Pan-Mesenteric Thrombosis Causing Portal Biliopathy: Case Series from a Tertiary Pancreato-Biliary Center

Abstract

Purpose: Portal biliopathy is a term used to describe biliary ductal and gallbladder wall abnormalities seen in patients with portal hypertension. These changes are seen in patients with extrahepatic portal vein obstruction (EHPVO). We report a case series of portal biliopathy diagnosed and treated endoscopically in our institution. A 35-year-old male with a past medical history significant for idiopathic portal hypertension requiring splenectomy and splenorenal shunt during childhood and chronic portal vein thrombosis, was found to have elevated ALP of 1,015 U/L on annual check-up without jaundice or pruritus. Exam showed no signs of chronic liver disease. Other liver enzymes were mildly elevated. His liver tests had previously been normal. He underwent magnetic resonance cholangiopancreatography (MRCP), which showed intrahepatic biliary dilatation and mesenteric edema (Figure 1A and 1B). Transabdominal ultrasonography showed cirrhosis and cavernous transformation. Investigations for the etiology of cirrhosis, hypercoagulable state, and myeloproliferative disorders were unremarkable. Endoscopic ultrasonography (EUS) revealed no mass at the stricture. He underwent endoscopic retrograde cholangiopancreatography (ERCP), which showed a highgrade stricture in the distal bile duct that was treated with temporary biliary stenting (Figure 1C). Postoperatively, he had significant improvement in the liver enzyme levels (Figure 1D). He remained asymptomatic at a 2-year follow-up visit. Between 2000 and 2012, we identified four patients with portal biliopathy. Clinical presentation, liver enzymes, and imaging findings are demonstrated in Table 1. This case series highlights the approach to an uncommon cause of biliary obstruction. Portal biliopathy is more common in patients with EHPVO than in patients with idiopathic portal hypertension or cirrhosis. For patients with EHPVO, who develop biliary symptoms, abnormal ALP activity or dilated bile ducts, MRCP is the initial investigation of choice. Patients with evidence of biliary obstruction by MRCP should undergo EUS to delineate the etiology of the obstruction. If portal biliopathy is confirmed, the treatment of choice is porto-caval shunting. ERCP with temporary biliary stent placement is the treatment of choice in patients who are not candidates for the porto-caval shunting.FigureTable 1: Case series of portal biliopathy at University of Minnesota