3. Fundamentals of Pediatric Surgery Edited by Peter Mattei,
MD, FAAP, FACS The Childrens Hospital of Philadelphia,
Philadelphia, PA, USA
4. Editor Peter Mattei, MD, FAAP, FACS Assistant Professor of
Surgery, University of Pennsylvania School of Medicine, Division of
General, Thoracic and Fetal Surgery, The Childrens Hospital of
Philadelphia, Philadelphia, PA, USA ISBN 978-1-4419-6642-1 e-ISBN
978-1-4419-6643-8 DOI 10.1007/978-1-4419-6643-8 Springer New York
Dordrecht Heidelberg London Springer Science+Business Media, LLC
2011 All rights reserved. This work may not be translated or copied
in whole or in part without the written permission of the publisher
(Springer Science+Business Media, LLC, 233 Spring Street, New York,
NY 10013, USA), except for brief excerpts in connection with
reviews or scholarly analysis. Use in connection with any form of
information storage and retrieval, electronic adaptation, computer
software, or by similar or dissimilar methodology now known or
hereafter developed is forbidden. The use in this publication of
trade names, trademarks, service marks, and similar terms, even if
they are not identified as such, is not to be taken as an
expression of opinion as to whether or not they are subject to
proprietary rights. While the advice and information in this book
are believed to be true and accurate at the date of going to press,
neither the authors nor the editors nor the publisher can accept
any legal responsibility for any errors or omissions that may be
made. The publisher makes no warranty, express or implied, with
respect to the material contained herein. Printed on acid-free
paper Springer is part of Springer Science+Business Media
(www.springer.com)
5. To my wife, partner, and best friend, Kim, for her support
and encouragement every day, and To Kim, Gina, Peter, Joey, and
Michael, for the inspiration and hope for the future I derive from
watching them grow, learn and dream.
6. wwwwwww
7. vii Fundamentals of Pediatric Surgery, like its predecessor
Surgical Directives: Pediatric Surgery, provides practicing
pediatric surgeons and adult general surgeons with authoritative
discourses that were written by recognized experts and cover the
fundamental principles of clinical pediatric surgery. The goal of
the editor and the authors is simple: provide readers with a unique
resource consisting of practical and clinically oriented chapters
that reflect the real- world experience of expert pediatric
surgeons. Given the pace of new advances in Pediatric Surgery, we
felt the time was right for the book to be updated and improved.
The result of these improvements and enhancements is Fundamentals
of Pediatric Surgery. Fundamentals of Pediatric Surgery is based on
a simple but important philosophy: provide a practical and
up-to-date resource for the practicing surgeon detailing the
specific needs and special considerations surrounding the surgical
care of children. We especially wanted to con- vey this information
in an accessible and pleasing format. Written by an experienced
surgeon or clinician, each chapter has been carefully edited to
maintain continuity in style and format while preserving the unique
voice of the experienced and knowledgeable contributing author.
This new edition also includes highlighted textboxes that emphasize
important points and critical concepts along with a list of
suggested reading. Finally, every chapter is followed by the
editors comments, which are intended to provide more in-depth
analysis, a distinct opinion, or simply additional useful
information. In addition to serving as a useful reference for
pediatric surgeons and general surgeons in clinical practice,
Fundamentals of Pediatric Surgery is also specifically designed to
be used by general surgical residents rotating in pediatric surgery
and chief residents who have chosen to obtain further specialized
training in a Pediatric Surgery fellowship program. The American
Board of Surgery and the Accreditation Council for Graduate Medical
Education (ACGME) consider experience in the clinical aspects of
pediatric surgery a necessary and important aspect of the education
and training of the general surgeon and every General Surgery
resident is expected to participate in a Pediatric Surgery rotation
during their residency. These rotations are typically brief but can
be quite hectic, with little time to read a comprehensive pediatric
surgical textbook, especially when what one really needs is a
practical guide to the everyday care of the pediatric surgical
patient. The monographs provided by Fundamentals of Pediatric
Surgery are concise and easy to read, filled with detailed and
relevant information that can help you care for the patient in the
clinic today or as a consultation on the Pediatrics service. The
goal is not to describe every possible management strategy, but
rather at least one reasonable and proven approach endorsed by an
experienced surgeon in a context that includes a discus- sion of
the underlying principles of care and essential issues to be
considered when faced with a particular clinical entity. The
Pediatric Surgery fellow will find this book to be a rich and
up-to-date source of perti- nent information related to the actual
day-to-day care of the child with a surgical disease pro- cess.
Furthermore, it will provide the foundation for what will
undoubtedly prove to be an exciting and life-long education in the
complexities of the surgical care of children. Finally, it
Preface
8. viii Preface is intended to be a valuable resource and study
guide for preparation for the written and oral American Board of
Surgery certifying examinations in Pediatric Surgery. It is our
sincere hope that Fundamentals of Pediatric Surgery, designed with
the more advanced practitioner in mind, will prove to be a useful
and valuable complement to the many excellent pediatric surgical
texts currently available. Philadelphia, Pennsylvania Peter Mattei,
MD, FAAP, FACS November 2010
9. ix This book is the result of a team effort that includes
the support and encouragement of my Surgeon-in-Chief, N. Scott
Adzick, the help and accommodations of my partners in the Division
of General, Thoracic and Fetal Surgery, and the time and expertise
of our administra- tive assistants. I was inspired to produce this
book by my many excellent teachers and men- tors when I was a
resident and pediatric surgery fellow, and over the years I have
been motivated to forge ahead by the experience of being a teacher
and mentor to the many excellent pediatric surgery fellows and
general surgery residents I have had the privilege to help train
over the years. I must also acknowledge the hard work and
dedication of the pediatric surgeons and other experts in the field
who have contributed chapters for this text and, more importantly,
their continued devotion to a career of working with children who
need our help and who ultimately make it all worthwhile.
Acknowledgments
10. xi Part I Perioperative Care 1 Preoperative Assessment and
Preparation..........................................................
3 Ari Y. Weintraub and Lynne G. Maxwell 2 Prenatal Diagnosis and
Genetic
Counseling........................................................
17 R. Douglas Wilson 3 Epidural and Regional
Anesthesia.......................................................................
23 Arjunan Ganesh and John B. Rose 4 Enteral
Nutrition....................................................................................................
27 L. Grier Arthur and Shaheen J. Timmapuri 5 Parenteral
Nutrition..............................................................................................
33 Aaron P. Garrison and Michael A. Helmrath 6 Fast-Track
Protocols..............................................................................................
37 Peter Mattei 7 Quality Improvement, Education, and Outcomes
Research in Pediatric
Surgery...............................................................................................
41 Steven Teich and Marc P. Michalsky Part II Critical Care 8
Shock.......................................................................................................................
49 John J. McCloskey 9 Electrolyte
Disorders.............................................................................................
57 Patrick J. Javid 10 Vascular
Access......................................................................................................
65 Stephen G. Murphy 11 Acute Kidney
Injury..............................................................................................
73 Peter A. Meaney and Kevin E.C. Meyers 12 Respiratory Failure and
Mechanical
Ventilation................................................ 83 Todd
J. Kilbaugh 13 Extracorporeal Membrane
Oxygenation.............................................................
91 Edmund Y. Yang Contents
11. xii Contents Part III Trauma 14 Pediatric Trauma
Resuscitation...........................................................................
103 Thane Blinman 15 Head
Trauma..........................................................................................................
