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Cloacal Malformations Within the field of pediatric colorectal and pelvic reconstruction, the most complex anatomic problem a pediatric surgeon can face is that of a cloacal malformation. The urinary tract, gynecologic system, and distal bowel all fuse into a single common channel which must be surgically reconstructed. The care of patients with a cloaca is daunting, and the spectrum of anatomic variations very wide. In this book about cloaca, the authors have brought organization to the complex, providing basic principles of care, from fetal and newborn management to the reconstruction of bowel, gynecologic systems, and urinary tracts as well as the care of patients after surgery to puberty and beyond. This is a comprehensive account of all elements of care for the cloaca patient. It includes the preoperative evaluation and surgical planning, the anorectal malformation (ARM) index, surgical reconstruction, urological evaluation and longterm care. Also covered are the gynecologic evaluation and intervention, neurological implications, bowel management and the long-term management of patients with cloacal malformations. Case studies provide valuable insights into a wide range of clinical scenarios, never previously categorized so comprehensively. These studies enable the reader to understand and gain experience from detailed descriptions. Fully illustrated, in color, and with further resources available online, this is a unique resource for all clinicians within the pediatric team caring for patients with cloacal malformations. In addition, specialists across numerous fields, including colorectal surgery, urology, gynecology, and gastrointestinal motility, as well as neonatology, orthopedics, neurosurgery, anesthesia, radiology, psychology, social work, nutrition, and nursing, will find this practical clinical text and its online resources to be an invaluable resource when dealing with the complex needs of the cloaca patient.

Cloacal Malformations Case Studies

Marc A. Levitt, MD

Chief, Division of Colorectal and Pelvic Reconstruction Children’s National Hospital Professor of Surgery and Pediatrics The George Washington University School of Medicine Washington, DC, USA Associate Editors

Tamador Yassen Al-Shamaileh Andrea T. Badillo Allison C. Mayhew Teresa L. Russell Briony K. Varda Richard J. Wood

Cover image: Mandy Root-Thompson First edition published 2024 by CRC Press 2385 NW Executive Center Drive, Suite 320, Boca Raton, FL 33431 and by CRC Press 4 Park Square, Milton Park, Abingdon, Oxon, OX14 4RN CRC Press is an imprint of Taylor & Francis Group, LLC © 2024 Marc A. Levitt This book contains information obtained from authentic and highly regarded sources. While all reasonable efforts have been made to publish reliable data and information, neither the author[s] nor the publisher can accept any legal responsibility or liability for any errors or omissions that may be made. The publishers wish to make clear that any views or opinions expressed in this book by individual editors, authors or contributors are personal to them and do not necessarily reflect the views/opinions of the publishers. The information or guidance contained in this book is intended for use by medical, scientific or health-care professionals and is provided strictly as a supplement to the medical or other professional’s own judgement, their knowledge of the patient’s medical history, relevant manufacturer’s instructions and the appropriate best practice guidelines. Because of the rapid advances in medical science, any information or advice on dosages, procedures or diagnoses should be independently verified. The reader is strongly urged to consult the relevant national drug formulary and the drug companies’ and device or material manufacturers’ printed instructions, and their websites, before administering or utilizing any of the drugs, devices or materials mentioned in this book. This book does not indicate whether a particular treatment is appropriate or suitable for a particular individual. Ultimately it is the sole responsibility of the medical professional to make his or her own professional judgements, so as to advise and treat patients appropriately. The authors and publishers have also attempted to trace the copyright holders of all material reproduced in this publication and apologize to copyright holders if permission to publish in this form has not been obtained. If any copyright material has not been acknowledged please write and let us know so we may rectify in any future reprint. Except as permitted under U.S. Copyright Law, no part of this book may be reprinted, reproduced, transmitted, or utilized in any form by any electronic, mechanical, or other means, now known or hereafter invented, including photocopying, microfilming, and recording, or in any information storage or retrieval system, without written permission from the publishers. For permission to photocopy or use material electronically from this work, access www.copyright.com or contact the Copyright ­C learance Center, Inc. (CCC), 222 Rosewood Drive, Danvers, MA 01923, 978–750–8400. For works that are not available on CCC please contact [email protected] Trademark notice: Product or corporate names may be trademarks or registered trademarks and are used only for identification and explanation without intent to infringe. ISBN: 978-1-032-23053-5 (hbk) ISBN: 978-1-032-21547-1 (pbk) ISBN: 978-1-003-27544-2 (ebk) DOI: 10.1201/9781003275442 Typeset in Palatino LT Std by Apex CoVantage, LLC Access the Instructor and Student Resources: www.routledge.com/cw/levitt

