- Localised cystic dilatation of all or part of the common bile duct
- 80% present in childhood
- Most common in Japan (1 in 1000 live births)
- Relatively rare in Western Europe (1 in 100,000 live births)
- Male : female ratio is 1:4
- Aetiology unknown
Pathology
- Cyst wall consists of fibrous tissue without muscle
- May contain up to 2 litres of fluid
- Often associated with distal common bile duct stenosis
- If not diagnosed they can progress to biliary fibrosis, cirrhosis and liver failure
- Three types are described
Presentation
- May be diagnosed prenatally on ultrasound
- 25% present as neonates with prolonged jaundice and cholestasis
- 75% later in childhood with triad of:
- Abdominal pain
- Abdominal mass
- Intermittent jaundice
- Differential diagnosis includes biliary atresia and neonatal hepatitis
Investigation
- Abdominal ultrasound usually reveals the cyst
- Cyst can also be imaged by ERCP or PTC
- Radioisotope scanning may show delayed biliary excretion and accumulation in the cyst
Complications
- Recurrent cholangitis
- Hepatic fibrosis
- Biliary cirrhosis and portal hypertension
- Rupture with biliary peritonitis
- Pancreatitis
- Hepatic abscess
- Gall stones
- Carcinoma of the biliary tree
Treatment
- Type I and II cysts
- Cyst should be resected and a hepaticojejunostomy performed
- Prevents anastomotic stricture and malignancy in the cyst
- Postoperative cholangitis is uncommon
- Type III cysts
- Cholecystectomy and choledochojejunostomy or choledochoduodenostomy
Prognosis
- If diagnoses early liver fibrosis regresses and normal hepatic function can be expected
- Prognosis is poor if advanced disease with portal hypertension
Bibliography
Howard E R. Hepatobiliary surgery in children. In: Johnson C D, Taylor I eds. Recent advances in
surgery 23. Edinburgh, Churchill Livingston, 2000: 69-84.
Kabra V, Agrawal M, Adulka T K, Dixit V K, Agrawal A K, Shukla V K.
Choledochal cyst: a changing pattern of presentation. Aust NZ J Surg 2001; 71:
159-161
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