111 Gregory G. Heuer and Phillip B. Storm 16 Neck
Injuries..........................................................................................................
117 Peter T. Masiakos and George C. Velmahos 17
Burns.......................................................................................................................
123 Gail E. Besner 18 Abdominal
Trauma................................................................................................
135 Michael L. Nance 19 Thoracic
Trauma...................................................................................................
145 Martin S. Keller 20 Spine
Trauma.........................................................................................................
151 Robert W. Letton 21 Vascular
Injury......................................................................................................
157 Barbara A. Gaines 22 Pediatric Hand
Injuries.........................................................................................
161 Roger Cornwall 23 Child
Abuse............................................................................................................
169 Richard A. Falcone, Jr. and Kathi Makoroff Part IV Head and
Neck 24 The Critical
Airway...............................................................................................
177 Karen B. Zur 25
Bronchoscopy.........................................................................................................
185 Ian N. Jacobs 26 Cystic Neck
Masses................................................................................................
195 Oluyinka O. Olutoye 27 Disorders of the Thyroid and
Parathyroid..........................................................
203 William T. Adamson 28 Cervical
Lymphadenopathy..................................................................................
213 Rajeev Prasad and L. Grier Arthur Part V Esophagus 29
Esophageal Atresia and Tracheo-Esophageal
Fistula......................................... 223 Jean-Martin
Laberge 30 Long-Gap Esophageal
Atresia..............................................................................
233 Pietro Bagolan and Francesco Morini
12. xiiiContents 31 Esophageal
Replacement.......................................................................................
247 Lewis Spitz 32 Esophageal
Injuries...............................................................................................
253 Kristin N. Fiorino and Petar Mamula 33 Foregut
Duplications..............................................................................................
267 Pablo Laje 34
Achalasia.................................................................................................................
273 J. Duncan Phillips Part VI Thorax and Mediastinum 35 Patent
Ductus
Arteriosus......................................................................................
283 Stephanie Fuller and Peter J. Gruber 36 Vascular Compression
Syndromes.......................................................................
289 Mark L. Wulkan 37 Congenital Lung
Lesions.......................................................................................
293 Bill Chiu and Alan W. Flake 38 Thoracoscopic Biopsy and
Lobectomy of the Lung............................................
299 Sanjeev Dutta and Craig T. Albanese 39 Diseases of the Pleural
Space................................................................................
305 Keith A. Kuenzler 40 Pectus
Deformities..................................................................................................
313 M. Ann Kuhn and Donald Nuss 41 Mediastinal
Masses................................................................................................
323 Richard D. Glick Part VII Stomach and Small Intestine 42
Gastroesophageal Reflux
Disease.........................................................................
333 Thane Blinman 43 Hypertrophic Pyloric
Stenosis..............................................................................
341 Marjorie J. Arca and Jill S. Whitehouse 44 Surgical Enteral
Access.........................................................................................
347 Tim Weiner and Melissa K. Dedmond 45 Duodenal
Atresia....................................................................................................
353 Keith A. Kuenzler and Steven S. Rothenberg 46 Intestinal
Atresias..................................................................................................
359 Peter F. Nichol and Ari Reichstein 47 Abdominal Cysts and
Duplications......................................................................
365 Patricia A. Lange
13. xiv Contents 48 Anomalies of Intestinal
Rotation..........................................................................
373 Franois I. Luks 49 Necrotizing
Enterocolitis.......................................................................................
381 Cynthia A. Gingalewski 50 Short Bowel
Syndrome..........................................................................................
387 Thomas Jaksic, Brian A. Jones, Melissa A. Hull, and Shimae C.
Fitzgibbons 51 Meconium
Ileus......................................................................................................
395 Peter Mattei 52
Intussusception.......................................................................................................
401 John H.T. Waldhausen 53 Meckels
Diverticulum...........................................................................................
409 Melvin S. Dassinger, III 54 Bariatric
Surgery...................................................................................................
415 Joy L. Collins 55 Chronic Abdominal
Pain.......................................................................................
425 Frazier W. Frantz 56 Crohns
Disease......................................................................................................
437 Peter Mattei 57 Ileostomy and
Colostomy......................................................................................
443 Oliver S. Soldes Part VIII Colon, Rectum, and Anus 58
Constipation...........................................................................................................
453 Linda Nicolette 59 Perianal
Disease.....................................................................................................
461 Cynthia D. Downard 60 Pilonidal Cyst
Disease............................................................................................
467 Daniel P. Doody 61 Hirschsprung
Disease............................................................................................
475 Jacob C. Langer 62
Appendicitis............................................................................................................
485 Shawn D. Safford 63 Ulcerative Colitis and Familial
Polyposis............................................................
491 Stephen E. Dolgin 64 Anorectal
Malformations......................................................................................
499 Marc A. Levitt and Alberto Pea
14. xvContents Part IX Abdominal Wall, Peritoneum, and
Diaphragm 65
Gastroschisis...........................................................................................................
515 Aimen F. Shaaban 66
Omphalocele...........................................................................................................
523 Kenneth W. Liechty 67 Eventration of the
Diaphragm..............................................................................
531 Samuel Z. Soffer 68 Congenital Diaphragmatic
Hernia.......................................................................
535 Peter Mattei 69 Uncommon
Hernias...............................................................................................
543 Shaheen J. Timmapuri and Rajeev Prasad 70 Umbilical Disorders
and
Anomalies.....................................................................
547 Adam J. Kaye and Daniel J. Ostlie 71 Peritoneal
Dialysis..................................................................................................
553 Danny Little and Monford D. Custer Part X Liver, Biliary Tree,
Pancreas, and Spleen 72 Neonatal
Hyperbilirubinemia...............................................................................
561 Clyde J. Wright and Michael A. Posencheg 73 Biliary
Atresia........................................................................................................
567 Peter C. Minneci and Alan W. Flake 74 Surgical Therapy of
Disorders of Intrahepatic
Cholestasis............................... 575 Peter Mattei 75
Cholecystitis............................................................................................................
579 Andr Hebra and Aaron Lesher 76 Choledochal
Cysts..................................................................................................
587 Greg M. Tiao 77 Hepatic
Resection...................................................................................................
593 Heung Bae Kim 78 Portal
Hypertension...............................................................................................
599 Jaimie D. Nathan, Kathleen M. Campbell, Greg M. Tiao, Maria H.
Alonso, and Frederick C. Ryckman 79 Congenital
Hyperinsulinism.................................................................................
611 N. Scott Adzick 80 Disorders of the
Pancreas......................................................................................
617 Marshall Z. Schwartz and Michael S. Katz 81 Disorders of the
Spleen..........................................................................................
625 Melissa E. Danko and Henry E. Rice
15. xvi Contents Part XI Genitourinary 82 Vesicoureteral
Reflux.............................................................................................
635 Pasquale Casale 83 Renal
Abnormalities..............................................................................................
641 Pierluigi Lelli-Chiesa and Gabriele Lisi 84 Penile Anomalies
and
Circumcision.....................................................................
651 Douglas A. Canning 85 Inguinal Hernia and
Hydrocele............................................................................
663 Andr Hebra and Joshua B. Glenn 86 Undescended
Testis................................................................................................
673 Pasquale Casale and Sarah M. Lambert 87 The Diagnosis and
Management of Scrotal
Pain................................................ 679 Stephen A.
Zderic 88 Cloacal
Exstrophy..................................................................................................
685 Michael C. Carr 89 Disorders of Sex
Development..............................................................................
