Since marked asymmetry is present, the prob-
frontal sinus. We have found that patients with mild lem is usually identified early by parents or caregiv-
deformities (those “too mild for surgery”) may go on ers. Physical findings include a palpable ridge over
to develop these late forehead changes, potentially the affected suture and flattening (plagiocephaly)
leading to severe psychosocial distress. They are fre- on the side of the synostotic suture. Contralateral
quently told at that point that there are no options frontal bossing may be seen. The nasal root is devi-
(“now it’s too late to do surgery”). ated toward the affected side; the ipsilateral superior
The diagnosis of complete or partial sagittal orbital rim is also skewed superiorly and laterally.
synostosis can usually be made with confidence on The ear is usually displaced anteriorly on the side of
clinical grounds alone. We do not recommend rou- the involved suture, thereby decreasing the distance
tine preoperative computed tomography (CT) scans.8 between the ear and the lateral canthus as compared
Plain radiographs or CT scans are obtained for atypi- with the other side.
cal cases or upon parental request. We prefer proto- We have used uni- and bilateral FOA for the treat-
cols for CT that limit radiation exposure. Magnetic ment of unilateral coronal synostosis9 and offer early
resonance imaging (MRI) is indicated for patients (<€3 months of age) endoscopic strip craniectomy with
with significant developmental disorders or struc- postoperative orthotic treatment. Design and fabrica-
tural brain abnormalities identified on CT. tion of the helmet, with careful follow-up, are critical.10

Metopic Synostosis Lambdoid Synostosis

Metopic synostosis may cause a spectrum of cranial Lambdoid synostosis usually results in a variety of
and facial abnormalities. The degree of deformity is findings; however, diagnosis on clinical grounds
likely related to the time and extent of suture clo- alone may be difficult, and therefore CT with three-
sure. Early, complete closure may cause severe defor- dimensional reconstruction may become necessary.
mity: hypotelorism, restriction or “pinching” at the Viewed anteriorly, the face may have a “windswept”
pterion, a palpable metopic ridge, and restricted appearance. A palpable ridge may be felt over the
frontal bone growth. This deformity is made worse involved lambdoid suture. The ear is usually dis-
as compensatory expansion occurs at the parietal placed posteriorly and inferiorly toward the closed
region bilaterally. This triangular head shape is suture. Significant contralateral parietal bossing is
described as “trigonocephaly.” It should be noted typical. The posterior asymmetry frequently leads
that trigonocephaly, scaphocephaly, dolichocephaly, to a head tilt. Physical therapy may be instituted to
plagiocephaly, brachycephaly, and similar descrip- eliminate this, but persistent asymmetry may cause
tions are physical findings and not diagnoses. These the tilt to persist.
findings may not be symmetric. Unfortunately, many Since the suboccipital skull base is asymmetric in
parents are told that these are “only cosmetic” prob- lambdoid synostosis, we believe that the reconstruc-
lems, and correction may not be pursued. We rec- tion must utilize a “bandeau” (the posterior analog
ognize that these disorders may cause significant to the bandeau used in the reconstruction of metopic
social problems and strongly advocate for surgical and coronal synostosis). With early diagnosis, open
treatment. The quality of the reconstruction will be or endoscopic strip craniectomy with postoperative
measured by most on cosmetic terms. orthotic treatment may be sufficient.
We believe that, while endoscopic suturectomy
for solitary metopic ridging (without any temporal Multisutural Synostosis
pinching) is feasible and sometimes requested by
parents, true trigonocephaly is best corrected with Multisutural synostosis is unusual and may cause
a fronto-orbital advancement and anterior calvarial significant, atypical deformities. Synostosis with
vault remodeling. Bitemporal pinching requires cre- superimposition of cranial deformation due to intra-
ation of a supraorbital bandeau that extends from uterine or extrauterine factors may also cause atypi-
the anterior aspect of the middle cranial fossa to the cal or unusual findings; supplemental imaging may
temporoparietal area. As is true for the other cra- be required to clarify the diagnosis and for treatment
niosynsostosis surgeries, resorbable hardware and/ planning. Depending upon the age at presentation,
or resorbable sutures are used, and metal wires or cranial vault remodeling or endoscopic approaches
titanium plates are avoided. are feasible and safe.11
116 Section III.Aâ•… Malformations of the Scalp and Skull

Goals and Advantages of Selected Surgical zag can then be closed as multiple sequential V-to-Y
Approaches advancements, facilitating closure. In contrast to many
adult exposures, we find that pediatric coronal flaps are
The goal of treatment is to improve cranial shape adequate when raised in a subperiosteal plane.
and to minimize facial differences, allowing for social The supraorbital nerves (the ophthalmic division of
integration and development. For cases presenting the trigeminal nerve) are most commonly found to pass
with papilledema or elevated ICP, expansion of the through a notch in the superior orbital rim and are eas-
cranial volume is necessary. A wide range of options ily preserved during the subperiosteal dissection of the
are available; recommendations account for patient coronal flap. In some cases, however, the supraorbital
age, presentation, available resources, and, above all, nerves will pass through a foramen above the superior
family preference and expectations. orbital rim. In order to preserve the nerves, the notch is
We advocate a balanced approach regarding the converted to a foramen with a small osteotome and the
decision for endoscopic versus open approaches nerve is delivered and protected.
