A 20-year-old man presents with repeated episodes of haematemesis. There is no history of jaundice or liver decompensation. On examination, the significant findings include moderate splenomegaly and presence of oesophagal varices. There is no ascites or peptic ulceration. The liver function tests are normal. What is the most likely diagnosis

CAROLI'S DISEASE is congenital, nonfamilial, multiple, irregular, dilatation of the intrahepatic ducts with stenotic segments in between. It is associated with congenital hepatic fibrosis and medullary sponge kidney. * It can also be included under type V choledochal cyst.it is a hepatic cause of poal hypeension. ref: SRB&;s manual of surgery, ed 3, pg no 569