693 Thomas F. Kolon 90 Vagina: Diseases and
Treatment...........................................................................
701 Edward J. Doolin Part XII Surgical Oncology 91
Neuroblastoma.......................................................................................................
709 Natasha E. Kelly and Michael P. La Quaglia 92 Wilms
Tumor..........................................................................................................
715 Peter F. Ehrlich 93 Adrenal
Tumors.....................................................................................................
725 Daniel von Allmen 94
Rhabdomyosarcoma..............................................................................................
729 Ravi S. Radhakrishnan and Richard J. Andrassy 95 Sacrococcygeal
Teratoma......................................................................................
735 Helene Flageole 96 Ovarian
Tumors.....................................................................................................
741 Kirk W. Reichard 97 Pediatric Testicular
Tumors..................................................................................
749 Ismael Zamilpa and Martin A. Koyle 98 Soft Tissue
Tumors.................................................................................................
755 Roman M. Sydorak and Harry Applebaum
16. xviiContents 99 Liver
Tumors..........................................................................................................
761 Rebecka L. Meyers 100 Musculoskeletal Surgical
Oncology......................................................................
773 Jenny M. Frances and John P. Dormans Part XIII Skin and Soft
Tissues 101 Subcutaneous
Endoscopy......................................................................................
785 Sanjeev Dutta 102 Benign Skin
Lesions...............................................................................................
795 Michael D. Rollins and Sheryll L. Vanderhooft 103 Atypical Nevi
and Malignant
Melanoma.............................................................
805 Kenneth W. Gow 104 Necrotizing Soft Tissue
Infections........................................................................
815 Eric R. Scaife 105 Hemangiomas and Vascular
Malformations.......................................................
819 David W. Low 106 Disorders of the
Breast..........................................................................................
829 Mary L. Brandt Part XIV Transplantation 107 Kidney
Transplantation.........................................................................................
839 Peter L. Abt and H. Jorge Baluarte 108 Liver
Transplantation............................................................................................
847 Maria H. Alonso 109 Intestinal
Transplantation.....................................................................................
857 Thomas M. Fishbein Part XV Miscellaneous 110 Gastrointestinal
Bleeding......................................................................................
865 Katherine J. Deans 111 Fetal
Surgery..........................................................................................................
871 Foong-Yen Lim and Timothy M. Crombleholme 112 Disorders of the
Abdominal Aorta and Major
Branches................................... 881 Omaida C. Velazquez
113 Ventricular Shunts for
Hydrocephalus................................................................
887 Gregory G. Heuer and Phillip B. Storm 114 Conjoined
Twins.....................................................................................................
893 Gary E. Hartman
Index..................................................................................................................................
901
17.
18. xix Peter L. Abt, MD Department of Surgery, University of
Pennsylvania, Childrens Hospital of Philadelphia, Hospital of the
University of Pennsylvania, Philadelphia, PA, USA William T.
Adamson, MD Department of Surgery, University of North Carolina
School of Medicine, North Carolina Childrens Hospital, Chapel Hill,
NC, USA N. Scott Adzick, MD Department of Surgery, Childrens
Hospital of Philadelphia, University of Pennsylvania School of
Medicine, Philadelphia, PA, USA Craig T. Albanese, MD, MBA
Department of Surgery, Stanford University, Lucile Packard
Childrens Hospital, Stanford, CA, USA Maria H. Alonso, MD Division
of Pediatric and Thoracic Surgery, Cincinnati Childrens Hospital
Medical Center, Cincinnati, OH, USA Richard J. Andrassy, MD
Department of Surgery, Memorial Hermann Hospital, MD Anderson
Cancer Center, Houston, TX, USA Harry Applebaum, MD Division of
Pediatric Surgery, David Geffen School of Medicine at UCLA, Kaiser
Permanente Los Angeles Medical Center, Los Angeles, CA, USA
Marjorie J. Arca, MD Department of Surgery, Medical College of
Wisconsin, Childrens Hospital of Wisconsin, Milwaukee, WI, USA L.
Grier Arthur, MD Division of Pediatric General, Drexel University,
Thoracic and Minimally Invasive Surgery, St. Christophers Hospital
for Children, Philadelphia, PA, USA Pietro Bagolan, MD Department
of Medical and Surgical Neonatalology, Bambino GESU Childrens
Hospital, Piazza S. Onofrio, 4, Roma 00165, Italia H. Jorge
Baluarte, MD Division of Pediatric Nephrology, Childrens Hospital
of Philadelphia, Philadelphia, PA, USA Gail E. Besner, MD
Department of Surgery, Ohio State University College of Medicine,
Nationwide Childrens Hospital, Columbus, OH, USA Contributors
19. xx Contributors Thane Blinman, MD General, Thoracic and
Fetal Surgery, The Childrens Hospital of Philadelphia, 34th and
Civic Center Blvd., 5 Wood, Philadelphia, PA 19104, USA Mary L.
Brandt, MD Department of Pediatric Surgery, Baylor College of
Medicine, Texas Childrens Hospital, Houston, TX, USA Kathleen M.
Campbell, MD Department of Gastroenterology, Cincinnati Childrens
Hospital Medical Center, Hepatology and Nutrition, Cincinnati, OH,
USA Douglas A. Canning, MD Department of Surgery, Division of
Urology, University of Pennsylvania School of Medicine, Childrens
Hospital of Philadelphia, Philadelphia, PA, USA Michael C. Carr, MD
Division of Urology, Childrens Hospital of Philadelphia,
Philadelphia, PA, USA Pasquale Casale, MD Department of Urology,
Childrens Hospital of Philadelphia, Philadelphia, PA, USA Bill
Chiu, MD Department of Surgery, Childrens Hospital of Philadelphia,
Philadelphia, PA, USA Joy L. Collins, MD Department of Pediatric
General and Thoracic Surgery, University of Pennsylvania, Childrens
Hospital of Philadelphia, Philadelphia, PA, USA Roger Cornwall, MD
Division of Orthopedic Surgery, Cincinnati Childrens Hospital
Medical Center, Cincinnati, OH, USA Timothy M. Crombleholme, MD
Department of Pediatric Surgery, Cincinnati Childrens Foundation,
University of Cincinnati College of Medicine, Cincinnati Childrens
Hospital Medical Center, Cincinnati, OH, USA Monford D. Custer, MD
Division of Pediatric Surgery, Childrens Hospital at Scott and
White, Temple, TX, USA Melissa E. Danko, MD Department of Surgery,
Duke University Medical Center, Durham, NC, USA Melvin S.
