Uncommon co-occurrence of ileal atresia and total colonic aganglionosis in two unrelated newborns.

We report two recent cases of distal ileal atresia associated with total colonic aganglionosis (TCA). It is well known that ileal atresia and Hirschsprung's disease (HD) are individually frequent causes of intestinal obstruction. However, the association of both these diseases is an extremely rare event. To our knowledge, only 19 cases of ileal atresia associated with HD have been described so far. When a child is diagnosed having ileal atresia, the possibility of associated TCA should be considered. Therefore, intra-operative staged biopsies should be sent for histological examination in order to rule out or confirm this very rare co-occurrence.