Management of congenital female genital tract anomalies related to primary amenorrhea and/or cyclic abdominal pain: A retrospective cohort study

Abstract

Introduction

Surgical treatment for patients with complex female genital anomalies (FGA) depends on the specific type of anomaly, its level of complexity, the symptoms experienced by the patient, and a thorough understanding of the embryological origins of the anomaly. The aim of this study is to analyze the results of the surgical treatment of young female patients with complex FGA presented with primary amenorrhea and/or cyclic pelvic pain and present their treatment algorithm.

Material and Methods

This retrospective cohort study analyzed prospectively collected data of adolescents and young women with FGA presented with primary amenorrhea and/or cyclic pelvic pain. Forty-eight patients of 11 different types of FGA, evaluated and treated in our Department between January 2007 and June 2025, were included.

Results

All patients were treated surgically. The design of the surgical treatment was based on the existing uterine body, cervical, and vaginal anatomy. Immediate surgery was performed in patients with obstructive pain, whereas nonobstructive cases were treated near anticipated sexual activity. Neovagina creation was performed in 26 cases of vaginal aplasia; isthmo-neovagina anastomosis was performed in one patient with cervical aplasia and a normal uterus and in two patients with a rudimentary cavity, while excision of the rudimentary cavity was performed in a third one. Two patients developed rectovaginal fistula, which was managed successfully. Surgical treatment of a patient with an obstructed bicorporeal complete uterus combined with cervical and vaginal aplasia was unsuccessful, leading to hysterectomy. All transverse vaginal septae were excised, one through posterior colpotomy. One patient with a complete bicorporeal uterus and unilateral cervical aplasia underwent successful anastomosis and another one underwent excision of the obstructed uterine body. Rudimentary cavities of unicorporeal uteri were removed.

Conclusions

Surgical treatment of FGA presenting with primary amenorrhea and/or cyclic pelvic pain should be anatomy-driven, with priority given to symptom relief and preservation of reproductive potential by restoring genital tract continuity, when feasible, or removing obstructed parts.