Adult choledochal cyst.

OBJECTIVE

Choledochal cyst is a rare disease in adults. Excellent results have been achieved with proper management. However, malignancy complicating choledochal cyst still carries a dismal prognosis despite radical surgery. The aim of this study was to review the clinical course and operative results of the disease in adulthood, with emphasis on the occurrence of malignancy.

METHODS

A retrospective review of adult patients who underwent surgery for choledochal cysts in a 12-year period was performed.

RESULTS

Of the 25 adults, 80% were female and the median age was 30 years. A total of 32% of patients had previous drainage procedures for their choledochal cyst disease. Malignancy was noted in five patients (20%). There were significantly more males among the patients with malignancy than among those with benign disease (60% vs 10%), a greater number of older patients (median age, 39 vs 27 years) and more impaired liver function tests, but there was no difference in terms of incidence of previous drainage procedures between the two groups. There was no operative mortality; operative morbidity was 36%. No significant long-term complications were noted in the benign group but three of the five patients with malignancy died or developed recurrence within a few months after surgery.

CONCLUSION

The prognosis for patients with malignancy complicating choledochal cyst remains poor despite aggressive radical surgery such as Whipple's operation or additional hepatectomy. Malignancy should be suspected in older and male patients who present with jaundice or impaired liver function tests. Total cyst excision and hepaticojejunostomy is an effective and safe treatment for patients with the common type I choledochal cyst in order to reduce the chance of subsequent development of malignancy.