CAROLI DISEASE

Course overview

CAROLI DISEASE IS A RARE INHERITED DISORDER INVOLVING SEGMENTAL DILATATION OF LARGE, INTRAHEPATIC BILE DUCTS WHICH APPEAR AS CYSTS ON IMAGING AND HISTOPATHOLOGIC EXAM. THE GENE INVOLVED IS THE SAME GENE RESPONSIBLE FOR AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE (ARPKD); HENCE, THESE CONDITIONS COMMONLY CO-OCCUR. CAROLI DISEASE IS USUALLY DIAGNOSED DURING ADOLESCENCE, AND PATIENTS TYPICALLY PRESENT WITH RECURRENT EPISODES OF CHOLANGITIS. THIS ACTIVITY DESCRIBES THE PATHOPHYSIOLOGY OF CAROLI DISEASE AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN ITS MANAGEMENT.