ROKITANSKY SYNDROME OR CERVICOVAGINAL ATRESIA ?

Elsevier

Available online 11 May 2023

Journal of Minimally Invasive GynecologyAuthor links open overlay panel, , , , ABSTRACTStudy 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 at 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 therapeutic option, safe and effective but requires accurate preoperative and intraoperative evaluation of rudimentary uterine structures.

Section snippetsINTRODUCTION

With the widespread use of laparoscopy in pediatrics and adolescent gynecology, increasingly Müllerian anomalies are being identified that do not easily fit into the categories of reference classifications (1 Buttram, 2 Pfeifer). It is true that Müllerian duct malformations exhibit wide variability in anatomical presentation and represent a continuum of embryonic changes that occur at different stages of fusion-canalization-resorption. (3 Ludwin), however, the identification of a precise

MATERIALS AND METHODS

This was an observational study performed at two tertiary centres having expertise in managing Mullerian anomalies with reconstructive surgery using minimal access techniques. This study was approved by the Ethics Committee of I.R.C.C.S. S.Raffaele Hospital, Milano (protocol code GARA, register number CE 73/INT/2021).

Between 2008 and 2021, 187 patients with vaginal agenesis were examined and treated at the National University of Milan (2010-2017) and the San Raffaele Life and Health University

RESULTS

All patients and their parents accepted the risk of conservative therapy in a counsellimg session. The anatomic malformation and available treatment options were discussed in details. The advantages and disadvantages of conservative surgical treatment were also discussed, emphasing the limited experience with conservative approaches. The primary outcomes were the creation of a neovagina > 6 cm in lenght, allowing easy insertion of two fingers, and the achievement of a menstrual flow. Surgical

DISCUSSION

In the eight cases described by us, the diagnosis generally adopted by most of the authors would have been that of "Rokitansky syndrome" and the recommended solution would have been the construction of a neovagina and surgical removal of rudimentary cavitated horns. Our study instead shows that in some cases of vaginal agenesis associated with the presence of cavitated rudimentary uterine horns it is possible, in addition to the creation of a neovagina, to recover menstrual function as well. In

CONCLUSIONS

An accurate preoperative and intraoperative evaluation of patients with vaginal agenesis is of paramount importance to identify the presence of rudimentary cavitated uterine horns. In these cases, laparoscopically assisted hornvestibular anastomosis may be considered considered a safe and effective therapeutic option.

Reference

1. Buttram Jr, Veasy C.; Gibbons, William E. Müllerian anomalies: a proposed classification (an analysis of 144 cases). Fertility and sterility, 1979, 32.1: 40-4

2. Pfeifer

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© 2023 Published by Elsevier Inc. on behalf of AAGL.

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