102例不同类型阴道斜隔综合征的临床特征  

Clinical features of 102 patients with different types of Herlyn-Werner-Wunderlich syndrome

作　　者：蒋建发[1] 易水晶[1] JIANG Jianfa;YI Shuijing(Department of Obstetrics and Gynecology,Third Xiangya Hospital,Central South University,Changsha 410013,China)

机构地区：[1]中南大学湘雅三医院妇产科,长沙410013

出　　处：《中南大学学报（医学版）》2023年第4期550-556,共7页Journal of Central South University ：Medical Science

基　　金：湖南省自然科学基金(2022JJ40744);中南大学湘雅三医院汇智育才计划(YX202112)。

摘　　要：目的:阴道斜隔综合征(Herlyn-Werner-Wunderlich syndrome,HWWS)是一种少见的女性生殖道畸形,国内外少有大样本的研究报道。该综合征临床表现多样,认识不足可能延误诊治。本研究分析不同类型HWWS患者的临床特点,以期提高妇产科医师对该病的诊治水平。方法:回顾性分析2009年10月1日至2022年4月5日在中南大学湘雅三医院妇产科住院治疗的HWWS患者的临床资料。收集患者的年龄、病史、体格检查和影像学检查结果、治疗情况等进行分析。将患者分为无孔斜隔型(I型)、有孔斜隔型(II型)和无孔斜隔合并子宫颈瘘管型(III型),比较不同类型HWWS患者的临床特点。结果:共纳入102例HWWS患者,年龄10~46岁,其中I型37例(36.27%),II型50例(49.02%),III型15例(14.71%)。所有患者均于月经初潮后诊治,诊断年龄(20.5±7.4)岁。3种类型HWWS患者的诊断年龄和病程的差异均有统计学意义(均P<0.05)。I型患者的诊断年龄[(18.0±6.0)岁]最小,且病程最短(中位病程6个月),而III型患者的诊断年龄[(22.9±9.8)岁]最大,病程最长(中位病程48个月)。I型患者的临床表现以痛经为主,II型及III型患者以异常阴道出血为主。在102例患者中,67例(65.69%)患者为双子宫,33例(32.35%)为纵隔子宫,2例(1.96%)为双角子宫。绝大部分患者为斜隔侧的肾缺如,仅1例患者为斜隔侧的肾发育不良。45例(44.12%)患者斜隔位于左侧,57例(55.88%)患者斜隔位于右侧。3种类型HWWS患者在子宫形态、泌尿系统畸形、盆腔肿块及斜隔侧别上的差异均无统计学意义(均P>0.05)。6例(5.88%)患者合并卵巢巧克力囊肿,4例(3.92%)合并盆腔脓肿,5例(4.90%)合并输卵管积水。所有患者均行阴道斜隔切除术,其中42例患者因无性生活史,行无损伤处女膜宫腔镜下阴道斜隔切开术,余60例患者行传统的阴道斜隔切开术。获得102例中的89例患者的随访资料,随访时长为术后1个月至12年不等。89例�Objective:Herlyn-Werner-Wunderlich syndrome(HWWS)is a rare female genital tract malformation,and there are few large sample studies reported at home and abroad.The clinical manifestations of this syndrome are diverse,and insufficient understanding may delay the diagnosis and treatment of the patients.This study aims to analyze the clinical characteristics of different types of HWWS patients,and to improve the diagnosis and treatment of HWWS.Methods:The clinical data of patients with HWWS who were hospitalized in the Department of Obstetrics and Gynecology,Third Xiangya Hospital of Central South University from October 1,2009 to April 5,2022 were retrospectively analyzed.The age,medical history,physical examination,imaging examination and treatment of the patients were collected for statistical analysis.The patients were divided into an imperforate oblique vaginal septum type,a perforate oblique vaginal septum type,and an imperforate oblique vaginal septum and cervical fistula type.The clinical characteristics of different types of HWWS patients were compared.Results:A total of 102 HWWS patients were enrolled,with age of 10−46 years old,including 37(36.27%)patients with type I,50(49.02%)type II,and 15(14.71%)type III.All patients were diagnosed after menarche,with an average age of(20.5±7.4)years.There were significant differences in the age of diagnosis and course of disease among the 3 types of HWWS patients(both P<0.05).Patients with type I had the youngest age of diagnosis[(18.0±6.0)years]and the shortest course of disease(median course of 6 months),while patients with type III had the oldest age of diagnosis[(22.9±9.8)years]and the longest course of disease(median course of 48 months).The main clinical manifestation of type I was dysmenorrhea,and the main clinical manifestation of type II and type III was abnormal vaginal bleeding.Of the 102 patients,67(65.69%)patients had double uterus,33(32.35%)had septate uterus,and 2(1.96%)had bicornuate uterus.The vast majority of patients had renal agenesis on the

关 键 词：阴道斜隔综合征 子宫畸形 痛经 肾缺如 

分 类 号：R711.1[医药卫生—妇产科学]