Decompensated liver failure due to portal hypertension as a result of hepatic arteriovenous malformations

Portal hypertension is usually seen because of liver cirrhosis, causing a plethora of symptoms such as ascites and oesophageal varices. However, altered hepatic vasculature can affect the portal venous pressure and thereby cause portal hypertension, giving rise to similar symptomology. This paper presents a case of recurring severe gastrointestinal (GI) bleeding, ascites and oesophageal varices in a patient with hepatic arteriovenous malformations (HAVM). Physical examination, liver biopsy, clinical imaging and genetic testing disproved hereditary haemorrhagic telangiectasia (HHT) and liver cirrhosis. Bevacizumab (BVZ) was initiated on the basis of experience from treating vascular malformations in HHT patients. The patient has not shown signs of GI bleeding since the initiation of BVZ. Genetic testing detected a mutation in the EPHB4 gene of previously unknown significance, but a connection with vascular malformations has been suggested in the literature. Collectively, this case calls for considering hepatic vascular malformations in patients with non-cirrhotic portal hypertension.