COLONIC ATRESIA COMBINED WITH HIRSCHSPRUNGS-DISEASE - A DIAGNOSTIC AND THERAPEUTIC CHALLENGE

Three new cases of patients with co-occurrence of colonic atresia and Hirschsprung's disease and a review of the literature (n = 5) are pres ented in this report. All patients (n = 8) except one were full-term i nfants who had no other significant anomalies. The preterm infant had associated tetralogy of Fallot in addition to Hirschsprung's disease a nd colonic atresia. Six patients had atresia of the ascending colon, a nd two had atresia of the colon to splenic flexure. All colonic atresi as were diagnosed neonatally; however, there was mean delay of 15.4 mo nths (range, 1 mo to 5 years) in diagnosing associated Hirschsprung's disease. There were two deaths. A careful examination of the resected specimen to rule out Hirschsprung's disease is recommended. Performing a rectal biopsy must be considered for patients who initially were tr eated for colonic atresia and who have a slow return of normal gut fun ction. Copyright (C) 1995 by W.B. Saunders Company