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Living Donor Liver Transplantation for Caroli's Disease: A Report of Two Cases
Author(s) -
Klaus Steinbrück,
Marcelo Enne,
Reinaldo Fernandes,
J. M. G. Martinho,
L.F. Pacheco-Moreira
Publication year - 2011
Publication title -
isrn surgery
Language(s) - English
Resource type - Journals
eISSN - 2090-5793
pISSN - 2090-5785
DOI - 10.5402/2011/106487
Subject(s) - medicine , liver transplantation , living donor liver transplantation , surgery , transplantation , disease , intrahepatic bile ducts , liver disease , rare disease , pediatrics , gastroenterology , bile duct
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.

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