Choledochal cyst is rarely diagnosed in adulthood. When complicated by biliary tract malignancy, the disease has a distinct presentation and carries a dismal prognosis despite radical surgical resection.
Tertiary referral center.
A retrospective study was performed on 30 adult patients who presented with choledochal cyst from January 1, 1989, to December 31, 2000.
Main Outcome Measures
The clinical presentation, management, and outcome of patients with and without biliary tract malignancy.
Nine patients (30%) had biliary tract malignancy complicating choledochal cyst (group A). Compared with 21 patients without malignancy (group B), group A patients had a significantly higher incidence of previous internal drainage operations for choledochal cyst (P = .049) and presentation with cholangitis (P = .03). Four patients in group A underwent pancreaticoduodenectomy and 3 received a palliative biliary drainage operation. The overall median survival of patients in group A was 12 months. Complete excision of choledochal cyst and Roux-en-Y hepaticojejunostomy were performed for all patients in group B, among whom 2 underwent concomitant hemihepatectomy. The operative morbidity and mortality were 14% and 0%, respectively, and there were no long-term complications with a median follow-up of 66 months.
Biliary tract malignancy complicating choledochal cyst in adults should be suspected in patients with a history of internal drainage of choledochal cyst and presentation with cholangitis. Complete excision of choledochal cyst with Roux-en-Y hepaticojejunostomy is the treatment of choice for patients without malignancy and can be performed with low operative morbidity and absence of long-term complications in adult patients.