Current approach of patients with Mayer-Rokitansky-Küster-Hauser syndrome

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Obstet Gynecol Sci. 2025;68(1):90-91
Publication date (electronic) : 2024 November 20
doi : https://doi.org/10.5468/ogs.24099
1Department of Gynaecological Oncology, Metaxa Cancer Hospital, Piraeus, Greece
2Department of Obstetrics and Gynecology, Helena Venizelou Hospital, Athens, Greece
3Athens Colorectal Laboratory, Athens, Greece
4Department of Colorectal Surgery, Royal Devon and Exeter NHS Foundation Trust, Exeter, UK
Corresponding author: Ioannis D. Gkegkes, MD, PhD Department of Colorectal Surgery, Royal Devon and Exeter NHS Foundation Trust, Barrack Rd, Exeter EX2 5DS, UK E-mail: ioannisgkegkes@gmail.com
Received 2024 April 6; Accepted 2024 November 11.

Dear Editor, it was with great interest that we read the article titled “clinical features and management of women with Mayer-Rokitansky-Küster-Hauser syndrome in a Thai population” by Matemanosak et al. [1]. The authors summarized their twenty-year experience with a cohort of 96 patients with Mayer-Rokitansky-Küster-Hauser syndrome.

We would like to thank the authors for presenting the classification description of anatomical variations in their patients and highlighting the fact that the degree of vaginal atresia can be negatively related to the patients’ sexual and psychological health. Regarding patient management, it is notable that in this cohort, approximately 50% of the patients remained untreated because they had not engaged in sexual intercourse (although some may question whether there may be a relationship between psychological consequences and difficulties with intercourse). Furthermore, no difficulties with sexual intercourse were noted in 41.7% of patients using either self-dilatation therapy or coital dilatation. Only a minority of patients (8.3%) underwent vaginal reconstruction.

Conservative management includes self-dilatation or Vecchietti vaginoplasty to generate progressive traction on the vaginal stump. Surgical formation of the neovagina using mucous/cutaneous, peritoneal, or ileal/sigmoid grafts or internal thigh fasciocutaneous cutaneous flaps is another approach [2]. We would also like to highlight that uterine transplantation can be offered to such patients as an option for uterine factor infertility [3]. Around thirty live births after uterine transplantation have been described to date [4].

Current recommendations emphasize dilatation therapy as the first-line treatment for vaginal agenesis and/or coital dilatation in patients who are able to regularly engage in sexual intercourse. Further studies investigating the criteria for surgical intervention and functional outcomes are needed to apply a safe, acceptable technique that can enable patients to engage in early sexual intercourse with a low incidence of shrinkage and a high rate of patient satisfaction.

We would like to thank the authors for sharing their valuable experience.

Notes

Conflict of interest

No potential conflicts of interest relevant to this article were reported.

Ethical approval

None.

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None.

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None.

References

1. Matemanosak P, Peeyananjarassri K, Klangsin S, Wattanakumtornkul S, Dhanaworavibul K, Choksuchat C, et al. Clinical features and management of women with Mayer-Rokitansky-Küster-Hauser syndrome in a Thai population. Obstet Gynecol Sci 2024;67:314–22.
2. Uccella S, Galli L, Vigato E, D’Alessio C, Di Paola R, Garzon S, et al. New neovagina-creating technique on the basis of a fasciocutaneous flap for Müllerian agenesis. Fertil Steril 2024;122:382–4.
3. Iavazzo C, Gkegkes ID. Possible role of DaVinci robot in uterine transplantation. J Turk Ger Gynecol Assoc 2015;16:179–80.
4. Veroux M, Scollo P, Giambra MM, Roscitano G, Giaquinta A, Setacci F, et al. Living-donor uterus transplantation: a clinical review. J Clin Med 2024;13:775.

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