Liver atrophy associated with monolobar Caroli's disease

Area of Science:

• Hepatobiliary Surgery
• Gastroenterology
• Medical Imaging

Background:

• Caroli's disease is a rare congenital disorder characterized by saccular dilatation of the intrahepatic bile ducts.
• Monolobar Caroli's disease (Type I) can present with complications such as recurrent cholangitis.
• The atrophy-hypertrophy complex is not typically associated with Caroli's disease.

Observation:

• A 30-year-old male presented with recurrent cholangitis.
• Imaging revealed localized, right-sided, saccular biliary dilatation within a liver of normal overall size.
• Intraoperative findings confirmed severe right lobar atrophy, with the resected lobe weighing only 140 grams.

Findings:

• This case highlights the unusual co-occurrence of the atrophy-hypertrophy complex in monolobar Caroli's disease.
• The resected right lobe demonstrated significant atrophy, contrasting with the biliary dilatation characteristic of Caroli's disease.
• Surgical challenges included distorted hilar vascular anatomy and posterior displacement of the right hepatic duct orifice.

Implications:

• This report expands the understanding of Caroli's disease presentation and associated pathologies.
• Recognition of this complex is crucial for surgical planning and management of affected patients.
• Further research may elucidate the underlying mechanisms linking biliary dilatation with lobar atrophy in this condition.