Nephrol Dial Transplant (2002) 17: 1113-1115
© 2002 European Renal Association-European Dialysis and Transplant Association
Case Reports
Caroli's disease of the liver in a renal transplant recipient
Vadakethu T. Ninan1,
Mangalathillam R. N. Nampoory,1,
Kaivilayil V. Johny2,
Ram K. Gupta1,
Imre Schmidt1,
Prasad M. Nair1 and
Jaber Al-Ali1
1 Department of Medicine, Mubarak Al-Kabeer Hospital and
2 Faculty of Medicine, Kuwait University, Kuwait
Keywords: Caroli's disease; clinical course; renal transplant
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Introduction
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Caroli's disease is a rare inherited disorder of the liver,
characterized by saccular and cystic dilatation of intra-hepatic
biliary ducts. Association of this abnormality with autosomal
recessive polycystic kidney disease (ARPKD) has been described
[
1]. This abnormality may remain asymptomatic and undetected
throughout life but often presents in adolescence or later with
episodes of bacterial cholangitis, complicated by multiple biliary
calculi. Very little is known about the clinical course of Caroli's
disease in renal transplant recipients. We report here our experience
with a case of Caroli's disease of the liver diagnosed in a
renal transplant recipient who presented with recurrent bouts
of cholangitis after renal transplantation.
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Case
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Miss FA, a 25-year-old single female had been diagnosed with
ARPKD at the age of 15 years, based on clinical and ultrasonographic
features. Over the subsequent years of follow up, her renal
function declined progressively and at the age of 20 years she
was started on maintenance haemodialysis. She underwent a live
unrelated donor renal transplantation a few months later at
another centre and came to our unit for follow up. Maintenance
immunosuppression consisted of prednisolone 10 mg once daily,
cyclosporin 70 mg twice daily, and azathioprine 50 mg once daily.
During the following 2 years she had two rejection episodes
which were successfully reversed with courses of i.v. methylprednisolone.
Two years post-transplant she was treated for an episode of
Escherichia coli septicaemia. The source of the infection was
not clear. An ultrasound of the abdomen carried out at the time
showed findings which were interpreted as multiple cysts in
the liver. There were no symptoms localized to the liver and
further investigations were not undertaken at that stage. She
remained stable and asymptomatic on follow up till 5 years post-transplant
when she was admitted with fever, upper abdominal pain, and
vomiting. On physical examination, an enlarged tender liver
was palpable. Computerized tomography (CT) of the abdomen showed
dilated intra- and extra-hepatic ducts with multiple biliary
calculi (Figure 1
). An endoscopic retrograde cholangiopancreatography
(ERCP) was undertaken and this showed dilated common bile duct
(CBD) and intra-hepatic ducts with marked saccular dilatation,
involving both lobes of the liver. The CBD had three calculi
and there were numerous calculi seen as filling defects in the
dilated intra-hepatic ducts. These features were consistent
with Caroli's disease of the liver. Endoscopic sphincterectomy
was carried out and CBD calculi were extracted. The calculi
in the intra-hepatic ducts could not be removed. The clinical
events, laboratory results, and antibiotics administered in
this admission and subsequent hospital admissions are summarized
in Table 1
. In a span of about 4 months she was admitted into
the hospital five times with fever. Except for an occasional
transient rise of serum creatinine, the renal transplant function
remained stable throughout these episodes. In the fifth episode,
fever was unresponsive to treatment. Ultrasound examination
and gallium scan did not reveal any localized liver abscess.
Under these circumstances she was referred for a liver transplantation.
An orthotopic liver transplantation from a cadaveric source
was successfully carried out and she has remained well over
the subsequent 4 years of follow up. After the liver transplant,
the maintenance immunosuppression was changed and consisted
of prednisolone 5 mg daily and tacrolimus 1 mg twice daily.
Recent investigations showed her serum creatinine to be stable
at 170 µmol/l, with normal liver enzymes, serum albumin,
and bilirubin.
