[Total colonic form of Hirschsprung disease. Treatment and long-term follow-up in 16 cases]

Abstract

From 1971 to 1994, 16 cases of total colon Hirschsprung's disease were treated at the University Hospital in Rennes. Diagnosis have been at 2 days to 3 months. Two children had a family history of Hirschsprung disease among which one associated megacolon and multiple endocrine neoplasia. This family had a mutation of the RET proto oncogene. Six children died before complete surgical cure, among whom 4 before total parenteral nutrition. Six were treated according to Lester Martin, 3 according to Duhamel, and 1 to Swenson. Diarrhea and occlusions happened during the first postoperative years. None had any enterocolitis. Eight of 9 followed children are continent. Technique had no influence on long term outcome. Early neonatal occlusion management seems to decrease enterocolitis's incidence. We abandoned Lester's technique and kept Duhamel's technique. The problems encountered during ileostomy period do not encourage us to forward the age of definitive surgery procedure.