Recurrent Biliary Obstruction Secondary to Portal Vein Thrombosis With Cavernous Transformation and the Role of Cholangioscopy in Diagnosis: Case Report

Abstract

Portal vein thrombosis with cavernous transformation is a rare cause of biliary obstruction. Portal biliopathy is a term that refers to biliary abnormalities associated with portal vein hypertension and is most commonly caused by extrahepatic portal vein thrombosis. Patients may be asymptomatic, but can also present with abdominal pain, jaundice, and fever. We describe a case of biliary obstruction secondary to portal vein thrombosis with cavernous transformation who presented with recurrent jaundice. A 61 year-old female with history of coronary artery disease presented with generalized weakness and jaundice of three days duration. She had associated nausea and vomiting. Physical examination was notable for scleral icterus. Initial work-up was revealed markedly elevated total bilirubin and alkaline phosphatase. Abdominal and MRCP showed extrahepatic and intrahepatic biliary ductal dilatation due to short segment stenosis of the distal common bile duct. CT scan of the abdomen with IV contrast demonstrated a large tortuous splenic vein as well as numerous dilated veins extending into the region of the porta hepatis. Bilirubin and alkaline phosphatase levels rapidly improved and the patient was discharged with a plan for follow-up MRCP. Two weeks later, she presented with recurrence of her jaundice. ERCP was performed which showed the common bile duct and intrahepatic ducts to be dilated, and a plastic stent was placed after which the total bilirubin and alkaline phosphatase levels decreased. She then developed cholangitis and underwent ERCP with cholangioscopy, which showed extrinsic compression at the mid bile duct likely from the portal venous cavernoma, establishing the diagnosis of portal biliopathy. A metal stent was placed. Subsequent Doppler ultrasound of the liver revealed multiple venous collaterals seen in the porta hepatis, consistent with cavernous transformation. The patient was started on warfarin with resolution of her symptoms and normalization tests. She has had no recurrence at 4 months of follow-up. Workup was negative for an underlying hypercoagulablity. Portal vein thrombosis with cavernous transformation may rarely be associated with the development of portal biliopathy. There should be high suspicion for this diagnosis in people who present with obstructive jaundice and have risk factors for thrombosis. Cholangioscopy may be helpful in making the diagnosis. Endoscopic biliary stenting may be uitilized for treatment.Figure: CT abdomen with contrast suggestive of portal vein thrombosis.Figure: MRCP showing a marked extrahepatic and intrahepatic ductal dilatation.Figure: Doppler ultrasound of the liver revealed multiple venous collaterals seen in the porta hepatis, consistent with cavernous transformation.