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Fig. 1. Non-enhanced CT image of the patient's liver, showing saccular dilatation of intra-hepatic biliary ducts involving both lobes of the liver. Biliary calculi, some of which are calcified, are seen as high density material in the lumen of the dilated ducts.
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Discussion
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Caroli described two forms of congenital dilatation of the intra-hepatic
tree, and these were associated with renal cystic disease. One
of these forms has pure ductal ectasia without significant hepatic
fibrosis and is termed Caroli's disease of the liver. When ductal
abnormalities are associated with periportal fibrosis the disease
is called Caroli's syndrome [
2]. Ductal ectasia predisposes
to bile stagnation and formation of biliary calculi, which increases
risk of cholangitis. The association of Caroli's disease with
ARPKD has been well described. Waters
et al. [
3] in a study
of 15 children with ARPKD found that 60% of them had associated
asymptomatic Caroli's disease of the liver. Several investigations
have indicated that hepatic ductal plate and renal tubular differentiation
share genetic determinants, and this may explain the association
between these diseases [
4]. Caroli's disease usually presents
as a symptomatic clinical problem in adolescence or later in
life. The fact that our patient developed episodes of cholangitis
only after renal transplantation raises the concern that the
immunosuppressed state predisposed her to development of infection.
Preventing recurrent infections in patients with Caroli's disease
with prophylactic antibiotics has sometimes been possible but
very often this strategy is unsuccessful [
5]. Results of surgical
procedures aimed at improving biliary drainage to prevent infections
have also not been encouraging. Our patient had diffuse involvement
of both lobes of the liver and hence localized resection of
the affected area was not possible. She had recurrent episodes
of cholangitis at short intervals, and fever during the fifth
episode was unresponsive to antibiotics. An orthotopic liver
transplant was the appropriate treatment in this situation for
this patient.
Orthotopic liver transplantation has been successfully carried out as treatment in some cases of Caroli's syndrome in the non-renal transplant setting [6,7]. We did not find any previous report on the clinical course of Caroli's disease in renal transplant recipients. ARPKD can progress to end-stage renal disease, and in view of its association with Caroli's disease of the liver, it is probable that more patients like ours will be encountered in whom both a renal and liver transplant could become necessary. Renal transplant recipients who develop recurrent bouts of cholangitis should undergo investigations to detect Caroli's disease, as appropriate therapy can lead to a good outcome.
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Notes
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Correspondence and offprint requests to: Dr M. R. N. Nampoory,
PO Box 1427, Hawally, Code No. 32015, Kuwait. Email:
nampoory{at}hotmail.com 
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References
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Desmet VJ. Ludwig symposium on biliary disorders-part I. Pathogenesis of ductal plate abnormalities. Mayo Clin Proc1998; 73: 80–89[Abstract]
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Waters K, Howman-Gibes, Rossleigh M, Ureu R, Knight J. Intrahepatic bile duct dilatation and cholestasis in autosomal recessive polycystic kidney disease. Demonstration with hepatobiliary scintigraphy. Clin Nucl Med1995; 20: 892–895[Web of Science][Medline]
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D'Agata ID, Jonas MM, Peres Aatvde BR, Guay-Woodford LM. Combined cystic disease of the liver and kidney. Semin Liver Dis1994; 14: 215–228[Web of Science][Medline]
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Burt MJ, Chambers ST, Strack MF, Troughton WD. Two cases of Caroli's disease, diagnosis and management. J Gastrenterol Hepatol1994; 9: 194–197[Web of Science][Medline]
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Schiano TD, Fiel MI, Miller CM, Bodenheimer HC, Jr, Min AD. Adult presentation of Caroli's syndrome treated with orthotopic liver transplantation. Am J Gastrenterol1997; 92: 1938–1940
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Sans M, Rimola A, Navasa M et al. Liver transplantation in patients with Caroli's disease and recurrent cholangitis. Transplant Intl1997; 10: 241–242
Received for publication: 10. 5.01
Accepted in revised form: 15. 1.02
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