title: Hirschsprung's disease. Evaluation of mortality and long-term function in 260 cases.
abstract: This report describes 260 patients treated for Hirschsprung's disease. There were 213 boys (82%) and 47 girls (18%). Age at diagnosis was younger than 30 days in 106 patients (41%), 1 month to 1 year in 90 patients (35%), and older than 1 year in 64 patients (25%). Diagnosis was achieved with barium enema and rectal biopsy. Aganglionosis involved the rectum or rectosigmoid in 174 patients (67%), the left colon in 38 patients (15%), and the proximal colon in 23 patients (9%); 25 patients (9%) had total colonic aganglionosis. Enterocolitis occurred in 47 cases (18%). Following an initial colostomy or ileostomy, a definitive pull-through procedure was performed in 247 patients (95%) (modified Duhamel in 185, Soave in 25, Swenson procedure in 15, and anomyectomy/sphincterotomy in 22); the overall survival rate was 93.8% (244 of 260 patients). An increased mortality was associated with Down syndrome, total colonic aganglionosis, and enterocolitis. Long-term follow-up (mean, 6 years 10 months) was available in 103 patients who underwent a Duhamel procedure. Sixty-seven (65%) had normal bowel function, 28 (27%) occasionally used enemas or stool softeners, and eight (8%) had severe constipation or soiling. Bowel habits improved with time and were considered normal in 58% of patients at less than 5 years of follow-up and in 88% of patients at more than 15 years of follow-up. The Duhamel operation is a very effective definitive procedure for Hirschsprung's disease. Long-term follow-up is an important component of patient care.
authors.affiliation: Department of Surgery, Indiana University School of Medicine, Indianapolis.
authors.forename: F J
authors.initials: FJ
authors.lastname: Rescorla
authors.forename: A M
authors.initials: AM
authors.lastname: Morrison
authors.forename: D
authors.initials: D
authors.lastname: Engles
authors.forename: K W
authors.initials: KW
authors.lastname: West
authors.forename: J L
authors.initials: JL
authors.lastname: Grosfeld
mesh_terms: D000714:Anastomosis, Surgical; D002423:Cause of Death; D003106:Colon / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006627:Hirschsprung Disease / Q000401:mortality* / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012007:Rectum / Q000601:surgery; D015996:Survival Rate; D016896:Treatment Outcome