Which genetic condition involves segmental saccular dilatation of intrahepatic ducts?

Caroli's Disease is characterized by the presence of segmental saccular dilatation of the intrahepatic bile ducts. This condition is a congenital disorder that results in cystic dilations or outpouchings of the bile ducts within the liver. The dilatations can be segmental, meaning they affect specific areas or segments of the liver's bile duct system, rather than being uniformly distributed.

As a result of this dilatation, patients may experience symptoms such as recurrent episodes of cholangitis (infection of the bile ducts) and may have a higher risk for complications, including liver dysfunction or the development of biliary stones. The recognition of this pattern of intrahepatic bile duct dilation is crucial for diagnosis and management.

In contrast, the other conditions listed do not primarily feature segmental saccular dilatation of the intrahepatic ducts. Mirizzi Syndrome involves obstructive jaundice due to a stone in the cystic duct compressing the common bile duct, cholangitis is primarily an infection that occurs within existing bile duct structures, and biliary atresia is a condition where there are absent or underdeveloped bile ducts, leading to a different pathology.