Spontaneous perforation of the biliary tract and portal vein thrombosis in infancy

Spontaneous biliary perforation (SBP) is an uncommon cause of surgical jaundice in the first few weeks of life and is characterised by the occurrence of a punched-out defect in the bile duct, typically where the cystic duct joins the common hepatic duct. In most cases the site occurs anteriorly and bile leaks into the general peritoneal cavity. We now describe two cases of SBP where the perforation occurred posteriorly, limiting the leak and resulting in delayed recognition. Surgical management in both cases consisted of hepaticojejunostomy-en-Roux. Both cases were complicated, although not immediately, by portal vein thrombosis and one by chylous ascites. Posterior SBP, presumably due to their intimate anatomical relation with the portal vein, seem predisposed to such complications.