Surgical management of Caroli disease in a low-mid income country: a single-center study and review of literature.

Abstract

Introduction: Caroli disease is an uncommon congenital condition characterized by non-obstructive intrahepatic bile duct dilation. When coupled with liver fibrosis or cirrhosis, it is termed Caroli syndrome. This disorder can lead to the development of gallstones, inflammation of the bile ducts, and an elevated susceptibility to cholangiocarcinoma. Typically, Caroli disease presents with involvement in less than 20% of the liver, predominantly affecting a single lobe (either left or right). Monolobar disease can often be effectively addressed through liver resection, while bilobar disease may necessitate the consideration of liver transplantation.

Methods: A retrospective study was undertaken involving patients diagnosed with Caroli disease who underwent liver resection. The research included cases from Surgery B Department at Ibn Sina University Hospital in Rabat, covering the period from January 2010 to January 2023.

Results: Nine patients who underwent liver resection for Caroli disease were identified, with an average age of 54 years (range: 17-76), and 44.4% (n = 4) being females. The study comprised 6 cases with disease limited to the left lobe and 3 to the right. The average time interval between initial symptoms and the definitive diagnosis was 4 years (range: 0-24 years). Surgical procedures included left lobectomy in 4 cases, left hepatectomy in 3 cases, right hepatectomy in 1 case, and sub-segmentectomy in 2 cases. Biliodigestive anastomosis was performed in 4 cases. Complications occurred in 2 patients (22.2%), and synchronous cholangiocarcinoma was observed in a single case (11.1%).

Conclusion: Consideration of Caroli disease as part of the differential diagnosis is crucial in cases of recurrent cholangitis. Liver resection stands out as the treatment of choice for patients with localized Caroli disease. The critical importance of early intervention is highlighted by the potentially fatal consequences of delayed diagnosis or treatment.