Sexuality in women with Mayer-Rokitansky-Küster-Hauser syndrome

Czech version

Authors: Chmel Roman Jr. ^1,2; Nováčková Marta ¹; Chubanovová Nikoleta ¹; Pastor Zlatko ¹
Authors‘ workplace: Gynekologicko-porodnická klinika 2. LF UK a FN Motol, Praha ¹; LF UK, Plzeň ²
Published in: Ceska Gynekol 2021; 86(3): 194-199
Category: Review Article
doi: https://doi.org/10.48095/cccg2021194

Overview

Objective: Evaluation of the quality of sexual life of women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) after neovagina creation using dilation and surgical techniques.

Methods: Literature search of articles published in the years 2000–2021 using the databases Web of Science, Google Scholar and PubMed by key words: Mayer-Rokitansky-Küster-Hauser syndrome, neovagina, female sexuality and Female Sexual Function Index. We used a standardized international Female Sexual Function Index and other quantitative and qualitative parameters of sexual satisfaction to assess sexual function and overall satisfaction in women with neovagina.

Results: It is possible to develop functional neovagina in women with MRKHS by either conservative or surgical techniques. The choice of the method used depends on the experience and skill of the surgeon, on the technical possibilities and equipment of the department, and on the anatomical predispositions and preferences of the patient. Satisfaction with sexual life also depends on the psychosexual maturity and sexual demands of both partners as well as on regular vaginal rehabilitation and quality of long-term psychological support. The functional results of dilation and surgical methods are comparable according to objective criteria. They only differ in individual aspects which correspond to the overall personality traits as well as to the character and quality of cohabitation.

Conclusion: Women with MRKHS are primarily handicapped by coital insufficiency and inability to reproduce naturally. Creating a neovagina technically allows them to realize satisfactory coitus, but in most cases, they are still frustrated by the primary problem of „physical indisposition“ and complicated path to motherhood.

Keywords:

Mayer-Rokitansky-Küster-Hauser syndrome – neovagina – Female Sexual Function Index – female sexuality

Sources

1. Oppelt P, Renner SP, Kellermann A et al. The clinical aspects of Mayer-Rokitansky-Kuester-Hauser syndrome: recommendations for clinical diagnosis and staging. Hum Reprod 2006; 21 (3): 792–797. doi: 10.1093/humrep/dei381.

2. Saravelos SH, Cocksedge KA, Li TC. Prevalence and diagnosis of congenital uterine anomalies in women with reproductive failure: a critical appraisal. Hum Reprod Update 2008; 14 (5): 415–429. doi: 10.1093/humupd/dmn018.

3. Folch M, Pigem I, Konje JC. Müllerian agenesis: etiology, diagnosis, and management. Obstet Gynecol Surv 2000; 55 (10): 644–649. doi: 10.1097/00006254-200010000-00023.

4. Ventriglio A, Bhugra D. Sexuality in the 21st century: sexual fluidity. East Asian Arch Psychiatry 2019; 29 (1): 30–34.

5. McQuillan SK, Grover SR. Systematic review of sexual function and satisfaction following the management of vaginal agenesis. Int Urogynecol J 2014; 25 (10): 1313–1320. doi: 10.1007/s00192-013-2316-3.

6. Pastor Z, Fronek J, Novackova M et al. Sexual life of women with Mayer-Rokitansky-Küster-Hauser syndrome after laparoscopic Vecchietti’s vaginoplasty. Sex Med 2017; 5 (2): e106– –e113. doi: 10.1016/j.esxm.2016.12.003.

7. Committee on Adolescent Health Care. ACOG Committee Opinion No. 728: Müllerian agenesis: diagnosis, management, and treatment. Obstet Gynecol 2018; 131 (1): e35–e42. doi: 10.1097/AOG.0000000000002458.

8. Carrard C, Chevret-Measson M, Lunel A et al. Sexuality after sigmoid vaginoplasty in patients with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril 2012; 97 (3): 691–696. doi: 10.1016/j.fertnstert.2011.12.015.

