Indiana University School of Medicine Indianapolis, IN
Introduction: Portal biliopathy is one of the rare complications of cirrhotic portal hypertension. We present a case of a patient with underlying cirrhosis presenting with biliary obstruction and bleeding in setting of biliary varices.
Case Description/Methods: A 60-year-old male with history of decompensated cirrhosis and heavy alcohol use was admitted for inpatient evaluation for liver transplant. His labs were remarkable for total bilirubin of 13.7, AST 66 of, ALT of 52 and alkaline phosphatase of 102. Patient reported several days of melena prior to admission but hemoglobin remained stable and there was no evidence of bleeding after admission. MRI abdomen completed as part of his pre-transplant workup showed severe intrahepatic biliary duct dilation with multifocal stricturing and beaded appearance of the bile ducts. Patient underwent endoscopic retrograde cholangiopancreatography (ERCP) for further evaluation. ERCP showed multiple filling defects on the cholangiogram that disappeared with contrast injection. Biliary sphincterotomy and balloon sweep of the biliary duct was negative other than blood clots. The filling defects remained in place despite multiple sweeps. Digital cholangioscopy (Spyglass) was introduced into bile duct that showed mucosal changes consistent with biliary varices. Four days post-procedure the patient developed melena with acute anemia. Repeat ERCP showed evidence of bleeding from sphincterotomy site and again biliary tree sweep revealed intraductal blood clots. Spyglass was introduced again with evidence of biliary varices without active bleeding. A covered metal stent was placed to help control post-sphincterotomy bleeding. The bleeding stabilized and the patient’s total bilirubin dropped from 14.2 to 8.9 over the following three days. CT A/P showed patent vessels with no evidence of portal vein thrombosis.
Discussion: Portal biliopathy is a biliary abnormality caused by portal cavernous malformation and biliary varices in patients with portal hypertension1. Biliary varices have been reported to be more common in patients with portal vein thrombosis rather than cirrhotic portal hypertension2,3. Majority of patients are asymptomatic, but a smaller subset of patients can present with jaundice, fever, or strictures. This diagnosis can be frequently overlooked which highlights the need to keep a high index of suspicion for this rare diagnosis when the cause for biliary obstruction or bleeding in patients with cirrhosis is unclear.
