CC BY 4.0 · European J Pediatr Surg Rep. 2022; 10(01): e45-e48
DOI: 10.1055/s-0041-1742154
Case Report

Hindgut Duplication in an Infant with Omphalocele–Exstrophy–Imperforate Anus–Spinal Defects (OEIS) Complex

1   Department of Surgery, Boston Children's Hospital, Boston, Massachusetts, United States
,
Farokh R. Demehri
1   Department of Surgery, Boston Children's Hospital, Boston, Massachusetts, United States
,
Craig W. Lillehei
1   Department of Surgery, Boston Children's Hospital, Boston, Massachusetts, United States
,
Joseph G. Borer
2   Department of Urology, Boston Children's Hospital, Boston, Massachusetts, United States
,
Benjamin C. Warf
3   Department of Neurosurgery, Boston Children's Hospital, Boston, Massachusetts, United States
,
Belinda H. Dickie
1   Department of Surgery, Boston Children's Hospital, Boston, Massachusetts, United States
› Author Affiliations

Abstract

Introduction The congenital anomaly of omphalocele, cloacal exstrophy, imperforate anus, and spinal abnormalities (OEIS complex) is rare but well recognized. Hindgut duplications are also uncommon and are not known to be associated with OEIS. We describe a neonate with OEIS who was found to have fully duplicated blind-ending hindguts.

Case Report A premature infant boy with OEIS underwent first-stage closure on day of life 6, which included excision of the omphalocele sac, separation of the cecal plate and bladder halves, tubularization of the cecal plate, hindgut rescue with end colostomy, and joining of the bladder halves. Cecal plate inspection revealed two hindgut structures that descended distally, one descended midline into the pelvis along the sacrum and the second laterally along the left border of the sacrum. Both lumens connected to the cecal plate and had separate mesenteries. In an effort to maximize the colonic mucosal surface area, the hindgut segments were unified through a side-to-side anastomosis, creating a larger caliber hindgut. The cecal plate was tubularized and an end colostomy was created. Bowel function returned and he was discharged home on full enteral feeds.

Discussion This case represents a cooccurrence of two extremely rare and complex congenital anomalies. The decision to unify the distinct hindguts into a single lumen was made in an effort to combine the goals of management for both OEIS and alimentary duplications. The hindgut is abnormal in OEIS and should be assessed carefully during repair.



Publication History

Received: 12 May 2020

Accepted: 16 October 2020

Article published online:
10 March 2022

© 2022. 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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