About 75% of all choledochal cysts present in childhood. The classical presentation of abdominal pain, jaundice and right upper quadrant mass occurs in about 33% of patients. There may be acute cholangitis - fever, right upper quadrant tenderness, jaundice and leukocytosis, biliary cirrhosis or diffuse peritonitis following rupture of the cyst.
Carcinoma may develop in the cyst.
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