Atlas of Pediatric Surgical Techniques 9781336241565, 133624156X, 9788480865562, 8480865563

Citation preview

Atlas of Pediatric Surgical Techniques

ERRNVPHGLFRVRUJ

Other Volumes in the Surgical

Techniques Atlas Series

Atlas of Endocrine Surgical Techniques Edited by Quan-Yang Duh, MD, Orlo H. Clark, MD, and Electron Kebebew, MD

Atlas of Breast Surgical Techniques Edited by V. Suzanne Klimberg, MD

Atlas of Surgical Techniques for the Upper Gastrointestinal Tract and Small Bowel Edited by Jeffrey R. Ponsky, MD, and Michael J. Rosen, MD

Atlas of Thoracic Surgical Techniques Edited by Joseph B. Zwischenberger, MD

Atlas of Cardiac Surgical Techniques Edited by Frank W. Selke, MD, and Marc Ruel, MD

Atlas of Minimally Invasive Surgical Techniques Edited by Stanley W. Ashley, MD, and Ashley Haralson Vernon, MD

Atlas of Trauma/Emergency Surgical Techniques Edited by William Cioffi, Jr., MD

Atlas of Surgical Techniques for the Colon, Rectum, and Anus Edited by James W. Fleshman, MD

Atlas of Surgical Techniques for the Hepatobiliary Tract and Pancreas Edited by Reid B. Adams, MD

Atlas of Pediatric Surgical Techniques A Volume in the Surgical Techniques Atlas Series Editors Dai H. Chung, MD

Professor and Lee Endowed Chair in Pediatric Surgery Department of Pediatric Surgery Professor, Department of Cancer Biology Vanderbilt Children’s Hospital Vanderbilt University Medical Center Nashville, Tennessee

Mike K. Chen, MD

Professor, Department of Surgery and Pediatrics Director, Division of Pediatric Surgery Program Director, Pediatric Surgery Fellowship Children’s Hospital of Alabama University of Alabama at Birmingham Birmingham, Alabama

Series Editors Courtney M. Townsend, Jr., MD

Professor and John Woods Harris Distinguished Chairman Robertson-Poth Distinguished Chair in General Surgery Department of Surgery The University of Texas Medical Branch Galveston, Texas

B. Mark Evers, MD

Professor and Vice-Chair for Research Department of Surgery Markey Cancer Foundation Endowed Chair Director, Markey Cancer Center University of Kentucky Lexington, Kentucky

ERRNVPHGLFRVRUJ

1600 John F. Kennedy Blvd. Ste 1800 Philadelphia, PA 19103-2899

ATLAS OF PEDIATRIC SURGICAL TECHNIQUES

ISBN: 978-1-4160-4689-9

Copyright © 2010 by Saunders, Inc., an imprint of Elsevier Inc. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Details on how to seek permission, further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions. This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein).

Notices Knowledge and best practice in this field are constantly changing. As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary. Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein. In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility. With respect to any drug or pharmaceutical products identified, readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications. It is the responsibility of practitioners, relying on their own experience and knowledge of their patients, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions. To the fullest extent of the law, neither the Publisher nor the authors, contributors, or editors, assume any liability for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein. ISBN: 978-1-4160-4689-9

Acquisitions Editor: Judith Fletcher Developmental Editor: Rachel Miller Publishing Services Manager: Patricia Tannian Senior Project Manager: John Casey Designer: Steven Stave

Working together to grow libraries in developing countries Printed in China Last digit is the print number: 9  8  7  6  5  4  3  2  1 

www.elsevier.com | www.bookaid.org | www.sabre.org

Contributors Maria H. Alonso, MD

Dai H. Chung, MD

Associate Professor of Surgery Surgical Assistant Director Liver Transplantation Division of Pediatric & Thoracic Surgery Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Professor and Lee Endowed Chair in Pediatric Surgery Department of Pediatric Surgery Professor, Department of Cancer Biology Vanderbilt Children’s Hospital Vanderbilt University Medical Center Nashville, Tennessee

Richard G. Azizkhan, MD, PhD (Hon)

Andrew M. Davidoff, MD

Surgeon-in-Chief Lester W. Martin Chair in Pediatric Surgery Professor of Surgery and Pediatrics Cincinnati Children’s Hospital Medical Center University of Cincinnati College of Medicine Cincinnati, Ohio

Professor, Department of Surgery and Pediatrics University of Tennessee College of Medicine Chairman, Department of Surgery St. Jude Children’s Research Hospital Memphis, Tennessee

Naira Baregamian, MD, MMS Pediatric Surgery Research Fellow Department of Surgery The University of Texas Medical Branch Galveston, Texas

Elizabeth A. Beierle, MD

Bryan J. Dicken, MSc, FRCSC, FAAP Assistant Professor of Surgery Division of Pediatric Surgery Stollery Children’s Hospital Edmonton, Alberta, Canada

Belinda Hsi Dickie, MD, PhD

Associate Professor of Surgery and Pediatrics University of Alabama at Birmingham Birmingham, Alabama

Colorectal Fellow Division of Pediatric General and Thoracic Surgery Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Deborah F. Billmire, MD

Richard A. Falcone, Jr., MD, MPH

Professor Department of Surgery, Section of Pediatric Surgery Indiana University Attending Surgeon James Whitcomb Riley Hospital for Children Indianapolis, Indiana

Associate Professor of Surgery Division of Pediatric and Thoracic Surgery Cincinnati Children’s Hospital Medical Center Department of Surgery, University of Cincinnati Cincinnati, Ohio

Mary L. Brandt, MD

Mary E. Fallat, MD

Professor, Michael E. DeBakey Department of Surgery Baylor College of Medicine Houston, Texas

Hirikati S. Nagaraj Professor and Division Chief Pediatric Surgery University of Kentucky Louisville, Kentucky

Mike K. Chen, MD

Jason S. Frischer, MD

Professor, Department of Surgery and Pediatrics Director, Division of Pediatric Surgery Program Director, Pediatric Surgery Fellowship Children’s Hospital of Alabama University of Alabama at Birmingham Birmingham, Alabama

Assistant Professor of Surgery University of Cincinnati College of Medicine Department of Pediatric General & Thoracic Surgery Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio v

vi    Contributors

John M. Gatti, MD

Michael D. Josephs, MD

Associate Professor Director of Minimally Invasive Urology Department of Surgery and Urology Children’s Mercy Hospital University of Missouri at Kansas City School of Medicine Kansas City, Missouri; Staff Surgeon Department of Surgery Children’s Mercy South Overland Park, Kansas; Staff Surgeon Department of Urology University of Kansas Medical Center Kansas City, Kansas

Attending Physician Dell Children’s Medical Center of Central Texas Austin, Texas

Michael J. Goretsky, MD Associate Professor of Clinical Surgery and Pediatrics Eastern Virginia Medical School Surgeon, Children’s Hospital of The King’s Daughters Norfolk, Virginia

Michael H. Hines, MD, FACS Associate Professor, Cardiothoracic Surgery and Pediatrics Wake Forest University School of Medicine Director of ECMO and Perfusion Services Wake Forest University Baptist Medical Center Winston-Salem, North Carolina

Ronald B. Hirschl, MD Professor of Pediatric Surgery Head, Section of Pediatric Surgery Surgeon-in-Chief, C.S. Mott Children’s Hospital University of Michigan Health System Ann Arbor, Michigan

Thomas H. Inge, MD, PhD, FACS, FAAP Associate Professor of Surgery and Pediatrics Surgical Director, Comprehensive Weight Management Center Director, Center for Bariatric Research and Innovation Division of Pediatric General and Thoracic Surgery Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Saleem Islam, MD, MPH Associate Professor Director, Pediatric Minimally Invasive Surgery Division of Pediatric Surgery, Department of Surgery University of Florida, College of Medicine Gainesville, Florida

Timothy D. Kane, MD Clinical Director Associate Professor of Surgery Division, Pediatric General and Thoracic Surgery Children’s Hospital of Pittsburgh University of Pittsburgh School of Medicine Pittsburgh, Pennsylvania

Akemi L. Kawaguchi, MD Assistant Professor of Clinical Surgery University of Southern California Keck School of Medicine Attending Surgeon, Pediatric Surgery Childrens Hospital Los Angeles Los Angeles, California

Anne C. Kim, MD, MPH Pediatric Surgery Research Fellow University of Michigan Ann Arbor, Michigan

Eugene S. Kim, MD, FACS, FAAP Assistant Professor of Surgery and Pediatrics Division of Pediatric Surgery Section of Hematology-Oncology Baylor College of Medicine Attending Surgeon Texas Children’s Hospital Houston, Texas

Keith A. Kuenzler, MD Assistant Professor of Surgery New York University School of Medicine Director, Minimally Invasive Surgery Division of Pediatric Surgery New York, New York

Jacob C. Langer, MD Professor of Surgery University of Toronto Faculty of Medicine Chief and Robert M. Filler Chair Paediatric General and Thoracic Surgery Hospital for Sick Children Toronto, Ontario, Canada

Contributors    vii

Marc Levitt, MD

Alberto Peña, MD

Associate Professor of Surgery University of Cincinnati Associate Director, Colorectal Center Cincinnati Children’s Hospital Cincinnati, Ohio

Professor of Surgery University of Cincinnati Director of Colorectal Center Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Peter B. Manning, MD

Thomas Pranikoff, MD

Professor of Surgery and Pediatrics University of Cincinnati College of Medicine Director, Cardiothoracic Surgery Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Associate Professor of Surgery and Pediatrics Section of Pediatric Surgery Wake Forest University School of Medicine Attending Surgeon Department of Pediatric Surgery Brenner Children’s Hospital Winston-Salem, North Carolina

Tory A. Meyer, MD Subspecialty Chief Pediatric Surgery Dell Children’s Medical Center of Central Texas Austin, Texas

Vincent Mortellaro, BS, MD Department of Surgery College of Medicine University of Florida Gainesville, Florida

J. Patrick Murphy, MD Professor of Surgery University of Missouri at Kansas City Chief of Section, Urology Department of Surgery Children’s Mercy Hospital Kansas City, Missouri

Jaimie D. Nathan, MD Assistant Professor of Surgery and Pediatrics Division of Pediatric and Thoracic Surgery Cincinnati Children’s Hospital Medical Center Department of Surgery University of Cincinnati College of Medicine Cincinnati, Ohio

Donald Nuss, MB, ChB, FRCSC, FACS Professor of Clinical Surgery and Pediatrics Eastern Virginia Medical School Surgeon, Children’s Hospital of The King’s Daughters Norfolk, Virginia

Frederick J. Rescorla, MD Professor of Surgery Indiana University School of Medicine Surgeon-in-Chief Department of Surgery Riley Hospital for Children Indianapolis, Indiana

Marleta Reynolds, MD Lydia J. Fredrickson Professor of Pediatric Surgery Northwestern University Feinberg School of Medicine Children’s Memorial Hospital Chicago, Illinois

Richard Ricketts, MD Professor of Surgery Emory University School of Medicine Chief, Division of Pediatric Surgery Children’s Healthcare of Atlanta Atlanta, Georgia

Frederick C. Ryckman, MD Professor of Surgery University of Cincinnati College of Medicine Pediatric Surgeon Division of Pediatric General and Thoracic Surgery Vice President for System Capacity and Peri-Operative Operations Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Bradley J. Segura, MD, PhD Daniel J. Ostlie, MD Professor of Surgery Children’s Mercy Hospital and Clinics Kansas City, Missouri

Assistant Professor of Surgery Department of Pediatric Surgery St. Louis Children’s Hospital St. Louis, Missouri

viii    Contributors

Robert C. Shamberger, MD

Greg M. Tiao, MD

Robert E. Gross Professor of Surgery Department of Surgery Harvard Medical School Chief of Surgery Children’s Hospital Boston Boston, Massachusetts

Associate Professor of Surgery Director, Liver Transplantation Division of Pediatric & Thoracic Surgery Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Stig Somme, MD Assistant Professor Department of Pediatric Surgery The Children’s Hospital University of Colorado, Denver Medical School Aurora, Colorado

Shawn D. St. Peter, MD Director, Center for Prospective Clinical Trials Department of Surgery Children’s Mercy Hospital Kansas City, Missouri

Charles J.H. Stolar, MD Director, Division of Pediatric Surgery Surgeon-in-Chief Morgan Stanley Children’s Hospital New York–Presbyterian Hospital Columbia University Medical Center New York, New York

Daniel H. Teitelbaum, MD Professor of Pediatric Surgery Mott Children’s Hospital University of Michigan Ann Arbor, Michigan

Daniel von Allmen, MD Director, Division of Pediatric General and Thoracic Surgery Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Brad W. Warner, MD Jessie L. Ternberg Distinguished Professor of Pediatric Surgery Department of Surgery Washington University School of Medicine Surgeon-in-Chief St. Louis Children’s Hospital St. Louis, Missouri

Mark L. Wulkan, MD Surgeon-in-Chief Children’s Healthcare of Atlanta Associate Professor of Surgery and Pediatrics Program Director, Pediatric Surgery Emory University School of Medicine Atlanta, Georgia

To our families Kimberleye, Camryn, Kaley, Elizabeth And all infants and children we care for

This page intentionally left blank

Foreword “A picture is worth a thousand words.” —Anonymous This atlas is for practicing surgeons, surgical residents, and medical students for their review and preparation for surgical procedures. New procedures are developed and old ones are replaced as technologic and pharmacologic advances occur. The topics presented are contemporaneous surgical procedures with step-by-step illustrations, along with the preoperative and postoperative considerations, as well as pearls and pitfalls taken from the personal experience and surgical practice of the authors. Their results have been validated in their surgical practices involving many patients. Operative surgery remains a manual art in which the knowledge, judgment, and technical skill of the surgeon come together for the benefit of the patient. A technically perfect operation is the key to this success. Speed in operation comes from having a plan and devoting sufficient time to completion of each step in order, one time. The surgeon must be dedicated to spending the time to do it right the first time; if not, there will never be enough time to do it right at any other time. Use this atlas, study it for your patients. “An amateur practices until he gets it right; a professional practices until she can’t get it wrong.” —Anonymous COURTNEY M. TOWNSEND, JR., MD B. MARK EVERS, MD

xi

This page intentionally left blank

Preface Pediatric surgery remains as a broad general surgical specialty caring for infants and children. Pediatric surgeons are faced with the challenges of dealing with a wide spectrum of complex pathology involving multiple organ systems in patients ranging from neonates to young adults. Despite surgeons having 2 years of rigorous clinical fellowship training, numerous pediatric surgical procedures are handled only occasionally due to the infrequency of a disease process. Conversely, other commonly performed procedures are not routine in a variety of patients, from very small premature neonates to extremely large adolescents, and therefore a thorough grasp of knowledge of surgical anatomy and physiology is required to operate safely. Mastery of all pediatric surgical procedures is a demanding lifelong learning process that requires refining operative techniques throughout a surgical career. For young surgical residents in training, as well as established surgeons who may only occasionally manage delicate pediatric surgical patients, concise and clear illustrations of pediatric surgical procedures can be instrumental to the careful preoperative planning and precise completion of operations. In this first edition of Atlas of Pediatric Surgical Techniques, we have put together a collection of a broad spectrum of pediatric surgical operations. The key steps of the procedure are described in a concise bullet-style format, highlighted by colorful illustrations and intraoperative photographs. Each chapter includes interesting Pearls and Pitfalls and a short list of pertinent references. Contributing authors come from all facets of our field, from academic centers to private surgical groups, each sharing a wealth of knowledge acquired from years of experience to illustrate pediatric surgical techniques. We believe this comprehensive atlas will nicely complement standard textbooks in pediatric surgery. We would like to thank our contributing authors and acknowledge the dedicated professionalism of our colleagues at Elsevier. Developmental Editors Rachel Miller and Sarah Myer, and Publishing Director Judith Fletcher have been instrumental to the completion of this atlas. DAI H. CHUNG, MD MIKE K. CHEN, MD

xiii

This page intentionally left blank

Contents Section

I General

CHAPTER

1

Vascular Access, 2 Deborah F. Billmire

CHAPTER

2

Extracorporeal Membrane Oxygenation Cannulation, 14 Thomas Pranikoff and Michael H. Hines

Section

II Head and Neck

CHAPTER

3

Thyroglossal Duct Cyst, 28 Michael D. Josephs

CHAPTER

4

Branchial Anomalies, 34 Tory A. Meyer

Section

III Thoracic

CHAPTER

5

Esophageal Atresia with Tracheoesophageal Fistula, 44 Richard Ricketts

CHAPTER

6

Cervical Esophagostomy, 54 Richard Ricketts

CHAPTER

7

Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula, 59 Mark L. Wulkan

CHAPTER

8

Esophageal Replacement, 66 Belinda Hsi Dickie and Richard G. Azizkhan

CHAPTER

9

Esophageal Replacement: Gastric Tube Pull-Up, 76 Saleem Islam and Ronald B. Hirschl

CHAPTER

10 Congenital Diaphragmatic Hernia and Eventration

of the Diaphragm, 83

Jason S. Frischer, Keith A. Kuenzler, and Charles J. H. Stolar CHAPTER

11

Surgical Treatment of Chest Wall Deformities: Nuss Procedure, 97 Michael J. Goretsky and Donald Nuss

CHAPTER

12

Surgical Treatment of Chest Wall Deformities: Open Repair, 104 Robert C. Shamberger

CHAPTER

13

Patent Ductus Arteriosus, 113 Peter B. Manning

xv

xvi    Contents CHAPTER

14 Congenital Lung Anomalies, 120 Elizabeth A. Beierle and Mike K. Chen

Section

IV Abdomen

CHAPTER

15

Duodenal Obstruction, 132 Daniel von Allmen

CHAPTER

16 Malrotation: Ladd Procedure, 138 Brad W. Warner

CHAPTER

17

Meconium Disease, 143 Mary L. Brandt

CHAPTER

18 Hirschsprung Disease: Transanal Pull-Through, 151 Stig Somme and Jacob C. Langer

CHAPTER

19 Hirschsprung Disease: Soave (Open and

Laparoscopic-Assisted) and Duhamel Techniques, 161 Anne C. Kim and Daniel H. Teitelbaum

CHAPTER

20 Hirschsprung Disease: Swenson Pull-Through

Procedure, 177 Marleta Reynolds

CHAPTER

21

Imperforate Anus, 185 Marc Levitt and Alberto Peña

CHAPTER

22 Cloaca, 206 Marc Levitt and Alberto Peña

CHAPTER

23 Biliary Atresia, 220 Jaimie D. Nathan and Frederick C. Ryckman

CHAPTER

24 Choledochal Cysts, 232 Greg M. Tiao

CHAPTER

25 Gastroschisis and Omphalocele, 242 Richard A. Falcone, Jr.

CHAPTER

26 Laparoscopic and Open Pyloromyotomy, 253 Shawn D. St. Peter and Daniel J. Ostlie

CHAPTER

27 Anti-Reflux Procedures, 266 Bradley J. Segura and Timothy D. Kane

CHAPTER

28 Bariatric Surgery, 279 Thomas H. Inge

CHAPTER

29 Splenectomy, 290 Vincent Mortellaro, Elizabeth A. Beierle, and Mike K. Chen

Section

V Genitourinary

CHAPTER

30 Inguinal Hernias and Hydroceles, 303 Akemi L. Kawaguchi and Eugene S. Kim

CHAPTER

31

Cryptorchidism, 316 Mary E. Fallat

CHAPTER

32 Testicular Torsion, 328 John M. Gatti and J. Patrick Murphy

Contents    xvii Section

VI Tumors

CHAPTER

33 Wilms Tumor, 339 Andrew M. Davidoff

CHAPTER

34 Neuroblastoma, 355 Naira Baregamian and Dai H. Chung

CHAPTER

35 Sacrococcygeal Teratoma, 364 Bryan J. Dicken and Frederick J. Rescorla

CHAPTER

36 Hepatic Tumors, 374 Maria H. Alonso

This page intentionally left blank

SECTION

I

General

CHAPTER

1 

Vascular Access Deborah F. Billmire

Step 1: Surgical Anatomy



The six central veins include the internal jugular, subclavian, and femoral veins. In most children, these are symmetric and paired.  Children with congenital heart disease, splenia syndromes, and variants of esophageal atresia have an increased incidence of anatomic variants in the subclavian veins that are relevant to central access procedures (Fig. 1-1).  Children with congenital heart disease and situs abnormalities have an increased incidence of variants of the inferior vena cava that are relevant (Fig. 1-2).

Step 2: Preoperative Considerations



What is the purpose of the access, and could therapy be achieved without central access? How long will the access be needed, and would a nontunneled or tunneled line be more appropriate?  Does the patient have current evidence of infection?  Does the patient have clinical indicators of coagulopathy or receive any medications that impact coagulation status?  Has the patient had previous central lines that may have resulted in venous thrombosis?  Can the patient be taken to the operating room so that optimal sterile conditions and fluoroscopy will be available? 

Step 3: Operative Steps

1.  General Concepts



2

In general, insertion of most central lines in children is best achieved in the operating room with the patient under anesthesia and using fluoroscopy. It is not uncommon, however, to

Chapter 1  •  Vascular Access   3

Left innominate vein Right innominate vein

Left SVC Right SVC Azygous vein

Right Atrium

Left SVC

Left Atrium

Coronary sinus

IVC

A Figure 1-1 

Right SVC

B

Normal

Isolated left superior vena cava

Coronary sinus

C

Persistent left superior vena cava (with normal superior vena cava)

IVC

Normal

A Figure 1-2 

Double IVC (below renal veins)

B

Double IVC (previously called Absent IVC) (below renal veins; persistence of azygos/hemiazygus above renal veins) C

4   Section I  •  General be asked to provide a central line in the emergency room or intensive care unit for an unstable patient who requires immediate access and is not stable enough for transport.  In these patients, the use of bedside Doppler examination or ultrasound may be helpful in assessing position and patency of veins. This is particularly useful in children who have had multiple previous central lines. With Doppler probe, a good venous signal that varies with respiration suggests patency of the jugular and femoral systems, and the path of the signal may be mapped out using a skin marker. Good augmentation of the venous signal with compression of the leg also suggests patency of the femoral system.  Bedside ultrasound may also demonstrate a patent vein that can be mapped using a skin marker or observed directly during venipuncture if a sterile probe is available.  Operative records should be reviewed for information regarding previous placement of central lines.  Regardless of the method or site of placement, a confirming radiographic image of the final result should be obtained.  Catheter size and number of lumens should be minimized to reduce risk of thrombosis and infection.

2.  Temporary Central Lines



Temporary lines are inserted by percutaneous Seldinger technique directly into the vein and are generally acceptable for 2 to 3 weeks.  The vein is accessed percutaneously using a thin-walled needle. After aspiration of nonpulsatile venous blood, the wire is advanced well into the vein.  Fluoroscopy is done to confirm proper wire placement in the central vein.  If venous blood is obtained, but the wire does not advance easily and fluoroscopy is available, contrast solution may be injected either through the needle or after replacing the needle over the wire with an angiocatheter and confirming continued ability to aspirate blood. This may demonstrate previously unrecognized thrombosis or congenital anatomic variants. If fluoroscopy is not available, repeat venipuncture, or an alternative site is needed.  Once the wire has easily passed, the dilator is placed over the wire just deep enough to allow the tip of the dilator to pass through the skin and soft tissues. The wire should remain inserted well beyond the tip of the dilator.  The dilator is removed, and the preflushed catheter is placed to an appropriate depth.  Precautions against venous embolism should be used at all sites.  Ability to aspirate blood and flush easily in all lumens should be confirmed before securing the line in place.

3.  Tunneled Central Lines



Tunneled lines are preferred for access anticipated to be longer than 2 to 3 weeks. These lines end as external catheters or as subcutaneous reservoirs (Fig. 1-3).  Percutaneous or cutdown technique may be used.  The entry site is enlarged enough to allow the catheter tubing to be brought from a separate exit site.  The exit site should be chosen several centimeters away in a location that will create a smooth pathway and a convenient place for the dressing. 

Chapter 1  •  Vascular Access   5

Figure 1-3 

6   Section I  •  General



The catheter may be either pulled through the tunnel antegrade with a blunt probe or pushed through the lumen of a Frazier tip sucker that is passed retrograde down the tunnel (Fig. 1-4).  The cuff is positioned 1 cm above the exit site to allow for later removal without the need for additional incision.  Once the catheter is tunneled to the exit site, it is cut to length.  For percutaneous access, the peelaway or obturator sheath is advanced over the wire with fluoroscopic assistance (Fig. 1-5).  The obturator and wire are removed, and the catheter is advanced down the sheath (Fig. 1-6).  The sheath is peeled away, and fluoroscopy is used again to confirm the proper course and position of the catheter (Fig. 1-7).  Ability to aspirate and flush the catheter is confirmed.  The entry-site wound is closed with subcuticular monofilament suture.  A single nonabsorbable monofilament suture is used to secure the catheter at the exit site after confirming ability to aspirate and flush all lumens.  For subcutaneous reservoirs, the reservoir should be placed in a flat location and secured to the fascia.

4.  Access by Anatomic Site

Neck  The available sites in the neck include the internal jugular and external veins (Fig. 1-8). The external jugular is accessed by cutdown technique and the internal jugular by either cutdown or percutaneous method.  The patient is positioned with a transverse roll beneath the shoulders to achieve mild extension, and the head is turned slightly to the side opposite the planned access. Skin preparation should include the anterior chest and both sides of the neck.  For all neck sites, the patient is placed in Trendelenburg position. External Jugular Cutdown  Most usable external jugular veins are visible on inspection. The vein overlies the sternocleidomastoid muscle. A small transverse incision is made in midneck directly over the visible vein.  With a minimal amount of blunt dissection, the vein is identified and fine absorbable ties are passed for proximal and distal control.  When the catheter is brought through the tunnel, care is taken to ensure that the tubing enters the neck incision in a gentle arc instead of a sharp turn that will kink the catheter.

Chapter 1  •  Vascular Access   7

A

B

Figure 1-4 

Figure 1-5 

Figure 1-6 

External jugular vein

Facial vein

Internal jugular vein Subclavian vein

Figure 1-7 

Figure 1-8 

8   Section I  •  General



The tubing is cut to length by following the expected course from the incision, to the clavicle, to the midline, and to the angle of Louis. A bevel is made to facilitate introduction into the vein.  The distal ligature is tied. It is helpful to place a few drops of lidocaine on the vessel to reduce vasospasm. An anterior venotomy is made, and the catheter is threaded into the vein.  Occasionally the catheter will be difficult to pass at the subclavian junction. In this situation it is often helpful to withdraw the catheter until the bevel is seen, rotate it 180 degrees, and repass the catheter.  Confirmation of the catheter in the superior vena cava by fluoroscopy should be obtained, and the catheter should aspirate and flush easily.  The proximal tie is ligated around the catheter and vein, and the neck wound is closed.  The catheter is secured at the exit site. Internal Jugular Cutdown  Preparation and position are similar to that for external jugular cutdown. If the procedure was initiated as an external jugular and the vein is not suitable, the same incision can be extended slightly for better exposure.  Blunt dissection should be carried through the sternocleidomastoid muscle. If this is done between the sternal and clavicular heads, dissection is minimized and the vein is visible just deep to the muscle.  Careful blunt dissection is done to isolate the vein, and proximal and distal control is achieved using silk ties. If a visible facial branch is seen, this may be controlled and is used as the point of access to the vein. In small neonates, this branch may be too small or angled for convenient use.  The catheter is drawn through the tunnel to the neck wound, taking care to make a gentle arc.  The catheter length is chosen as a path directly from the incision to the angle of Louis and cut with a slight bevel.  There is no need to ligate the vein in almost all patients, including small premature infants. The stay sutures are placed under traction, and a pursestring suture of 6-0 Prolene is placed on the anterior wall of the vein.  A small venotomy is made, and the catheter is introduced and threaded into the vein.  Fluoroscopy is done to confirm catheter position, and ability to aspirate and flush the catheter is assured.  The pursestring suture is tied down securely, but ability to slide the catheter at the venotomy site is confirmed.  The muscle is closed over the arc of the catheter to minimize mobility of the catheter. The subcutaneous tissue and skin are closed.  The catheter is secured at the exit site.

Chapter 1  •  Vascular Access   9

Internal Jugular, Percutaneous  In adolescent patients, it is sometimes helpful to have the patient elevate the head from the bed before anesthesia is administered. The separate heads of the sternocleidomastoid muscle are more easily seen, and the triangular space formed by their adjacent bundles is visible for marking. This can be particularly helpful when placing the larger hemodialysis catheters.  The patient is positioned supine with a generous transverse roll beneath the shoulders and the neck well extended. The head is turned to the opposite side.  If ultrasound or Doppler is not used, the palpable carotid pulse serves as a landmark. The needle enters the skin at the apex of the muscular triangle lateral to the pulse at a 30-degree angle from the skin and is slowly advanced while aspirating. The trajectory should follow a course aiming at the ipsilateral nipple. When venous blood is obtained, the wire is placed through the needle, and fluoroscopy is used to confirm venous position.  For a tunneled line, the exit site is planned for a flat area on the anterior chest wall below the clavicle. Subclavian Vein, Percutaneous  In children, the subclavian vein is the most commonly used percutaneous access. The right side is preferable, if possible. The left side may be used if there is a recent history of infected right-side line, ventriculoperitoneal shunt, venous occlusion, or difficulty in accessing the right side.  The patient is positioned supine in Trendelenburg with a transverse roll beneath the shoulder to achieve a neutral position of the shoulders. The neck is extended with the head in the midline.  Skin preparation should include both sides of the chest and the neck.

10   Section I  •  General



By palpation, the “crook” of the clavicle is identified. The needle is introduced through the skin just lateral to the crook and directed at the inferior margin of the clavicle. As soon as the needle is under the margin of the clavicle, the tip should be aimed at the sternal notch and slowly advanced using continuous suction. The trajectory of the needle should be parallel with the floor, and no pressure on the needle should be needed to maintain position (Fig. 1-9).  When venous blood is obtained, the wire should be passed through the needle with fluoroscopic confirmation of venous position.  If the wire passes to the opposite subclavian vein, withdraw the tip of the wire back to the ipsilateral subclavian, place a finger in the sternal notch, and press downward to distort the innominate junction and advance the wire.  If the wire passes to the ipsilateral jugular, withdraw the tip back to the ipsilateral subclavian, rotate the head to the ipsilateral side, press downward with a finger at the base of the neck just medial to the sternocleidomastoid, and advance the wire.  For tunneled lines, the exit site is most commonly on the anterior chest wall. For active toddlers, it is sometimes helpful to exit on the upper back by tunneling over the shoulder (Fig. 1-10). Femoral Vein  The femoral vein is used less often because of concerns regarding contamination in infants and toddlers and concern about kinking and obstruction in patients who are old enough to sit. It is more likely to be used in emergent situations in the intensive care unit or in patients with a coagulopathy. Access by cutdown or percutaneous technique is possible (Fig. 1-11).  Femoral cutdown via the saphenous vein  The patient is placed supine with the hips flat.  A wide preparation is done to include both groins and the abdomen up to the umbilicus.  A small transverse incision is made medial to the pulse 1 cm below the inguinal ligament in infants and 2 to 3 cm below the inguinal ligament in children or adolescents.  Dissection is carried down through subcutaneous tissue to identify the saphenous vein.  The vein is isolated and controlled proximally and distally with fine absorbable ties. Lidocaine is dripped onto the vessel to reduce spasm.  A tunneled line of matched size is brought through a separate exit site to the incision. In most cases, it is advantageous to have the tunnel on the abdominal wall so that the exit site is above the diaper.  The vein is ligated distally, and a small venotomy is made.  The catheter is trimmed to length (incision to costal margin) and introduced through the venotomy.  Fluoroscopy is used to confirm position in the inferior vena cava below the diaphragm.  The catheter should aspirate and flush easily.  The proximal tie is ligated around the vein and catheter.  The incision is closed in layers with absorbable suture.  The catheter is secured at the exit site with monofilament suture and a secure sterile dressing is applied.

Chapter 1  •  Vascular Access   11

Figure 1-9 

Figure 1-10 

Ascending lumbar vein

Femoral vein

Saphenous vein

Figure 1-11 

12   Section I  •  General



Percutaneous femoral access Puncture the skin 1 or 2 cm below the inguinal ligament and medial to the palpable pulse. The needle should be angled 30 degrees above the skin, and the trajectory should point toward the umbilicus. Advance slowly, aspirating continually until venous blood is obtained. The wire is advanced under fluoroscopic guidance into the inferior vena cava.  The catheter is secured at the exit site with monofilament suture, and a secure sterile dressing is applied. 

Step 4: Postoperative Care



A confirming radiographic image should be done after all access procedures either with fluoroscopy or plain radiography.

Arterial Access

Radial Artery  The radial artery is the most frequently accessed peripheral artery.  Before cannulation, an Allen’s test should be done to confirm patency of collateral circulation. The radial and ulnar arteries are compressed simultaneously, followed by release of the ulnar compression. If the collateral circulation is adequate, the hand will become pink.  The hand should be taped to a padded arm board with a roll under the wrist to aid in extension.  The area is prepped with antiseptic. Using palpation of the pulse medial to the radial head or ultrasound guidance, the artery is accessed percutaneously with a 22- or 24-gauge angiocatheter, depending on the size of the patient. The catheter is advanced.  The angiocatheter is secured with tape, leaving the fingers exposed for monitoring.  The line should be removed as soon as it is no longer needed and should be removed promptly if there is any evidence of ischemia. Posterior Tibial Artery  The posterior tibial artery is a peripheral access site that is often used in small infants. It is palpable just posterior to the medial malleolus at the ankle.  The foot is restrained on a padded armboard with a roll under the ankle and the foot in gentle plantar flexion.  The area is prepared with antiseptic. Using palpation of the pulse posterior to the medial malleolus or ultrasound guidance, a 22- or 24-gauge angiocatheter is used to access the artery and is then advanced.  The catheter is taped in place, leaving the toes exposed for monitoring.  The line should be removed as soon as it is no longer needed and should be removed promptly if there is any evidence of ischemia.

Chapter 1  •  Vascular Access   13

Step 5: Pearls and Pitfalls



Tunneled silicone catheters that remain intravascular but become malpositioned with the tip in the internal jugular or opposite subclavian vein in many cases may be repositioned without return to the operating room. Under fluoroscopy, a burst injection of normal saline with volume limited to 2 to 5 mL through a Luer lock syringe may “flip” the tip back to proper position in the superior vena cava. Success of this technique has been seen with catheter sizes ranging from 2.7 to 7 French.  “Pinch-off sign” occurs when a subclavian catheter is inserted medially, causing compression of the catheter between the clavicle and first rib. This is visible on x-ray as a compressed segment of the catheter. It is associated with increased risk of catheter fracture with leakage or embolization.  Cardiac tamponade, although rare, may occur during placement of a central line by any approach or as a delayed complication from catheter migration. Any unexplained, abrupt change in cardiopulmonary status should raise suspicion of this problem, even if the chest x-ray is unchanged.  Femoral lines placed by percutaneous or cutdown technique may enter the lumbar venous plexus through the ascending lumbar veins. This may be suspected on plain x-ray by a catheter path in the midline overlying the spine and confirmed by a lateral film showing the catheter path posterior to the vertebral bodies.

Bibliography

Aitken DR, Minton JP. The “pinch-off sign”: A warning of impending problems with permanent subclavian catheters. Am J Surg 1984;148:633-636. Bagwell CE, Salzberg AM, Sonnino RE, et al. Potentially lethal complications of central venous catheter placement. J Pediatr Surg 2000;35(5):709-713. Lavandosky G, Gomez R, Montes J. Potentially lethal misplacement of femoral central venous catheters. Crit Care Med 1996;24(5):893-896. Mowery N, Billmire DF, Schamberger M, et al. Incidence of persistent left superior vena cava in esophageal atresia. J Pediatr Surg 2006;41:484-486. Singer RL, Wolfson PJ. Experience with umbilical artery cutdowns in neonates. Pediatr Surg Int 1990;5:295-297. Skandalakis JE. The superior and inferior venae cavae. In Skandalakis JE, Gray SW, eds. Embryology for surgeons, 2nd ed. Baltimore: Williams & Wilkins, 1994; pp. 1032-1051. Van Engelenburg KCA, Festen C. Cardiac tamponade: A rare but life-threatening complication of central venous catheters in children. J Pediatr Surg 1998;33:1822-1824. Warner BW, Ryckman FC. A simple technique to redirect malpositioned Silastic central venous catheters. J Parenter Enter Nutr 1992;16(5):473-476, 1992.

CHAPTER

2 

Extracorporeal Membrane Oxygenation Cannulation Thomas Pranikoff and Michael H. Hines

Step 1: Surgical Anatomy



Within the carotid sheath, the internal jugular vein is anterior and lateral and the common carotid artery is medial and posterior. The vagus nerve lies posterior and between these two structures.  In the femoral triangle, below the inguinal ligament, the femoral vein lies medial to the artery. More distally, the vein moves posterior to the artery.

Step 2: Preoperative Considerations—Patient Management before Extracorporeal Life Support



Patients who require extracorporeal life support (ECLS) are critically ill, and proper preparation before initiating ECLS is challenging.  Adequate monitoring and nursing care are essential, and required equipment (cannulas, surgical instruments, circuit and components) and personnel (operating room and ECLS) must be available.  The ability to transport the patient safely with adequate ventilation and hemodynamic support should be considered.  The decision of where to cannulate the patient (e.g., in the intensive care unit [ICU], operating room [OR], emergency department) needs to be thought out carefully.  Most institutions will have pre-ECLS orders that need to be initiated, including ordering blood and platelets.  The patient should be anesthetized to facilitate safe cannulation, avoid anxiety and discomfort, and reduce the likelihood of air embolus. We use a combination of fentanyl and rocuronium.  After the vessels have been surgically exposed or a guidewire placed for percutaneous access, the patient is anticoagulated with heparin (100 units/kg for 3 minutes) before cannulation.

14

Chapter 2  •  Extracorporeal Membrane Oxygenation Cannulation   15

Type of Support



Extracorporeal support is provided in two principal ways:  Venovenous (VV) bypass, which provides excellent respiratory support  Venoarterial (VA) bypass, which provides both cardiac and respiratory support.  VA bypass removes blood from the systemic venous circulation, usually from the right atrium via the right internal jugular vein, and returns the blood to the systemic arterial circulation in the aortic arch via the right common carotid artery.  In VV bypass, blood is drained from the venous circulation and returned to the venous circulation either through a single double-lumen catheter in the right atrium via the jugular vein or by using two cannulas in the jugular and femoral veins.  Most cases of respiratory failure can be managed with VV bypass if cardiac function is adequate. This may be difficult to determine in the typical hypoxemia patient who is on highpressure ventilation, which depresses cardiac function.  After ECLS is begun and airway pressures are decreased, cardiac output increases and inotropic support can usually be weaned. VV bypass offers several advantages over VA bypass:  The avoidance of arterial cannulation eliminates potential arterial embolization and ischemia.  VV bypass eliminates need for arterial ligation or repair,  It preserves blood flow and improves oxygenation to pulmonary circulation with beneficial vasodilatory effect.  It produces no hemodynamic effects, particularly no increase in afterload.

Cannula Considerations



During ECLS it is important to use a drainage (venous) cannula with the largest lumen and shortest length possible because venous drainage is achieved only by gravity siphon.  In this system, if preload is adequate, the limiting factor determining maximum flow is cannula resistance, which is directly proportional to the length and inversely proportional to the fourth power of the luminal radius. This simple relationship becomes more complicated for devices that are not uniformly shaped.  Cannula size is based on the outer diameter. Identically sized cannulas may vary in inner diameter according to the wall thickness of the material used.  Venous cannulas generally have both end and side holes to allow flow even if the end of the cannula is occluded.  Arterial cannulas generally have only end holes to prevent arterial injury from ejected blood.  The cannula should resist kinking while remaining flexible and thin-walled to offer the least resistance possible.  Wire-wound cannulas (e.g., Biomedicus) are resilient to kinking, whereas the thin-walled double-lumen cannulas are more prone to kink.  Vascular access for ECLS in neonates is particularly challenging because of their small vessels. The route of access depends on the method used. VA bypass is indicated when both cardiac and pulmonary support is required and in neonates if access for VV support cannot be obtained (i.e., the vein is too small to accept a 12 French cannula).

16   Section I  •  General 

For VA access, the preferred site for venous drainage is the right atrium via the right internal jugular vein. The arterial infusion is directed at the aortic arch via the right common carotid artery.  For VV access, a double-lumen cannula is placed into the right atrium via the right internal jugular vein. This technique is limited by the size of the vein because the smallest doublelumen VV cannula available is 12 French. For larger children (>10 kg), single-lumen cannulas may be placed into the right internal jugular vein and left or right femoral vein.  Single-lumen cannulas are available in sizes ranging from 8 French for neonates to 29 French for adult-sized patients.  Double-lumen cannulas are available in various sizes: 12 to 18 French (Origen Biomedical, Inc., Austin, TX) and 31 French (Avalon Laboratories, LLC, Rancho Dominguez, CA).

Selection of Technique



The VA bypass requires an open technique for arterial ligation to prevent leakage around the cannula and possible distal embolization from flow past the cannula.  In infants and small children, the carotid artery is usually safe to ligate distally without major sequelae.  VV bypass can be performed via a percutaneous or open technique. Although jugular vein ligation is usually tolerated, there is evidence that it may produce high venous pressure, which can lead to cerebral ischemia.  Because the size of the vessel in relation to the cannula is unknown, vessel disruption is a risk when percutaneous access is used. For this reason, our preferred method is the semiopen technique. This technique requires a small incision to see the size of the vein as an aid to selecting the correct cannula size (usually 12 or 15 French in a newborn).  Cannula insertion can also be viewed through this incision if desired.  With this technique, vessel ligation is not used; this has several advantages: cephalad flow into the cannula increases the amount of deoxygenated blood available to enter the bypass circuit, the vessel may remain patent after decannulation (and can be recannulated if needed), and kinking of the cannula at the vessel is reduced because the vessel is not fixed to the cannula with a ligature, which can act as a fulcrum around which the cannula kinks. Also, adjustment of cannula depth is much simpler.

