Choledochal Cyst
Laura Webb Lamps, MD
Key Facts
Terminology
Cystic dilatation of biliary tract, usually extrahepatic
Clinical Issues
Usually presents in childhood; most patients female
Most common in Japan
Jaundice, abdominal pain, mass are common findings
Markedly increased risk of carcinoma
Macroscopic Features
Type I (segmental or diffuse fusiform dilatation of common bile duct) is most common
Microscopic Pathology
Thickened, fibrotic cyst wall most commonly lined by biliary epithelium
Liver biopsy specimen shows nonspecific changes of acute or chronic biliary obstruction
 Anteroposterior radiograph during percutaneous cholangiogram shows fusiform dilatation of the common bile duct with rapid change in caliber at the sphincter of Oddi, confirming type I choledochal cyst. |
 This section of choledochal cyst from a resection specimen shows a thickened fibrotic wall with chronic inflammation and ulcerated overlying epithelium. |
TERMINOLOGY
Definitions
Cystic dilatation of biliary tract, usually extrahepatic
ETIOLOGY/PATHOGENESIS
Unknown
Possible congenital malformation
Majority of patients have abnormal pancreaticobiliary junction
Long common channel between distal common bile duct and pancreatic duct
May allow pancreatic secretions to reflux into bile ducts, possibly causing damage and eventual dilatation
No specific inheritance pattern
Occasionally coexists with congenital hepatic fibrosis, biliary dysgenesis, extrahepatic cystic disease
CLINICAL ISSUES
Epidemiology
Age
Usually presents before age 10
Can manifest at any age, however
Gender
75% of patients are female
Ethnicity
Rare in United States (1 in 13,000 live births)
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