A 40-year-old woman presents with recurrent right upper quadrant pain, jaundice, and cholangitis. MRCP reveals multiple intrahepatic biliary strictures with saccular dilatations and a normal extrahepatic duct. Which classification system best describes the pattern of her disease and guides surgical planning?
- A Bismuth-Corlette classification
- B Todani classification of choledochal cysts
- C Amsterdam criteria for primary sclerosing cholangitis
- D Caroli disease — Type V in Todani classification ✓
Explanation
Caroli disease is characterised by non-obstructive saccular (cystic) dilatations of the intrahepatic bile ducts in a segmental or diffuse distribution with a normal extrahepatic duct. It corresponds to Todani Type V choledochal cyst classification. Caroli syndrome (Caroli disease + congenital hepatic fibrosis) carries portal hypertension risk. When disease is confined to a lobe or segment, hepatic resection is curative; diffuse disease requires liver transplantation. The Bismuth-Corlette classification applies to hilar cholangiocarcinoma. PSC shows multifocal strictures on MRCP but is an autoimmune-inflammatory condition without true saccular dilatations.
Reference: Bailey & Love's Short Practice of Surgery, 27th ed.
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Written and medically reviewed by the StethoPrep medical team.