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GI RADIOLOGY Table of Contents   
Year : 2007  |  Volume : 17  |  Issue : 4  |  Page : 254-256
Images: Monolobar Caroli's disease

Department of Radiology, Thomas Jefferson University Hospital, Philadelphia 19107, USA

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Keywords: Caroli′s disease; CT; MRCP

How to cite this article:
Verma SK, Mitchell DG. Images: Monolobar Caroli's disease. Indian J Radiol Imaging 2007;17:254-6

How to cite this URL:
Verma SK, Mitchell DG. Images: Monolobar Caroli's disease. Indian J Radiol Imaging [serial online] 2007 [cited 2020 Jun 4];17:254-6. Available from:
Caroli's disease is the eponymous designation for congenital nonobstructive dilatation of the large intrahepatic bile ducts. [1] This rare and incompletely delineated entity was first described by Caroli et al. in 1958. [2] Caroli's disease may be multifocal and diffuse or may be localized to a lobe or segment of the liver, usually the left. Most cases are associated with congenital hepatic fibrosis. Medullary sponge kidneys may occur occasionally. [3] The incidence of focal monolobar Caroli's disease is rare; less than 20% of cases are reported in literature . [4] The use of noninvasive techniques such as USG, CT scan, isotope scan, and magnetic resonance cholangiopancreatography (MRCP) helps in the diagnosis. Invasive techniques such as endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC) are, therefore, now rarely required. Histopathological examination of the resected specimen is still the gold standard for diagnosing monolobar Caroli's disease.

   Case Report Top

A 23-year-old man presented with right upper quadrant abdominal pain of three months' duration. The pain was intermittent and cramping and occurred after meals. He denied having had any fever, chills, or malaise. On physical examination, there was no fever or evidence of jaundice. The liver was normal. The spleen was not palpable. Liver function tests such as bilirubin, prothrombin time, alkaline phosphatase, and albumin were normal; levels of serum glutamate oxaloacetate transaminase (SGOT) and serum glutamate pyruvate transaminase (SGPT) were mildly elevated. The renal parameters were normal.

Color Doppler USG showed saccular dilatation of the intrahepatic ducts, with abnormal flow signal within [Figure - 1]A. Contrast-enhanced CT scan of the abdomen showed an abnormal area consisting of multiple tortuous, tubular structures in the posterior segment of the right lobe of the liver, some of which contained portal radicles (central dot sign) [Figure - 1]B. MRI of the abdomen showed a rounded tangle of dilated nonenhancing cystic tubular structures [Figure - 2]A in the right lobe of the liver, with focal cystic dilatation of the intrahepatic bile ducts and a normal common bile duct [Figure - 2]. In all the studies, there was no evidence of portal hypertension. The common bile duct and gall bladder were normal.

These findings suggested a segmental anomaly of dilated ducts. The patient underwent a right hepatic lobectomy subsequently. Sectioning through the parenchyma revealed a 5.1 ◊ 5.0 ◊ 4.9 cm irregular area of cystic, dilated ducts, which were filled with thick, green material. Histological examination of the cysts showed hyperplasia of the biliary epithelium but no dysplasia [Figure - 3]. The typical stromal component of a cystadenoma was not present. There was no evidence of malignancy. This appearance was consistent with right-sided monolobar Caroli's disease.

   Discussion Top

Caroli's disease results from a bile duct malformation, which involves neonatal occlusion of the hepatic artery, leading to bile duct ischemia, cystic dilatation, and abnormal growth rate of the developing biliary epithelium and its supporting connective tissue. [5] Incomplete resorption of the circular plates leads to the formation of multiple primitive bile ducts surrounding portal vein radicles. [1] It is believed to have an autosomal recessive inheritance pattern and usually presents with intermittent abdominal pain and hepatomegaly. [6] Cholangitis, cholelithiasis, biliary abscess, septicemia, liver cirrhosis, and cholangiocarcinoma (upto 7%) are all potential complications. [6]

The dilated sacculi or cystic spaces are anechoic on USG and hypodense on CT scan. The fibrovascular bundles, containing portal vein radicles and a branch of the hepatic artery bridging the saccule, appear as a central dot or a linear structure on CT, enhancing with contrast. [7] This 'central dot sign' is also seen on USG. [8] CT scan demonstrates the extent of disease well. MRI provides information about the severity, location, and extent of liver involvement. In our case, this information was useful for planning the correct therapeutic strategy.

