[Clinical analysis of 6 cases with atypical vulvovaginal malformations]

To investigate clinical features and management of complicated or rare vulvovaginal anomalies derived from urogenital sinus malformation.

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.

This kind of vulvovaginal 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 bimanual examination, one orifice located under clitoris, the other orifice was anus. No vaginal 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 (infrasphincteric), presenting with distal vaginal atresia and urethrovaginal fistula; type III was high communication (suprasphincteric) with distal vaginal atresia and more proximal urethrovaginal 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 was to reconstruct perineal appearance, release the obstruction, and repair the defect and ultimately recovering the normal function. The alternative surgical methods should be considered individually.

The vulvovaginal 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.