Related Experiment Video
Updated: May 30, 2026
Laparoscopic Left Liver Sectoriectomy of Caroli's Disease Limited to Segment II and III
Published on: February 27, 2009
Liver transplantation in a 7-month-old girl with Caroli's disease
Robin D Kim1, Linda Book, Allah Haafiz
1Department of Surgery, Section of Transplantation and Hepatobiliary Surgery, University of Utah School of Medicine, Salt Lake City, UT 84132, USA. robin.kim@hsc.utah.edu
Insights
Caroli
Area of Science:
- Hepatology and Gastroenterology
- Rare genetic disorders
- Pediatric liver disease
Background:
- Caroli's disease is a rare autosomal recessive liver disorder causing bile duct dilatation.
- It presents with biliary obstruction, cholangitis, and potential liver abscesses.
- Caroli's syndrome includes congenital hepatic fibrosis and portal hypertension.
Observation:
- The study reports on the youngest patient diagnosed with Caroli's syndrome.
- This patient required intervention due to the severity of the condition.
- The case highlights the challenges in managing pediatric Caroli's syndrome.
Findings:
- Successful liver transplantation was performed on the youngest reported patient with Caroli's syndrome.
- The patient experienced a positive outcome post-transplantation.
- A review of recent literature on Caroli's syndrome management was conducted.
Implications:
- Liver transplantation is a viable and effective treatment for severe Caroli's syndrome, even in very young patients.
- Early diagnosis and intervention are crucial for improving outcomes.
- This case expands the understanding of liver transplantation as a therapeutic option for pediatric liver diseases.
Abstract:
Caroli's disease (including Caroli's syndrome) is a rare autosomal recessive disorder of the liver characterized by diffuse cystic dilatation of the intrahepatic bile ducts. The disease may present at any age and is characterized by recurrent episodes of biliary obstruction, cholangitis, hepaticolithiasis, and liver abscesses. Caroli's syndrome is further associated with congenital hepatic fibrosis and portal hypertension. Patients with recurrent complications or cirrhosis may die because of recurrent infection, portal hypertension, liver failure, or cholangiocarcinoma. Liver transplantation is the treatment of choice for these complicated patients. Here we describe the youngest reported patient with Caroli's syndrome treated successfully using liver transplantation and review the recent literature.