Contents

Prefaceix Acknowledgementsxi About the Author Associate Editors Chapter Authors, Medical Illustrators, and Contributors Books by the Author Ode to the Colorectal Trainee Online Resources Abbreviations Used in This Book

xiii xv xvii xxi xxiii xxv xxvii

PART 1 OVERVIEW

1

1 History of cloacal malformations Tamador Yassen Al-Shamaileh and Marc A. Levitt 2 Overview of cloacal malformations Tamador Yassen Al-Shamaileh, Andrea T. Badillo, and Marc A. Levitt 3 Preoperative evaluation of cloacal malformations and surgical planning Tamador Yassen Al-Shamaileh, Allison C. Mayhew, Briony K. Varda, and Marc A. Levitt 4 The anorectal malformations (ARM) index Andrea T. Badillo, Briony K. Varda, and Marc A. Levitt 5 Surgical reconstruction in cloacal malformations Tamador Yassen Al-Shamaileh, Allison C. Mayhew, Briony K. Varda, and Marc A. Levitt 6 Urological evaluation and long-term management in cloacal malformations Briony K. Varda 7 Gynecologic evaluation and intervention in cloacal malformations Allison C. Mayhew and Veronica Gomez-Lobo 8 Neurosurgical implications of cloacal malformations John Myseros 9 Bowel management of patients with cloacal malformations Kristina Booth, Stephanie Vyrostek, Katie Worst, and Marc A. Levitt 10 Long-term outcomes of patients with cloacal malformations Tamador Yassen Al-Shamaileh, Briony K. Varda, and Marc A. Levitt

2 7 25 30 36 63 77 87 92 97

PART 2  CASE STUDIES

105

Case 1

106

A patient with prenatal diagnosis of a cloacal malformation Andrea T. Badillo, Tamador Yassen Al-Shamaileh, Briony K. Varda, and Marc A. Levitt

v

Contents

SECTION 1  TOTAL UROGENITAL MOBILIZATION (TUM) Case 2

Case 3 Case 4

117

 otal urogenital mobilization (TUM) in a patient with cloacal malformation T and borderline common channel and urethral lengths 118 Laura Tiusaba, Briony K. Varda, Tamador Yassen Al-Shamaileh, Christina Ho, and Marc A. Levitt A patient with a cloacal malformation, a short common channel, and a high rectum 131 Teresa L. Russell, Christina Feng, Tamador Yassen Al-Shamaileh, and Marc A. Levitt A patient with cloacal malformation, short common channel, and tethered cord 141 Laura Tiusaba, Briony K. Varda, Tamador Yassen Al-Shamaileh, Christina Ho, and Marc A. Levitt

SECTION 2  UROGENITAL (UG) SEPARATION 

149

Case 5

150

Case 6 Case 7 Case 8 Case 9

Urogenital separation vs. TUM Teresa L. Russell, Tamador Yassen Al-Shamaileh, and Marc A. Levitt A patient with cloacal malformation, a long common channel, and a high rectum Tamador Yassen Al-Shamaileh, Elizaveta Bokova, and Marc A. Levitt A patient with cloacal malformation, a long common channel, and long urethra Shimon E. Jacobs, Christina Feng, Tamador Yassen Al-Shamaileh, and Marc A. Levitt A patient with cloacal malformation, a short common channel, and short urethra Richard J. Wood Laparoscopic approach to a patient with cloacal malformation Prathima Nandivada, Erin Mcnamara, and Belinda Dickie

SECTION 3  SPECIAL CASES

162 177 184 189 195

Case 10 A patient with a slightly hypospadic urethral meatus: (Cloaca type 1) 196 Tamador Yassen Al-Shamaileh and Marc A. Levitt Case 11 A patient with cloacal malformation, a rectourethral fistula, and distal vagina atresia 204 Teresa L. Russell, Tamador Yassen Al-Shamaileh, Allison C. Mayhew, and Marc A. Levitt Case 12 A patient with a cloacal malformation with urethral atresia 215 Tamador Yassen Al-Shamaileh, Elizabeth Bokova, and Marc A. Levitt Case 13 A patient with a posterior cloacal variant: Urogenital sinus with perineal fistula 223 Tamador Yassen Al-Shamaileh and Marc A. Levitt Case 14 A patient with cloacal malformation, Mu``llerian anomalies, and vesicoureteral reflux230 Athanasios Tyraskis, Laura Tiusaba, Shimon E. Jacobs, Briony K. Varda, and Marc A. Levitt Case 15 A patient with cloacal exstrophy 239 Laura Tiusaba and Caitlin Smith Case 16 Bowel management in a patient with a cloacal malformation 245 Kristina Booth, Stephanie Vyrostek, Katie Worst, and Marc A. Levitt

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Contents

APPENDICES Available online only, please visit www.Routledge.Com/cw/levitt Appendix A Treatment timeline for patients with cloacal malformations Tamador Yassen Al-Shamaileh, Bhupender Yadav, and Marc A. Levitt Appendix B Cloacagram Tamador Yassen Al-Shamaileh and Marc A. Levitt Appendix C Examination under anesthesia sample operative note Tamador Yassen Al-Shamaileh and Marc A. Levitt Appendix D Electrical stimulation of the anus Tamador Yassen Al-Shamaileh and Marc A. Levitt Appendix E Operating room preparation Tamador Yassen Al-Shamaileh and Marc A. Levitt Appendix F Postoperative care instructions  Tamador Yassen Al-Shamaileh and Marc A. Levitt Appendix G Applied anatomy Teresa L. Russell, Tamador Yassen Al-Shamaileh, and Marc A. Levitt Appendix H New gynecologic anatomic diagrams Allison C. Mayhew and Marc A. Levitt Index255

