Management of extra hepatic portal venous obstruction (EHPVO): current strategies

Extrahepatic portal venous obstruction (EHPVO) is the commonest cause of portal hypertension and variceal bleeding in children. Though mortality related to variceal bleeding is uncommon, morbidity due to massive splenomegaly with hypersplenism, growth failure, ectopic varices like rectal varices and portal biliopathy is significant. A significant proportion of cases in adults are due to procoagulant state but the same has not been documented in children. Studies in children have shown that hereditary or acquired coagulation disorders do not play a role in the pathogenesis of EHPVO in children. Regarding endotherapy for variceal bleeding, there is no doubt that band ligation is superior to sclerotherapy. Nevertheless, a combination of band ligation followed by sclerotherapy has shown to be superior to either modality in children with EHPVO. Growth retardation due to growth hormone resistance is common in children with EHPVO. Diminished portal blood flow results in decreased insulin delivery to the liver and thereby decreased production of insulin-like growth factor-1 (IGF-I) and insulin-like growth factor binding protein-3 (IGFBP-3). Improvement of growth after restoration of hepatic blood flow with mesenteric-left-portal bypass or Rex shunt, has been documented. Portal biliopathy is universal in adults and common in children but symptomatic cases are mainly in adults; thereby suggesting a progressive nature of the condition. Symptomatic biliary obstruction can be managed endoscopically but shunt surgery followed by biliary bypass (if necessary) seems to be the best management option. With the availability of the most physiological shunt (mesenteric-left-portal bypass or Rex) the management paradigm of EHPVO has changed from endotherapy to primary shunt surgery.