Hirschsprung's disease. Evaluation of mortality and long-term function in 260 cases
F. J. Rescorla, A. M. Morrison, D. Engles, K. W. West and J. L. Grosfeld
Department of Surgery, Indiana University School of Medicine, Indianapolis.
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.