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Preface

Patients with anorectal malformations (ARM), Hirschsprung disease (HD), fecal incontinence from a variety of causes, colonic motility disorders, and a myriad of other conditions comprise the field of pediatric colorectal and pelvic reconstruction. In this group, the most complex anatomic problem a surgeon can face is that of a cloacal malformation, in which the urinary tract, gynecologic system, and distal bowel all fuse into a single common channel which must be surgically reconstructed. The care of such patients with a cloaca is daunting, and the spectrum of anatomic variations very wide. In this book about cloaca, we have attempted to bring some organization to the complexity, providing basic principles of care, from fetal and newborn management to the reconstruction to bowel and urinary care after surgery to puberty and beyond. Such patients require input from specialists across numerous fields throughout their lives, which may include colorectal surgery, urology, gynecology, and gastrointestinal (GI) motility, as well as orthopedics, neurosurgery, anesthesia, pathology, radiology, psychology, social work, nutrition, and nursing. Having met many parents with newborns diagnosed with colorectal problems, it is clear to me that no parent ever expects that their child would have a problem with urination, gynecologic function, or stooling. These physiologic capacities are taken for granted, and when told that their child has a problem in these areas, they are usually shocked that something like this could even occur. When discussing with these parents the necessity of surgery to correct their child’s anatomy, none of them focus on the surgical technique and elegance of the reconstruction, which are the primary surgical goals. All parents are instead most concerned about whether the surgeon will create an anatomy that will allow their child to remain clean and dry for urine and stool and have expected gynecologic function. As surgeons, it is our moral obligation to remember this. We always need to understand the outcome the family desires. As proud as we are of our surgical skills, it is the functional outcome that matters most to our patients and their families. In my 30 years thus far as a surgeon working in this field and toiling away with the goal of improving my patients’ quality of life, I have developed and learned from others the key aspects of care of the patient with a cloacal malformation. I, together with the wonderful collaborators I have worked with on this book, and with the help of the incredible team with whom I work every day, have attempted to capture these principles, in a case-oriented approach. That approach, which I learned from my father who wrote books about neurology in the same format, I find is most capably absorbed and retained by the reader. Our goal is to help other caregivers understand the daily struggle we experience in our work caring for these complex patients, and to teach to the readers of this book the skills and tricks to achieve positive results. I want to take this moment to thank my family, my wife, Shary, my children, Sam, Raquel, and Jess, my parents, Eva (z”l) and Larry (Mom and Dad), my siblings, Adam and Lora, my acquired siblings, Sharone, Steph, Harley (z”l), and Becky, and my in-laws, Sandy (z”l) and Abe, my cousin Andrea for your tireless support, devotion, and love. I would have achieved very little without you.

Marc A. Levitt, MD

ix

Acknowledgements

The first time I  saw a cloaca operation was in 1992 as a final year medical student, at the age of 25, on an elective with Alberto Peña prompted by my interest in pediatric surgery. I vividly remember thinking there would be no way I could ever develop the understanding or the skills to repair such a complex malformation. Since then, after caring for approximately 1000 such patients, patterns emerged, and it was my desire to write down the reproducible concepts I had learned that the idea for this book emerged. Many individuals over the past 30 years helped me understand this rare condition, and to those individuals I wish to say “thank you”. I would like to first acknowledge Alberto Peña for his influence on my education caring for complex problems over thousands of colorectal cases we helped together, as his student, trainee, and partner, as well as Hardy Hendren with whom I had many fascinating discussions on his frequent collaborations with Alberto. I would like to additionally acknowledge the reconstructive surgeons with whom I have worked over the years, Curt Sheldon, Shumyle Alam, Lesley Breech, Jason Frischer, Belinda Dickie, Rama Jayanthi, Daniel Dajusta, Christina Ching, Molly Fuchs, Geri Hewitt, Kate McCracken, Richard Wood (one of this book’s editors), Hans Pohl, Briony Varda (editor), Christina Ho, Veronica Gomez-Lobo, Allison Mayhew (editor), Andrea Badillo (editor), and Christina Feng with whom I have cared for many patients and who helped develop protocols that have de-mystified this condition. To the radiologists who revolutionized the anatomic understanding of cloaca, without whom a definitive well-planned reconstruction would not be possible – thank you to Steven Kraus, Daniel Podberesky, Neil Johnson, John Racadio, Manish Patel, Eva Rubio, Greg Bates, Brent Adler, Ben Thompson, Bhupender Yadav, Ranjith Vellody, and Karun Sharma. I want to thank the trainees who inspire those around them daily and make me a better surgeon, because they force regular analysis of our ideas and push the lines of progress. Tamador Yassen Al-Shamaileh (editor), a former trainee, and our wonderful research coordinator, Teresa Russell (editor), both worked tirelessly to get the concept of this book onto paper. And, finally, it must be stated that the care of these complex patients is managed collaboratively with our incredible nursing and advanced practice nursing colleagues, in the operating room, on the in-patient ward, and in the out-patient clinic, with the goals to maximize urologic, gynecologic, and colorectal outcomes, and without whom no surgeon in the reconstructive world would accomplish very much at all.

