Choledochal Cyst in Childhood

Chun, Yong Soon; Jung, Sung Eun; Lee, Seong Cheol; Park, Kwi Won; Kim, Woo Ki

Abstract

BACKGROUND: Choledochal cysts are not so rare in far east Asian countries as in western countries and are found mainly in children. Reflux of pancreatic juice to the bile duct through anomalous pancreaticobiliary ductal union (APBDU) is considered to be an important etiologic factor. METHOD: To study the clinical characteristics of choledochal cysts in children and the significance of APBDU in this disease, we reviewed 80 choledochal cyst cases. APBDU is defined as a long common channel (>1.5 cm).
RESULTS
Ages ranged from 19 days to 13 years, and the male to female ratio was 1 to 4 which revealed a predominance among females. The symptoms, in order of frequency, were abdominal pain (63%), jaundice (48%), and abdominal mass (26%); classic triads were present in 3 cases (4%). The diagnosis was made mainly with ultrasonography. Todani type I (88%) and IV (11%) were the two most common types of cysts. An operative cholangiography was performed in 80 cases, among which an analysis of the status of the pancreaticobiliary ductal union was possible in 21 cases. A long common channel (>1.5 cm) was found in all 21 cases, 16 cases being of the P- type and 5 cases of the B- type. A cyst excision and hepaticojejunostomy was performed in 79 cases of type I and type IV cysts, and a liver biopsy only was performed in one case of a type V cyst. A common bile duct stone was associated with one case and cholangitis was associated with two cases as postoperative complications. One patient expired due to heart disease. Seventy-ine patients were followed up for a mean period of 7.2 years after the operation. Seventy-ight patients are healthy now, and liver function tests have returned to normal level. One Carolie's disease patient has been followed up outpatiently without bad liver function. An intraoperative liver biopsy was performed in 73 cases. The results were normal in 3 cases; cholestasis was noted in 2 cases, inflammation in 26, fibrosis in 38, and cirrhosis in 4.
CONCLUSIONS
APBDU seems to play an important role in the pathogenesis of choledochal cysts, especially type I and type IV cysts. Excision of the cyst is the procedure of choice for treatment because it eliminates the APBDU and prevents both hepatic cirrhosis and the potential for developing a malignancy.