Caroli disease is an infrequent congenital pathology that is part of the spectrum of fibrocystic diseases, characterized mainly by malformation of bile ducts. These patients often have stones and cholangitis, which respond poorly to conventional endoscopic retrograde cholangiopancreatography. To date, there is little evidence on the usefulness of cholangioscopy in this disease, so we describe the experience of performing cholangioscopy in a patient with hepatolithiasis and Caroli disease.
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