based on multiple factors, including age, diagnosis, Hemostasis is carefully maintained throughout
and goals of treatment. We consider the ideal endo- the dissection with cautery, bone wax, and other
scopic case a young patient, less than 3 months of hemostatic agents (Gelfoam, thrombin, FlowSeal).
age, with sagittal synostosis. The thin bone, rapid The dura is carefully inspected for its integrity and
cranial growth, and simplicity of the required ortho- hemostasis during the procedure. Immediately after
sis (symmetric application of force along the antero- bone removal, a moist sponge is placed on the dura;
posterior [AP] axis), and excellent long-term results it is then peeled back, and small bleeding points are
support this approach. Other diagnoses (such as controlled with bipolar cautery. Unintended duroto-
unilateral coronal or lambdoid synostoses), when mies, unusual in nonsyndromic cases, are repaired
made early enough, may also lend themselves to primarily with 4–0 Nurolon (Ethicon, Somerville, NJ,
endoscopic strip craniectomy with postoperative USA) sutures. Bone that is removed is kept in saline
orthotic treatment; however, careful follow-up and during the procedure, while the surgical field is kept
skilled orthotic fabrication are needed. We have not wet as well to prevent tissue desiccation.
used, and do not advocate, endoscopic approaches Bone fixation is achieved with resorbable plates
for bilateral coronal synostosis, older patients (more placed in positions where structural rigidity is
than 3 to 4 months of age), or syndromic synostoses. required; resorbable sutures are used elsewhere.
We cover all bony defects with autologous bone har-
vested in the form of split calvarial bone grafts where
Surgical Considerations necessary. Rib grafts are very rarely required. When
the superior lateral orbital rim is advanced during
Communication with the operating room team is the surgery (metopic and coronal), the periorbita is
emphasized. A standardized checklist, including the carefully incised lateral to V1 in order to allow expan-
planned procedure, critical steps, needed equip- sion of the soft tissue over the advanced bone. Upon
ment, expected blood loss, potential for venous air closure, the soft tissue must be carefully redraped.
embolism, and other concerns, is reviewed. Surgery Rarely, pericranial or galeal scoring is required. We
is performed under general anesthesia with oro- do not use drains routinely. Galea and skin are closed
endotracheal intubation. Arterial line, Foley catheter, with resorbable sutures. A head dressing is placed
and two peripheral intravenous catheters are placed. and is kept in place for 48 hours. Although rare, che-
Tranexamic acid (TXA), 50 mg/kg loading dose, fol- mosis affecting greater than one-third of the palpe-
lowed by an infusion of 5 mg/kg/hr, is used in most bral fissure of the conjunctiva may require temporary
cases to decrease blood loss.12 We do not routinely tarsorrhaphies with resorbable sutures.
use diuretics for brain relaxation in nonsyndromic Bumps and irregularities are typically palpable
cases. For metopic and coronal synostosis (anterior postoperatively, especially about 10 months postop-
correction), the patient is placed in a comfortable eratively, as resorbable plates and pins begin to dis-
supine position in a well-padded pediatric horse- solve. We have not needed to remove these and have
shoe headrest. For sagittal and lambdoid synostosis found this process to be self-limited.
(posterior correction), the patient is placed in a com-
fortable prone position in a well-padded pediatric Deformational Plagiocephaly
horseshoe headrest, with care to avoid pressure on
the globes. Deformational changes in head shape, by definition,
The workhorse for surgical exposure in the repair of are due to extrinsic forces resulting in cranial mold-
craniosynostosis is the coronal incision. The design of ing. To minimize the risk of SIDS (sudden infant death
our incision has evolved over more than 15 years from syndrome) in the early 1990s, the American Academy
a common zigzag to a curvilinear design. The zigzag, of Pediatrics’ Back to Sleep campaign encouraged par-
however, may be useful for syndromic cases where the ents to keep their babies in a supine position in their
intracranial volume needs to be augmented. The zig- cribs. The malleability of the infant cranium is nec-
12â•… Nonsyndromic Synostosis: Overview 117
essary to navigate the birth canal, but it also makes tarsorrhaphy. Positioning must also allow for rapid
the infant cranium vulnerable to continued external access to the airway.
forces. In fact, this is understood and exploited in Venous obstruction is avoided by preventing
many cultures, where cranial molding is performed excessive neck flexion or direct neck compression
to produce a variety of head shapes that the cultures and reverse Trendelenberg position when needed.
