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

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.

Surgical Management of Congenital Intrahepatic Bile Duct Dilatation, Caroli's Disease and Syndrome

“…13,39 LT enables (i) a complete resection of diffuse congenital intrahepatic bile ducts whatever the extent, (ii) to treat underlying liver disease, and (iii) to prevent cholangiocarcinoma degeneration. 12,34,41 In addition, in the present series, 70% of the recipients underwent a biliary reconstruction using a Roux-en-Y loop. As previously reported, 13 we recommend LT only in patients with diffuse bilobar forms of the disease complicated by repeated sepsis or to patients with cirrhosis or portal hypertension.…”

Caroli Disease: A Case Report Observed at the Cocody Teaching Hospital of Abidjan (Cote D’Ivoire)

“…When she is associated with a congenital hepatic fibrosis, she is called Caroli syndrome. In this case, clinical manifestations are those of congenital hepatic fibrosis including collateral venous circulation, esophageal varices and a spleno-megaly [3] [7]. However, there are some isolated cases [3] [7].…”

“…It's a recessive autosomal affection characterized by a localized or a diffused cystic dilation of intra-hepatic bile ducts [2]. Clinical manifestations of the disease are variable, different from the childhood to the second or the third decade of life, generally diagnosed in adult people [3] [4] [5] [6]. When she is associated with a congenital hepatic fibrosis, she is called Caroli syndrome.…”

“…In this case, clinical manifestations are those of congenital hepatic fibrosis including collateral venous circulation, esophageal varices and a spleno-megaly [3] [7]. However, there are some isolated cases [3] [7]. The prognosis factors depend on its complications: cholangitis, cirrhosis and cholangiocarcinoma [1] [4].…”

“…During the clinical examination, we found a likely cirrhotic hepatomegaly associated to an edematous-ascitic syndrome. Biological exams showed an infectious syndrome with a hyperleukocytosis at 13.000 per mm 3 , a hyperneutrophilia at 9.600 per mm 3 . Serological viral markers of B, C and D hepatitis were negative.…”