Multiple Cerebral Aneurysms in an Adult With Autosomal Recessive Polycystic Kidney Disease

Autosomal recessive polycystic kidney disease (ARPKD) is a rare form of fibrocystic kidney disease that affects approximately 1 in 20,000 live births.1,2 The classic presentation is with prominent hepatic impairment caused by hepatic fibrosis in addition to renal impairment that is more aggressive than that seen in autosomal dominant polycystic kidney disease (ADPKD). Caroli syndrome is the eponym given to the combination of ARPKD, hepatic fibrosis, portal hypertension, and intrahepatic bile duct dilatation.