The challenges of closing an ileostomy in patients with total intestinal aganglionosis after small bowel transplant

Pediatr Surg Int. 2018 Jan;34(1):113-116. doi: 10.1007/s00383-017-4216-y. Epub 2017 Nov 23.

Abstract

We present the case of a 14-year-old male with a history of small bowel transplantation for long segment Hirschsprung's disease who underwent Duhamel ileorectal pull-through procedure. In post-transplant, the patient had no restrictions and was not TPN-dependent. To improve his quality of life, he and his family were interested in closing the ileostomy and undergoing pull-through surgery. The complexity of the case includes the presence of an aganglionic rectal segment-a short root of the mesentery due to the small bowel transplant-and significant immunosuppression. At the moment, he is continent, doing well, and has not had any remarkable complications.

Keywords: Hirschsprung’s disease; Small bowel transplantation; Total intestinal aganglionosis.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Digestive System Surgical Procedures*
  • Hirschsprung Disease / surgery*
  • Humans
  • Ileostomy*
  • Immunosuppressive Agents / therapeutic use
  • Intestine, Small / transplantation*
  • Male

Substances

  • Immunosuppressive Agents

Supplementary concepts

  • Aganglionosis, total intestinal