The appearance of monolobar Caroli's disease may be confused with primary sclerosing cholangitis, recurrent pyogenic cholangitis, polycystic liver disease, choledochal cyst, biliary papillomatosis and, occasionally, focal obstructive biliary dilatation. [9] MRI is superior to USG and CT scan in its ability to differentiate between these conditions. [10]

Direct cholangiography is rarely necessary for an accurate diagnosis [11] and both ERCP and PTC carry a high risk of bacterial cholangitis. [12] Histological examination of the resected specimen may be valuable and will confirm the diagnosis in equivocal cases. Benign lesions do not show dysplasia of the epithelium of the biliary ducts; instead, hyperplasia and ulceration are the common pathological manifestations. [13]

The aim of therapy is to decrease the morbidity and mortality associated with the recurrent cholangitis, hepatic abscess, and cholangiocarcinoma. Lobar or segmental resection may lead to a complete cure when the disease is confined to one lobe. In our patient, lobectomy relieved symptoms. In the diffuse form, conservative management and/or biliary drainage procedures are effective in decompressing the biliary tract and avoiding complications. Orthotopic liver transplantation is the best alternative, with excellent long-term results. [13]

   References Top

1.Desmet VJ. Congenital diseases of intrahepatic bile ducts: Variations on the theme ductal plate malformation. Hepatology 1992;16:1069-83.  Back to cited text no. 1    
2.Caroli J, Soupault R, Kossakowski J, Plocker L, Paradowska M. The polycystic conge'nitale dilatation of bile ducts intrahe'patiques: Test classification. Semin Hop Paris 1958;34:488-95.  Back to cited text no. 2    
3.Mall JC, Ghahremani GG, Boyer JL. Caroli's disease associated with congenital hepatic fibrosis and renal tubular ectasia. Gastroenterology 1974;66:1029-35.  Back to cited text no. 3    
4.Giovanardi RO. Monolobar Caroli's disease in an adult: Case report. Hepatogastroenterology 2003;50:2185-7.  Back to cited text no. 4    
5.Marchal GJ, Desmet VJ, Proesmans WC, Moerman PL, Van Roost WW, Van Holsbeeck MT, et al. Caroli disease: High-frequency US and pathologic findings. Radiology 1986;158:507-11.  Back to cited text no. 5    
6.Taylor AC, Palmer KR. Caroli's disease. Eur J Gastroenterol Hepatol 1998;10:105-8.  Back to cited text no. 6    
7.Choi BI, Yeon KM, Kim SH, Han MC. Caroli disease: Central dot sign in CT. Radiology 1990;174:161-3.  Back to cited text no. 7    
8.Seth AK, Chawla Y, Dhiman RK, Gupta S, Suri S, Dilawari JB. Caroli's disease: A central dot means a lot. Trop Gastroenterol 1997;18:165-6.  Back to cited text no. 8    
9.Mortelι KJ, Ros PR. Cystic focal liver lesions in the adult: Differential CT and MR imaging features. Radiographics 2001;21:895-910.  Back to cited text no. 9    
10.Pavone P, Laghi A, Catalano C, Materia A, Basso N, Passariello R. Caroli's disease: Evaluation with MR cholangiopancreatography (MRCP). Abdom Imaging 1996;21:117-9.  Back to cited text no. 10    
11.Lucaya J, Gomez JL, Molino C, Atienza JG. Congenital dilatation of the intrahepatic bile ducts (Caroli's disease). Radiology 1978;127:746.  Back to cited text no. 11    
12.Missavage AE, Sugawa C. Caroli's disease: Role of endoscopic retrograde cholangiopancreatography. Am J Gastroenterol 1983;78:815-7.  Back to cited text no. 12    
13.Dagli U, Atalay F, Sasmaz N, Bostanoπlu S, Temuηin G, Sahin B. Caroli's disease: 1977- 1995 experiences. Eur J Gastroenterol Hepatol 1998;10:109-12.  Back to cited text no. 13    

Correspondence Address:
Sachit K Verma
Department of Radiology, Thomas Jefferson University Hospital Philadelphia, PA 19107
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-3026.36870

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  [Figure - 1], [Figure - 2], [Figure - 3]

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