Intestinal atresia and Hirschsprung's disease

Intestinal atresia associated with Hirschsprung's disease has been reported in only 26 cases (20 of small bowel and 6 of colon). Three additional patients are reported, two with associated myelomenigocoele. The significance of the myelomeningocoele and possible aetiological mechanisms of these associations are discussed with particular reference to the role of a vascular accident or the embryological failure of migration of nerve cells. The most likely cause is a volvulus proximal to the aganglionic bowel resulting in the associated atresia.Offprint requests to: S. Cywes