find desirable. While these deformational changes
usually correct on their own, developmental delays,
hydrocephalus, torticollis, and cephalohematomas Timeout, Checklists, and Complication
may contribute to their persistence. It is, therefore, Management
imperative to recognize deformational changes early
and to differentiate them from craniosynostosis. Significant risks are present during every portion of sur-
Although craniosynostosis will likely require surgi- gery for cranial synostosis, from airway management,
cal correction in infancy, deformational plagioceph- positioning (hypertension, extremities, cervical spine),
aly rarely requires surgery. However, persistence of and fluid management to the removal and reconstruc-
deformational forces may prevent full correction, tion of the calvaria and facial bones. Anticipation of
and then surgery may be necessary. Differentiat- potential problems is the best way to avoid them.
ing abnormal head shapes without craniosynostosis
from abnormal head shapes with craniosynostosis
is crucial but occasionally difficult. How to diagnose Planning of Skin Incision
and treat deformational or positional plagiocephaly
is addressed elsewhere. A stealth or zigzag scalp incision can be fashioned for
Molding helmets may be applied to aid in the cosmesis.
correction of asymmetry, but, because of the stigma, We strive for minimal bone destruction by using
expense, and inconvenience, we use them in a burr holes just large enough to establish the epidural
minority of cases. Our indications for prescribing a plane and to allow introduction of the footplate
molding helmet include severe asymmetry (particu- attachment of the drill with the smallest avail-
larly facial), late presentation, failure of conservative able diameter bit (B5 on Midas Rex electric drill,
measures, and parental preference. Medtronic, Minneapolis, MN, USA). Whenever pos-
sible, we use the fontanelle as an entry point. Poten-
tial sites of unintended durotomy are at the region of
the cribriform plate, the anterior sagittal sinus, fora-
12.2╇ Operative Detail and men, and the dural fold around the sphenoid wing,
particularly in cases of coronal synostosis.
Preparation Significant hemorrhage is usually the result of
Operative planning—correct procedure for the unintended violation of a venous sinus. Potential sites
patient and surgeon; communication with the include the sagittal sinus, torcula, and transverse/sig-
operative team; expected procedure, length, comor- moid sinuses. Gentle tamponade and application of
bidities, potential problems—in surgery for cranial Gelfoam, thrombin, FloSeal, and/or Surgicel are usually
synostosis is truly a team effort. Careful explanation sufficient. Like geology, hemostasis is often a study of
of the rationale, expected outcomes, and possible time and pressure. Reverse Trendelenberg position may
risks of surgery is essential. Communication among also help, although care must be taken to avoid entrain-
the surgical team is paramount to ensuring good ment of air. Attempts at bipolar cautery may result in
outcomes and safe surgery. Initial explanation of the paradoxical enlargement of the defect and increased
planned procedure, positioning, expected duration, bleeding. Rarely, direct suture repair or suture applica-
potential blood loss, and surgical adjuncts prevents tion of a muscle plug or dural tack-up is needed.
unnecessary delays or prolongation of the procedure.
12.2.2╇ Salvage and Rescue
12.2.1╇ Expert Suggestions and Given the potential for unexpected blood loss, directed
Comment donor or other banked blood products are available
for immediate use and are called for as the patient
Key Steps of the Procedure and Operative enters the operating room. Adequate venous access
Nuances is ensured prior to initiation of the procedure, as is
an arterial line for continuous blood pressure moni-
Positioning must prevent compression or impinge- toring. An experienced pediatric anesthesiologist is
ment upon eyes, neck, and extremities. The corneas a critical team member, given the potential for rapid
are protected by use of tape, inserts, or temporary physiological changes.
118 Section III.Aâ•… Malformations of the Scalp and Skull

12.3╇ Outcomes and Postoperative References


Course ╇1. Selber J, Reid RR, Chike-Obi CJ, et al. The changing
epidemiologic spectrum of single-suture synostoses.
