先天性胆管囊肿 Todani Ⅱ型：法国外科协会的欧洲多中心研究及文献复习。

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

机构信息

*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.

出版信息

Ann Surg. 2015 Jul;262(1):130-8. doi: 10.1097/SLA.0000000000000761.

DOI:10.1097/SLA.0000000000000761

PMID:24979598

Abstract

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.

摘要

目的

本研究旨在分析患有胆管憩室（即 Todani Ⅱ型先天性胆管囊肿）患者的临床表现、手术治疗方法和长期预后。

背景

该病发病率占所有报道的先天性胆管囊肿病例的 0.8%至 5%之间，但其临床表现、治疗方法和结局的相关信息匮乏。

方法

法国外科协会开展了一项多中心欧洲回顾性研究。将患者的病历资料纳入一个网站数据库中。对诊断性影像学研究、手术和病理报告进行了中心审核。

结果

在 350 例先天性胆管囊肿患者中，有 19 例（5.4%）为Ⅱ型，其中 17 例为成人（89.5%），2 例为儿童。胆管憩室位于肝外胆管的上段、中段和下段分别为 11 例（58%）、4 例（21%）和 4 例（21%）。6 例（31.6%）患者表现为复杂型，包括 1 例同时性癌。所有患者均行憩室切除术，11 例（58%）需要联合切除肝外胆管，复杂型临床表现有助于预测该术式。无死亡病例。89.5%的患者（中位随访时间：52 个月）长期预后良好。