Step 3: Operative Steps—Cannula Insertion for Neonatal ECLS

VV/VA Cannulation: Open Technique

Preoperative Vascular cannulation and decannulation are performed in the neonatal ICU with the patient under adequate sedation and neuromuscular blockade.  Neuromuscular blockade is especially important in preventing the potentially lethal complication of an air embolus during introduction of the venous cannula.  Heparin sodium (100 units/kg) is drawn up for subsequent administration. Anesthesia  Local anesthesia is administered by infiltration of 1% lidocaine. 

Chapter 2  •  Extracorporeal Membrane Oxygenation Cannulation   17

Operation Position of Patient  The patient is placed supine with the head turned to the left. A roll is placed transversely beneath the shoulders.  The endotracheal tube is positioned to prevent kinking under the drapes during the procedure. This can be accomplished by using a piece of suction tubing split lengthwise and placed over the tube at the connector to prevent kinking.  The chest, neck, and right side of the face are aseptically prepared and draped. Incision  A transverse cervical incision about 2 to 3 cm long is made one fingerbreadth above the clavicle over the lower aspect of the right sternocleidomastoid muscle. Exposure of the Carotid Sheath  The platysma muscle and subcutaneous tissues are divided with electrocautery, and the sternocleidomastoid muscle is exposed.  Dissection is continued bluntly between the sternal and clavicular heads of the muscle.  The omohyoid muscle will be seen superiorly. It may be necessary to divide the omohyoid muscle tendon to expose the carotid sheath. Two alternating self-retaining retractors are placed. Dissection of the Vessels  The carotid sheath is opened and the internal jugular vein, common carotid artery, and vagus nerve are identified and isolated.  Manipulation of the vein should be minimized to avoid inducing spasm, which makes introduction of a large venous cannula difficult.  There is often a branch on the medial aspect of the internal jugular vein, and this branch must be ligated. Ligatures of 2/0 silk are placed proximally and distally around the internal jugular vein. The common carotid artery lies medial and posterior and has no branches, which makes its dissection proximally and distally safe. Ligatures of 2/0 silk are also placed around the carotid artery. The vagus nerve should be identified.  Once vessel dissection is completed, heparin (100 units/kg) is administered intravenously and 3 minutes allowed for circulation. During this waiting period, papaverine is instilled into the wound to enhance vein dilatation. Arteriotomy/Venotomy  For VA bypass, the arterial cannula is chosen (usually 10 French) and marked with a 2-0 silk ligature, left uncut, at a point that will allow the tip of the cannula to lie at the ostium of the brachiocephalic artery (about 2 to 2.5 cm).  The venous cannula (usually 12 to 14 French) is similarly marked at a point equal to the distance from the venotomy to the right atrium (roughly 6 to 7 cm).

18   Section I  •  General



An obturator is placed into the venous cannula to prevent blood from flowing out through the side holes during introduction into the vessel.  The common carotid artery is ligated distally. Proximal control is obtained with the use of an angled ductus clamp.  A transverse arteriotomy is made near the distal ligature. Full-thickness stay sutures of 6-0 polypropylene are placed on the proximal edge of the artery to prevent subintimal dissection during cannula insertion.  Following arterial cannulation, a venotomy is performed in similar fashion. Gentle retraction of the caudal ligature around the vein precludes the need for a ductus clamp during venotomy and venous cannulation. Stay sutures are also not routinely necessary for venous cannulation. Cannula Placement (Fig. 2-1)  The cannulas are carefully placed into the artery and vein and secured using two circumferential 2-0 silk ligatures.  A small piece of Silastic vessel loop can be left inside the ligatures to protect the vessels from injury during decannulation, when the ligatures are sharply divided. The ends of the marking ligatures are tied to the most distal circumferential ligature for extra security.  Immediately after each cannula is secured, it is carefully de-aired via back-bleeding and filling with heparinized saline.  For VV bypass, the double-lumen cannula is placed into the venotomy and advanced to place the tip in the mid-right atrium. It is crucial to maintain the arterial reinfusion (red) port anteriorly while securing for proper orientation to minimize recirculation. Wound Closure  The wound is irrigated with saline, and hemostasis is obtained. The skin is closed with continuous monofilament suture. The wound is dressed with gauze.  The cannula is sutured to the skin with several 2-0 silk sutures; special attention should be directed to affixing the cannulas securely to the bed.

Chapter 2  •  Extracorporeal Membrane Oxygenation Cannulation   19

Artery

Figure 2-1    Surgeon’s view from head

Vein

20   Section I  •  General

VV Cannulation: Semi-open Technique

Incision and Vein Exposure  A transverse cervical incision approximately 1.5 to 2 cm long is made 2 cm above the right clavicle between the heads of the sternocleidomastoid muscle.  The platysma is divided with electrocautery, and the anterior surface of the internal jugular vein is exposed with minimal dissection. The vessel is observed, and an appropriately sized VV ECMO cannula is selected. Guidewire Placement (Fig. 2-2)  The cannula skin exit position is selected so that the cannula will lie behind the right ear when the head is returned to the midline.  The needle and catheter are placed through the skin 2 cm superior to the incision and into the internal jugular vein to enter either under the skin flap or just inside the incision. The needle is removed, and a 0.035-inch-diameter guidewire is advanced and the catheter withdrawn.  A Teflon guiding obturator is placed over the guidewire into the vessel and right atrium. The skin exit is slightly enlarged with a scalpel.  Fluoroscopy is very helpful to observe appropriate guide wire placement, as well as during dilation and cannula advancement. Cannula Placement (Fig. 2-3)  Heparin (100 units/kg) is administered and 3 minutes allowed for circulation.  The selected cannula is advanced over the Teflon obturator into the vein under direct vision to confirm entrance into the vein.  The arterial (red) port of the cannula must be directed anteriorly to allow the arterial blood to cross the tricuspid valve and minimize recirculation of circuit blood.  The tip of the cannula is placed 6 to 8 cm from the skin. Wound Closure and Cannula Fixation  The relatively low venous pressure allows adequate hemostasis around the venotomy site without any ligature. This prevents kinking of the thin-walled cannula, which often occurs at the area of a ligature if it is used around the vessel.  Repositioning of the cannula requires only removing the skin sutures, repositioning the cannula, and replacing skin sutures.  The cannula is fixed to the skin with several 2-0 silk sutures. The incision is closed using a monofilament suture. Decannulation  After respiratory failure has resolved to allow ventilation without extracorporeal support, decannulation can be performed by removing the skin sutures, pulling the venous cannula, and holding pressure on the catheter exit site for 5 minutes or until bleeding stops.  Care must be taken to remove the entire cannula rapidly to prevent air from entering the side holes while the end of the cannula remains in the vessel.

Chapter 2  •  Extracorporeal Membrane Oxygenation Cannulation   21

Figure 2-2 

Figure 2-3 

22   Section I  •  General

Cannula Insertion for Pediatric ECLS



Children older than infants have different bypass needs, similar to those of adults. Vessels are larger and make more options available for access.  VV bypass is still used preferentially for respiratory support. VA bypass is preferred for cardiac support, including postoperative patients who do not wean from cardiopulmonary bypass after heart surgery.  Children who are not yet old enough to walk have very small femoral vessels that are unsuitable for use in bypass access. For this reason, in this group (weight less than 10 kg), a doublelumen cannula in the jugular vein for VV bypass or single cannulas in the jugular vein and carotid artery must be used for VA bypass.  Occasionally a small child with respiratory failure has a jugular vein that is too small to allow a large enough double-lumen cannula for adequate flow on VV bypass and must be placed on VA bypass.

Venovenous Bypass



As described, VV bypass in small children can be achieved using a double-lumen cannula either placed by a modified Seldinger technique, as described already, or entirely percutaneously if the vein is judged to be adequate to receive the cannula.  Children who weigh more than 10 kg usually have large enough veins to use a two-cannula technique by placing cannulas in both the femoral and jugular veins.  The selection of cannulas is again based on two criteria: (1) the largest cannula that the vein will accept based on judgment and (2) a large enough drainage cannula to allow for enough flow (100 mL/kg/min), which can be estimated by the M number provided by the manufacturer.  The issue of which cannula to use for drainage and reinfusion has two options. The jugular vein cannula will often allow more drainage. If the end of this cannula is in the atrium and preload is adequate, it can drain until the atrium collapses around the cannula and the pump flow is interrupted by servoregulation. Flow is thought to be greater in this situation because the atrium is spherical compared with the cylindrical shape of the femoral or iliac vein. However, if pump blood is reinfused into the femoral vein cannula, recirculation is often significant. This may be due to the direction of blood draining into the right atrium from the inferior vena cava being directed preferentially into the jugular cannula before mixing occurs.  Rich and colleagues showed that draining blood from the femoral cannula and reinfusing into the jugular cannula result in higher arterial saturation (i.e., oxygen delivery), even though the total flow achievable is less because recirculation is minimal.  We prefer this method of bypass and try to use a femoral cannula that reaches the intrahepatic vena cava, which is large and does not collapse.

Chapter 2  •  Extracorporeal Membrane Oxygenation Cannulation   23 Venoarterial Bypass



For cardiac failure, most pediatric patients are cannulated through the neck using the jugular vein and carotid artery by cutdown.  Patients placed on bypass after cardiac surgery may use their cannulation sites in the chest that are used for cardiopulmonary bypass.

Cannula Insertion for Adolescent ECLS

Venovenous Bypass



Cannulation for adolescent VV bypass uses two cannulas placed in the jugular and femoral veins. These cannulas can be placed safely by a percutaneous method. A large cannula (23 to 29 French) should be placed for drainage and a somewhat smaller cannula (21 to 23 French) for venous reinfusion.  It is especially important for the drainage cannula to have side holes in addition to an end hole to maximize flow and allow flow to continue if the end becomes obstructed.  An adult-sized double-lumen cannula has been introduced by Avalon Laboratories and is available in sizes up to 31 French, which is suitable for most adult patients, even those who are obese.  Recirculation is a problem that can be solved as described in the preceding section on pediatric cannulation.

Venoarterial Bypass



In adolescents VA bypass can be achieved using many different cannulation schemes. Jugular vein to carotid artery bypass as used in infants has been used successfully and works well, especially for combined cardiac and pulmonary support. It provides very good perfusion to all branches of the aortic arch and distal aorta, but it increases afterload by increasing aortic pressure. Ligation of the cerebral artery may cause cerebral edema.  Jugular vein to femoral artery bypass provides adequate distal perfusion, but this approach can fail to perfuse the aortic arch in situations where the native cardiac function is good. If the blood ejected from the left ventricle is desaturated because of pulmonary dysfunction, the aortic arch might not receive well-oxygenated pump blood, and the result will be hypoxemia in the upper half of the body. This problem can be solved by adding an additional perfusion cannula to the venous circulation to create venoarteriovenous bypass, which increases oxygenation of the right ventricular blood much like VV bypass and provides the hemodynamic support of VA bypass. The increased afterload from VA bypass may prevent the failing left ventricle from ejecting blood and result in high left atrial pressure, causing pulmonary edema. This situation can be managed by draining blood from the left atrium into the venous side of the bypass circuit either from direct cannulation of the left atrium by thoracotomy or by catheter-based balloon atrial septostomy.  Arterial cannulation can be performed either percutaneously or by direct cutdown of the vessel. With either method, if the cannula is large enough to diminish flow, distal ischemia may result. Several methods of managing this type of ischemia have been described. 

24   Section I  •  General 

Placement of a distal perfusion catheter can be used with the open technique by placing a connector with a side port and placing small tubing directed into the vessel distally at the cutdown site. With the percutaneous technique, an arterial line can be placed into either the dorsalis pedis or posterior tibial artery by cutdown and the distal pressure measured. If the pressure is less than 50 mm Hg, the catheter can be perfused by a line from the perfusion limb of the circuit.  Decannulation can be performed similarly to previously described methods for the vein (direct pressure for percutaneously placed line, ligation of the jugular vein for cutdown placement).  Arterial decannulation is more complicated. Direct pressure may be all that is needed for percutaneously placed arterial cannulas. The larger the cannula is in relation to the artery, the more likely that a pseudoaneurysm or arterial stenosis will result.  An alternative to this method is venous patch angioplasty, a technique used for removing arterial cannulas placed by cutdown. In this technique, the vessel is controlled by a clamp, and the cannula is removed. A diamond-shaped patch of vein is then sutured into the defect, which both closes the hole and prevents stricture at the repair site.

Transthoracic Cannulation



In certain circumstances, cervical or femoral access for VA extracorporeal support is either not possible or not practical, particularly when treating patients who have not been weaned from cardiopulmonary bypass or who have undergone post-sternotomy resuscitation. In these circumstances direct cannulation of the arterial and venous system is performed using techniques and cannulas that are standardly used with cardiopulmonary bypass.  Pursestring sutures of some sort are placed in the ascending aorta, usually directly in the right atrium, and brought through snares that allow the suture to be tightened around the cannula and secured, preventing leaking of blood around the cannula, and, in the case of the venous side, preventing entraining of air into the system.  While in the operating room, the cannulas are usually lightly secured to the drapes or left lying on the field; it is critical to secure the cannulas in a more “permanent” fashion when providing more prolonged extracorporeal support, particularly for safety during transport. In general, this involves suturing the cannula to the chest wall at some point and then closing the wound with an artificial dressing usually made of some sort of plastic or elastic, with the cannulas exiting between the suture line between the material and the skin.  If the patient becomes more lightly sedated and moves or attempts to breathe or cough, the sternal edges can separate and put tension on the cannulas, risking dislodgement. This can be prevented by either using continuous neuromuscular blockade or by using one or two heavy sutures or sternal wires to bridge the distance between the sternal edges. This prevents spreading of the distance between the edges if the patient coughs or fights the endotracheal tube. We have found this to provide more than adequate stabilization of the support apparatus and prefer it over neuromuscular blockade with their potential sequelae.

Step 4: Postoperative Care



It is critical that the cannulas are well secured to the patient’s skin as well as to the bed so that tension does not pull on the cannula. Failure to adequately secure the cannula may cause malpositioning or inadvertent decannulation.  The wound should be dressed with a dry, sterile dressing.

Chapter 2  •  Extracorporeal Membrane Oxygenation Cannulation   25

Step 5: Pearls and Pitfalls



Cannulation of patients for ECMO can be quite challenging, and problems are frequently encountered. By adequately preparing the patient, complications can usually be avoided.  Proper training and support of the surgeon performing these procedures will allow most of these problems to be managed without poor outcomes.

Difficulty Threading the Venous Cannula



This type of difficulty can occur because the vein is too small, the catheter is too large, or there is a left-sided superior vena cava without an innominate vein.  The clavicle or first rib can sometimes obstruct if the patient’s head is hyperextended or hyperrotated.  Try to reposition the head. Also, severe mediastinal shift may be present with diaphragmatic hernia or pneumothorax or effusion.

Vein Division



Especially in small newborns, it may be difficult to introduce the venous cannula. During attempts to do this, the vein may become divided, which will make further attempts to introduce the cannula more difficult.  Vascular control is the primary goal, which is best done using a vascular clamp.  Once vascular control has been achieved, placing a guidewire may be helpful to introduce the cannula.  Placing stay sutures to provide traction during cannula placement will help.  A ligature should be placed around the vein to tie in the cannula.  At decannulation, a pursestring suture may be used to control bleeding.

Proximal Vein Lost in Mediastinum



During a difficult venous cannulation, when the cannula does not thread easily, sudden loss of resistance may be due to division of the vein, which may invert into the mediastinum.  Bleeding can be controlled by using direct pressure with a finger. If the vein end can be retrieved with a forceps, cannulation may be performed as above with vein division.  Otherwise, if no other suitable vein is available for access, median sternotomy and access via a thoracic approach may be needed.  If other access is available, control can almost always be achieved by suturing fascia to cover the hole where the vein was lost and applying direct pressure.

26   Section I  •  General

No Flow After Catheter Placement



If there is no flow after placement of the cannula, the cannula and circuit tubing should be examined for kinking.  Chest radiography or fluoroscopy should be used to assess the position of the venous cannula and reposition or replace as needed.

Intrathoracic Vein Perforation



Sudden cessation of flow with hemodynamic instability is of concern for intrathoracic vessel perforation. This situation requires immediate median sternotomy and vascular repair, with subsequent open cannulation.

Bibliography

Foley DS, Swaniker F, Pranikoff T, Bartlett RH, Hirschl R. Percutaneous cannulation for venovenous extracorporeal life support (ECLS). J Pediatr Surg 2000;35:943-947. Lazar EL, Abramson SJ, Weinstein S, et al. Neuroimaging of brain injury in neonates treated with extracorporeal membrane oxygenation: Lessons learned from serial examinations. J Pediatr Surg 1994;29:186-191. Miskulin J, Annich G, Grams R, et al. Venous-arteriovenous cannulation for adult ECMO patients with cardiogenic shock. 14th Annual ELSO Conference, September 10-12, 2004, Chicago, IL. Montoya JP, Merz SI, Bartlett RH. A standardized system for describing flow/pressure relationships in vascular access devices. Trans Am Soc Artif Intern Organs 1991;37:4-8. Peek GJ, Firmin RK, Moore HM, et al. Cannulation of neonates for venovenous extracorporeal life support. Ann Thoracic Surg 1996;61:1291-1292. Pranikoff T, Hirschl RB. Neonatal extracorporeal membrane oxygenation. In: Carter DC, Russell RCG, eds. Rob and Smith’s operative surgery, 6th ed. London: Butterworth-Heinemann, 2005. Pranikoff T, Hirschl RB, Remenapp R, Swaniker F, Bartlett RH. Venovenous extracorporeal life support via percutaneous cannulation in 94 patients. Chest 1999;115:818-822. Pranikoff T, Hirschl RB. Neonatal extracorporeal membrane oxygenation. In: Carter DC, Russell RCG, eds. Rob and Smith’s operative surgery, 5th ed. London: Butterworth-Heinemann, 1995. Rich PB, Awad SS, Crotti S, Hirschl RB, Bartlett RH, Schreiner RJ. A prospective comparison of atrio-femoral and femoro-atrial flow in adult venovenous extracorporeal life support. J Thorac Cardiovasc Surg 1998;116:628-632. Schumacher RE, Barks JD, Johnston MV, et al. Right-sided brain lesions in infants following extracorporeal membrane oxygenation. Pediatrics 1988;82:155-161. Sinard JM, Merz SI, Hatcher MD, et al. Evaluation of extracorporeal perfusion catheters using a standardized measurement technique—the M-number. Trans Am Soc Artif Intern Organs 1991;37:60-64. Streltz LJ, Bej MD, Graziani LJ, et al. Utility of serial EEGs in neonates during extracorporeal membrane oxygenation. Pediatr Neurol 1992;8:190-196. UK collaborative randomized trial of neonatal extracorporeal membrane oxygenation. UK Collaborative ECMO Trial Group. Lancet 1996;348:75-82. Walker LK, Short BL, Traystman RJ. Impairment of cerebral autoregulation during venovenous extracorporeal membrane oxygenation in the newborn lamb. Crit Care Med 1996;24:2001-2006.

SECTION

II

Head and Neck

CHAPTER

3 

Thyroglossal Duct Cyst Michael D. Josephs

Step 1: Surgical Anatomy



Thyroglossal duct cyst traditionally manifests as a painless midline anterior cervical mass that often moves with swallowing (Fig. 3-1).  The cyst occasionally communicates with the skin as a draining sinus, and its tract always extends through the center of the hyoid bone to terminate at the tongue base (Fig. 3-2). Operative extirpation of the entire cyst and sinus tract, including the involved portion of the hyoid bone, is critical to avoid a recurrence.  The greater horns of the hyoid bone may be palpated bilaterally and moved side to side, which will result in cyst movement (Fig. 3-3) and help to ensure a proper anatomic resection and avoid injuring the larynx.

Step 2: Preoperative Considerations



The diagnosis of thyroglossal duct cyst is made by a history and physical examination, although an ultrasound may help to differentiate this lesion from a dermoid cyst, epidermoid cyst, or median ectopic thyroid. An extensive radiographic and laboratory evaluation is unnecessary.  Surgical excision is indicated on identification and is easiest in the absence of infection. Most infections can be treated with oral antibiotics targeting Haemophilus influenzae, Staphylococcus aureus, and Staphylococcus epidermidis, but occasionally the abscess is medically refractory and requires drainage. Elective resection is then best reserved for a time when the wound has healed and the inflammation is minimized.

28

Chapter 3  •  Thyroglossal Duct Cyst   29

Foramen cecum Epiglottis

Mandible

Tract Hyoid bone

Cricoid cartilage

Cyst Thyroid cartilage

Thyroid gland

Figure 3-2 

Figure 3-1 

Figure 3-3 

30   Section II  •  Head and Neck

Step 3: Operative Steps

Anesthetic Induction



General endotracheal intubation is preferred for safe airway management. Because of the possibility of digital palpation of the tongue base during the operation (see next comment), the endotracheal tube should be secured in a reliable fashion.  A laryngeal mask airway (LMA) may also be considered, although its use is discouraged. Because the origin of the cyst’s tract must be ligated, the anesthesiologist or an assistant may need to insert a finger into the mouth to reflect the tongue base anteriorly. This maneuver would disrupt the seal on the LMA and hinder ventilation if the patient is not breathing spontaneously.

Positioning



The patient is placed supine with the neck extended. This is facilitated by placing a roll transversely behind the shoulders. The bed is then tilted into the reverse Trendelenburg position (Fig. 3-4).  Draping should allow easy access to the mouth if transoral palpation of the base of the tongue becomes necessary.

Incision



The Sistrunk procedure remains the operation of choice for the management of thyroglossal duct cysts.  A transverse incision is made directly over the mass and along a natural skin crease for improved cosmesis. Care should be taken to avoid entering the wall of the cyst.  If previous drainage was required for an infected cyst, the skin may be quite adherent to the cyst. Excising a bit of adherent skin may be helpful.  The length of the incision is usually limited to the diameter of the mass, although this length can be increased if additional exposure is required (Fig. 3-5).  Self-retaining retractors are generally not required and, if used, tend to necessitate a larger incision.  The cyst is separated from the surrounding tissue nearly circumferentially, and the sinus tract to the hyoid bone is identified posteriorly and preserved (Fig. 3-6).  The strap muscles are reflected off the body of the hyoid adjacent to where it is penetrated by the sinus tract. The hyoid is dissected circumferentially on both sides and then divided with scissors or bone cutters just lateral to the penetrating sinus tract (Fig. 3-7, A).  Dissection of the tract is continued proximally and cephalad toward its origin at the foramen cecum, where it is suture ligated (Fig. 3-7, B). This facilitates a complete en bloc resection.

Chapter 3  •  Thyroglossal Duct Cyst   31

Figure 3-4 

Figure 3-5 

Figure 3-6 

A

Figure 3-7 

B

32   Section II  •  Head and Neck

Figure 3-8 

Closing



If the cyst wall is violated, then the wound is copiously irrigated (Fig. 3-8). The platysma muscle is reapproximated using interrupted absorbable sutures. The subcutaneous tissue and skin are then closed by the surgeon’s method of choice. For this I prefer subdermal sutures of 4-0 Vicryl followed by 5-0 Monocryl subcuticular closure. Finally, a dry, sterile occlusive dressing is applied.  If the surgical field remains dry, consideration can be given toward intraoperative administration of ketorolac in addition to the usual narcotic analgesics. 

Step 4: Postoperative Care

 

Narcotic analgesics are often required in the recovery room. Almost uniformly, patients can be discharged home on the day of surgery with oral narcotics, nonsteroidal anti-inflammatory agents, or both.

Step 5: Pearls and Pitfalls



Despite the low incidence of carcinoma and recurrence, every effort should be made to resect the entire thyroglossal duct cyst and tract to prevent the development of these occurrences.  Recurrent disease is related to incomplete resection and postoperative infection and can be managed by secondary Sistrunk operation with limited base-of-tongue resection, central neck dissection with core resection of the tongue base, or suture-guided transhyoid pharyngotomy.  Although surgeons dislike reporting this, inadvertent resection of a portion of the thyroid cartilage or tracheal ring is a serious complication that must be avoided. Meticulous attention to and identification of anatomic landmarks, particularly the greater horns of the hyoid bone, ensure that this complication is averted.

Chapter 3  •  Thyroglossal Duct Cyst   33

Bibliography

Kaselas C, Tsikopoulos G, Chortis C, Kaselas B. Thyroglossal duct cyst’s inflammation. When do we operate? Pediatr Surg Int 2005;1:991-993. Ostlie DJ, Burjonrappa SC, Snyder CL, et al. Thyroglossal duct infections and surgical outcomes. J Pediatr Surg 2004;39:396-399. Perkins JA, Inglis AF, Sie KC, Manning SC. Recurrent thyroglossal duct cysts: a 23-year experience and a new method for management. Ann Otol Rhinol Laryngol 2006;115:850-856. Tracy TF Jr, Muratore CS. Management of common head and neck masses. Semin Pediatr Surg 2007;16:3-13.

CHAPTER

4 

Branchial Anomalies Tory A. Meyer

Step 1: Surgical Anatomy



The course of branchial cysts, sinuses, and fistulas depend on the branchial arch from which they are derived (first, second, third, or fourth).  The extent of branchial anomalies varies considerably, from small sinuses or cysts limited to the subcutaneous tissue (Figs. 4-1 and 4-2) to large inflammatory masses extending to the pharynx.  First branchial anomalies appear in the preauricular or submandibular area and may course through the parotid gland, insinuate around the facial nerve trunk or branches, and connect to the external auditory canal.  Second branchial anomalies are seen along the anterior border of the sternocleidomastoid muscle and may ascend through the deep tissues of the neck above the hyoid, between the internal and external carotid arteries, adjacent to the hypoglossal and glossopharyngeal nerves, and terminate in the tonsillar fossa or other nasopharyngeal areas.  Although the theoretical course of third and fourth branchial anomalies has been described as descending into the mediastinum, this course has not been noted in vivo. These rare lesions have more recently been described by their pharyngeal terminus as pyriform fossa sinuses. Externally they may be evident along the anterior border of the sternocleidomastoid, usually on the left side of the neck, and pass through the thyroid, posterior to the internal carotid, adjacent to the recurrent laryngeal nerve, through the inferior constrictor muscle, and connecting to the pyriform fossa.  Branchial anomalies may contain keratinizing or nonkeratinizing squamous epithelium or pseudostratified columnar respiratory epithelium.

Step 2: Preoperative Considerations



Most common initial signs of branchial anomalies are an asymptomatic cystic mass, chronically draining sinus, or recurrently infected lesion of the neck.  Occasionally it may manifest in infants with respiratory distress or stridor resulting from the swelling of cyst, impinging on the airway.  Sinuses of the pyriform fossa may manifest as recurrent neck abscesses or acute thyroiditis.

34

Chapter 4  •  Branchial Anomalies   35

Figure 4-1 

Figure 4-2 

36   Section II  •  Head and Neck



Preoperative imaging may help distinguish a branchial anomaly from other lesions, such as a cystic hygroma, lymphoma, neurofibromatosis, lymphadenopathy, carotid body tumor, tuberculous adenitis, lipoma, hemangioma, thyroglossal duct cyst, ectopic thyroid, thymic cyst, or a dermoid.  Ultrasound or computed tomography scan with intravenous contrast may demonstrate gas in a cystic structure and may be useful to delineate the course of the lesion and possible thyroid involvement.  Barium swallow may show the origination and extent of fistula or sinus.  Antibiotics (with drainage, if necessary) should be administered until clinical signs of infection have resolved and before removal to minimize risks of complications and recurrence.  Removal of a branchial anomaly before repeat infections will aid dissection in clean surgical planes.  Goal of surgery is complete excision to prevent recurrence.  Laryngoscopy or telescopic pharyngoscopy is useful for identification or cannulation of a pharyngeal fistula.  Recent studies suggest that chemical or thermal cauterization of the internal opening may be adequate treatment for pyriform fossa sinus anomalies.  Resection of all but the simplest anomalies limited to the subcutaneous tissue should be done with the patient under general anesthesia with an endotracheal tube rather than a laryngeal mask airway.

Step 3: Operative Steps

First Branchial Anomalies



Intraoperative facial nerve monitoring should be considered. Injection of the tract through the external auditory canal with methylene blue or instrumentation with a small probe facilitates tract identification.  A preauricular cervicomastoid incision is made and may be extended as necessary.  The superficial lobe of the parotid is mobilized with clear identification of the facial nerve as it emerges from the stylomastoid foramen and enters the parotid.  Lateral parotidectomy may be necessary to see the nerve and branchial anomaly adequately.  The cyst is dissected free from surrounding tissues and the tract followed along its course, which may be posterior to the parotid (type I), or intraparotid (type II) (Fig. 4-3). The tract may ultimately connect with the external auditory canal.  A segment of the external auditory canal, including skin and cartilage, is taken with the cyst. The external auditory canal wound may be packed open.  The wound is closed in layers with a subcuticular skin closure and appropriate dressing applied. 

Chapter 4  •  Branchial Anomalies   37

Facial nerve

External auditory canal

Parotid gland

A First branchial cleft anomaly (I) Figure 4-3 

B

First branchial cleft anomaly (II)

38   Section II  •  Head and Neck

Second Branchial Anomalies

Large Cysts  With the neck extended, large cysts are approached through an oblique neck incision following Langer lines along the superior portion of the swelling (see Fig. 4-5).  The cyst is freed up circumferentially, paying careful attention to the jugular and carotid vessels and the hypoglossal nerve (XII) posteriorly.  The facial vein and branches of the jugular vein may be ligated as necessary.  If a tract is present, it should be freed up from attachments as it courses over the hypoglossal nerve (XII), between the internal and external carotid arteries, beneath the posterior belly of the digastric muscle, and over the glossopharyngeal nerve (IX) to enter the pharynx in the tonsillar fossa.  The tract is suture ligated at the level of the pharynx with absorbable suture.  The wound is closed in layers, and a drain is not usually necessary. Fistulas and Small Cysts  With the neck extended, a small transverse elliptical incision is made around the skin opening in the lower neck.  A small probe or Prolene suture may be passed along the course of the tract and into the pharynx to help with identification of the tract. The suture can be tied to a wad of gauze and used for traction on the tract.  As the tract ascends in the neck, it is dissected free from surrounding tissues as far superiorly as possible, at which time an additional transverse incision is made (stair-step incision) and the fistulous remnant is delivered through this more superior wound (Fig. 4-4).  This process is repeated until the tract turns into the deep tissues, at which point the incision may need to be extended to allow deeper dissection.  Similar to large second branchial cysts, dissection continues around the fistulous tract, paying careful attention to preservation of the hypoglossal and glossopharyngeal nerves at the lower margin and the branches of the carotid arteries medially and laterally (Fig. 4-5).  The fistulous tract is ligated at the level of the pharynx with absorbable suture, and the wounds are closed and dressed.

Third and Fourth Branchial Anomalies (Pyriform Fossa Sinus Tracts)



The traditional approach is open excision of the entire fistulous tract; however, recent studies have shown that cauterization of the pyriform sinus opening alone can be effective. Open Operation  With the neck extended, direct laryngoscopy is performed to identify the sinus tract opening in the pyriform sinus.

Chapter 4  •  Branchial Anomalies   39

Figure 4-4 

Hypoglossal nerve

Glossopharyngeal nerve Vagus nerve Initial incision 2nd Branchial anomaly

Carotid artery

Figure 4-5 

40   Section II  •  Head and Neck



A Fogarty catheter is inserted into the sinus and secured to aid with identification of the tract (Fig. 4-6).  If an external opening is present, it may be injected with methylene blue or instrumented with a lacrimal duct probe to facilitate identification during dissection.  A generous oblique neck incision is made at the level of the thyroid cartilage.  The strap muscles are retracted, and the cyst or tract is dissected circumferentially to the thyroid gland, through which it passes.  Resection of the lateral portion of the thyroid or a thyroid lobectomy is performed to include the fistulous tract.  The recurrent laryngeal nerve is clearly identified and preserved because the course of the fistula may be parallel to the recurrent laryngeal nerve.  The superior parathyroid gland is identified and preserved.  The most commonly seen tract courses superiorly into the pyriform sinus.  Alternatively, the tract is followed superior to the hypoglossal nerve and posterior to the carotid. It will pass superior or inferior to the superior laryngeal nerve, depending on its cause as a third or fourth branchial anomaly, respectively.  The tract is then dissected as it courses inferiorly, anterior, or lateral to the recurrent laryngeal nerve, then piercing the inferior constrictor muscle at the level of the base or apex of the pyriform sinus.  A portion of the thyroid cartilage may be removed, or the inferior constrictor may be opened to adequately view this portion of the tract.  Using absorbable suture, the tract is closed where it enters the pyriform sinus.  The wound is closed in layers, and a drain is not generally necessary.

Endoscopic Sinus Cauterization



Cauterization may be performed at the time of drainage of neck abscess or subsequently when acute infection has resolved.  Laryngoscopy is used to visualize the pyriform sinus (Fig. 4-7).  A small Fogarty catheter is passed into the opening of the sinus, and the tract is dilated enough to allow passage of a coagulating instrument into the opening.  An insulated probe or ball coagulating tip is passed into the sinus opening and activated on low power till tissue blanching occurs.  The superficial portion of the opening is coagulated again to ensure closure of the tract.  To prevent perforation of the hypopharynx, avoid overcoagulation.

Step 4: Postoperative Care



Recurrence is much more likely if the pathology specimen does not contain an epitheliumlined tract.  Long-term follow-up is necessary to monitor for recurrence.

Chapter 4  •  Branchial Anomalies   41

Endotracheal tube

Epiglottis

Pyriform sinus Fogarty into sinus opening Endoscopic view of hypopharynx

Figure 4-6 

Figure 4-7 

42   Section II  •  Head and Neck

Step 5: Pearls and Pitfalls



Infection should be treated before surgical intervention for removal of branchial anomalies.  Mastery of the anatomy of the neck and possible courses of these anomalies are essential for successful resection.  Complete surgical extirpation is the traditional approach for preventing recurrence.  Complications include injury to the hypoglossal, glossopharyngeal, vagus, superior laryngeal, and recurrent laryngeal nerves. In addition, injury to the carotid or jugular vessels and formation of a pharyngocutaneous fistula may occur.  Although the long-term recurrence rate is not well established, endoscopic sinus cauterization may be adequate treatment for pyriform sinus anomalies (third and fourth branchial anomalies) with a lower complication rate.

Bibliography

Acierno SP, Waldhausen JHT. Congenital cervical cysts, sinuses, and fistulas. Otolaryngol Clin North Am 2007;40:161-176. Houck J. Excision of branchial cysts. Operative Techn Otolaryngol 2005;16:213-222. James A, Stewart C, Warrick P, Tzifa C, Forte V. Branchial sinus of the piriform fossa: reappraisal of third and fourth branchial anomalies. Laryngoscope 2007;117(11):1920-1924. Jordan JA, Graves JE, Manning SC, McClay JE, Biavati MJ. Endoscopic cauterization for treatment of fourth branchial cleft sinuses. Arch Otolaryngol Head Neck Surg 1998;124(9):1021-1024. Liberman M, Kay S, Emil S, et al. Ten years’ experience with third and fourth branchial remnants. J Pediatr Surg 2002;37:685-690. Pereira KD, Davies JN. Piriform sinus tracts in children. Arch Otolaryngol Head Neck Surg 2006;132:1119-1121. Sai Prasad TR, Chong Cl, Mani A, et al. Acute suppurative thyroiditis in children secondary to pyriform sinus fistula. Pediatr Surg Int 2007;23(8):779-783. Schroeder JW, Mohyuddin N, Maddalozzo J. Branchial anomalies in the pediatric population. Otolaryngol Head Neck Surg 2007;137(2):289-295. Smith CD. Cysts and sinuses of the neck. In Grosfeld JL, O’Neill JA, Coran AG, Fonkalsrud EW, eds. Pediatric surgery, 6th ed. Philadelphia: Mosby Elsevier, 2006; pp. 861-869. Tracy TJ, Muratore CS. Management of common head and neck masses. Semin Pediatr Surg 2007;16(1):3-13. Verret DJ, McClay J, Murray A, Biavati M, Brown O. Endoscopic cauterization of fourth branchial cleft sinus tracts. Arch Otolaryngol Head Neck Surg 2004;130(4):465-468. Waldhausen JHT, Tapper D. Head and neck sinuses and masses. In Ashcraft KW, Holcomb GW, Murphy JP, eds. Pediatric surgery, 4th ed. Philadelphia: Saunders, 2005; pp. 1054-1057.

SECTION

III

Thoracic

CHAPTER

5 

Esophageal Atresia with Tracheoesophageal Fistula Richard Ricketts

Step 1: Surgical Anatomy



The proximal esophagus will be of variable length, and the distal tracheoesophageal fistula (TEF) is usually at or slightly above the carina.  The vagus nerve courses along the trachea, tracheoesophageal groove, and the distal esophagus (Fig. 5-1).  The proximal esophagus receives its blood supply from the thyroid arteries, and the distal esophagus from segmental branches directly off the aorta.

Step 2: Preoperative Considerations



The diagnosis is frequently established in utero because of associated maternal polyhydramnios.  The neonate may present with coughing, drooling, and respiratory distress shortly after delivery.  Infants with pure esophageal atresia (EA) will be scaphoid because of absence of gas in the gastrointestinal tract.  Neonates with a TEF may become distended, especially if they are placed on positive pressure ventilation.  A babygram radiograph with a tube in the upper pouch establishes the diagnosis of EA or EA-TEF in a vast majority of neonates (Fig. 5-2). Contrast studies are rarely required.  One must evaluate the baby for the VACTERL complex.  Vertebral: Spine films  Anorectal: Physical examination for patent anus  Cardiac: Echocardiogram to evaluate cardiac anatomy as well as identify position of the aortic arch. Surgical approach through the right or the left side of the chest is determined by the side of the aortic arch. In general, the arch is on the left and access for repair is through the right side of the chest.

44

Chapter 5  •  Esophageal Atresia with Tracheoesophageal Fistula   45

Trachea

Clavicle (cut) 1st rib (cut)

Vagus nerve Azygos vein Esophagus

Figure 5-1 

Esophageal atresia with TEF

Figure 5-2 

Isolated esophageal atresia

46   Section III  •  Thoracic



TE: Plain babygram radiograph with a catheter (bougie or nasogastric [NG]) in upper pouch is sufficient. Many surgeons routinely perform a rigid bronchoscopy as part of the operative procedure before proceeding with the repair to assess for a possible proximal TEF.  Renal: Ultrasound  Limb: Physical examination for radial hypoplasia (Fig. 5-3)  The distance between the two ends of the esophagus can be estimated by placing a blunt tipped bougie (Hurst dilator or small chest tube) into the upper pouch (with mild downward pressure) and taking a chest x-ray; assume that the distal esophagus is at or slightly above the level of the carina. In patients with pure EA who have already had a gastrostomy tube placed, instilling contrast into the stomach during the same maneuver will demonstrate the level of the distal esophagus (Fig. 5-4).  The timing of repair depends on the infant’s size and condition:  Primary repair: Infant has no significant cardiac or pulmonary problems and weighs more than 1000 g.  Delayed primary repair d Infant weighs less than 1000 g  Support with total parenteral nutrition (TPN) or G-tube feeds  Constant suction on upper pouch d Short-term cardiac or pulmonary problems (pneumonia) that can be resolved in 2 to 3 weeks d “Long gap”  TPN or G-tube feeds and bouginage with suction on upper pouch  Staged repair: Divide TEF and repair EA later d Extremely premature infant d Severe pneumonia d Requirement for high-pressure ventilation d Complex congenital heart disease (CHD) with cardiac decompensation (cyanotic CHD)  Esophageal substitution: Cervical esophagostomy with substitution at a later date d Extremely long gap (EA with no distal esophagus in chest and upper pouch at the thoracic inlet) d Failed repair

Step 3: Operative Steps

Anesthetic Considerations



End of the endotracheal tube should be in the midtrachea to reduce likelihood of intubating the distal TEF.  Use low ventilatory pressures to minimize distention of the stomach.

Chapter 5  •  Esophageal Atresia with Tracheoesophageal Fistula   47

Figure 5-3 

Figure 5-4 

48   Section III  •  Thoracic

Positioning



The infant is placed in an extreme lateral decubitus position (almost prone) with the side opposite the aortic arch up (Fig. 5-5).

Incision and Access



The incision extends from just below the tip of the scapula posteriorly to a point midway between the scapula and vertebral column (Fig. 5-6).  The 4th intercostal space is entered; the serratus anterior muscle is spared.  An extrapleural approach is used. The plane is developed with a freer elevator initially and then with moistened “micro-Kittner” dissectors (folded neuropatties on a mosquito clamp) until a small Finochietto retractor can be placed.  The parietal pleura is rolled off the azygos vein, which is then divided between suture ligatures. The TEF will usually be located at or slightly above the level of the azygos vein.  The TEF, distal esophagus, vagus nerve, and proximal esophagus are then identified, and a determination is made about the feasibility of primary repair.

