Rokitansky Syndrome or Cervicovaginal Atresia?

Abstract

Study objective: To investigate the possibility of conservative management of rudimentary uterine horns associated with vaginal agenesis.

Design: Observational study on cohort of consecutive cases treated with the same criteria from 2008 to 2021.

Setting: Two academic institutions and teaching hospitals in Milan, Italy.

Patients: Eight patients with vaginal agenesis associated with rudimentary cavitated uterine horns treated by the same team and postoperatively followed.

Interventions: All the subjects underwent the same standardized surgical procedure: laparoscopy, intraoperative ultrasound, and horn-vestibular direct anastomosis. Postoperatively vaginoscopy was performed every 6 months.

Measurements and main results: The postoperative course was generally uneventful and the mean hospital stay was 4.3 ± 2.5 (SD) days. All the patients began to menstruate a few months after the operation. Menstrual flows were light but regular. All patients had a neovaginal length > 4 cm at 1 year postoperatively, reaching approximately 6 cm at 2 years. During the follow-up period, 5 patients were sexually active without dyspareunia. In all cases, surgery restored the continuity of the neovagina and uterine horn through the creation of a "vaginal-horn fistula tract."

Conclusion: In patients with vaginal agenesis associated with the presence of a uterine cavitary horn, it is possible to recover not only sexual activity but also menstrual function. The horn-vestibular anastomosis may be considered a valid, safe, and effective therapeutic option but requires accurate preoperative and intraoperative evaluation of rudimentary uterine structures.

Keywords: Cervicovaginal atresia; Müllerian anomalies; Neovagina; Rokitansky syndrome; Uterine rudimentary horns.