Hyperbilirubinemia Due to Extrinsic Biliary Compression From Direct Intrahepatic Portocaval Shunt Performed for Portal Hypertensive Bleeding

Abstract

Direct intrahepatic portocaval shunt (DIPS) is a procedure involving intravascular stent placement through the caudate lobe of the liver for management of portal hypertension. It is employed less frequently than TIPS, and extrinsic biliary compression due to DIPS has never been described in the literature. A 65 year old female with history of non-cirrhotic fibropolycystic liver disease and PVT with cavernoma and sinistral portal hypertension underwent DIPS and ileal variceal embolization for massive ileal variceal bleeding. TIPS was attempted but unsuccessful, so DIPS was subsequently performed. Pre-procedural alkaline phosphatase (AP) was 56U/L, total bilirubin (TB) 1.1mg/dL, and direct bilirubin (DB) was 0.4mg/dL. Post-procedurally she was noted to have AP 326U/L, TB 9.8mg/dL, and DB 6.9mg/dL. There was report of dark stools, with initial concern of hemobilia versus bilhemia. Imaging revealed stable mild intrahepatic and common bile duct (CBD) dilatation. Given new fevers and possible enhancement of CBD, she was treated with piperacillin-tazobactam for presumed cholangitis with subsequent improvement and was discharged home. She returned ten days later with alkaline phosphatase of 2,925U/L with total bilirubin of 41.9mg/dL and direct bilirubin of 29.8mg/dL. MRI revealed intra- and extra-hepatic biliary dilatation to the mid-CBD felt to be due to extrinsic compression by the crossing DIPS. ERCP revealed CBD stenosis which did not appear to be related to crossing DIPS but was stented. Afterwards, her AP peaked at 3,062U/L with TB 56.9mg/dL and DB 38.1mg/dL. Liver biopsy revealed bland cholestasis. Her liver chemistries started to improve. Due to persistent fevers, ERCP was repeated for stent exchange and revealed sigmoid-shaped stenosis in the upper CBD due to extrinsic compression from the crossing DIPS, thus revealing the ultimate diagnosis. Stent was replaced and AP subsequently improved to 104U/L with TB 1.4mg/dL.The differential of hyperbilirubinemia after DIPS procedure includes hemolysis (typically presents with indirect hyperbilirubinemia), as well as hepatic decompensation (increase in all liver chemistries), bilhemia from biliary-venous fistula (isolated hyperbilirubinemia), infection (often a mixed picture), and biliary compression (cholestatic picture). This complicated case illustrates the difficulty in diagnosis of extrinsic biliary compression in a patient with known mild biliary dilation, requiring a second ERCP to make the final diagnosis.2187_A Figure 1. MRCP image of the 1.9 cm segmental narrowing of the mid common bile duct, thought to be related to the crossing DIPS.2187_B Figure 2. MRCP image of 1.9cm segmental narrowing of CBD secondary to extrinsic compression by the crossing TIPS