Hirschprung's disease: long-term follow-up of 65 cases.

The author reviews 65 cases of Hirschsprung's disease seen and treated in Saskatoon between 1951 and 1981. The annual incidence of this disease is 1/4000 live births. Overall mortality was 25% and related to two factors: (a) delay in diagnosis and surgical treatment with a high incidence of enterocolitis in the earlier years of the series; (b) a mortality of 83% in patients with small bowel aganglionosis. Thirty-five resections were done for colonic aganglionosis; there was one death that occurred 20 years ago, indicating that surgery is safe in this disease, but delay in treatment is not. Of six patients with small bowel aganglionosis, only three had resection and only one of these survived. The two patients who died had very high small bowel aganglionosis, probably incompatible with survival. The Rehbein type of procedure proved useful in high colonic aganglionosis where, by necessity, anastomosis was done at up to 7 cm above the white line of the pelvis, with excellent end results. There was a 35% incidence of Hirschsprung's disease in females; 37% had high aganglionosis (above the sigmoid). Low (white line) anastomosis was done in three patients available for 12-year follow-up. All have some degree of incontinence.