Role of Cholangioscopy in a Patient With Hepatolithiasis and Caroli Disease

Abstract

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.

Figure 1.

T2-weighted images obtained from cholangioresonance, showing slight lobulation of the hepatic contours and atrophy of the right hepatic lobe, associated with the loss of normal morphology of the intrahepatic bile ducts, with the presence of irregular cystic dilations and some defects inside them that are consistent with stones (marked with arrows in A, B, and C). (C) An image is also observed inside the intrahepatic bile duct that corresponds to the portal vein central point sign found in Caroli disease (indicated by the arrowhead). (D) Cholangiographic sequence showing multiple defects in the intrahepatic bile duct and the cystic dilatations described in the previous images.

Figure 2.

Cholangioscopy with evidence of multiple hepatolithiasis, extraction of stones with electrohydraulic lithotripsy, and extraction of fragments with a basket, with subsequent resolution of hepatolithiasis.