A young boy presented with severe hematemesis. On examination, there was no hepatomegaly, Mild splenomegaly is present. Endoscopy shows esophageal varices. The most probable diagnosis -

Splenomegaly, variceal bleeding in the absence of hepatomegaly suggest the diagnosis of non-cirrhotic portal fibrosis. Non-Cirrhotic Portal Fibrosis Its incidence is very high in India accounts for 15% to 18% of all patients with portal hypertension. Aetiology : Infection → It was suggested that E.coli can cause NCPF Exposure to chemicals → Chronic ingestion of Arsenic is incriminated in causing NCPE Vinyl Chloride toxicity can also cause NCPF Clinical features : Symptoms : Patients are usually young in 3rd or 4th decade of life. Gastrointestinal haemorrhage is the most common presenting symptom seen in 60-90% of patients. Lump in the left side of Abdomen d/t enlargement of the spleen Jaundice & Ascites uncommon and if they do occur it is for a short period of time. Signs Splenomegaly (usually massive) The liver usually normal in size Sometimes mild ascites can be seen. Endoscopy : Esophageal varices are detected in over 90% of patients Rectal varices in 70% of patients.