Caroli's syndrome leading to splenic artery aneurysm: a rare presentation.

Splenic artery aneurysm is a rare disorder (0.7%) that arises mainly as a sequlae to portal hypertension. Other causes of splenic artery aneurysm are atherosclerosis, arterial wall injury due to trauma, pancreatitis, and medial dysplasias of the wall. However, though Caroli's disease is known to cause portal hypertension, the rise of vascular pressure leading to aneurysm is not yet reported (extensive Medlar search failed to reveal any publication). Every effort should be made to diagnose this condition as early as possible because 25% of ruptured splenic aneurysms are fatal. A unique case of Caroli's disease giving rise to splenic artery aneurysm and its possible pathogenesis is reported.