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Successful liver transplantation in a child with Caroli's disease.

Authors
  • Meier, Constance
  • Deutscher, Jens
  • Müller, Susanna
  • Haluany, Karin
  • Fangmann, Josef
  • Siekmeyer, Werner
  • Richter, Thomas
  • Kiess, Wieland
Type
Published Article
Journal
Pediatric Transplantation
Publisher
Wiley (Blackwell Publishing)
Publication Date
Jun 01, 2008
Volume
12
Issue
4
Pages
483–486
Identifiers
DOI: 10.1111/j.1399-3046.2007.00879.x
PMID: 18179642
Source
Medline
License
Unknown

Abstract

CD is a rare autosomal recessive disease, characterized by multifocal cystic dilatation of intrahepatic bile ducts. The course of the disease is characterized by intrahepatic cholelithiasis, recurrent episodes of cholangitis, because of cholelithiasis, hepatic abscesses often ending in death caused by uncontrolled infection. Other conditions such as choledochal cyst and renal cystic disease are frequently associated, and patients have a higher risk for the development of cholangiocarcinoma. Endoscopic drainage of the bile duct is palliative and ineffective. OLT appears to be the treatment of choice. In monolobar cases partial liver resection has been shown to be a curative therapeutic option. We report on the course of disease in a Turkish girl who was diagnosed with CD in the neonatal period. At the age of 8.2 yr, she received OLT and is in good health 57 months post-transplantation.

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