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CC BY 4.0 · European J Pediatr Surg Rep. 2024; 12(01): e45-e49
DOI: 10.1055/a-2351-9413
Case Report

Achieving Digestive Autonomy and Gastrointestinal Continuity in a Patient with Short Bowel Syndrome Secondary to Concomitant Jejunal Atresia and Small Intestinal Hirschsprung's Disease

Alejandro R. Velasquez
1   Department of Colorectal and Pelvic Reconstruction, Children's National, Washington, District of Columbia, United States
2   The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, United States
,
Thomas O. Xu 
3   Department of Surgery, Colorectal and Pelvic Reconstructive Surgery, Children's National Hospital, Washington, District of Columbia, United States
,
Yu-Ting Liu
3   Department of Surgery, Colorectal and Pelvic Reconstructive Surgery, Children's National Hospital, Washington, District of Columbia, United States
,
Sulaiman Kidwai
4   Department of Surgery, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, United States
,
Teresa L. Russell 
1   Department of Colorectal and Pelvic Reconstruction, Children's National, Washington, District of Columbia, United States
,
Laura Tiusaba 
3   Department of Surgery, Colorectal and Pelvic Reconstructive Surgery, Children's National Hospital, Washington, District of Columbia, United States
,
Krystal Artis
1   Department of Colorectal and Pelvic Reconstruction, Children's National, Washington, District of Columbia, United States
,
Anthony Sandler
1   Department of Colorectal and Pelvic Reconstruction, Children's National, Washington, District of Columbia, United States
,
Andrea Badillo
1   Department of Colorectal and Pelvic Reconstruction, Children's National, Washington, District of Columbia, United States
,
Marc A. Levitt
3   Department of Surgery, Colorectal and Pelvic Reconstructive Surgery, Children's National Hospital, Washington, District of Columbia, United States
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Abstract

Concomitant presentation of jejunal atresia and Hirschsprung's disease is rare and places children at high risk for developing short bowel syndrome and parenteral nutrition dependence, which can affect the feasibility/timing of pull-through. A patient was born with jejunal atresia with a delayed diagnosis of Hirschsprung's disease. After several procedures and bowel resections, the patient was ultimately left with an end jejunostomy and long Hartman's pouch with short bowel syndrome, dependent on parenteral nutrition. The patient initially presented to our institution at age 2 with failure to thrive secondary to an obstructed/dilated jejunostomy and mild enterocolitis of their defunctionalized segment. The patient subsequently underwent completion of subtotal colectomy and revision of jejunostomy utilizing a serial transverse enteroplasty to manage the dilated bowel and gain length. The patient was able to wean off parenteral nutrition and achieve nutritional autonomy by age 5. Following this, the patient was able to undergo an ileoanal pull-through. After the pull-through, the patient was able to pass stool independently and suffered no major complications to date. Serial transverse enteroplasty can be successfully utilized in patients with a history of Hirschsprung's disease and jejunal atresia to achieve nutritional autonomy and ultimately reestablish gastrointestinal continuity with pull-through.

Keywords

STEP procedure - total colonic Hirschsprung's disease - short bowel syndrome - Hirschsprung's pull-through - jejunal atresia


Publication History

Received: 21 December 2023

Accepted: 29 February 2024

Accepted Manuscript online:
25 June 2024

Article published online:
16 July 2024

© 2024. 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/)

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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