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Comparative Study
. 2015 Mar;17(3):278-83.
doi: 10.1111/hpb.12330. Epub 2014 Oct 19.

Clinical classification of Caroli's disease: an analysis of 30 patients

Zhong-Xia Wang  1 , Yong-Gang Li, Rui-Lin Wang, Yong-Wu Li, Zhi-Yan Li, Li-Fu Wang, Hui-Ying Yang, Yun Zhu, Yao Wang, Yun-Feng Bai, Ting-Ting He, Xiao-Feng Zhang, Xiao-He Xiao
Affiliations
Comparative Study

Clinical classification of Caroli's disease: an analysis of 30 patients

Zhong-Xia Wang et al. HPB (Oxford). 2015 Mar.

Abstract

Background: Caroli's disease (CD) is a rare congenital disorder. The early diagnosis of the disease and differentiation of types I and II are of extreme importance to patient survival. This study was designed to review and discuss observations in 30 patients with CD and to clarify the clinical characteristics of the disease.

Methods: The demographic and clinical features, laboratory indicators, imaging findings and pathology results for 30 patients with CD were reviewed retrospectively.

Results: Caroli's disease can occur at any age. The average age of onset in the study cohort was 24 years. Patients who presented with symptoms before the age of 40 years were more likely to develop type II CD. Approximately one-third of patients presented without positive signs at original diagnosis and most of these patients were found to have type I CD on pathology. Anaemia, leucopoenia and thrombocytopoenia were more frequent in patients with type II than type I CD. Magnetic resonance cholangiopancreatography (MRCP) and computed tomography (CT) examinations were most useful in diagnosing CD.

Conclusions: No typical symptoms, signs or laboratory indicators are able to distinguish CD from other conditions. Both MRCP and CT were most valuable in diagnosis. The two types of CD may be differentiated by age of onset and routine blood tests.

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Figures

Figure 1
Figure 1
Histopathology of a liver biopsy from a patient with type I Caroli's disease shows cystic dilatation of the segmental intrahepatic bile ducts. (Haematoxylin and eosin stain; original magnification ×10)
Figure 2
Figure 2
Histopathology of a liver biopsy from a patient with type II Caroli's disease shows hepatic and dense portal fibrosis, along with dilated bile ducts. (Haematoxylin and eosin stain; original magnification ×10)
Figure 3
Figure 3
(a) Computed tomography in a 16-year-old girl with Caroli's disease showing central dotting (arrow). (b) Magnetic resonance cholangiopancreatography in the same patient shows the connection between the secular ectasias and the normal biliary tract (arrow)
See this image and copyright information in PMC

References

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