Choledochoceles in children: The elephant abnormality

Abstract

A choledochocele is a rare congenital abnormality of the extrahepatic bile duct characterised by localised dilatation of the distal common bile duct within the wall of the duodenum. We present two children with a long history of undiagnosed abdominal pain and subtle signs of cholestasis. Specific investigations included magnetic resonance cholangiopancreatography (MRCP) that led to a possible diagnosis of choledochocele, and then confirmatory cholangiograms (ERCP and open). Both children required intervention to unroof the dilatation allowing improved pancreato-biliary drainage and complete resolution of symptoms. Elephants have no gallbladder, instead they have a localised dilatation of the distal common bile duct within the wall of the duodenum – analogous to the situation described here. • Choledochoceles may have non-specific symptoms making them difficult to suspect. • Cholangiogram is the gold standard to confirm the diagnosis. • Drainage of the choledochocele into the duodenum resolves symptoms and can be done endoscopically or surgically. • Biopsy should always be taken as a malignancy risk exists, at least in adults.