Hirschsprung disease-Bowel function beyond childhood

Abstract

Hirschsprung disease is a developmental defect of the enteric nervous system characterized by lack of enteric neurons in the distal hindgut. There are numerous reports on short-term outcomes indicating that impaired bowel function is common. Recently, several controlled studies show that bowel function outcomes are affected beyond childhood, in adolescents and adults, compared with healthy control subjects. Constipation and fecal incontinence are common. The impaired bowel function appears to have a negative impact on quality of life, although, a majority of patients have adapted to their symptoms. On the other hand, Hirschsprung disease seems to have limited impact on education and occupation in adult life. The aim of this review was to summarize current knowledge of bowel function outcome beyond childhood in patients with Hirschsprung disease.

Keywords: Bowel function; Constipation; Fecal incontinence; Hirschsprung disease; Quality of life; Soiling.