Marc A. Levitt, MD

xi

About the Author

Marc A. Levitt  focuses his career on enhancing the care of children with colorectal and pelvic reconstructive needs. He has cared for children from all 50 of the United States and from over 75 countries and has performed more than 15,000 pediatric colorectal procedures. He has written four textbooks and this is his fifth, as well as over 300 scientific articles in this subject area. His work extends to educating students, surgeons, other medical colleagues, and nurses, as well as helping to develop integrated centers throughout the world to ensure that all children have access to quality colorectal care. He is actively engaged in helping patients, their doctors, and nurses in resource-limited locations through the organization Colorectal Team Overseas, www.ctoverseas.org. He is the Chief of the Division of Colorectal and Pelvic Reconstruction at Children’s National Hospital, in Washington DC, a uniquely integrated team of pediatric colorectal surgeons, urologists, gynecologists, gastroenterologists, nurses, and advanced practice providers, and is a Professor of Surgery and Pediatrics at The George Washington University School of Medicine.

xiii

Associate Editors

Tamador Yassen Al-Shamaileh, MD Pediatric Surgery Consultant King Hussein Cancer Center Assistant Professor, Faculty of Medicine Mutah University Amman, Jordan Andrea T. Badillo, MD Associate Chief Division of Colorectal and Pelvic Reconstruction Pediatric Surgeon Divisions of Colorectal and Pelvic Reconstruction and General and Thoracic Surgery Program Director Pediatric Colorectal Fellowship Department of Surgery Children’s National Hospital, Washington, DC, USA Associate Professor of Surgery and Pediatrics The George Washington University School of Medicine Allison C. Mayhew, MD Director, Pediatric and Adolescent Gynecology Pediatric and Adolescent Gynecologist Division of Colorectal and Pelvic Reconstruction Department of Surgery Children’s National Hospital, Washington, DC, USA

Assistant Professor of Gynecology The George Washington University School of Medicine Teresa L. Russell, MS Senior Clinical Research Coordinator Divisions of Urology and Colorectal and Pelvic Reconstruction Department of Surgery Children’s National Hospital, Washington, DC, USA Briony K. Varda, MD, MPH Co-Director, Children’s National Spina Bifida Program, Divisions of Urology and Colorectal and Pelvic Reconstruction Department of Surgery Children’s National Hospital, Washington, DC, USA Assistant Professor of Urology and Pediatrics The George Washington University School of Medicine Richard J. Wood, MD Chief, Department of Pediatric Colorectal and Pelvic Reconstructive Surgery Nationwide Children’s Hospital, Columbus, OH, USA Professor of Surgery The Ohio State University, Columbus, OH, USA

xv

Chapter Authors, Medical Illustrators, and Contributors

Tamador Yassen Al-Shamaileh, MD Associate Editor Part 1, Chapters 1–3, 5, 6, and 10. Part 2, Cases 1–7 and 10–13. Appendices A–G. Andrea T. Badillo, MD Associate Editor Part 1, Chapters 2 and 4. Part 2, Case 1. Elizaveta Bokova, MD Research Fellow, Division of Colorectal and Pelvic Reconstruction, Department of Surgery Children’s National Hospital, Washington, DC, USA Part 2, Cases 6 and 12. Kristina M. Booth, FNP Advanced Practice Nurse Center for Colorectal and Pelvic Reconstruction Nationwide Children’s Hospital, Columbus, OH, USA Part 1, Chapter 9. Part 2, Case 16. Belinda Dickie, MD, PhD Director, Colorectal and Pelvic Malformation Center Co-Director, Congenital Diaphragmatic Hernia Program Assistant Professor, Assistant in Surgery Department of Surgery Boston Children’s Hospital and Harvard Medical School Boston, MA, USA Part 2, Case 9. Christina Feng, MD Surgeon, Divisions of Colorectal and Pelvic Reconstruction and General and Thoracic Surgery Department of Surgery Children’s National Hospital, Washington, DC, USA Part 2, Case 7.

Veronica Gomez-Lobo, MD Director, Pediatric and Adolescent Gynecology Director, Positive Reevaluation of Urogenital Differences (PROUD) Service Pediatric and Adolescent Gynecologist Division of Colorectal and Pelvic Reconstruction Department of Surgery Children’s National Hospital, Washington, DC, USA Director, Pediatric and Adolescent Gynecology National Institute of Child Health and Human Development Bethesda, MD, USA Part 1, Chapter 7. Shimon E. Jacobs, MD Colorectal Surgical Fellow, Division of Colorectal and Pelvic Reconstruction, Department of Surgery Children’s National Hospital, Washington, DC, USA Part 2, Cases 7 and 14. Marc A. Levitt, MD Author Part 1, Chapters 1–6, 9, and 10. Part 2, Cases 1–7, 10–14, and 16. Appendices A–G. Allison C. Mayhew, MD Associate Editor Part 1, Chapters 3, 5, and 7. Cases 11 and 14. Appendix H. Erin McNamara, MD, MPH Associate Program Director and Co-Director Spina Bifida Center Associate Director, Colorectal and Pelvic Malformation Center Instructor of Surgery Boston Children’s Hospital and Harvard Medical School Boston, MA, USA Part 2, Case 9.

