CONCLUSIONS
A definitive diagnosis of Caroli syndrome is only possible through CT or
MRCP or histological examination of the liver specimen, which remains a
challenge in the resource-limited settings. It is important to consider
Caroli’s syndrome in the differential diagnosis in patients presenting
with features of portal hypertension. Delay in treatment can lead to
severe life threatening hematemesis, cholangitis or even cancer. Slow
and silent progression of the disease warrants regular follow-up.