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

Authors

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

Document Type

Journal Article

Publication Date

1-1-2024

Journal

European journal of pediatric surgery reports

Volume

12

Issue

1

DOI

10.1055/a-2351-9413

Keywords

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

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.

APA Citation

Velasquez, Alejandro R.; Xu, Thomas O.; Liu, Yu-Ting; Kidwai, Sulaiman; Russell, Teresa L.; Tiusaba, Laura; Artis, Krystal; Sandler, Anthony; Badillo, Andrea; and Levitt, Marc A., "Achieving Digestive Autonomy and Gastrointestinal Continuity in a Patient with Short Bowel Syndrome Secondary to Concomitant Jejunal Atresia and Small Intestinal Hirschsprung's Disease" (2024). GW Authored Works. Paper 5272.
https://hsrc.himmelfarb.gwu.edu/gwhpubs/5272

Department

School of Medicine and Health Sciences Student Works