Journal
AMERICAN JOURNAL OF ROENTGENOLOGY
Volume 216, Issue 6, Pages 1530-1538Publisher
AMER ROENTGEN RAY SOC
DOI: 10.2214/AJR.20.23522
Keywords
bile ducts; Caroli disease; congenital lithiasis; MRI
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This study aimed to assess the MRCP features of Caroli disease, finding that diffuse CD patients often had congenital hepatic fibrosis and a central dot sign, while localized CD patients were commonly associated with intrahepatic biliary calculi and left liver atrophy.
OBJECTIVE. The purpose of this multicenter retrospective study was to assess the MRCP features of Caroli disease (CD). MATERIALS AND METHODS. Sixty-six patients were identified from 2000 to 2019. The inclusion criteria were diagnosis of diffuse or localized CD mentioned in an imaging report, presence of intrahepatic bile duct (IHBD) dilatation, and having undergone an MRCP examination. The exclusion criteria included presence of obstructive proximal biliary stricture and having undergone hepatobiliary surgery other than cholecystectomy. Histopathology records were available for 53 of the 66 (80%) patients. Diffuse and localized diseases were compared by chi-square and t tests and Kaplan- Meier model. RESULTS. Forty-five patients had diffuse bilobar CD ((five pediatric patients [three girls and two boys] with a mean [+/- SD] age of 8 +/- 5 years [range, 1-15 years] and 40 adult patients [26 men and 14 women] with a mean age of 35 +/- 11 years [range, 20-62 years]) and 21 patients had localized disease (12 men and 9 women; mean age, 54 +/- 14 years). Congenital hepatic fibrosis was found only in patients with diffuse CD (35/45 [78%]), as was a central dot sign (15/35 [43%]). IHBD dilatation with both saccular and fusiform features was found in 43 (96%) and the peripheral funnel-shaped sign in 41 (91%) of the 45 patients with diffuse CD but in none of the patients with localized disease (p <.001). Intrahepatic biliary calculi were found in all patients with localized disease but in only 16 of the 45 (36%) patients with diffuse CD (p <.001). Left liver atrophy was found in 18 of the 21 (86%) patients with localized disease and in none of the patients with diffuse CD (p <.001). The overall survival rate among patients with diffuse CD was significantly lower than that among patients with localized disease (p =.03). CONCLUSION. Diffuse IHBD dilatation with both saccular and fusiform features associated with the peripheral funnel-shaped sign can be used for the diagnosis of CD on MRCP. Localized IHBD dilatation seems to be mainly related to primary intrahepatic lithiasis.
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