Introduction In 5–10% of Hirschsprung’s disease (HD) cases the aganglionic segment involves the entire colon and may extend into the small bowel. Long-term results after surgical treatment are not always good (mortality rate for this group exceeds 65% in most series). We report our experience with two patients with different short-term outcome.
Case 1 A two-day-old boy admitted with bowel obstruction and operated on for colonic and ileocecal valve atresia, performing cecostomy. HD was not suspected initially, and the patient underwent a ceco-colonic anastomosis one month later, suffering from anastomosis dehiscence and chemical peritonitis. Partial ileo-colonic resection, ileostomy and side-to-end ileo-colonic anastomosis were carried out, showing histological diagnosis of total colonic aganglionosis (TCA). Now (at 7 month-old), the patient does well (weight/height >p3), with definite procedure still to be done.
Case 2 A two-day-old boy with intestinal obstruction underwent urgent laparotomy, finding ileocecal hernia with partial resection and ileostomy. Postoperative ischemic enterocolitis occurred, carrying out resection of entire colon and 65 cm of small intestine, both aganglionic. Boley modified procedure, creating a 20 cm side-to-side ileocolostomy with the aganglionic descending colon, was performed. Complications included near total parenteral nutrition dependence, cholestatic hepatopathy, severe perianal excoriation due to liquid and frequent stools, blood infections etc. The patient died at 7 months of age because of bacterial sepsis.
Comments TCA diagnosis is a clinical challenge for paediatricians. Accurate histological diagnosis and meticulous preoperative bowel management are necessary. Nevertheless, the length of the aganglionosis seems to have the greatest impact on overall surgical outcome of HD.