Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2011:2011:106487.
doi: 10.5402/2011/106487. Epub 2011 Apr 27.

Living Donor Liver Transplantation for Caroli's Disease: A Report of Two Cases

Klaus Steinbrück  1 , Marcelo Enne, Reinaldo Fernandes, Jose M Martinho, Lúcio F Pacheco-Moreira
Affiliations
Case Reports

Living Donor Liver Transplantation for Caroli's Disease: A Report of Two Cases

Klaus Steinbrück et al. ISRN Surg. 2011.

Abstract

Caroli's disease (CD) is a rare autosomal recessive disorder characterized by intrahepatic cystic dilatation of the bile ducts. Patients with bilobar or progressive disease may require orthotopic liver transplantation (OLT). In the MELD era, living donor liver transplantation (LDLT) raised as the ultimate treatment option for these patients, once their MELD score is usually low. Herein, we describe 2 cases of patients (a 2-year-old girl and a 19-year-old teenager) that successfully underwent LDLT as a treatment for diffuse CD. The good postoperative courses of the two cases indicate that LDLT is a feasible option in the treatment of this disorder, even in complicated or early age patients.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Preoperatory magnetic resonance of patient 1, showing the intrahepatic cystic dilatation of the bile ducts, especially on the right liver.
Figure 2
Figure 2
Preoperatory magnetic resonance of patient 2, reveling diffuse cystic dilatation of the bile ducts.
Figure 3
Figure 3
Inspection of liver specimen of patient 2 confirmed intrahepatic bile ducts' cystic dilatation.
See this image and copyright information in PMC

References

    1. Vachell HR, Stevens WM. Case of intrahepatic calculi. British Medical Journal. 1906;1:434–436. - PMC - PubMed
    1. Caroli J, Soupault R, Kossakowski J, Plocker L, Paradowska Congenital polycystic dilation of the intrahepatic bile ducts; attempt at classification. La Semaine des Hôpitaux. 1958;34(8):488–495. - PubMed
    1. Todani T, Watanabe Y, Narusue M. Congenital bile duct cysts. Classification, operative procedures, and review of thirty seven cases including cancer arising from choledochal cyst. American Journal of Surgery. 1977;134(2):263–269. - PubMed
    1. Taylor ACF, Palmer KR. Caroli’s disease. European Journal of Gastroenterology and Hepatology. 1998;10(2):105–108. - PubMed
    1. Grumbach R, Bourillon J, Auvert JP. Maladie fibro-kystique du foie avec hypertension portale chez l’enfant. La Semaine des Hôpitaux. 1954;74:p. 30.

Publication types

LinkOut - more resources