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CC BY 4.0 · European J Pediatr Surg Rep. 2021; 09(01): e41-e45
DOI: 10.1055/s-0041-1728719
Case Report

A Novel Surgical Approach for the Management of Cloacal Exstrophy with a Giant Omphalocele

Caitlin A. Smith
1   Department of General and Thoracic Surgery, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, Washington, United States
,
Jeffrey R. Avansino
1   Department of General and Thoracic Surgery, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, Washington, United States
,
Paul Merguerian
2   Department of Urology, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, Washington, United States
,
Victoria Lane
3   Department of Surgery, The Great North Children's Hospital, Royal Victoria Infirmary, Newcastle, United Kingdom
,
Marc Levitt
4   Division of Colorectal and Pelvic Reconstructive Surgery, Children's National Hospital, Washington, District of Columbia, United States
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Abstract

Cloacal exstrophy is a rare malformation that presents as a lower midline abdominal wall defect which affects the gastrointestinal and genitourinary systems. The components of cloacal exstrophy characteristically include omphalocele, exstrophy of perineal structures, and imperforate anus. Most of these patients also have renal anomalies such as pelvic kidney, fused kidneys, or solitary kidneys. This congenital condition can also be associated with spinal issues, such as spinal dysraphism. When combined with spinal defects, it is referred to as the omphalocele, exstrophy, imperforate anus, and spinal defects (OEIS) complex, and is one of the most challenging surgical conditions to manage. Here, we present a unique case of a low-birth-weight patient with OEIS and a liver containing giant omphalocele and the novel surgical technique used to manage her cloacal exstrophy whereby the cecal plate was not separated from the bladder halves, but rather left for an autoaugment, and the ileum was connected to the hindgut.

Keywords

cloacal exstrophy - bladder exstrophy - anorectal malformation


Publication History

Received: 23 September 2020

Accepted: 04 March 2021

Article published online:
18 May 2021

© 2021. 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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