Spectrum of congenital anomalies associated with biliary atresia

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 recommended.