Which among the following is the inheritance pattern of Caroli's syndrome?
High-Yield Explanation
Caroli's syndrome is a congenital syndrome and often follows an autosomal recessive pattern of inheritance. It is associated with peripoal hepatic fibrosis and cystic kidney disease. Caroli's disease is characterised by saccular dilatation of segmental intrahepatic bile ducts, without other hepatic abnormalities. Both patients presents with right upper quadrant pain, recurrent bouts of cholangitis, and formation of calculi in the dilated ducts. Diagnosis i smade by MRCP or contrast cholangiography. Ref: Teaching Atlas of Interventional Radiology: Non-Vascular Interventional Procedures edited by Saadoon Kadir, page 178.