BILIARY, ANORECTAL AND ESOPHAGEAL ATRESIA - A NEW ENTITY

A unique case of newborn biliary atresia associated with esophageal at resia and tracheoesophageal fistula, ano-rectal atresia, Reovirus type 3 infection and an early switch of fetal into adult hemoglobin is rep orted. At birth, the infant, who had only one umbilical artery, was op erated on by primary anastomosis of the esophagous, and descending ccl ostomy. At six weeks of age the baby underwent a ''Kasai hepatic porto enterostomy-Type I'' for a EHBA Type III, Subtype C2, Subgroup O (''ap lasia'' of all extrahepatic biliary ducts, including the gallbladder). The absence of an artery branch for the left lobe of the liver was ob served. Histologically, the liver showed a hyperplasia of the intrahep atic bile ducts due to persistence of an excess of embryologic bile du cts in ''ductal plate malformation'' (DPM). Specific Reovirus type 3 a ntibodies were found in both the mother's and baby's sera. In the post operative period the infant developed rapid and severe liver failure a nd underwent a successful liver transplantation. Although in most case s EHBA appears to be a perinatal event due to a necro-inflammatory pro cess of unknown etiology, cases associated with complex extrahepatic a nomalies, may be due ta different pathogenetic mechanisms supported by different causative agents operating very early in the fetal period. Viral infection seems to be the most reliable etiology.