Choledochal cyst: A Common cause of cholangitis in Pediatrics

Abstract

Choledochal cysts (CC) are a congenital cystic dilation of the extrahepatic and/or intrahepatic bile ducts that approximately 80% of the patients are diagnosed in childhood. Despite the diversity of etiologies, the main elements of the predominantly types I and IV, which constitute the majority of all types, have become clearer. The clinical presentation varies from jaundice in young patients to non-specific abdominal pain in older patients, but morbidity increases with complications such as angiocholitis, pancreatitis, perforation, hepatitis, liver failure and malignancy in late-diagnosed patients. MRCP is considered the gold standard diagnostic tool today, capable of accurately assessing biliary anatomy. The therapeutic approach has progressed over the years. It is based on the removal of the entire cyst and reconstruction of the remaining bile ducts up to drainage.