LAUSR

Background Cavernous transformation of the portal vein is a rare entity and usually is a sequela of EHPV. We report a case of a 21 year old Filipino female presented with a history of intermittent hematemesis which started when she was 1 year old. Pre-natal and birth history was unremarkable. She had recurrent episodes of upper GI bleeding even at a young age. No other stigmata of liver disease were noted. Endoscopic band ligation bleeding varices was done. Liver function tests, Hepatitis B and C screening were negative. Abdominal MRI and CT scan revealed portal cavernoma and absence of cirrhosis. Methods We report a case of a young female adult in whom the diagnosis of CTPV was made after extensive workup for the case of her recurrent upper GI bleeding and signs of portal hypertension in the absence of cirrhosis and other stigmata of chronic liver diseases. Results Complete blood count revealed bicytopenia and adequate platelets with microcytic, hypochromic anaemia. PBS revealed Microcytic cells with hypochromicity, anisocytosis and poikilocytosis. Liver function tests, Hepatitis B and C screening were negative. Fibroscan of the liver revealed intermediate fibrosis. Colour flow Doppler ultrasound of the abdomen is compatible with portal hypertension of the portal vein and splenomegaly. MRI of the whole abdomen revealed portal hypertension with marked splenomegaly, cavernous transformation of the portal vein and collateral vessel formation. There’s also focal biliary ectasia and cholecystolithiasis. Repeat gastroscopy revealed 3 esophageal varices. Conclusions In conclusion, NCPH is the most common cause of portal hypertension next to cirrhosis. Two disease entities in NCPH, namely NCPF/IPH and EHPVO are distinct diseases, presenting with features of PHT – variceal bleed, splenomegaly and near normal liver functions. Likely pathogenesis is early age, portal inflammation/infection in a prothrombotic individual although we still have to work up this patient for thrombotic risk. The diagnosis needs exclusion of cirrhosis in NCPF/IPH and presence of portal cavernoma in EHPVO. Slow hepatic dysfunction due to parenchymal extinction and portal biliopathy is a late event in EHPVO. Effective management of PHT and its complications results in excellent 5 and 10 years survival.