Liver transplantation consequential to Caroli's syndrome: a case report

Area of Science:

• Hepatology
• Gastroenterology
• Rare Diseases

Background:

• Caroli's disease involves congenital dilatations of intrahepatic bile ducts.
• It can manifest as cystic structures communicating with the biliary tree.
• Associated conditions include congenital hepatic fibrosis and polycystic kidney lesions.

Purpose of the Study:

• To present a case of recurrent cholangitis secondary to Caroli's syndrome.
• To highlight the unique instance of liver transplantation for this condition at the center.

Main Methods:

• Case report of a 44-year-old woman.
• Analysis of hepatectomy sample post-liver transplantation.
• Pathological examination of bile duct malformations.

Main Results:

• The patient experienced recurrent cholangitis due to Caroli's syndrome.
• Liver transplantation was performed, showing large cystic dilatations in segments VII and VIII.
• Pathology confirmed congenital dilatation of intrahepatic bile ducts and congenital hepatic fibrosis.

Conclusions:

• Orthotopic liver transplantation is a therapeutic option for diffuse or uncontrollable Caroli's syndrome.
• It serves as a final option for cases unresponsive to other treatments or with significant complications.
• This case underscores the importance of transplantation in managing severe manifestations of Caroli's disease.