Extrahepatic portal venous obstruction, although rare in the western world, is a common cause of major and life threatening upper gastrointestinal bleeding among the poor in developing countries. Patients have large spleens and stunted growth. The diagnosis is easily confirmed by Doppler ultrasonography. Endoscopy sclerotherapy is the best option for the control of acute variceal bleeding. For secondary prophylaxis of bleeding, the choice lies between repeated sclerotherapy and a portosystemic shunt. We believe that due consideration should be given to performing a splenectomy and a lienorenal shunt. Performed by experienced surgeons, it carries a low operative mortality of 1%, a rebleeding rate of about 10%, removes the large spleen, reverses hypersplenism, and is not followed by portosystemic encephalopathy. Most importantly, it is a onetime procedure particularly suited to those who have little access to blood transfusion and sophisticated medical facilities. 1. Introduction Extrahepatic portal venous obstruction (EHPVO) is accompanied by replacement of the extrahepatic portal vein by a cavernoma with or without thrombosis of the intrahepatic portal, splenic, or superior mesenteric veins. In developing countries, EHPVO has been reported to be the most common cause of upper gastrointestinal bleeding (UGIB) in children (70% in some reports) and is also a common cause of variceal bleeding in adults [1]. In western countries, EHPVO is second only to cirrhosis as a cause of portal hypertension, but its relative incidence is much lower compared with that in the developing countries. Its aetiology is still not clear but has been attributed to umbilical sepsis after birth with thrombosis extending to the portal system via the patent umbilical vein or portal pyaemia following intra-abdominal sepsis. However, notwithstanding a lack of knowledge about its cause, most children and adults with EHPVO are generally from the so-called lower economic strata [2]. Variceal bleeding in EHPVO usually occurs in the first or second decade of life [15]. However, the outcome after a bleed is better compared to bleeding in cirrhotics (if adequate blood replacement facilities are at hand), because patients with EHPVO have normal liver function (and histology) which helps them to sustain bleeding episodes without decompensation [16]. However mortality rates of between 5 and 30% have been reported for a single bleeding episode because of the large volumes of blood lost in patients who do not have access to sophisticated medical facilities including blood transfusion [17]. Till
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