Gastrointestinal function after surgical correction of Hirschsprung's disease: long-term follow-up in 81 patients.

Background: Despite significant developments in understanding the pathological anatomy and physiology of Hirschsprung's disease (HD), the follow-up of children with HD remains far from clear. Treatment results for HD are not uniformly successful and the interpretation of the result by the parents and child may differ from that of the surgeon. Our goal is to analyze quality of life and differences in functional outcomes in children operated on using the pediatric incontinence / constipation scoring system (PICSS) validated in a regulatory group.
Methods: A retrospective questionnaire was performed for all children operated for HD between 1995 and 2017 at the Pediatric Surgery Unit. The use of PICSS defined children with continence or incomplete constipation.
Results: 125 patients were operated on for HD between 1995 and 2017 in our Pediatric Surgery Unit and 81 families gave their consent to participate. The median age at the interview was 94.5 months (range 12-283 months). Complete aganglionosis was in 2% of cases. Treatments included a Soave procedure in 96%. Colostomy was performed in 15.8% (13/81). Decompressive enterostomy was performed in a child. The rate of normal bowel function was 66.6% (n = 54). The percentage of cases with incomplete continence and with constipation were 4.9% (n = 4) and 16% (n = 13), respectively.
Conclusions: HD results aren't always as good as surgeons may perceive; long-term follow-up is important. Over time, most schoolage children improve significantly for fecal continence, but it can also be acquired in late adolescence.