[Extensive Hirschsprung's disease associated with intestinal malrotation].

Hirschsprung's disease (HD) involves the entire colon in less than 5% of cases, and the association of extensive HD with intestinal malrotation is very rare. This association of symptoms may delay both diagnosis and treatment. An infant presented with an intermittent occlusive syndrome that began neonatally. Intestinal malrotation was diagnosed radiologically, and treated surgically when the child was 2 months old. However, a chronic occlusion persisted. Biopsies of the rectum and the appendix demonstrated an absence of neurons in intestinal plexi. When the child was 17 months old, ileostomy and surgical excision of the segment affected by HD (the colon and terminal ileum) were performed. An ileoanal anastomosis was performed at the age of 29 months, with favorable outcome. The persistence of symptoms of intestinal occlusion after attempted treatment of intestinal malrotation must therefore suggest the possibility of associated HD in a young child.