12.3.1╇ Postoperative Considerations Plast Reconstr Surg 2008;122(2):527–533
╇2. Ardalan M, Rafati A, Nejat F, Farazmand B, Majed M,
Immediate postoperative care focuses on the early El Khashab M. Risk factors associated with cranio-
detection and treatment of postoperative complica- synostosis: a case control study. Pediatr Neurosurg
2012;48(3):152–156
tions, hemorrhage at the operative site, airway prob-
lems, and pain. Our patients are transported directly ╇3. Vasco G, Baranello G, Ricci D, et al. Longitudinal assess-
ment of visual development in non-syndromic cranio-
from the operating theater to the pediatric intensive
synostosis: a 1-year pre- and post-surgical study. Arch
care unit. Serial hemoglobin is obtained until stable, Dis Child 2008;93(11):932–935
asymptomatic levels above 7 g/dL are tolerated. If
╇4. Da Costa AC, Walters I, Savarirayan R, Anderson VA,
persistent hemorrhage is suspected based on clinical Wrennall JA, Meara JG. Intellectual outcomes in
or laboratory data, a CT scan is obtained to rule out children and adolescents with syndromic and non-
a postoperative hematoma. Return to the operating syndromic craniosynostosis. Plast Reconstr Surg
room for delayed hemorrhage has been extremely 2006;118(1):175–181, discussion 182–183
rare in the authors’ combined experience. ╇5. Becker DB, Petersen JD, Kane AA, Cradock MM, Pil-
Some degree of swelling at the operative site gram TK, Marsh JL. Speech, cognitive, and behavioral
is inevitable and may be a source of consider- outcomes in nonsyndromic craniosynostosis. Plast Re-
able, understandable parental anxiety. For anterior constr Surg 2005;116(2):400–407
approaches, periorbital edema may result in tempo- ╇6. Jimenez DF, Barone CM. Endoscopic technique for sagit-
rary eye closure. Elevation of the head by bed posi- tal synostosis. Childs Nerv Syst 2012;28(9):1333–1339
tioning or use of a car seat may hasten resolution. ╇7. Goodrich JT, Tepper O, Staffenberg DA. Craniosynos-
Neurologic complications of surgery for nonsyn- tosis: posterior two-third cranial vault reconstruction
dromic craniosynostosis are exceptionally rare, as using bioresorbable plates and a PDS suture lattice
are general medical problems.13 in sagittal and lambdoid synostosis. Childs Nerv Syst
Delayed complications of surgery are most com- 2012;28(9):1399–1406
monly minor aesthetic concerns related to the inci- ╇8. Fearon JA, Singh DJ, Beals SP, Yu JC. The diagnosis and
sion, small osseous irregularities, and, less often, treatment of single-sutural synostoses: are computed
tomographic scans necessary? Plast Reconstr Surg
persistence or progression of the initial problem.14
2007;120(5):1327–1331
Scar spread and areas of alopecia are more common
╇9. Selber JC, Brooks C, Kurichi JE, Temmen T, Sonnad SS,
in cases that require tension for final closure. Most
Whitaker LA. Long-term results following fronto-orbital
are amenable to simple scar revision on an elective reconstruction in nonsyndromic unicoronal synostosis.
basis. Minor bony irregularities tend to improve Plast Reconstr Surg 2008;121(5):251e–260e
over time as calvarial growth proceeds and the scalp 10. Jimenez DF, Barone CM. Endoscopic technique for coro-
thickens. Exceptionally, a persistent bone defect may nal synostosis. Childs Nerv Syst 2012;28(9):1429–1432
require secondary repair with a graft; split-thickness 11. Jimenez DF, Barone CM. Multiple-suture nonsyndrom-
calvarial graft is usually sufficient. Persistent bony ic craniosynostosis: early and effective management
prominences may be simply burred down if they using endoscopic techniques. J Neurosurg Pediatr
cause aesthetic problems. 2010;5(3):223–231
Recurrence of the initial abnormal appearance may 12. Goobie SM, Meier PM, Pereira LM, et al. Efficacy of
also occur, fortunately uncommonly. In our experience, tranexamic acid in pediatric craniosynostosis surgery:
recurrence is usually due to inadequacy of the initial a double-blind, placebo-controlled trial. Anesthesiol-
surgical approach; for example, simple suturectomy ogy 2011;114(4):862–871
in an older patient with sagittal synostosis. For endo- 13. Goodrich JT. Craniofacial surgery: complica-
scopic cases, failure may relate to poor compliance tions and their prevention. Semin Pediatr Neurol
with, or poor fabrication of, the postoperative orthotic 2004;11(4):288–300
device. 14. Foster KA, Frim DM, McKinnon M. Recurrence of syn-
In summary, nonsyndromic craniosynostoses are ostosis following surgical repair of craniosynostosis.
gratifying to treat. With proper patient selection, Plast Reconstr Surg 2008;121(3):70e–76e
planning, and execution, complications are rare and
patient satisfaction is high. A variety of techniques—
“open,” purely endoscopic, and endoscopic-assisted—
are widely used and accepted. Choice of the optimal
procedure must account for the initial deformity,
patient age, and surgeon and parental preference.
13 Sagittal Synostosis Repair Surgery
D. Douglas Cochrane and Peter Albert Woerdeman

13.1╇ Introduction and Background • If needed, to neutralize the effect of raised


intracranial pressure if it is present or
Craniosynostosis is a condition in which one or develops subsequently
more of the fibrous sutures in an infant skull devel- Although there is evidence that developmental
ops abnormally or prematurely fuses by ossification, progression may differ in infants and children with
thereby changing the growth pattern and shape of sagittal synostosis, sagittal synostosis surgery does
the skull. Isolated, nonsyndromic sagittal synostosis not significantly change this trajectory.4–6
is the most common form of craniosynostosis and
is found in about 1 in 2,000 births. As a result, the
largest surgical experience has been accumulated in 13.1.2╇ Treatment Goals
addressing this dysmorphology.1,2 Although at least
20% of craniosynostosis cases are caused by specific The degree of skull dysmorphology varies among
single-gene mutations or chromosome abnormali- individual patients with sagittal synostosis. In addi-
ties, isolated nonsyndromic sagittal craniosynosto- tion to biparietal narrowing and dolichocephaly,
sis is not directly associated with gene mutations or frontal or occipital bossing commonly occurs. It is
chromosome abnormalities. At this time, molecular rare in isolated, nonsyndromic sagittal synostosis
genetic diagnosis is not routinely performed.3 to have both occipital and frontal bossing. The pri-
Multiple procedures have been described since the mary goal of surgical treatment is the normalization
original bilateral parasagittal craniectomies (1890, of appearance by enlarging the biparietal diameter
Marc-Lannelongue) and the pioneering work of Lane relative to the long axis of the head and ensuring
in 1892 (vertex craniectomy with biparietal osteoto- normal cranial volume. Due to the variation in shape
mies): from strip craniectomies to the more exten- among patients, the surgical intervention for indi-
sive, invasive, and complex surgeries, such as bilateral vidual patients should be tailored to the skull shape
parietal flap craniectomies, sagittal strip with circular and include maneuvers to normalize that shape.
