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The Egyptian Journal of Radiology and Nuclear Medicine (2014) 45, 49–52
Egyptian Society of Radiology and Nuclear Medicine
The Egyptian Journal of Radiology andNuclearMedicine
www.elsevier.com/locate/ejrnmwww.sciencedirect.com
CASE REPORT
Monolobar Caroli’s disease with renal cysts: Case
report
* Corresponding author. Tel.: +91 9418500500.
E-mail addresses: [email protected] (S. Thakur), dr.sushma.
[email protected] (S. Makhaik), [email protected]
(A. Chauhan), [email protected] (A. Jhobta),
[email protected] (C.S. Thakur), [email protected]
(R.G. Sood).1 Tel.: +91 9418157788.2 Tel.: +91 9418033264.3 Tel.: +91 9418100180.4 Tel.: +91 9418481310.5 Tel.: +91 9418206920.
Peer review under responsibility of Egyptian Society of Radiology and
Nuclear Medicine.
Production and hosting by Elsevier
0378-603X � 2014 Production and hosting by Elsevier B.V. on behalf of Egyptian Society of Radiology and Nuclear Medicine.
http://dx.doi.org/10.1016/j.ejrnm.2013.10.007Open access under CC BY-NC-ND license.
Shruti Thakur *, Sushma Makhaik 1, Arun Chauhan 2, Anupam Jhobta 3,
Charu S. Thakur 4, R.G. Sood 5
Department of Diagnostic Radiology and General Surgery, Indira Gandhi Medical College and Hospital, Shimla 171001,Himachal Pradesh, India
Received 10 July 2013; accepted 28 October 2013Available online 18 November 2013
KEYWORDS
Caroli’s disease;
Intrahepatic;
Cholangitis;
Cholangiocarcinoma
Abstract Caroli’s disease is autosomal recessive, non-obstructive dilatation of intrahepatic biliary
ducts. The exact etiology is unclear. Two variants of Caroli’s disease are well known-simple; in
which bile ducts are dilated without hepatic fibrosis and the second type which is associated with
congenital hepatic fibrosis along with its sequelae, also known as Caroli’s syndrome. Simple
Caroli’s disease without hepatic fibrosis is quite rare. The importance of recognizing this disease
as a cause of biliary stasis is its frequent association with lithiasis, recurrent cholangitis, liver
abscesses, cirrhosis and cholangiocarcinoma.� 2014 Production and hosting by Elsevier B.V. on behalf of Egyptian Society of Radiology and Nuclear
Medicine. Open access under CC BY-NC-ND license.
1. Introduction
Caroli’s disease or communicating cavernous ectasia of the
intrahepatic bile ducts is a rare autosomal recessive disorderwhich occurs due to the abnormal development of the intrahe-patic bile ducts secondary to intrauterine vascular insult (1). Itis characterized by non-obstructive dilatation of the intrahe-
patic biliary radicles. Caroli’s disease may be diffuse or maybe localized to a lobe or a segment of the liver, usually the left(2). The disease is recognized in its two forms-simple (classic);
in which the intrahepatic biliary duct dilatation is seen withouthepatic fibrosis and Caroli’s syndrome (fibrous); in which thereis hepatic fibrosis in addition that may be accompanied by the
cascade of events that follow portal hypertension. In contrastenhanced computed tomography (CECT), the ‘central dotsign’ representing the vascular bundle in dilated biliary duct
is pathognomonic. Early recognition of the disease and its
50 S. Thakur et al.
complications is required for timely management of thepatient.
2. Case report
A 27-year-old female presented with intermittent right upperquadrant pain for the past five months. There was no history
of fever or jaundice. The blood tests were unremarkable exceptfor slightly raised levels of SGOT and SGPT. The generalphysical examination was also normal with no hepatomegaly
or splenomegaly. On ultrasound of the abdomen, multiple tor-tuous tubular anechoic structures were seen in the right lobe ofthe liver. These were communicating with the right biliary tree
(Fig. 1a). On Doppler imaging, the right portal vein brancheswere accompanying these dilated channels and some of thesedilated channels were also showing traversing septae
Fig. 1b Doppler ultrasound shows portal vein branches (dashed
black arrow) accompanying these dilated biliary ducts (white
arrow). A fibrovascular septa is also seen traversing one of these
dilated ducts (black arrow).
Fig. 1a Gray scale ultrasound in a 27-year-old female with
monolobar Caroli’s disease shows dilated, anechoic, intercommu-
nicating biliary channels in the right hepatic lobe (white arrow).
(Fig. 1b). The left biliary ducts, common hepatic duct,common bile duct and gall bladder were normal. Contrast en-hanced CT abdomen showed saccular dilatation of biliary rad-
icals which were confined to the right lobe (Fig. 2a and b). Asmall enhancing dot representing portal vein branch was seenin the center of some of these cystic dilatations giving the typ-
ical ‘central dot’ sign (Fig. 3). The left and extrahepatic biliarytree and the gall bladder were normal (Fig. 4). Both the kid-neys showed multiple small simple cysts (Fig. 5a and b). There
was no evidence of hepatic mass or portal hypertension onboth Doppler and CT imaging. Esophageal varices, spleno-megaly or ascites were not seen. The radiographic imagingwas consistent with simple Caroli’s disease in localized form.
