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Case Reports
. 2015 Jun;21(2):175-9.
doi: 10.3350/cmh.2015.21.2.175. Epub 2015 Jun 26.

Caroli's disease misdiagnosed as intraductal papillary neoplasm of the bile duct

Dae Hoe Gu  1 , Min Seon Park  1 , Chang Ho Jung  1 , Yang Jae Yoo  1 , Jae Young Cho  1 , Yun Ho Lee  1 , Yeon Seok Seo  1 , Hyung Joon Yim  1 , Soon Ho Um  1 , Ho Sang Ryu  1
Affiliations
Case Reports

Caroli's disease misdiagnosed as intraductal papillary neoplasm of the bile duct

Dae Hoe Gu et al. Clin Mol Hepatol. 2015 Jun.

Abstract

Caroli's disease is a rare autosomal-recessive disorder caused by malformation of the ductal plate during embryonic development. Although it is present at birth, Caroli's disease is typically not diagnosed until between the second and fourth decades of life, as it was in the present patient. Here we report a rare case of Caroli's disease limited to one liver segment, which was initially misdiagnosed as an intraductal papillary neoplasm of the bile duct. The asymptomatic patient was treated with liver segmentectomy.

Keywords: Caroli's disease; Intraductal papillary neoplasm of the bile duct; Segmentectomy.

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Conflict of interest statement

Conflicts of Interest: The authors have no conflicts to disclose.

Figures

Figure 1
Figure 1. Abdominal computed tomography (CT), magnetic resonance imaging (MRI), and magnetic resonance cholangiopancreatography (MRCP) findings. Localized bileduct dilatation in segment 5 (arrow) presented as a high signal intensity in T2-weighted MRI but without a demonstrable intraductal mass lesion evident in the images. There was no evidence of liver cirrhosis (A: pre-enhancement phase of CT, B: portal phase of CT, C: T2- weighted MRI, D: MRCP).
Figure 2
Figure 2. Gross findings of the resected liver. (A) There were no grossly remarkable findings on the surface of the resected liver. (B) No stones or mass was found in the cut surface of the serial section, and a diffuse parenchymal hemorrhagic soft dilated lesion was noted. The dilatation of the intrahepatic duct appeared in clusters. Hemorrhagic peliosis was seen in the periductal area, and the liver parenchyma had a slight greenish color that was suggestive of cholestasis.
Figure 3
Figure 3. Microscopy findings of the resected specimen. Bile ducts were dilated and had thickened walls (asterisks). Ectasias result in a predisposition to repeated attacks of cholangitis with complications such as intrahepatic lithiasis, amyloidosis, and cholangiocarcinoma, but they were not present in this case. The lining epithelium (arrows) comprised columnar and cuboidal cells (hematoxylin and eosin stain, A: ×40, B: ×200).
See this image and copyright information in PMC

References

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