Choledochal Cysts: Contemporary Diagnosis, Surgical Management, and Long-Term Surveillance

Abstract

Choledochal cysts, increasingly referred to as congenital biliary dilatation, are uncommon abnormalities of the biliary tree associated with biliary stasis, pancreatobiliary reflux, recurrent inflammation, pancreatitis, cholangitis, stone formation, progressive hepatobiliary injury, and an increased lifetime risk of biliary tract malignancy. Although many cases are diagnosed during childhood, adult presentation remains clinically relevant because delayed diagnosis is often associated with complicated disease, chronic inflammation, biliary stones, adhesions, and greater concern for cholangiocarcinoma or gallbladder carcinoma. Contemporary evaluation relies on ultrasonography, magnetic resonance cholangiopancreatography, computed tomography in selected cases, and therapeutic or problem-solving use of endoscopic retrograde cholangiopancreatography. Complete cyst excision with cholecystectomy and biliary reconstruction remains the cornerstone of treatment for most extrahepatic choledochal cysts. However, important controversies persist regarding the timing of surgery in prenatally diagnosed neonates, hepaticoduodenostomy versus Roux-en-Y hepaticojejunostomy, the role of laparoscopic and robotic surgery, and the optimal strategy for long-term surveillance. This narrative review provides a contemporary overview, updated through May 2026, of classification, pathogenesis, diagnostic evaluation, operative principles, reconstruction strategies, minimally invasive approaches, postoperative complications, malignancy risk, and follow-up.