Hirschsprung's disease, colonic atresia, and absent hand: a new triad

Abstract

Colonic atresia (CA) has been reported in association with Hirschsprung's Disease (HSCR) on 11 previous occasions. In most reported cases the atresia has involved the right side of the colon; but in this, the twelfth case report, the atresia involved the left side of the colon, with aganglionosis of the entire distal gut. In addition, the child had an absent hand. The authors believe that this triad has not been reported previously. The literature is reviewed, and possible mechanisms are discussed.