Question

An 18-year-old boy presents with massive hematemesis, and the spleen is evidently enlarged up to the umbilicus. He is then found to have varices on esophagoscopy. What could be the diagnosis?

• A. Budd-Chiari syndrome
• B. Veno-occlusive disease
• C. Non-cirrhotic portal fibrosis
• D. Cirrhosis of the liver

Hint:

Massive, well tolerated splenomegaly with normal liver function in a young patient points to non-cirrhotic portal fibrosis.

Answer 1

The Correct Option is C

Step 1: Understanding the Question:
An 18-year-old boy has massive hematemesis (vomiting blood), a spleen enlarged all the way to the umbilicus, and varices on esophagoscopy. We need the diagnosis that best fits this picture.

Step 2: Key Formula or Approach:
Massive splenomegaly plus esophageal varices in bleeding points to portal hypertension. The job here is to pick which cause of portal hypertension best fits a young patient with a huge spleen but no other clue pointing to liver failure.

Step 3: Detailed Explanation:
Budd-Chiari syndrome is caused by hepatic vein outflow obstruction and usually presents with painful hepatomegaly, ascites, and abdominal pain, not with a spleen this massive as the leading feature.
Veno-occlusive disease affects the small hepatic veins, often after bone marrow transplant or exposure to certain toxins, and again presents mainly with tender hepatomegaly and ascites rather than a dramatic splenomegaly.
Cirrhosis of the liver in an 18 year old would usually come with signs of chronic liver cell failure, such as jaundice, ascites, and a shrunken or nodular liver, none of which is mentioned here.
Non-cirrhotic portal fibrosis (NCPF) is a condition seen mostly in young people from the Indian subcontinent, where portal hypertension develops from fibrosis of the small portal vein branches while the liver cells themselves stay healthy. Patients present exactly like this: a massive, well tolerated splenomegaly, repeated bouts of hematemesis from esophageal varices, and normal liver function, because the liver parenchyma is spared.

Step 4: Final Answer:
The clinical picture of a young patient with a huge spleen, variceal bleeding, and preserved liver function fits non-cirrhotic portal fibrosis best.