A 12 year old boy presents with hemetemesis, melena and mild splenomegaly. There is no obvious jaundice or ascites. The most likely diagnosis is
Correct Answer: EHPVO
Description: Extrahepatic poal venous obstruction (EHPVO) EHPVO is a vascular disorder of the liver Obstruction of the extrahepatic poal vein with or without involvement of the intrahepatic poal veins or splenic or SMV Most common site of obstruction is at the confluence of splenic vein and SMV EHPVO is the most common cause of upper Gastrointestinal bleeding in children Accounts for almost 70: of pediatric patients with poal hypeension Clinical features It can present in two forms Recent EPHVO : Abdominal pain, ascites or fever, may be asymptomatic Chronic EPHVO : Repeated, well tolerated bleeding episodes from esophageal varices Ectopic varices are common (duodenum, anorectal region, biliary tree and gallbladder) manifest as obscure GI bleed, bleeding per rectum or biliary obstruction Moderate splenomegaly is universal and may be a presenting feature A propoion of children have growth retardation Ref: Blumga 5th edition Pgno : 1099-1105
Category:
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