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; Thomas O. Xu; Yu-Ting Liu; Sulaiman Kidwai; Teresa L. Russell; Laura Tiusaba; Krystal Artis; Anthony Sandler; Andrea Badillo; Marc A. Levitt

doi:10.1055/a-2351-9413

European Journal of Pediatric Surgery Reports, Table of Contents

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

¹Department of Colorectal and Pelvic Reconstruction, Children's National, Washington, District of Columbia, United States

²The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, United States

,

Thomas O. Xu

³Department of Surgery, Colorectal and Pelvic Reconstructive Surgery, Children's National Hospital, Washington, District of Columbia, United States

,

Yu-Ting Liu

³Department of Surgery, Colorectal and Pelvic Reconstructive Surgery, Children's National Hospital, Washington, District of Columbia, United States

,

Sulaiman Kidwai

⁴Department of Surgery, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, United States

,

Teresa L. Russell

¹Department of Colorectal and Pelvic Reconstruction, Children's National, Washington, District of Columbia, United States

,

Laura Tiusaba

³Department of Surgery, Colorectal and Pelvic Reconstructive Surgery, Children's National Hospital, Washington, District of Columbia, United States

,

Krystal Artis

¹Department of Colorectal and Pelvic Reconstruction, Children's National, Washington, District of Columbia, United States

,

Anthony Sandler

¹Department of Colorectal and Pelvic Reconstruction, Children's National, Washington, District of Columbia, United States

,

Andrea Badillo

¹Department of Colorectal and Pelvic Reconstruction, Children's National, Washington, District of Columbia, United States

,

Marc A. Levitt

³Department of Surgery, Colorectal and Pelvic Reconstructive Surgery, Children's National Hospital, Washington, District of Columbia, United States

› Author Affiliations

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

Full Text

References

References
1 Moore SW. Total colonic aganglionosis in Hirschsprung disease. Semin Pediatr Surg 2012; 21 (04) 302-309
2 Kawaguchi AL, Guner YS, Sømme S. et al; American Pediatric Surgical Association Outcomes and Evidence-Based Practice (OEBP) Committee. Management and outcomes for long-segment Hirschsprung disease: a systematic review from the APSA Outcomes and Evidence Based Practice Committee. J Pediatr Surg 2021; 56 (09) 1513-1523
3 Osuchukwu OO, Rentea RM. Ileal Atresia. StatPearls; 2023
4 Pimenta Santos L, Coimbra D, Cunha C, Lopes MF. Oesophageal atresia with tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease: outcome of a rare phenotype unusual association of diseases/symptoms. BMJ Case Rep 2019; 12: 226675
5 Lauwers P, Moens E, Wustenberghs K. et al. Association of colonic atresia and Hirschsprung's disease in the newborn: report of a new case and review of the literature. Pediatr Surg Int 2006; 22 (03) 277-281
6 Gupta M, Beeram MR, Pohl JF, Custer MD. Ileal atresia associated with Hirschsprung disease (total colonic aganglionosis). J Pediatr Surg 2005; 40 (09) e5-e7
7 Payen E, Talbotec C, Chardot C. et al. Outcome of total colonic aganglionosis involving the small bowel depends on bowel length, liver disease, and enterocolitis. J Pediatr Gastroenterol Nutr 2022; 74 (05) 582-587
8 Nakayuenyongsuk W, Barnes D, Martin B, Christofferson M, Kerner J. The runs: sudden copious ostomy output in an acolonic Hirschsprung disease patient with short gut syndrome. Dig Dis Sci 2019; 64 (01) 56-59
9 Goslin B, Brown A, Robertson D. Gastroschisis, ileal atresia, and Hirschsprung's disease in a newborn: the first reported case. J Pediatr Surg 2012; 47 (11) 2134-2136
10 Gauderer MW, Rothstein FC, Izant Jr RJ. Ileal atresia with long-segment Hirschsprung's disease in a neonate. J Pediatr Surg 1984; 19 (01) 15-17
11 Atqiaee K, Hiradfar M, Mashhadi MP, Khanghah AS. Ileal atresia and total colonic Hirschsprung disease in a 36-week neonate: a case report. Clin Case Rep 2023; 11 (03) e7079
12 Janik JP, Wayne ER, Janik JS, Price MR. Ileal atresia with total colonic aganglionosis. J Pediatr Surg 1997; 32 (10) 1502-1503
13 Lally KP, Chwals WJ, Weitzman JJ, Black T, Singh S. Hirschsprung's disease: a possible cause of anastomotic failure following repair of intestinal atresia. J Pediatr Surg 1992; 27 (04) 469-470
14 Pironi L. Definition, classification, and causes of short bowel syndrome. Nutr Clin Pract 2023; 38 (Suppl. 01) S9-S16
15 Laughlin DM, Friedmacher F, Puri P. Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatr Surg Int 2012; 28 (08) 773-779
16 Yeh YT, Tsai HL, Chen CY. et al. Surgical outcomes of total colonic aganglionosis in children: a 26-year experience in a single institute. J Chin Med Assoc 2014; 77 (10) 519-523
17 Ikawa H, Masuyama H, Hirabayashi T, Endo M, Yokoyama J. More than 10 years' follow-up to total colonic aganglionosis–severe iron deficiency anemia and growth retardation. J Pediatr Surg 1997; 32 (01) 25-27
18 Kim HB, Fauza D, Garza J, Oh JT, Nurko S, Jaksic T. Serial transverse enteroplasty (STEP): a novel bowel lengthening procedure. J Pediatr Surg 2003; 38 (03) 425-429
19 Ching YA, Fitzgibbons S, Valim C. et al. Long-term nutritional and clinical outcomes after serial transverse enteroplasty at a single institution. J Pediatr Surg 2009; 44 (05) 939-943
20 Muff JL, Sokolovski F, Walsh-Korb Z. et al. Surgical treatment of short bowel syndrome-the past, the present and the future, a descriptive review of the literature. Children (Basel) 2022; 9 (07) 1024
21 Bischoff A, Levitt MA, Peña A. Total colonic aganglionosis: a surgical challenge. How to avoid complications?. Pediatr Surg Int 2011; 27 (10) 1047-1052