occipital and parietal wedge craniectomies, and vari- After sagittal craniosynostosis repair surgery,
ous other types of calvarial vault remodeling. After patients are followed with cephalic morphometry
more than 100 years of craniosynostosis surgery and (head circumference, cephalic index, and scalp sur-
dozens of peer-reviewed papers on the subject, con- face measurements: ear-to-ear and nasion-to-inion
troversy still remains regarding the best procedure for distances) and funduscopy for early recognition of
the treatment of sagittal synostosis. possible secondary synostosis or craniostenosis.
In this chapter, we share our experience in the
surgical repair of sagittal synostosis.
13.1.3╇ Alternate Procedures
13.1.1╇ Indications for Surgical Because the natural course of head shape develop-
Intervention ment in sagittal synostosis will not normalize over
time, in contrast to nonsynostotic plagiocephaly, there
The indications for surgical repair of sagittal synos- are no well-studied alternatives to surgery (with or
tosis are twofold: without postoperative helmet treatment). Although
some calvarial contour normalization was found in a
• To normalize the head shape and therefore small series of very young unoperated patients after
the appearance the use of molding helmets,7 we do not think “external
119
120 Section III.Aâ•… Malformations of the Scalp and Skull
molding” without surgical intervention should be con- we recommend a minimal vertex craniectomy (uti-
sidered as a first-line treatment at this time. lizing the endoscope if necessary), often augmented
with small anterior and posterior biparietal wedge
craniectomies, followed by helmeting to define an
optimal calvarial shape.
13.2╇Advantages In the older infant (> 8 months of age) with iso-
lated sagittal synostosis, more radical reshaping is
Since normalization of skull shape is the treatment goal, recommended and is achieved with an open vertex
most patients will attain an acceptable head shape (not craniectomy, including releasing biparietal wedge
recognized as abnormal in public) regardless of the craniectomies with or without bilateral barrel oste-
surgical maneuvers performed. Postoperatively, head otomies and with or without an occipital remodeling
shape does show a tendency to drift minimally toward craniotomy. We do not do staged or “front-and-back”
the original dolichocephalic shape.8 However, this does procedures because forehead reshaping occurs over
not result in a degree of abnormality in shape detect- time, with or without helmeting, as the result of cra-
able to the untrained or unaware eye. niofacial growth.
There is no clear advantage of one surgical Basic craniotomy instrumentation and powered
approach over another with respect to eventual cos- tools for burr holes and osteotomies are all that are
metic outcome or surgical complications or on risk of needed. A head lamp, surgical loupes, and suction
infection, dural and brain injury, or poor bone refor- cautery facilitate adequate vision for simple ver-
mation. Since the needs of patients are individually tex craniectomy done through anterior and poste-
specific, there is no single right, optimal procedure rior coronal incisions. If endoscopes are to be used
for all. Often, the surgeon’s repertoire of procedures to assist in the extradural dissection, we have used
is largely based on training and philosophy, and they a 4-mm 30° endoscope (Karl Storz, Tuttlingen, Ger-
may range from simple suturectomy with or without many). In minimally invasive procedures, bone is cut
postoperative molding with a helmet to complete with straight or curved Mayo scissors, and Goldman
calvarial remodeling. The frequency of complica- septum scissors are used for thicker bone.
tions, the need for transfusion, and the time to recov-
ery do differ among the various surgical techniques.
The more radical the operation, the more time con- 13.3.2╇ Expert Suggestions and
suming, the more likely the need for transfusion, and Comments
the longer the hospital stay.9
The operation type and techniques used to normal-
ize head shape are based on the patients’ specific
13.2.1╇Contraindications skull shape and age.
There are only relative contraindications to surgical
intervention. If patients are medically unstable, elec- 13.3.3╇ Key Steps of the Procedure and
tive sagittal synostosis repair surgery should not be
performed at that time. Furthermore, if blood trans-
Operative Nuances
fusion is refused, we offer simple craniectomy (with
1. Following anesthetic induction and
or without biparietal wedge craniectomies) and hel-
intubation, one or two IVs and an arterial line
meting rather than more extensive operations. In
are placed; no urinary catheter, central line,
patients with metabolic bone disease, poorer results
or precordial monitoring is used. Prophylactic
for bone regeneration should be anticipated. There-
antibiotics are given.
fore, one might judge metabolic bone disease a rela-
tive contraindication. 2. Positioning should be as simple as possible. For
a minimal vertex craniectomy, the patient is
positioned either lateral on a padded beanbag
(Fig. 13.1a–c), in the “sphinx” position
13.3╇ Operative Detail and (Fig.€13.1d–f), or prone in a padded horseshoe
headrest in a near-neutral position (Fig. 13.1g,h).
