1440 An Uncommon Cause of Biliary Obstruction in a Patient With Budd-Chiari Syndrome

Abstract

INTRODUCTION: Biliary varices (BVs) are an infrequent complication of chronic portal hypertension (PHTN). PHTN causes collateral paracholedochal veins to dilate and protrude into the common bile duct (CBD), forming varices. Most cases of BVs are asymptomatic and are therefore likely underdiagnosed. However, protrusion of BVs into the CBD can lead to clinically significant biliary obstruction. CASE DESCRIPTION/METHODS: A 34-year-old female with polycythemia vera and Budd-Chairi syndrome status-post portocaval shunt on chronic warfarin presented with a 1-day history of right upper quadrant pain and non-bloody, non-bilious emesis. She was febrile to 39.9 C and had a soft, but distended abdomen without icterus or asterixis. Labs were notable for total bilirubin 4.1 mg/dL, direct bilirubin 1.2 mg/dL, AST 79 U/L, ALT 69 U/L, INR 1.8, and alkaline phosphatase of 207 U/L. Abdominal ultrasound with Doppler demonstrated a patent portacaval shunt, splenomegaly, and stable prominent portosystemic varices causing limited visualization of the CBD. ERCP for further visualization showed a filling defect of the middle third of the CBD. A biliary stent was deployed, causing hemorrhage within the CBD, raising the possibility of an intraductal varix. Hemostasis was achieved with subsequent metal stent placement. Further investigation with venography displayed multiple infrahepatic IVC collateralization and two regions of significant stenosis along the portal-IVC stent and intrahepatic IVC. This resulted in diversion of blood flow through the azygos system and ultimately giving rise to her CBD varices. Balloons were used for venoplasty resulting in successful reduction of the regions of stenosis and improvement of the pressure gradients. DISCUSSION: The presence of BVs can complicate surgical and endoscopic procedures of the biliary tract. In this case, endoscopic intubation of the duct during ERCP caused unexpected hemobilia as BVs were not suspected in this patient. Data on management of biliary varices is limited. In our patient, stent placement was performed to both achieve hemostasis and relieve the biliary obstruction. Venography was done to diagnose and treat IVC stenosis. This ultimately improved pressure gradients in the collateral circulation and should prevent progression of BVs. History of PHTN should increase clinical suspicion for BVs in patients presenting with biliary obstruction. If a biliary tract procedure is required, pre-procedure imaging for variceal screening should be considered to prevent complications.