Management Portal hypertension		Portal hypertension is defined as a portal vein (PV) pressure > 5 mm Hg or PV-to-hepatic vein gradient > 10 mm Hg. The site of the PV obstruction can be presinusoidal (e.g. post UV catheterization in the neonate), postsinusoidal (e.g. Budd-Chiari syndrome) or sinusoidal (e.g. cirrhosis). Cirrhosis is the most common cause of portal hypertension in pediatrics. Portal hypertension can be diagnosed on physical exam by the presence of enlarged portal-to-systemic collateral veins. Periumbilical vein collaterals are manifested by superficial abdominal wall veins that radiate from the umbilicus - the caput medusa. Perirectal collaterals lead to the development of hemorrhoids. Splenomegaly from splenic vein collaterals is common, but remember in the embryonic type of biliary atresia, there may be asplenia, polysplenia or situs inversus. Esophageal varices, from esophageal vein collaterals, are best seen on endoscopy. Ultrasound is useful in evalutaing the size of the portal and splenic veins. Doppler flow studies are especially helpful. Portal blood flow away from the liver is associated with severe portal hypertension. Creation of a portosystemic shunt is the only way to reduce the PV pressure. An important consideration is to be certain the shunt surgery does not compromise a potential future liver transplant procedure. Complications are worsening hepatic failure from the decreased portal blood flow and precipitation of hepatic encephalopathy. Return to top of page