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

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.

Hirschsprung's disease: case report

“…Patients with a ganglion segment smaller than 20 to 40 centimeters became more likely to use Total Parenteral Nutrition (TPN) due to the small part of the absorptive intestinal surface after removal of the affected segment. The only way to avoid TPN is intestinal transplantation, aiming to improve the quality of life and decrease morbidity and mortality [7]. It is noted that even if the entire affected segment can be removed, there is no complete recovery of the patients, thus, it becomes necessary to better understand the pathophysiology of the disease to improve the therapeutic techniques, aiming at the total cure of the patient, reducing the risk of sequelae [2].…”

Total collectomy in adult woman with Hirschsprung's Disease: a case report

“…The newborn usually has an acute obstructive abdomen or constipation, associated with abdominal distention and paradoxical diarrhea. In older children, the most common form of presentation is chronic constipation [2,7,8].…”