Diagnosis and Treatment of Choledochal Cysts: A Comprehensive Review with a Focus on Choledochocele

Choledochal cysts (CCs) are cystic dilations of intrahepatic and/or extrahepatic bile ducts. Around 80% of CCs are diagnosed within the first decade of life. These complex clinical entities are extremely rare, especially in the Western population. CCs are frequently classified according to the Todani classification. CCs may be asymptomatic or present as acute pancreatitis and/or cholangitis, biliary obstruction, or malignancy. Therefore, the diagnosis relies primarily on abdominal imaging modalities, mainly magnetic resonance cholangiopancreatography. Management is tailored based on the cyst morphology and the patient’s clinical characteristics, with surveillance, surgery, and interventional endoscopy being the most frequent management options. While the surgical approach is the most frequently employed, type III CCs (also known as choledochocele) are frequently managed endoscopically, and novel endoscopic, minimally invasive treatment options are rapidly emerging.