S2427 Umbilical Variceal Hemorrhage: An Uncommon First Presentation of Cirrhosis

Abstract

INTRODUCTION: Ectopic varices are dilated portosystemic collateral veins that develop outside of the gastro-esophageal region. They occur most commonly in the small bowel, colon, and peritoneum. Management of ectopic varices is complex given their diversity of location, presentation and complications. Our case describes a life-threatening hemorrhage from an umbilical varix as a first presentation of cirrhosis, and its management challenges. CASE DESCRIPTION/METHODS: A 64-year-old man without known liver disease presented to the hospital with spontaneous bleeding from his umbilicus and syncope. Two weeks prior to admission, he noted bleeding from a periumbilical purple blister that was sutured at an outside hospital, but he developed a second lesion in the area which bled on the day of admission. His history was notable for obesity, hypertension and peripheral artery disease, with left above the knee amputation and a right superficial femoral artery stenting. Relevant medications included Aspirin and Ticagrelor. He was drinking half a pint of whiskey and 2–3 beers daily. His physical exam showed a reducible umbilical hernia with an adjacent bulge that was actively bleeding. He had grade 2 hepatic encephalopathy (HE) with asterixis. Labs were notable for hemoglobin 5.6 g/dL (baseline 11 g/dL), platelets 110,000/mm3, bilirubin 0.7 g/dL, albumin 2.1 g/dL and INR 1.2. Abdominal CT revealed cirrhosis, splenorenal shunt and an ectopic varix coursing through an umbilical hernia. His echocardiogram showed enlarged right ventricle. He was treated with octreotide, evaluated by interventional radiology, and several therapeutic options were considered. Given his anatomical features, liver function and comorbidities, a small caliber transjugular intrahepatic portosystemic shunt (TIPS) was inserted to allow coil embolization of the varix. At the time of TIPS, his MELD-Na score was 15 and Child-Pugh class B. He had no further bleeding, encephalopathy or cardiac complications, and was discharged after one week. DISCUSSION: Ectopic varices are uncommon and their management can be challenging as local treatment is not effective against preventing rebleeding. TIPS, embolization, sclerotherapy, or balloon occlusion retrograde transvenous obliteration can be used, based on patients’ characteristics. In our patient with HE and cardiac dysfunction, a small caliber TIPS with adjunctive variceal embolization was well tolerated and provided definitive therapy for bleeding from ectopic varices.