xvii

Chapter Authors, Medical Illustrators, and Contributors

John Myseros, MD Vice Chief, Department of Neurosurgery Children’s National Hospital, Washington, DC, USA Part 1, Chapter 8. Prathima Nandivada, MD Surgeon, Department of Surgery Assistant Professor of Surgery Boston Children’s Hospital and Harvard Medical School Boston, MA, USA Part 2, Case 9. Teresa L. Russell, MS Associate Editor Part 2, Cases 3, 5, and 11. Appendix G. Caitlin Smith, MD Co-Director, Reconstructive Pelvic Medicine Program Associate Program Director, General Surgery Residency Assistant Professor Seattle Children’s Hospital and the University of Washington Seattle, WA, USA Part 2, Case 14. Laura Tiusaba, MD Colorectal Surgical Fellow, Division of Colorectal and Pelvic Reconstruction, Department of Surgery Children’s National Hospital, Washington, DC, USA Part 2, Cases 2, 4, 14, and 15. Athanasios Tyraskis, MD Colorectal Surgical Fellow, Division of Colorectal and Pelvic Reconstruction, Department of Surgery Children’s National Hospital, Washington, DC, USA Part 2, Case 14. Briony K. Varda, MD, MPH Associate Editor Part 1, Chapters 3–6, 10. Part 2, Cases 2 and 4. Stephanie Vyrostek, RN, BSN Program Manager Center for Colorectal and Pelvic Reconstruction Nationwide Children’s Hospital, Columbus, OH Part 1, Chapter 9. Part 2, Case 16.

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Richard J. Wood, MD Associate Editor Part 2, Case 8. Katherine Worst, CPNP-AC Advanced Practice Clinical Manager, Division of Colorectal and Pelvic Reconstruction Department of Surgery Children’s National Hospital, Washington, DC, USA Part 1, Chapter 9. Part 2, Case 16.

MEDICAL ILLUSTRATORS Tamador Yassen Al-Shamaileh, MD Associate Editor Mandy Root-Thompson, MS Medical Illustrator and Owner MedDrawStudio LLC. Columbus, OH, USA

CONTRIBUTORS Julie M. Choueiki, MSN, BSN, RN Senior Program Manager, Division of Colorectal and Pelvic Reconstruction, Department of Surgery Children’s National Hospital, Washington, DC, USA Christina Ho, MD Co-Director, Spina Bifida Program Department of Urology Children’s National Hospital, Washington, DC, USA Meghan Mesa, BSN, RN, CPNRN, CPN Nurse Clinical Coordinator, Division of Colorectal and Pelvic Reconstruction, Department of Surgery Children’s National Hospital, Washington, DC, USA Hans Pohl, MD Chief, Division of Urology Co-Director, Spina Bifida Program Pediatric Urologist, Department of Urology Children’s National Hospital, Washington, DC, USA

Chapter Authors, Medical Illustrators, and Contributors

Arianna Sidoti, BS Surgery Resident, Department of Surgery Weill Cornell Medicine New York, NY, USA Ranjith Vellody, MD Director, Department of Interventional Radiology Interventional Radiologist, Department of Interventional Radiology Children’s National Hospital, Washington, DC, USA

Hendt Versteegh, MD, PhD Pediatric Surgery Resident, Department of Pediatric Surgery Erasmus MC, Sophia Children’s Hospital Rotterdam, Netherlands Bhupender Yadav, MD Interventional Radiologist Department of Interventional Radiology Children’s National Hospital, Washington, DC, USA

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Books by the Author

Pediatric Colorectal and Pelvic Surgery: Case Studies Victoria A. Lane, Richard J. Wood, Carlos Reck-Burneo, and Marc A. Levitt ISBN: 9781138031777 (2017, CRC Press) Fetal Incontinence and Constipation in Children: Case Studies Onnalisa Nash, Julie M. Choueiki, and Marc A. Levitt ISBN: 9780367151614 (2020, CRC Press) Pediatric Colorectal and Pelvic Reconstructive Surgery Alejandra Vilanova-Sanchez and Marc A. Levitt ISBN: 9780367136475 (2020, CRC Press) Pediatric Colorectal Surgery: Tips & Tricks Marc A. Levitt ISBN: 9780367693176 (2022, CRC Press)

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Ode to the Colorectal Trainee

In the realm of colorectal a surgical quest, Where our trainees put skills to the test, Colorectal fellows, steadfast and strong, In their capable hands, hope thrives along.

Another, a master of surgical skill, Their hands, a testament, with a resolute will, With precision and finesse, they pave the way, Inspiring others, day after day.

With hands steady and minds ever keen, They embark on a journey, unseen, A symphony of collaboration, in perfect accord, A dance of skill, where lives are restored.