Division of the TEF and Mobilization of the Esophagus



Encircle the distal esophagus near the TEF with a vessel loop, taking care not to injure the proximal bronchi (beware of the right mainstem bronchus going posteriorly) or vagus nerve (Fig. 5-7).  Elevate the esophageal side of the TEF with a vessel loop. Pinch the fistula close with forceps, and have the anesthesiologist inflate the lungs to ensure that the anatomy has been correctly identified and that the bronchus or trachea has not been mistaken for the fistula.  Start a 5-0 Prolene suture on the tracheal side of the TEF, and sequentially divide the TEF and sew the tracheal side closed with the 5-0 Prolene suture using a simple running stitch. Tie the suture, and then run it back and tie it to the original starting point, creating an airtight tracheal closure.  Mobilize the distal esophagus no more than 2 cm so as not to disrupt too much of the segmental blood supply.  Mobilize the proximal esophagus after placing a stay stitch at its end, up to the thoracic inlet if necessary; the blood supply is intramural and will not be compromised. A combination of sharp and blunt dissection is used, taking care to stay directly on the esophageal wall so as not to enter the membranous portion of the trachea or damage the recurrent laryngeal nerves. This dissection may expose a proximal TEF, which if found is primarily repaired.

Chapter 5  •  Esophageal Atresia with Tracheoesophageal Fistula   49

Tip of scapula

Nipple

Figure 5-6 

Figure 5-5 

Proximal esophagus

TEF

Vagus nerve

Figure 5-7 

50   Section III  •  Thoracic

Anastomosis (Fig. 5-8)



A single-layer, end-to-end anastomosis with fine suture is performed. Two “corner” stitches are placed with the knots on the outside. A center stitch and one stitch between the center and each “corner,” with knots on the inside, are then placed. The sutures are then pulled up and crossed to eliminate tension on the one being tied. Be sure that each stitch is fullthickness and includes mucosa. This completes the “back-row” anastomosis.  For the anterior row of sutures, place a center stitch (including mucosa on each side) with the knot on the outside.  Pull up on this stitch as the anesthesiologist passes down a transanastomotic 6 or 8 French NG feeding tube. Avoid creating a submucosal false channel.  Complete the anterior row of the anastomosis with additional full-thickness stitches with the knots on the outside.  Separate the suture lines on the esophagus and the trachea by interposing some mediastinal soft tissue held in place with 5-0 chromic suture.

Closing



Place a 10 or 12 French chest tube near, but not touching, the anastomosis (Fig. 5-9). To keep it in the desired location, a 5-0 chromic suture may be used to sew it to the inside of the chest wall. Bring it out through a separate stab wound incision and connect to a 2-cm water seal (not to suction).  Place saline into the chest, and inflate the lungs to ensure that the fistula closure on the tracheal side is complete. Close the chest in the standard fashion.  Secure the transanastomotic feeding tube in place. Do not replace it “blindly” if it comes out.

Step 4: Postoperative Care



Keep the head of the bed slightly elevated, and suction the oropharynx and the endotracheal tube as necessary. Do not suction beyond 8 cm so as not to traumatize the repaired fistula.  Wean from the ventilator, and extubate as soon as possible.  Intravenous fluids are run at maintenance rates or less; do not flood the lungs! Provide nutrition via TPN and lipids; NG drip feeds may be started via transanastomotic feeding tube on postoperative day 2.  A contrast swallow is obtained on postoperative day 5. If there is no leak, commence oral feeds and disconnect the chest tube. If it shows a minor, “contained” leak, keep the patient NPO (nothing by mouth), and repeat the study in 1 week. If it shows an anastomotic disruption, consider re-exploration for attempted repair or conversion to esophagostomy and eventual replacement.  Prophylactic histamine-2 (H2) blockers to prevent anastomotic strictures are routinely used.  Obtain a contrast swallow 6 to 8 weeks postoperatively. If it shows a stricture, begin a program of esophageal dilatations.

Chapter 5  •  Esophageal Atresia with Tracheoesophageal Fistula   51

Figure 5-8 

Figure 5-9 

52   Section III  •  Thoracic

Step 5: Pearls and Pitfalls



Position the patient almost prone, and keep the incision posterior; this gives excellent visualization and exposure to the esophagus and TEF.  Do not mobilize the distal esophagus more than a few centimeters because to do so would disrupt the segmental blood supply to that portion of the esophagus and cause ischemia, leading to anastomotic breakdown or stricture formation.  The proximal esophagus can be dissected all the way up to the neck because it has an intramural blood supply and therefore will not become ischemic.  Keep the dissection directly on the esophageal wall (upper pouch) to avoid damage to the vagus nerves and recurrent laryngeal nerves.  Be sure to include mucosa with each stitch, or you will get a leak or a stricture.  Avoid excessive tension on the anastomosis.  Consider a spiral or circular myotomy on the upper pouch to lessen anastomotic tension if necessary.  A myotomy is most easily done with a #5 Fogarty catheter inflated in the upper pouch (Fig. 5-10).  Avoid entry into the mucosa.  Consider use of the Foker technique (Foker et al., 2005) for extreme long-gap atresia.  Do not approximate the ribs too tightly because to do so might lead to fused ribs and subsequent scoliosis.  Avoid deep (> 8 cm) esophageal and tracheal suctioning, which can cause anastomotic dehiscence.  Use prophylactic H2 blockers to help prevent anastomotic strictures caused by gastroesophageal reflux.  Aggressively dilate esophageal strictures (once per week); do an antireflux procedure for recalcitrant strictures.

Bibliography

Foker JE, Kendall TC, Carlton K, et al. A flexible approach to achieve a true primary repair for all infants with esophageal atresia. Semin Pediatr Surg 2005;14:8-15. Morrow SE, Ashcraft KW. Esophageal atresia. In Ziegler MM, Azizkhan RG, Weber TR, eds. Operative pediatric surgery. New York: McGrawHill, 2003; pp 349-354. Raffensperger JG. Esophageal atresia and tracheoesophageal stenosis. In Raffensperger JG, ed. Swenson’s pediatric surgery, 5th ed. Norwalk, CT: Appleton and Lange, 1990; pp. 697-717. Ricketts RR, Luck SR, Raffensperger JG. Circular esophagomyotomy for primary repair of long-gap esophageal atresia. J Pediatr Surg 1981;16:365-369. Schwartz MZ. An improved technique for circular myotomy in long-gap esophageal atresia. J Pediatr Surg 1983;18:833-834.

Chapter 5  •  Esophageal Atresia with Tracheoesophageal Fistula   53

Figure 5-10 

CHAPTER

6 

Cervical Esophagostomy Richard Ricketts

Step 1: Surgical Anatomy



The approach is usually through the left side.  Note the relationship of the trachea, esophagus, recurrent laryngeal nerve, and the thoracic duct (Fig. 6-1).

Step 2: Preoperative Considerations



Use the left side of the neck for future esophageal replacement because this procedure will be done through the left chest, the mediastinum, or in the substernal position.  Use the right side of the neck for the Kimura esophageal lengthening procedure (Kimura and Soper, 1994).  Preoperative prophylactic antibiotics should be given.

Step 3: Operative Steps

Anesthetic Induction

 

54

Endotracheal intubation and relaxation are used. The anesthesiologist should pass a blunt tipped bougie down the esophagus; this will aid in identifying the esophagus.

Chapter 6  •  Cervical Esophagostomy   55

Contents of carotid sheath Esophagus Recurrent laryngeal nerve Trachea Thoracic duct

Figure 6-1 

56   Section III  •  Thoracic

Positioning

 

Place the patient supine with a roll under the shoulders to extend the neck. Turn the head slightly to the contralateral (usually right) side.

Incision



A 2-cm incision is made 1 cm above the clavicle, extending from near the midline to the medial border of the clavicular head of the sternocleidomastoid muscle.  Divide the clavicular head of the sternocleidomastoid muscle.  Use rongeurs to remove a portion of the manubrium for better exposure.

Identify the Esophagus



Retract the common carotid artery, internal jugular vein, and vagus nerve laterally. Locate the esophagus by palpating the bougie within it. Do not confuse this with the endotracheal tube!  Dissect the soft tissues away from the esophageal wall using blunt and sharp dissection. Limit the use of electrocautery, which could damage the vagus or recurrent laryngeal nerves.  Encircle the esophagus with a vessel loop; beware of the contralateral nerves.  Continue the dissection distally, staying right on the esophageal wall, until the end of the esophagus is found.  Place a 3-0 silk traction suture on the end of the esophagus and dissect the esophagus proximally up to the pharynx using blunt and sharp dissection. 

Create the Stoma



Bring the esophagus out through the lateral end of the incision.  Attach the wall of the esophagus to the platysma muscle circumferentially with 5-0 silk or Vicryl sutures. Use muscular bites only, and do not enter the esophageal lumen. Make the stoma as wide as possible.  Close the remaining wound (if any) in layers; a drain is not necessary. Apply Dermabond over the skin closure unless the stoma takes up the entire wound.  Mature the stoma with full-thickness bites of the esophageal wall to the skin with fine absorbable sutures (Figs. 6-2 and 6-3).  Calibrate the stoma with a Hegar dilator. It should be at least a #10 Hegar dilator size.  Dress with ample dry gauze, or apply a stoma appliance to collect the saliva.

Chapter 6  •  Cervical Esophagostomy   57

Figure 6-2 

Figure 6-3 

58   Section III  •  Thoracic

Step 4: Postoperative Care



Dilate (“calibrate”) the stoma twice daily with Hegar dilators because these stomas tend to contract and close.  “Sham” oral feedings may commence on the second or third postoperative day (assuming that the wound, if any, has healed).

Step 5: Pearls and Pitfalls



Take special care when dissecting the esophagus away from the posterior (membranous) wall of the trachea.  Stay directly on the esophagus to avoid nerve damage.  Dissect more length than you think you need.  Make the ostomy as wide as possible; they tend to shrink.  Avoid full-thickness bites of the esophagus (except for maturing the stoma), or you will get side-hole fistulas.  Teach the parents how to dilate (calibrate) the stoma; they should do this once or twice per day for several months.

Bibliography

Coran AG, Behrendt DM, Weintraub WH, et al. Esophageal atresia. In Coran AG, et al, eds. Surgery of the neonate. Boston: Little, Brown and Company, 1978; pp. 45-57. Kimura K, Soper RT. Multistage extrathoracic esophageal elongation for long gap esophageal atresia. J Pediatr Surg 1994;29:566-568.

7 

CHAPTE R Chapter 7  •  Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula   59

Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula Mark L. Wulkan

Step 1: Surgical Anatomy



The fistula usually lies in proximity to the azygos vein. A moderately stiff tube should be placed through the mouth into the upper pouch to facilitate identification.  Placement in the semiprone position greatly facilitates the operation by allowing the lung to drop away from the operative field (the posterior mediastinum). 

Step 2: Preoperative Considerations



Consideration should be given to reserving the thoracoscopic technique for patients who weigh more than 2000 g and are clinically stable until you become comfortable with the technique.  Fistula ligation alone can be undertaken thoracoscopically.

Step 3: Operative Steps

Anesthetic Induction



If the anesthesiologist attempts to place the endotracheal tube past the fistula, care must be taken to avoid a right mainstem intubation, which can cause problems when the right-sided chest is insufflated and the right lung is collapsed. 59

60   Section III  •  Thoracic 

There is usually no need for bronchial blockers or other techniques to perform single lung ventilation.  The same issues of anesthetic induction for open repair of esophageal atresia with tracheoesophageal fistula apply for thoracoscopic repair.

Positioning



The patient is placed in a nearly prone position. Position the patient for success! You will have a much greater chance of completing the operation thoracoscopically if the patient is positioned for a thoracoscopic procedure, not for conversion. Port Positioning  The ports are positioned to facilitate dissection and suturing (Fig. 7-1).  The “baseball diamond” should be maintained.  Be careful positioning the most cephalad port site so that the patient’s arm does not interfere with mobility. 

Establishment of Pneumothorax



The chest is accessed first through the planned camera port site with a Veres needle (Fig. 7-2)  Initial insufflation is with 3 to 4 mm Hg of pressure using CO2.

Trocar Placement



Generally, 3.5-mm trocars are used.  A 5-mm trocar may be used for the cephalad (surgeon’s right hand) port.

Dividing the Azygos Vein



The vein can be dissected and divided. Hook cautery is usually sufficient (Fig. 7-3).  Impedance feedback bipolar cautery or ultrasonic energy (such as the Ligasure, Valleylab, Boulder, CO) may also be used with less collateral damage (although this requires a 5-mm port).  Clips are not recommended because they can dislodge or entangle sutures during the anastomosis. 

Chapter 7  •  Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula   61

Right hand Camera Left hand

Figure 7-1 

Figure 7-2 

Figure 7-3 

62   Section III  •  Thoracic

Dissecting the Fistula



The fistula is identified by following the distal esophagus up to the trachea (Fig. 7-4). The distal esophagus can be identified by watching for respiratory variations.  Care should be taken to identify and preserve the vagus nerve. 

Dividing and Ligating the Fistula (Fig. 7-5)

 

The fistula can be “test clamped” to make sure you have the correct structure. The fistula can be sutured or clipped. There have been reports of tracheal migration of metal clips. I prefer to use plastic clips or suture (Fig. 7-6).

Mobilization of Upper Pouch



The upper pouch is mobilized while anesthesia places gentle pressure on a tube in the pouch. This maneuver is used to determine whether there is adequate length to complete the anastomosis.  The pouch can be mobilized off the trachea if necessary.  An adequate enterotomy is made at the end of the pouch (Fig. 7-7). This often entails removing the tip of the pouch. There is a tendency to make an enterotomy that is too small if no tissue is removed, which may lead to stricture.

Chapter 7  •  Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula   63

Figure 7-4 

Figure 7-5 

Figure 7-6 

Figure 7-7 

64   Section III  •  Thoracic

Anastomosis



Care must be taken to ensure that you have mucosa in each bite. Three back wall (medial wall) sutures are placed with the knots inside the lumen.  Each suture is tied after it is placed (Fig. 7-8).  If there is tension, two sutures can be placed with “slip knots.” The sutures are then alternately cinched down.  Two corner sutures are placed with the knots outside the lumen.  A small feeding tube is then passed transnasally or transorally by anesthesia through the anastomosis into the stomach (Fig. 7-9).  The anterior (lateral wall) sutures are placed (usually three or four) with the knots tied outside the lumen (Fig. 7-10).  The anastomosis is inspected and any additional sutures are placed as needed.  A chest tube is placed (Fig. 7-11).  Skin is closed with Steri-strips or glue. 

Step 4: Postoperative Care



Postoperative care is the same as that after open procedures.  Feeding is begun through the transanastomotic feeding tube on postoperative day 1.  An esophagram is obtained between postoperative days 5 and 7.  Transoral feeding is begun after a negative esophagram and the chest tube is removed.

Step 5: Pearls and Pitfalls



Don’t be afraid to place the patient nearly prone: position the patient for success, not conversion.  Secure the trocars at the skin site with a suture.  Minimize tension at the anastomosis by adequate mobilization.

Bibliography

Holcomb GW, Rothenberg SS, Bak K, et al. Thoracoscopic repair of esophageal atresia and tracheoesophageal fistula: a multi-institutional analysis. Ann Surg 2005;242(3):422-428; discussion 428-430. Rothenberg SS. Thoracoscopic repair of esophageal atresia and tracheo-esophageal fistula. Semin Pediatr Surg 2005;14(1):2-7. van der Zee D, Bax NMA. Thoracoscopic repair of esophageal atresia with distal fistula. Surg Endosc 2003;17(7):1065-1067.

Chapter 7  •  Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula   65

Figure 7-8 

Figure 7-10 

Figure 7-9 

Figure 7-11 

CHAPTER

8 

Esophageal Replacement Belinda Hsi Dickie and Richard G. Azizkhan

Colonic Interposition Step 1: Surgical Anatomy



Preserve as much native proximal esophagus as possible, especially the cricopharyngeus. Preservation of the lower esophageal sphincter is ideal; the colonic interposition conduit should bridge the defect in a course that is straight and as short as possible to encourage dependent drainage.  The portion of the colon to be used for esophageal replacement depends largely on the length required. Anatomic considerations could determine whether colonic interposition is possible or an alternative should be used; prior symptomatic short colon could cause exacerbation of intestinal problems if used as a conduit.  An isoperistaltic conduit consisting of transverse and descending colon in the left hemithorax in the retrohilar position is best suited for long-gap esophageal atresia. Vascular supply is based on the left colic artery and collaterals from the marginal artery of Drummond to the transverse colon (Fig. 8-1, A).  An alternative conduit is the ascending or transverse colon segment based on the ileocolic and middle colic artery, respectively (Fig. 8-1, B). 

Step 2: Preoperative Considerations



Before any replacement procedure is done, a thorough radiologic evaluation of the stomach and gastrointestinal tract is essential.  Mechanical bowel preparation and oral antibiotic should be administered the night before surgery.  Perioperative parenteral antibiotics are administered.

66

Chapter 8  •  Esophageal Replacement   67

Marginal artery of Drummond

Left colic artery

A Figure 8-1 

Ileocolic artery Left colic artery

B

Marginal artery of Drummond

68   Section III  •  Thoracic

Step 3: Operative Steps

Anesthetic Induction



Consider inserting a thoracic epidural for postoperative analgesic management.

Positioning



The patient is placed supine in a 45-degree right lateral decubitus position for optimal access to cervical, abdominal, and thoracic approaches.

Incision



An upper midline abdominal incision is used to access the colon. Gastrocolic ligament is divided to free the transverse colon. Descending and sigmoid colon are mobilized by dividing lateral peritoneal attachments.  Mesentery of the transverse and descending colon is incised by dividing the left branch of the middle colic artery and preserving the marginal arcade and the left colic artery and its arcades (see Fig. 8-1, A).  The phrenoesophageal ligament is incised; dissection of the esophagus along the hiatus, gastroesophageal junction, and into the mediastinum is performed.  The length of the colon conduit is determined, and then the colon is divided into the transverse and descending regions, retaining the left colic blood supply. If the right colon is used as the conduit, the ileocolic vessels are preserved (see Fig. 8-1, B).  A left lateral thoracotomy (through the 5th and 6th intercostal spaces) is done to give access to the chest for further dissection of the esophagus and subsequent placement of the colonic conduit.  Figure 8-2 demonstrates the configuration of the colon conduit before placement in the retrohilar or substernal position.  The colonic segment and its pedicle are brought anterior to the pancreas behind the stomach and through an incision made in the gastrohepatic ligament and then passed through the esophageal hiatus or through a lateral incision in the diaphragm. The retrohilar tunnel is created lateral to the descending aorta behind the aortic arch.  The upper end of the colonic segment is brought through Sibson fascia posterior to the subclavian vessels and lateral to the carotid sheath. Figure 8-3, A provides a lateral perspective of the colon conduit.  If a retrohilar approach is not possible, the colonic conduit can also be positioned retrosternal (Fig. 8-3, B).  A delayed proximal anastomosis may be indicated if the blood supply of the cervical portion of the conduit is marginal. This can be performed several weeks later. 

Chapter 8  •  Esophageal Replacement   69

Site of gastrostomy

Neck incision

Site of cologastric anastomosis

Figure 8-2 

A Figure 8-3 

B

70   Section III  •  Thoracic

Closing



When the entire esophagus is replaced, a cervical esophagocolostomy (or pharyngocolostomy) and a cologastrostomy are constructed.  A Heineke-Mikulicz pyloroplasty and a gastrostomy are routinely done before closure.  A Penrose drain in the cervical wound below the plastyma and a dependently placed chest tube in the left chest are secured before closure.

Step 4: Postoperative Care



Patients should be monitored in the intensive care unit postoperatively and kept on NPO (nothing by mouth) status; parenteral nutrition is administered.  A water-soluble contrast is obtained on postoperative day 7.  If no leak is observed, the cervical drain and chest tube can be removed and the patient fed.  If a leak is noted, an additional 2 weeks of NPO and repeat contrast study are done.  Most leaks are at the cervical anastomosis and usually close spontaneously; Around 50% result in stricturing that requires subsequent dilatations. The overall leak rate is between 25% and 35%.

Step 5: Pearls and Pitfalls



When dividing the colon, back bleeding should be noted at both the proximal and distal ends of the segment, indicating a good blood supply.  Identifying and preserving the recurrent laryngeal nerve and thoracic duct are important in dissection in the neck.  A thoracic incision can be avoided in a retrosternal positioned conduit by creating the tunnel with a dual approach from the abdomen and the neck.  Long-term follow-up is required. As many as 15% to 20% of patients will require operative intervention because of development of significant redundancy in the colonic conduit resulting in severe stasis or obstruction  Gastroesophageal reflux is unpredictable, and if it becomes problematic, a partial-wrap fundoplication should be considered.

Chapter 8  •  Esophageal Replacement   71

Gastric Transposition Step 1: Surgical Anatomy



The advantages of a whole-stomach transposition compared with the reverse gastric tube interposition include the simplicity of a single anastomosis and a long vascularized conduit without suture lines.  In reverse gastric tube transposition, a preexisting gastric volume minimum of 200 to 250 mL is required to ensure that there is an adequate residual gastric reservoir after tube construction. This is especially important in patients with long-gap esophageal atresia, in whom the stomach is usually small.

Step 2: Preoperative Considerations



A preoperative upper gastrointestinal contrast study should be performed to document gastric volume and determine whether the stomach is large enough for a gastric tube interposition to be performed.  Mechanical bowel preparation and oral antibiotics should be administered to prepare the colon in case a gastric conduit is not possible.

Step 3: Operative Steps

Anesthetic Induction



Consider insertion of an epidural for postoperative pain management.

Positioning



The patient should be placed supine in a 45-degree right lateral decubitus position on the operating table.

72   Section III  •  Thoracic

Incision



The procedure begins with an abdominal exploration and mobilization of the stomach and duodenum. If present, the gastrostomy tube is dismantled and the site closed. The stomach and gastroesophageal junction are mobilized, ensuring preservation of the blood supply and avoiding injury to the spleen.  Extensive kocherization of the duodenum is done to allow for sufficient mobilization of the stomach as well as to help with the pyloroplasty  The left gastric and short gastric vessels are divided, leaving the supply to the stomach dependent on the gastroepiploic and right gastric arteries. The omentum is separated off the stomach (Fig. 8-4).  The gastroesophageal junction and distal esophagus are mobilized to the level of the carina through the esophageal hiatus. The esophagogastric junction is divided and the cardia oversewn.  Once the apex of the gastric fundus can reach past the clavicles and the pylorus overlies the xiphoid process, sufficient mobilization of the stomach is completed (Fig. 8-5).  A low anterior cervical incision is used to mobilize the remaining esophagus and to create the tunnel for the gastric transposition to pass. Carotid sheath and trachea are retracted laterally and the larynx and trachea medially. Care is taken to identify and protect the recurrent laryngeal nerves.  The gastric transposition is best placed in the position of the native esophagus (most direct route to the cervical esophagus). A left lateral thoracotomy may facilitate positioning the stomach in the chest (Fig. 8-6).  The stomach can be passed retrosternally (potentially longer and more tortuous route) or via retrohilar route. The retrohilar position requires enlarging of the esophageal hiatus and careful identification of the recurrent laryngeal nerve to prevent injury.  To position the gastric conduit in the retrohilar position requires opening the diaphragm at the esophageal hiatus to allow the graft to be mobilized. A left thoracotomy is required to place the stomach in correct position behind the left lung. To create adequate space in the neck, a sternal fascial incision or removal of the clavicular head is sometimes required.  Pyloroplasty is done to improve gastric emptying.

Closing



A cervical Penrose drain and left chest tube are placed before closing.

Chapter 8  •  Esophageal Replacement   73

Esophageal branch

Right gastric artery

Left gastroepiploic artery

Figure 8-4  Figure 8-5 

A Figure 8-6 

B

74   Section III  •  Thoracic

Step 4: Postoperative Care

 

Patients should be monitored closely in the intensive care unit postoperatively and kept NPO. A contrast study is obtained 1 week postoperatively to test for leaks.  If no leak is observed, the cervical drain and chest tube can be removed and the patient fed.  If a leak is noted, an additional 2 weeks of NPO status and a repeat contrast study at that time are required.

Step 5: Pearls and Pitfalls



Any residual esophageal stump should be resected because, if left in place, it could cause torque on the gastric transposition as it is drawn up into the chest.  The native esophagus in the case of caustic ingestion should be removed to reduce the longterm risk of malignancy.  Gastric transposition carries a mortality rate of 5% to 7% but has a high success rate of 88% to 90%. Risk of peptic esophagitis is significant if there is any intrathoracic stasis.  A partial gastric transposition performed in the right side of the chest for primary treatment of long-gap esophageal atresia has been described.

Chapter 8  •  Esophageal Replacement   75

Bibliography

Ahmed A, Spitz L. The outcome of colonic replacement of the esophagus in children. Progr Pediatr Surg 1986;19:37-54. Ahmad SA, Sylvester JG, Hebra A, et al. Esophageal replacement using the colon: Is it a good choice? J Pediatr Surg 1996;31: 1026-1031. Anderson KD, Noblett H, Belsey R, Randolph JG. Long-term follow-up of children with colon and gastric tube interposition for esophageal atresia. Surgery 1992;1111:131-136. Azar H, Chrispin AR, Waterston DJ. Esophageal replacement with transverse colon in infants and children. J Pediatr Surg 1971; 66:333-342. Choi RS, Lillehei CW, Lund DP, et al. Esophageal replacement in children who have caustic pharyngoesophageal strictures. J Pediatr Surg 1971;6:3-9. Gross RE, Firestone FN. Colonic reconstruction of the esophagus in infants and children. Surgery 1967;61:955-964. Kelly JP, Shackelford GD, Roper CL. Esophageal replacement with colon in children; functional results and long-term growth. Ann Thorac Surg 1983;36:634-643. Poppeo E, Coosemans W, De Leyn P, Denette G, Van Raemdonck D, Lerut T. Esophageal replacement with colon in children using either the intrathoracic or retrosternal route: an analysis of both surgical and long-term results. Surg Today 1997;27:729-734. West KW, Vane DW, Grosfeld JL. Esophageal replacement in children: Experience with thirty-one cases. Surgery 1986;100:751-757.

CHAPTER

9 

Esophageal Replacement: Gastric Tube Pull-up Saleem Islam and Ronald B. Hirschl

Step 1: Surgical Anatomy



Esophageal atresia remains the most common reason for esophageal replacement in children. The stomach will usually have a normal configuration and, in cases of pure esophageal atresia, will have a gastrostomy tube in place at the time of esophageal replacement. The stomach in pure atresia is diminutive at birth and requires several months of feedings before attaining a normal size.  A key element of the anatomy is the blood supply to the stomach. The transposed stomach will rely on the right gastric and the right gastroepiploic arteries, which must be preserved (Fig. 9-1).  The left gastric and the short gastric arteries are ligated to mobilize the stomach. It is prudent to temporarily occlude flow to the left gastric artery using a bulldog clamp before ligation to ensure that the stomach will have adequate perfusion from the remaining vessels. If the perfusion appears inadequate, an alternate esophageal substitution is required.  Another important anatomic point is that the most cephalad portion of the stomach will be the fundus, not the gastroesophageal junction (GEJ).  In cases of atresia, the esophageal bed will be without scarring or adhesions unless previous attempts at repair have been made. Significant scar formation may be present in children with severe caustic injury to the esophagus and in cases where there is a history of perforation or prior attempts at a replacement procedure.  In some cases, a cervical esophagostomy may be present at the time of surgery. This will usually be on the left side of the lower neck, which is more desirable because the path through the mediastinum is shorter to this side. It is critical to be aware of the vagus and the recurrent laryngeal nerves at the time of surgery. Both recurrent laryngeal nerves can be injured and will result in severe respiratory compromise because of vocal cord paralysis.

76

Chapter 9  •  Esophageal Replacement: Gastric Tube Pull-up   77

Right gastric artery

Right gastroepiploic artery

Figure 9-1 

78   Section III  •  Thoracic

Step 2. Preoperative Considerations



We recommend preoperative admission for bowel preparation in case the stomach is not usable for esophageal replacement and the colon is required.  Children with a history of atresia may have concomitant cardiac defects and should have a cardiology workup done as needed.  Blood should be available for the procedure. If there is consideration for using the colon, a preoperative barium enema is useful to evaluate the anatomy.

Step 3. Operative Steps

Anesthesia



After induction, an arterial catheter for monitoring should be placed. Additionally, a central venous line may be helpful for intraoperative monitoring as well as for postoperative care.  Abdominal pain may be well controlled in older children using an epidural anesthetic.  A bladder catheter is placed to monitor urine output. 

Positioning



The cervical anastomosis is typically performed on the left side of the neck; thus we position the patient in a modified supine position with a roll beneath the upper back to expose the neck. In addition, the left shoulder and upper arm are prepared in the field with a stockinette on the lower arm to allow manipulation during the case as needed.  The preparation is from the chin and below the ear to the pubic bone.  Bakes dilators are useful to help identify a proximal atretic esophagus in the neck for patients without a cervical esophagostomy.

Chapter 9  •  Esophageal Replacement: Gastric Tube Pull-up   79

Incisions



A cervical incision is required to identify the proximal esophagus. In some cases, there will be an existing cervical esophagostomy.  The incision is made on the anterolateral surface of the left neck, approximately 2 to 3 cm above the clavicle, and is placed between the heads of the sternocleidomastoid.  Dissection is carried out between these heads, and the medial aspect usually needs to be transected for adequate exposure.  The trachea is identified, and the tracheoesophageal groove is seen just below, where care is taken to identify the recurrent laryngeal nerve. At this point, it is helpful to have a bougie or a Bakes dilator in the esophageal pouch (especially for an atresia) to identify it.  When the esophagus has been identified, traction stitches can be placed in it to retract it laterally; care needs to be exercised to prevent injury to the opposite recurrent laryngeal nerve.  Use of electrocautery near the recurrent laryngeal nerves is minimized. Dissection is continued behind the trachea and anterior to the spine to circumscribe the esophagus.  The abdominal incision may be midline or left subcostal.  A gastrostomy tube is usually present and will be taken down and oversewn.  The blood supply to the stomach is carefully assessed, and bulldog clamps may be placed on the left gastric artery while taking down the short gastric vessels at least 1 to 2 cm away from the gastroepiploic artery.  The left gastric artery will also have to be ligated to allow the stomach to reach the cervical region.  In most cases, the stomach will retain an excellent blood supply, which is verified by the temporary clamping.  The distal esophageal end is then dissected free from the hiatus, and blunt dissection is carried out into the mediastinum. Dissection may be tedious in cases where scarring and adhesions from prior procedures or antecedent perforations are present. In cases of atresia, the stump is mobilized and transected with a stapler and then oversewn.  The highest point of the stomach is usually the fundus and not the GEJ. Adequacy of the length is verified by placing the mobilized stomach over the chest and neck.

80   Section III  •  Thoracic



At this point, a pyloromyotomy or pyloroplasty is performed to assist in gastric emptying. A pyloromyotomy will be adequate for most patients and is greatly preferred to maintain gastric length (Fig. 9-2). A Kocher maneuver is performed to gain additional length.  Mediastinal dissection is carried out bluntly from the hiatal area below and from the neck above (Fig. 9-3). In smaller children, this may be accomplished by using fingers alone, but in older patients a sponge stick or blunt Kelley clamp will be needed.  In some cases in which there is excessive scar tissue or a native esophagus that cannot be removed safely, a left thoracotomy may need to be performed as well.  Once the dissection is completed, the area is stretched with two or three fingers to make room for the stomach. The hiatus must often be opened to allow the stomach to pass successfully. It is critical that the stomach passes to the left of the aortic arch.  A large chest tube (28 to 36 French) is then passed from the neck down to the abdomen through the mediastinal space that was created. This is attached to the fundus of the stomach with a 0 silk stitch through the tube and stomach. To prevent twisting of the stomach during transposition, two sutures of different colors may be placed on the fundus before carefully pulling it up into the cervical region. There is usually no tension on the transposed stomach when placed in the neck area.  To decrease tension on the anastomosis, the stomach is sutured to the sternocleidomastoid and the strap muscles in infants and the prevertebral fascia in older children. The hiatus is sutured to the stomach to prevent herniation.  Anastomosis between the stomach and the cervical esophagus is performed in a single layer with absorbable suture. A nasogastric (NG) tube of sufficient size (14 to 16 French) is placed with the tip located at the level of the hiatus, above the pyloroplasty (Fig. 9-4). We may perform a jejunostomy for enteral nutrition, particularly in cases of esophageal atresia, where oral aversion may exist.  The neck wound is closed in layers with absorbable suture and a soft Penrose drain left in the anastomotic area to drain any leaks.  We routinely check for vocal cord motion before leaving the operating room.

Step 4: Postoperative Care



Patients are intubated at least overnight and possibly longer because edema can develop in the cervical tracheal area as a result of the dissection.  Acute gastric dilation can occur if the NG does not function well, and this may cause respiratory difficulties. Antibiotics are administered for at least 24 hours.  A contrast study is performed on postoperative day 6-7 and the NG tube as well as the Penrose drain can be removed if no leak is found. Jejunal tube feedings may be started on postoperative day 2 or 3 at a slow continuous rate.  Oral diet is started when the contrast study demonstrates no leak. In patients with esophageal atresia, reinitiating oral feeding may take a prolonged time and require extensive therapy.

Chapter 9  •  Esophageal Replacement: Gastric Tube Pull-up   81

Divided GE junction

Pyloroplasty

Closed gastrostomy

Figure 9-2 

Figure 9-3 

Figure 9-4 

82   Section III  •  Thoracic

Step 5: Pearls and Pitfalls



The left cervical anastomosis provides the shortest route for the transposed stomach. Parents should be cautioned that a left thoracotomy may be required in cases of severe adhesions.  Leaks are seen from the cervical anastomosis in about 25% to 50% of cases, but almost all will heal spontaneously.  In cases where a cervical esophagostomy is performed, consideration should be given for sham feedings to ameliorate oral aversion issues.  Care must be exercised to prevent damaging the recurrent laryngeal nerve on either side. Dissection is done directly on the esophageal wall.  The abdominal portion of the operation may be performed laparoscopically and the mediastinal dissection with a blunt dissector from below. 

Bibliography

Hirschl RB, Yardeni D, Oldham K, et al. Gastric transposition for esophageal replacement in children. Ann Surg 2002;236:531-541. Spitz L. Gastric replacement of the esophagus. In Spitz L, Coran AG, eds. Pediatric surgery, 6th ed. London: Edward Arnold, 2007; pp. 145-152. Spitz L, Kiely E, Pierro A. Gastric transposition in children—a 21-year experience. J Pediatr Surg 2004;39:276-281.

10 

T E RDiaphragm   83 Chapter 10  •  Congenital Diaphragmatic Hernia and EventrationC HofA Pthe

Congenital Diaphragmatic Hernia and Eventration of the Diaphragm Jason S. Frischer, Keith A. Kuenzler, and Charles J. H. Stolar

Congenital Diaphragmatic Hernia Step 1: Surgical Anatomy



Congenital diaphragmatic hernia (CDH) typically refers to a defect in the posterolateral diaphragm, known as the foramen of Bochdalek. The abdominal viscera translocate through this defect into the chest during fetal development.  The diaphragm is a musculotendinous dome-shaped construction that separates the thoracic and peritoneal cavities. The central tendon is a fibrous structure that accounts for about one third of the total surface area of the diaphragm and is the insertion point for the crural fibers that pass around the aorta and the esophagus and continue to the ligament of Treitz.  The diaphragm develops during weeks 4 through 8 of gestation from several fused embryonic components, including the septum transversum, pleuroperitoneal membranes or folds, and the mesentery of the esophagus. The septum transversum forms the central tendon. Closure of the pleuroperitoneal membranes completes the formation of the fetal diaphragm. It is hypothesized that the posterolateral defect in the diaphragm is a result of incomplete or absent fusion of these membranes. As a consequence, the abdominal viscera migrate into the chest during the first trimester.  The ipsilateral lung is affected more than the contralateral lung, but both are affected. The microstructural abnormality features compromised pulmonary arterial divisions, muscular hypertrophy of the intra-acinar arterioles, decreased bronchiolar units, and consequently a decreased surface available for gas exchange. Affected infants are born with both pulmonary hypoplasia and pulmonary hypertension. The pulmonary hypoplasia can be severe enough to preclude survival without fetal circulation, but successful management of the pulmonary hypertension can enable to infant to thrive.  Incidence of CDH varies from 1 in 2200 to 1 in 5000 births. Most series report that around 80% of cases occur on the left side and 20% will contain a hernia sac.

83

84   Section III  •  Thoracic

Step 2: Preoperative Considerations

Diagnosis



The diagnosis is typically made antenatally. The infant is usually full term and manifests respiratory distress immediately.  Prenatal ultrasound demonstrates a fetus with bowel, liver, or spleen in the chest and shifting of the mediastinum.  At birth the diagnosis is confirmed by chest radiograph showing loops of bowel in the thoracic cavity with contralateral mediastinal shift.  The chest is usually barrel-shaped, and the abdomen is scaphoid.

Resuscitation



Prompt intubation and nasogastric decompression should be the first steps in the resuscitation process.  Ventilator strategies depend on the clinical situation.  Patients should always be allowed to breathe spontaneously, and paralytic agents should be avoided to minimize barotrauma.  Ventilator settings range from low rates with modest peak airway pressures to high-rate ventilation with negligible peak pressures. High-frequency oscillating ventilation is an excellent strategy as well.  Standard monitoring should be immediately instituted with the addition of preductal and postductal arterial oxygen saturation (Sao2).  Most of these infants have significant pulmonary hypertension leading to right-to-left shunting at the patent ductus arteriosus, and therefore blood gas monitoring via the umbilical artery (postductal) is often misleading.  Preductal partial pressure of arterial oxygen (Pao2) and the partial pressure of carbon dioxide (Pco2) are more accurate predictors of the lungs’ ability to provide meaningful gas exchange.  Arterial and venous accesses are required to assist in the resuscitation of the infant in respiratory distress.  An echocardiogram is obtained with special attention to signs of pulmonary hypertension. Pharmacologic supports that either dilate the pulmonary vasculature or assist in improving right-sided heart function are useful. Agents such as nitric oxide, iloprost, epoprostenol, sildenafil, dobutamine, and milrinone have all been used to help improve the pulmonary hypertension or its effects.  Extracorporeal membrane oxygenation (ECMO, discussed in Chapter 2) is an adjunct in the preoperative infant who has already demonstrated evidence of adequate lung function but who then deteriorates secondary to pulmonary hypertension. The likelihood that this infant would tolerate surgery is low, and therefore the added cardiopulmonary support will allow the infant to recover from the acute pulmonary hypertensive crisis. Repair is typically performed when ECMO parameters are near the time of decannulation. The infant is usually weaned off ECMO support within 1 to 2 days after closure of the diaphragmatic defect.

Chapter 10  •  Congenital Diaphragmatic Hernia and Eventration of the Diaphragm   85

Step 3: Operative Steps

Anesthesia



An orotracheal or nasotracheal tube is used to secure the airway. A nasotracheal tube has the theoretical advantage of lower incidence of airway complications, but an orotracheal tube is easier to place and more commonly used.  A pressure-cycled infant ventilator is preferred over the conventional anesthesia circuit to provide mechanical ventilation to the infant.  Muscle paralysis and narcotics are administered and complemented with intravenous anesthetic agents on an as-needed basis.  Continuous oxygen saturation monitoring, both preductal and postductal, is critical, as are blood gas observations of hypercapnia and ventilator adjustments as needed.

Positioning



The operative room should be warmed, and focus should include keeping the patient warm at all times. Plastic drapes are used to cover the child outside the operating field.  The infant is placed in the supine position on a heating apparatus.  A small roll is used to elevate the ipsilateral subcostal region.

86   Section III  •  Thoracic

Incision



A subcostal incision is made on the side of the hernia. The incision is made approximately one fingerbreadth below the costal margin. While incising the anterior abdominal wall musculature and fascia, be careful to leave enough fascia and muscle on the cephalad aspect of the wound to provide for an adequate closure (Fig. 10-1).  Electrocautery is used to ensure hemostasis because of the potential need for ECMO and anticoagulation. 

Hernia Reduction



The upper portion of the wound is retracted in a cephalad direction along with the upper rim of diaphragm. The entire defect should be exposed. Carefully reduce the abdominal contents back into the abdominal cavity, or eviscerate the contents during the repair.  The bowel should be placed in the abdominal cavity in an organized fashion, focusing attention on the orientation of the mesentery.  Reduction of the liver deserves vigilance because of the extremely fragile nature of the neonatal liver. Similar caution should be made when reducing the spleen and care taken with its attachments to the pancreas and colon.

Repair of the Diaphragm



Identify the anterior rim of the diaphragm and trace it medially to free up the mesothelial covering. The diaphragmatic edge should be completely mobilized.  Primary repair is accomplished by using interrupted simple sutures with nonabsorbable material (Fig. 10-2).  If the repair is too large for primary repair, a prosthetic patch can be created using 1-mmthick Gore-Tex. The patch should be sized carefully and secured with monofilament interrupted sutures (Fig. 10-3).

Chapter 10  •  Congenital Diaphragmatic Hernia and Eventration of the Diaphragm   87

Figure 10-1 

Figure 10-2 

Figure 10-3 

88   Section III  •  Thoracic

Closing



The abdomen is closed in layers. The loss of abdominal domain can potentially make wound closure challenging. One should not be reticent about placing either a patch or a silo to close the abdomen.  A chest tube is usually not necessary after repair of CDH.  Infants repaired on ECMO, where anticoagulation is required, and those with a pneumothorax or unexpected bleeding are the only patients who require an ipsilateral tube thoracostomy. 

Step 4: Postoperative Care



The preoperative care strategy is reinstituted in the postoperative period. Spontaneous respiration and permissive hypercapnia are the mainstays of the respiratory strategy.  Narcotics are necessary to ensure adequate analgesia.  Similar to other laparotomies, sufficient circulating volume must be maintained with intravenous fluids, and adequate hemoglobin must be maintained for oxygen delivery.  Mechanical ventilation should be converted to nasal-prong continuous positive airway pressure assisted ventilation when the infant stabilizes and subsequently weaned as tolerated.  Parenteral nutrition is provided until respiratory status improves, the neonate has return of bowel function, and the infant is weaned to full enteral feeds. 

