Management of Hirschsprung's disease: curative surgery before 3 months of age

Abstract

Curative surgery of Hirschsprung's disease (HD) was performed in 32 infants younger than 3 months of age from January 1, 1977, December 31, 1986. There were 24 males, seven of whom presented with total colonic aganglionosis. Preoperative relief of obstruction was carried out by only enemas in 25 patients (81%), with addition of total parenteral nutrition of 8 to 27 days in case of severe enterocolitis. Colostomy was never performed as a routine procedure. Preoperative morbidity occurred in three cases: one enterocolitis and two sigmoid performations that were successfully treated by colostomy. Weight at operation ranged from 3.3 to 6.0 kg (mean, 4.9 kg). Swenson's (25), Duhamel's (5), and Soave's procedures (2) were performed. There was no mortality. Follow-up varied from 2 to 10 years. Postoperative morbidity occurred in five patients (one anastomotic leak, two stenosis, one volvulus, and one recurrent enterocolitis). All other patients are considered as definitively cured. Comparison with data in the literature permits one to advocate primary corrective treatment of HD without decompression in infants less than 3 months of age.