Preparation The lateral decubitus position is less complex
from an anesthetic perspective. In older infants,
13.3.1╇ Preoperative Planning and or when a more radical open vertex craniectomy
Special Equipment is planned, the prone position on a horseshoe
headrest is always chosen.
The decision on the operative technique to be
employed is largely based on the age of the infant. The patient is secured to the table with “bum
In the younger infant with simple sagittal synostosis, and chest” straps, and the head is fixed to the head-
13â•… Sagittal Synostosis Repair Surgery 121
rest with an adhesive U-drape. This prevents fluids prep in the hair is absorbed with sterile towels
from running onto the face during the operation and prior to draping.
avoids pressure and moisture skin breakdown. This 4. Scalp incision (Fig. 13.1): For minimal vertex
technique also stabilizes the head to the headrest. craniectomy, two 5– to 6-cm-long coronal
Eyes, forehead, maxillae, and body pressure points are incisions are made: one just posterior to the
checked to ensure no local pressure,10 and the stabil- anterior fontanelle and one just in front of the
ity of the patient on the table is checked by putting posterior fontanelle. Alternatively, a wider
the table through any movements that will be neces- coronal incision at the midpoint of the sagittal
sary during the operation, prior to draping. Should the suture or a “lazy S”-shaped midline incision
patient shift on the table, adjustments in the securing over the sagittal suture can be used.
straps are made and the position retested. For an open vertex craniectomy, a bicoronal
3. Prepping and draping: After minimal hair zigzag incision is made from behind the ear,
clipping, we do a double 2% chlorhexidine crossing the midline in the anterior third of
gluconate solution skin prep, carefully the sagittal suture, and to behind the other
avoiding any dripping of prep solution under ear. The lateral zigzags are curved at least
the U-drape onto the skin and eyes. The last twice the width of the eventual scar, and the
prep is allowed to dry, and any excess liquid incision comes straight over the vertex.

a d g

b e

c f

Fig. 13.1â•… (a) Positioning of the head in sagittal craniosynostosis repair surgery. (b) The lateral head position, with the side of the
head resting on a doughnut-shaped gel pad. (c) The head is fixed with adhesive U-drape before sterile draping. (d) A lateral view of
the “sphinx” position. (e) The head is placed on a gel-padded beanbag or Doro headrest (PMI, Frieburg, Germany) and fixed with
adhesive U-drape. (f) Frontal view of the “sphinx” position after sterile draping. (g) The prone position with the forehead resting on
a padded horseshoe head holder before draping. (h) After draping.
122 Section III.Aâ•… Malformations of the Scalp and Skull
After local anesthesia (0.25% xylocaine and a
1/400,000 adrenaline) administration, the scalp inci-
sion is made with either fine-tip cautery or scalpel.
With the pericranium left intact, a subgaleal scalp
dissection is done, with attention to meticulous
hemostasis.
5. Bone removal: Access to the extradural
space at the coronal suture can be achieved
with either bilateral burr holes or dissection
through the coronal suture. Bilateral burr
holes are placed anterior to the lambdoid
suture. Burr holes are fashioned with a
craniotome using either an acorn (6-mm)
or match head (2.2-mm) burr. The holes are
placed parasagittally, 1.5 to 2 cm off midline.
Ideally, the planned parasagittal osteotomies
do not cross the location of the commonly
present parietal emissary veins.
b
In the minimal procedure, after the vertex scalp is
elevated in the subgaleal plane, only as wide as nec-
essary for the parasagittal osteotomies, anterior and
posterior craniectomies are fashioned to include the
fused suture under direct vision. This provides access
to the extradural space, from anterior and posterior
trajectories for the scope or dissector. Using direct
visualization with a headlight and loupes (or, less
commonly, with the endoscope), the parasagittal
osteotomies are made starting with either the cra-
niotome or scissors, depending on access provided
by the scalp incision and bone thickness (Fig. 13.2b).
The osteotomies continue from either anteriorly or
posteriorly, with cuts from the opposite direction Fig. 13.2â•… Oblique, 3D-rendered view with semitransparent
made to join at the mid-parietal level. An osteotomy scalp showing skin incisions and osteotomies. (a) Open bone
across the midline posteriorly is made with a cranio- work, including circular occipital remodeling of interparietal
tome, scissors, or blade cut after the dura is freed. occipital bone. (b) Minimally invasive bone work.
