Bile duct cysts in adults: a multi-institutional retrospective study. French Associations for Surgical Research.

Objective: To review the features of adult patients undergoing surgery for bile duct cysts, focusing on the anatomy of the biliary tree as well as the long-term outcome.

Summary Background Data: Bile duct cysts (BDCs) are uncommon in Western countries, and the majority of reported cases originate from Asia. Japanese authors have emphasized the frequent association of extra- and intrahepatic bile duct dilatations, but grading of patients based on Todani's classification is often hindered by the absence of an accurate definition of types IC and IVA cysts. Moreover, despite the increasing use of extrahepatic cyst excision, little is known about the long-term outcome in patients with intrahepatic bile duct involvement.

Methods: Forty-two adult patients with BDC were treated between 1980 and 1992 in 17 institutions of the French Associations for Surgical Research. Clinical presentation, radiologic presurgical evaluation, and surgical procedures were analyzed. The long-term postsurgical outcome was derived from patient charts, attending physicians, or direct patient contact.

Results: Twelve patients (30%) had recurrent abdominal pain or jaundice from childhood. Seven (17%) had undergone prior cystenterostomy. Twenty-one (50%) had a Todani-type IVA cyst with extra- and intrahepatic bile duct involvement. Of these, nine had segmental, exclusively left-sided intrahepatic bile duct dilatation. Biliary carcinoma was encountered in five patients (12%). Extrahepatic cyst excision with a Roux-en-Y hepaticojejunostomy was performed in 34 patients with type I or IV cysts. The overall operative mortality rate was 2.4%. Long-term results were clearly correlated with cyst type: during a mean follow-up of 8.4 years, 11 of 12 patients (92%) treated by cyst excision for type I cyst remained free of symptoms, whereas 31% of patients who underwent surgery for type IV cyst had episodic or severe cholangitis with intrahepatic stones.

Conclusions: In patients with BDC, particular attention must be given to the associated intrahepatic bile duct dilatations. We propose a modification of Todani's classification to distinguish cystic, segmental, and fusiform dilatations of the intrahepatic biliary tree in type IV cysts. In patients with segmental left intrahepatic cystic dilatations, combined left liver lobectomy and extrahepatic cyst excision is suggested to decrease late postsurgical biliary complications.