Caroli's Syndrome: A Case Report and Literature Review

Area of Science:

• Hepatology
• Gastroenterology
• Medical Imaging

Background:

• Caroli's syndrome (CS) is a rare congenital fibro-polycystic liver disorder characterized by intra-hepatic bile duct dilatation.
• It typically affects the left hepatic lobe, with fewer than 200 reported cases since 1958.

Observation:

• A 21-year-old male presented with upper GI bleeding and pallor, initially without signs of chronic liver disease.
• Esophageal varices were identified, prompting investigation for portal hypertension.
• The patient had a history of consanguineous parental marriage.

Findings:

• Imaging studies including ultrasound, CT, and MRCP revealed CS localized to the right hepatic lobe.
• Portal hypertension was the predominant clinical manifestation.
• Liver biopsy confirmed right-sided cystic dilations and congenital hepatic fibrosis.

Implications:

• This case underscores the importance of considering rare conditions like Caroli's syndrome in patients presenting with unexplained GI bleeding and portal hypertension.
• Right-lobe-dominant CS, though rare, can manifest with significant complications.
• Advanced imaging techniques are crucial for accurate diagnosis of intra-hepatic biliary anomalies.