Because of increased risk of cholangiocarcinoma, the patientwas offered hepatic lobectomy as the treatment which the pa-tient had denied at present. So the patient is managed with
Fig. 2 (a and b) Contrast enhanced CT images (axial, sagittal)
show hypoechoic saccular dilatations in right lobe of the liver
communicating with right hepatic duct consistent with dilated
biliary radicles (white arrow).
Fig. 3 CECT axial image (magnified view) shows the enhancing
portal vein radical (black arrow) in the center of the dilated biliary
duct (white arrow) giving a typical ‘central dot’ sign.
Fig. 4 Coronal CECT shows normal caliber of common hepatic
and common bile ducts (black arrow).
Fig. 5 (a and b) Axial CECT shows small cortical cysts in right
(Fig. 5a, black arrow) and left kidneys (Fig. 5b, black arrow).
Monolobar Caroli’s disease with renal cysts: Case report 51
analgesics along with close follow up for the possibilities ofcomplications.
3. Discussion
Caroli’s disease is a rare congenital disorder characterized by
focal or diffuse saccular dilatation of intrahepatic biliary rad-icals. It was first described by Caroli et al. in 1958 (3). It is alsoclassified as type V choledochal cyst (4). The estimated inci-
dence is 1 in 1,000,000 population. Most cases follow autoso-mal recessive inheritance. There is no sex predilection (5).
The exact pathogenesis is unclear but it is suggested that a
prenatal vascular accident causing occlusion of the hepatic ar-tery is the triggering factor (6). This compromises biliary duc-tal circulation and leads to complete or partial derangement inthe normal remodeling of the ductal plate which is a layer of
hepatic precursor cells that surrounds the portal vein branches
and is the anlage of intrahepatic bile ducts. Varying severity ofdestructive inflammation and segmental dilatation of the bile
ducts follow (7). It should be remembered that the remodelingof the hepatic ductal plates begins in utero from the hilum tothe periphery of the liver with successive development of the
larger ducts first, then the segmental ducts, the interlobularducts and finally the smallest bile ductules (1). If the biliarymalformation involves the ductal plate of the large centralintrahepatic ducts only, it causes the simple type of Caroli’s
disease. If the ductal plates of both the central and the smallerperipheral intrahepatic ducts are malformed, Caroli’s diseasewith congenital hepatic fibrosis results known as Caroli’s syn-
drome (1).Although the disorder is congenital, the patient is asymp-
tomatic in first two decades of life and may remain so for
the entire life. Intermittent abdominal pain is the most com-mon symptom. Other presenting features are fever and jaun-dice. The patients with Caroli’s disease and congenitalhepatic fibrosis may reveal features of portal hypertension like
ascites and esophageal variceal hemorrhage (5). The signifi-cance of recognizing Caroli’s disease as a cause of biliary stasisis its frequent association with various complications. The
common ones are stone formation and cholangitis. Bile ductdilatation leads to bile stagnation and then to lithiasis. Recur-rent cholangitis is the primary cause of morbidity and mortal-
ity. Less frequent complication is cholangiocarcinoma with anincidence of 7–14% (8). Our patient had come with intermit-tent abdominal pain and the disease was recognized before
the development of any complication while the most commonmode of presentation is acute cholangitis seen in 64% of cases(5).
Non invasive imaging techniques like ultrasound, CT and
MRCP help in the diagnosis. Therefore invasive procedures
52 S. Thakur et al.
like ERCP and PTC are usually not required. On sonography,the dilated biliary channels are anechoic and on CT arehypodense. The diagnosis rests on demonstration of continuity
of these saccular dilatations with the biliary tree. The fibrovas-cular bundle which comprises of portal vein radical and branchof hepatic artery can be seen traversing through these biliary
dilatations as enhacing septae or central dot giving the ‘centraldot’ sign which can be seen on both contrast enhanced CT andDoppler. MRI provides better information regarding location,
severity and extent of the disease. However the histopatholo-gical examination of the resected specimen is the goldstandard.
The differentials to be considered are primary sclerosing
cholangitis (PSC), recurrent pyogenic cholangitis, polycysticliver disease, biliary papillomatosis and sometimes obstruc-tive dilatation (5). Biliary ductal dilatation, stenosis, intrahe-
patic calculi and malignancy may be associated with Caroli’sdisease as well as with PSC, however, in PSC the dilatationis rarely saccular and is typically more isolated and fusiform
which is not the characteristic of Caroli’s disease. All thesebiliary changes are also seen with recurrent pyogenic cholan-gitis and this differential is difficult to exclude on imaging
alone. However, in recurrent pyogenic cholangitis the patientis septic while it is not so in simple Caroli’s disease. In poly-cystic hepatic disease, the cysts do not communicate withbile ducts (8). MRI is superior to CT for differentiating
these conditions.Various renal disorders like autosomal dominant polycystic
kidney disease, autosomal recessive polycystic kidney disease,
medullary sponge kidney and medullary cystic disease maybe associated (1). The treatment depends upon the form ofthe disease. The diffuse form required orthotopic hepatic
transplantation while in focal form, lobectomy is sufficient.
4. Conclusion
Caroli’s disease is a rare but important cause of biliary stasis.The patient should be offered appropriate surgical intervention
otherwise close follow up is mandatory for the possible devel-opment of the complications.
Conflict of interest
There is no conflict of interest to disclose.
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