Near-total intestinal aganglionosis in the Waardenburg-Shah syndrome

Both pigmentation and otic defects of Waardenburg Syndrome and Hirschsprung 's disease have a common origin in neural crest cells and were described in 1951 and 1887, respectively. The clinical manifestations of both in the sa me patient were described in 1981 in 12 infants so afflicted. The authors p resent such a case of long segment aganglionosis in a 15-day-old Marshalles e girl with Waardenburg-Shah syndrome and discuss diagnosis, treatment, and prognosis. J Pediatr Surg 34:1853-1855. Copyright (C) 1999 by W.B. Saunder s Company.