Neonatal hepatitis and extrahepatic biliary atresia in the same sibship

An instance of the rare occurrence of neonatal hepatitis and extrahepatic biliary atresia in the same sibship is reported. The older brother with neonatal hepatitis developed jaundice at the age of 4 days and had clay-colored stools from early infancy. Cholangiography by exploratory laparotomy at the age of 3 months showed a normal bile duct pattern. After laparotomy, jaundice rapidly disappeared, and stools became yellow. His liver function has been normal since age 6 months to the present (6 years old). The younger brother developed jaundice and clay-colored stools at the age of 1 month. The diagnosis of extrahepatic biliary atresia was made at laparotomy at the age of 4 1/2 months. Hepatojejunostomy was performed with successful bile drainage, although he had frequent attacks of ascending cholangitis since operation. These cases support a recent hypothesis that neonatal hepatitis and extrahepatic biliary atresia may be produced by the same disease process.