One-stage neonatal pull-through to treat Hirschsprung's disease

Traditionally Hirschsprung's disease has been treated by a three-stage procedure. This approach has been associated with stoma problems, three episodes of hospitalization, and a theoretical disadvantage, because of the delayed passage of feces via the rectum, in controlling defecation. The aim of this study was to assess the results of one-stage neonatal pull-through for the treatment of Hirschsprung's disease.

This was a 10-year retrospective review of all patients treated with a neonatal pull-through for Hirschsprung's disease. Information was obtained from the patients' records. All descriptive data are expressed as mean +/- standard deviation.

Fifty-one patients were treated in the 10-year period studied. The mean gestational age was 39.6 +/- 1.7 weeks, weight was 3.3 +/- 0.54 kg, with 76% being boys. The surgery was performed at 10.3 +/- 5.8 days of age. A Swenson procedure was carried out in 38 patients and a Soave in 13. The extent of the disease was rectosigmoid in 39, and the remaining were sigmoid, descending colon, and total colonic. The mean length of the surgery was 181 +/- 40 minutes. Blood transfusions were required in 13 patients with a mean volume of 55 mL given. The complications observed were: an anastomotic leak in two cases, requiring laparotomy and colostomy (both in patients with Down's syndrome), and wound infection in three cases. There were two delayed deaths both associated with other congenital anomalies. In total, three patients required further surgery; two had colostomies, one requiring a redo pull-through and one had an ileostomy for enterocolitis. The total length of hospital stay was 20.4 +/- 11.2 days. Mean follow-up was 3.73 +/- 2.9 years. In 21 patients the follow-up was greater than 4 years and in this group none were totally incontinent. Four patients complained of soiling at least once a week, and 17 had normal bowel control.

Hirschsprung's disease can be successfully treated in the neonatal period with a one-stage pull-through. The short- and long-term results are as good as those with the three-stage procedure, with the child usually benefitting by not having a stoma and a shorter hospital stay.