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 -: (A) Veno occlusive disease, (B) Budd chiari syndrome, (C) Cirrhosis liver, (D) Non cirrhotic portal fibrosis
Answer is D. 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.