Caroli's disease pathophysiology

Caroli's disease Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Caroli's disease from other Diseases
Epidemiology and Demographics
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Physical Examination
Laboratory Findings
CT
MRI
Echocardiography or Ultrasound
Other Imaging Findings
Treatment
Medical Therapy
Surgery
Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Caroli's disease pathophysiology On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Caroli's disease pathophysiology All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Caroli's disease pathophysiology
CDC on Caroli's disease pathophysiology
Caroli's disease pathophysiology in the news
Blogs on Caroli's disease pathophysiology
Directions to Hospitals Treating Caroli's disease
Risk calculators and risk factors for Caroli's disease pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Pathophysiology

• Biliary cysts have been described throughout the biliary system, and are classified according to their location and anatomy.
• Many biliary cysts are congenitally acquired, perhaps as a consequence of unequal proliferation of epithelial cells during embryonic biliary duct development. Some biliary cysts are acquired, and some may develop in association with anatomic variations that lead to abnormally high ductal pressures in association with other predisposing factors.
• Type I (75-85%)

• Choledochal cysts - cystic dilatation of the common bile duct (CBD)
• Often presents during infancy with significant liver disease.

• Type II (2-3%)

• Diverticulae anywhere in the extrahepatic ducts.
• Presenting symptoms’s may reflect cystic compression of nearby structures.

• Type III (1-5%)

• Choledochocele cysts
• Often present with pain and obstructive jaundice; many have pancreatitis.

• Type IV (18-20%)

• IVA – Multiple cysts in the Intra- and Extra-hepatic ducts
• IVB – Multiple cysts in the Extra-hepatic ducts only

• Type V – Caroli’s Disease - rare

• Single or Multiple Intrahepatic bile duct cysts