9. Rosen R, Brown C, Heiman J et al. The Female Sexual Function Index (FSFI): a multidimensional self-report instrument for the assessment of female sexual function. J Sex Marital Ther 2000; 26 (2): 191–208. doi: 10.1080/009262300278597.

10. Fedele L, Bianchi S, Frontino G et al. The laparoscopic Vecchietti‘s modified technique in Rokitansky syndrome: anatomic, functional, and sexual long-term results. Am J Obstet Gynecol 2008; 198 (4): 377.e1–e6. doi: 10.1016/j.ajog.2007.10.807.

11. Hořejší J. Congenital developmental defects of derivates of müllerian ducts. Endocr Dev 2012; 22: 251–270. doi: 10.1159/000331689.

12. Chmel R Jr, Pastor Z, Mužík M et al. Syndrom Mayer-Rokitansky-Küster-Hauser – ageneze dělohy a pochvy: aktuální znalosti a terapeutické možnosti. Ceska Gynekol 2019; 84 (5): 386–392.

13. Dabaghi S, Zandi M, Ilkhani M. Sexual satisfaction in patients with Mayer-Rokitansky-Küster-Hauser syndrome after surgical and non-surgical techniques: a systematic review. Int Urogynecol J 2019; 30 (3): 353–362. doi: 10.1007/s00192-018-3854-5.

14. Kang J, Chen N, Song S et al. Sexual function and quality of life after the creation of a neovagina in women with Mayer-Rokitansky-Küster-Hauser syndrome: comparison of vaginal dilation and surgical procedures. Fertil Steril 2020; 113 (5): 1024–1031. doi: 10.1016/j.fertnstert.2020.01.017.

15. Giannesi A, Marchiole P, Benchaib M et al. Sexuality after laparoscopic Davydov in patients affected by congenital complete vaginal agenesis associated with uterine agenesis or hypoplasia. Hum Reprod 2005; 20 (10): 2954–2957. doi: 10.1093/humrep/dei152.

16. Fedele L, Frontino G, Restelli E et al. Creation of a neovagina by Davydov‘s laparoscopic modified technique in patients with Rokitansky syndrome. Am J Obstet Gynecol 2010; 202 (1): 33.e1–e6. doi: 10.1016/j.ajog.2009.08.035.

17. Callens N, De Cuypere G, De Sutter P et al. An update on surgical and non-surgical treatments for vaginal hypoplasia. Hum Reprod Update 2014; 20 (5): 775–801. doi: 10.1093/humupd/ dmu024.

18. Vatsa R, Bharti J, Roy KK et al. Evaluation of amnion in creation of neovagina in women with Mayer-Rokitansky-Kuster-Hauser syndrome. Fertil Steril 2017; 108 (2): 341–345. doi: 10.1016/j.fertnstert.2017.05.026.

19. Bianchi S, Frontino G, Ciappina N et al. Creation of a neovagina in Rokitansky syndrome: comparison between two laparoscopic techniques. Fertil Steril 2011; 95 (3): 1098–1100.e1–e3. doi: 10.1016/j.fertnstert.2010.11.032.

20. Aly AM. Functional outcome after McIndoe vaginoplasty. Egypt J Plast Reconstr Surg 2012; 36 (1): 19–24.

21. Fotopoulou C, Sehouli J, Gehrmann N et al. Functional and anatomic results of amnion vaginoplasty in young women with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril 2010; 94 (1): 317–323. doi: 10.1016/j.fertnstert.2009. 01.154.

22. Panici PB, Maffucci D, Ceccarelli S et al. Autologous in vitro cultured vaginal tissue for vaginoplasty in women with Mayer-Rokitansky-Küster-Hauser syndrome: anatomic and functional results. J Minim Invasive Gynecol 2015; 22 (2): 205–211. doi: 10.1016/j.jmig.2014.09.012.

