MIRIZZI SYNDROME

Abstract

Mirizzi Syndrome is an unusual presentation of obstructive jaundice. In this rare entity gallstone impaction in Hartmann's pouch or in the cystic duct causes external compression of the common hepatic duct, resulting in biliary obstruction. First described by Mirizzi in 1948. This syndrome has been reported in 0.7-1.4% of all adult patients undergoing cholecystectomy. Rare in children, never been reported in infants. 2 months old male infant presented with gradual increasing direct hyperbilirubinemia. He was born at full term with gastrochisis, corrected by surgery shortly after birth. There was no resection of intestine during surgery. Received TPN for 3 weeks after birth. He was sent home at 1 month of age. Direct bilirubin at discharge was 3.2, normal alkaline phosphatase (AP), mildly elevated AST and ALT. At presentation direct bili was 5.2, AST 186, ALT 216, AP 421. Unremarkable CBC, BMP, and CRP. Blood cultute, urine culture, TORCH study, and viral hepatitis screening was negative. Hepatobiliary ultrasonography (US) showed mild hepatomegaly, contracted gall bladder, mild dilatation of common bile duct. Given the negative infectious workup, the US findings raise the suspicion of biliary atresia. Liver biopsy was done, which revealed obstructive cholestasis. Liver biopsy findings supported the diagnosis of biliary atresia. Pediatric Surgery was consulted for Kasai procedure. Intraoperative cholangiogram before Kasai revealed a small stone at the junction of the cystic and common hepatic duct that was causing extrinsic compression on the common hepatic duct. Exploration of CBD revealed no stones or other cuases of obstruction. The diagnosis of the Mirizzi syndrome was made. Cholecystectomy was done and a T tube was placement. T tube cholangiogram showed good flow. Direct hyperbilirubin level back to normal over the next few weeks. Though rare, Mirizzi syndrome can occur in infant and should be considered in the differential diagnosis of cholestatic jaundice.