Choledochal cysts (CCs) are congenital cystic dilatation of extrahepatic and/or intrahepatic bile ducts. CCs are more common in Asian population, the cause is still unknown. Although the etiology is controversial, the main elements in the natural historical emergence of the type I and type IV, which make up the majority of all types, have become clearer. The majority of CCs are diagnosed in childhood. Clinical presentation varies from jaundice in young patients to nonspecific abdominal pain in older, but morbidity increases with complications such as cholangitis, pancreatitis, perforation, hepatitis, liver failure, and malignancy in delayed diagnosed patients. MRCP is considered the current gold standard diagnostic modality that is able to accurately assess biliary anatomy. Although the treatment approach has been formed over the years, it still has not reached the last state. Eventually, the removal of the entire cyst and the reconstruction of the remaining biliary tract to drainage is the current treatment approach. But the dilemma is the way of reconstruction procedure (hepaticoduodenostomy or hepaticojejunostomy). All patients should be followed up for a long period of time, regardless of the surgery method.
Part of the book: Pediatric Surgery, Flowcharts and Clinical Algorithms