23. Lloyd J, Crouch NS, Minto CL et al. Female genital appearance: „normality“ unfolds. BJOG 2005; 112 (5): 643–646. doi: 10.1111/j.14 71-0528.2004.00517.x.

24. Adamiak-Godlewska A, Skorupska K, Rechberger T et al. Urogynecological and sexual functions after Vecchietti reconstructive surgery. Biomed Res Int 2019; 2019: 2360185. doi: 10.1155/2019/2360185.

25. Kimberley N, Hutson JM, Southwell BR et al. Well-being and sexual function outcomes in women with vaginal agenesis. Fertil Steril 2011; 95 (1): 238–241. doi: 10.1016/j.fertnstert. 2010.05.061.

26. Csermely T, Halvax L, Sárkány A et al. Sexual function after modified laparoscopic Vecchietti‘s vaginoplasty. J Pediatr Adolesc Gynecol 2011; 24 (3): 147–152. doi: 10.1016/j.jpag.2010.12. 008.

27. Both S, Kluivers K, Kate-Booij MT et al. Sexual response in women with Mayer-Rokitansky-Küster-Hauser syndrome with a nonsurgical neovagina. Am J Obstet Gynecol 2018; 219 (3): 283.e1–283.e8. doi: 10.1016/j.ajog.2018.07.012.

28. Weijenborg PT, Kluivers KB, Dessens AB et al. Sexual functioning, sexual esteem, genital self-image and psychological and relational functioning in women with Mayer-Rokitansky-Küster-Hauser syndrome: a case-control study. Hum Reprod 2019; 34 (9): 1661–1673. doi: 10.1093/humrep/dez130.

29. Liu X, Liu M, Hua K et al. Sexuality after laparoscopic peritoneal vaginoplasty in women with Mayer-Rokitansky-Kuster-Hauser syndrome. J Minim Invasive Gynecol 2009; 16 (6): 720–729. doi: 10.1016/j.jmig.2009.07.018.

30. Communal PH, Chevret-Measson M, Golfier F et al. Sexuality after sigmoid colpopoiesis in patients with Mayer-Rokitansky-Kuster-Hauser Syndrome. Fertil Steril 2003; 80 (3): 600–606. doi: 10.1016/s0015-0282 (03) 00796-9.

31. Labus LD, Djordjevic ML, Stanojevic DS et al. Rectosigmoid vaginoplasty in patients with vaginal agenesis: sexual and psychosocial outcomes. Sex Health 2011; 8 (3): 427–430. doi: 10.1071/SH10105.

32. Liao LM, Conway GS, Ismail-Pratt I et al. Emotional and sexual wellness and quality of life in women with Rokitansky syndrome. Am J Obstet Gynecol 2011; 205 (2): 117.e1–e6. doi: 10.1016/j.ajog.2011.03.013.

33. Minto CL, Liao KL, Conway GS et al. Sexual function in women with complete androgen insensitivity syndrome. Fertil Steril 2003; 80 (1): 157–164. doi: 10.1016/s0015-0282 (03) 00501-6.

34. Masters WH, Johnson VE. Human sexual response. Toronto: Bantam Books 1966.

35. Callens N, De Cuypere G, Wolffenbuttel KP et al. Long-term psychosexual and anatomical outcome after vaginal dilation or vaginoplasty: a comparative study. J Sex Med 2012; 9 (7): 1842––1851. doi: 10.1111/j.1743-6109.2012.02747.x.

36. Chmel R, Novačková M, Pastor Z et al. The interest of women with Mayer-Rokitansky-Küster-Hauser syndrome and laparoscopic Vecchietti neovagina in uterus transplantation. J Pediatr Adolesc Gynecol 2018; 31 (5): 480–484. doi: 10.1016/j.jpag.2018.04.007.

37. Chmel R, Pastor Z, Matecha J et al. Uterine transplantation in an era of successful childbirths from living and deceased donor uteri: current challenges. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2020; 164 (1): 115–120. doi: 10.5507/bp.2019.020.