Associated developmental anomalies of the anterior end of the neural crest: Hirschprung's disease/Waadrenburg syndrome

Three patients are described in whom Hirschsprung's disease was associated, in varying degree, with deafness, disturbance of facial configuration and deficient pigmentation of the skin and its appendages; expressing developmental defects of the anterior end of the neural crest. The anomalies described are seen, not as separate and fortuitously associated lesions, but rather as different aspects of a single developmental disturbance of the anterior end of the neural crest. In sporadic reports[14-17] [as shown in text] a substantial experience of such patients is recorded, though texts on pediatric surgery rarely quote this