Step 5: Pearls and Pitfalls



When making a subcostal incision, be sure to leave sufficient tissue on the cephalad portion of the wound to allow for adequate closure.  With using a patch for large defects, the ribs can be used for anchoring the lateral aspect. On the medial side, if there is a paucity of tissue, one must be creative and anchor to structures around the hiatus or pericardium.  Postoperative volvulus is a rare complication, and therefore correction of the nonrotated bowel is unnecessary. Division of Ladd’s bands, with or without appendectomy, is discouraged because of the increased incidence of bleeding if the patient needs to go on ECMO.

Chapter 10  •  Congenital Diaphragmatic Hernia and Eventration of the Diaphragm   89

Thoracoscopic CDH Repair Step 1: Surgical Anatomy

See page 85 for a description of the anatomy.

Step 2: Preoperative Considerations



As with other diseases, early efforts to apply minimally invasive techniques to CDH began with older children. As advances in optics and instrumentation have been made, it has been possible to perform successful neonatal repairs. Although no clear advantage has been demonstrated yet, we consider a thoracoscopic approach for all newborns with CDH who are hemodynamically stable and able to tolerate the low-pressure pneumothorax required.  Specifically, these patients are newborns on conventional ventilator, requiring low supplemental oxygen and pressure support settings.  As our experience has increased, we have successfully repaired CDH defects on the left and right sides, both primarily and with synthetic patch material.  Monitoring and support lines are identical to those described for the open repair.

Step 3: Operative Steps

Positioning



The infant is placed in the lateral decubitus position. An axillary roll is placed, and bony prominences are padded.  Elevating the infant off the operating table on narrow stack of pads or towels will allow the thoracoscopic instruments a greater range of motion.  The surgeon stands at the infant’s head with the video screen positioned over the legs in direct line of vision. 

90   Section III  •  Thoracic

Trocar Placement



Three 5-mm radially expandable sleeves and trocars are used. The first port is placed in the posterior axillary line caudal to the level of the scapula.  A 4-mm, 30-degree scope is used.  The pneumothorax is slowly increased to 5 mm Hg while monitoring for ventilatory changes.  The two working ports are then inserted under thoracoscopic visualization. These are placed several centimeters to either side of the first port, one to two rib spaces caudally. 

Reduction of Viscera and Repair of Defect



The viscera are gently reduced into the abdomen using cotton-tipped “endo-peanut” instruments.  Insufflation assists not only in the reduction but also in the maintenance of operative space by keeping the viscera from returning to the thorax.  A hernia sac, if found, is completely excised so that the diaphragmatic edges can be properly identified and apposed.  Primary repair is done from the medial to lateral aspects using 2-0 braided nonabsorbable sutures on standard taper needles. We prefer to place these sutures in simple fashion. Alternatively, one may elect to use “U” stitches with or without pledgets.  Identical to the open repair, a synthetic patch may be placed into a large defect or as an “onlay” over a weaker area. The patch material is cut to proper size and then rolled tightly so that it will pass through the 5-mm port (mineral oil or other lubrication may facilitate its passage).

Optional Pericostal Sutures



Completion of a primary or patch repair may require pericostal sutures.  Once the rib is identified, a tiny adjacent skin incision is made using a #11 blade.  The suture is then passed through this incision through the intercostal space and into the thorax on a “ski” needle.  The suture is retrieved and passed with the thoracoscopic needle driver through the diaphragm or patch and then driven back out of the chest on the other side of the rib.  Sutures are tied extracorporeally with the knots buried subcutaneously beneath the tiny skin incisions, which are subsequently closed with Steri-strips.

Chapter 10  •  Congenital Diaphragmatic Hernia and Eventration of the Diaphragm   91

Step 4: Postoperative Care

See page 90 for a description of the postoperative care.

Step 5: Pearls and Pitfalls



Both intracorporeal and extracorporeal knot tying may be useful.  Similar to the open technique, placement of sutures around the ribs can be used to anchor a patch in the lateral aspect. These sutures may be placed through a separate small incision made directly over the identified ribs.

Eventration of the Diaphragm Step 1: Surgical Anatomy



Eventration of the diaphragm refers to a radiographic finding of an abnormally elevated hemidiaphragm that can either be congenital or acquired.  Congenital eventrations can either be due to birth trauma, Erb’s palsy, or secondary to an anatomic abnormality of the diaphragm, in which case the muscle is typically thinned or may be absent.  Acquired eventrations are usually secondary to phrenic nerve injury during repair of congenital cardiac defects. These lesions, like those of birth trauma or Erb’s palsy, are associated with normal diaphragm musculature.

Step 2: Preoperative Considerations

Diagnosis



Initial workup includes a chest radiograph preferably with an anteroposterior and lateral view, which is imperative.  A right-sided eventration is suspected when the ipsilateral hemidiaphragm is greater than two rib levels higher than the left.  A left sided eventration is entertained when the left hemidiaphragm is one rib level above the right diaphragm.  The diagnosis is confirmed by dynamic visualization of the diaphragm using either fluoroscopy or ultrasonography of the chest and observing the movement of the diaphragm.  Absent or paradoxical elevation of the diaphragm on inspiration is diagnostic of eventration.

92   Section III  •  Thoracic

Associated Anomalies



Structural cardiac anomalies are associated with patients who have congenital eventration of the diaphragm; therefore an echocardiogram should be obtained.  Intestinal rotational anomalies and gastric volvulus have been reported in patients with diaphragmatic eventration; if symptoms warrant, an upper gastrointestinal contrast study would be prudent.

Indications for Repair



Recurrent symptoms and respiratory distress are absolute indications for repair. Diaphragm level above the fourth intercostal space or inability to wean from the ventilator would be an indication.  When phrenic nerve injury is suspected, a waiting period before instituting operative therapy is reasonable. 

Chapter 10  •  Congenital Diaphragmatic Hernia and Eventration of the Diaphragm   93

Step 3: Operative Steps

Anesthesia



The anesthetic approach is standard for any infant. Intravenous and inhaled agents may be used.  Although not mandatory, establishing single-lung ventilation on the contralateral side would assist in making the eventration repair easier. This is especially true for the thoracic approach.

Positioning



Positioning depends on the approach. A lateral decubitus position is used for a thoracic approach.  Supine positioning is appropriate for an abdominal approach. 

Incision



A lateral muscle-sparing thoracotomy is made in the seventh intercostal space for an isolated unilateral eventration.  A transverse upper abdominal incision is used for an eventration associated with malrotation or for the rare instance of a bilateral eventration.

94   Section III  •  Thoracic

Repair of the Eventration



Open techniques as well as minimally invasive techniques through the abdominal and thoracic approach may be used to repair an eventration.  The first step is to grasp the diaphragm and manipulate the muscle to determine the amount necessary to create a taut repair (Fig. 10-4).  Non-absorbable suture is used to plicate the muscle with several full-thickness bites in order to craft a pleating of the diaphragm (Fig. 10-5).  A chest tube is usually not necessary after repair of diaphragmatic eventrations.

Step 4: Postoperative Care



Postoperative care is similar to that of congenital diaphragmatic hernia repair but is generally simpler because of the absence of pulmonary hypertension.

Step 5: Pearls and Pitfalls

 

Thoracoscopic approach can be used by experienced endoscopic surgeons. A taut repair is vital to minimize recurrent symptoms.

Chapter 10  •  Congenital Diaphragmatic Hernia and Eventration of the Diaphragm   95

Figure 10-4 

Figure 10-5 

96   Section III  •  Thoracic

Bibliography

Boloker J, Bateman DA, Wung JT, et al. Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnia/ spontaneous respiration/elective repair. J Pediatr Surg 2002;37:357-366. Hines MH. Video-assisted diaphragm plication in children. Ann Thorac Surg 2003;76:234-236. Lally KP, Harting MT. Surgical management of neonates with congenital diaphragmatic hernia. Semin Pediatr Surg 2007;16:109-114. Lally KP, Lally PA, Van Meurs KP, et al. Treatment evolution in high-risk congenital diaphragmatic hernia: ten years’ experience with diaphragmatic agenesis. Ann Surg 2006;244:505-513. Stolar CJH, Dillon PW. Congenital Diaphragmatic Hernia and Eventration. In Grosfeld JL, O’Neill JA Jr, Fonkalsrud EW, Coran AG, eds. Pediatric Surgery, 6th ed. Philadelphia: Mosby Elsevier, 2006; pp 931-954. West SD, Wilson JM. Follow up of infants with congenital diaphragmatic hernia. Semin Perinatol 2005;29:129-133. Yang EY, Allmendinger N, Johnson SM, et al. Neonatal thoracoscopic repair of congenital diaphragmatic hernia: selection criteria for successful outcome. J Pediatr Surg 2005;40:1369-1375. Yazici M, Karaca I, Arikan A, et al. Congenital eventration of the diaphragm in children: 25 years’ experience in three pediatric surgery centers. Eur J Pediatr Surg 2003;13:298-301.

11 

H A P TProcedure   ER Chapter 11  •  Surgical Treatment of Chest Wall Deformities: CNuss 97

Surgical Treatment of Chest Wall Deformities: Nuss Procedure Michael J. Goretsky and Donald Nuss

Step 1: Surgical Anatomy



Pectus excavatum is the most common chest wall deformity in children. Its incidence is estimated at approximately 1 in 100 children, and it has a male-to-female ratio of 3-4:1.  Morphologic distinctions in pectus excavatum simplify the diagnosis and aid in the surgical correction. The deformities may be small in diameter and deep, “cup-shaped” or of large diameter and shallow, “saucer-shaped,” or eccentric.  The use of more than one bar may be beneficial in patients older than 16 years with a stiff chest or in those with a severe depression or major sternal torsion.

Step 2: Preoperative Considerations



A complete history and physical examination are performed on all patients. All patients are encouraged to perform exercises designed to strengthen the chest and back muscles and improve exercise tolerance, along with maintaining proper posture.  Determination of a severe pectus excavatum and the need for repair includes two or more of the following criteria:  A Haller computed tomography (CT) index greater than 3.2 with cardiac compression  Pulmonary function studies that indicate restrictive or obstructive airway disease  A cardiology evaluation showing that the compression is causing murmurs, mitral valve prolapse, cardiac displacement, or conduction abnormalities on the echocardiogram or electrocardiogram (ECG) tracings  Documentation of progression of the deformity with associated physical symptoms other than isolated concerns of body image  Our experience has shown that the optimal age for repair is 10 to 14 years because patients’ chests are still soft and malleable. At this age, they show quick recovery, a rapid return to normal activities, and excellent results. After puberty, the flexibility of the chest wall is 97

98   Section III  •  Thoracic

decreased and often requires the insertion of two bars. It also takes the patients longer to recover at this age. However, we have performed the procedure in patients up to age 31 years with good results.

Step 3: Operative Steps

Anesthetic Induction



A first-generation cephalosporin is used for antibiotic coverage and continued until discharge.  Standard endotracheal anesthesia is used for surgical correction, and epidural analgesia is used both as an adjunct during surgery and continued for pain control for an average of 3 days postoperatively.  The arms are abducted at the shoulders, and care is taken to pad all pressure points and keep the upper extremities extended without tension on the brachial plexus.

Before Incision



The length of the pectus bar is determined by measuring the distance from the right midaxillary line to the left midaxillary line and subtracting 2 cm or 1 inch. The measurement is done over the area of the deepest depression that is still part of the sternum.  The bar is bent to the desired convex configuration, making note that the center of the bar should have a negligible bend to allow greater stability (Fig. 11-1).

Surgical Procedure



A thoracoscope is inserted into the right side of the chest two intercostal spaces below the planned bar placement to check that the internal anatomy corresponds with the external markings and to look for unexpected pathology.  Lateral thoracic incisions are made in the region of the midaxillary line, and subcutaneous tunnels are created to the greatest apex of the pectus deformity (X) (Fig. 11-2). These “X’s” represent the entrance and exit sites of the bar from the chest. They are in the intercostal space that is in the same level as the deepest depression, and care should be taken that they are placed medial to the greatest apex of the chest.  Skin tunnels are created above the muscle, starting from each of the lateral thoracic incisions to the top of the pectus ridge on each side. To minimize superficial skin retraction, the tunnel can be created between the pectoralis major and minor muscles. The tunnels should be created so that the entry and exit sites of the bar from inside the chest are medial to the top of the pectus ridge on each side.

Chapter 11  •  Surgical Treatment of Chest Wall Deformities: Nuss Procedure   99

X

X

X

Figure 11-2  Figure 11-1 

100   Section III  •  Thoracic



With the thoracoscope in place, a tonsil clamp is inserted into the subcutaneous tunnel on the right, and a blunt thoracostomy is created at the site marked “X” while taking care not to injure the intercostal vessels, mammary vessels, lung, or pericardium (Fig. 11-3).  Under continued thoracoscopic visualization, a Lorenz introducer (W. Lorenz Surgical, Jacksonville, FL) is inserted into the chest through the right tunnel and thoracostomy site at the top of the pectus ridge. With great care and thoracoscopic guidance, the pleura and pericardium are dissected off the under surface of the sternum, creating a substernal tunnel. The introducer is slowly advanced across the mediastinum and brought out through the corresponding intercostal space on the left and advanced out of the incision on the contralateral side. Again, this exit site is medial to the top of the pectus ridge (see Fig. 11-3)  A 30-degree scope facilitates visualization during the substernal dissection, and care is taken to keep the point of the dissector underneath the sternum at all times to push the heart out of the way of the dissection plane. During the dissection, the ECG monitor should be turned to maximum volume to listen for any ectopy or arrhythmias.  When the introducer is fully in place, the sternum is elevated by lifting the introducer on each side, thus correcting the pectus excavatum. The sternum is brought out of its depressed position by lifting the introducer several times. This is facilitated by pressing down on the lower chest wall while lifting the introducer. This maneuver flexes the cartilage, and in our experience it helps to minimize intercostal muscle stripping when the bar is placed and facilitates correction of sternal torsion, if it is present.  Once the sternal depression has been corrected, umbilical tape is attached to the introducer, and the introducer is slowly withdrawn from the chest cavity with the umbilical tape attached.  The pectus bar that was previously bent into a convex shape is then attached to the umbilical tape. It is then slowly pulled through the right subcutaneous tunnel under thoracoscopic visualization and guided through the substernal tunnel with its convexity facing posteriorly until it merges on the contralateral side.  The pectus bar is positioned inside the chest with its convexity facing posteriorly and an equal amount of bar protruding on each side. Using the specially designed bar flippers, the bar is rotated 180 degrees, giving instant correction to the pectus deformity. The sides of the bar should be resting comfortably against the musculature and should not be too tight or too loose. If the bar does not fit snugly on each side because of pressure on the middle, the bar can be reflipped and molded as necessary while still in place in the chest.

Stabilization of the Bar



The bar is stabilized by attaching a stabilizer to the left end of the bar and wiring the bar and stabilizer together with no. 3 surgical steel wire. The stabilizer and bar are also secured by placing numerous interrupted absorbable sutures through the holes in the bar and adjacent fascia. An additional stabilizing technique uses a laparoscopic “auto-suture” needle to place multiple “0” absorbable sutures around the bar and underlying ribs with thoracoscopic guidance (Fig. 11-4).

Chapter 11  •  Surgical Treatment of Chest Wall Deformities: Nuss Procedure   101

X

X

Figure 11-3 

X

PDS sutures

Figure 11-4 

X

102   Section III  •  Thoracic

Closure



Meticulous hemostasis is obtained, and then the pockets are closed in three layers with absorbable suture.  The chest is evacuated of CO2 by placing the patient in Trendelenburg position with the left side down and the anesthesiologist giving positive ventilatory pressure. By cutting the tubing of the trocar and placing it in water below the level of the patient, one can confirm nearcomplete evacuation when the bubbles stop. Rarely is a chest tube required.

Step 4: Postoperative Care



Perioperative antibiotics are continued until discharge on the 4th or 5th day. An epidural catheter is used for 3 or 4 days and then transitioned to intravenous and oral pain medications. Patients are discharged receiving a combination of narcotic, anti-inflammatory, and muscle-relaxant medications.  For the first 6 weeks, patients are prohibited from playing sports but are encouraged to do deep-breathing exercises and to ambulate. At 6 weeks after the repair, they are encouraged to resume normal activities, and at 3 months they may resume competitive sports. Heavy contact sports such as football, boxing, and ice hockey are prohibited for a minimum of 6 months. Our preference is that they avoid heavy contact sports while the bar(s) is in place, but other aerobic sports are encouraged.

Chapter 11  •  Surgical Treatment of Chest Wall Deformities: Nuss Procedure   103

Step 5: Pearls and Pitfalls



Proper bending of the bar is crucial so that there is a gentle curve to the bar. The entry and exit sites must be medial to the greatest apex of the chest; otherwise, the bar is unstable and can flip and strip the intercostal muscles.  In older patients or those with a stiff chest or major torsion, two bars are helpful to distribute the pressure and get a more stable repair. One bar has to be under the bony sternum, and although it is desirable, the second bar need not be.  Any patient with a history of potential allergies should be tested for metal allergy and, if positive, should have titanium bars made. In any postoperative patient with atypical symptoms, including skin reactions, pleural or pericardial fluid, or culture-negative wound collections, one should consider a metal allergy as the cause.  The Lorenz introducer comes in three sizes. For patients with a deep pectus or severe torsion, the largest introducer facilitates a safer dissection because of the greater acuity of the angle of the introducer. 

Bibliography

Croitoru DP, Kelly RE Jr, Nuss D, et al. Experience and modifications update for the minimally invasive Nuss technique for pectus excavatum repair in 303 patients. J Pediatr Surg 2002;37:437-445. Kelly RE, Shamberger RC, Mellins RB, et al. Prospective multicenter study of surgical correction of pectus excavatum: design, perioperative complications, pain, and baseline pulmonary function facilitated by Internet-based data collection. J Am Coll Surg 2007;205(2): 205-216. Lawson ML, Cash TF, Akers RA, et al. A pilot study of the impact of surgical repair on disease—specific quality of life among patients with pectus excavatum. J Pediatr Surg 2003;38:916-918. Nuss D, Kuhn MA, Obermeyer RJ. Our approach: MIS repair of pectus excavatum. Contemp Surg 2007;63:444-451.

CHAPTER

12 

Surgical Treatment of Chest Wall Deformities: Open Repair Robert C. Shamberger

Step 1: Surgical Anatomy



The severity and configuration of the pectus excavatum deformity vary widely. The depression involves posterior displacement of the sternum as well as the costal cartilages. The depression may also be linked with a protrusion of one side creating a “mixed” deformity.  In teenagers, the posterior curvature of the ribs often involves part of the bony ribs as well as the cartilaginous portion.  Another key consideration for these patients is the degree of asymmetry of the excavatum configuration. In some patients there is a marked asymmetry, often with a much shorter anteroposterior (AP) diameter of the chest on the right than on the left side. The sternum in these patients is often rotated with the right side down, and the ribs may actually take off at almost a right angle from the sternum. Surgical repair should address all of these anatomic components.  The severity of the depression is often assessed using the Haller index, in which the transverse diameter of the chest (Fig. 12-1 A–B) is divided by the shortest distance between the sternum and the spine (Fig. 12-1 C–D). Indices greater than 3.25 are generally considered significant and appropriate for repair.

Step 2: Preoperative Considerations



Many studies of the cardiopulmonary effects of the pectus excavatum have been completed in the last two decades. A “restrictive” pulmonary defect is often present in patients with an excavatum configuration.  On physiologic testing, decreased maximal energy expenditure is seen in many with the most severe deformity, and during exercise the tidal volume is often decreased from normal. Patients often find the appearance of the more severe depressions quite distressing and frequently push for repair. 104

Chapter 12  •  Surgical Treatment of Chest Wall Deformities: Open Repair   105

C

A

B D

Figure 12-1 

106   Section III  •  Thoracic

Step 3: Operative Steps

Anesthetic



General endotracheal anesthesia is required, and many institutions currently use an epidural catheter as well for intraoperative and postoperative pain control.

Positioning



The patients’ arms are best placed at their side to maintain symmetry and to allow the surgeon access to the entire side of the patient during the surgical repair.  Most repairs include insertion of a metal strut, so perioperative antibiotics are used, and I prefer a povidone-iodine impregnated drape or other skin barrier to minimize exposure of the skin.

Incision



A transverse incision is used unless a prior sternotomy scar is present (Fig. 12-2). In females, the incision is placed in the inframammary crease. I mark this with the patient sitting in the preoperative area to ensure that it is correctly identified. The incision must not extend onto the breast, which can produce “tethering” of the scar between the breasts.  Skin flaps are then developed superiorly to the level of the highest cartilage to be resected and inferiorly to allow exposure of the triangular insertion of the rectus muscle onto the base of the sternum.  The pectoral muscles are mobilized off the sternum beginning at their insertion on the sternum, and care is taken to avoid injury of the periosteum, which in teenagers can result in significant bleeding (see Fig. 12-2). The muscle flaps are elevated to the lateral extent of the deformity. Ellis (1997) has described performing this flap elevation mobilizing the pectoral muscles with the skin flap rather than as separate layers.  This step is facilitated by first developing the plane directly anterior to one of the costal cartilages; electrocautery is used to divide the insertion of the pectoral muscles onto the sternum and then inserting an empty knife handle in the more lateral areolar plane just anterior to the costal cartilage, followed by a right angle retractor. I then repeat the process to the space in front of the costal cartilage either superior or inferior to this site and then retract the muscle flap anteriorly with two right-angle retractors. With electrocautery, one can then divide the salmon-colored muscle between the two retractors off from its insertion on the intercostal muscles, developing a plane laterally. One should avoid injury to the silvery fascia overlying the intercostal muscles to avoid hemorrhage and entry into the pleural cavity. 

Chapter 12  •  Surgical Treatment of Chest Wall Deformities: Open Repair   107

Elevation of pectoral flaps

3 4

Skin incision

5

A Figure 12-2 

B

108   Section III  •  Thoracic



Once the pectoral muscle flaps are mobilized, resection of the deformed costal cartilages is performed. This is achieved by placing a transverse incision directly over the midportion of the costal cartilages passing just through the perichondrium (Fig. 12-3).  Perichondrial elevation is achieved using a perichondrial elevator defining the plane between the costal cartilage and the perichondrium. This is facilitated by incising the perichondrium with electrocautery at its anterior junction with the sternum, which allows the perichondrial sheath then to be reflected either superiorly or inferiorly to enhance exposure of the perichondrium on the posterior aspect of the rib.  In children in their early teens, the growth plate for the rib at the costochondral junction is preserved by leaving a segment of the cartilage attached to the rib to allow continued growth (Fig. 12-4).  In older teenagers who have completed most or all of their growth, the most anterior portion of the osseous ribs is often involved in the posterior depression, at least on the right side. In this situation, I extend the excision of the rib out onto the osseous portion and divide the rib with an angled bone cutter at the point where the rib is at the apex of its height. Dissection of the perichondrial sheath is facilitated by remembering that ribs 2 and 3 are fairly flat with a narrow depth, ribs 4 and 5 are almost circular in their transverse configuration, and ribs 6 and 7 are narrow and deeper (see Fig. 12-4). Fonkalsrud and Mendoza (2006) described a method of repair in which only segments of the deformed costal cartilages are removed.  After the costal cartilages have been resected on each side, a transverse osteotomy is created at the superior aspect of the deformity on the sternum. I perform this using a Hall air drill to perforate the anterior plate of the sternum with two osteotomies approximately 3 to 5 mm apart or wider for larger teenagers (Fig. 12-5).  The underlying cancellous bone is then mobilized with a perichondrial elevator or osteotome so that the segment of bone can then be elevated anteriorly.  The base of the sternum is then elevated to fracture the posterior plate of the sternum. This maximizes the flexibility of the sternum and the ability to obtain a complete correction.  The lowest portion of the sternal depression is then identified. This is generally the point of insertion of the 4th or 5th ribs. A tunnel is created at this site posterior to the sternum to allow passage of the retrosternal strut. At this time, the sternum is elevated anteriorly with a large towel clip to increase the distance between the posterior aspect of the sternum and the pericardium.

Chapter 12  •  Surgical Treatment of Chest Wall Deformities: Open Repair   109 Bed of third cartilage 1 Excise costal cartilage leaving growth plate and a segment of the costal cartilage

Manubrium Retracted pectoralis major

2

2

Gladiolus 3

3

4 4 Perichondrial incision 5

Divide cartilage junction over perichondrial elevator Xiphoid

6

7

Cross sectional area of the costal cartilages

Incision at junction with sternum

Figure 12-3  Ribs 2 and 3

Figure 12-4 

1

1

2

2 Closure 3

3

4

4

5

5

6

6

7

7 30-35° Before

Figure 12-5 

After

Ribs 4 and 5

Ribs 6 and 7

110   Section III  •  Thoracic



Using electrocautery, the perichondrial sheath at the selected 4th or 5th space is then divided from its junction with the sternum. A Schnidt clamp is used to dissect bluntly between the sternum and the pericardium until the retrosternal space is traversed. On the opposite side of the sternum, the perichondrial sheath is again divided from the sternum to allow the Schnidt to come through that opening. The sternum is then elevated, placing the Schnidt on the anterior surface of the bony ribs on each side.  The retrosternal strut is then configured into a “gull-wing” configuration so that the sternum will sit in the posterior depression of the strut as shown in Fig. 12-6. The strut is passed behind the sternum in a concave up position and with the strut flat relative to the sternum. After it is in position on each side on top of the ribs, it is then grasped with Kelly clamps and rotated 180 degrees. This should achieve an optimal correction for the sternum. If additional anterior elevation is required, the strut can be removed and repassed after altering its configuration or it can be bent further using handheld plate benders.  To prevent lateral motion, the strut is secured with a heavy absorbable suture traversing a portion of the periosteum of the underlying rib and coming through one of the holes on the end of the strut. I secure the strut at both ends (see Fig. 12-6). Care is taken at this point to ensure that the ends of the strut are flush with the chest wall and not protruding into the skin, which will cause discomfort.

Closure



Once hemostasis is confirmed, the wound is then closed, bringing the pectoral muscle flaps over the strut and sternum, joining them in the midline attached to the underlying periosteum of the sternum. At the inferior end of the sternum, it is important to join the muscle flaps to the rectus muscle fascia to achieve a water-tight closure.  I generally use one Hemovac drain deep to the pectoral muscle flaps to avoid significant fluid collection. Skin flaps are then closed in three layers with absorbable sutures. Excellent correction of the deformity is generally achieved using this method.

Step 4: Postoperative Care



Perioperative antibiotics are limited to two postoperative doses.  With successful epidural anesthesia, patients are able to ventilate well, and postoperative pulmonary morbidity should be limited.  Patients are encouraged to ambulate the following day.  Limited pneumothoraces are generally well tolerated and rarely require aspiration or placement of thoracostomy tubes.

Chapter 12  •  Surgical Treatment of Chest Wall Deformities: Open Repair   111

Costal cartilage Sternum

Part 1 Retrosternal strut

Perichondrial sheath

Part 2

Part 3

Figure 12-6 

112   Section III  •  Thoracic

Step 5: Pearls and Pitfalls



When the subcutaneous flaps are mobilized, the pectoral muscle fascia should be preserved intact with the muscle to maintain the strength and integrity of the muscle flap for closure. This is important because the flap will often be under some tension when it is stretched over the anteriorly displaced sternum.  When sharply incising the perichondrium of the costal cartilages, stay in the middle of the cartilage. It is much easier to start the plane of dissection on the flat surface of the cartilage than on the side. Use a knife to make the perichondrium incision because it allows easier dissection of the perichondrium from the cartilage than if electrocautery is used for the incision.  When dividing the costal cartilages and the perichondrial sheath to create the retrosternal tunnel, stay directly adjacent to the sternum to avoid injury to the internal mammary vessels, which are 1 to 1.5 cm lateral to the sternum.

Bibliography

Aronson DC, Bosgraaf RP, Merz EM, et al. Lung function after the minimal invasive pectus excavatum repair (Nuss procedure). World J Surg 2007;31:1518-1522. Baronofsky ID. Technique for the correction of pectus excavatum. Surgery 1957;42:884-890. Beiser GD, Epstein SE, Stampfer M, et al. Impairment of cardiac function in patients with pectus excavatum, with improvement after operative correction. N Engl J Med 1972;287:267-272. Cahill JL, Lees GM, Robertson HT. A summary of preoperative and postoperative cardiorespiratory performance in patients undergoing pectus excavatum and carinatum repair. J Pediatr Surg 1984;19:430-433. Ellis DG. Experience with a variation of the transverse incision in chest wall deformity correction. J Pediatr Surg 1997;32:728-729. Fonkalsrud EW, Mendoza J. Open repair of pectus excavatum and carinatum deformities with minimal cartilage resection. Am J Surg 2006;191:779-784. Haller JA Jr, Kramer SS, Lietman SA. Use of CT scans in selection of patients for pectus excavatum surgery: a preliminary report. J Pediatr Surg 1987;22:904-906. Kaguraoka H, Ohnuki T, Itaoka T, et al. Degree of severity of pectus excavatum and pulmonary function in preoperative and postoperative periods. J Thorac Cardiovasc Surg 1992;104:1483-1488. Malek MH, Berger DE, Housh TJ, et al. Cardiovascular function following surgical repair of pectus excavatum: a meta-analysis. Chest 2006;130:506-516. Malek MH, Fonkalsrud FW, Cooper CB. Ventilatory and cardiovascular responses to exercise in patients with pectus excavatum. Chest 2003;124:870-882. Shamberger RC, Welch KJ. Surgical repair of pectus excavatum. J Pediatr Surg 1988;23:615-622. Welch KJ. Satisfactory surgical correction of pectus excavatum deformity in childhood: a limited opportunity. J Thorac Surg 1958;36:697-713.

13 

C H A P T EArteriosus   R Chapter 13  •  Patent Ductus 113

Patent Ductus Arteriosus Peter B. Manning

Step 1: Surgical Anatomy



Because the dissection performed is typically limited, it is essential to identify key aspects of the juxtaductal anatomy before proceeding with ductal closure.  The ductus arteriosus may be larger than the distal aortic arch, particularly in premature infants, and may be mistaken for the arch itself. Because the aortic arch follows a course in the mediastinum from right to left, the distal arch and isthmus typically lie in a slightly deeper plane, just superior to the ductus arteriosus. As a result of these factors, the ductus may be mistaken for the aortic arch itself, leading to unnecessary additional dissection and injury to other structures, such as the left pulmonary artery, the recurrent laryngeal nerve, or the aorta itself.  With the typical dissection, the left pulmonary artery is not completely exposed, although its location should be appreciated at the superior aspect of the hilum of the lung.  The phrenic nerve should be well anterior of the area of dissection, lying on the thymus in the superior mediastinum and coursing anterior to the pulmonary hilum.  The vagus nerve is typically easily identified just anterior to the descending aorta. The recurrent laryngeal nerve leaves the vagus to loop around the inferior margin of the ductus arteriosus. Its relationship to the ductus arteriosus is the most reliable method of confirming the identity of the ductus, although its location results in its injury being the most common complication of juxtaductal operations.

Step 2: Preoperative Considerations



Indications for ductal closure generally fall into two main categories dictated by the size of the ductus and the age of the child.  Congestive heart failure in the premature infant population is the most common indication for surgical ductal closure. Whereas echocardiography may demonstrate the presence and approximate size of the patent ductus, the decision to intervene should be based on clinical symptoms of congestive heart failure (respiratory compromise caused by excessive pulmonary blood flow, failure to maintain consistent weight gain, compromise of systemic perfusion manifested as renal insufficiency, or necrotizing enterocolitis). Echocardiographic evidence of significant pulmonary volume overload, such as left atrial and left ventricular 113

114   Section III  •  Thoracic

enlargement, and a chest radiograph showing cardiac enlargement and prominent pulmonary vasculature support the clinical findings.  In older children a medium-sized to large patent ductus may lead to chronic congestive heart failure symptoms, the risk of developing pulmonary hypertension, and ultimately Eisenmenger physiology. In the current era, the incidence of bacterial endarteritis is extremely low, although bacterial endarteritis was responsible for a large portion of the mortality from patent ductus arteriosus (PDA) in the pre-antibiotic era.  Choices for management  Because ductal patency is related to prostaglandin metabolism, medical closure is possible using the prostaglandin synthetase inhibitors indomethacin or other nonsteroidal antiinflammatory drugs (NSAIDs). Only premature infants in the first week or two of life predictably respond to NSAID therapy, although the use of NSAIDs may be contraindicated because of renal insufficiency or concern about bleeding complications.  Catheter-based therapy using Gianturco coils or the Amplatzer ductal occluder device has become the standard therapy, with few exceptions, for PDA in children beyond the newborn period.  Video-assisted clip occlusion of the patent ductus has been described in premature infants and in older children and is the preferred strategy in some centers. With this technique, the learning curve is often greater and the incidence of complications or incomplete ductal closure is often higher.  Open surgical closure may be performed in patients of any age and can include clip ligation, simple or multiple ligation, or division with oversewing. Choice of closure method depends on surgeon preference, the diameter and length of the ductus, and the patient’s age and size.

Step 3: Operative Steps



Because the premature infant is the patient most commonly requiring surgical closure, the discussion will focus primarily on this population.  Transfer of the patient from bed to bed or transport from site to site within a hospital entails as much risk to the fragile premature neonate as does the operation itself. For this reason, many centers perform PDA closure in the neonatal intensive care unit, even in units unattached to a pediatric hospital. When it is preferred to perform the procedure in the operating room suite, the infant should be maintained in his or her own isolette, preferably one with an overhead warmer. The infant’s head is positioned toward the open end to allow access by the anesthesiologist.  Avoiding hypothermia is essential while operating on premature infants and may be very challenging in the operating room environment. Likewise, ventilation may be difficult because of retraction on the left lung and the underlying lung disease. Proper endotracheal tube position and adequacy of ventilation must be confirmed after positioning in the right lateral decubitus position and before draping for the procedure. Limited, expedient dissection is important in preventing major physiologic compromise in these patients.  A posterolateral left 3rd or 4th interspace thoracotomy gives the best exposure to the juxtaductal region (Fig. 13-1). A muscle-sparing thoracotomy typically provides adequate exposure if it is preferred by the surgeon.  Retracting the left lung anteriorly and inferiorly exposes the posterior mediastinum. Two small malleable retractors (one on the upper lobe above the pulmonary hilum and a smaller one on the lower lobe posteriorly below the pulmonary hilum) can be clamped to the chest retractor and will afford excellent exposure (Fig. 13-2).

Chapter 13  •  Patent Ductus Arteriosus   115

Figure 13-1 

Phrenic nerve Vagus nerve

Descending aorta

Figure 13-2 

116   Section III  •  Thoracic



Dissection is limited to the region of the ductus at its aortic end and is most easily begun by opening the pleura for 1 to 1.5 cm overlying the descending aorta, staying at least a few millimeters posterior to the vagus nerve. Gentle blunt and electrocautery dissection along the anterior aspect of the aorta should lead first to the ductus itself (Fig. 13-3). Limited dissection superior to the ductus should be carried out to confirm the identity and location of the distal aortic arch, which lies in a slightly deeper plane and is often smaller than the ductus itself, which may lead to confusion.  The superior and inferior aspects of the ductus itself are dissected at the aortic end of the ductus, which will result in the recurrent laryngeal nerve being pushed out of harm’s way toward the pulmonary end of the ductus. The ductus need not be circumferentially dissected if clip occlusion is the planned method of closure, but enough dissection is needed to be able to see well the tip of the clip close beyond the deep edge of the ductus to avoid ductal injury during clip application. Retraction of the proximal descending aorta anteriorly will allow posterior dissection of the back wall of the ductus if this is needed for methods that use circumferential dissection.  Management of the PDA itself  Division of the ductus between vascular clamps with oversewing of the ends is the only method that can confidently be predicted to result in a 100% closure rate, but this technique is appropriate only for larger children with relatively longer ductal lengths.  Closure by Ligaclip may be used with open or thoracoscopic techniques. It is the simplest and most appropriate method in the premature infant. It is important to recognize that clips close first at their tips and then in the middle. It is essential to be able to dissect enough of the ductus to see that the tip does not close on the ductal wall itself and perforate it; rather, it must come together beyond the ductal wall. This may result in the need to use a larger clip size than expected from the initial exposure. Positioning the legs of the clip applier along the superior and inferior aspect of the ductus and then pushing the applier against the descending aorta while lifting the latter structure posteriorly and upward will allow good visualization of clip closure and also will minimize the risk of injury to the recurrent laryngeal nerve, which will tend to stay near the pulmonary end of the ductus.  Simple ligation of a large ductus in a premature infant may be more difficult because of the need to perform circumferential dissection and the inability to feel when the suture is tied down securely without tearing through the fragile ductal wall, potentially resulting in a higher rate of incomplete closure.  In older children, a multiple ligation technique may be performed using simple ligatures along the length of the ductus or ligatures secured by taking fine adventitial bites in the ductal wall to prevent slipping toward the center (Fig. 13-4).  In the absence of injury to the lung during exposure, a chest tube typically does not need to be left following the procedure. In older children, this improves postoperative comfort and typically allows for more expedient discharge from the hospital.

Chapter 13  •  Patent Ductus Arteriosus   117

Phrenic nerve Vagus nerve

Descending aorta

Figure 13-3 

Vagus nerve Recurrent laryngeal nerve Descending aorta

Figure 13-4 

118   Section III  •  Thoracic

Step 4: Postoperative Care



Older children should be able to be discharged from the hospital within 24 to 48 hours following ductal closure. Resumption of a normal diet and adequate pain control are the typical driving factors.  Ligation of a hemodynamically important ductus in a premature infant typically results in a rise in diastolic and mean arterial pressures noted immediately. Studies have demonstrated improvements in pulmonary mechanics soon after ductal occlusion and a variable response in left ventricular performance with an acute increase in ventricular afterload. The need for initiation or escalation of inotropic support is observed in up to a third of these patients. Chronic lung disease is frequent in this population even after ductal ligation, leading some to suggest using this therapy earlier given its demonstrated safety even in very-low-birthweight neonates.

Step 5: Pearls and Pitfalls



It is critical to understand the anatomy of the juxtaductal aorta and the pulmonary hilum and the distortion that may be caused with a large patent ductus. Dissection should be minimized for expedient conduct of the procedure, but definitive identification of the pertinent anatomy cannot be compromised. Confusion has led to such complications as ductal injury, left pulmonary artery ligation, or aortic ligation.  Recurrent laryngeal nerve injury is best prevented by limiting dissection to the aortic end of the ductus and by avoiding handling the anterior flap of pleura where the vagus nerve could be grasped above the level of recurrent laryngeal nerve exit.  The risk of postoperative chylothorax may be lessened by limiting dissection to the peri­ adventitial plane of the vascular structures and the use of low-energy cautery during much of the dissection.

Chapter 13  •  Patent Ductus Arteriosus   119

Bibliography

Gould DS, Montenegro LM, Gaynor JW, et al. A comparison of on-site and off-site patent ductus arteriosus ligation in premature infants. Pediatrics 2003;112(6 Pt 1):1298-1301. Hillman ND, Mavroudis C, Backer CL. Patent ductus arteriosus. In Mavroudis C, Backer CL, eds. Pediatric cardiac surgery, 3rd ed. Philadelphia: Mosby, 2003; pp. 223–233. Jacobs JP, Giroud JM, Quintessenza JA, et al. The modern approach to patent ductus arteriosus treatment: complementary roles of videoassisted thoracoscopic surgery and interventional cardiology coil occlusion. Ann Thorac Surg 2003;76(5):1421-1427. Moin F, Kennedy KA, Moya FR. Risk factors predicting vasopressor use after patent ductus arteriosus ligation. Am J Perinatol 2003;20(6):313-320. Rao PS. Percutaneous closure of patent ductus arteriosus: state of the art. J Invasive Cardiol 2007;19(7):299-330. Raval MV, Laughon MM, Bose CL, Phillips JD. Patent ductus arteriosus ligation in premature infants: who really benefits, and at what cost? J Pediatr Surg 2007;42(1):69-75. Schneider DJ, Moore JW. Patent ductus arteriosus. Circulation 2006;114(17):1873-1882. Zbar RI, Chen AH, Behrendt DM, et al. Incidence of vocal fold paralysis in infants undergoing ligation of patent ductus arteriosus. Ann Thorac Surg 1996;61(3):814-816.

CHAPTER

14 

Congenital Lung Anomalies Elizabeth A. Beierle and Mike K. Chen

Step 1: Surgical Anatomy

r

Congenital malformations of the lungs in neonates and infants are uncommon. The three conditions most commonly encountered are discussed herein: congenital lobar emphysema, cystic adenomatoid malformations (CCAMs), and pulmonary sequestrations (both extralobar and intralobar). r Many of these lesions are asymptomatic and are discovered on routine prenatal ultrasound or postnatal imaging studies. Occasionally they cause acute respiratory distress in the neonate and require immediate surgical intervention. r Eventually, many CCAMs and intralobar pulmonary sequestrations become symptomatic with recurrent pneumonia, pulmonary abscess, pneumothorax, or hemoptysis, leading to the need for surgical intervention.

Step 2: Preoperative Considerations

r

120

Congenital lobar emphysema is the overdistention of one or more lobes of the lung. It usually results from an absence of bronchial cartilage but may occur from mucus plugging and progressive air trapping in ventilated patients. Associated anomalies of the heart and great vessels are common.  Infants may present with respiratory distress, diminished breath sounds, hyperresonance over the involved thorax, and evidence of mediastinal shift.  Chest radiographs are usually diagnostic, with hyperlucency of the involved lobe, collapse or atelectasis of the other lobes, and shift of the heart toward the opposite thorax. Fig. 14-1 is a chest radiograph demonstrating overexpanded congenital lobar emphysema of the right middle lobe and significant compression of the right upper and lower lobes.  If the infant is in severe respiratory distress, the surgeon should urgently proceed to operation; however, asymptomatic congenital lobar emphysema may regress, and observation of those infants is warranted.

