CHOLEDOCHAL CYST

CHOLEDOCHAL CYST

Choledochal cysts are congenital dilatations of the intra- and/ or extrahepatic biliary system. The pathogenesis is unclear. Anomalous insertions of the biliary–pancreatic junction are frequently observed, but whether or not these play a role in the pathogenesis of the condition is unclear. Todani and colleagues proposed a classification of cystic disease of biliary tract ( Figure 71.24 ). Patients may present at any age with jaundice, fever, abdominal pain and a right upper quadrant mass on examina tion; 60% of cases are diagnosed before the age of 10 years. Pancreatitis is not an infrequent presentation in adults. P atients with choledochal cysts have an increased risk of developing c holangiocarcinoma, with the risk varying directly with the age at diagnosis. Takuji Todani , b. 1931, Department of Surgery , Okayama University Medical School, Okayama, Japan, modified Alonso-Lej’s classification of choledochal cysts in 1977. USG confirms the presence of an abnormal cyst and mag - netic resonance imaging (MRI)/MRCP will reveal the anat - - omy , in particular the relationship between the lower end of - the bile duct and the pancreatic duct. CT is also useful for delineating the extent of intra- or extrahepatic dilatation. Radical excision of the cyst is the treatment of c hoice, with reconstruction of the biliary tract using a Roux-en-Y loop of jejunum. Complete resection is important because of an asso - ciation with the later de velopment of cholangiocarcinoma. Resection and Roux-en-Y reconstruction is also associated with a reduced incidence of stricture formation and recur - rent cholangitis. Type III needs endoscopic management with sphincterotomy . It should be accompanied by biopsy of the cyst epithelium to exclude dysplasia in symptomatic cases and in young patients without symptoms.

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