Dassinger, III, MD Division of Pediatric Surgery, University of
Arkansas for Medical Sciences, Arkansas Childrens Hospital, Little
Rock, AR, USA Katherine J. Deans, MD, MHSc Department of Surgery,
Division of General Thoracic and Fetal Surgery, University of
Pennsylvania, Childrens Hospital of Philadelphia, Philadelphia, PA,
USA Melissa K. Dedmond, PA-C Department of Pediatric Surgery,
University of North Carolina, UNC Hospitals, Chapel Hill, NC,
USA
20. xxiContributors Stephen E. Dolgin, MD Albert Einstein
College of Medicine, Schneider Childrens Hospital, 269-01 76 Ave 1,
New Hyde Park, NY 11040, USA Daniel P. Doody, MD Department of
Pediatric Surgery, Massachusetts General Hospital, Harvard Medical
School, Boston, MA, USA Edward J. Doolin, MD, BS Chemistry
Department of Pediatric General and Thoracic Surgery, Childrens
Hospital of Philadelphia, Philadelphia, PA, USA John P. Dormans, MD
Department of Orthopedics, Childrens Hospital of Philadelphia,
Philadelphia, PA, USA Cynthia D. Downard, MD, MMSc Department of
Surgery, University of Louisville, Kosair Childrens Hospital,
Louisville, KY, USA Sanjeev Dutta, MD, MA Department of Surgery,
Lucile Packard Childrens Hospital, Stanford University, 780 Welch
Road, Svite 206, Stanford, CA 94305, USA Peter F. Ehrlich, MD, MSc
Department of Pediatric Surgery, University of Michigan, CS Mott
Childrens Hospital, Ann Arbor, MI, USA Richard A. Falcone, Jr., MD,
MPH Pediatric General and Thoracic Surgery, University of
Cincinnati, Cincinnati Childrens Hospital Medical Center,
Cincinnati, OH, USA Kristin N. Fiorino, MD Department of Pediatric
Gastroenterology, Childrens Hospital of Philadelphia, Philadelphia,
PA, USA Thomas M. Fishbein, MD Georgetown University Hospital,
Transplant Institute, Washington, DC, USA Shimae C. Fitzgibbons, MD
Department of Surgery, Harvard Medical School, Childrens Hospital
Boston, Boston, MA, USA Helene Flageole, MD, MSc, FRCSC, FACS
Department of Surgery, McMaster Childrens Hospital, 1200 Main
Street, Hamilton, ON # L8N325, Canada Alan W. Flake, MD Department
of Surgery, Childrens Hospital of Philadelphia, Philadelphia, PA,
USA Jenny M. Frances, MD, MPH Department of Orthopedic Surgery, New
York University Hospital for Joint Diseases, New York, NY, USA
Frazier W. Frantz, MD Department of Pediatric Surgery, East
Virginia Medical School, Childrens Hospital of The Kings Daughters,
Norfolk, VA, USA Stephanie Fuller, MD Department of Cardiothoracic
Surgery, Childrens Hospital of Philadelphia, 34th Street &
Civic Center Boulevard, Ste. A2NWAD, Philadelphia, PA 19103,
USA
21. xxii Contributors Barbara A. Gaines, MD Childrens Hospital
of Pittsburgh of UPMC, University of Pittsburgh, Pittsburgh, PA,
USA Arjunan Ganesh, MBBS Department of Anesthesiology, University
of Pennsylvania, Childrens Hospital of Philadelphia, Philadelphia,
PA, USA Aaron P. Garrison, MD Department of General Surgery,
University of North Carolina at Chapel Hill, Chapel Hill, NC, USA
Cynthia A. Gingalewski, MD Department of General Surgery, George
Washington University, Childrens National Medical Center,
Washington, DC, USA Joshua B. Glenn, MD Department of Pediatric
Surgery, Vanderbilt University Childrens Hospital, TN, USA Richard
D. Glick, MD Department of Pediatric Surgery, Albert Einstein
College of Medicine, Schneider Childrens Hospital, New Hyde Park,
NY, USA Kenneth W. Gow, MD, MSc, FRCSC, FAAP, FACS Department of
Surgery, Childrens Hospital and Regional Medical Center, University
of Washington, 4800 Sand Point Way NE, MIS W-7729, PO Box 5371,
Seattle, WA 98105, USA Peter J. Gruber, MD, PhD Department of
Pediatric Surgery, Childrens Hospital of Philadelphia,
Philadelphia, PA 19140, USA Gary E. Hartman, MD, MBA Department of
Pediatric Surgery, Stanford University School of Medicine, Lucile
Packard Childrens Hospital, Stanford, CA, USA Andr Hebra, MD
Department of Surgery, Medical University of South Carolina,
Childrens Hospital, 96 Jonathan Lucas Street, Charleston, SC 29425,
USA Michael A. Helmrath, MD Department of Pediatric Surgery,
University of North Carolina at Chapel Hill, Chapel Hill, NC, USA
Gregory G. Heuer, MD, PhD Department of Neurosurgery, University of
Pennsylvania, The Childrens Hospital of Philadelphia, 877 N. 30th
St. Philadelphia, PA 19130, USA Melissa A. Hull, MD Department of
Surgery, Harvard Medical School, Childrens Hospital Boston, Boston,
MA, USA Ian N. Jacobs, MD Department of Otolaryngology, University
of Pennsylvania School of Medicine, Childrens Hospital of
Philadelphia, Philadelphia, PA, USA Thomas Jaksic, MD, PhD
Department of Pediatric Surgery, Childrens Hospital Boston, Boston,
MA, USA
22. xxiiiContributors Brian A. Jones, MD Department of Surgery,
Harvard Medical School, Childrens Hospital Boston, Boston, MA, USA
Patrick J. Javid, MD Department of Surgery, Seattle Childrens
Hospital, University of Washington, Seattle, WA, USA Michael S.
Katz, MD Department of Pediatric Surgery, St. Christophers Hospital
for Children, Philadelphia, PA, USA Adam J. Kaye, MD Department of
Pediatric Surgery, Childrens Mercy Hospital, 2401 Gillham Road,
Kansas City, MO 64108, USA Martin S. Keller, MD Department of
Pediatric Surgery, Washington University, St. Louis Childrens
Hospital, St. Louis, MO, USA Natasha E. Kelly, MD Department of
Pediatric Surgery, Memorial Sloan Kettering Cancer Center, New
York, NY 10065, USA Todd J. Kilbaugh, MD Department of
Anesthesiology and Critical Care, University of Pennsylvania,
Childrens Hospital of Philadelphia, Philadelphia, PA, USA Heung Bae
Kim, MD Department of Surgery, Pediatric Transplant Center, Harvard
Medical Center, Childrens Hospital Boston, Boston, MA, USA Thomas
F. Kolon, MD Department of Pediatric Urology, University of
Pennsylvania School of Medicine, Childrens Hospital of
Philadelphia, Philadelphia, PA, USA Martin A. Koyle, MD, FACS, FAAP
Department of Pediatric Urology, University of Washington, Seattle
Childrens Hospital, Seattle, WA, USA Keith A. Kuenzler, MD
Minimally Invasive Pediatric Surgery, NYU Langone Medical Center,
New York, NY 10016, USA M. Ann Kuhn, MD Department of Pediatric
Surgery, Eastern Virginia Medical School, Childrens Hospital of the
Kings Daughters, Norfolk, VA, USA Michael P. La Quaglia, MD
Department of Surgery, Weill Cornell University Medical School,
Memorial Sloan-Kettering Cancer Center, New York, NY, USA
Jean-Martin Laberge, MD, FRCSC, FACS Department of Pediatric
General Surgery, McGill University, Montreal Childrens Hospital of
the McGill Health Care Centre, Montreal, QC, Canada Pablo Laje, MD
Department of General Pediatric and Thoracic Surgery, Childrens
Hospital of Philadelphia, Aapt K-1103, Philadelphia, PA 19144,
USA
23. xxiv Contributors Sarah M. Lambert, MD Department of
Urology, Childrens Hospital of Philadelphia, Philadelphia, PA, USA
Patricia A. Lange, MD Department of Surgery, University of North
Carolina, Chapel Hill, UNC Hospitals, Chapel Hill, NC, USA Jacob C.
