The Right Colon Patch Graft Procedure for Extensive Intestinal Aganglionosis

抄録

Extensive intestinal aganglionosis is rare and very difficult to diagnose and treat. The condition is often fatal. A 6-month-old boy who had undergone ileostomy for extensive intestinal aganglionosis was referred to our department. We applied an aganglionic right colon onlay patch to the aganglionic intestine to enhance absorption of water and electrolytes. Three months after the ileocolostomy, the definitive operation, a Swenson-type procedure, was performed. The mesocolon to the onlay patch could be divided because blood supply was adequate from the ileal mesentery via the intestinal wall. Postoperatively, the onlay patch segment appeared normal on colonoscopy and bowel habit was improved. Although the patient still requires parenteral nutrition support due to the short bowel, the right colon onlay patch procedure enables him to be cared for at home and provides an opportunity for normal growth and development.