Chapter 14  •  Congenital Lung Anomalies   121

Figure 14-1 

122   Section III  •  Thoracic

r

CCAMs derive from the abnormal branching of the immature bronchioles during lung development. They are commonly seen in the lower lobes and derive their blood supply from the pulmonary vessels; most have a communication with the tracheobronchial tree.  CCAMs are commonly diagnosed on prenatal ultrasound. Large lesions may result in fetal nonimmune hydrops caused by compression of the mediastinum and vena cava obstruction. Spontaneous regression may occur in 40% to 80% of prenatally detected lesions.  The most common presenting symptom is respiratory distress. Initial imaging studies include chest radiographs.  Lesions seen on ultrasound might not be detected by plain films, and computed tomography (CT) or magnetic resonance imaging (MRI) may be helpful. Common radiologic findings include large cystic spaces in the hemithorax with mediastinal shift, or multiple cysts may be seen.  Figures 14-2 and 14-3 are of a neonate with a congenital cystic adenomatoid malformation of the left lower lobe. The left lung is overexpanded with multiple cystic areas present. There is significant shift of the mediastinum to the right hemithorax. The stomach bubble and nasogastric tube are situated below the diaphragm, aiding in the differential diagnosis of congenital diaphragmatic hernia.  Symptomatic neonates should undergo urgent surgical excision. Infants who remain asymptomatic should undergo elective resection at around 6 to 12 months of age to avoid the risks of neonatal anesthesia. All CCAMs that do not spontaneously involute in the first few months of life should be removed because of the risk of recurrent pneumonias and the potential for malignant transformation. r Pulmonary sequestrations are cystic masses of nonfunctioning lung tissue with no connection to the tracheobronchial tree. They typically derive their blood supply from a systemic artery originating from the thoracic or abdominal aorta and may have systemic venous connection to the superior vena cava or azygos or hemiazygos veins. They exist in two forms: extralobar and intralobar. An extralobar sequestration is covered by separate visceral pleura, is completely discrete from the normal lung, and is most commonly seen in the left lower chest. Associated anomalies are common and include chest wall deformities, congential heart defects, and congenital diaphragmatic hernia.  Neonates may be asymptomatic or have respiratory distress, pneumonia, feeding difficulties, or congestive heart failure resulting from arteriovenous shunting. Intralobar sequestrations are found within the surrounding normal lung tissue, most commonly in the left lower lobe. These also have a systemic arterial supply but commonly drain through the pulmonary veins. The most common presenting symptom in a neonate is respiratory distress.  Initial imaging studies include chest radiographs that commonly show a left-sided lower lobe mass or consolidation. Additional imaging, including CT and MRI, may be helpful for definitive diagnosis and to outline the large systemic artery arising from the subdiaphragmatic aorta (Fig. 14-4, arrow).  Neonates with respiratory distress should undergo urgent surgical resection. Older infants and children may have recurrent respiratory infections and should have an elective resection after the infection has been treated. Asymptomatic neonates whose lesions appear to have regressed on ultrasound should undergo follow-up imaging studies. Those neonates with asymptomatic pulmonary sequestration should undergo elective resection, as suggested for CCAM.

Chapter 14  •  Congenital Lung Anomalies   123

Figure 14-2 

Figure 14-3 

Figure 14-4 

124   Section III  •  Thoracic

Step 3: Operative Steps

General

r

After the induction of general anesthesia, placement of an endotracheal tube, and obtaining adequate intravenous access, the infant is positioned for a posterolateral thoracotomy. r Single lung ventilation may be accomplished by selective mainstem intubation, a bronchial blocker, or double-lumen endotracheal tube, but it is not absolutely required. Occasionally single lung ventilation is not tolerated well in infants. r Posterolateral thoracotomy is the most frequently used approach and is useful for almost all pulmonary lesions requiring resection in childhood. The child is placed with the affected side up, down leg flexed at 90 degrees, a towel placed between the legs, and an axillary roll positioned to prevent stretching and vascular compromise of the dependent arm and shoulder. The ipsilateral arm is elevated toward the head and supported on blankets or pillows, taking care to avoid stretching the brachial plexus. Antiseptic preparation of the skin and preoperative antibiotic prophylaxis is used. A gently curvilinear incision is made starting at the anterior axillary line and extended posteriorly (Fig. 14-5). The fifth intercostal space is used for lower lobectomy and extrapulmonary sequestrations, and the fourth is used for upper lobectomy. To avoid the late effects of scoliosis and chest wall deformities, care is taken in performing a muscle-sparing thoracotomy and avoiding the division of the trapezius, rhomboid, and paraspinal muscles. The chest is opened along the appropriate intercostal space, and a Finochietto rib spreader is placed for visualization. Once the lesion has been identified, the procedure is continued with the correct lobectomy. r The basic principles of pulmonary lobectomy involve dissection and division of the arterial supply, followed by the venous drainage, and finally the bronchus. A detailed explanation of all pulmonary resections is beyond the scope of this text; therefore we refer you to the selected references and will describe only a left lower lobectomy in detail because it is the most commonly affected lobe.

Left Lower Lobectomy

r

The major fissure of the lung is opened by retracting the upper lobe superiorly. The pulmonary artery is located at the base of the fissure, lying in the same plane as the fissure (Fig. 14-6, A). Once the artery is located, the perivascular space is entered and the superior segmental artery and its branches and the basilar artery and its branches are dissected free. Before completion of the arterial dissection, the major fissure may need to be completely divided, a measure that is usually amenable to a stapling device. The branches of the artery are doubly ligated or suture ligated and divided.

Chapter 14  •  Congenital Lung Anomalies   125

Figure 14-5 

126   Section III  •  Thoracic

r

Next the lung is reflected anteriorly to expose the posterior hilum and the inferior pulmonary ligament. The ligament is divided, and the vein is exposed. In the case of a pulmonary sequestration, the systemic artery usually courses through the inferior pulmonary ligament, and this artery must be identified, doubly ligated, or suture ligated and divided before division of the inferior pulmonary ligament. The vein and its branches are doubly ligated or suture ligated and divided (Fig. 14-6, B). r The upper lobe is then retracted anteriorly, and the lower lobe bronchus is exposed. The upper lobe pulmonary artery is dissected free from the underlying lower lobe bronchus, and the bronchus is dissected free. The origin of the upper lobe bronchus must be identified and preserved. The lower lobe bronchus is occluded before division, and the upper lobe is observed for adequate inflation. The lower lobe bronchus is then divided and closed in segments using monofilament sutures (Fig. 14-6, C) or using a stapling device. Once the bronchus is divided, the specimen may be removed from the chest. r The chest is then filled with warm saline, and gentle positive pressure is applied to identify any air leaks from the bronchial closure. A chest tube is placed into the chest through a separate stab incision in the anterior axillary line approximately two intercostal spaces caudal to the incision. The ribs are reapproximated with interrupted absorbable suture, the chest wall muscles with running absorbable suture. The chest tube is placed to 10 to 15 cm H2O suction, and a sterile dressing is applied to the wound.

Thoracoscopic Lobectomy

r

With advances in minimally invasive techniques and equipment, thoracoscopic pulmonary lobectomy is possible in almost any age or size of a child. Most of these procedures can be accomplished using 5-mm trocars and small, 3.5-mm, instruments. The long-term benefit of these procedures is the avoidance of late musculoskeletal complications that are seen with thoracotomy, but as of this date, definitive long-term data addressing this outcome are lacking.

Chapter 14  •  Congenital Lung Anomalies   127

Left pulmonary artery Aorta Sup. segmental branch Basilar branch

Left pulmonary artery

Upper lobe

Upper lobe

Lower lobe

A Left pulmonary artery Suture

Left pulmonary vein Left main bronchus

B

Left lower lobe bronchus Suture Left pulmonary vein

C Figure 14-6 

Lower lobe

Ligated/ divided sup. segmental branch

128   Section III  •  Thoracic



Single lung ventilation, as described previously herein, is achieved, but those infants and children who do not tolerate single lung ventilation are not candidates for this procedure. The patient is positioned laterally, and the surgeon and assistant stand on the same side, facing the patient’s abdomen. There is a single video monitor placed on the opposite side of the patient. A valved 5-mm port is placed in the midaxillary line about the fifth intercostal space. A controlled pneumothorax is created by insufflating 4 torr CO2 at 1 L/min, which allows excellent visualization of the chest without the need for manual lung retraction. Two to three additional ports are placed in a triangular fashion, and 3.5-mm instruments are used (Fig. 14-7). The techniques follow those of the open procedure, mainly division of the pulmonary fissure, dissection and ligation of the arteries, veins, and the bronchus. The pulmonary vessels and the systemic vessels of a pulmonary sequestration may be sealed and divided with the Ligasure. This instrument is useful for vessels 9 mm or smaller. It is also useful in division of the fissure. Larger vessels may require endoclips, suture ligation, or an Endo-GIA stapler. The bronchus may be handled with an Endo-GIA stapler, but the chest cavity of many infants is too small to accommodate this device. In those instances, the bronchus may be divided and sutured intracorporeally with interrupted absorbable sutures. The posterolateral port incision may be enlarged to remove the specimen, or a retrieval bag may be used. A 12 French chest tube may be placed through one of the trocar sites and placed to 10 to 15 mm H2O suction.

Step 4: Postoperative Care

r

The infant is allowed to feed ad libitum the following morning as long as there is no gastric distention. Antibiotics are continued until the chest tube is removed. The chest tube is maintained on 10 to 15 cm H2O suction until there is no air leak and output is minimal. At that time the tube is placed to H2O seal and a chest radiograph is obtained to ensure that no pneumothorax is present. If air does not accumulate in the chest cavity, the tube is removed and a follow-up chest radiograph is obtained to ensure that the lung remains expanded. These children are usually seen in follow-up in the clinic about 4 weeks after their procedure.

Step 5: Pearls and Pitfalls

r

Most of these children do extremely well, and major complications such as empyema and bronchopleural fistula are rare.

Chapter 14  •  Congenital Lung Anomalies   129

Figure 14-7 

130   Section III  •  Thoracic

Congenital Lobar Emphysema

r

The surgeon must be prepared for immediate thoracotomy in these children because occasionally the child will acutely decompensate after the induction of anesthesia. r Opening the chest will reduce the tension effects of the emphysematous lobe and allow adequate ventilation of the contralateral normal lung. In older infants and children, obstruction of the bronchus from a radiolucent foreign body leads to air trapping and a clinical picture that may mimic congenital lobar emphysema, and diagnostic bronchoscopy is helpful.

Congenital Cystic Adenomatoid Malformations

r

This entity, especially when it occurs on the left side, may be confused with congenital diaphragmatic hernia. Noting the location of the stomach bubble and nasogastric tube beneath the diaphragm aids in the diagnosis. If the diagnosis remains in question, an upper gastrointestinal study or CT scan looking for loops of bowel in the chest may also be helpful.

Pulmonary Sequestration

r

The most important aspect of surgical intervention for this entity is adequate and early control of the systemic arterial supply. A number of extralobar sequestrations (15%) will be found within or below the diaphragm and may be mistaken for a neuroblastoma. These lesions are amenable to laparoscopic removal, again with meticulous attention to control of the systemic arterial supply. About 10% of sequestrations may have connections to the gastrointestinal tract, and preoperative upper gastrointestinal series is helpful in defining this anatomy.

Bibliography

Albanese CT, Rothenberg SS. Experience with 144 consecutive pediatric thoracoscopic lobectomies. J Laparoendosc Adv Surg Tech 2007; 17:339-341. Duncombe GJ, Dickinson JE, Kikiros CS. Prenatal diagnosis and management of congenital cystic adenomatoid malformation of the lung. Am J Obstet Gynecol 2002;187:950-954. Hood M. Techniques in general thoracic surgery. Philadelphia: Saunders, 1985; pp. 101-133. Laberge JM, Puligandia P, Flageole H. Asymptomatic congenital lung lesions. Semin Pediatr Surg 2005;14:16-33. Mei-Zahav M, Konen O, Manson D, Langer JC. Is congenital lobar emphysema a surgical disease? J Pediatr Surg 2006;41:1058-1061, 2006. Rothenberg SS. Experience with thoracoscopic lobectomy in infants and children. J Pediatr Surg 2003;38:102-104.

SECTION

IV

Abdomen

CHAPTER

15 

Duodenal Obstruction Daniel von Allmen

Step 1: Surgical Anatomy

r

In congenital duodenal atresia, the obstruction is distal to the ampulla of Vater in 85% of patients. A complete obstruction may be a luminal web (most common), an atresia connected by a fibrous cord, or complete separation of proximal and distal segments (Fig. 15-1). r The ampulla is located close to the medial wall of the web, and care must be taken to avoid injuring it during repair. r A fenestrated duodenal web with incomplete obstruction may not be noted at birth but can be seen later with feeding intolerance or obstruction when solid foods are started. r Annular pancreas may accompany duodenal atresia. The annular portion of the pancreas can contain a significant pancreatic duct (Fig. 15-1, D). r A “windsock” deformity can suggest a more distal obstruction (Fig. 15-2). r Esophageal atresia may accompany duodenal atresia. r Preduodenal portal vein may cause obstruction of the duodenum. r Familial duodenal atresia occurs as part of Feingold syndrome.

Step 2: Preoperative Considerations

r

The differential diagnosis includes malrotation with volvulus, small bowel atresia, and Hirschsprung disease in patients with bilious vomiting. In patients with nonbilious emesis, the differential includes pyloric atresia and antral web. r Prenatal ultrasound and postnatal plain films show classic “double-bubble” configuration with a dilated first portion of the duodenum (Fig. 15-3). r About 30% of patients with duodenal atresia have trisomy 21. r About 20% of patients with duodenal atresia have cardiac anomalies.

132

Chapter 15  •  Duodenal Obstruction   133

A

B

D

C Figure 15-1 

Common duct

Web

Dilated duodenum

Figure 15-2 

Figure 15-3 

134   Section IV  •  Abdomen

Step 3: Operative Steps

r

Open and laparoscopic techniques are described, and both achieve the same anatomic result. The patient is positioned in the supine position for both approaches. r The objective of surgery in most cases is to bypass the obstruction rather than resect it, thus avoiding injury to the ampulla of Vater or annular pancreas. r Open surgery is done through a right-sided transverse supraumbilical incision. Trocar placement for the laparoscopic approach is shown in Fig. 15-4. r The hepatic flexure is mobilized, and the duodenum is Kocherized to provide exposure to the first, second, and third portion of the duodenum. r The level of obstruction is identified by transition from the dilated proximal segment to the collapsed unused distal bowel. If an annular pancreas is present, the level of obstruction is beneath the gland and bypass is performed from the bowel proximal and distal to the pancreatic tissue. r After placing traction sutures, a transverse duodenotomy is made on the anti-mesenteric portion of the dilated proximal duodenum and a longitudinal incision on the anti-mesenteric border of the distal duodenum (Fig. 15-5). r Prior to anastomosis the distal bowel is cannulated with an 8-10 French catheter and saline infused and followed to the ileocecal valve to rule out a distal obstruction. r A “diamond” anastomosis is created using absorbable sutures beginning with the back wall and progressing anteriorly (Fig. 15-6) r Laparoscopically, sutures or U-clips can be used to fashion the identical anastomosis. r Abdominal closure is achieved with absorbable sutures.

Chapter 15  •  Duodenal Obstruction   135

3 mm working port 3 mm working ports

5 mm camera port (45° laparoscope)

Figure 15-4 

Figure 15-5 

Figure 15-6 

136   Section IV  •  Abdomen

Step 4: Postoperative Care

r

Prophylactic antibiotics are discontinued within 24 hours. Patients are extubated in the operating room unless co-morbidities such as cardiac anomalies require continued ventilatory support. r Nasogastric decompression is continued until output decreases and feedings can begin. r Feedings may have to advance slowly because of poor emptying of the dilated stomach and proximal duodenum. r Long-term outcome is good unless cardiac or chromosomal anomalies impact survival. Complications requiring reoperation are uncommon. r

Step 5: Pearls and Pitfalls

r

The level of obstruction may be obscured by a “windsock” deformity that results in dilation of the duodenum distal to the obstruction. r In some cases the lateral portion of the web can be resected to relieve the obstruction without injury to the ampulla if it is clearly visualized. r If the ampulla is distal to the obstruction (15%) and close to the duodenotomy, care must be taken to avoid common duct ligation or injury when placing the posterior row of duodenoduodenostomy sutures. r A transanastomotic feeding tube may facilitate faster initiation of post-operative enteral feedings (Fig. 15-7). r Patients with significant co-morbidities may benefit from gastrostomy tube placement in anticipation of poor oral feeding. r Minimize bag-mask ventilation or nasal continuous positive airway pressure, which may disrupt the anastomosis in the early postoperative period. r A small transanastomotic feeding tube can be passed adjacent to the gastrostomy tube in small infants to create a functional gastrojejunostomy tube.

Bibliography

Escobar MA, Ladd AP, Grosfeld JL, et al. Duodenal atresia and stenosis: long-term follow-up over 30 years. J Pediatr Surg 2004;39:867871; discussion 867-871. Naik-Mathuria B, Olutoye OO. Foregut abnormalities. Surg Clin North Am 2006;86:261-284. Spilde TL, St Peter SD, Keckler SJ, et al. Open vs laparoscopic repair of congenital duodenal obstructions: a concurrent series. J Pediatr Surg 2008;43:1002-1005.

Chapter 15  •  Duodenal Obstruction   137

Figure 15-7 

CHAPTER

16 

Malrotation: Ladd Procedure Brad W. Warner

Step 1: Surgical Anatomy

r

The term malrotation describes several related abnormalities characterized by the failure of the intestine to assume its normal anatomic position. r The normal anatomic position of the intestine is achieved during the 10th week of gestation when the herniated intra-abdominal contents return to the abdominal cavity and begin a counterclockwise rotation about the superior mesenteric artery (Fig. 16-1). r The final result of this normal rotational process is a retroperitoneal duodenum with the ligament of Treitz in the left upper quadrant at or close to the level of the antrum of the stomach. The beginning of the small intestine (jejunum) is located to the left of midline in the left upper quadrant and the cecum fixed in the right lower quadrant. The mesentery of the normally fixed small intestine is broad based, extending from the left upper quadrant to the right lower quadrant of the abdomen and is at low risk for volvulus (Fig. 16-2). r The most common forms of malrotation are nonrotation and incomplete rotation, which predispose the patient to duodenal obstruction and volvulus (Fig. 16-3).

Step 2: Preoperative Considerations

r

The presentation of malrotation ranges from the acute onset of bilious emesis in the infant or child, feeding intolerance caused by duodenal obstruction, or an incidental finding on imaging studies. r Bilious emesis in the neonate is a surgical emergency until proven otherwise and mandates an upper gastrointestinal evaluation with a contrast study regardless of findings on plain films. The diagnosis of volvulus in this setting is made by demonstration of obstruction with contrast tapering to a bird’s beak. r The child diagnosed with volvulus should receive fluids and broad-spectrum antibiotics en route to the operating room for reduction of the volvulus and a Ladd procedure. r When the diagnosis of malrotation is made in the setting of feeding intolerance or incidentally, an elective Ladd procedure is appropriate. 138

Chapter 16  •  Malrotation: Ladd Procedure   139

Ligament of Treitz Superior mesenteric artery Prearterial Postarterial

180°

A

B

Cecum

90°

C Figure 16-1 

D

Ligament of Treitz Ascending colon

Descending colon

Cecum

A Figure 16-2 

Figure 16-3 

B

140   Section IV  •  Abdomen

Step 3: Operative Steps

r

In the case of the child with volvulus, adequate volume resuscitation should occur before the induction of general anesthesia. r Prophylactic antibiotics for skin flora are adequate in the setting of an elective Ladd procedure; a broad-spectrum antibiotic should be given to the patient with volvulus. r The infant or child is placed in the supine position, and a transverse right upper quadrant incision is made. The level of the incision is at the midway point between the subcostal margin and the umbilicus. r The bowel is eviscerated and, if a volvulus is encountered, the intestine is rotated counterclockwise and inspected for evidence of ischemia (Fig. 16-4). If the bowel is not obviously necrotic, it should be covered with warm moistened laparotomy pads and re-examined after 10 to 15 minutes. Grossly necrotic bowel should be resected. If the remaining intestine is questionable, a second-look laparotomy is scheduled 24 to 48 hours later. r A Ladd procedure is then performed by dividing the peritoneal attachments between the cecum and the retroperitoneum that overlies the duodenum, so called Ladd bands (see Fig. 16-4). r The duodenum is then mobilized, followed by widening of the narrow small bowel mesentery to reduce the risk of volvulus (see Fig. 16-4). r The mesoappendix is then taken using electrocautery in the infant or ties in the older child. The appendix itself is then inverted with a lacrimal probe and double-ligated with a 2-0 absorbable suture. r The small intestine is then placed in the abdomen to the right of the midline with the colon to the left of the midline. r The abdominal wall is then closed in layers.

Step 4: Postoperative Care

r

The infant or child with a volvulus and compromised bowel requires monitoring, nasogastric decompression, intravenous antibiotics, and further resuscitation in an intensive care setting. r In the patient with questionable or a large amount of ischemic bowel, a second-look laparotomy is typically undertaken on postoperative day 1 or 2, and decisions then must be made with regard to further resection, stoma formation or, unfortunately, in some situations redirection of care when the insult is deemed incompatible with life. r In the uncomplicated Ladd procedure, a proximal ileus is common after a Ladd procedure and the stomach is decompressed until nasogastric aspirates have decreased and become clear. At that time the child’s is allowed to begin feeding.

Chapter 16  •  Malrotation: Ladd Procedure   141

A

B

C Figure 16-4 

D

142   Section IV  •  Abdomen

Step 5: Pearls and Pitfalls

r

A normal or nonspecific bowel gas pattern should not delay further radiographic investigation in an infant or child with bilious emesis. A delay of even a few hours can be catastrophic. r Recurrent volvulus can occur despite completion of a Ladd procedure. Infants and children with bilious emesis and abdominal distention should be carefully evaluated for possible recurrent volvulus or bowel obstruction. Urgent reoperation may be necessary.

Bibliography

Aiken JJ, Oldham KT. In Ashcraft KW, Holcomb GW, Murphy JP, eds. Pediatric surgery, 4th ed. Philadelphia: Saunders Elsevier, 2005; pp. 435-447. Spitz L, Bax NM, van der Zee DC. Malrotation. In Spitz L, Arnold G, Coran AG, eds. Operative pediatric surgery, 6th ed. New York: Hodder Arnold, 2006; pp. 383-392.

17 

C H A P T E R Disease   143 Chapter 17  •  Meconium

Meconium Disease Mary L. Brandt

Meconium Ileus Step 1: Surgical Anatomy

r

Meconium ileus is an obturator (intraluminal) obstruction caused by thick, viscous meconium, most commonly in patients with cystic fibrosis. r Accurate prenatal diagnosis of meconium ileus is difficult.  The most common ultrasound findings are echodense bowel and bowel dilatation.  Fetal magnetic resonance imaging has been suggested as an additional tool for prenatal diagnosis of the underlying cause of echodense bowel because it can differentiate between meconium ileus, atresia, bowel duplication, and other pathologic conditions.  Absence of the gallbladder may also be associated with cystic fibrosis.  Maternal polyhydramnios may occur in some cases.

Step 2: Preoperative Considerations

r

The passage of meconium in healthy term infants usually occurs within the first 24 hours, but this is prolonged by prematurity. In one study of infants weighing less than 1250 g and less than 32 weeks’ gestation, the mean time to first stool was 44 hours, and the 75th percentile was 10 days. r Clinical presentation  The presentation varies depending on whether the obstruction is simple (uncomplicated) or complex (complicated).  Simple meconium ileus refers to an intraluminal obstruction of the bowel without complication, which is much more common than complicated meconium ileus.  Soon after birth, these infants have failure to stool, bilious emesis, and abdominal distention.  Complicated meconium ileus refers to meconium ileus with perforation, volvulus, or atresia.  Perforation or volvulus with necrosis can cause peritonitis and the associated systemic changes. 143

144   Section IV  •  Abdomen 

If a volvulus occurs antenatally, the bowel can absorb and at birth the patient will have a small bowel atresia.  If there is a patent processus vaginalis, meconium may be visible in the scrotum, which can manifest as calcifications on radiographs. r Provide intravenous fluid resuscitation, place an orogastric or nasogastric (NG) tube, and correct any electrolyte imbalance.

Step 3: Treatment

r

All infants who are stable enough should undergo a contrast enema. If the patient has complicated meconium ileus, knowing that the colon is patent before surgery is important.  The contrast enema may be therapeutic as well as diagnostic for simple meconium ileus.  A KUB (kidneys, ureters, and bladder) examination should be performed 12 hours after the contrast enema; if obstruction is still present, the contrast enema can be repeated. r The first use of a contrast enema to relieve the obstruction of meconium ileus was reported in 1969 by Noblett. He defined seven “requirements” that still hold true today:  Rule out other causes of obstruction with an initial diagnostic enema.  The patient must have uncomplicated meconium ileus.  The procedure must be performed under careful fluoroscopic control.  The patient should receive parenteral antibiotics before the procedure.  A pediatric surgeon should be present during the procedure.  The infant should be fully resuscitated.  All preparations should be in place in case emergency surgery is needed. 

Operative Considerations

r

Indications for surgery All neonates with complicated meconium ileus  Those with uncomplicated meconium ileus who have failed two or three therapeutic enemas r Goal is to evacuate the inspissated, thick meconium from the bowel. r The goal is accomplished in most cases by one of three simple measures: a needle enterostomy, an open enterostomy, or access through the appendix. This procedure can be tedious and can easily take an hour or longer.  A needle enterostomy involves multiple injections slanted through the wall of the bowel with a small-gauge needle to deliver saline, dilute Gastrografin, and/or 2% to 4% N-acetylcysteine. Once the meconium is liquefied by one or all of these substances, it can be manually passed into the colon and out the rectum.  Alternatively, a small enterotomy can be performed, usually just proximal to the junction of the dilated and nondilated bowel.  An appendectomy can be performed and the open appendiceal stump used as an enterotomy. r Removal of the meconium can be facilitated by judicious and gentle use of either a Foley or Fogarty catheter (Fig. 17-1). r Using the appendix for removal of the meconium has a slight theoretical advantage in that it prevents future appendiceal pathology (Fig. 17-2). 

Chapter 17  •  Meconium Disease   145

Figure 17-1 

Figure 17-2 

146   Section IV  •  Abdomen

r

For most patients, once the meconium has been evacuated, the enterotomy or appendiceal stump can be closed primarily.  If there is tenacious meconium that cannot be removed, or if there is a question of the ability of the enterotomy or appendiceal stump to heal, a T-tube (enterotomy) or Malecot (appendicostomy) can be placed to create a controlled fistula (Fig. 17-3). A 10 to 14 French T-tube is trimmed, and extra holes are cut before insertion. It is held in place by two pursestring sutures, and the bowel is sutured to the abdominal wall as well. In patients who have had a T-tube placed in the terminal ileum, daily irrigation with N-acetylcysteine is used to promote and maintain patency of the terminal ileum. Once pancreatic enzymes have been started and spontaneous defecation established, the tube can be removed 2 to 3 weeks after surgery. The fistula tract will then spontaneously close. r In cases of complex meconium ileus or severely ischemic bowel in simple meconium ileus, bowel resection may be indicated.  Primary anastomosis historically has been frowned on but can be done if the bowel is decompressed well distally and there is no evidence of ischemia.  An end ileostomy and mucous fistula is the safest and most diverting ostomy. r The Bishop-Koop and Santulli stomas were developed to make stoma closure easier and to give access to the bowel. The Bishop-Koop procedure (Fig. 17-4, A) involves resection of the segment of ileum obstructed by the meconium and anastomosing the end of the proximal bowel (ileum) to the side of the distal bowel. The end of the distal bowel (ileum) is brought out as a decompressive ileostomy that can also be used for irrigation. The Santulli (Fig. 17-4, B) refers to the formation of a proximal enterostomy, and the distal end is anastomosed to the side of the proximal bowel. These procedures are uncommonly performed today but can be useful in patients with densely adherent intraluminal meconium who are at risk for postoperative obstruction or with dramatically different caliber of proximal and distal bowel. r Free-flowing meconium peritonitis, which occurs with a late-term in utero perforation, can be treated by evacuation and irrigation. In this setting, there is a chemical and minimally contaminated peritonitis. The bowel can usually be separated for inspection, and a decision of how to treat the perforation can be made, usually to resect and bring out stomas. Primary anastomosis is not indicated in the setting of meconium peritonitis. r Meconium pseudocyst occurs when there is an in utero perforation weeks to months before birth. Calcification of the pseudocyst wall is often seen, and a bowel that is involved cannot be separated from this dense inflammatory response. These neonates are best treated with diversion and control of the perforation. The procedure should evacuate distal meconium and leave as much bowel length as possible. Surgery in the setting of a meconium pseudocyst may range from just placing a tube enterostomy into a well-formed perforation to an extensive resection of the pseudocyst and involved bowel.

Step 4: Postoperative Care

r

Neonatology and pulmonary specialists are critical. r If an enema was successful in evacuating the viscous meconium, N-acetylcysteine (Mucomyst) is started (5 mL per NG tube every 6 hours). Pancreatic enzymes are started when feeds are started beginning with a dose of 450 to 900 units of lipase per gram of fat. Breast milk or easily absorbed infant formulas such as Pregestimil are preferred. r Residual meconium can be evacuated postoperatively by instilling N-acetylcysteine through the NG tube or, if a T-tube was placed, through the T-tube. r As soon as spontaneous defecation is established, an elemental formula should be ordered and pancreatic enzymes started.

Chapter 17  •  Meconium Disease   147

Figure 17-3 

A Figure 17-4 

B

148   Section IV  •  Abdomen Step 5: Pearls and Pitfalls

r

Meconium ileus occurs most commonly in patients with cystic fibrosis. This is one of very few diagnoses associated with abdominal distention at birth, before the infant swallows any air. r On physical examination, the abdomen is nontender and may feel “doughy.” Dilated bowel loops and peristaltic waves may be visible. There may also be a “putty sign”; that is, an indentation made by a finger into the bowel loop filled with meconium may still be visible after pressure is released. r Around 10% of infants with jejunal atresia will have cystic fibrosis. r

Distal Intestinal Obstructive Syndrome Step 1: Surgical Anatomy

r

The term meconium ileus equivalent has now been replaced by the term distal intestinal obstructive syndrome (DIOS). r The key distinction for these patients is between constipation and DIOS. r The current, and most clinically relevant, definition of DIOS is fecal obstruction unresponsive to usual laxatives. r Patients with DIOS are almost exclusively older than 5 years of age. r Most patients develop DIOS because of a change in their routine, such as insufficient oral enzymes, noncompliance, or a recent change in enzymes. r One of the most significant risk factors is a previous episode of DIOS; recurrence is reported in more than 80% of patients. r Symptoms include chronic crampy abdominal pain, anorexia, early satiety, vomiting, weight loss, and change in stool pattern. r On physical examination, the abdomen will be distended, and there is usually a palpable fecaloma in the right lower quadrant. r Plain radiograph of the abdomen will show a copious amount of fecal material in the right colon, usually with a “soap bubble” appearance.

Step 2: Preoperative Considerations

r

DIOS can lead to intussusception, with the heavy stool acting as a lead point; appendicitis, with the lumen of the appendix occluded by the inspissated stool; and volvulus. r The differential diagnosis of DIOS includes these complications as well as adhesive small bowel obstruction, fibrosing colonopathy, Crohn disease, and small bowel fistula. r Treatment of patients with DIOS begins with intravenous hydration and correction of electrolyte imbalances. r An NG tube may be placed, initially for decompression and subsequently to deliver solubilizing agents. r Rectal contrast enemas (Gastrografin, Hypaque or Omnipaque +/− Tween 80), and oral N-acetylcysteine are given to loosen and liquefy the inspissated stool. r Once some stool begins to pass, Golytely can be used to complete evacuation of the stool.

Chapter 17  •  Meconium Disease   149

Step 3: Surgery

r

If surgery is necessary, the appendix should be removed and the stump used to flush the stool into the colon. If an appendectomy has already been performed, an enterotomy can be used instead to evacuate the stool. r For patients with recalcitrant recurrence of DIOS, an appendicostomy or button cecostomy may be indicated.

Step 4: Postoperative Care

r

Following resolution of the acute event, it is important to prevent recurrence by adjusting enzyme therapy, concentrating on good oral hydration and using stool softeners/hydrating agents such as MiraLax.

Step 5. Pearls and Pitfalls

r

Other risk factors for DIOS include binge eating or other dietary indiscretions, medications that slow gut transit, fasting, respiratory infection, and fluid loss from warm weather or exercise. r DIOS can also occur after surgery, and prophylaxis for patients with cystic fibrosis should be considered in the perioperative period after any procedure.

Other Meconium-Related Disorders Meconium Plug Syndrome

r

Meconium plug syndrome is an obstruction of the colon (rather than the terminal ileum) that occurs in 1:500 to 1:1000 live births and was classically described by Clatworthy in 1956 as a “transient form of distal colonic or rectal obstruction in newborn infants caused by inspissated, immobile meconium.” r Meconium plug has a high association with cystic fibrosis and Hirschsprung disease. Up to 25% of patients with meconium plug will subsequently be shown to have cystic fibrosis. r The risk factors for isolated meconium plug syndrome include prematurity, hypotonia, hypermagnesemia, respiratory distress, sepsis, maternal diabetes, and hypothyroidism. r Patients with meconium plug syndrome are usually full-term infants whose symptoms include vomiting, distention, and failure to pass meconium. r Contrast enemas are not only diagnostic but therapeutic as well. Only rarely is a second enema needed, and surgery is almost unheard of in this syndrome.

150   Section IV  •  Abdomen

Meconium Disease of Prematurity

r

Premature infants pass meconium later than term infants. On occasion, like meconium ileus or meconium plug syndrome, the meconium becomes obstructive, and these infants become symptomatic. r The diagnosis of meconium disease of prematurity is usually made when the infant is between 5 and 30 days of age. r These infants are often symptomatic before diagnosis. They have a distended but otherwise benign abdomen and are not systemically ill. r Abdominal radiographs resemble those of meconium ileus, with dilated loops and a paucity or absence of air-fluid levels. r Contrast enemas should be obtained for diagnostic as well as therapeutic reasons. r Very dilute Gastrografin (1:5) or iso-osomolar contrast is recommended. r In these tiny and vulnerable patients, a trip to radiology has risks. r Although some physicians contend that these risks are worth the benefit of fluoroscopic guidance, others start with a bedside technique. r Although some physicians have suggested the use of small-volume, frequent saline enemas to evacuate meconium in low-birth-weight infants, this method was shown to be ineffective in a prospective, randomized trial. r Likewise, the use of N-acetylcysteine via NG tube is probably ill advised because of the risk of hypernatremia in these fragile patients. r Unlike term infants with meconium plug syndrome, infants with meconium disease of prematurity have no increased risk of cystic fibrosis or Hirschsprung disease. r

Bibliography

Clatworthy HW, Howard WH, Lloyd J. The meconium plug syndrome. Surgery 1956;39:131-142. Dimmitt RA, Moss RL. Meconium diseases in infants with very low birth weight. Semin Pediatr Surg 2000;9(2):79-83. Docherty JG, Zaki A, Coutts JAP, et al. Meconium ileus: a review 1972-1990. Br J Surg 1992;79(6):571-573. Escobar MA, Grosfeld JL, Burdick JJ, et al. Surgical considerations in cystic fibrosis: a 32-year evaluation of outcomes. Surgery 2005;138(4):560-571, discussion 571-572. Meetze WH, Palazzolo VL, Bowling D, et al. Meconium passage in very-low-birth-weight infants. JPEN J Parenter Enteral Nutr 1993;17(6):537-540. Noblett HR. Treatment of uncomplicated meconium ileus by Gastrografin enema: A preliminary report. J Pediatr Surg 1969;4:190-197. Rescorla FJ, Grosfeld JL. Contemporary management of meconium ileus. World J Surg 1993;17(3):318-325. Rubinstein S, Moss R, Lewiston N. Constipation and meconium ileus equivalent in patients with cystic fibrosis. Pediatrics 1986;78(3):473-479.

18 

C H A P Pull-Through   TER Chapter 18  •  Hirschsprung Disease: Transanal 151

Hirschsprung Disease: Transanal Pull-Through Stig Somme and Jacob C. Langer

Step 1: Surgical Anatomy

r

Three important landmarks to consider for transanal pull-through: The anorectal squamocolumnar junction (dentate line)—The mucosal incision should be made high enough that the transitional epithelium is preserved. If this epithelium is damaged, sensation will be abnormal and continence will be impaired.  The submucosal plane of the rectum—This method is relatively bloodless and permits the surgeon to prevent injury to the vagina or prostate during the initial dissection.  The plane along the outside of the rectal wall—Also relatively bloodless. The principal reason for staying in this plane is that it will prevent injury to pelvic structures. Blood vessels to the rectum must be divided just as they enter the rectal wall. r The location of the pathological transition zone should be determined before beginning the anal dissection. r In children with a more proximal transition zone, the normally innervated bowel might not reach the anus. In these cases a number of maneuvers can be performed, including dividing the lateral attachments of the descending colon, taking down the splenic flexure, and dividing mesenteric vessels proximally so that blood supply is maintained via the marginal vessels. 

Step 2: Preoperative Considerations

r

The diagnosis of Hirschsprung disease is suspected based on the clinical picture of intestinal obstruction, constipation, or enterocolitis. A contrast enema often shows a “transition zone.” However, around 10% of newborns with rectosigmoid Hirschsprung disease and all children with total colonic aganglionosis do not exhibit this finding. r The diagnosis must be confirmed with a rectal biopsy reviewed by an experienced pathologist. The biopsy should be obtained from the posterior wall of the rectum about 1 cm proximal to the anorectal squamocolumnar junction (dentate line). The diagnosis is based on the absence of ganglion cells in an adequate specimen. A false-positive diagnosis may occur if the biopsy is taken too close to the dentate line or if there is not enough submucosa. There is controversy regarding the need for routine cholinesterase staining. 151

152   Section IV  •  Abdomen

r

Between the initial diagnosis of Hirschsprung disease and the definitive operation, rectal stimulations and irrigations should be performed as needed. If necessary, the infant can be discharged and scheduled for an elective operation at a later date. If this course is chosen, the parents must be taught how to perform rectal stimulations or irrigations before discharge and must be educated to recognize the signs of enterocolitis. r In premature infants, it may be difficult for the pathologist to interpret a rectal biopsy because of the prematurity of the ganglion cells. These infants should be managed with rectal stimulation or irrigations or both until term, when a suction biopsy can be obtained. r If long-segment Hirschsprung disease is suspected based on a radiologic transition zone extending proximal to the midtransverse colon or into the small bowel, we recommend performing a diverting stoma in the normally innervated colon or small bowel, followed by a Duhamel pull-through at a second stage after the stool has thickened. r We do not perform formal bowel preparation for infants with Hirschsprung disease because they often become distended as a result of the distal functional obstruction. Instead, we recommend a washout in the operating room before beginning the operation.

Step 3: Operative Steps

Anesthesia

r

General anesthesia with endotracheal intubation is used. A single-shot caudal block is achieved at the beginning of the operation and again at the end of the procedure. A caudal catheter should not be used because it is located too close to the surgical site. r Prophylactic antibiotics, covering both coliforms and anaerobic organisms, should be given before the incision is made. r

Chapter 18  •  Hirschsprung Disease: Transanal Pull-Through   153

Position

r

The infant is placed in lithotomy position, with the legs abducted at the hip joint and support under the knees to gain access to both the anus and to the abdomen. r The infant is moved to the foot of the table so that the operating surgeon can sit on a stool below the child. r During the perineal portion of the procedure, Trendelenburg positioning is helpful for visualization. r A headlight is helpful to optimize visualization. r A Foley catheter is optional. If a catheter is not used, the bladder can be intermittently emptied using a Coudé maneuver.

Irrigation

r

Warm, diluted Betadine solution is used through a red rubber catheter to irrigate the colon until it is clean.

154   Section IV  •  Abdomen

Leveling Biopsy

r

We begin by making a supraumbilical incision, opening the fascia in a transverse fashion, including ligation and division of the umbilical vein. r A Hegar dilator is carefully introduced through the anus and used to push the sigmoid colon toward the umbilical incision, where it is grasped with a Babcock clamp (Fig. 18-1). Care is taken to identify which part of the sigmoid is proximal and which is distal. r A full-thickness biopsy is obtained from the antimesenteric border of the colon for immediate frozen-section evaluation. The biopsy site is closed using a running 4-0 PDS in a single layer, and the tail is left long for easy identification if additional biopsies are necessary. If a transition zone is identifiable by direct vision, the first biopsy is taken about 2 cm proximal to the transition zone. It must be recognized that the pathological transition zone is sometimes more proximal than either the grossly visible or radiologically evident transition zone. r While waiting for the results of the frozen section, the mesentery to the colon distal to the biopsy can be divided either by ligation or by bipolar cauterization close to the bowel wall (Fig. 18-2). r If the initial biopsies are aganglionic, additional biopsies must be obtained more proximally until normal ganglion cells are found. r In cases with a more proximal transition zone, the umbilical incision can be used to divide the lateral attachments of the descending colon, take down the splenic flexure, or divide mesenteric vessels proximally so that blood supply is maintained via the marginal vessels.

Pull-Through

r

Four 4-0 silk sutures are placed to evert the anus (Fig. 18-3). Some surgeons prefer the Lonestar retractor, but we find that it tends to tear through the tissue in infants and is therefore more traumatic. r With a nasal speculum in the anus, a circumferential mucosal incision is made using a needletip cautery (Fig. 18-4). The mucosal incision should be made 0.5 cm above the dentate line in an infant and up to 1.0 cm above the dentate line in an older child.

