Spectrum of congenital anomalies associated with biliary atresia

Abstract

Several congenital anomalies have been reported in association with biliary atresia. We have analysed the type and frequency of anomalies observed over a 10-year period in consecutive patients operated for extrahepatic biliary atresia at our institution. Of the 107 infants who underwent a laparotomy and surgical correction of biliary atresia, 9 (8.4%) showed significant associated anomalies. Among them, 5 (55.5%) had splenic malformations, 6 (66.6%) had digestive anomalies in the form of malrotation, Meckel’s diverticulum and jejunal atresia. One patient had the classical polysplenia syndrome. Our follow-up period was limited (i.e. 5 months) during which time 3 of the 9 (33.3%) patients with associated anomalies became jaundice-free. A higher incidence of malformations found in association with biliary atresia supports the congenital theory and a more thorough search for these anomalies is recommeded.