If present, any wormian bone or bone present ante-
rior to the lambda can then be removed under direct
vision. The scalp is further dissected for anterior and the coronal sutures and posteriorly to and over the
posterior parietal wedge osteotomies made paral- occipital prominence, exposing the coronal, lamb-
lel to the coronal and lambdoid sutures. Scissors are doid, and squamosal sutures laterally.
adequate for these osteotomies. They do not need to Accessing the extradural space either with burr
cross the squamosal suture. holes or through the coronal sutures, a vertex craniec-
Hemostasis is achieved under direct vision using tomy is performed using a craniotome and the fused,
bipolar cautery on the dura and subgaleal vessels nonadherent sagittal suture and adjacent parietal
and with bone wax and/or monopolar cautery on bone can be elevated from the dura and reflected on
the diploic space. Floseal Matrix (Baxter Healthcare, the lambdoid sutures. The vertex bone can then be
Deerfield, IL, USA) can be used for fast hemostasis sharply dissected at the lambda. This technique should
in difficult-to-reach bleeding. Hemostasis must be be avoided if there is a wormian bone at the posterior
meticulous. fontanelle or the lambda is angled in such a way that
In the older infants and those with prominent the vertex bone would cause compression and occlu-
occipital prominence, the more radical open ver- sion of the superior sagittal sinus during the maneu-
tex craniectomy is performed with the patient in ver. In this situation, the posterior parietal osteotomy
the prone position (Fig. 13.1g,h). The same anes- should be done using a craniotome and the bone at the
thetic precautions are taken as described for the lambda should be elevated under direct vision.
minimal procedure. A bicoronal incision is used as When the occipital bone is deformed, the pos-
previously described. Scalp flaps are mobilized to terior parietal bones are often concave, creating
13â•… Sagittal Synostosis Repair Surgery 123
the clinical biparietal narrowing. This narrowing a mold made at 10 days following surgery, when
can be addressed by excising these depressions postoperative swelling has, in most cases, resolved.
(wedge osteotomies) (Fig. 13.2a). If the occipital When the helmet is fitted, shims may be necessary
deformity is to be addressed, the lambdoid sutures if the anteroposterior (AP) dimension has further
must be freed from the dura through their whole decreased as swelling has resolved. Biparietal shims
length. The bilateral occipital craniotomy is done may be needed to achieve a secure fit. Patients are
in a circular fashion, elevating the interparietal seen every 3 weeks by the orthotist, and as the AP
portion of the occipital bone. This bone flap can be dimension increases, the helmet liner is shaved, the
either left off or remodeled (flattened using radial shell is adjusted to accommodate the new size goal,
cuts) and repositioned (sutured to the posterior and biparietal shims are removed.
parietal bones). Helmeting is maintained for at least 6 months.
If there is not spontaneous widening of the ver- Only one helmet has been necessary in our patients
tex craniectomy defect, narrow wedge osteotomies to date. Since the helmet’s shell is adjustable, it does
or barrel staves are made parallel to the coronal not need to be remade. The inner foam insert can
suture as far as to the squamosal suture and the be carved to address areas of pressure. The weight
parietal bones are hinged laterally on the squamo- of the helmet may be a concern for very young
sal suture and contoured using the Tessier bone patients; however, to the present, a standard helmet
bending forceps. The posterior parietal wedge has proven adequate.
osteotomies can be “narrowed” with sutures. This
causes the parietal bone to buckle and gives an
immediate wider biparietal diameter and appear- 13.3.4╇ Hazards, Risks, Avoidance of
ance. Hemostasis is as described previously and Pitfalls
must be meticulous.
In general, the mortality and morbidity rates of
6. Scalp closure: Closure of the scalp is done
sagittal synostosis repair surgeries are very low.1,11
in two layers. We use Vicryl (Ethicon,
Nevertheless, great care is always needed during
Somerville, NJ, USA) 3–0 interrupted stitches
positioning to avoid pressure sores, ocular injury,
subcutaneously and dissolvable running
and slipping as table position changes.
Vicryl Rapide 4–0 for skin closing. The skin
Proper exposure of the extradural space and
is then sprayed with an adhesive OpSite
hemostatic control are needed to avoid dural tears,
spray dressing (Smith-Nephew, Mississauga,
sagittal sinus hemorrhaging, or air embolism. One
Ontario, Canada). Postoperatively, the head
should anticipate that the dura and sinus will be
of the patient is raised 20 to 30°. Average
adherent at nonfused segments of the sagittal, coro-
skin-to-skin time for the minimal procedure
nal, and lambdoid sutures and at the lambda. Dural
is about 1 hour, for the more radical open
dissection from those parts of the bone is more diffi-
procedure about 1.5 hours.
cult and therefore prone to dural tearing. Such dissec-
7. Postoperative helmet therapy: Calvarial tion should be done under direct vision. Smoothing
growth will conform to the inner shape of the bone edges after osteotomy and craniectomies
a helmet worn postoperatively. By creating might lower the risk of impinging the dura or skin
a helmet with an optimal inner shape, the postoperatively. To decrease the risk of postoperative
posthelmeting clinical results have been bleeding from the scalp, we like to elevate the head
made predictable. The goal of helmeting 20–30° degrees postoperatively.
is a cephalic index of approximately 0.8.