Together they strive, these surgical peers, Banishing doubts and conquering fears, In the realm of colorectal, they stand tall, Writing stories of healing, beyond recall.

The most complex conditions they face, With knowledge and precision, they embrace, Learning from masters, seeking wisdom’s gain, Embracing challenges, pushing through the strain.

And the nurses, their supporters and more, Their dedication unwavering, their spirits soar, They’re the biggest fans, or critics true, No matter what, their love shines through.

From pre-dawn rounds to the twilight’s fall, They answer the summons, duty’s call, Patiently learning, embracing the trade, Beneath their wings, new surgeons are made.

Oh, colorectal training, a sacred domain, Where dedication and knowledge attain, The surgeons’ comrades, by their side, With each incision, they help to guide.

One, the beacon of unwavering light, Their knowledge, a beacon shining so bright, With wisdom and expertise, they impart, Guiding the way, with a caring heart.

May our trainee’s journeys unfold, With tales of triumph, untold, As they leave their mark on patient and peer, Their legacy resonating, crystal clear. Written by Meghan Mesa, BSN, RN, CPN

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Online Resources

APPENDICES AVAILABLE ONLINE ONLY, PLEASE VISIT WWW.ROUTLEDGE.COM/CW/LEVITT Appendix A

Treatment timeline for patients with cloacal malformations Tamador Yassen Al-Shamaileh and Marc A. Levitt

Appendix B

Cloacagram Tamador Yassen Al-Shamaileh and Marc A. Levitt

Appendix C

Examination under anesthesia sample operative note Tamador Yassen Al-Shamaileh and Marc A. Levitt

Appendix D

Electrical stimulation of the anus Tamador Yassen Al-Shamaileh and Marc A. Levitt

Appendix E

Operating room preparation Tamador Yassen Al-Shamaileh and Marc A. Levitt

Appendix F

Postoperative care instructions Tamador Yassen Al-Shamaileh and Marc A. Levitt

Appendix G

Applied anatomy Teresa L. Russell, Tamador Yassen Al-Shamaileh, and Marc A. Levitt

Appendix H

New gynecologic anatomic diagrams Allison C. Mayhew and Marc A. Levitt

xxv

Abbreviations Used in This Book

3D three-dimensional ACE antegrade continence enema AFI amniotic fluid index AP anterior-posterior APGAR appearance, pulse, grimace, activity, and respiration ARM anorectal malformation(s) ARSM  American Society for Reproductive Medicine ASD atrial septal defect BID “bis in die” (twice daily) CAH congenital adrenal hyperplasia CC common channel CIC clean intermittent catheterization CKD chronic kidney disease CPAP continuous positive airway pressure CSF cerebrospinal fluid CT computerized tomography CXR chest radiography DMSA dimercapto succinic acid DSD disorders of sexual development EBC expected bladder capacity for age ECHO echocardiogram EF esophageal fistula eGFR estimated glomerular filtration rate ESRD end-stage renal disease EUA examination under anesthesia G2P1 “gravida 2, para 1” GA gestational age GFR glomerular filtration rate GI gastrointestinal GU genitourinary HD Hirschsprung disease HM Heineke–Mikulicz (anoplasty) IC ileocolic artery IMA inferior mesenteric artery IMV inferior mesenteric vein IR interventional radiology LUQ left upper quadrant

MACE Malone antegrade colonic enema MAG3 mercaptoacetyltriglycine MC middle colic artery MRI magnetic resonance imaging NCA nurse-controlled analgesia NG nasogastric NICU neonatal intensive care unit NPO “nil per os” (nothing by mouth) NS normal saline OEIS omphalocele exstrophy imperforate anus spinal defect complex OR operating room PACU post-anesthesia care unit PC pubococcygeal PCA patient-controlled analgesia PCPLC Pediatric Colorectal and Pelvic Learning Consortium PDS polydioxanone sutures PICU pediatric intensive care unit POD postoperative day PRN “Pro re nata” PSARP posterior sagittal anorectoplasty PSARVUP posterior sagittal anorectovaginourethroplasty PVR post-void residuals RC right colic artery RLQ right lower quadrant RUQ acute right upper quadrant RVF rectovestibular fistula SCD sequential compression device SFU Society of Fetal Urology SIS small intestinal submucosa SMA superior mesenteric artery SP suprapubic (tube) SSI surgical site infection TEF tracheoesophageal fistula TID “ter in die” (three times a day) TUM total urogenital mobilization UDS urodynamic study testing

xxvii

Abbreviations Used in This Book

UG urogenital UGS urogenital separation UPJO ureteropelvic junction obstruction US ultrasound UTI urinary tract infection UVJO ureterovesical junction obstruction

xxviii

VACTERL  vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities VCUG voiding cystourethrogram VSD ventricular septal defect VUR vesicoureteral reflux