Chapter 18  •  Hirschsprung Disease: Transanal Pull-Through   155

Figure 18-1 

Figure 18-2 

Figure 18-3 

Figure 18-4 

156   Section IV  •  Abdomen

r

Multiple 4-0 silk sutures are placed into the proximal edge of the mucosal incision for retraction. These sutures are brought together and secured with one straight hemostat. r The dissection of the muscular cuff then proceeds using the needle-tip cautery (Fig. 18-5). The correct plane is in the submucosa, between the mucosa and the circular muscle fibers. It has a white appearance and is relatively avascular. r The submucosal dissection is carried proximally for approximately 2 cm. At this point the muscular cuff is incised and divided circumferentially (Fig. 18-6), taking care to stay immediately on the outside of the rectal muscle. r The dissection then proceeds proximally on the outside of the rectum, again taking care to stay immediately on the outside of the rectal muscle (Fig. 18-7). The rectal blood supply is divided as it enters the wall of the bowel. Most blood vessels can be “pinch-burned” (cauterized) and then transected. Larger vessels can be ligated and divided. r Eventually the peritoneal reflection is encountered and divided so that the bowel that is pulled through is intraperitoneal. At this point, it is important to maintain the orientation of the rectum so that it is not twisted. A Kelly clamp can be placed in the vertical plane to maintain the orientation, which will prevent inadvertent 360-degree rotation of the bowel. Some surgeons also place sutures periodically along the antimesenteric surface of the rectum to ensure correct orientation. r When the leveling biopsy site (proximal to the transition zone with confirmed ganglion cells) has been reached, the pull-through is extended for another 2 to 3 cm proximally to ensure that the transition zone is not included in the anastomosis (Fig. 18-8). r The anterior half of the circumference of the normally innervated bowel is transected. The anastomosis is performed using interrupted 4-0 Vicryl sutures, starting with the 12, 3, and 9 o’clock positions. Bowel transection is then completed, the specimen passed off the field, and the 6 o’clock stitch is placed. The rest of the anastomosis is performed using a “divide and conquer” technique, bisecting the space between the initial stitches and then continuing to divide the spaces between sutures until the anastomosis is complete. It is important to obtain full-thickness bites of the pulled-through colon and generous bites of the underlying muscle on the distal rectum. It is also critical to ensure that the distal bites include only rectal mucosa and not transitional epithelium or skin (Fig. 18-9). r When the anastomosis has been completed, a gentle digital examination is performed to ensure that it is patent and that there is no twist. In the very small infant, the anastomosis might not accept a finger, in which case an appropriate-sized Hager dilator should be passed.

Chapter 18  •  Hirschsprung Disease: Transanal Pull-Through   157

Figure 18-6 

Figure 18-5 

Figure 18-8 

Figure 18-7 

Figure 18-9 

158   Section IV  •  Abdomen

Closure

r

After changing gloves and gowns, the surgeon closes the umbilical incision. We use a single layer of running 3-0 PDS, starting from both ends and working toward the middle. r The skin is closed with interrupted subcutaneous 5-0 absorbable sutures and Steri-strips. The wound is infiltrated with 0.25% bupivacaine with epinephrine. r The eversion sutures are removed from the anus, and Polysporin is applied. r A nasogastric tube is not necessary, and no drains are placed.

Step 4: Postoperative Care

r

Postoperatively the infant is observed for post-anesthesia problems, as well as for evidence of bleeding, abdominal distention, or fever. r Feeding can be started as soon as the infant arrives in the nursery unit or on the surgical ward. We usually start with clear fluids such as a pediatric oral electrolyte solution and advance to a regular diet as tolerated. r Postoperative pain is managed with 10 mg/kg of acetaminophen given orally every 4 hours. In unusual cases, particularly if the caudal block was not effective, intravenous narcotics may be necessary. r The infant can be discharged home as soon as there is evidence of bowel function and toleration of full feedings. r Before the infant is discharged, detailed instructions are provided with regard to signs and symptoms of enterocolitis, which can occur even within the first week postoperatively. Enterocolitis is the most common reason for readmission and is the most common cause of death in patients who have undergone a pull-through for Hirschsprung disease. r The parents should also be instructed about how to prevent perineal excoriation from the frequent and loose bowel movements that most children experience early after a pull-through. Routine protection of the perineal skin with Ihle’s paste (a 25% zinc oxide–based barrier) and stoma powder is commonly recommended, although many other effective barrier creams exist. A stomal therapist can be very helpful in providing support and advice to the parents with respect to this common problem. r The first postoperative visit is in 7 to 14 days. At this visit we calibrate the anastomosis using Hegar dilators. In infants a no. 10 dilator is adequate, and in older children a no. 12 Hegar should easily pass through the anastomosis. A small amount of bleeding can be expected. The patient is brought back again weekly over the next 6 to 8 weeks for calibration as the anastomosis heals. Although many surgeons instruct parents to dilate the anastomosis postoperatively on a daily basis, we use this approach only if the anastomosis is tight during the weekly calibration.

Chapter 18  •  Hirschsprung Disease: Transanal Pull-Through   159

Step 5: Pearls and Pitfalls

r

An accurate histologic diagnosis is necessary before a pull-through is performed. If there is any question about the diagnosis, the pull-through should be performed at a later time after multiple full-thickness leveling biopsies have been obtained and the samples have been examined by an experienced pathologist. r For surgeons who prefer the use of laparoscopy to the umbilical incision, the operation is essentially the same as described above. r Children who initially have profound malnutrition, severe enterocolitis, or massively dilated proximal bowel should be managed with a preliminary colostomy in normally innervated colon. Once the reason for the colostomy has resolved, the child can still undergo a transanal pull-through. In this situation the colostomy in taken down and used as the pull-through bowel. The rest of the procedure is done as described above. r For children with long-segment Hirschsprung disease, we prefer to perform a Duhamel procedure rather than a transanal pull-through. If the transition zone is proximal to the midtransverse colon during the leveling biopsies, we bring a colostomy or ileostomy in normally innervated bowel. In most cases, the stoma is brought out through the umbilical incision. The umbilical incision can be used again when the colectomy and pull-through are ultimately performed, resulting in an excellent cosmetic appearance. r The transanal pull-through is more difficult and treacherous in a child with massive dilatation of the rectum. This can be particularly problematic in older children (older than 2 years) with a delayed diagnosis and short-segment disease, in whom the rectum is both dilated and thickened. In these children, a Duhamel procedure may be a better choice.

160   Section IV  •  Abdomen

r

Attention to the technical details is extremely important to ensure a good outcome. These details include the following:  Avoid twisting of the bowel.  Avoid stretching of the sphincter during the dissection. This is accomplished by pulling the bowel firmly down so that the operation is done outside the anus rather than placing retractors within the anus and stretching the sphincter unnecessarily.  Create the rectal muscular cuff to be no more than 2 cm long. If the cuff is made longer, it is important to divide it or excise a strip of it posteriorly so as to avoid constriction of the pull-through bowel by the cuff.  Ensure that the anastomosis is performed proximal to the dentate line so that the transitional epithelium, which is so important in normal sensation and therefore in continence, is not disturbed.

Bibliography

Dasgupta R, Langer JC. Transanal pull-through for Hirschsprung’s disease. Semin Pediatr Surg 2005;14:64-71. Langer JC, Durrant AC, de la Torre ML, et al. One-stage transanal Soave pull-through for Hirschsprung’s disease: a multicenter experience with 141 children. Ann Surg 2993;238:569-576. Nasr A, Langer JC. Evolution of the technique in the transanal pull-through for Hirschsprung disease: effect on outcome. J Pediatr Surg 2007;42:36-39.

19 

C H A P T ETechniques   R Chapter 19  •  Hirschsprung Disease: Soave and Duhamel 161

Hirschsprung Disease: Soave (Open and Laparoscopic-Assisted) and Duhamel Techniques Anne C. Kim and Daniel H. Teitelbaum

Open Endorectal (Soave) Pull-Through Step 1: Surgical Anatomy



The endorectal pull-through procedure essentially requires the removal of the rectal mucosa and submucosa to create an aganglionic cuff through which normal ganglionic intestine is brought through. In most cases, this can be performed as a primary or one-stage procedure, avoiding the need for a leveling colostomy. Advantages of an endorectal dissection include the avoidance of trauma to sensory nerves in the rectum and preservation of the internal sphincter.  To have a complete understanding of the surgical anatomy, some consideration should be given to the histology of the colon.  Underlying the normal mucosa is a submucosal layer, which includes a muscular layer and accompanying submucosal (Meissner) plexus; deep to this is yet another muscular layer with its accompanying myenteric (Auerbach) plexus.  In Hirschsprung disease, the ganglia in both the submucosal and myenteric plexuses are absent for some length proximal to the dentate line.  The exact relation of the dentate line to the most distal extent of the aganglionic segment was initially characterized by Aldridge and Campbell in 1968. They defined a “hypoganglionic segment” in which ganglia were sparse, but still present, that extended, on average, for about 0.5 cm cranial to the dentate line. Indeed, the length of this segment was determined for each layer of the neural plexus, from an average of 4 mm for the myenteric plexus, 7 mm for the deep mucous plexus, and 10 mm for the superficial mucous plexus.

161

162   Section IV  •  Abdomen



Therefore, suction rectal biopsies taken about 2 cm cranial to the dentate line should provide definitive diagnosis of the disease. In addition, starting the level of dissection from 0.5 cm proximal to the dentate line will ensure that the entire aganglionic segment is removed.  The muscular complex surrounding the anal canal includes the internal sphincter arising as the continuation of the circular smooth muscle of the rectum; this ends 2.6 to 6.6 mm caudal to the dentate line. Surrounding this layer is a striated muscular complex called the levator ani, contiguous with the external sphincter that begins just caudal to the internal sphincter. Therefore, the endorectal mucosal dissection should continue to 5 mm cranial to the dentate line in a neonate and 1 cm above this line in older children. By maintaining this distance, the entire sphincteric complex is easily spared from inadvertent damage, decreasing the risk of incontinence.

Step 2: Preoperative Considerations



Even in neonates, serial rectal washouts should be performed with 10 mL/kg of normal saline accompanied by digital dilation. It should be emphasized that these are washouts, not enemas. This requires that a large-bore catheter be placed above the aganglionic region and left in place to allow the evacuation of stool. The fluid for or the irrigation performed most immediately before the operation should also include 1% neomycin. In addition, parenteral antibiotics covering both skin flora and enteric organisms should be administered within a half hour of initial incision as well as two doses postoperatively.  Older infants and children should also undergo a formal bowel preparation. Two days before surgery, a clear liquid diet should be initiated. On the day before surgery, a polyethylene glycol (PEG ~3500 molecular weight) oral solution should be given. Because children with Hirschsprung disease cannot spontaneously evacuate stool, serial rectal washouts must be performed every 4 to 6 hours on the day the PEG solution is administered.

Step 3: Operative Steps

Positioning and Preparation



A nasogastric tube is placed after induction of anesthesia. The patient should be placed in lithotomy position, with the buttocks brought to the edge of the table and propped on a folded towel. The legs should be carefully positioned on wooden skis or leg supports with proper padding and care paid to pressure points. Both the abdomen and perineum should be prepared with antiseptic solution in standard fashion before draping both areas to allow for access to the anus and entire abdomen. Alternatively, in infants, the entire body from the upper abdomen to the feet may be prepped in total, and feet and legs placed in stockinettes (Fig. 19-1). One may then have an assistant elevate both legs during the transanal anastomosis portion of the procedure. Once the patient is prepped, a Foley catheter should be inserted into the urinary bladder. Before the incision is made, the entire table should be placed in a slight Trendelenburg position.

Chapter 19  •  Hirschsprung Disease: Soave and Duhamel Techniques   163

Figure 19-1 

164   Section IV  •  Abdomen

Incision



A left lower quadrant hockey-stick type incision should be made and encompass the preexisting colostomy, if present.

Leveling



The classic appearance of the proximal (ganglionic) bowel shows an extremely hypertrophied muscular wall, with a loss of the taenia coli (Fig. 19-2). The transition zone between aganglionic and ganglionic bowel can be made by a combination of visual inspection and a series of frozen sections. Once the presence of normal ganglion cells is identified, the bowel should be transected with a stapling device above the transition zone (ideally about 5 cm proximal or cranial to this point). This step is recommended because the level of aganglionosis can vary around the circumference of the colon, and proceeding more proximally will help to ensure that the selected bowel will have essentially normal pathology throughout. Both the proximal bowel and distal bowel are then mobilized, with the latter dissected to around 2 to 4 cm above the peritoneal reflection. Traction sutures are then placed at the end of the proximal colonic segment to facilitate the pull-through.

Endorectal Dissection



Beginning on the distal bowel, a 2-cm segment just below the level of the peritoneal reflection should be cleared of serosa, mesentery, and pericolonic fat. The seromuscular layer is then incised by electrocautery down to the level of the submucosa. This incision is extended circumferentially using a hemostat. Dissection is carried further using a Kitner to perform blunt dissection, or in infants or neonatal patients a cotton-tipped applicator can be used effectively for this purpose (Fig. 19-3).  Once established, this plane of dissection is continued distally. Upward pulling on the traction sutures of the distal rectum is necessary to provide helpful countertraction. A helpful addition is the placement of other traction sutures into each quadrant of the muscle cuff as the dissection progressively develops (Fig. 19-4). Without the application of this countertraction, the dissection becomes ineffective, and one cannot proceed distally to an adequate level. Electrocautery should be used to coagulate larger communicating vessels between the submucosa and muscular cuff. The dissection should be carried out to within 0.5 cm of the dentate line in neonates and approximately 1 cm in older children.  Of note, this dissection can be carried out via a transanal approach, but if the dissection is begun transabdominally, it is most easily continued in this manner to maintain the proper plane. In addition, employment of the transabdominal approach minimizes the stretch placed on the anal sphincters by the retractors required to provide exposure. Once the dissection is complete, the posterior portion of the muscular cuff is split posteriorly and carried down as distally as possible, almost to the same level as the endorectal dissection.

Chapter 19  •  Hirschsprung Disease: Soave and Duhamel Techniques   165

A

B

Figure 19-2 

Figure 19-3 

Figure 19-4 

166   Section IV  •  Abdomen Pull-Through



At this point, one surgeon moves to the perineal field and places retractors (phrenic or armynavy) at the anal verge. A ring forceps or Kelly clamp is then inserted into the rectum, and the assistant in the abdominal field will place the end of the mucosal or submucosal tube into the clamp. Eversion of the dissected segment is then performed, and the end of the tube is regrasped with a clamp and held in traction (Fig. 19-5).

Anastomosis



An incision should then be made on the anterior half of the mucosal or submucosal tube, 0.5 cm proximal to the dentate line. A Kelly clamp should be placed through this opening and used to grasp the traction sutures on the proximal bowel (Fig. 19-6). Particular attention should be paid to avoid twisting the bowel as it is pulled through the muscular cuff. The placement of two differently colored sutures on the mesenteric and anti-mesenteric sides of the ganglionic bowel will help the surgeon identify and correct any twisting that may occur during the pull-through process.  The anastomosis is begun by incising the anterior half of the ganglionic colon and suturing it to the anterior cuff of the anal mucosa using 4-0 polyglactin suture. Sutures placed at each corner and in midline are then used as traction sutures by the assistant as the surgeon places interrupted sutures to complete the anastomosis quadrant by quadrant.  The posterior portion of the anastomosis should be completed by incising one quarter of the remaining ganglionic colon and everted mucosal or submucosal segment.  Exposure by the assistant is facilitated by outward traction placed on the sutures that have been placed into each quadrant.  The final quadrant of the anastomosis should be completed and inspected. The neo-rectum can be inverted by applying gentle upward traction on the colon. Rectal examination can then be performed to palpate the anastomosis 1.5 to 2 cm proximal to the anal verge. After a change of gloves, attention should be redirected to the abdominal field.  The seromuscular portion of the pulled-through segment can then be attached to the muscular cuff to prevent early postoperative prolapse. No drain is placed because significant oozing is rarely seen.

Step 4: Postoperative Care



The nasogastric tube can typically be removed at the end of the operation. Care following the endorectal pull-through is that practiced for any patient who has undergone colon resection with a low anastomosis. It is advisable to allow no rectal examinations or medications per rectum for at least 2 to 3 weeks. All attempts should be made to provide postoperative pain relief, as well as to normalize diet and provide stool softeners as soon as possible. As mentioned previously, two doses of antibiotics with skin and enteric flora coverage should be administered postoperatively. Follow-up and careful calibration of the anastomosis with Hegar dilators should be performed at frequent clinic visits beginning at 3 weeks postoperatively.

Chapter 19  •  Hirschsprung Disease: Soave and Duhamel Techniques   167

Figure 19-5 

Figure 19-6  Photo courtesy of Dr. Essam Elhalaby, Tanta University, Egypt.

168   Section IV  •  Abdomen

Step 5: Pearls and Pitfalls



A general way to determine adequate mobilization of the proximal colonic segment is to stretch it over the pubis to the anal verge. If more length is needed, it may be helpful to divide the inferior mesenteric artery near its origin, preserving the marginal arterial arcade.  Concern over the subsequent problems with stool evacuation caused by the presence of an abnormally functioning internal anal sphincter has led some surgeons to divide not only the posterior wall of the muscular cuff but also the cranial portion of the internal sphincter. This appears to cause few, if any, problems with continence.  As mentioned, it is helpful to use different colored sutures on the end of the proximal, ganglionic bowel to maintain its orientation.  Although complete strictures of the anastomosis are uncommon, the anastomosis should be gently sized with a Hegar dilator at each clinic visit. In general, at the initial visit (about 3 weeks postoperatively), a neonatal pull-through should accept a size 7 to 9 Hegar. Subsequent visits should gradually increase this size to between 10 and 12.

Laparoscopic-Assisted Endorectal (Soave) Pull-Through Step 1: Surgical Anatomy

See preceding Soave technique.

Step 2: Preoperative Considerations



Preoperative washouts and, depending on the patient’s age, bowel preparation are identical to that for the endorectal pull-through, as detailed above. Use of preoperative antibiotics is the same as that with the open endorectal pull-through. Careful consideration should be given to positioning, for if the lower extremities are too high, they will interfere with the surgeon’s ability to manipulate the laparoscopic instruments. It is often easier to prep the entire lower half of the infant into the operative field, and keep the legs in a supine position, or hanging just off the bed, during the laparoscopic portion of the procedure. One could then have an assistant elevate the lower extremities once the surgeons are ready to perform the perineal portion of the procedure.  At the beginning of the case, the surgeon generally stands on the patient’s right, while the assistant stands at the foot of the operating table. Placement of the monitor on the left of the patient near the foot of the bed is optimal (Fig. 19-7).

Chapter 19  •  Hirschsprung Disease: Soave and Duhamel Techniques   169

Anesthesiologist

Monitor

Operating surgeon

Assistant surgeon

Figure 19-7 

170   Section IV  •  Abdomen

Step 3: Operative Steps

Incision



An umbilical port should be placed first, followed by trocars in the right upper quadrant and the remaining one in the suprapubic area.

Leveling



Before mobilization of the colon, it is necessary to determine the level of aganglionosis, which begins by visually inspecting for a transition zone and taking a seromuscular biopsy just above this level. This is most easily accomplished by grasping the serosa with the nondominant hand and sharply cutting into the intestinal wall until the level of the submucosa is reached. One then proceeds to dissect the bowel bluntly in this plane until a 0.5-cm piece of muscularis is obtained. If a perforation occurs, one can simply close this hole with an intracorporeally placed suture.  If one cannot identify a transition zone, one should take the first biopsy just above the peritoneal reflection and proceed proximally at about 10-cm intervals as needed. One should also consider performing an appendectomy early in such a case, particularly if specimens continue to show absence of ganglia, as the child may have total colonic aganglionosis.

Dissection



Dissection and leveling of the aganglionic segment proceed as in the open technique, with the assistant holding the camera and the upper trocar grasping the rectum for exposure and retraction. Surgical clip appliers may be used to ligate blood vessels, with small vessels controlled by cautery (Fig. 19-8). Following mobilization, the surgeon and assistant should switch places. The transanal dissection, pull-through, and anastomosis then proceed, the details of which are given in the chapter on the transanal approach (see Chapter 18).

Chapter 19  •  Hirschsprung Disease: Soave and Duhamel Techniques   171

Figure 19-8 

172   Section IV  •  Abdomen Step 4: Postoperative Care



Postoperative care is as detailed in the section on endorectal pull-through.

Step 5: Pearls and Pitfalls



Ideally the two working ports should each be a hand’s width away from the umbilical port; however, if the inferior port is placed too low, the mobility of the trocar will be limited by the lower extremities and iliac crest. An ideal way to assess whether one has achieved adequate mobilization of the ganglionic bowel is to confirm that it can be easily be pulled down to the lowest portion of the pelvis before beginning the transanal dissection.  It is also important to mark carefully the transition zone of the colon with a suture during the laparoscopic portion of the case to allow for easy confirmation of this level during the transanal portion of the procedure.

Duhamel Pull-Through Step 1: Surgical Anatomy



The Duhamel technique was advanced in 1956 to avoid the tedious pelvic dissection of the Swenson procedure and to protect the nervi erigentes, which may be found lateral and anterior to the rectum. The procedure has undergone several modifications, the most important of which was by Martin, and included the use of an automatic stapling device. It is fairly straightforward and continues to be popular today. Despite its relative simplicity, several key technical points must be followed.  As with other pull-through procedures, ganglionic bowel is brought down to less than 1 cm proximal to the dentate line. To preserve the autonomic nerve plexus to the genitourinary system, very little manipulation of the rectum is performed anteriorly.  In the past, the child often had a leveling colostomy, which was placed several months previously. This served to decompress the bowel and return it to normal caliber. The operation was generally performed when the child was 6 to 12 months of age with a weight of 10 kg. With the use of smaller endo-stapling devices, the procedure can also be performed primarily in the newborn period.

Step 2: Preoperative Considerations



The child is admitted the day before the surgery for a mechanical bowel preparation as well as oral antibiotics. Care must be taken to give adequate rectal and colonic washouts because stool is often inspissated in the distal rectum. It is necessary to do a rectal examination on the child before the pull-through to ensure that no residual stool is present. Preoperative antibiotics should be given.

Chapter 19  •  Hirschsprung Disease: Soave and Duhamel Techniques   173



A nasogastric tube is placed after induction of anesthesia. The child is placed in a supine position, and the patient is prepared circumferentially from the abdomen to the feet. Stockinettes are placed around each foot, and a Foley catheter is inserted into the bladder after the patient has been prepared and draped. Excellent exposure is obtained by assistants supporting and flexing the lower extremities at the hips during the anal anastomosis. Alternatively, the child can be placed in stirrups or on skis.

Step 3: Operative Steps

Incision



A hockey stick or oblique incision is made to incorporate the colostomy, if present.

Leveling



The transition zone between aganglionic and ganglionic bowel can be made by a combination of visual inspection and a series of frozen sections. Once the presence of normal ganglion cells is identified, the bowel should be transected with a stapling device above the transition zone (ideally approximately 5 cm proximal or cranial to this point) to ensure that the remaining bowel will have essentially normal innervation. Both the proximal bowel and distal bowel are then mobilized, with the latter mobilized to approximately 2 to 4 cm above the peritoneal reflection. Traction sutures are then placed at the end of the proximal colonic segment to facilitate the pull-through.

Retrorectal Dissection



Blunt dissection in the posterior midline is used to create a retrorectal space down to the level of the pelvic floor. Dissection is considered complete once an assistant’s finger, inserted only 1 to 1.5 cm into the anus, can be palpated from the abdominal field. While a blunt clamp may help the dissection, an index finger can easily be used to develop this plane.  Following completion of the retrorectal dissection, the aganglionic bowel is transected with a stapler at the level of the peritoneal reflection. Tacking sutures are placed on each edge of the staple line to facilitate anterior retraction of this segment during the pull-through.  Polypropylene and polyglactin sutures should be used to mark the mesenteric and antimesenteric surfaces of the ganglionic bowel. This will help the surgeon to maintain the orientation of the bowel as it is pulled through.

174   Section IV  •  Abdomen

Pull-Through



If the legs are not already in skis, they should be drawn upward by an assistant to allow a clear view of the anus. The surgeon should move to the perineal portion of the surgical field and place narrow anal retractors to be held by assistants in the abdominal field. Although the instruments used in the perineal portion of the procedure should be treated as contaminated, there is no need to maintain a separate sterile field with separate setups. However, gloves should be changed once the anastomosis is created.  Cautery is used to create a full-thickness incision on the posterior wall of the rectum, 0.5 cm (1 cm in older infants) proximal to the dentate line. This distance should be carefully maintained by curving the incision appropriately when extended in each direction. To maintain proper orientation of the bowel, the inferior and superior edges of the incision are respectively marked with undyed and dyed polyglactin sutures. These sutures are placed in the midline, and with one on each edge; hemostats are used to hold each suture in position.  From the perineal field, a long clamp is inserted into the retrorectal space and toward the abdominal field. The clamp is used to grasp the tacking sutures on the distal ganglionic bowel, and the surgeon in the abdominal field guides the bowel to help prevent rotation of the bowel as it is pulled through (Fig. 19-9).  The staple line is then excised on the anterior half of the ganglionic colon, and a single-layered anastomosis is begun using the three previously placed polyglactin sutures. Each stitch should be carefully placed to ensure that the anterior wall of the anus is not incorporated into any of the sutures.

Anastomosis



After completing the anterior half of the anastomosis, the remainder of the staple line is excised, and the anastomosis is completed.  One arm of an automatic stapling device is placed into the native anal canal while the other is placed into the neorectum (Fig. 19-10). The stapler is fired directly in the midline, and the suture line is checked for hemostasis. A long (80 mm) stapling device is generally preferred; a smaller endo-stapler is used in newborns.  It is common for a single staple application to be insufficient to complete the full length of the anastomosis of ganglionic and aganglionic bowel (Fig. 19-10, B). This can be remedied by firing the stapler from the abdominal field. The staple line of the aganglionic rectum is opened, and a small enterotomy is made in the ganglionic colon at a similar level to allow placement of a reloaded stapler. The anastomosis must be digitally examined to ensure that it is complete. Huge fecalomas can form if a bridge or spur remains between bowel segments.  The opened staple line is then sutured to the enterotomy in the ganglionic colon in two layers. Care is taken not to leave a blind-ending stump of aganglionic bowel because this could lead to the development of a large fecaloma. The neorectum may or may not be reperitonealized, and the abdomen is closed. The nasogastric tube typically can be removed at the end of the procedure.

Chapter 19  •  Hirschsprung Disease: Soave and Duhamel Techniques   175

Figure 19-9 

A Figure 19-10 

B

176   Section IV  •  Abdomen

Step 4. Postoperative Steps



Postoperative care is as detailed in the section on endorectal pull-through, except that dilation with Hegars may not be necessary.

Step 5. Pearls and Pitfalls



As mentioned previously, it is of paramount importance to ensure completion of the anastomosis between the aganglionic and ganglionic portions of the bowel.  Care must be taken to ensure that there is no twisting of the pulled-through segment; as mentioned, coordination between the assistant and operating surgeon is critical.

Bibliography

Aldridge RT, Campbell PE. Ganglion cell distribution in the normal rectum and anal canal: a basis for the diagnosis of Hirschsprung’s disease by anorectal biopsy. J Pediatr Surg 1968;3:475-490. Teitelbaum DH, Coran AG. Hirschsprung disease, operations. In Spitz L, Coran AG, eds. Operative pediatric surgery, 6th ed. London: Hodder Headline Group, 2006; pp. 553-563. Teitelbaum DH, Coran AG. Hirschsprung’s disease and related neuromuscular disorders of the intestine. In Grosfeld JL, O’Neil JA, Fonkalsrud EW, Coran AG, eds. Pediatric surgery, 6th ed. Philadelphia: Mosby Elsevier, 2006; pp. 1514-1547.

20 

C H A P T E R Procedure   177 Chapter 20  •  Hirschsprung Disease: Swenson Pull-Through

Hirschsprung Disease: Swenson Pull-Through Procedure Marleta Reynolds

Step 1: Surgical Anatomy



Hirschsprung disease is the clinical manifestation of the absence of ganglion cells of the involved intestine. Ganglion cells are absent from the submucosal (Meissner) plexus as well as the intramuscular (Auerbach) plexus. In addition, there is an increase in the longitudinal nerve fibers, which stain intensely for acetylcholinesterase.  The incidence of Hirschsprung disease ranges from 1 in 4000 to 1 in 7000 live births, with a male-to-female ratio of 4:1.  The sporadic occurrence of Hirschsprung disease accounts for 80% to 90% of cases. There has been an explosion of genetic information regarding the complex inherited pattern of Hirschsprung disease and its association with neurocristopathies.  In 80% of affected patients, the aganglionic segment of colon extends from the anus to the sigmoid colon. A longer segment of colon is involved in 10% to 15% of patients. Total colon Hirschsprung disease occurs in between 3% and 10% of patients, with extension into the small bowel in less than 3% of patients.  Trisomy 21, trisomy 18, Ondine curse, and a variety of other cristopathologic disorders are associated with Hirschsprung disease.  The diagnosis is most frequently made in the newborn period. The newborn has signs of bowel obstruction, including failure to pass meconium in the first 48 hours of life, abdominal distention, and vomiting. In infancy and childhood, symptoms of chronic constipation with or without episodes of explosive diarrhea can be present.

177

178   Section IV  •  Abdomen

Step 2: Preoperative Considerations

Neonates



Physical examination reveals a full-term infant with a distended abdomen. Examination of the perineum reveals an anus in the correct position, and rectal examination reveals an appropriate anal and rectal diameter. Withdrawal of the digit often results in the production of explosive liquid stool.  Plain abdominal film reveals a distal bowel obstruction with dilated large bowel (Fig. 20-1). Barium enema reveals a narrow distal segment with dilated proximal bowel (Fig. 20-2). A postevacuation film reveals retained contrast material (Fig. 20-3).  Suction rectal biopsy should be obtained with the request for acetylcholinesterase staining.

Infants and Children



Physical examination reveals a distended abdomen, normally placed anus, a rectal examination of normal caliber, a collapsed rectum, and production of explosive stool with removal of the digit. Barium enema reveals a collapsed distal colon and a dilated proximal colon. Postevacuation films reveal retained contrast material.  Suction rectal biopsy in infants older than 3 months is usually inadequate for diagnosis, and a full-thickness rectal biopsy will need to be obtained. This involves a preoperative mechanical bowel preparation and general anesthetic.

Management



Most infants can be treated with colonic irrigations once or twice per day before scheduling a definitive procedure. Timing for a pull-through procedure is surgeon dependent, with some performing the procedure in the first several weeks of life and others waiting until infants are 2 to 3 months of age and weigh at least 10 pounds.  If colonic irrigations do not sufficiently decompress the bowel and the infant is unable to tolerate feedings and gain weight, consideration should be made for the placement of a temporary colostomy.  Infants with long-segment Hirschsprung disease or total colonic Hirschsprung disease are less likely to thrive with a colonic irrigation management scheme and will need a temporary colostomy placed in the colon or small bowel, where normal ganglion cells are present. A Kimura patch should be considered in infants with total colon Hirschsprung disease as a two-step procedure preceding pull-through.

Chapter 20  •  Hirschsprung Disease: Swenson Pull-Through Procedure   179

Figure 20-1 

Figure 20-2 

Figure 20-3 

180   Section IV  •  Abdomen Step 3: Operative Steps



A preoperative admission is planned for a mechanical bowel preparation consisting of colonic irrigations every 4 hours and installation of a sufficient quantity of Go-Litely by oral or nasogastric route. A central venous line is placed for intravenous hydration, blood sampling, and postoperative total parenteral nutrition.  The patient is placed on the operating table in the supine position, and the colon is irrigated with normal saline and dilute Betadine. Parenteral antibiotics are administered. The infant is placed at the end of the table in the frog leg position; an older child is placed in a lithotomy position. The abdomen and the perineum are prepared and a Foley catheter placed.  A transanal Swenson pull-through is appropriate for most infants with rectosigmoid Hirschsprung disease. Minimally invasive techniques can be used alone or in combination with the transanal approach in infants or children with more proximal colonic involvement.

Transanal Operative Technique



Four stay sutures are placed at the mucocutaneous junction and epinephrine injected at the dentate line (Fig. 20-4). A circumferential incision is then made through the full thickness of the bowel wall just proximal to the dentate line. Stay sutures are placed through the full thickness of the bowel wall circumferentially, and bipolar cautery is used to dissect proximally.  Traction is placed on the bowel with the stay sutures, and the dissection is continued using the bipolar cautery to ligate and divide the feeding vessels staying on the bowel wall (Fig. 20-5). The transition zone can be visualized as the bowel begins to increase in size and thickness.  Full-thickness biopsy of the bowel wall at this level will identify the transition zone with the presence of a few ganglion cells and hypertrophied nerves.  Dissection is continued proximally until normal ganglion cells are identified. Full-thickness biopsies are taken in the anterior and posterior aspects of the bowel, with different-colored sutures placed on the anterior surface and the posterior surface to maintain orientation.  At the point where normal ganglion cells are identified anteriorly and posteriorly and when there is no evidence of hypertrophied nerves, three fourths of the circumference of the bowel is divided (Fig. 20-6). Sutures are placed through the muscularis in four quadrants. A fullthickness anastomosis is then created with absorbable sutures (Fig. 20-7). The remainder of the bowel is divided and removed and the anastomosis completed (Fig. 20-8).  A laparoscope can be introduced through the umbilicus to evaluate the orientation of the pulled-through bowel. In patients in whom the involved bowel extends more proximally than the sigmoid colon, minimally invasive techniques can be used to mobilize the colon and ensure that the pulled-through intestine has an adequate blood supply.

Operative Technique: Minimally Invasive Assisted



After a pneumoperitoneum is created, two 5-mm and one 12-mm ports are placed. The suspected transition zone is identified and biopsies are taken to identify the presence of

Chapter 20  •  Hirschsprung Disease: Swenson Pull-Through Procedure   181

Figure 20-4 

Figure 20-5 

Figure 20-6 

Figure 20-7 

Figure 20-8 

182   Section IV  •  Abdomen

normal ganglion cells proximal to the transition zone (Fig. 20-9). Once this is established, the bowel is divided with the Endo-GIA stapling device.  The pelvic peritoneum is incised and the mobilization of the rectum is accomplished by cauterizing the vessels with the harmonic scalpel on the bowel wall. This dissection is carried distally to 1 cm from the dentate line.  The proximal bowel is mobilized to provide enough length to reach the anus. If necessary, mesenteric vessels are clipped at the base of the mesentery, preserving the arcade of vessels to the bowel to be pulled-through.  The rectum is everted at the anus, and an incision is made 1 cm proximal to the dentate line over three fourths of the circumference.  A clamp is placed through this opening, and the normal proximal colon is pulled through the incision under direct vision.  Muscularis sutures are placed in four quadrants, and the distal colon is divided. A fullthickness anastomosis is created with absorbable sutures. The distal bowel is amputated and the final quarter of anastomosis completed.

Step 4: Postoperative Care



Parenteral antibiotics continue for a minimum of 72 hours. Nasogastric drainage is instituted intraoperatively and continues until there is return of bowel function. The diet is then advanced as tolerated.  A Foley catheter is used for intraoperative and postoperative monitoring of urine output and is removed on postoperative day 3.  Parents are given instructions and supplies for care of a significant diaper rash. 

Follow-up Care



The patient is seen 2 weeks following discharge, and a thorough physical examination is performed. No formal rectal examination is performed at this time. A gentle sizing of the rectal opening with small Hegar dilators occurs at the 4-week visit.  Any evidence of obstruction from postoperative edema is treated with gentle irrigations at home, with an examination under anesthesia in the operating room when indicated.  Postoperative stricture formation is identified at the postoperative visits and is treated with examination under anesthesia in the operating room. Late signs of dysmotility or relative obstruction are treated with examination under anesthesia and gentle dilation of the internal sphincter. Postoperative irrigations may be necessary in some infants, however, and if the need for irrigations or repeat dilation ensues, consideration should be made for re-evaluation of the extent of resection and possible repeat full thickness biopsy.

Chapter 20  •  Hirschsprung Disease: Swenson Pull-Through Procedure   183

Figure 20-9 

184   Section IV  •  Abdomen



It should be emphasized to the family to return to the hospital immediately for any high fever associated with abdominal distention, lethargy, or a decrease in bowel movements. The families should be educated to watch for these life-threatening signs of enterocolitis.

Step 5: Pearls and Pitfalls



At the time of pull-through procedure, biopsies of the anterior and posterior aspect of the involved bowel will help avoid the complication of retained aganglionic bowel.  Meticulous dissection and bipolar cautery use on the bowel wall will prevent injury to surrounding nerves.  The need for increased awareness of possible enterocolitis, which is treated with immediate irrigation and general stretching of the internal sphincter under anesthesia, decreases as the child grows. Incidents of enterocolitis and the need for intervention usually decrease by the 3rd or 4th year following pull-through procedure.  Early Kimura patch followed by Swenson pull-through procedure greatly reduces the need for total parenteral nutrition and associated liver failure in infants with total colon Hirschsprung disease.

Bibliography

Curran TJ, Raffensperger JG. The feasibility of laparoscopic Swenson pull-through. J Pediatr Surg 1994;29(9):1273-1275. Curran TJ, Raffensperger JG. Laparoscopic Swenson pull-through: a comparison with the open procedure. J Pediatric Surg 1996;31(8): 1155-1157. Dasgupta R, Langer JC. Transanal pull-through for Hirschsprung’s disease. Semin Pediatr Surg 2005;14(1):64-71. Nishijima E, Kimura K, Tsugawa C, Muraji T. The colon patch graft procedure for extensive aganglionosis: long-term follow-up. J Pediatr Surg 1998;33(2):215-219. Swenson O. Hirschsprung’s disease. In Raffensperger JG, ed. Swenson’s pediatric surgery, 4th ed. New York: Appleton-Century-Crofts, 1980; pp. 507-531. Teitelbaum DH, Coran AG. Hirschsprung’s disease and related neuromuscular disorders of the intestine. In Grosfeld JL, O’Neill JA, Fonkalsrud EW, Coran AG, eds. Pediatric surgery, 6th ed., vol. 2. Philadelphia: Elsevier, 2006; pp. 1514-1559. Weidner BC, Waldhausen JH. Swenson revisited: a one-stage, transanal pull-through procedure for Hirschsprung’s disease. J Pediatr Surg 2003;38(8):1208-1211.

21 

CHAPTER Chapter 21  •  Imperforate Anus   185

Imperforate Anus Marc Levitt and Alberto Peña

Step 1: Surgical Anatomy



Anorectal malformations occur in 1 in 4000 neonates and are found slightly more commonly in boys than in girls. The most common defects in girls are rectovestibular fistula followed by perineal fistula and persistent cloaca. The most common in boys is a rectourethral fistula followed by perineal fistula. Rectobladder neck fistulas in boys occur in 10% of patients, and imperforate anus without fistula in both boys and girls represents only 5% of the entire group of defects.  A therapeutically oriented classification includes the following.

Boys



Perineal (cutaneous) fistula Rectourethral fistula (bulbar and prostatic)  Rectobladder neck fistula  Imperforate anus without fistula  Rectal atresia 

Girls



Perineal (cutaneous) fistula Vestibular fistula  Imperforate anus without fistula  Rectal atresia  Persistent cloaca 

185

186   Section IV  •  Abdomen Step 2: Preoperative Considerations

Associated Defects

Sacrum and Spine The sacrum is often abnormal, and the degree of sacral development correlates with the quality of sphincter and nerves and thus the final functional prognosis.  Traditionally the number of vertebrae was the criterion for evaluating the sacrum. One missing vertebra does not have important diagnostic implications. More than two absent vertebrae represent a poor prognostic sign.  To improve the prognostic accuracy based on sacral abnormalities, a sacral ratio was created that expresses the degree of sacral development. For this measurement, three lines are drawn: line A extends across the uppermost portion of the iliac crests; line B joins both inferior and posterior iliac spines; and line C runs parallel to lines A and B and passes through the lowest radiologically visible sacral point. In normal children the ratio of the distances BC:AB is between 0.7 and 0.8 in both anteroposterior and lateral projections (Fig. 21-1). Children with anorectal malformations suffer from different degrees of sacral hypodevelopment, with the ratio varying between 0 and 0.8. A ratio of less than 0.4 usually signifies a poor functional prognosis. Urogenital Defects  The frequency of associated urogenital defects varies from 25% to 50%. Patients with persistent cloaca or rectobladder neck fistulas have a 90% chance of having a significant associated urologic abnormality; infants with minor defects (perineal fistula) have a less than a 10% chance.  The most common urologic malformation associated with imperforate anus is absent kidney, followed by vesicoureteric reflux. Hydronephrosis, urosepsis, and metabolic acidosis from poor renal function represent the main sources of mortality in neonates with anorectal malformations.  Patients with anorectal malformations should undergo an ultrasound study of the abdomen during the first 24 hours after birth, and if this study shows any abnormalities, a thorough urologic evaluation is indicated. Other Defects  Other congenital malformations commonly associated with anorectal malformations include esophageal atresia, duodenal atresia, and cardiovascular defects. 

Management of Anorectal Malformations During the Neonatal Period



Two important questions must be answered during the first 24 hours of life: what are the associated anomalies, and what operation is required—a newborn pull-through or a colostomy? Males  The decision-making algorithm for the management of male newborns with anorectal malformations is shown in Fig. 21-2.  Associated malformations must be investigated. In more than 80% of boys, perineal inspection and urinalysis provide enough information to make a clinical diagnosis. If a perineal fistula exists, the patient may be treated with a minimal posterior sagittal anorectoplasty. The presence of a flat bottom and meconium in the urine are indications for a diverting colostomy. The colostomy decompresses the intestine in the neonatal period, provides access for a contrast study to define the anorectal anatomy, and will subsequently provide protection against infection during the healing process after the definitive repair.

