1923 Bleeding Varices in a Non-Cirrhotic

Abstract

INTRODUCTION: Extra-hepatic portal vein obstruction (EHPVO) presents with manifestations of portal hypertension, including variceal bleed. Cirrhosis is the leading cause of portal hypertension (90%), and EHPVO can mask as cirrhosis, especially in a patient with risk factors such as alcohol abuse and incarceration. CASE DESCRIPTION/METHODS: 37 y/o male with h/o DVT and alcohol abuse presented to our ER for abdominal pain and hematemesis. He was a chronic alcoholic but quit drinking 6 months prior. On admission, he was afebrile, mildly tachycardic but not hypotensive. Examination showed non-specific diffuse abdominal tenderness and jaundice. Labs showed Hb 5.8 g/dL, platelets 66/mcL, INR 2.13. Liver enzymes were normal, except T Bili of 5.1 mg/dL, and albumin 3.8 g/dL. Entire cirrhotic work-up was negative. CT abdomen showed nodular liver, splenomegaly, mild ascites, and gastroesophageal varices. Doppler US showed hepatopedal flow within the main portal vein. Triple-phase CT showed portal vein thrombosis (PVT) with cavernous transformation. EGD revealed large esophageal varices with red wale sign, which were banded. No gastric or duodenal varices. Colonoscopy revealed diffuse melena up to the terminal ileum, but no varices. Liver biopsy was negative for cirrhosis. The patient was found to have a heterozygous Factor V Leiden (FVL) gene mutation. Anticoagulation was not initiated due to recent bleed. He was re-admitted, unconscious after massive GI bleeding at home. Repeat banding was performed. SMA angiography and portography showed complete occlusion of the main SMA, splenic vein, and PV. DISCUSSION: For patients with portal hypertension secondary to portal vein thrombosis, the Bevano VI guidelines on portal hypertension recommend primary prophylaxis with non-selective beta-blockers and endoscopic variceal ligation. Failure of primary prophylaxis requires secondary prophylaxis with TIPS. However, surgical devascularization, such as the modified Sugiura procedure, is a great option with lower rates of re-bleeding and higher rates of survival compared to surgical shunts. Decompressive portal therapy is an important treatment aspect for our patient due to his need for lifelong anticoagulation in the setting of inherited thrombophilia.