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 preoperative evaluation and surgical planning, the anorectal malformation (ARM) index, surgical reconstruction, urological evaluation and long-term 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 when dealing with the complex needs of the cloaca patient.


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 developing 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, and nurses and is a Professor of Surgery and Pediatrics at The George Washington University School of Medicine.


Associate Editors


Tamador Al-Shamaileh, MD

Pediatric Surgery Consultant

Assistant Professor, Department of General Surgery, Faculty of Medicine

Mu'tah University, Al-Karak, Jordan

University of Jordan, Amman, Jordan



Andrea T. Badillo, MD

Director, Division of Colorectal and Pelvic Reconstruction, Pediatric Surgeon,

Divisions of Colorectal and Pelvic Reconstruction and General and Thoracic Surgery, and 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



Part One: Overview

1. History of Cloacal Malformations

Tamador Al-Shamaileh, Marc A. Levitt

2. Overview of Cloacal Malformations

Tamador Al-Shamaileh, Andrea T. Badillo, Marc A. Levitt

3. Preoperative Evaluation and Surgical Planning

Tamador Al-Shamaileh, Allison C. Mayhew, Briony K. Varda, Marc A. Levitt

4. The ARM Index

Andrea T. Badillo, Briony K. Varda, Marc A. Levitt

5. Surgical Reconstruction

Tamador Al-Shamaileh, Allison C. Mayhew, Briony K. Varda, Marc A. Levitt

6. Urological Evaluation and Long-Term Management in Cloacal Malformations

Briony K. Varda, Tamador Al-Shamaileh, Marc A. Levitt

7. Gynecologic Evaluation and Intervention in Cloacal Malformations

Allison C. Mayhew

8. Neurosurgical Implications

John Myseros

9. Bowel Management

Kristina Booth, Stephanie Vyrostek, Katie Worst, Marc A. Levitt

10. Long-Term Outcomes of Patients with Cloacal Malformations

Tamador Al-Shamaileh, Briony K. Varda, Marc A. Levitt

Part Two: Case Studies

Case 1 A patient with a prenatal diagnosis of a cloacal malformation

Andrea T. Badillo, Tamador Al-Shamaileh, Briony K. Varda, Marc A. Levitt

Section 1 Total Urogenital Mobilization (TUM)

Case 2 TUM in a patient with a cloacal malformation and borderline common channel and urethral lengths

Laura Tiusaba, Briony K. Varda, Tamador Al-Shamaileh, Marc A. Levitt

Case 3 A patient with a cloacal malformation, a short channel, and a high rectum

Teresa L. Russell, Christina Feng, Tamador Al-Shamaileh, Marc A. Levitt

Case 4 A patient with a cloacal malformation, a short common channel, and a tethered cord

Laura Tiusaba, Briony K. Varda, Tamador Al-Shamaileh, Marc A. Levitt

Section 2 Urogenital (UG) Separation

Case 5 UG Separation vs. TUM in a patient with a borderline cloacal malformation who underwent a UG separation

Teresa L. Russell, Tamador Al-Shamaileh, Marc A. Levitt

Case 6 A patient with a cloacal malformation, a long common channel, and a high rectum

Tamador Al-Shamaileh, Elizaveta Bokova, Marc A. Levitt

Case 7 A patient with a cloacal malformation, a long common channel, and a long urethra

Shimon E. Jacobs, Christina Feng, Tamador Al-Shamaileh, Marc A. Levitt

Case 8 A patient with a cloacal malformation, a short common channel, and a short urethra

Richard J. Wood

Case 9 Laparoscopic approach to a patient with a cloacal malformation

Prathima Nandivada, Erin McNamara, Belinda Dickie

Section 3 Special Cases

Case 10 A patient with a cloacal malformation and a slightly hypospadic urethral meatus (Cloacal type 1)

Tamador Al-Shamaileh, Marc A. Levitt

Case 11 A patient with a cloacal malformation, a rectourethral fistula, and distal vaginal atresia

Teresa L. Russell, Tamador Al-Shamaileh, Allison C. Mayhew, Marc A. Levitt

Case 12 A patient with a cloacal malformation with urethral atresia

Tamador Al-Shamaileh, Elizaveta Bokova, Marc A. Levitt

Case 13 A patient with a posterior cloacal variant: a urogenital sinus with perineal fistula

Shimon E. Jacobs, Tamador Al-Shamaileh, Marc A. Levitt

Case 14 A patient with a cloacal malformation, Mullerian anomalies, and vesicoureteral reflux

Athanasios Tyraskis, Laura Tiusaba, Shimon Jacobs, Allison C. Mayhew, Marc A. Levitt

Case 15 A patient with cloacal exstrophy

Laura Tiusaba, Caitlin Smith, Briony K. Varda

Case 16 Bowel management in a patient with cloacal malformation

Kristina Booth, Stephanie Vyrostek, Katie Worst, Marc A. Levitt

Appendices

Appendix A Treatment Timeline for Patients with Cloacal Malformations

Tamador Al-Shamaileh, Marc A. Levitt

Appendix B Cloacagram

Tamador Al-Shamaileh, Marc A. Levitt

Appendix C Examination Under Anesthesia Sample Operative Note

Tamador Al-Shamaileh, Marc A. Levitt

Appendix D Electrical Stimulation of the Anus

Tamador Al-Shamaileh, Marc A. Levitt

Appendix E Operating Room Preparation

Tamador Al-Shamaileh, Marc A. Levitt

Appendix F Postoperative Care Instructions

Tamador Al-Shamaileh, Marc A. Levitt

Appendix G Applied Anatomy

Teresa L. Russell, Tamador Al-Shamaileh, Marc A. Levitt

Appendix H New Gynecologic Anatomic Diagrams

Allison C. Mayhew