Choledochal cysts are a congenital anomaly, and they show dilatation of the intra- or extrahepatic biliary tree. These cysts are uncommon in Western countries, but are not rare in Asian countries. Choledochal cysts are classified into five groups based on location or shape of the cysts. Types I and IV-A cysts are the most common types, which are associated with anomalous pancreaticobiliary junction (APBJ), but other cysts are not associated with APBJ. Types I and IV-A cysts appear to belong to a different category from other cysts embryologically. Type I and IV-A cysts accompany anomalies of the pancreas. Type I and IV-A cysts might occur when left ventral anlage persists, and with disturbed recanalization of the common bile duct. Endoscopic retrograde cholangiopancreatography is the gold standard for detecting APBJ, but it is an invasive procedure. Magnetic resonance cholangiopancreatography (MRCP) is a non-invasive imaging tool for detecting pancreatic and biliary trees. MRCP is the first-choice modality for diagnosing choledochal cysts and APBJ in pediatric patients. Cystoenterostomy is been performed because of high complication and mortality rates. Complete excision of the cysts with Roux-en-Y hepatojejunostomy is a standard procedure for choledochal cysts to prevent postoperative complications, including development of cancer. In this study, we review classification, pathogenesis, diagnosis, and treatment of types I and IV-A choledochal cysts.
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