Pediatric choledochal cysts: diagnosis and current management

Choledochal cysts are rare congenital disorders first described by Vater and Ezler in 1723. Their exact etiology remains incompletely understood; however, an anomalous pancreaticobiliary union (APBDU) and subsequent reflux of biliary contents into the biliary tree are thought to play a role. Accordingly, APBDU-associated choledochal cyst patients are significantly more likely to have evidence of hepatitis, cholangitis or pancreatitis and pathologically confirmed inflammation. In 1977, Todani and colleagues modified the original Alonso-Lej classification to include five types of CC. Type I and IV are the most common and most likely to be associated with malignancy. The majority of choledochal cysts are diagnosed in childhood. Clinical presentation varies and most often consists of nonspecific abdominal pain. Diagnosis is typically accomplished using multimodality imaging techniques including computed tomography, magnetic resonance imaging, ultrasound and MRCP. The use of diagnostic PTC and ERCP in CC has been largely replaced by MRCP. Appropriate management consists of prompt, complete cyst excision followed by restoration of biliary enteric continuity when necessary. Minimally invasive CC resection in the pediatric population has demonstrated acceptable outcomes. Prognosis is generally excellent; however, malignancy risk remains higher than the general population even after complete surgical excision.