Congenital abnormality of the biliary ducts characterized by the dilation of intra or extra hepatic ducts or both
MC related to an anomlaous pancreaticobiliary junction, which leads to weakness and dilation of the biliary wall due to the reflux of alkaline pancreatic secretions into the biliary tree
History / PE:
Symptoms depend on age of presentation
Abdominal pain, jaundice, recurrent pancreatitis (kids)
Vague epigastric/RUQ pain, cholangitis, palpable mass (adults)
Diagnosis:
Cystic extra hepatic mass
Increased bilirubin
Mild elevations of amylase and lipase
Ultrasound
Complications:
Cholangiocarcinoma
Differential Diagnosis:
Caroli's syndrome (intrahepatic dilation of ducts)
Biliary atresia (presents earlier in life)
Pseudopancreatic cyst (rarely causes RUQ mass)
