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

ABSTRACT

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