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choledochal cyst; biliary cyst (includes: choledochocele, Caroli's disease)
Classification:
- type I:
- most common (80-90%)
- saccular or fusiform dilatation of a portion or entire common bile duct with normal intrahepatic duct
- type II:
- isolated diverticulum protruding from the common bile duct
- type III: (choledochocele)
- dilatation of duodenal portion of common bile duct
- type IVa:
- multiple dilatations of the intrahepatic & extrahepatic biliary tree
- type IVb:
- multiple dilatations involving only the extrahepatic bile ducts
- type V: (Caroli's disease)
- cystic dilatation of intrahepatic biliary ducts
Etiology:
- cystic dilation of the bile ducts
Epidemiology:
- uncomnon
- less uncommon in East Asians
Clinical manifestations:
- most present in 1st year of life; adult presentation is rare
- classic triad in a minority of patients
- intermittent abdominal pain
- jaundice
- right upper quadrant abdominal mass
Special laboratory:
- endoscopic retrograde cholangiopancreatography (ERCP)
- dilation of the common bile duct without evidence of obstructing lesion
Radiology:
- ultrasonography
- computed tomography
- magnetic resonance imaging
Complications:
- cholangitis
- biliary cancer (2%)
Management:
- surgical excision with formation of a roux-en-Y anastomosis to the biliary duct
General
cyst
biliary disease
congenital anomaly (birth defect)
References
- Wikipedia: Choledochal cysts
http://en.wikipedia.org/wiki/Choledochal_cysts
- Sawyer MAJ
eMedicine (Medscape): Imaging in Choledochal Cyst
http://emedicine.medscape.com/article/366004-overview
- Sawyer MAJ
eMedicine (Medscape): Choledochal Cyst
http://emedicine.medscape.com/article/172099-overview
- Medical Knowledge Self Assessment Program (MKSAP) 16
American College of Physicians, Philadelphia 2012