Choledochal Cystic Diseases

Cystic disease of the intrahepatic and extrahepatic bile ducts results in rare malformations with a variable presentation. The majority of patients present during childhood with symptoms of abdominal pain, cholangitis, and an abdominal mass. A palpable mass is unusual in adults, and adult patients tend to present with recurrent cholangitis, pancreatitis, or rarely portal hypertension. The cause of this disorder also is debated, with both congenital and acquired origins postulated. The gold standard for the treatment of choledochal cysts is complete excision with the establishment of biliary flow into the gastrointestinal tract. The well described malignant potential of the cyst and the high rate of recurrent cholangitis with internal drainage procedures mandate cyst excision when possible. In the event of extensive scarring or malignant changes of the cyst, the posterior wall of the cyst may be left in situ to avoid endangering the portal vessels, which are found posteriorly. Alternatively, various endoscopic or percutaneous interventions may provide symptomatic relief. However, every effort should be directed towards complete resection of the cyst and the re-establishment of biliary-enteric continuity.