Congenital choledochal malformation: Not just a problem for children

Abstract

Choledochal cysts remain relatively uncommon in Western Europe and the US, although they are appreciably more common in Asia. Their aetiology remains obscure although abnormalities of the pancreaticobiliary junction may be the primary pathology in some, with biliary dilatation following reflux of activated pancreatic secretions. Most anomalies will present in childhood with obstructive jaundice or abdominal pain; a proportion may only present for the first time during adulthood, and some of these will show malignant transformation. The classical triad of pain, jaundice and a palpable mass is, however, not common. Complete cyst excision, where possible, and biliary reconstruction remain the aims for most types although there is still some controversy about the type of reconstruction. Laparoscopic reconstruction is possible but is still very much an advanced technique. The risk of long-term problems post-surgery is significant. Whether this is due to recurrent pancreatitis secondary to the retained common channel and/or a distal stump, or due to the development of biliary tract malignancy is still a cause for concern and indicates the need for adequate, prolonged follow-up.