Langer, MD, FRCSC Hospital for Sick Children, Division of Thoracic
and General Surgery, University of Toronto, 1526555 University
Avenue, Toronto, ON, M5GF 1X8, Canada Pierluigi Lelli-Chiesa, MD
Department of Pediatric Surgery, Gabriele dAnnunzio of
Chieti-Pescara, Santo Spirito Hospital, Pescara, Italy Aaron
Lesher, MD Department of Surgery, Medical University of South
Carolina, Charleston SC, USA Robert W. Letton, Jr., MD Oklahoma
University Health Sciences Center, Childrens Hospital of Oklahoma,
Oklahoma City, OK, USA Marc A. Levitt, MD Colorectal Center for
Children, Cincinnati Childrens Hospital Medical Center, Pediatric
Surgery, 3333 Burnet Avenue, ML 2023, Cincinnati, OH 45229, USA
Kenneth W. Liechty, MD Departments of General Thoracic and Fetal
Surgery, University of Pennsylvania, Childrens Hospital of
Philadelphia, Philadelphia, PA, USA Foong-Yen Lim, MD Department of
Pediatric Surgery, Cincinnati Childrens Hospital Medical Center,
3333 Burnet Avenue, MLC 11025, Cincinnati, OH 452293090, USA
Gabriele Lisi, MD, PhD Department of Pediatric Surgery, Gabriele
dAnnunzio of Chieti-Pescara, Santo Spirito Hospital, Pescara, Italy
Danny Little, MD Division of Pediatric Surgery, Scott and White
Hospital, 615 West Garfield Avenue, Temple, TX, USA and Department
of Surgery, Texas A&M Health Science Center, Temple, TX, USA
David W. Low, MD Department of Surgery, Division of Plastic
Surgery, University of Pennsylvania School of Medicine, Childrens
Hospital of Philadelphia, Philadelphia, PA, USA Franois I. Luks,
MD, PhD Warren Halpert Medical School of Brown University,
Providence, RI, USA, Division of Pediatric Surgery, Hasbro
Childrens Hospital, 2, Dudley Street, Suite 180, Providence, RI
02905, USA Kathi Makoroff, MD Department of Pediatrics, Cincinnati
Childrens Hospital Medical Center, Cincinnati OH, USA Petar Mamula,
MD Department of Endoscopy, University of Pennsylvania, Childrens
Hospital of Philadelphia, Philadelphia PA, USA Peter T. Masiakos,
MS, MD, FACS, FAAP Department Pediatric Surgery, Pediatric Trauma
Unit, 55 Fruit Street, Warren 1155, Boston, MA 02114, USA
24. xxvContributors Peter Mattei, MD, FAAP, FACS Assistant
Professor of Surgery, University of Pennsylvania School of
Medicine, Division of General, Thoracic and Fetal Surgery,
Childrens Hospital of Philadelphia, Philadelphia, PA, USA Lynne G.
Maxwell, MD Department of Anesthesiology, University of
Pennsylvania, Childrens Hospital of Philadelphia, Philadelphia, PA,
USA John J. McCloskey, MD Department of Anesthesiology and Critical
Care Medicine, Childrens Hospital of Philadelphia, Philadelphia,
PA, USA Peter A. Meaney, MD, MPH Department of Anesthesia and
Critical Care, University of Pennsylvania, Childrens Hospital of
Philadelphia, Philadelphia, PA, USA Kevin E.C. Meyers, MB BCh
Department of Pediatrics and Nephrology, Childrens Hospital of
Philadelphia and University of Pennsylvania, Philadelphia, PA, USA
Rebecka L. Meyers, MD Primary Childrens Medical Center, 100 North
Medical Drive, Svite 2600, Salt Lake City, UT 84113, USA Marc P.
Michalsky, MD Department of Pediatric Surgery, Ohio State
University, Nationwide Childrens Hospital, Columbus. OH, USA Peter
C. Minneci, MD Department of Surgery, Childrens Hospital of
Philadelphia, 34th Street & Civic Center Boulevard, Wood 5,
Philadelphia, PA 19104, USA Francesco Morini, MD Department of
Medical and Surgical Neonatology, Bambino Gesu Childrens Hospital
Research Institute, Rome, Italy Stephen G. Murphy, MD Department of
Surgery, DuPont Hospital for Children, Wilmington, DE, USA Michael
L. Nance, MD Department of Surgery, Childrens Hospital of
Philadelphia, Philadelphia, PA, USA Jaimie D. Nathan, MD Division
of Transplantation, Division of Pediatric and Thoracic Surgery,
University of Cincinnati, Cincinnati Childrens Hospitals Medical
Center, Cincinnati, OH, USA Peter F. Nichol, MD, PhD Department of
Surgery, University of Wisconsin School of Medicine and Public
Health, Madison, WI, USA Linda Nicolette, MD Department of
Pediatric Surgery, Presbyterian Hospital, Albuquerque, NM, USA
Donald Nuss, MB, ChB Department of Surgery, Eastern Virginia
Medical School, Childrens Hospital of The Kings Daughters, Norfolk,
VA, USA
25. xxvi Contributors Oluyinka O. Olutoye, MD, PhD Division of
Pediatric Surgery, Michael E. DeBakey Department of Surgery, Baylor
College of Medicine, Texas Childrens Hospital, Houston, TX, USA
Daniel J. Ostlie, MD Department of Pediatric Surgery, University of
Missouri Kansas City, Childrens Mercy Hospital and Clinics, Kansas
City, MO, USA Alberto Pea, MD University of Cincinnati, Childrens
Hospital of Cincinnati, Cincinnati, OH, USA J. Duncan Phillips, MD
Department of Surgery, University of North Carolina, Chapel Hill,
North Carolina Childrens Hospital, Chapel Hill, NC, USA Michael A.
Posencheg, MD Department of Neonatology, University of Pennsylvania
School of Medicine, Hospital of the University of Pennsylvania,
Philadelphia, PA, USA Rajeev Prasad, MD, FACS, FAAP Department of
Pediatric General Surgery, Drexel University College of Medicine,
St. Christophers Hospital for Children, Philadelphia, PA, USA Ravi
S. Radhakrishnan, MD, MBA Department of Surgery, MD Anderson Cancer
Center, Memorial Hermann Hospital, 6431 Fannin Street, MSB 4200,
Houston, TX 77030, USA Kirk W. Reichard, MD Thomas Jefferson School
of Medicine, Alfred I. DuPont Hospital for Children, Wilmington,
DE, USA Ari Reichstein, MD Department of Surgery, University of
Wisconsin School of Medicine and Public Health, Madison, WI, USA
Henry E. Rice, MD Division of Pediatric Surgery, Duke University,
Duke University Medical Center, Durham, NC, USA Michael D. Rollins,
MD Department of Surgery, Division of Pediatric Surgery, Primary
Childrens Medical Center, University of Utah School of Medicine,
100 North Mario Capecchi Drive, Suite 2600, Salt Lake City, UT
841131100, USA John B. Rose, MD Department of Anesthesiology,
University of Pennsylvania, Childrens Hospital of Philadelphia,
Philadelphia, PA, USA Steven S. Rothenberg, MD Columbia University,
Rocky Mountain Hospital for Children, Denver, CO, USA Frederick C.
Ryckman, MD Division of Pediatric and Thoracic Surgery, Cincinnati
Childrens Hospital Medical Center, Cincinnati, OH, USA Shawn D.
