Congenital Extrahepatic Biliary Atresia

Case 1. The elder of two sibs, this boy was born on May 21, 1965 to unrelated healthy parents aged 29 (mother) and 30 years (father). Birthweight was 2820 g.; delivery was by forceps. His mother had 'influenza' between the fifth and sixth months of the pregnancy which had been otherwise normal. There was no previous foetal loss. Four generations of the family, comprising, in addition to the parents, 19 second degree relatives and 69 more distant kin, were reviewed without uncovering any significant findings. Jaundice, which rapidly deepened, was present at birth. Laparotomy at 6 weeks showed the liver to be enlarged, smooth, and tense, with thin fibrous cords representing the hepatic, the common bile, and the cystic ducts which were all completely free of bile. The gall-bladder was small and collapsed though the lumen contained some thick green bile. A diagnosis of extrahepatic biliary atresia was made and a cholecystgastroenterostomy was successfully performed. The patient was readmitted at 9 months with a respiratory infection, deep jaundice, and colourless faeces: despite antibiotic therapy he died 10 days later. Necropsy was not performed. Case 2. The second boy in the sibship, bom June 22, 1967, weighed 2876 g. at birth which followed an uneventful pregnancy and normal delivery. He was jaundiced, had colourless faeces, and his abdomen was distended by an enlarged liver. Laparotomy at 5 weeks showed a rudimentary cystic duct and a small lax gallbladder containing 2 ml. of bile-stained viscous fluid. Other parts of the portal duct system were not identified.