Adult's congenital bile duct cysts

Congenital bile duct cysts (CBDC) is a rare congenital malformation. It results from an anomaly of the biliopancreatic junction (AJBP). This condition is often diagnosed at a young age. Although, it can be asymptomatic and discovered only at an advanced age. The aim of our work was to describe the diagnosis, therapeutic and evolutionary aspects of BVCD through a series of 11 cases diagnosed in adult cases.

This is a descriptive, retrospective and monocentric study. It collects patients operated for CBDC between 01/08/1999 and 30/06/2009.

The mean age was 45.3 years. Two men and nine women. The right hypochondria pain has been reported by all patients. On physical examination, jaundice was noted in five cases and the rest of the examination was normal. Biology showed cholestasis in six cases and cytolysis in four cases. Only one patient had hyperamylasaemia (five times normal). The preoperative diagnosis of a VBCD was reported in eight cases. Peroperative cholangiography (OCP) allowed to make the diagnosis and classify the CBDC according to the classification of Todani. Microscopic examination demonstrated three cases of associated gallbladder adenocarcinoma. The operative follow-up was simple for ten cases. A case of surgical recovery was necessary due to infection of necrotic pancreatitis. The mean follow-up was 40.6 months with extremes from one month to seven years. Only one case of death has been reported. No patient has presented a later biliary tract degeneration. Only one case of secondary biliary cirrhosis due to repeated attacks of angiocholitis has been reported.

CBDC is rare. It must be suspected at any age. It is characterized by the increased risk of degeneration. Radiological examinations can suspect the diagnosis in younger and asymptomatic patients to ensure a well-conducted and timely surgical treatment.