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CC BY 4.0 · European J Pediatr Surg Rep. 2023; 11(01): e1-e4
DOI: 10.1055/s-0043-1761206
Case Report

Cloacal Malformation with Associated Urethral Atresia

Tamador Al-Shamaileh
1   Department of General Surgery, King Hussein Cancer Center, Amman, Jordan
,
Laura Tiusaba 
2   Division of Colorectal and Pelvic Reconstruction, Department of Surgery, Children's National Hospital, Washington, District of Columbia, United States
,
Shimon Eric Jacobs 
2   Division of Colorectal and Pelvic Reconstruction, Department of Surgery, Children's National Hospital, Washington, District of Columbia, United States
,
Teresa Lynn Russell 
2   Division of Colorectal and Pelvic Reconstruction, Department of Surgery, Children's National Hospital, Washington, District of Columbia, United States
,
Elizaveta Bokova
2   Division of Colorectal and Pelvic Reconstruction, Department of Surgery, Children's National Hospital, Washington, District of Columbia, United States
,
Hans G. Pohl
3   Department of Urology, Children's National Hospital, Washington, District of Columbia, United States
,
Briony K. Varda
3   Department of Urology, Children's National Hospital, Washington, District of Columbia, United States
,
Christina Ho
3   Department of Urology, Children's National Hospital, Washington, District of Columbia, United States
,
Christina Feng
2   Division of Colorectal and Pelvic Reconstruction, Department of Surgery, Children's National Hospital, Washington, District of Columbia, United States
,
Andrea Badillo
2   Division of Colorectal and Pelvic Reconstruction, Department of Surgery, Children's National Hospital, Washington, District of Columbia, United States
,
Marc A. Levitt
2   Division of Colorectal and Pelvic Reconstruction, Department of Surgery, Children's National Hospital, Washington, District of Columbia, United States
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Abstract

Introduction Cloacal malformations comprise a heterogeneous group of anomalies that are considered the most complex anorectal malformations (ARMs) in females. Precise evaluation to identify the unique anatomy prior to reconstruction with collaboration between colorectal surgeons, urologists, and gynecologists is vital. Here, we present a rare anatomical variation in a patient with a cloacal malformation which affected operative and postoperative management.

Case description A 6-year-old female with cloaca who underwent colostomy, vaginostomy, and vesicostomy as a newborn presented for reconstruction. Her VACTERL workup was negative except for an atretic right kidney. Her ARM index included the cloaca, a normal spine, and sacrum with a lateral sacral ratio of 0.7, predicting good potential for bowel continence. Cystoscopy through the vesicostomy showed a small bladder with normal ureteral orifices, and a closed bladder neck, with no identifiable urethra. A cloacagram showed an atretic common channel, a single small vagina, and a rectum below the pubococcygeal line. The patient underwent a posterior sagittal anorectovaginourethroplasty, vaginal patch using rectum, rectoplasty, and perineal body reconstruction. The urethra was not amenable to reconstruction, so the vesicostomy was preserved and a future Mitrofanoff was planned.

Conclusion Urethral atresia is a rare and challenging finding in cloaca patients, and a vesicostomy is needed to drain urine in the newborn period. Preoperative examination under anesthesia, cystoscopy, vaginoscopy, and cloacagram are crucial to identify the precise anatomy and to plan accordingly.

Keywords

urethra atresia - cloaca - rectal patch


Publication History

Received: 26 May 2020

Accepted: 13 December 2020

Article published online:
07 February 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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