Morbidity
Caroli disease is typically found in Asia and diagnosed in persons under the age of 22. Cases have also been found in both infants and adults. As medical imaging technology improves, diagnostic age decreases. Morbidity is common and is caused by complications of cholangitis, sepsis, choledocholithiasis, and cholangiocarcinoma. These morbid conditions often prompt the diagnosis. Portal hypertension may be present, resulting in other conditions including splenomegaly, hematemesis and melena. These problems can severely affect the patient's quality of life. In a ten year period between 1995 and 2005, only ten patients were surgically treated for Caroli disease, with an average patient age of 45.8 years.
After reviewing 46 cases of Caroli disease before 1990, it was found that 21.7% of the cases were the result of an intraheptic cyst or non-obstructive biliary tree dilation, 34.7% were linked with congenital hepatic fibrosis, 13% were isolated choledochal cystic dilation, and the remaining 24.6% had a combination of all three.
Mortality is indirect and caused by complications. After cholangitis occurs, patients typically die within approximately 5–10 years.
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