BILE-DUCT CYSTS IN ADULTS - A MULTIINSTITUTIONAL RETROSPECTIVE STUDY

Objective To review the features of adult patients undergoing surgery for bile duct cysts, focusing on the anatomy of the biliary tree as we ll as the long-term outcome. Summary Background Data Bile duct cysts ( BDCs) are uncommon in Western countries and the majority of reported c ases originate from Asia. Japanese authors have emphasized the frequen t association of extra- and intrahepatic bile duct dilatation, but gra ding of patients based on Todani's classification is often hindered by the absence of an accurate definition of types IC and IVA cysts. More over, despite the increasing use of extrahepatic cyst excision, little is known about the long-term outcome in patients with intrahepatic bi le duct involvement. Methods Forty-two adult patients with BDC were tr eated between 1980 and 1992 in 17 institutions of the French Associati ons for Surgical Research. Clinical presentation, radiologic presurgic al evaluation, and surgical evaluation, and surgical procedures were a nalyzed. The long-term postsurgical outcome was derived from patient c harts, attending physicians, or direct patient contact. Results Twelve patients (30%) had recurrent abdominal pain or jaundice from childhoo d. Seven (17%) had undergone prior cystenterostomy. Twenty-one (50%) h ad a Todani-type IVA cyst with extra- and intrahepatic bile duct invol vement. Of these, nine had segmental, exclusively left-sided intrahepa tic bile duct dilatation. Biliary carcinoma was encountered in five pa tients with type I or IV cysts. The overall operative mortality rate w as 2.4%. Long-term results were clearly correlated with cyst type: dur ing 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% o f patients who underwent surgery for type IV cyst had episodic or seve re 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 classifi cation to distinguish cystic, segmental, and fusiform dilatations of t he intrahepatic biliary tree in type IV cysts. In patients with segmen tal left intrahepatic cystic dilatations, combined left liver lobectom y and extrahepatic cyst excision is suggested to decrease late postsur gical biliary complications.