A mutation in the OFD1 gene is seen in polycystic kidney disease that is linked to intracranial hypertension.

Dr. Manjula Roumie*

We describe a very unusual instance of type I polycystic kidney disease and choledochal cysts. Due to polycystic kidney disease, a 48-year-old woman was reliant on hemodialysis for chronic renal failure. By chance, an ultrasound revealed that she also had a dilated common bile duct. An endoscopic retrograde cholangiopancreatography without visualisation of the pancreatic duct revealed a spindle-shaped, dilated common bile duct (type I choledochal cyst). The choledochal cyst was removed from her. A cholangiogram performed during surgery revealed no evidence of contrast medium reflux into the pancreatic duct. The aspirated bile from the bile duct did not have an increased amylase level. The presence of renal fibro polycystic disease may prevent pancreaticobiliary dysfunction from becoming a factor in the aetiology of choledochal cysts. In these situations, choledochal cyst management is still debatable and needs more discussion.