The challenges of closing an ileostomy in patients with total intestinal aganglionosis after small bowel transplant
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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.
KeywordsTotal intestinal aganglionosis Small bowel transplantation Hirschsprung’s disease
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflicts of interest.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
Informed consent was obtained from all individual participants included in the study.
- 14.Amerstorfer EE, Fasching G, Till H, Huber-Zeyringer A, Höllwarth ME (2015) Long-term results of total colonic agangliosis patients treated by preservation of the aganglionic right hemicolon and the ileo-cecal valve. Pediatr Surg Int 31(8):773–780. https://doi.org/10.1007/s00383-015-3743-7 CrossRefPubMedGoogle Scholar