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  • • 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

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Epidemiology

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  • • Onset of symptoms usually in infancy or childhood

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Symptoms and Signs

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  • • Jaundice

    • Fever

    • Pain

    • Palpable right upper quadrant mass

    • Hepatomegaly

    • Bleeding varices

    • Asymptomatic

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Laboratory Findings

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  • • Hyperbilirubinemia

    • Elevated alkaline phosphatase

    • Leukocytosis

    • Elevated amylase and lipase

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Imaging Findings

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  • • 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

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  • • Type of choledochal cyst

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  • • History and physical exam

    • Liver function tests

    • CBC

    • Amylase and lipase

    • Abdominal US

    • ERCP or MRCP (adults)

    • HIDA scan or MRCP (children)

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Surgery

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

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

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Treatment Monitoring

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

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Complications

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  • • Biliary stricture or leak

    • Cholangitis

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References

Vercruysse R, Van den Bossche MR. Choledochal cyst in adults. Acta Chir Belg. 1998;98:220.  [PubMed: 9830548]
Watanatittan S, Niramis R: Choledochal cyst: review of 74 pediatric cases. J Med Assoc Thai 1998;81:586.  [PubMed: 9737111]

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