34例胆管消失综合征的单中心诊疗经验

目的探讨胆管消失综合征(VBDS)的病因及诊疗经验。方法回顾性分析20年来成都市第六人民医院普外科经肝穿刺病理学检查确诊的34例VBDS患者的临床、内镜逆行性胰胆管造影术(ERCP)、磁共振胰胆管造影术(MRCP)检查、病理及随访资料。结果 34例VBDS患者的平均年龄(35.5±6.3)岁,男女之比为2.1∶1,临床表现以黄疸、皮肤瘙痒为主要症状,常见病因有:先天性畸形、恶性病变、缺血因素、免疫因素、感染因素、药物因素、中毒因素、特发性等。早期治疗以激素及对症观察治疗为主,晚期需要肝移植治疗。随访1~18年,2例(5.9%)治愈,4例(11.8%)黄疸时好时发,11例(32.4%)长期轻度到中度黄疸,17例(50.0%)死亡。结论 VBDS成年人多见,多为后天因素所致;儿童少见,多为感染和先天性胆道发育不良。不同病因引起的VBDS预后和转归不同。早期干预预后较好,发展至终末期肝病需要肝移植。ERCP和MRCP结合临床有助于早期诊断。

Experience of 34 cases of vanishing bile duct syndrome ： a single center

Objective To investigate etiology and treatment of the vanishing bile duct syndrome （VBDS）.Methods The data of 34 cases of VBDS,which were diagnosed by liver biopsy pathology in our hospital during the past 20 years were analyzed retrospectively.Results The mean age of 34 cases of VBDS was （35.5 ± 6.3） years old,male to female ratio was 2.1∶ 1.Jaundice and pruritus were the main symptoms.Common causes were congenital malformations,malignant lesions,ischemic factors,immune factors,infectious factors,drug factors,poisoning factors and idiopathic.Hormones and symptomatic treatment were the main therapy in the early stage,liver transplantation was needed in the end stage.During 1 ～ 18 years follow-up,there were 2 patients （5.9％） healing,4 patients （11.8％） with sometimes jaundice,11 patients （32.4％） with long-term mild to moderate jaundice and 17 patients （50.0％） dead.Conclusion Incidence of VBDS are more in men than women.VBDS are more common in adults,mostly due to acquired factors,and less common in children,mostly due to infections and congenital biliary hypoplasia.The prognosis and outcome of different VBDS are different due to the causes.Early intervention is good prognosis,but liver transplantation is required when to the end-stage.ERCP and MRCP combined with clinical data could diagnose earlier than pathological diagnosis.