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

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.