An 18-year-old female with recurrent esophageal variceal bleeding.

An 18-year-old female with recurrent esophageal variceal bleeding due to extrahepatic portal venous obstruction was referred to us for surgical management. On general physical examination she was pale. There was no jaundice or lymphadenopathy. Abdominal examination disclosed a massive splenomegaly (12 cm below the costal margin). Laboratory tests revealed a hemoglobin of 85 g/L, a total leucocyte count of 4.2×109/L and the platelets were 30×109/L. Liver function tests and other biochemical investigations were within normal limits. Color Doppler sonography revealed multiple collateral channels replacing the portal vein (portal cavernoma) and massive splenomegaly. Furthermore, it demonstrated multiple, small foci of echogenic nodules with no acoustic shadowing in the spleen. Non-enhanced CT divulged massive splenomagaly with a pressure effect on the ipsilateral kidney and multiple, discrete, millimetric hyperdense spots in the spleen (Figure 1). A contrast-enhanced splenoportovenogram confirmed the presence of portal cavernoma, esophageal varices, multiple perisplenic collaterals and extensive thrombosis of the splenoportal venous axis. A diagnosis of extrahepatic portal vein obstruction leading to portal hypertension was made.