A 23 year old male has presented with repeated episodes of hematemesis. There is no history of jaudice or liver decompensation. On examination ther significant findings include splenomegaly (8 ems below costal margin), and presence of esophageal varices. There is no ascites or peptic ulceration. The liver function tests are normal. The most likely diagnosis is
Answer is B (Non cirrhotic poal fibrosis) : Features of poal hypeension in an adult with a large splenomegaly (8cm below coastal margin) with no history of jaundice or liver decompensation suggests a diagnosis of Non-cirrhotic poal hypeension. Non cirrhotic poal hypeension encompasses two distinct pathological condition that present with similar clinical features. Distinction between the two conditions should ideally he made byliwther investigations as the similarly in presentation makes clinical criteria unreliable. Nevehless if we do have to choose one single best option the following clues may be helpfitl. NCPF NCPF presents in young adults most commonly during the third and fouh decade Mode of presentation The onset of symptoms is gradual Mass in left upper quadrant (splenomegaly) is about 4 times more common in NCPF than EHPH Extrahepatic poal hypeension llPH may present in two age groups children (Ist & 2nd decade) (congenital malformations) Adults (4th & 5th decade) (thrombotic event) The onset is gradual in children where the cause is congenital malformation The onset is acute in adults where the cause is usually a thrombotic even Mass in left upper quadrant (splenomegaly) is four times less common in patients with EHPH Thus a young adult presenting with a gradually progressive cause and a moderate to massive splenomegaly suggests the diagnosis of Non cirrhotic poal fibrosis.
Extahepatic poal venous obstruction
Non cirrhotic poal fibrosis
Cirrhosis
Hepatic venous outflow tract obstruction