Long term follow after surgical treatment of intrahepatic biliary cysts (types IV & V)

Abstract

Background: Intrahepatic Biliary Cysts (IHBC) are rare congenital lesions of the biliary tract. Types 4A and 5 (Caroli Disease) are frequently confused but both entities present distinct therapeutic options. Methods: Between 1991 and 2017, 38 patients with cysts dilatations were operated. 89,5% were CD. Diagnosis was performed by US, CT, ERCP, MRI. The diagnosis was confirmed by histology. Results: 63,2% patients were female, average age was 47,42 years (20-71). 97,3% symptomatic. Cholangitis was the main presented symptom (96%). The left hemiliver was involved in 67,6%. Bilateral disease in 21,6% Surgical procedures performed were: A) Unilateral IHBC Type 5: left lateral sectionectomy (LLS) 12, left hepatectomy (LH) 16 and right hepatectomy (RH) 5 B) Bilateral IHBC Type 5: RH 1, LH 4 and hepaticojejunostomy (HY) 2. C) Unilateral IHBC Type 4A: LH 1. D) Bilateral IHBC Type 4A: LH 1 and percutaneous treatment 2. Morbidity was 28,9%. 81,6% of patients followed for >5 years. After a median follow-up of 172 months 97,4% of patients are alive and free of symptoms. Diagnosis of CD was confirmed by histology. Cholangiocarcinoma was present in one patient (2,6%). Conclusion: IHBC in Argentina are more common in females with left hemiliver involvement. Surgical resection is the best curative option in unilateral disease with long term survival free of symptoms and complications. In cases of bilateral disease types 5 or 4A cysts, hepaticojejunostomy could be an altermative treatment before indication of liver transplantation.