Chapter 21  •  Imperforate Anus   187

A

B

C

Figure 21-1 

Newborn male - Anorectal malformation Perineal inspection

20–24 hrs

* Spine

* Sacrum

* Kidney U/S

* Spinal U/S

* Urinalysis

* Cardiac echo

* R/O esophageal atresia Re-evaluation and cross-table lateral film

Perineal fistula

Rectal gas below coccyx No associated defects

Anoplasty

Consider PSARP with or without colostomy

Figure 21-2 

Rectal gas above coccyx Associated defects Abnormal Sacrum Flat bottom

Colostomy

188   Section IV  •  Abdomen 

It is important to wait 20 to 24 hours before deciding what operation to do. These patients do not show abdominal distention during the first few hours of life, and even if a perineal fistula is present, meconium is not usually seen on the perineum until 20 to 24 hours after birth. Radiologic evaluations are inaccurate because of the presence of meconium in the blind pouch of the intestine, interfering with the migration of air into the pouch and giving a false image of a high defect. A significant amount of intraluminal rectal pressure is required to reach a level high enough to overcome the voluntary muscle tone that keeps the most distal part of the rectum compressed.  Shortly after birth, intravenous fluids are administered and a nasogastric tube is inserted to keep the stomach decompressed, thus avoiding the risk of vomiting and aspiration. Antibiotics are administered, and an ultrasound study of the abdomen is performed to rule out the presence of other anomalies (mainly urologic). A piece of gauze is placed on the tip of the penis, and the nurses are instructed to check for particles of meconium on this gauze.  If after 20 to 24 hours of observation there is no clinical evidence indicating the need for a colostomy or a perineal operation, the patient should have a cross-table lateral film with the patient in the prone position to determine the position of the rectal pouch. The anal dimple is marked. If the rectum is visible below the coccyx, the patient can undergo a primary newborn repair, provided the surgeon is experienced with this technique. If the image is questionable, it is preferable to perform a diverting colostomy.  The patient is discharged to home after recovery from colostomy placement. If the patient is growing well and has no other associated defects (cardiovascular or gastrointestinal) that require treatment, he is readmitted at 6 to 12 weeks of age for a posterior sagittal anorectoplasty after undergoing a distal colostogram. This early repair can be performed safely only if the surgeon has experience in dealing with the delicate anatomy of the infant.  Performing the definitive repair early in life has important advantages for the patient, including less time with an abdominal stoma, less size discrepancy between proximal and distal stomas at the time of colostomy closure, simpler anal dilatation, and no recognizable psychological sequelae from painful perineal maneuvers. In addition, at least theoretically, placing the rectum in the right location early in life may represent an advantage in terms of acquired local sensation.  Some surgeons have proposed primary repair of all anorectal malformations during the neonatal period without a protective colostomy. There is no question that this can be done and that it has the potential of avoiding the morbidity related to the formation and closure of a colostomy. The disadvantages include the fact that the tissue planes of the neonate are not as well defined as in older patients. Also, the diagnostic tests used to determine the level of the defect are not accurate enough, and the surgeon is actually subjecting the patient to a blind exploration of the perineum. If the rectum is located high in the abdomen, the surgeon may damage other structures during the search for the rectum. Such structures include the posterior urethra, seminal vesicles, vas deferens, bladder neck, and ectopic ureters. In addition, there is a risk of dehiscence and infection because the stool is not diverted. Females  A decision-making algorithm for the initial management of females is shown in Fig. 21-3. Perineal inspection usually provides more information in girls than in boys. The principle of waiting 20 to 24 hours before making a decision is again valuable.  The presence of a single perineal orifice is pathognomonic for a cloaca. Because of their complexity, these defects are dealt with in a separate chapter.  Perineal inspection may reveal the presence of a vestibular fistula (Fig. 21-4, in prone position), which is the most common condition in girls. In cases with rectovestibular fistula, the rectal orifice is located within the vestibule and outside the hymen. A true rectovaginal fistula is an extremely rare anomaly.  These patients can undergo a primary repair via a posterior sagittal approach, either in the newborn period or following a period of dilatations, provided that the surgeon has adequate experience and that a meticulous technique is used. Our preference is to perform the repair during the newborn period. A colostomy followed by the definitive repair is an acceptable and safe approach. These fistulas are usually large enough to decompress the gastrointestinal

Chapter 21  •  Imperforate Anus   189

Newborn female - Anorectal malformation

R/O serious, potentially lethal associated defects

-Sacrum

-Kidney and abdominal U/S

-Esophagus

-Spinal U/S (tethered cord)

-Cardiac echo

-Lumbar spine

Perineal Inspection

Single perineal orifice Cloaca

Perineal fistula

Vestibular fistula

24 hours

-UroL evaluation

Cross table lateral X-Ray

-R/O hydrocolpos Colostomy Drain hydrocolpos Urinary diversion (if necessary)

No visible fistula (1500 U/mL), ferritin (>142 ng/mL), vanillymandelic acid (VMA)/homovanillic acid (HVA) ratio less than 1, N-myc amplification, chromosome 1 short arm (p) deletion, DNA ploidy, decreased CD44 expression, increased tyrosine kinase A expression, increased multidrug resistance–associated protein, and unbalanced 11q loss of heterozygosity.  Screening markers include urinary catecholamine metabolites, HVA, and VMA.

355

356   Section VI  •  Tumors Staging



Computed tomography (CT) scan of chest, abdomen, and pelvis is typically performed at initial workup. 99m  The occult areas of metastasis can be assessed by technetium 99m (Tc MDP) scan.  Tumor viability, disease extent, recurrence, and metastatic disease can also be determined with the use of the radiolabeled metaiodobenzylguanidine (MIBG) scintigraphy.  Plain radiographs of areas of bony tenderness are obtained to evaluate for cortical bone metastasis.  Bilateral posterior iliac crest bone marrow aspiration is performed.  Tissue biopsy should be obtained via methods of needle, incisional, or excisional approach of the primary or metastatic lesions to assess for tumor behavior and diagnosis before the initiation of therapy.  Paraneoplastic syndromes and associated abnormalities include opsoclonus-myoclonus syndrome, vasoactive intestinal peptide–mediated severe secretory syndrome, central hypoventilation syndrome (Ondine’s curse), DiGeorge syndrome, and congenital heart disease.

Imaging



Routine imaging studies include chest radiographs, ultrasonography, CT, and magnetic resonance imaging (MRI).  The preferred diagnostic tool for initial imaging, staging, and the detection of metastases is CT (Fig. 34-1).  Calcifications are noted in about 85% of cases.  In patients with thoracic tumors or tumors with intraspinal extensions, MRI is thought to be more helpful.

Differential Diagnoses



Differential diagnoses include Wilms tumor (calcification is uncommon, invades renal vein and inferior vena cava [IVC], displaces vessels), neonatal adrenal hemorrhage (avascular, decreased echogenicity on ultrasonography), pheochromocytoma, and adrenocortical carcinomas.

Step 3: Operative Steps

Anesthesia



General anesthesia with intravascular pressure monitoring is used.  Epidural anesthesia is frequently used for effective pain control.

Chapter 34  •  Neuroblastoma   357

Figure 34-1 

358   Section VI  •  Tumors

General Surgical Principles

Abdominal Tumors   Transverse supraumbilical or chevron incision is used.  A thoracoabdominal approach is used for large right-sided tumors adherent to the diaphragm or liver or in tumors with both intrathoracic and intra-abdominal components.  Adequate surgical exposure, often using a surgical retractor system (e.g., Omni), is essential (Fig. 34-2).  Gentle exploration of the abdominal cavity should be performed, assessing for the primary tumor as well as for any displaced adjacent structures.  Most intra-abdominal tumors arise from suprarenal regions.  Identify and establish major vascular control. The IVC above and below the tumor is exposed.  Stage III and IV tumors can involve encasement of major vascular structures, demanding meticulous dissection to avoid injury.  For right-sided tumors, the renal vein and artery are mobilized and vessel loops are placed around them.  Dissection plane is established between the tumor and the IVC.  Proper exposure and ligation of right adrenal venous drainage to IVC and left adrenal venous to left renal vein are essential. Fig. 34-3 demonstrates vessel loops placed around the left renal vein.  Arterial branches are controlled using silk ties as well as transfixing suture ligatures.  Maintenance of proper tissue dissection plane during tumor resection is critical.  Tumor does not typically invade the tunica media of major blood vessels.  Meticulous hemostasis is essential to successful outcome (Fig. 34-4).  Nephrectomy should be avoided unless substantial tumor burden will be left behind.  Adequate exposure of the retroperitoneum is essential for proper assessment and resection of intra-abdominal neuroblastoma.

Chapter 34  •  Neuroblastoma   359

Figure 34-2 

Figure 34-3 

Figure 34-4 

360   Section VI  •  Tumors



For advanced-stage tumors, initial biopsy is performed for tissue diagnosis and analysis for tumor markers. Laparoscopic approach has been advocated (Fig. 34-5). Cervical and Thoracic Tumors   Localized tumors are approached via a low transverse collar incision, whereas those with mediastinal or thoracic extension require a combined thoracic and supraclavicular approach.  Isolated thoracic tumors are resected via an open or thoracoscopic approach.  Unlike abdominal tumors, cervical and thoracic neuroblastomas usually do not encase major vessels.  Horner syndrome may result from stellate ganglion involvement. Spinal Tumors   The dumbbell tumors are the extradural extensions of the intrathoracic paraspinal neuroblastoma. Surgical management is controversial and presents a significant challenge in deciding whether the thoracic or spinal component should be resected first (Fig. 34-6).  With preoperative chemotherapy and radiotherapy, these tumors are less friable, and the risk of intraspinal bleeding and perioperative neurologic complications is reduced.  In the absence of a cord compression, surgical excision of primary tumor is preferred.  In patients with cord compression or acute paralysis, decompressive laminectomy should be performed by neurosurgery before primary tumor resection is done. Pelvic Tumors   In general, these tumors are located deep in the presacral region, in close proximity to the pelvic nerves and hypogastric vessels.  Pfannenstiel incision with bilateral extensions to the anterior superior iliac spines may be preferred.  Iliac artery, sciatic nerve, pelvic autonomic nerves, ureters, and major visceral structures must be properly identified and preserved.

Chapter 34  •  Neuroblastoma   361

A

B

Figure 34-5 

Figure 34-6 

362   Section VI  •  Tumors

Step 4: Postoperative Care



Initial monitoring in an acute or intermediary care setting is usually required for patients who have undergone major resections.  Hemodynamic monitoring using arterial and central venous lines is essential because of significant third-space fluid sequestration.  Pain management can be effectively achieved with epidural catheter.  Careful assessment and maintenance of nutritional support are critical.  A postoperative ileus should be anticipated for a few days.  Other potential complications include chylous ascites resulting in significant morbidity, intestinal obstruction or ischemia, and major organ injury during extensive resections following chemotherapy in advanced-stage tumors.

Chapter 34  •  Neuroblastoma   363

Step 5: Pearls and Pitfalls



Thorough and accurate determination of staging at diagnosis is essential.  Evaluate for complete resectability of early stage tumors.  Multidisciplinary treatment approach is crucial.  Determine advanced-stage tumors that require preresection adjuvant therapy.  Adequate tissue biopsy for histology and determination of tumor markers must be done.  Tumor rupture and spillage must be avoided, considering how friable neuroblastoma tumors tend to be with common areas of tumor necrosis.  Lymph node sampling and clearance in neuroblastoma remain controversial.  Neuroblastomas tend to engulf and surround vessels, typically displacing kidney, and are highly vascular.  It is also a continuous spectrum with more benign counterparts, such as ganglioneuroma and ganglioneuroblastoma.  Skin metastases appear as “blueberry muffins,” skull base fracture-like “raccoon eyes,” and radiographic appearance of massive liver metastases is described as pepper syndrome.

Bibliography

Attiyeh EF, London WB, Mosse YP, et al. Chromosome 1p and 11q deletions and outcome in neuroblastoma. N Engl J Med 2005;353: 2243-2253. Bowen K, Chung DH. Recent advances in neuroblastoma. Curr Opin Pediatr 2009;21:350-356. Brodeur GM. Neuroblastoma: biological insights into a clinical enigma. Nature Rev Cancer 2003; 3:203-216. Ishola, TA, Chung DH. Neuroblastoma. Surg Oncol 2007;16:149-156. Von Allmen D, Grupp S, Diller L, et al. Aggressive surgical therapy and radiotherapy for patients with high-risk neuroblastoma treated with rapid sequence tandem transplant. J Pediatr Surg 2005; 40:936-941.

CHAPTER

35 

Sacrococcygeal Teratoma Bryan J. Dicken and Frederick J. Rescorla

Step 1: Background and Surgical Anatomy



Sacrococcygeal teratomas (SCTs) are the most common extragonadal germ cell tumors in children. The incidence is approximately 1 in 35,000 live births, with females accounting for 75% of cases.1  SCTs are composed of elements from one or more of the embryonic germ cell layers and contain tissue foreign to the anatomic site of origin. Mature teratomas consist of well-differentiated tissues, whereas immature teratomas contain varying amounts of immature tissue such as neuroectoderm.2 Histologically, teratomas are classified according to their stage of immaturity.3  SCTs present in two clinical patterns:  Neonates with large external, predominantly benign lesions  Children between infancy and 4 years of age with predominantly malignant lesions arising primarily within the pelvis  When the tumor is found before 1 month of age, the risk of malignancy is about 5%, whereas when the tumor is discovered between 1 and 12 months of age, the risk of malignancy is 60%; in children older than 1 year, 75% of tumors are malignant.3  The most commonly applied anatomic classification, adopted by the American Academy of Pediatrics (AAP), is based on the degree of intrapelvic extension (Fig. 35-1).4

Step 2: Preoperative Considerations

Neonatal



Generally the tumors are noted to have a characteristic mass protruding from the sacral region (Fig. 35-2).  This lesion is often detected by prenatal ultrasound. If the external portion is larger than 5 cm, abdominal delivery should be considered so as to avoid dystocia and tumor rupture.2

364

Chapter 35  •  Sacrococcygeal Teratoma   365

I

II

III

IV

Figure 35-1 

Figure 35-2 

366   Section VI  •  Tumors Infant and Pediatric Patients



Generally older children have no external portion noted at birth and present secondary to tumor compression of the bladder or rectum or a palpable rectal mass.  Preoperative evaluation should focus on evaluating the degree of pelvis and abdominal extension by the following:  Clinical assessment: A complete physical examination, including a digital rectal examination to evaluate the presacral region  Anteroposterior and lateral radiographs: Pelvis and spine radiographs may reveal a bony defect, suggesting dural communication. Calcifications are a frequent finding and in general are less common in malignant lesions.  Radiologic studies: Generally ultrasound is adequate to delineate pelvic or abdominal extension. When a significant abdominal component is identified or concerns arise as to malignant potential, double-contrast computed tomography is useful to demonstrate the relation of the tumor to the rectum and urinary tract, as well as to exclude distant metastasis. Magnetic resonance imaging is particularly useful in evaluating anatomic relation to adjacent structures and possible infiltration into the spinal canal (Fig. 35-3).3  Laboratory investigations: An elevated serum alpha-fetoprotein (AFP) level is commonly associated with malignant tumors (yolk sac or endodermal sinus tumor, embryonal carcinoma, or undifferentiated germ cell tumor).2  There is an increasing frequency of malignancy with higher AAP anatomic classification (Table 35-1).4

Step 3: Operative Steps

Positioning



About 46.7% of SCTs have a significant external component (AAP class I) and can generally be approached in a prone jack-knife position.1 This can be accomplished using a gel roll to support the face and head in neutral position, along with a thoracic gel roll, with the arms in a swimmer’s position. The pelvis is generously elevated using additional gel rolls under the anterior superior iliac spine, with the hips flexed and abducted and the knees slightly flexed and the feet supported with a soft roll (Fig. 35-4).  The balance of SCTs have a significant pelvic or abdominal component (AAP classes II-III) or are contained entirely within the presacral space (AAP class IV). These patients are best approached initially through a supine position, with the dissection beginning in the abdomen.2 Surgical preparation should extend from the nipples to the toes to facilitate intraoperative patient manipulation.

Table 35-1.  Frequency of Malignancy Associated with Higher American Academy of Pediatrics Anatomic Classification AAP TYPE

MALIGNANT (%)

METASTASIS (%)

I

8

0

II

21

6

III

34

20

IV

38

8

Chapter 35  •  Sacrococcygeal Teratoma   367

Figure 35-3 

Figure 35-4 

368   Section VI  •  Tumors

Operative Technique



The abdominal approach through a lower transverse muscle–sparing incision enables mobilization of the pelvic portion of the tumor as well as early ligation of the sacral artery (Fig. 35-5) and access to the distal aorta in very large or vascular lesions.  Use of self-retaining retractors to pack the bowel away from the operative field, followed by lateralization of sigmoid colon, exposes the sacral promontory. Incising the peritoneal reflection with electrocautery at the promontory exposes the median sacral artery, which can be easily ligated at its origin.  Dissection continues inferiorly in the avascular presacral space down to the level of the pelvic floor. The tumor should then be meticulously separated from the rectum with a combination of sharp and blunt dissection by electrocautery. Dissection should take place immediately adjacent to the tumor capsule. Care should be taken to avoid inadvertent division of the levator complex making up the pelvic floor as it is splayed laterally over the SCT.  Lateral and anterior dissection should take place adjacent to the tumor capsule, with care to avoid unnecessary margins that could result in bladder or sexual dysfunction resulting from injury to the sympathetic and parasympathetic nerves.  On completion of the abdominal dissection, the patient is repositioned in prone jack-knife position as described previously.

Posterior Perineal Approach



In a prone jack-knife position, an inverted “V” or inverted chevron incision is made with the apex focused on the sacrococcygeal joint (Fig. 35-6). The incision sweeps inferolaterally around the tumor to encompass the mass and abnormal or ischemic skin.  With the use of electrocautery, dissection is carried down through the subcutaneous tissue down to the tumor capsule posteriorly and gluteal muscles laterally. The gluteal muscles are typically splayed over the SCT and should be retracted laterally to allow mobilization of the tumor with blunt and sharp dissection.  The posterior dissection is carried down to the coccyx, where the coccyx is freed from the sacrum. Care must be taken to identify and ligate the sacral artery if not completed during an abdominal approach. The sacrococcygeal joint is divided with heavy scissors, leaving the coccyx attached to the teratoma.  A large red rubber catheter (10 or 12 Foley) or Hegar dilator may be inserted into the rectum to help guide separation of the tumor from the rectum (Fig. 35-7). Care should be taken to avoid excessive traction on the levator complex.

Chapter 35  •  Sacrococcygeal Teratoma   369

Sacral artery

Figure 35-5 

Figure 35-6 

Figure 35-7 

370   Section VI  •  Tumors



Wound closure begins with reapproximation of the midline levator to the presacral fascia with interrupted sutures. A passive drain may be placed to avoid seroma formation. Finally, the gluteal muscles are reconstructed in the midline, and excessive skin is excised. The subcutaneous tissue is closed with an absorbable suture, followed by a subcuticular absorbable skin closure (Fig. 35-8).

Step 4: Postoperative Care



Antibiotic prophylaxis is not necessary postoperatively. Surgical drains (if placed) should be removed on postoperative day 2 or 3 or when drainage is minimal.  Initiation of enteral feeds may be commenced on return of bowel function and advanced to enteral goals as tolerated.  Neonates with mature and immature teratomas are managed with observation, with predicted recurrence rates of 10% to 21% after complete resection of SCT.2  Tumor recurrence should be treated with surgical resection where feasible, and the patient should be treated with multiagent chemotherapy.5  Older patients frequently have unresectable lesions. After initial biopsy, these patients should undergo neoadjuvant platinum-based chemotherapy, followed by delayed surgical resection.2  Follow-up should entail the following:  Thorough physical examination of the abdomen, pelvis, buttocks region, and a rectal examination to palpate the presacral region.  Serial AFP levels should be obtained to ensure normal levels by 9 months of age in neonates.  Physical examination and serial AFP should be conducted every 3 months for 3 years after initial resection.  Functional complications following surgical resection of SCT include the following: 6  Fecal soiling (25%) in children older than 3 years of age.  Urinary incontinence (16%) or urinary tract dysfunction secondary to pelvic nerve injury during pelvic dissection.7

Chapter 35  •  Sacrococcygeal Teratoma   371

A

B Figure 35-8 

372   Section VI  •  Tumors

Step 5: Pearls and Pitfalls



Preoperative imaging is crucial in assessing respectability, directing surgical positioning, and excluding unexpected findings, such as myelomeningocele, spinal defects, and urinary tract obstruction.  Utilization of a large red rubber catheter or Hegar dilator placed into the rectum may greatly facilitate safe dissection of the tumor from the rectum.  Early identification and ligation of the median sacral artery enable early vascular control and minimize intraoperative blood loss (see Fig. 35-5).  Complete resection of the coccyx in continuity with the SCT greatly reduces local recurrence rates from 37% to around 10% to 20%.  Careful dissection immediately adjacent to the tumor capsule minimizes inadvertent injury to the sympathetic and parasympathetic pelvic plexus and should reduce long-term urinary, fecal, and possible sexual dysfunction.

Chapter 35  •  Sacrococcygeal Teratoma   373

References

1. Rescorla FJ, Sawin RS, Coran AG, et al. Long-term outcome for infants and children with sacrococcygeal teratoma: a report from the Childrens Cancer Group. J Pediatr Surg 1998;33(2):171-176. 2. Rescorla FJ. Pediatric germ cell tumors. Semin Surg Oncol 1999; 16(2):144-158. 3. Skinner MA. Germ cell tumors. In Keith T, Oldham PMC, Foglia RP, Skinner MA, eds. Principles and practice of pediatric surgery, vol. 1: Lippincott Williams & Wilkins, 2005; pp. 637-648. 4. Altman RP, Randolph JG, Lilly JR. Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. J Pediatr Surg 1974; 9(3):389-398. 5. Rescorla F, Billmire D, Stolar C, et al. The effect of cisplatin dose and surgical resection in children with malignant germ cell tumors at the sacrococcygeal region: a pediatric intergroup trial (POG 9049/CCG 8882). J Pediatr Surg 2001; 36(1):12-17. 6. Currarino G, Coln D, Votteler T. Triad of anorectal, sacral, and presacral anomalies. AJR Am J Roentgenol 1981; 137(2):395-398. 7. Boemers TM, van Gool JD, de Jong TP, Bax KM. Lower urinary tract dysfunction in children with benign sacrococcygeal teratoma. J Urol 1994; 151(1):174-176.

CHAPTER

36 

Hepatic Tumors Maria H. Alonso

Step1: Surgical Anatomy



The typical benign tumors of the liver that require resection are mesenchymal hamartoma, adenoma, and focal nodular hyperplasia. Hepatoblastoma is the most common malignant liver tumor of childhood; it usually occurs within the first 3 years of life. Hepatocellular carcinoma, sarcoma, and epithelioid hemangioendothelioma, a low- to intermediate-grade malignant tumor, is less common and generally is seen in older children.  The liver is separated into nine anatomic segments, each with an independent portal venous and arterial inflow and biliary and hepatic venous drainage. This segmental anatomy forms the basis for the design and implementation of a successful anatomic resection of the liver (Fig. 36-1).  The hepatic veins divide the liver into four sections: right anterior and posterior, left medial, and lateral. The left and middle hepatic veins form a common trunk before entering the vena cava in 60% of patients.  The right hepatic artery and portal vein enter the liver parenchyma shortly after branching. The left hepatic duct and left portal vein have a longer course after branching. Together they course to the base of the umbilical fissure, where they join the left hepatic artery before entering the liver parenchyma (Fig. 36-2).

Step 2: Preoperative Considerations



In the case of malignant lesions, most commonly hepatoblastoma, there should be an expectation of paired inflow and outflow structures that remain intact with an adequate resection margin. In most of these cases, the resection will be anatomic (i.e., along one of the planes outlined by the hepatic veins). Ligation of the appropriate arterial and portal venous inflow produces a demarcation line that correlates with the safe line of division. Benign lesions, in many situations, can be resected along nonanatomic lines.  Cross-sectional imaging for evaluation the tumor and its relationship to the vasculature of the liver is the key to preoperative planning. This imaging can be a computed tomography scan or a magnetic resonance imaging. The use of ultrasound with duplex scanning can be helpful for evaluation of both portal and hepatic venous anatomy, and it can be used intraoperatively as well. Good-quality imaging studies should identify replaced or accessory arterial anatomy and the presence of an accessory right hepatic vein. 374

Chapter 36  •  Hepatic Tumors   375

Right posterior section

Figure 36-1 

Figure 36-2 

Right anterior section

Left medial section

Left lateral section

376   Section VI  •  Tumors



Routine laboratory evaluation including blood count and renal panel would be expected, especially for children who are between rounds of chemotherapy, to ensure bone marrow recovery. Blood should be available for the procedure. Prophylactic parenteral antibiotics are given within 30 minutes of incision time and as appropriate throughout the length of the procedure.  There is definite user preference for selected equipment, but certain types of equipment can improve exposure and minimize blood loss.  A mechanical retractor such as a Thompson retractor (Thompson Surgical Instruments, Inc.) or a Buchwalter will simplify exposure, especially for suprahepatic vena cava and right hepatic resections.  The ultrasonic dissector, water-jet dissector, electrocautery, and the argon-beam coagulator can all be useful during the parenchyma dissection. The dissectors can assist in exposure of the vessels and ducts within the substance of the liver so that these structures can be easily identified and ligated.  Stapling devices can also be used to divide larger blood vessels and parenchyma.

Step 3: Operative Steps



General endotracheal anesthesia is administered, and peripheral intravenous access is obtained. A nasogastric tube and a urinary catheter are placed for decompression and drainage.  For children with planned anatomic resections or major nonanatomic resections, arterial and central lines are placed for monitoring and postoperative management. Consideration is given to intraoperative maintenance of a low central venous pressure to minimize bleeding during the division of the hepatic soft tissue.  Patient positioning is supine with the arms at the sides; the draping allows access to the lower chest and the abdomen down to the pubis. The “baby table extension” is useful for infants and toddlers.  The incision is generally bilateral subcostal with upper midline extension (Fig. 36-3). The upper midline extension provides excellent exposure of the suprahepatic vena cava, and the subcostal portion can be adjusted, depending on the specific anatomy of the tumor. For more limited resections, a subcostal or bilateral subcostal incision may suffice.  The basic principles for all anatomic liver resections include control of the inflow and outflow vessels and parenchyma transection. The methods to achieve these principles are similar regardless of the particular lobe or segment of the liver being removed. For purposes of this section, the right hepatectomy will be used as the standard to illustrate the principles previously listed.  The liver is carefully inspected and palpated to identify the tumor and any unexpected lesions and their relationship to the vascular structures. This must be completed before resection is begun.  The ligamentum teres is divided along with the falciform ligament to expose the hepatic veins. The right lobe of the liver is mobilized by dividing the right triangular ligament. The right lobe should be mobile enough to be rotated out of the abdominal cavity (Fig. 36-4).  The foramen of Winslow is palpated for identification of a replaced or accessory right hepatic artery and for the presence of any significant adenopathy (Fig. 36-5).  The cystic duct and cystic artery are divided. The gallbladder is removed at this time for better visualization of the hilar structures. Cholecystectomy is not absolutely necessary at this particular time; however, right hilar exposure may be improved.

Chapter 36  •  Hepatic Tumors   377

Typical incision

Figure 36-3 

Figure 36-4 

Figure 36-5 

378   Section VI  •  Tumors



The cystic duct stump is rotated anteriorly and medially to expose the portal vein behind the common bile duct, and the portal vein can be followed cephalad to its bifurcation. Ligation of small caudate branches will increase the mobility of the short right portal vein segment and allow for better control before ligation. The right hepatic artery should be encountered just anterior to the right portal vein branch. Both of these structures can be divided at this stage. Completion of this inflow division should produce a clear line of demarcation within the hepatic parenchyma to guide the soft tissue resection. The right hepatic duct can be left intact to be divided during the parenchyma dissection or identified and ligated at this stage of the operation (Fig. 36-6). In the presence of a single right hepatic duct that is extrahepatic, ligation during the hilar dissection will certainly improve exposure of the right portal vein.  Mobilization of the right lobe is completed by separating the bare area completely from the diaphragm to expose the right hepatic vein and inferior vena cava further.  With the right lobe rotated medially, the hepatic veins passing from the posterior aspect of the right lobe to the vena cava are divided from the inferior border of the liver, continuing cephalad until the right hepatic vein is clearly seen. This dissection will often include a portion of the caudate lobe, which courses circumferentially around the vena cava and will need to be ligated and divided. Be aware of the possibility of an accessory right hepatic vein, which will be encountered during this portion of the procedure. Once the right hepatic vein is completely exposed, it can be divided using a stapling device or vascular clamps and sutures (Fig. 36-7).  The parenchyma transection is begun by scoring the capsule along the demarcation line using electrocautery. Many techniques are available for parenchyma transection, or as adjuncts, such as the ultrasonic dissector, the water-jet dissector, and the radiofrequency dissector. Older techniques such as the crush and clamp technique work well for most of the parenchyma with the assistance of the ultrasonic or water-jet dissector as the dissection approaches the hepatic veins. This is especially true in cases where the hepatic vein anatomy is unclear and the hepatic vein has not been controlled before beginning parenchyma transection. The safety of intermittent inflow occlusion using a Pringle maneuver has been documented. This technique may be used during the parenchyma division if needed.  Close inspection of the cut surface for hemostasis and bile leak is an important step before closure. The cut surface is coated with fibrin glue. Closed suction drainage along the cut surface is optional.  The abdominal wall is closed in layers with a running, absorbable suture.

Step 4: Postoperative Care



At least for the immediate postoperative period, children are usually managed in the pediatric intensive care unit (PICU), where they can be carefully monitored for bleeding. Pain management is achieved with intravenous narcotics administered by the PICU team or by the anesthesia pain service.  For children who have had a large volume of liver resected, electrolytes, phosphorus, blood count, and prothrombin time are checked for the first 2 to 3 days postoperatively.  An oral diet can be started in the first 2 to 3 days postoperatively.

Chapter 36  •  Hepatic Tumors   379

Figure 36-6 

Right hepatic vein

Diaphragm

IVC

Figure 36-7 

Right lobe of liver rotated anteriorly

380   Section VI  •  Tumors

Step 5: Pearls and Pitfalls



Control of the appropriate hepatic vein might not be achievable until the parenchyma is divided. This can be done safely as long as care is taken during that portion of the dissection to identify the hepatic vein anatomy before transaction is done.  On rare occasions the left portal vein branch arises from the right portal vein in the substance of the liver. This anatomic variation would be very important to identify if planning resection requiring division of the right portal vein.  Intraoperative ultrasound is a useful tool to delineate the relationship of the tumor to both the portal and hepatic veins. It can be especially helpful if hepatic vein control cannot be obtained before division of the hepatic parenchyma.  The anatomy of the biliary tree is quite variable, and an intraoperative cholangiogram can provide a roadmap that can make hilar dissection more comfortable.

Bibliography

Cho CS, Park J, Fong Y. Hepatic resection. In ACS surgery: Principles and practice. WebMD: 2007. De Ville de Goyet J, Otte J-B. Liver tumors and resections. In Stinger MD, Oldham KT, Mouriquand PDE, eds. Pediatric surgery and urology: Long-term outcomes, 2nd ed. New York: Cambridge University Press, 2006; pp. 799-814. Giuliante F, Nuzzo G, Ardito F, et al. Extraparenchymal control of hepatic veins during mesohepatectomy. J Am Coll Surg 206:496-502. Gonzalez RJ, Barnett CB Jr. A technique for safely teaching major hepatectomy to surgical residents. Am J Surg 2008;195:521-525. Poon RT. Current techniques of liver transection. HPB 2007;9:166-173. Poon RT. Recent advances in techniques of liver resection. Surg Technol Int 2004;13:71-77. Vollmer CM, Dixon E, Sahajpal A, et al. Water-jet dissection for parenchymal division during hepatectomy. HPB 2006;8:377-385. Yao P, Morris DL. Radiofrequency ablation-assisted liver resection: review of the literature and our experience. HPB 2006;8:248-254.

ERRNVPHGLFRVRUJ A Abdomen following anti-reflux procedures, 277f tumor in, 358-360 Abdominal pain, choledochal cyst associated with, 232-234 Abdominal wall defect, 242 Acetaminophen, 264 following transanal pull-through, 158 Actinomycin-D, 346-348 Adenoma, in liver, 374 Adolescent cannula insertion for, 23-24 obese, surgery for, 280 venous catheterization in, 9 venovenous bypass for, 23 weight loss surgery for, 280 Adrenal carcinoma, neuroblastoma and, 356 Adrenal gland, neuroblastoma and, 355 Adrenal hemorrhage, neuroblastoma and, 356 AFP. See Alpha-fetoprotein Aganglionic cuff, 161-162 Aganglionosis, level of, 170 Alkalosis, hypertrophic pyloric stenosis and, 254-256 Allen’s test, 12 Allergy, metal, 103 Alpha-fetoprotein, 366, 370 Amplatzer ductal occluder device, 114 Ampulla of Vater, 132, 134 Anal canal, 162 Anal sphincter, internal, 168 Anastomosis between stomach and cervical esophagus, 80 cervical gastric tube pull-up and, 78 transposed stomach and, 82 cologastric, 69f diamond, 134, 135f Duhamel pull-through and, 174 in bariatric surgery, 286, 287f Soave pull-through, stricture of, 168 Soave pull-through technique and, 166 tracheoesophageal fistula repair and, 50, 51f, 64, 65f transanal pull-through and, 156, 157f Anesthesia for anti-reflux procedures, 268 for bariatric surgery, 281 for cannula insertion, 6-8 for cervical esophagostomy, 54 for chest wall deformity repair, 98, 106 for congenital diaphragmatic hernia repair, 85 for cryptorchidism repair, 319 for esophageal atresia with tracheoesophageal fistula, 46 for esophageal replacement, 68, 71 for eventration of diaphragm repair, 94 for gastric tube pull-up, 78 for thoracoscopic repair of esophageal atresia, 59-60 for thoracoscopic repair of tracheoesophageal fistula, 59-60 for thyroglossal duct cyst, 30 for transanal pull-through, 152 Angioplasty, venous patch, 24 Anorectal malformation bucket-handle, 190, 191f incidence of, 185 incision for, 196 management of, 186-190 mortality of, 204 repair of, functional disorders associated with, 204

Anorectoplasty, 186 posterior sagittal, 188 Anti-reflux procedures, 266-278 abdomen following, 277f anesthesia for, 268 description of, 270-276 instrument placement for, 270, 271f instruments for, 269f patient positioning for, 269f placement of stitches in, 275f Anti-reflux valve, 240 Antiplatelet therapy, 299 Anus aganglionic colon and, 177 imperforate (See Imperforate anus) transanal pull-through and, 154-156, 155f Aorta descending, 115f, 117f juxtaductal, 118 Apnea, gastroesophageal reflux indicated by, 266-268 Appendectomy, meconium evacuation and, 144 Appendicitis, distal intestinal obstructive syndrome and, 148 Appendicostomy, 149 meconium removal and, 146 Appendix access through, meconium evacuation and, 144 meconium removal through, 144, 145f Arteriotomy, cannulation and, 17-18 Artery cannulation of, 24 colic, 67f cystic, 376 Drummond, 67f gastric, 73f, 76, 77f, 291f esophageal replacement and, 79 gastroepiploic, 73f, 76, 77f hepatic, 374, 375f, 378 ileocolic, 67f mesenteric, 139f posterior tibial, catheterization through, 12 pulmonary, 127f left lower lobectomy and, 124-126 patent ductus arteriosus repair and, 113 radial, catheterization through, 12 renal multiple, 339 palpation of, 344, 345f sacral ligation of, 369f, 372 sacrococcygeal teratoma repair and, 368, 369f spermatic, 316 splenic, 290, 291f identification of, 296 ligation of, 297f testicular, 316 Aspirin, 299 Auerbach plexus, colon and, 161

B Babygram radiograph, 44, 46 Bariatric surgery, 279-289 complications following, 286 intestinal bypass, 279 Barium enema, Hirschsprung disease diagnosis and, 178 Beckwith-Wiedemann syndrome, 244 Wilms tumor and, 342, 342t Bell-clapper deformity, 328, 329f, 334

Index

Bile duct biliary atresia and, 220 choledochal cyst and, 232 type I atresia of, 220 type III atresia of, 220 Bile duct plug, 222, 223f Biliary ascites, 240 Biliary atresia, 220-231 cholangiography and, 225f classification of, 220, 221f histologic findings associated with, 222, 223f incision for repair of, 224, 225f liver affected by, 224 repair of, sutures for, 229f Biliary ductule, 226, 227f Biliary obstruction, choledochal cyst as cause of, 232 Biliary tree, 380 extrahepatic, 239-240 Biliopancreatic diversion, 279 Bilious emesis malrotation indicated by, 138 meconium ileus associated with, 143 Biopsy full-thickness, 180 ganglion cells located with, 183f leveling, 154 seromuscular, 170 Wilms tumor, 351 Birth trauma, eventration of diaphragm and, 91 Bishop-Koop procedure, 146, 147f Bladder hemivagina attached to, 216 neck of, 200 BMI. See Body mass index Body mass index, weight loss surgery and, 280 Bowel aganglionic, 173 anastomosis and, 176 retained, 184 compromised, 140 dilatation of, meconium ileus associated with, 143 dilated proximal, 179f division of, in Swenson technique, 181f echodense, 143 ganglionic, 164, 165f, 172-173 anastomosis and, 176 mobilization of, 172 gastroschisis repair and, 244, 245f ischemic, 146 motility of, gastroschisis repair and, 252 necrotic, 140 obstruction of, 142 bariatric surgery and, 286 distal intestinal obstructive syndrome and, 148 radiography of, 178, 179f resection of, complex meconium ileus treated with, 146 rotation of, prevention of, 174, 175f twisting of, in open endorectal pull-through, 166 vaginal reconstruction with, 216, 217f Bowel atresia, 144 Bowel movement anorectal malformation repair and, 202 decrease in, enterocolitis indicated by, 184 following cloaca repair, 218 Branchial anomalies, 34-42 dissection and, 39f example of, 35f first, 36 fourth, 38-40 second, 38

381

382   Index Branchial anomalies (Continued) third, 38-40 type I, 37f type II, 37f Branchial cyst, 34 example of, 35f large, 37f, 38 Branchial fistula, 34 Branchial sinus, 34 Bronchiole, immature, 122 Bronchus anomalies of, 34-42 lower lobe, 126, 127f Bucket-handle anorectal malformation, 191f Button cecostomy, 149

C Canal of Nuck, 308 Cannula arterial, 15 double-lumen, 15-16, 22 drainage, 15-16 for adolescent extracorporeal life support, 23-24 for drainage, 22 for reinfusion, 22 guidewire placement for, 21f insertion of, for child, 22-23 jugular vein, 22 no flow after placement of, 26 placement of, 18, 19f, 20, 21f venous, 15-17 in neonate, 25 threading of, 25 wire-wound, 15 Cannulation anesthesia for, 14 arterial, 23 guidewire placement in, 20 intrathoracic vein perforation and, 26 oxygenation, extracorporeal membrane, 14-26 transthoracic, 24 venoarterial anesthesia for, 16 technique for, 16-18 venous, 25 vein division and, 25 venovenous anesthesia for, 16 semi-open technique for, 20 technique for, 16-18 Carcinoma embryonal, 366 hepatocellular, 374 Cardiac anomaly, eventration of diaphragm and, 92 Cardiac tamponade, central line placement and, 13 Cardiopulmonary system, pectus excavatum effect on, 104 Cardiovascular defect, anorectal malformations associated with, 186 Caroli disease, 232, 238 Carotid sheath, view of, 55f Catecholamine, urinary, neuroblastoma diagnosis and, 355 Catheter arterial access for, 12 central venous, 346 external, for tunneled central line, 4, 5f, 7f pinch-off sign and, 13 placement of, through jugular vein, 6-9 temporary central line, 4 tunneled central line, 4-6 venous, postoperative care following, 12 Caudal regression syndrome, 244 Cauterization in laparoscopic-assisted endorectal pull-through, 170, 171f of azygous vein, 60 of pyriform fossa sinus, 40 of pyriform sinus, 38 of urethra, 213f

Cautery, needle-tip, transanal pull-through and, 154, 155f, 156, 157f CCAM. See Congenital cystic adenomatoid malformation CdC. See Choledochal cyst CDH. See Congenital diaphragmatic hernia Cecostomy, 149 Cecum, 139f Central hypoventilation syndrome, 356 Central line temporary, 4 tunneled, 4-6 Cephalosporin, 98, 280 Cervical esophagostomy, 54-58, 76 anesthesia for, 54 Charcot triad, choledochal cyst associated with, 232 Chemotherapy central venous catheter for, 346 sacrococcygeal teratoma treatment and, 370 Wilms tumor treatment with, 346 Chest tube, tracheoesophageal fistula repair and, 64, 65f Chest wall deformity anesthesia for repair of, 98 Nuss procedure for, 97-103 incision in, 98, 99f open repair of, 104-112 anesthesia for, 106 incision for, 106-110, 107f thoracostomy in repair of, 98-100 Child anti-reflux procedures for, 266-268, 269f bowel preparation for, 162 cannula insertion for, 22-23 central line in, 2-4 congestive heart failure in, patent ductus arteriosus associated with, 114 esophageal replacement in, 76 hepatoblastoma in, 374 Hirschsprung disease in, 178 pectus excavatum in, 97 sacrococcygeal teratoma in, 364, 366 subclavian vein catheterization in, 9-10 surgical anatomy of, vascular access and, 2-4 testicular torsion in, 328, 334 venoarterial bypass for, 22 venovenous bypass for, 22 volvulus in, 138 Cholangiocarcinoma choledochal cyst associated with, 234, 239-240 choledochal cyst repair and, 239 Cholangiogram, 380 choledochal cyst diagnosed with, 234, 235f Cholangiography, of biliary atresia, 225f Cholangitis biliary atresia repair and, 230 choledochal cyst associated with, 234, 239-240 Choledochal cyst, 232-241 decompression of, 236, 237f definition of, 232 excision of, 240f subtypes of, 232, 233f type I, 234-236 dissection of, 234, 236 type II, 236 type III, 238 type IV, 234-236 type V, 238 Choledochocele, 232 Choledocholithiasis, 236 Cholelithiasis, splenectomy and, 292 Chylous ascites, 362 Cirrhosis biliary, choledochal cyst associated with, 234 biliary atresia associated with, 220, 222, 223f, 225f Cloaca, 206-219 characteristics of patients with, 209, 209t colostomy and, 210 common channel and, 212-214 defects associated with, 208 endoscopy for diagnosis of, 209 illustration of, 207f in neonate, 208

