A228 WHEN ASCITES & VARICEAL BLEEDING ARE NOT FROM CIRRHOSIS: A CASE OF MUTIPLE ARTERIOPORTAL FISTULAE CAUSING PORTAL HYPERTENSION

Abstract

Abstract Background Portal hypertension is usually due to increased resistance from cirrhosis. However, pressures can also be elevated due to increased flow. Aims To describe a peculiar case of non-cirrhotic portal hypertension. Methods A case report and literature review was performed. Results A 47-year-old previously well man presented with a 6 month history of rapidly progressive weight loss, ascites and variceal bleed. Workup ruled out common causes of primary liver disease. Initial imaging demonstrated a heterogenous liver, splenomegaly, ascites, patent hepatic/portal veins and multiple poorly defined low-density hepatic lesions with the largest measuring 2.1 cm. Transient elastography was 7.3 kPa (F1-mild fibrosis). At transjugular liver biopsy, hepatic venogram ruled out Budd-Chiari and hepatic vein pressure gradient was normal at 3–4 mmHg. Histology unfortunately showed hemangioma. A percutaneous liver biopsy suggested nodular regenerative hyperplasia, minimal fibrosis and mild cholestasis. Given worsening ascites, hyponatremia and 7 months of rapidly progressive decline, transjugular intrahepatic portosystemic shunt (TIPSS) was inserted. Intra-procedure, portal vein pressure was noted to be 51 mmHg, with a portosystemic gradient of 42 mmHg. Although numerous abdominal CT and MRI did not show AV shunting, ultrasound post-TIPSS showed hepatic pseudoaneurysms & arterioportal fistulae (APF). Direct angiogram showed numerous hepatic pseudoaneurysms and intrahepatic fistulae making embolization impossible. CT showed no evidence of pseudoaneurysms or fistulae outside of the liver. Workup for autoimmune rheumatological diseases and congenital telangiectatic syndromes were negative. Given the high pressures being directed through the new TIPSS, right heart failure is an ongoing concern. APF are rarely encountered causes of presinusoidal portal hypertension, with communications most commonly arising from the hepatic (65%) & splenic arteries (11%) & the portal vein. Causes include traumatic (28%), iatrogenic (16%), vascular/telangiectatic malformations (15%), tumors (15%), aneurysms (14%) & congenital disease. Endovascular embolization can be used to treat single lesions. In complex cases with mulitple APF, surgery and/or liver transplantation may be required. Conclusions We report a rare case of non-cirrhotic portal hypertension due to increased flow rather than increased resistance secondary to APF. Funding Agencies None