Safford, MD Department of Surgery, National Naval Medical Center,
Bethesda, MD, USA Eric R. Scaife, MD Department of Pediatric
Surgery, University of Utah, 100 N. Mario Capecchi Drive, Street
2600, Salt Lake City, UT 841131103, USA
26. xxviiContributors Marshall Z. Schwartz, MD Department of
Surgery, Drexel University College of Medicine, St. Christophers
Hospital for Children, Philadelphia, PA, USA Aimen F. Shaaban, MD
Department of Surgery, University of Iowa Carver College of
Medicine, University of Iowa Hospitals and Clinics, Iowa City, IA,
USA Samuel Z. Soffer, MD Albert Einstein College of Medicine,
Division of Pediatric Surgery, Schneider Childrens Hospital, 26901
76th Avenue, New Hyde Park, NY 11598, USA Oliver S. Soldes, MD
Department of Pediatric Surgery, Cleveland Clinic Foundation,
Cleveland, OH, USA Lewis Spitz, MB ChB, PhD, MD (Hon), FRCS,
FRCPCH, FAAP (Hon), FCS(SA) (Hon) Department of Paediatric Surgery,
Institute of Child Health, University College, London, Great Ormond
Street Hospital, London, UK Phillip B. Storm, MD Department of
Neurosurgery, Childrens Hospital of Philadelphia, Philadelphia PA,
USA Roman M. Sydorak, MD, MPH Department of Pediatric Surgery,
Kaiser Permanente Los Angeles Medical Center, 4760 Sunset
Boulevard, 3rd Floor, Los Angeles, CA 90027, USA Steven Teich, MD
Department of Pediatric Surgery, Ohio State University, Nationwide
Childrens Hospital, Columbus, OH, USA Greg M. Tiao, MD Department
of Pediatric and Thoracic Surgery, Cincinnati Childrens Hospital
Medical Center, Cincinnati, OH, USA Shaheen J. Timmapuri, MD
Department of Pediatric Surgery, Drexel University, St.
Christophers Hospital for Children, Philadelphia, PA, USA Sheryll
L. Vanderhooft, MD Department of Pediatric Dermatology, University
of Utah School of Medicine, Primary Childrens Medical Center, Salt
Lake City UT, USA Omaida C. Velazquez, MD, FACS Jackson Memorial
Medical Center, University of Miami Hospital 1611 NW 12th Avenue,
Holtz Building, Room 3016 (R-310), Miami, FL 33136, USA George C.
Velmahos, MD, PhD, MSEd Department of Surgery, Harvard Medical
School, Massachusetts General Hospital, Boston MA, USA Daniel von
Allmen, MD Department of Pediatric Surgery, University of North
Carolina, North Carolina Medical Hospital, Chapel Hill, NC, USA
John H.T. Waldhausen, MD Department of Surgery, University of
Washington, Childrens Hospital and Regional Medical Center,
Seattle, WA, USA
27. xxviii Contributors Tim Weiner, MD Department of Surgery,
University of North Carolina, UNC Hospitals, Chapel Hill, NC, USA
Ari Y. Weintraub, MD Department of Anesthesiology, University of
Pennsylvania, Childrens Hospital of Philadelphia, Philadelphia, PA,
USA Jill S. Whitehouse, MD Department of Pediatric Surgery, Medical
College of Wisconsin, Childrens Hospital of Wisconsin, Milwaukee,
WI, USA R. Douglas Wilson, MD, MSc Department of Obstetrics and
Gynecology, University of Calgary and Calgary Health Region,
Foothills Medical Center, Calgary, AB, Canada Clyde J. Wright, MD
Department of Pediatrics, Childrens Hospital of Philadelphia, 34th
Street and Civic Center Boulevard, Philadelphia, PA 19104, USA Mark
L. Wulkan, MD Department of Surgery, Childrens Healthcare of
Atlanta at Egleston, Emory Childrens Center, Atlanta, GA, USA
Edmund Y. Yang, MD, PhD Department of Pediatric General Surgery,
Vanderbilt Childrens Hospital, Nashville, TN, USA Ismael Zamilpa,
MD Department of Pediatric Urology, University of Washington,
Seattle Childrens Hospital, Seattle, WA, USA Stephen A. Zderic, MD
Department of Pediatric Urology, Childrens Hospital of
Philadelphia, Philadelphia, PA, USA Karen B. Zur, MD Department of
Otolaryngology, University of Pennsylvania School of Medicine,
Childrens Hospital of Philadelphia, Philadelphia, PA, USA
28.
29. Part I Perioperative Care
30.
31. 3P. Mattei (ed.), Fundamentals of Pediatric Surgery, DOI
10.1007/978-1-4419-6643-8_1, Springer Science+Business Media, LLC
2011 All patients presenting for surgical procedures under
anesthesia benefit greatly from a thorough
preanesthetic/preoperative assessment and targeted preparation,
which serve to optimize any coexisting medical conditions and
minimize the poten- tial for complications. An increasing number of
procedures are being performed on an outpatient basis, and the
preop- erative assessment and preparation often occurs in the sur-
geons office or even in the preoperative area on the day of
surgery. In addition to identifying outstanding medical issues that
may delay or lead to cancellation of their procedure on the
scheduled date, the preoperative assessment is an excel- lent
opportunity to prepare patients and families and to edu-
catethemaboutwhattoexpectduringandafteradministration of an
anesthetic. For pediatric patients in particular, where the
psychological needs of the patient differ depending on their age
and the surgery and recovery involves and affects the entire
family, the preoperative assessment has a crucial role in ensuring
a smooth perioperative experience. The goals of the preoperative
evaluation are to identify any active medical issues and to ensure
that the management of these conditions is optimized prior to
anesthesia and sur- gery. Unresolved medical issues are often
significant enough to warrant cancellation of procedures for
further diagnostic workup or treatment. It is obviously in the best
interest of all the involved parties to avoid this. Risks of
Anesthesia The risk of dying from general anesthesia can only be
extrap- olated from large series and appears to be as low as 1 in
250,000 in healthy patients. To put this in perspective for
parents, the risk of a motor vehicle collision on the way to the
hospital or surgery center is greater than the risk of death under
anesthesia. Common minor adverse effects including discomfort from
airway management and postoperative nausea and vomiting (PONV)
should be discussed, along with assurances that everything will be
done to prevent and treat these relatively common complaints.
TheAmericanSocietyofAnesthesiologists(ASA)physical status score is
a means of communicating the physical condi- tion of the patient.
