[Hirschprung' disease in Iceland 1969-1998.]

Hirschprung s disease (HD) is a congenital disease characterized by the absence of myenteric and submucosal ganglion cells in the distal alimentary tract and results in decreased motility in the affected bowel segment. The purpose of this study was to review the incidence, presentation, treatment and operative results in children with Hirschprung's disease in Iceland.

Thirteen infants with Hirschprung s disease (11 boys and two girls) were treated in Landspítalinn University Hospital between January 1969 and December 1998. The records of these patients were reviewed retrospectively.

The incidence of Hirschprung's disease in Iceland is 1/10,000 and 85% of those are boys. All the infants were born full term. No family history and no associated abnormalities were noted. The mean age at first admission was 20 (1-136) days and mean age at diagnosis was 166 (5-623) days. Swenson's pull-through (two- or three-stage procedure) was carried out in all patients at the mean age of 18.6 months. The extent of aganglionosis was rectosigmoid colon in 10 patients (77%) and one patient had total colonic aganglionosis. Postoperative complications occurred in seven patients (53%), adhesion ileus being the most common complication. Long term bowel function was satisfactory in 85% of the patients.

The incidence of Hirschprung's disease in Iceland is low. Mean age at diagnosis is six months. Sixty percent of the children are discharged with a wrong diagnosis after first admission to hospital and this could be improved by diagnosing the disease at an earlier stage. Postoperative complications are common but no deaths occurred. Bowel function following definitive correction is good compared to other studies.