Choledochal Cyst

• • Type I cysts (fusiform dilation of common bile duct [CBD]) account for 85-90%

  • Type II (true diverticula of CBD) 1-2% of cases

  • Type III (choledochocele—dilation of distal/intramural portion of CBD) < 2% of cases

  • Type IV (multiple cysts involving intrahepatic and extrahepatic ducts) as high as 15% of cases in some series

  • Type V (cystic malformation of intrahepatic ducts) rare

  • 3-5% incidence of carcinoma

Epidemiology

• • Onset of symptoms usually in infancy or childhood

Symptoms and Signs

• • Jaundice

  • Fever

  • Pain

  • Palpable right upper quadrant mass

  • Hepatomegaly

  • Bleeding varices

  • Asymptomatic

Laboratory Findings

• • Hyperbilirubinemia

  • Elevated alkaline phosphatase

  • Leukocytosis

  • Elevated amylase and lipase

Imaging Findings

• • US showing characteristic cystic dilation of biliary tree corresponding to type as well as proximal dilation in presence of obstruction

  • ERCP or magnetic resonance cholangiopancreatography (MRCP) showing cystic dilation corresponding to type and proximal obstruction in presence of obstruction

• • Type of choledochal cyst

• • History and physical exam

  • Liver function tests

  • CBC

  • Amylase and lipase

  • Abdominal US

  • ERCP or MRCP (adults)

  • HIDA scan or MRCP (children)

Surgery

• • Cyst excision and biliary reconstruction (types I-III)

  • Types IV and V individualized and may require partial hepatectomy if unilobar involvement

Treatment Monitoring

• • Surveillance for carcinoma since patients still at increased risk for remainder of biliary tree

Complications

• • Biliary stricture or leak

  • Cholangitis

References