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.
Veno occlusive disease
Budd chiari syndrome
Cirrhosis liver
Non cirrhotic portal fibrosis