特殊类型外阴阴道畸形六例临床分析

目的 分析6例特殊类型的外阴阴道畸形患者的临床特点,探讨其分型及处理.方法 回顾1998年3月至2008年3月北京协和医院收治的6例特殊类型外阴阴道畸形(即与泌尿生殖赛发育异常相关的外阴阴道畸形)患者的临床资料,根据胚胎发育学及解剖学特点对其进行分型,并总结其处理方式.结果 此类外阴阴道畸形罕见,也未被纳入目前的分类及诊断系统,但该类患者的临床表现具有娃著共性:月经正常来潮,但经血与尿液自同一孔道流出;妇科检查发现会阴仅见2个孔道,前方孔道位于阴蒂下方,似尿道外口,后方孔道为肛门,之间无类似阴道前庭和阴道外口的结构.本研究根据其特征分为3型,即:Ⅰ型(3例):小阴唇融合,即泌尿生殖窦末端存留;Ⅱ型(1例):远端尿道阴道瘘伴阴道闭锁(尿道外括约肌下型),阴道与尿道低位交通;Ⅲ型(2例):近端尿道阴道瘘伴阴道闭锁(尿道外括约肌上型),阴道与尿道高位交通,严重者可伴消化道及其他系统发育异常.6例患者均接受手术治疗,不同类型的畸形患者术式也不相同,但多可经1次手术完成,术后患者外阴形态接近正常,未见阴道狭窄.结论 特殊类型外阴阴道畸形结构复杂多样,对其进行分型有利于临床诊断和处理,但手术设计仍应个体化,在争取满意的解剖与功能恢复的同时,应兼顾美学、性学及心理学需求.

Clinical analysis of 6 cases with atypical vulvovaginal malformations

Objective To investigate clinical features and management of complicated or rare vulvovaginal anomalies derived from urogenital sinus malformation.Methods Medical documents of 6 cases with vulvovaginal anomalies treated in Department of Obstetrics and Gynecology in Peking Union Medical College Hospital in the past 10 years were reviewed retrospectively.According to embryological and anatomical characteristics,the classification system were established.Results This kind of vulvovngihal anomalies was very rare and not enrolled into current diagnosis and treatment system. All patients had a common clinical features:menstruation bleeding and urine from the same outflow tract;and two orifices on perineum were found by bimannal examination,one orifice located under clitoris,the other orifice Was anus.No vagihal vestibule and vulvar were observed between those two orifices.According to clinical features,those 6 cases were divided into 3 types:type Ⅰ Was persistence of distant urogenital sinus with clinical manifestation of simple labial fusion;type Ⅱ was low communication between vagina and urogenital sinus (infrasphincterie),presenting with distal vaginai atresia and urethrovagihal fistula;type Ⅲ was high communication (suprasphincteric) with distal vagihal atresia and more proximal urethrovavihal fistula.Type Ⅲ was frequently associated with severe rectum and anus anomaly,and other malformation as well.All patients were treated by surgical correction.Basically.the principle of operation wag to reconstruct perineal appearance,release the obstruction,and rear the defect and ultimately recovering the normal function.The alternative surgical methods should be considered individually.Conclusions The vulvovnginal anomalies derived from urogenital sinus malformation were complicated clinically.To establish classification system will be benefit for clinicians to make diagnosis and choose appropriate treatment. The individualized surgery should be performed on those patients to restore the structure and function,in the mean time,the factors associated with esthetics.sex and psychics should be considered.