PURPOSE OF REVIEW: Historically, surgical treatment of choledochal cyst consisted of cyst enterostomy. However, incomplete cyst excision can result in recurrent symptoms and malignant transformation within the cyst remnant. Accordingly, management of choledochal cyst now includes complete cyst excision whenever possible. We provide a review detailing the up to date management of choledochal cysts. We describe choledochal cyst-type specific surgical approaches, the impact of minimally invasive surgery in choledochal cyst therapy, and long-term sequelae of choledochal cyst management. RECENT FINDINGS: Treatment of choledochal cyst aims to avoid the numerous hepatic, pancreatic, or biliary complications that may occur. More recently, minimally invasive approaches are being used for the treatment of choledochal cyst with acceptable morbidity and mortality. Moreover, long-term follow up of choledochal cyst patients after resection has demonstrated that although the risk of biliary malignancy is significantly decreased after choledochal cyst resection, these patients may remain at a slightly increased risk of biliary malignancy even after excision. SUMMARY: Management of choledochal cyst and the operative conduct will depend upon the patient comorbidities and choledochal cyst subtype. However, given the complex nature of choledochal cyst and limited experience of most centers, these patients should be evaluated and treated at high-volume hepatopancreaticobiliary centers familiar with management of choledochal cyst.
