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Todani Type II Congenital Bile Duct Cyst: European Multicenter Study of the French Surgical Association and Literature Review

Ouaïssi, Mehdi MD, PhD*; Kianmanesh, Reza MD, PhD; Belghiti, Jacques MD, PhD; Ragot, Emilia MD; Mentha, Gilles MD, PhD§; Adham, Mustapha MD, PhD; Troisi, Roberto Ivan MD, PhD, FEBS; Pruvot, François-René MD, PhD**; Dugué, Laurent MD††; Paye, François MD, PhD‡‡; Ayav, Ahmet MD, PhD§§; Nuzzo, Gennaro MD¶¶; Falconi, Massimo MD‖‖; Demartines, Nicolas MD, FACS***; Mabrut, Jean-Yves MD, PhD†††; Gigot, Jean-François MD, PhD‡‡‡; the Working Group of the French Surgical Association

doi: 10.1097/SLA.0000000000000761
Original Articles

Objective: The purpose of the study was to analyze clinical presentation, surgical management, and long-term outcome of patients suffering from biliary diverticulum, namely Todani type II congenital bile duct cyst (BDC).

Background: The disease incidence ranges between 0.8% and 5% of all reported BDC cases with a lack of information about clinical presentation, management, and outcome.

Methods: A multicenter European retrospective study was conducted by the French Surgical Association. The patients' medical records were included in a Web site database. Diagnostic imaging studies, operative and pathology reports underwent central revision.

Results: Among 350 patients with congenital BDC, 19 type II were identified (5.4%), 17 in adults (89.5%) and 2 in children. The biliary diverticulum was located at the upper, middle, and lower part of the extrahepatic biliary tree in 11, 4, and 4 patients (58%, 21%, and 21%, respectively). Complicated presentation occurred in 6 patients (31.6%), including one case of synchronous carcinoma. Surgical techniques included diverticulum excision in all patients. Associated resection of the extrahepatic biliary tree was required in 11 cases (58%) and could be predicted by the presence of complicated clinical presentation. There was no mortality. Long-term outcome was excellent in 89.5% of patients (median follow-uptime: 52 months).

Conclusions: According to the present largest Western series of Todani type II BDC, the type of clinical presentation rather than BDC location, was able to guide the extent of biliary resection. Excellent long-term outcome can be achieved in expert centers.

Congenital biliary diverticulum, namely, Todani type II bile duct cyst, is a rare disease. Surgical resection is the treatment of choice, requiring associated bile duct resection in 58% of the patients with excellent long-term outcome. Synchronous cancer may be associated.

*Department of General and Digestive Surgery, Timone Hospital, Marseille, France

Department of Digestive and Endocrine Surgery, Robert Debré Hospital, Reims, France

Department of HBP Surgery, Beaujon Hospital, Clichy, France

§Department of Digestive Surgery, University Hospital of Geneva, Geneva, Switzerland

Department of HBP Surgery, Edouard Herriot Hospital, Lyon, France

Department of General and Hepato-Biliary Surgery, Liver Transplantation Service Surgery, Ghent University Hospital Medical School, Ghent, Belgium

**Department of HBP Surgery, Claude Huriez Hospital, Lille, France

††Department of Digestive Surgery, Saint Camille Hospital, Bry-sur-Marne, France

‡‡Department of General and Digestive Surgery, Saint-Antoine Hospital, Paris, France

§§Department of General and Digestive Surgery, University Hospital of Nancy, Nancy, France

¶¶Department of HBP Surgery, Gemelli University Hospital, Roma, Italy

‖‖Clinica Chirurgia del Pancreas, Universita Politecnica delle Marche A.O.U. Ospedali Riuniti, Torrette-Ancona, Italy

***Department of Digestive Surgery, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland

†††Department of Digestive Surgery and Liver Transplantation, La Croix-Rousse Hospital, Lyon, France

‡‡‡Department of Abdominal Surgery and Transplantation, Cliniques Universitaires Saint-Luc, Universite Catholique de Louvain (UCL), Brussels, Belgium.

Reprints: Jean-François Gigot, MD, PhD, Division of Hepato-Biliary and Pancreatic Surgery, Department of Abdominal Surgery and Transplantation, Cliniques Universitaires Saint-Luc, Universite Catholique de Louvain (UCL), Ave Hippocrate, 10, 1200 Brussels, Belgium. E-mail: jean-francois.gigot@uclouvain.be.

Disclosure: The authors declare no conflicts of interest.

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