The physical status score was never intended to represent a measure
of operative risk and serves primarily as a means of communication
among care providers (Table1.1). In addition, certain information
is essential and should be included in the preoperative assessment
of every patient: weight, blood pressure, oxygen saturation (SpO2 )
by pulse oximetry in both room air (and with supplemental O2 , if
applicable), allergies, medications, cardiac and murmur his- tory,
and previous subspecialty encounters. Patients who have previously
undergone general anes- thesia should be asked specifically
regarding a history of the adverse effects: emergence delirium,
PONV, difficult intubation, and difficult intravenous access. Keep
in mind that patients and parents are often very anxious about
recur- rence of these events. The family history should also be
reviewed for pseudocholinesterase deficiency (prolonged paralysis
after succinyl choline) or any first-degree relative who
experienced malignant hyperthermia. Airway/Respiratory System Many
congenital syndromes are associated with craniofacial abnormalities
that may complicate or even preclude routine airway management
techniques (Table1.2). In addition to a detailed physical
examination, a history of past intubations and details of the
methods used to secure the airway are even more useful in planning
an anesthetic. Some patients are given a difficult airway letter by
an anesthesiologist and this information should be shared with the
anesthesia care team in advance of the scheduled operation. In the
absence of such information, prior anesthetic records should be
obtained and reviewed to guide airway management. Chapter 1
Preoperative Assessment and Preparation Ari Y. Weintraub and Lynne
G. Maxwell A.Y. Weintraub(*) Department of Anesthesiology,
University of Pennsylvania, Childrens Hospital of Philadelphia,
34th Street and Civic Center Boulevard, Room 9329, Philadelphia, PA
19104, USA e-mail: [email protected]
32. 4 A.Y. Weintraub and L.G. Maxwell Asthma (reactive airways
disease) is one of the most common chronic diseases in children and
many periopera- tive procedures can exacerbate the disease. These
include induction and emergence from anesthesia and endotracheal
intubation. As with all chronic conditions, asthma should be
optimally medically managed prior to presenting for an operation or
anesthesia. In addition to regular appropriate use of controller
medications (inhaled corticosteroids,
intermediate-actingbronchodilators,leukotrienemodifiers), we
recommend that patients with asthma use their bron- chodilators
every 6h for 48h prior to anesthesia to mini- mize perioperative
bronchospasm. A history of a recent flare requiring oral
corticosteroids suggests poorly con- trolled disease and might
warrant delay of an elective pro- cedure until better control is
achieved. Some feel it is best to wait 46 weeks after an acute
exacerbation for associ- ated airway hyperreactivity to return to
baseline. Patients with persistent poorly controlled reactive
airways disease should be referred to their primary health care
provider or pulmonologist for strategies to improve their status.
These strategies sometimes include the administration of oral
corticosteroids. Children often have loose teeth as they transition
from their primary to secondary dentition, or due to poor oral
hygiene or an underlying disorder such as osteogenesis imperfecta
or ectodermal dysplasia. There is a significant risk of aspirating
a tooth that is accidentally displaced during orotracheal
intubation, so loose teeth should be electively removed at
induction. In some cases, it is best to recommend a preoperative
visit to a dentist. Obstructive sleep apnea is seen commonly in
patients with adenotonsillar hypertrophy, obesity, and some syn-
dromes. Symptoms (snoring, daytime somnolence), results of sleep
studies, and the need for noninvasive ventilation (CPAP, BIPAP)
should be included in the preoperative assessment as airway
obstruction should be anticipated in the postoperative period,
often making inpatient observation and monitoring necessary. One of
the most common questions confronting an anes- thesiologist is
whether to cancel a procedure because of an upper respiratory
infection. This can be a vexing problem for all parties involved,
and the decision is often a difficult one to make with confidence.
The patient with current or recent URI undergoing general
anesthesia is theoretically at increased risk of postoperative
respiratory complications, including lar- yngospasm, bronchospasm,
hypoxia, and apnea, with the patients under 2 years of age being at
greatest risk. However, anesthetic management can also be tailored
to reduce stimu- lation of a potentially hyperreactive airway. In
addition, can- cellation of a procedure can impose an emotional or
economic burden on the patient, family, physician, and hospital or
ambulatory surgical facility. Unless the patient is acutely ill, it
is often acceptable to proceed with the anesthetic. Patients with
high fever, wheezing, or productive cough may actually have a lower
respiratory tract infection and surgery is more likely to be
cancelled. Our approach is to discuss the urgency of the planned
procedure with the surgeon and to review the risks and benefits of
proceeding or rescheduling with the par- ents, including the
possibility that the child may have another URI at the time of the
rescheduled procedure. Allowing the Table1.1 American Society of
Anesthesiology (ASA) physical status (PS) classifications
Classification Definition Example PS 1 Normal healthy person PS 2
Mild systemic disease without functional limitations
Well-controlled asthma PS 3 Severe systemic disease Acute
lymphocytic leukemia PS 4 Severe systemic disease that is a
constant threat to life Extreme prematurity PS 5 Moribund patient,
unexpected to survive without the procedure Congenital heart
disease for initiation of ECMO PS 6 Brain-dead patient for organ
procurement E Suffix added for emergent procedures Table1.2
Syndromes and craniofacial abnormalities associated with difficult
ventilation or intubation Syndrome Associated airway features Apert
Craniosynostosis, midface hypoplasia BeckwithWiedemann syndrome
Macroglossia Crouzon Craniosynostosis, midface hypoplasia
FreemanSheldon (whistling face) syndrome Microstomia Goldenhar
syndrome Hemifacial microsomia, mandibular hypoplasia (uni- or
bilateral) KlippelFeil syndrome Limited cervical mobility
Mucopolysaccharide storage disorders Redundant facial, pharyngeal,
and supraglottic soft tissue; neck immobility Pierre-Robin sequence
Micrognathia, glossoptosis, cleft palate Treacher-Collins syndrome
Maxillary/mandibular hypoplasia Trisomy 21 (Down syndrome)
Macroglossia, subglottic stenosis, midface hypoplasia
33. 51 Preoperative Assessment and Preparation parents to
participate in the decision-making process when appropriate usually
leads to mutual satisfaction among all parties involved. The
patient with a difficult airway might require advanced airway
management techniques, which often necessitates additional OR time
and, in some cases, a planned period of postoperative mechanical
ventilation and an ICU stay. The laryngeal mask airway is now being
used routinely for general anesthesia. This technique allows the
patient to breathe spontaneously, with or without pressure support
from the anesthesia machine, and, in most cases, neuromus- cular
blocking agents are not used. Therefore, it is usually used for
cases where skeletal muscle relaxation is not needed for safe
conduct of the operation. Any requirement for mus- cle relaxation
should be discussed with the anesthesiologist in advance.
Cardiovascular At the time of the presurgical evaluation, up to 90%
of children are found to have an innocent murmur, probably due to
turbu- lent flow at the aortic or pulmonary roots or in the
subclavian or pulmonary arteries. Most of these children do not
require a car- diology consultation and can be safely observed.
These mur- murs are frequently episodic and are associated with a
normally split second heart sound, normal exercise tolerance, and
normal electrocardiogram. Concomitant medical problems such as
anemia and fever augment audibility of innocent murmurs because
they increase cardiac output. Nevertheless, a thorough history and
physical examina- tion will occasionally reveal findings that raise
greater con- cern in a child with a murmur: an infant with failure
to thrive or diaphoresis or tachypnea during feedings, or the older
child with dyspnea, tachypnea, exercise intolerance, or syn- cope.