PART

1

OVERVIEW

1

History of cloacal malformations TAMADOR YASSEN AL-SHAMAILEH AND MARC A. LEVITT

HISTORICAL MANAGEMENT OF A PATIENT WITH AN ANORECTAL MALFORMATION (ARM) Two thousand years ago, in the Babylonian Talmud, a reference is made to a baby born with imperforate anus. For the treatment of this baby, it is recommended to use a barley branch to scratch open the membrane covering the expected anal location (Figure 1.1). Aristotle documented the first reports of people and animals with aberrant perineal anatomy. Dating from the 3rd century before the common era (BCE), treatment consisted of dilation of the fistula tract or simply incising a thin membrane, which indicates that those cases were a low type of anorectal malformation (ARM). It is clear that all other malformations were not survivable. In the 18th century a Scottish surgeon, Benjamin Bell, reported that in a newborn with no anal opening, a finger-guided dissection into the perineum should be carried out until the operator encountered feces. Later, Alexis Littre from France was the first to perform a stoma in children, which became a life-saving maneuver for patients with a complex ARM. Jean Amussat, in 1835, was the first to suture rectal wall to the skin, thus creating the first anoplasty on record. In 1930, Wangensteen and Rice described the radiographic invertogram as a method for determining the level of the distal rectal atresia, which with key modifications, is now an invaluable tool in the management of ARM patients. F. Douglas Stephens made a significant contribution to the care of children born with ARM by performing the first anatomic studies in human autopsies. In 1953, Stephens proposed using a sacral approach followed by an abdominoperineal operation. The key to his procedure 2

Translation of relevant section: “Abaye said: If there is no haluk for an infant, a hemmed rag should be brought, and the hem tied round at the bottom and doubled over at the top. Abaye also said: Mother told me, an infant whose anus is not visible should be rubbed with oil and stood in the sun, and where it shows transparent it should be torn crosswise with a barley grain, but not with a metal instrument, because that causes inflammation.” Figure 1.1  Reproduction of a section of the Babylonian Talmud: Tractate Shabbath. Folio 134a. Short translation (English) of section that references details and treatment of imperforate anus.

was preservation of the puborectalis sling, considered vital to maintaining fecal continence. Over the next 25 years, different surgical techniques were described, with the common effort at protecting the puborectalis sling. DOI: 10.1201/9781003275442-2

History of management of patients with cloacal malformations

A student of Stephens, Justin Kelly, brought this technique to Boston where he went for training. A  co-trainee, Alberto Peña, learned this technique and then, upon returning to Mexico in 1972, modified it further. In collaboration with Peter Devries, he lengthened the perineal incision to improve exposure, and in 1980, for the first time a posterior sagittal approach for the treatment of imperforate anus was performed. With this approach, improved visualization facilitated the safe separation of the rectum from adjacent structures. This advance opened the surgical world of pediatric colorectal subspecialties, where problems too high to reach from below, and too low to reach from above were now more solvable.

HISTORY OF MANAGEMENT OF PATIENTS WITH CLOACAL MALFORMATIONS The management of cloacal malformations has advanced significantly over time. Many cases previously described as rectovaginal fistulae were actually cloacal malformations. Historically, patients with cloacal malformations were left untreated or managed with a colostomy alone. In 1953, Paul Swenson first reported the full repair of a cloaca with the creation of three separate openings for the urethra, vagina, and rectum. In 1959, Malcolm Gough described 18 cases of cloaca, and suggested either a pull-through of the rectum or colostomy without repairing the vagina. In 1966, William Snyder reported on seven patients with cloacal malformations, three of whom died, and described that he was able to repair the vagina in only one case. No technical details about the operations were described. With more understanding of this malformation, a new form of cloaca was recognized with the opening located at the site of the rectum rather than the site of the urethra. This was first described in 1974 in an article published by Klugo, Fisher, and Retik from Boston, where they did only a vaginoplasty. In 1986, this type of cloaca was named a “posterior cloaca” by Alberto Peña. The contributions of W. Hardy Hendren from Boston in the 1970s, 1980s, and 1990s to the world of cloaca were monumental. His work formed the basis for the management of patients with cloacal malformations in the modern era, providing publications with intricate detail that were the first that described definitive reconstruction of this anomaly. Hendren described a loop colostomy in the

transverse colon, and intermittent catheterization of the bladder or urine-filled vagina until a definitive repair was performed. He recommended avoiding urinary diversion, with the exception of a simple temporary suprapubic (SP) cystostomy. He was an advocate for repair of the three systems (urologic, gynecologic, and colorectal) at one setting, arguing that preliminary abdominoperineal pullthrough of the colon without repair of the genitourinary (GU) aspect of the problem can lead to urinary tract stasis and serious obstructive uropathy. This concept was truly visionary and is reaffirmed in today’s management focused on ensuring reliable bladder emptying. He insisted on conducting a full endoscopic evaluation prior to the repair, in order to determine the anatomy of the malformation. This is now considered standard in the operative preparation for patients with cloacal malformations. He also recommended that the urinary tract be repaired first when there is obstruction or massive reflux to prevent upper tract deterioration. The abdominoperineal approach was widely used with the understanding that the levator muscle was the only muscle responsible for fecal continence, and that the external anal sphincter was rudimentary or had no role. This led to the use of a supralevator, sacrococcygeal approach for mobilization of the terminal bowel and transection of a fistula, if present, which allowed for the blunt creation of a small retro-urethral translevator space through which the bowel could be pulled to a perineal incision for the creation of an anus (Figure  1.2). This approach had multiple problems – limited exposure, risk of injury to the presacral plexus or urogenital (UG) structures, and a high risk of fecal incontinence. With use of the posterior sagittal anorectoplasty (PSARP), the importance of the external anal sphincter, and use of an electrical stimulator to mark it were highlighted. This approach was used for the first time in a patient with a cloaca in 1982, termed a posterior sagittal anorectovaginourethroplasty (PSARVUP) (Figure  1.3). The PSARVUP used a meticulous sharp dissection in the midline from the sacrum to the perineum. This new approach allowed for direct exposure to the complex visceral arrangements as well as the voluntary muscle relevant to urinary and fecal continence and provided a unique opportunity to correlate the anatomy to the functional results. The PSARVUP is now considered standard for patients with cloaca, and abdominal approaches are reserved for cases that are unable to be completed using a posterior sagittal incision. 3

