A 22 year old male presented with repeated episodes of hematemesis. There is no history of jaundice or liver decompensation. On examination the significant findings include massive splenomegaly and presence of esophageal varices. There is no ascites or peptic ulceration. The liver function tests are normal. The most likely diagnosis is:-
Correct Answer: Non-cirrhotic poal fibrosis
Description: Non-Cirrhotic Poal HTN- Types* EHPVO (Extra Hepatic Poal Venous Obstruction)* NCPF (Non-Cirrhotic Poal Fibrosis)Similarities Between EHPVO and NCPF- No ascites- No jaundice- No encephalopathy- No hepatic failure - Varices present- Splenomegaly Difference Between EHPVO and NCPF EHPVO NCPF - 1st and 2nd order branches are involved- M/C site of obstruction - Confluence of splenic vein and Superior mesenteric vein- Seen in 1-2nd decade- Splenomegaly is less common (Mild to Moderate) - Involvement of 3rd and 4th outer branches.- Seen in 2nd-4th decade- Substances that increases the risk of NCPF* V - Vinyl chloride* A - Arsenic* C - Copper- Splenomegaly is more common (Moderate to Massive)
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