Cloaca (Continued) incidence of, 185, 206 persistent diagnosis of, 206-208 in female, 185 urogenital defect and, 186 repair of, 210-214 signs of, 188 treatment goals for, 206 Cloacagram, 209 Coagulopathy, liver transplant for, 230 Coccyx, resection of, 372 Cologastrostomy, 70 Colon aganglionic, 177 ascending, 139f Auerbach plexus of, 161, 177 descending, 66, 68, 139f division of, 67f, 68 esophageal replacement and, 66 histology of, 161-162 hypoganglionic segment of, 161 interposition of, 66-70 intramuscular plexus of, 177 Meissner plexus of, 161, 177 mobilization of, 168 sigmoid, 68, 177 submucosal plexus of, 161, 177 transverse, 66, 68 vagina replacement and, 214, 215f view of, 67f Colon conduit, configuration of, 68, 69f Colostography, 200 high-pressure distal, 190, 209 Colostomy anorectal malformations and, 194 cloaca repair and, 210 descending, 194 advantages of, 204 diverting, 186 following cloaca repair, 216-218 leveling, 168 loop, 194 disadvantages of, 204 prolapse and, 194 repair following, 188-190 transverse, disadvantages of, 204 Coma, following splenectomy, 300 Common channel cloaca repair and, 206, 207f, 210-214 size of, 206 Computed tomography (CT) hepatic tumor diagnosed with, 374 neuroblastoma diagnosed with, 356, 357f sacrococcygeal teratoma diagnosed with, 366 Wilms tumor diagnosed with, 340 Congenital cystic adenomatoid malformation, 120, 130 description of, 122 diagnosis of, 122 of left lower lobe, 122 radiograph of, 123f Congenital diaphragmatic hernia, 83-96 anesthesia for, 85 description of, 83 diagnosis of, 84 incidence of, 83 incision for, 86, 87f reduction of, 86 resuscitation and, 84 thoracoscopic repair of, 89-91 Congenital heart disease in child, 2 neuroblastoma associated with, 356 Congenital lobar emphysema, 120, 130 description of, 120-122 radiograph of, 121f Congestive heart failure patent ductus arteriosus and, 113-114 pulmonary sequestration indicated by, 122 Constipation, Hirschsprung disease associated with, 151-152

Index   383 Contrast enema distal intestinal obstructive syndrome diagnosed with, 148 Hirschsprung disease diagnosis and, 151-152 meconium ileus diagnosed with, 144 meconium plug syndrome diagnosed with, 149 Costal cartilage displacement of, 104 excision of, 109f perichondrial elevation and, 108 perichondrium of, 112 resection of, 108 Cough, gastroesophageal reflux indicated by, 266-268 Cricopharyngeus, colonic interposition and, 66 Cristopathologic disorders, 177 Crohn disease, distal intestinal obstructive syndrome and, 148 Crura, anti-reflux procedures and, 275f Cruroplasty, anti-reflux procedures and, 275f Cryptorchidism, 316-327 physical examination for, 316, 317f Cyst branchial, 34, 38 choledochal (See Choledochal cyst) nonparasitic, 293 parasitic, 293 splenic, 293 thyroglossal duct, 28-33 Cystic duct, 376 type III atresia of, 220 Cystic fibrosis absence of gallbladder and, 143 meconium ileus associated with, 143, 148 meconium plug syndrome and, 149

D Dartos muscle, 322, 324 Dartos pouch, 332, 333f neonatal testicular torsion and, 332 Death, following splenectomy, 300 Decannulation, 24 Dehydration, emesis as cause of, 253-254 Denys-Drash syndrome, Wilms tumor and, 342, 342t Diaphragm description of, 83 development of, 83 eventration of, 83-96 anesthesia for, 85, 93 repair of, 86, 87f sutures in repair of, 94, 95f Diaphragmatic crus, anti-reflux procedures and, 273f DiGeorge syndrome, 356 DIOS. See Distal intestinal obstructive syndrome Disseminated intravascular coagulation, following splenectomy, 300 Distal intestinal obstructive syndrome, 148-149 Doppler examination, vein patency determined with, 4 Down syndrome, 192, 201 Doxorubicin, 346-348, 350 Drainage cannula, 15-16 Ductus arteriosus, 113. See also Patent ductus arteriosus dissection of, 116 ligation of, 116, 117f Ductus deferens, 329f Duhamel procedure, 152, 159, 161-176 Dumbbell tumor, 360 Duodenal atresia, 132 anorectal malformations associated with, 186 esophageal atresia associated with, 132 Duodenotomy, transverse, 134, 135f Duodenum dilated, 133f kocherization of, 72 mobilization of, 141f obstruction of, 132-136 laparoscopic approach for, 135f radiograph of, 133f retroperitoneal, 138

E EA. See Esophageal atresia Echinococcus, 293 Echocardiography, Wilms tumor diagnosis with, 340 ECLS. See Extracorporeal life support ECMO. See Extracorporeal membrane oxygenation Eisenmenger physiology, patent ductus arteriosus associated with, 114 Embryonal carcinoma, 366 Emesis gastroesophageal reflux indicated by, 266-268 hypertrophic pyloric stenosis indicated by, 253-254 paradoxical aciduria caused by, 253-254 Emphysema, congenital lobar, 120, 130 Endarteritis, patent ductus arteriosus associated with, 114 Endodermal sinus tumor, 366 Endoscopic retrograde cholangiopancreatography, 234, 238 Endoscopy, cloaca diagnosis and, 209 Endotracheal intubation for cervical esophagostomy, 54 tracheoesophageal fistula repair and, 59-60 Enolase, neuron-specific, 355 Enterocolitis colostomy prior to transanal pull-through and, 159 following transanal pull-through, 158 Hirschsprung disease associated with, 151-152 signs of, 184 treatment of, 184 Enterostomy needle, 144 open, 144 Enterotomy meconium removal and, 146 meconium removal with, 149 Roux limb and, 284, 285f tracheoesophageal fistula repair and, 62, 63f Epididymis, 329f Epididymis torsion, scrotal pain caused by, 328t Epiglottis, 29f Epithelioid hemangioendothelioma, 374 Erb’s palsy, eventration of diaphragm and, 91 ERCP. See Endoscopic retrograde cholangiopancreatography Esophageal atresia, 44-52 anastomosis for, 50 anesthesia for, 46 anorectal malformations associated with, 186 duodenal atresia associated with, 132 esophageal replacement for, 76 in child, 2 in neonate, 44 incision for repair of, 48, 49f radiograph of, 45f repair of, 46 thoracoscopic repair of, 59-64 anesthesia for, 59-60 port positioning for, 60, 61f with tracheoesophageal fistula, radiograph of, 45f Esophageal hiatus, 271f Esophageal sphincter, colonic interposition and, 66 Esophagitis, gastroesophageal reflux indicated by, 266-268 Esophagocolostomy, 70 Esophagogastric junction, 72 Esophagostomy, 46 cervical, 54-58, 76, 82 anesthesia for, 54 stoma creation for, 56, 57f Esophagram, tracheoesophageal fistula repair and, 64 Esophagus anti-reflux procedures and, 275f atresia of, 44-52 dissection of, 58, 68 identification of, 56 length of, 46, 47f mobilization of, 48 replacement of, 66-75 anesthesia for, 68, 71, 78 colonic interposition and, 66-70 gastric tube pull-up for, 76-82 view of, 45f, 55f

Extracorporeal life support, 14 for child, 22-23 neonatal, 16-20 Extracorporeal membrane oxygenation, congenital diaphragmatic hernia and, 84 Extracorporeal membrane oxygenation cannulation, 14-26

F Failure to thrive, gastroesophageal reflux indicated by, 266-268 Falciform ligament, 376 Fallopian tube inguinal hernia repair and, 308 vaginal switch maneuver and, 216 Fecal biopsy, Hirschsprung disease diagnosis and, 151 Fecaloma, 174 Feeding tube placement of, in tracheoesophageal fistula repair, 64, 65f transanastomotic, 136, 137f Feingold syndrome, 132 Female anorectal malformation in, 185, 188-190, 189f, 194 repair of, 201-202 imperforate anus in, 190 inguinal hernia repair in, 308 Fentanyl, cannulation anesthesia with, 14 Fertility, cryptorchidism and, 326 Fever choledochal cyst associated with, 232 enterocolitis indicated by, 184 Fibrosing colonopathy, distal intestinal obstructive syndrome and, 148 Fistula branchial, 34, 38 perineal ( See Perineal fistula) pharyngeal, 36 rectal imperforate anus and, 194 rectobladder colostomy for, 194 rectobladder neck ( See Rectobladder neck fistula) rectourethral (See Rectourethral fistula) rectourinary, 190 side-hole, 58 small bowel, distal intestinal obstructive syndrome and, 148 tracheoesophageal ( See Tracheoesophageal fistula) Fistulous tract, 38, 39f, 40 dissection of, 39f Focal nodular hyperplasia, 374 Foker technique, 52 Foramen cecum, 29f Foramen of Bochdalek, 83 Foramen of Winslow, 376, 377f Fowler-Stephens first-stage orchiopexy, 320, 321f Frazier tip sucker, 6, 7f

G Gallbladder, 376 absence of, 143 biliary atresia indicated by, 222 Gallstones, hemolysis and, 292 Ganglion cell, Hirschsprung disease and, 177 Ganglioneuroblastoma, 363 Ganglioneuroma, 363 Gastrectomy, vertical sleeve, 279 Gastric banding, 279 Gastric pouch, creation of, in bariatric surgery, 284, 285f Gastric transposition, 71-74 mortality rate of, 74 partial, 74 Gastric tube pull-up, 76-82 anesthesia for, 78 Gastric volvulus, 92 Gastroesophageal junction, 72, 76, 81f

384   Index Gastroesophageal reflux, 266-268 diagnosis of, 266 esophageal replacement and, 70 Gastrografin, 144, 150 Gastrointestinal contrast study, 286 Gastrointestinal leak, bariatric surgery and, 286 Gastrointestinal tract, evaluation of, colonic interposition and, 66 Gastrojejunostomy, 284 Gastroplasty, 279 Gastroschisis, 242-252 definition of, 242 example of, 243f Gastrosplenic ligament, 290 Gastrostomy, site of, 69f GEJ. See Gastroesophageal junction Germ cell tumor, 364, 366 Gianturco coil, 114 Growth retardation, gastroesophageal reflux indicated by, 266-268

H Haemophilus influenzae, 28 Haller index, 97, 104, 105f Heart disease, in child, 2 Heineke-Mikulicz pyloroplasty, 70 Hemangioendothelioma, 374 Hematocolpos, 208 Hematologic disorders, 292 Hematometra, 208 Hemidiaphragm, elevated, 91 Hemihypertrophy, Wilms tumor and, 342 Hemiscrotum, 330, 331f in neonate, 330 Hemiuterus, 216, 217f Hemivagina blood supply to, 216 hydrocolpos drainage and, 210 vaginal switch maneuver and, 216 Hemolysis, gallstone formation and, 292 Hemolytic anemia, 299 Heparin, 280, 299 following cannulation, 14 vessel dissection and, 17 Hepatic duct, 378, 379f choledochal cyst dissection and, 236 type II atresia of, 220 type III atresia of, 220 Hepatic tumor, 374-380 incision for, 376, 377f surgery for, 376-378 Hepaticojejunostomy, 236, 237f stenosis of, 239 Hepatobiliary scintigraphy, 234 Hepatoblastoma, 374-376 Hepatomegaly, biliary atresia indicated by, 220-222 Hereditary spherocytosis, 292 Hernia bilateral, 303 congenital diaphragmatic ( See Congenital diaphragmatic hernia) hiatal, example of, 273f inguinal, 303-315 cryptorchidism associated with, 316 hydrocele and, 312 incidence of, 303 laparoscopic repair of, 308, 309f repair of, 304-308, 305f, 307f, 309f scrotal pain caused by, 328t ventral, 248, 249f delayed repair of, 250 Hernia sac, 90, 304, 306, 307f cryptorchidism and, 316 ligation of, 310, 320 spermatic cord separated from, 320, 321f HIDA. See Hepatobiliary scintigraphy Hirschsprung disease, 151-184 definition of, 177 diagnosis of, 151-152, 177

Hirschsprung disease (Continued) enterocolitis and, 158 incidence of, 177 long-segment, 159, 178 management of, 178 meconium plug syndrome and, 149 rectosigmoid, 180 total colonic, 178 Histamine-2 blocker, 50, 52 Hodgkin’s lymphoma, 293 Homovanillic acid, 355 Horner syndrome, 360 HPS. See Hypertrophic pyloric stenosis Human chorionic gonadotropin, 316 HVA. See Homovanillic acid Hydration, following pyloromyotomy, 264 Hydrocele, 303-315 communicating, 310 definition of, 310 inguinal hernia and, 312 noncommunicating, 310 of tunica vaginalis, 310 repair of, 311f, 313f scrotal pain caused by, 328t Hydrocolpos, 206-208 bilateral, vaginal switch maneuver and, 216 drainage of, 210 Hydronephrosis, 186 anorectal malformation mortality and, 204 cloaca associated with, 208 Hyoid bone, 28, 29f palpation of, 29f thyroglossal duct cyst and, 28 Hyperbilirubinemia biliary atresia indicated by, 220 liver transplant for, 230 Hypertension, Wilms tumor and, 342 Hypertrophic pyloric stenosis, 253-254 diagnosis of, 254 ultrasound of, 255f Hypoalbuminemia, liver transplant for, 230 Hypochloremia, hypertrophic pyloric stenosis and, 254-256 Hypokalemia emesis as cause of, 253-254 hypertrophic pyloric stenosis and, 254-256 Hypopharynx, view of, 41f Hypoplasia, radial, 46, 47f Hypothermia, patent ductus arteriosus repair and, 114

I Immune thrombocytopenic purpura, 292 Imperforate anus, 185-205 in female, 190 low, 190 urogenital defects associated with, 186 with rectovaginal fistula, 202 Imperforate anus without fistula, 192, 194, 201 in female, 185 in male, 185 Incision for anorectal malformation, 196 for biliary atresia repair, 224, 225f for cryptorchidism repair, 320-324 for hepatic tumor, 377f for nephrectomy, 342-344 for Nuss procedure, 98, 99f in perichondrium, 109f Indomethacin, ductal closure with, 114 Infant anti-reflux procedures for, 266-268 bowel preparation for, 162 congestive heart failure in, patent ductus arteriosus indicated by, 113-114 endorectal dissection in, 164, 165f endoscopy for, cloaca diagnosis and, 209 gastroschisis in, 242 Hirschsprung disease in, 178 lung anomalies in, 120

Infant (Continued) omphalocele in, 244 passage of meconium in, 143-144 premature ductus ligation for, 118 hernia repair in, 310 Hirschsprung disease in, 152 inguinal hernia in, 303-304 patent ductus arteriosus in, 113-116 pulmonary hypertension in, 83 pulmonary hypoplasia in, 83 respiratory distress in, 120 sacrococcygeal teratoma in, 366 venoarterial bypass for, 23 volvulus in, 140 Infection, post-splenectomy, 300 Inferior vena cava, Wilms tumor invasion of, 346 Intestinal atresia, gastroschisis repair and, 252 Intestinal bypass, 279 Intestine anomaly of, eventration of diaphragm associated with, 92 blind pouch of, 188 ganglionic, 161-162 malrotation of, 138-142, 139f mesentery of, 138, 139f obstruction of, Hirschsprung disease associated with, 151-152 rotation of, 139f Intrahepatic lithiasis, choledochal cyst repair and, 239 Intussusception distal intestinal obstructive syndrome and, 148 following Wilms tumor removal, 352 ITP. See Immune thrombocytopenic purpura IVC. See Inferior vena cava

J Jaundice biliary atresia indicated by, 220-222 choledochal cyst associated with, 232-234 Jejunojejunostomy, 282, 283f Jejunum bariatric surgery and, 282 blood supply of, 279

K Kasai portoenterostomy, 224, 226-228 Kidney absent, 186 contralateral, Wilms tumor and, 351 function of, Wilms tumor and, 340 horseshoe, 339 large Wilms tumor compression of, 349f removal of, 344 resection around, 349f solitary, 339 Wilms tumor and, 346-348 Wilms tumor and, 339 Kidney, ureter, and bladder examination, 144 Kimura esophageal lengthening procedure, 54 Kimura patch, 178, 184 Kocher maneuver, 80 KUB. See Kidney, ureter, and bladder examination

L Lactate dehydrogenase, 355 Ladd bands, 140, 141f Ladd procedure, 138-142 Laparoscopic-assisted endorectal pull-through, 168 surgeon positioning for, 164 Laparoscopy cryptorchidism repair with, 319 for abdominal tumor, 361f Laparotomy, cloaca repair with, 209, 214 Leveling biopsy, 154

Index   385 Lidocaine cannulation anesthesia with, 16 vasospasm treated with, 8, 10 Ligament falciform, 376 gastrosplenic, 290 inguinal, 304 phrenoesophageal, 68 Poupart, 304 splenorenal, 290 Ligament of Treitz, 138, 139f Ligamentum teres, 376 Liver anomaly in hilum of, 239-240 biliary atresia and, 224, 225f biopsy of, biliary atresia diagnosis and, 222 herniated, 250, 251f reduction of, 86 resection of, 376 right lobe of, 379f rotation of, 377f segments of, 374, 375f transplant of, 230 choledochal cyst treatment and, 238 tumor of, 374 Liver failure, Hirschsprung disease and, 184 Lobectomy hepatic, 238 left lower, 124-126, 127f pulmonary, 124 thoracoscopic, 126-128 Loop gastric bypass, 279 Lorenz introducer, 100, 101f, 103 Lung anomalies of, 120-130 congenital diaphragmatic hernia and, 83 ventilation of, 128 Wilms tumor in, 340, 342, 343f Lung disease, patent ductus arteriosus associated with, 118 Lymph node, sampling of Wilms tumor and, 351 Wilms tumor removal and, 344-346, 347f

M Magnetic resonance imaging (MRI) hepatic tumor diagnosed with, 374 neuroblastoma diagnosed with, 356 sacrococcygeal teratoma diagnosed with, 366, 367f Wilms tumor diagnosis with, 340 Male anorectal malformation in, 185, 187f, 190-192 inguinal hernia repair in, 304-308, 305f perineal fistula in, 190 rectourethral fistula in, 202 Malnutrition, colostomy prior to transanal pull-through and, 159 Malrotation definition of, 138 Ladd procedure for, 138-142 Marcaine, 268 Marginal artery of Drummond, 67f Meconium passage of, 143-144 presence of, 188 removal of, with catheter, 145f Meconium disease, 143-150 Meconium disease of prematurity, 150 Meconium ileus, 143-148 complex, bowel resection for, 146 complicated, 143-144 cystic fibrosis and, 148 definition of, 143 with perforation, 143-144 with volvulus, 143-144 Meconium ileus equivalent, 148 Meconium plug syndrome, 149 Meconium pseudocyst, 146 Megarectosigmoid, 194

Megasigmoid, 194 Meissner plexus, colon and, 161 Mesenchymal hamartoma, 374 Mesenteric defect, closure of, in bariatric surgery, 282, 283f Mesenteric vessels, 215f Mesentery bariatric surgery and, 282 staple placement in, in bariatric surgery, 282, 283f Mesocolon, bariatric surgery and, 282 Metabolic acidosis, 186 anorectal malformation mortality and, 204 cloaca associated with, 208 Metabolic alkalosis, emesis as cause of, 253-254 Metal, allergy to, 103 Methylprednisolone, 230 Müllerian anomaly, 208, 219 Müllerian structures, 214 Muscle levator rectobladder neck fistula and, 192 rectourethral fistula and, 192 pectoral, chest wall deformity open repair and, 106, 107f platysma, 32 sternocleidomastoid, branchial anomaly and, 34 Muscle complex anal canal and, 162 rectourethral fistula and, 192 Myotomy, tracheoesophageal fistula repair and, 52, 53f

N N-acetylcysteine, 144, 146, 148 Nasogastric tube, placement of, in gastric tube pull-up, 80, 81f Neck lesion of, 34-36 vascular access through, 6-12, 7f Neomycin, rectal washout and, 162 Neonatal adrenal hemorrhage, neuroblastoma and, 356 Neonate anorectal malformation in, 186 mortality of, 204 bilious emesis in, malrotation associated with, 138 cloaca in, 206-208, 219 complicated meconium ileus in, 144 congenital diaphragmatic hernia in, 83 endorectal dissection in, 164, 165f esophageal atresia in, 44 extracorporeal life support for, 16-20 female, anorectal malformation in, 189f hemiscrotum in, 330 Hirschsprung disease in, 178 liver in, 86 lung anomalies in, 120 male, anorectal malformation in, 186-188, 187f patent ductus arteriosus in, 118 pulmonary sequestration in, 122 rectal washout for, 162 sacrococcygeal teratoma in, 364 testicular torsion in, 328, 332 surgical intervention for, 330 thoracoscopic repair of congenital diaphragmatic hernia in, 89-91 tracheoesophageal fistula in, 44 vascular access for, 15 venous cannulation of, 25 volvulus in, 138 Nephrectomy incision for, 342-344 intussusception following, 352, 353f Nerve laryngeal, esophageal replacement and, 79 phrenic, 115f, 117f eventration of diaphragm and injury to, 91-92 injury to, eventration of diaphragm and, 92 patent ductus arteriosus repair and, 113

Nerve (Continued) recurrent laryngeal, 55f, 117f vagus, 44, 45f, 115f, 117f patent ductus arteriosus repair and, 113 tracheoesophageal fistula repair and, 48, 49f Nervi erigentes, 172 Neuroblastoma, 355-363 cervical, 360 imaging for, 356 incidence of, 355 staging of, 356 thoracic, 360 Wilms tumor and, 352 Neurocristopathy, 177 Neuromuscular blockade, 16 Nissen fundoplication, 266 laparoscopic, 266, 267f Non-Hodgkin’s lymphoma, 293 Nonsteroidal antiinflammatory drug (NSAID), ductal closure with, 114 Nuss procedure chest wall deformity treated with, 97-103 incision in, 98-100, 99f thoracostomy in, 98-102

O Obesity bariatric surgery for, 279 in adolescent, 280 Omentum, 72 bariatric surgery and, 281 open pyloromyotomy and, 260-262 Omphalocele, 242-252 definition of, 242 example of, 243f repair of fascial substitute for, 250, 251f skin closure and, 248, 249f Ondine’s curse, 177, 356 Open endorectal pull-through, 161-168, 167f dissection for, 164, 165f positioning for, 162, 163f Open endorectal technique, 161-176 OPSI. See Overwhelming post-splenectomy infection Opsoclonus-myoclonus syndrome, 356 Orchiectomy, 324 Orchiopexy, 318, 323f, 325f Fowler-Stephens, 320, 321f microvascular, 322 neonatal testicular torsion and, 332 suturing in, 325f Organ bag, 242, 243f, 244 Ovary, inguinal hernia repair and, 308 Overwhelming post-splenectomy infection, 300 Oxygenation, extracorporeal membrane, 14-26

P Pancreas annular, 132, 133f, 134 choledochal cyst dissection and, 235f, 236 windsock deformity of, 132, 133f Pancreatic duct, 133f Pao2. See Partial pressure of arterial oxygen Papaverine, vein dilation with, 17 Paradoxical aciduria, emesis as cause of, 253-254 Parietal peritoneum, 290 Parotid gland, branchial anomaly and, 34 Partial pressure of arterial oxygen, 84 Partial pressure of carbon dioxide, 84 Patent ductus arteriosus, 113-119. See also Ductus arteriosus indications for ductal closure in, 113-114 ligation for, 117f retraction for, 115f Pco2. See Partial pressure of carbon dioxide PDA. See Patent ductus arteriosus

386   Index Pectus bar, 98, 99f shape of, 100, 103 stabilization of, 100, 101f titanium, 103 Pectus excavatum cardiopulmonary effects of, 104 chest wall deformity and, 97 distinctions of, 97 severe, 97 severity of, 104 Pelvis, tumor in, 360 Penicillin, following splenectomy, 300 Pentalogy of Cantrell, 244 Pepper syndrome, 363 Peptic esophagitis, 74 Perichondrium, 108 incision of, 109f of costal cartilage, 112 Perineal fistula in female, 185, 194 repair of, 201 in male, 185, 190 incidence of, 185 incision for, 196 urogenital defect and, 186 Perineal orifice, 188 single, 206-208, 207f Periosteum, chest wall deformity open repair and, 106, 107f Peritoneum, 308 Peritonitis causes of, 143 choledochal cyst associated with, 234 meconium, 146 Pharyngeal fistula, 36 Pharyngocolostomy, 70 Pharynx, branchial anomaly and, 34 Pheochromocytoma, neuroblastoma and, 356 Phrenic nerve. See Nerve, phrenic Pinch-off sign, 13 Pneumonia aspiration, gastroesophageal reflux indicated by, 266-268 pulmonary sequestration indicated by, 122 Pneumoperitoneum, 180-182 Pneumothorax congenital diaphragmatic hernia repair and, 90 establishment of, 60, 61f Polyhydramnios, maternal, 143 Portal tract, edema of, 223f Prader orchidometer, 322 Predisposition syndrome, Wilms tumor and, 342, 342t Processus vaginalis, 303, 320 in neonatal testicular torsion, 332, 333f Pseudocyst intra-abdominal, 208 meconium, 146 splenic, 293 Pull-through Duhamel, 172-176 laparoscopic-assisted endorectal, 168-172 open endorectal, 161-168, 167f Soave, 161-168, 167f surgeon positioning for, 164 Swenson, 177-184 Pulmonary embolism, bariatric surgery and, 286 Pulmonary function, Wilms tumor and, 342 Pulmonary hilum, 118 Pulmonary hypertension infant, 83-84 patent ductus arteriosus associated with, 114 Pulmonary hypoplasia, infant, 83 Pulmonary lobectomy, 124 Pulmonary metastasis, Wilms tumor and, 352 Pulmonary sequestration, 120, 130 description of, 122 radiograph of, 123f Pyloromyotomy esophageal replacement and, 80 feeding management following, 262

Pyloromyotomy (Continued) laparoscopic, 253-265 instrument insertion in, 256, 257f patient positioning for, 256, 257f technique for, 258-262, 259f open, 253-265 incisions for, 258-260, 259f, 261f technique for, 260-262, 261f, 263f pain control following, 264 Pyloroplasty duodenum kocherization and, 72 esophageal replacement and, 80, 81f gastric emptying and, 72 Heineke-Mikulicz, 70 Pylorus hypertrophied, 253, 258, 259f identification of, 253 Pyocolpos, 208 Pyriform fossa sinus, 34 cauterization of, 40 Pyriform fossa sinus tract, 38-40 Pyriform sinus anomalies of, 42 cauterization of, 38, 40 visualization of, 41f

R Radiograph babygram, 44, 46 congenital cystic adenomatoid malformation, 123f of congenital lobar emphysema, 121f pulmonary sequestration, 123f Radiography, sacrococcygeal teratoma diagnosed with, 366 Radiolabeled metaiodobenzylguanidine scintigraphy, neuroblastoma diagnosed with, 356 Ranitidine, 286 Rectal atresia, 192 in female, 185 in male, 185 with stenosis, 201 Rectal fistula, imperforate anus and, 194 Rectobladder fistula, colostomy for, 194 Rectobladder neck fistula, 192, 200 in male, 185 incidence of, 185 rectum and, 196 Rectoperineal fistula complications associated with, 204 in female, 190 Rectourethral bulbar fistula, 196 Rectourethral fistula, 190, 192, 196-198 colostomy for, 194 in male, 185, 202 incidence of, 185 placement of rectum in, 199f sutures in repair of, 199f Rectourinary fistula, 190 Rectovaginal fistula, cloaca and, 206 Rectovesical fistula, urogenital defect and, 186 Rectovestibular fistula in female, 188 incidence of, 185 Rectum cloaca repair and, 210, 212-214 dissection of, 198 mobilization of, 200 placement of, in rectourethral fistula repair, 199f rectourethral fistula and, 192, 197f sacrococcygeal teratoma surgery and, 368, 369f tailoring of, 198 tapering of, 198 vagina common wall with, 201 vagina replacement and, 214, 215f Renal disease, Wilms tumor and, 340 Renal function anorectal malformation mortality and, 204 Wilms tumor and, 340

Respiratory distress cystic adenomatoid malformation indicated by, 122 following splenectomy, 300 pulmonary sequestration indicated by, 122 Respiratory failure, venovenous bypass for, 15 Retrosternal strut, 110, 111f Reverse gastric tube transposition, 71 Rib, chest wall deformity open repair and, 108 Rocuronium, cannulation anesthesia with, 14 Roux limb construction of, 279 enterotomy and, 284, 285f Roux-en-Y gastric bypass, 279 anastomosis patency and, 287f closure following, 286, 287f Roux-en-Y hepaticojejunostomy, 236, 237f Roux-en-Y jejunal limb, 228, 229f Roux-en-Y portoenterostomy, 224 RYGBP. See Roux-en-Y gastric bypass

S Sacral ratio, 186 Sacral region, sacrococcygeal teratoma and, 364, 365f Sacrococcygeal joint, sacrococcygeal teratoma surgery and, 368-370, 369f Sacrococcygeal teratoma, 364-373 classification of, 364, 365f closure following surgery for, 370, 371f example of, 365f frequency of malignancy of, 366t incidence of, 364 patient positioning for, 367f Sacrum, defects of, 186 Santulli stoma, 146, 147f Sarcoma, hepatic, 374 Scarpa’s fascia, 324 Scrotum acute, differential diagnosis of, 328, 328t closure of incision of, in orchiopexy, 324, 325f hydrocele and, 310 orchiopexy and, 322 pain in, 328 SCT. See Sacrococcygeal teratoma Seldinger technique, 4 Septum transversum, 83 Shimada system, 355 Shock, following splenectomy, 300 Shoe-shine maneuver, anti-reflux procedures and, 274, 275f Sickle cell disease, 292, 299 Silo, gastroschisis repair and, 244, 245f Simpson-Golabi-Behmel syndrome, Wilms tumor and, 342, 342t Sinus branchial, 34 pyriform, cauterization of, 40 pyriform fossa, 34 Sistrunk procedure, 30, 32 Small bowel fistula, distal intestinal obstructive syndrome and, 148 Soave pull-through, 167f laparoscopic-assisted endorectal, 168 Soave technique, 161-176 dissection for, 164, 165f Spectrin, 292 Spermatic cord, 329f hernia sac separated from, 320, 321f length of, 320 twisting of, 328 Spermatic cord torsion, scrotal pain caused by, 328t Spherocytosis, 299 hereditary, 292 Sphincter cloaca repair and, 210, 211f mechanism of, 203f Spinal cord, compression of, 360 Spine defects of, 186 tumor of, 360

Index   387 Spleen cystic lesions in, 299 cysts in, 293 location of, 291f position of, 290 pseudocysts in, 293 reduction of, 86 surgery on, causes of, 290 Splenectomy, 290-300 laparoscopic, 294-296 bag device used in, 297f cauterization in, 295f patient positioning in, 294, 295f puncture sites for, 294, 295f open, 298 partial, 293, 299 Splenia syndrome, in child, 2 Splenomegaly, non-Hodgkin’s lymphoma and, 293 Splenorenal ligament, 290 Sporadic hemihypertrophy, Wilms tumor and, 342t Staphylococcus aureus, 28 Staphylococcus epidermidis, 28 Stapler, in Duhamel pull-through anastomosis, 174, 175f Sternocleidomastoid muscle, branchial anomaly and, 34 Sternum displacement of, 104 elevation of, chest wall deformity repair and, 100 perforation of, 109f retrosternal strut and, 108, 111f Stoma calibration of, 58 creation of, for cervical esophagostomy, 56, 57f Stomach blood supply to, 76, 79 esophageal atresia and, 76 evaluation of, colonic interposition and, 66 fundus of, 79 mobilization of, 72, 73f omentum separated from, 72, 73f position of, 72, 73f transposition of, 71-74 twisting of, 80 Subcutaneous reservoir, 4, 5f Sudden infant death syndrome, gastroesophageal reflux associated with, 266-268 Surgery abdominal hemostasis in, 358, 359f retractor used in, 359f bariatric ( See Bariatric surgery) bilateral nephron-sparing, 348 for weight loss, 280 Surgical anatomy, vascular access and, 2 Swallowing, thyroglossal duct cyst and, 28, 29f Swenson procedure, 172 Swenson pull-through, 177-184 stricture formation and, 182 suture placement in, 181f transanal technique for, 180, 181f

T Taenia coli, loss of, 164 TEF. See Tracheoesophageal fistula Teratoma, sacrococcygeal. See Sacrococcygeal teratoma Testicle appearance of, in testicular torsion, 328, 329f hydrocele and, 310 incomplete fixation of, 328 Testicular implant, 326 Testicular necrosis, 330, 331f Testicular torsion, 328-336 absence of vascular flow associated with, 330, 331f contralateral fixation and, 334 in child, 334 in neonate, 332 intravaginal, 328, 329f midline scrotal incision for repair of, 334, 335f recurrent, 334

Testis appendix, scrotal pain caused by, 328t ascending, 326 blood supply to, 316 contralateral, in testicular torsion repair, 334 fixation of, in testicular torsion repair, 332, 333f, 334, 335f ischemia of, 322 measurement of, 322, 323f mobilization of, 322 minimization of, 322 movement of, in orchiopexy, 322, 323f placement of, in orchiopexy, 324, 325f remnant, 320 retractile, 326 undescended, 316 visualization of, 321f Testosterone, functioning testicle and, 316 Thalassemia, 292 Thoracic duct, view of, 55f Thoracoscope, chest wall deformity repair and, 98-100 Thoracoscopic lobectomy, 126-128 Thoracotomy eventration of diaphragm repair and, 93 interspace, 114, 115f left, 80 muscle-sparing, 114 posterolateral, 124 incision for, 124, 125f Thrombocytopenia, 292 Thrombocytosis, splenectomy and, 299 Thyroglossal duct cyst, 28-33, 29f anesthesia for, 30 diagnosis of, 28 incision for, 30, 31f irrigation of, 32, 32f positioning for, 30, 31f Thyroid, 29f lobectomy of, 40 Thyroid cartilage, resection of, 32 Thyroiditis, pyriform fossa sinus and, 34 Tongue, thyroglossal duct cyst and, 28 Trachea dissection of esophagus away from, 58 view of, 45f, 55f Tracheal ring, resection of, 32 Tracheoesophageal fistula, 44-52 anastomosis for, 50, 51f anesthesia for, 46 dissection of, 62 division and ligation of, 62, 63f division of, 48 identification of, 63f in neonate, 44 incision for repair of, 48, 49f positioning for repair of, 48, 49f repair of, 46 suture placement and, 64, 65f thoracoscopic repair of, 59-64 anesthesia for, 59-60 port positioning for, 60, 61f Transanal pull-through, 151-160, 155f, 157f anesthesia for, 152 causes of death following, 158 incision for, 155f landmarks for, 151 Trauma, scrotal pain caused by, 328t Triangular cord sign, 222, 223f Trimethoprim-sulfamethoxazole, 230 Trisomy 13, 244 Trisomy 13-15, 244 Trisomy 14, 244 Trisomy 16-18, 244 Trisomy 18, 177 Trisomy 21, 132, 177 Tumor abdominal, 358-360 hemostasis in surgery for, 358, 359f laparoscopy for, 361f cervical, 360 dumbbell, 360

Tumor (Continued) endodermal sinus, 366 germ cell, 364, 366 hepatic ( See Hepatic tumor) in vena cava, removal of, 347f intraureteral, 344, 345f pelvic, 360 scrotal pain caused by, 328t spinal, 360 resection of, 361f splenectomy and, 293 thoracic, 360 yolk sac, 366 Tumor thrombus, 346 Tunica albuginea, 324 Tunica vaginalis, 320, 329f hydrocele of, 310-315 neonatal testicular torsion and, 329f

U Ultrasound hepatic tumor diagnosed with, 374 hypertrophic pyloric stenosis diagnosed with, 254, 255f neuroblastoma diagnosed with, 356 sacrococcygeal teratoma diagnosed with, 366 vein patency determined with, 4 Wilms tumor diagnosed with, 340 Umbilical vessels, omphalocele repair and, 246-250, 247f Umbilicoplasty, 246 Umbilicus creation of, 246, 247f incision in, 261f Ureteral stent, 348 Urethra bulbar, 192, 198 cauterization of, 212, 213f cloaca repair and, 212-214, 213f near-atresia of, 208 prostatic, 192 rectourethral fistula and, 192 Urinary tract, cloaca repair and, 212-214 Urogenital defect, 186 Urogenital sinus, cloaca repair and, 212 cloaca repair and, 213f Urosepsis, 186 anorectal malformation mortality and, 204 cloaca associated with, 208 Ursodeoxycholic acid, 230, 238 Ursodiol, 286

V VA. See Venoarterial bypass Vagina cloaca repair and, 210, 212-214, 213f distended, 206-208 incomplete separation of, 201 rectum common wall with, 201 replacement of, 214-216, 215f, 217f Vaginal fistula, 202 Vaginal switch maneuver, 216, 217f Vagus nerve. See Nerve, vagus Vanillymandelic acid, 355 Vas deferens, 200, 306, 308 Vascular access, 2-13 arterial, 12 postoperative care following, 12 through neck, 6-12, 7f Vasoactive intestinal peptide-mediated severe secretory syndrome, 356 Vein ascending lumbar, 11f azygous cauterization of, 60 division of, 60, 61f tracheoesophageal fistula and, 59 view of, 45f

388   Index Vein (Continued) cannulation of, 24-25 central, 2 division of, cannulation and, 25 external jugular, catheterization of, 6-8 femoral, 2, 11f catheterization of, 10-12, 11f hepatic, 380 accessory, 378 illustration of, 379f liver segments and, 374 tumor thrombus above, 347f inferior vena cava, 3f innominate, 3f internal jugular, catheterization of, 8-9 intrathoracic, perforation of, 26 jugular, 2 mesenteric, 290 portal, 374, 375f, 378 anatomic variation in, 380 bifurcation of, 226, 227f position and patency of, 4 proximal, cannulation and, 25 pulmonary, 127f renal palpation of, 344, 345f removal of Wilms tumor in, 347f vessel loops placed around, 358, 359f Wilms tumor invasion of, 346 saphenous, 11f catheterization of, 10 splenic, 290, 291f, 296 ligation of, 297f subclavian, 2 catheterization of, 9-10, 11f Vena cava catheterization of, 8 hepatic vein and, 374 inferior, 379f removal of Wilms tumor in, 347f

Vena cava (Continued) superior, 3f catheterization of, 8 Wilms tumor extension into, 340, 341f Wilms tumor invasion of, 346 Venoarterial bypass, 23-24 carotid artery and, 22 description of, 15 extracorporeal support with, 15 for child, 22 for neonate, 15 open technique for, 16 technique for, 16 venovenous bypass compared to, 15 Venoarterial cannulation anesthesia for, 16 technique for, 16-18 Venotomy, cannulation and, 17-18 Venovenous bypass, 22 description of, 15 double-lumen cannula for, 16 extracorporeal support with, 15 for child, 22 in adolescent, 23 jugular vein and, 22 respiratory failure treated with, 15 technique for, 16 venoarterial bypass compared to, 15 Venovenous cannulation anesthesia for, 16 semi-open technique for, 20 technique for, 16-18 Ventilation, single lung, 124, 128 Vertebra, missing, 186 Vertical sleeve gastrectomy, 279 Vesicostomy, near-atresia of urethra and, 208 Vesicoureteric reflux, 186 Vestibular fistula in female, 185, 188, 189f, 190, 194, 195f repair of, 201 incision for, 196

Vincristine, 346-348 Viscera, reduction of, congenital diaphragmatic hernia repair and, 90 VMA. See Vanillymandelic acid Volvulus, 138, 140 diagnosis of, 138 meconium ileus and, 143-144 recurrent, 142 Vomiting, hypertrophic pyloric stenosis indicated by, 253-254 VV. See Venovenous bypass

W WAGR syndrome, Wilms tumor and, 342t Washout colonic, 172-173 for laparoscopic-assisted endorectal pull-through, 168 rectal, 162, 172-173 Weight loss, surgery for, 280 Wheezing, gastroesophageal reflux indicated by, 266-268 Wilms tumor, 339-354 bilateral, 346-348 biopsy of, 351 imaging of, 340 intussusception following removal of, 352, 353f kidney parenchyma compressed by, 348, 349f neuroblastoma and, 352, 356 predisposition syndromes and, 342t primary, resection of, 350 removal of, 344 rupture and spill of, 351 staging of, 350, 350t Windsock deformity, 132, 133f, 136

Y Yolk sac tumor, 366