History of cloacal malformations

Figure 1.2  The supralevator, sacrococcygeal approach for mobilization. Illustration (a) showing the anatomic view as seen through a sacral approach. The blind-ending terminal bowel is below the coccyx (not shown). A sound is in the urethra and a hemostat outlines the puborectalis sling looking down from above through the divided pubococcygeal fibers of the levator ani. (b) An empty Penrose drain is in the new external sphincter-puborectalis canal. (Illustrations adapted from Keisewetter, W. B. and Nixon, H. H. (1967). Reproduced with permission.)

Figure 1.3  Illustration showing posterior sagittal incision for a cloacal malformation, which runs from the coccyx to the perineum through the anal sphincter muscle fibers.

The common channel length gained importance over the years as a crucial determinant of the approach in treatment of patients with cloacal malformations. Hendren used a urogenital (UG) separation technique for all patients 4

with cloacal malformations, in which the vagina(s) was separated from the bladder neck and UG sinus. He emphasized a reconstruction of the urethra once the rectum and vagina were separated from it. A feared complication of this separation was the development of a vagino-urethral fistula in 10% of cases. Partial vaginal replacement with the distal colon was performed by Peña in 1987. This allowed for more favorable cosmetic and functional results for these patients. Later, he also promoted techniques such as complete vaginal replacement with small bowel, vaginal switch, and division of the megarectum longitudinally to create a neovagina and a neorectum to be used as options to reconstruct the vagina. In 1996, another revolutionary milestone was achieved, when Peña published a description of the total urogenital mobilization (TUM). This technique reduced operative time, but also preserved the blood supply to the urethra because there was no separation between the urethra and the vagina, instead the UG complex was mobilized as a unit. When Peña presented his paper “Total urogenital mobilization, an easier way to repair a cloaca” on this new technique in 1996, Hendren rose to the microphone, complimented Peña on the brilliant maneuver and offered only one key critique—he said: “I disagree with the title

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of your paper. Nothing about cloaca is easy!” Hendren had been given a preview of the technique on a visit by him to Schneider Children’s Hospital in New York where Peña worked. This book’s author was standing behind both of them as Peña drew diagrams about the idea and remembers the excitement in both surgeons’ eyes. The TUM was henceforth brought into favor for the repair of cloacal malformations; for patients with shorter common channels, this approach proved to be a success. For patients with long common channels, the TUM alone did not provide adequate mobility and therefore a “transabdominal TUM” was employed. This included a laparotomy with abdominal dissection followed by a pull-through. Unfortunately, this maneuver could result in pulling the bladder through the UG diaphragm, disrupting the normal support structures and leading to stress urinary incontinence. Moreover, when the complex could not be brought down adequately despite additional abdominal dissection the remaining option would be a UG separation of the previously mobilized complex. It was quickly observed that the urethra in particular could not withstand this degree of dissection, which caused urethral ischemia. Therefore, UG separation is best for longer common channels and TUM for shorter channels; the decision as to which procedure to perform can only be made with precise imaging of the cloaca prior to surgery. The experience applying TUM to a variety of channel lengths led to the publication of an algorithm for the surgical reconstruction of cloaca in 2010 anchored on common channel length. Specifically, patients with a common channel less than 3 cm should be repaired with a TUM via a posterior sagittal approach alone. Patients with a common channel between 3 and 5 cm should undergo TUM with or without a vaginal replacement. While patients with common channels greater than 5 cm require a UG separation wherein the vagina is dissected away from the common channel, the remaining defect is closed, and the common channel becomes the urethra. The latter may be further augmented by a laparotomy with additional dissection superiorly if needed. In 2018, the importance of urethral length – in addition to the length of the common channel – came into focus. This arose from a study establishing that the average urethral length in a series of female infants was 1.5 cm. The urethra in cloaca patients may be much shorter, and a TUM in a patient with a short urethra may be one cause for stress urinary incontinence postoperatively. The algorithm

for the surgical reconstruction of cloaca was thus modified to incorporate urethral length. Specifically, among patients with a 1–5 cm common channel, a urethral length >1.5 cm would indicate the TUM approach. Patients with a urethral