These findings warrant further evaluation, including an
electrocardiogram, chest X-ray, consultation with a pediatric
cardiologist, and, in some cases, an echocardiogram. Children with
congenital heart disease frequently undergo a general surgical
procedure. Assessment of the childs cur- rent health status
includes a full history and physical exami- nation and recent
evaluation by the childs cardiologist. This communication should
include: a full description of the original lesion, documentation
of any procedures performed for palliation or repair, residual
abnormalities such as an intracardiac shunt or valve abnormality,
current functional status, and results of the most recent
echocardiogram. Knowledge of the childs cardiac anatomy is
essential to assess the risk of paradoxical emboli and
endocarditis. Revised recommendations for antibiotic prophylaxis
that are substantially different from those promulgated over the
past 50 years were recently published by the American Heart
Association. Specifically, genitourinary and gastrointestinal
procedures have been eliminated from those requiring pro- phylaxis
and prophylaxis for dental and respiratory tract pro- cedures is
restricted to patients with: (1) unrepaired cyanotic congenital
heart disease, (2) congenital heart defect repaired with prosthetic
material, (3) cardiac transplantation, or (4) a history of
endocarditis. Endotracheal intubation itself is not an indication
for antibiotic prophylaxis (Table1.3). Patients with
hemodynamically insignificant lesions such as bicuspid aortic valve
or mitral valve prolapse no longer require pro- phylaxis for any
procedure. Patients with congenital heart disease repaired with
prosthetic material require prophylaxis only for the first 6 months
after repair, after which time endothelialization will have
occurred. This is true for VSD as well as ASD repairs as long as
there is no residual defect. Patients with prosthetic valves or
those palliated with shunts or conduits require prophylaxis. Some
cardiologists differ with these new guidelines. It is therefore
advisable to request a recommendation from the childs cardiologist
based on the childs condition and planned procedure. Although
antibiotic prophylaxis is frequently administered orally to adults,
it is usually given intravenously in children. When indicated, our
practice is to give the antibiotic intravenously at induction of
anesthesia, because the surgical preparation time generally allows
sufficient time to achieve adequate blood levels before the
incision is made. Starting an intravenous catheter in an awake
child solely to administer antibiotics for antibiotics is rarely,
if ever, necessary. Surgical patients with long QT syndrome (LQTS),
in which ion channels involved in repolarization function Table1.3
Cardiac conditions for which prophylaxis with dental or respiratory
tract procedures is recommended Congenital heart disease (CHD)a
Unrepaired cyanotic CHD, including palliative shunts and conduits
Completely repaired congenital heart defect with prosthetic
material or device, whether placed by surgery or by catheter
intervention, during the first 6 months after the procedureb
Repaired CHD with residual defects at the site or adjacent to the
site of a prosthetic patch or prosthetic device (which inhibit
endothelialization) Cardiac transplantation recipients who develop
cardiac valvopathy Prosthetic cardiac valves Previous infective
endocarditis a Except for the conditions listed above, antibiotic
prophylaxis is no longer recommended for any other form of CHD b
Prophylaxis is recommended because endothelialization of prosthetic
material occurs within 6 months of the procedure Source: data from:
Wilson W, Taubert KA, Gewitz M etal. Prevention of infective
endocarditis. Guidelines from the American Heart Association
Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee,
Council on Cardiovascular Disease in the Young, and the Council on
Clinical Cardiology, Council on Cardiovascular Surgery and
Anesthesia, and Quality of Care and Outcomes Research
Interdisciplinary Working Group. Circulation. Apr 2007;
doi:10.1161/CIRCULATIONAHA. 106.183095
34. 6 A.Y. Weintraub and L.G. Maxwell abnormally due either to
a congenital defect or drug effect, are at risk for torsades de
pointes, a potentially life-threatening ventricular tachycardia.
Congenital LQTS occurs in 1:5,000 individuals and can present at
any age with syncope, seizures or sudden cardiac death, usually
after an increase in sympa- thetic activity such as exercise or
emotional stress. Because volatile anesthetic agents and surgical
stress increase the risk of developing ventricular tachycardia, a
preoperative electro- cardiogram should be obtained in patients who
are symp- tomatic, have a family history of sudden death, or are
taking drugs which predispose to the condition (www.azcert.org/
medical-pros/drug-lists/drug-lists.cfm). A QTc of more than 470 ms
in males and 480 ms in females is diagnostic of LQTS. Cardiology
consultation should be obtained as preop- erative medical treatment
might be necessary. Any patient with congenital heart disease,
cardiomyopathy, arrhythmia, or unexplained syncope requires a
thorough cardi- ology evaluation before undergoing an elective
surgical proce- dure, especially one that requires a general
anesthetic. In fact, anesthetists at most institutions will require
that a letter of car- diology clearance be included in the medical
record before the day of surgery. This letter is written by the
consulting cardiolo- gist and should include a detailed discussion
of the anatomy of the defect, the current medical regimen, and
specific recom- mendations regarding the peri-operative care of the
patient. Gastroesophageal Reflux Disease The majority of infants
and a significant number of children have some degree of
gastroesophageal reflux and the diagnosis of gastroesophageal
reflux disease is increasing. Symptoms of GERD in infants and
children differ substantially from those seen in adults and are
often primarily respiratory in nature: cough, wheezing, or
pneumonitis. Yet, despite a theo- retical increase in the risk of
aspiration of gastric contents during the induction of anesthesia,
children with a history of GERD do not have an increased incidence
of pulmonary aspi- ration as long as fasting guidelines have been
followed. Unless there is a history of aspiration when fasting, an
intravenous rapid sequence induction is not usually indicated.
Patient with GERD should be taking appropriate chemoprophylaxis H2
-blocker or proton pump inhibitor) as prescribed by their primary
physician or gastroenterologist. Obesity Obesity is an increasing
problem in children, with a recent estimated incidence of 15%. As
in adults, obese children have an increased incidence of
obstructive sleep apnea, which can be associated with adverse
respiratory events in the perioperative period. Problems during
induction include difficult mask ventilation. Preoperative
evaluation of chil- dren with a body mass index of 30 or greater
should include a careful history of snoring and daytime somnolence.
Patients with suspected obstructive sleep apnea should be referred
to a pulmonologist for a sleep study and considered for therapy
with a positive-pressure breathing device. In addition to airway
and respiratory complications, obese patients have been found to
have an increased incidence of postoperative complications such as
infection, wound com- plications, and deep venous thrombosis when
compared to children of normal weight. Diabetes Approximately 1 in
500 people under age 20 has diabetes, however complications
requiring surgical intervention, such as cardiovascular disease,
are extremely rare in this age group. Nevertheless, patients with
diabetes present for routine and emergent surgery with the same
frequency as nondiabetic patients and their underlying diabetes
must be addressed. As with any other chronic illness, the medical
management of diabetes should be optimized before elec- tive
surgery and a plan for perioperative glucose and insu-
linmanagementshouldbeformulatedbytheendocrinologist and
anesthesiologist in joint fashion. The stresses of sur- gery and
its effects on a regular schedule can wreak havoc on normally
well-controlled diabetes if not properly man- aged. The goal of
perioperative management is no longer merely avoiding
life-threatening hypoglycemia and severe hyperglycemia but to
maintain euglycemia to the extent possible. Regimens of multiple
injections of long- and short-acting insulin are still common, but
many patients with diabetes have insulin pumps that deliver a
continuous subcutaneous infusion with on-demand boluses for
carbohydrate intake or correction of hyperglycemia. Typical
management includes the usual preoperative fast with clear liquids
up until 2h before the operation. Whenever possible, it is usually
best to schedule the diabetic patient as the first case of the day.
After consultation with the patients endocrinologist, the insulin
dosage regimen most often includes reduction of the long- or
moderate-acting insulin dose with a reduced or skipped short-acting
insulin dose on the morning of surgery. Insulin pump infusions may
be continued up until the time of surgery. Blood sugar should be
checked upon arrival. Hypoglycemia requires intervention but oral
treatment might require delaying the procedure due to fasting
guide- lines. Hyperglycemia (>250 mg/dL) should be treated
withsubcutaneous insulin or a bolus via the insulin pump.
35. 71 Preoperative Assessment and Preparation The presence of
urine ketones will usually lead to cancellation or delay of an
elective procedure. Intra-operative management depends on the
length of the procedure. Many institutions consider insulin pumps
unau- thorized medical devices and prohibit their use. For outpa-
tient procedures that take