Ideally, the helmet is worn 23 hours per
day unless there is scalp pressure or 13.3.5╇ Salvage and Rescue
irritation. Compressive molding, as is done
on other forms of calvarial deformity, is not Massive bleeding is possible in areas where the
performed. sagittal sinus is adherent to the sagittal suture or
We have experimented with locally fabricated lambda. Such adherence occurs in areas of a patent,
helmets but have had best quality and service normal suture and not in the areas where the suture
from the manufacturer producing bivalved cranial is fused. Hemorrhage can also occur from the diploic
remolding orthoses (Orthomerica, Orlando, FL, USA). and unrecognized parietal emissary veins. We usu-
Although stereographic imagery from craniofacial ally have blood for transfusion available. The secure
surface scanners can be used to define the start- positioning of the patient on the operating table
ing cranial morphology, our orthotic team prefers allows head-up or head-down positioning quickly to
to base the helmet on a cast mold. The patient has address blood loss or air entrainment.
124 Section III.Aâ•… Malformations of the Scalp and Skull

13.4╇ Outcomes and Postoperative References


Course ╇1. Lee HQ, Hutson JM, Wray AC, et al. Analysis of morbid-
ity and mortality in surgical management of craniosyn-
13.4.1╇ Postoperative Considerations ostosis. J Craniofac Surg 2012;23(5):1256–1261
╇2. Lattanzi W, Bukvic N, Barba M, et al. Genetic basis of
During the postoperative period, we elevate the single-suture synostoses: genes, chromosomes and
head 20 to 30° upright. Usually, patients who had clinical implications. Childs Nerv Syst 2012;28(9):
1301–1310
minimally invasive surgery go home within 24 to 48
hours. Patients who had more extensive surgery are ╇3. Johnson D, Wilkie AO. Craniosynostosis. Eur J Hum
Genet 2011;19(4):369–376
nursed on the ward for 3 to 4 days prior to discharge.
Follow-up assessments of wound healing are made ╇4. Arnaud E, Renier D, Marchac D. Prognosis for mental func-
tion in scaphocephaly. J Neurosurg 1995;83(3):476–479
at 4 to 6 weeks postoperatively and at the time of
helmet fitting and adjustment. ╇5. Da Costa AC, Anderson VA, Holmes AD, et al. Longitudi-
nal study of the neurodevelopmental characteristics of
treated and untreated nonsyndromic craniosynostosis
in infancy. Childs Nerv Syst 2013;29(6):985–995
13.4.2╇Complications
╇6. Speltz ML, Endriga MC, Mouradian WE. Presurgical
and postsurgical mental and psychomotor develop-
Patients who have had minimally invasive sagittal
ment of infants with sagittal synostosis. Cleft Palate
synostosis repair surgery have rarely required blood Craniofac J 1997;34(5):374–379
transfusion. In the more extensive procedures, 10 to
╇7. Sood S, Rozzelle A, Shaqiri B, Sood N, Ham SD. Effect
15% of patients need a blood transfusion either intra- of molding helmet on head shape in nonsurgically
operatively (rare) or postoperatively.12 The complica- treated sagittal craniosynostosis. J Neurosurg Pediatr
tion types and rates we have seen are similar to those 2011;7(6):627–632
reported by others.1,11 Clinically significant sutural ╇8. Agrawal D, Steinbok P, Cochrane DD. Long-term anthro-
fusion of previously normal coronal or lambdoid pometric outcomes following surgery for isolated sag-
sutures after a vertex craniectomy is uncommon. ittal craniosynostosis. J Neurosurg 2006;105(5, Suppl):
This is usually recognized because of the falloff of 357–360
head growth in the first 24 months following the ini- ╇9. Massimi L, Tamburrini G, Caldarelli M, Di Rocco C. Ef-
tial correction. Papilledema may be present. In most fectiveness of a limited invasive scalp approach in the
situations, the bone growth cannot keep pace with correction of sagittal craniosynostosis. Childs Nerv
brain growth, and craniostenosis results. A cranial Syst 2007;23(12):1389–1401
expansion operation may then be needed. Recurrent 10. Lee J, Crawford MW, Drake J, Buncic JR, Forrest C. An-
dolichocephaly of a degree meriting repeat opera- terior ischemic optic neuropathy complicating cranial
tion is also rare. In this situation, a radical remod- vault reconstruction for sagittal synostosis in a child. J
eling with parietal widening is needed. This can be Craniofac Surg 2005;16(4):559–562
done with a single operation or more gradually with 11. Alvarez-Garijo JA, Cavadas PC, Vila MM, Alvarez-Llanas A.
cranial distractors. Sagittal synostosis: results of surgical treatment in 210
patients. Childs Nerv Syst 2001;17(1-2):64–68
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14 Operative Techniques in Cranial
Vault Reconstruction: Nonsyndromic
Coronal Craniosynostosis
Christopher C. Chang, Derek M. Steinbacher, Charles C. Duncan,
and John A. Persing

14.1╇ Introduction and Background mation associated wi