Restricted accessReview articleFirst published online 2021-1
Psychological impact and health-related quality-of-life outcomes of Mayer–Rokitansky–Küster–Hauser syndrome: A systematic review and narrative synthesis
Mayer–Rokitansky–Küster–Hauser syndrome causes absence or underdevelopment of uterus and vagina, but women’s subjective experience remains understudied. This systematic review was conducted to examine the psychological and health-related quality-of-life outcomes of Mayer–Rokitansky–Küster–Hauser syndrome. In total, 22 articles identified through electronic search matched the inclusion criteria and were included in our review. Mayer–Rokitansky–Küster–Hauser syndrome may be associated with psychological symptoms and impaired quality of life, but especially with poor sexual esteem and genital image. Women may experience difficulties managing intimacy and disclosing to partners. Mothers may be perceived as overinvolved, with consequent negative emotions in women with the disease.
Bargiel-MatusiewiczKKroemekeA (2015) Personality traits and coping styles in women with Mayer-Rokitansky-Küster-Hauser syndrome. Archives of Medical Science11: 1244–1249.
2.
Bargiel-MatusiewiczKKroemekeAPolańskaK (2013) The relationship between neuroticism, coping styles and emotions in women with Mayer-Rokitansky-Küster-Hauser syndrome: A moderated mediation analysis. Polish Psychological Bulletin44: 1–8.
3.
BeanEJMazurTRobinsonAD (2009) Mayer-Rokitansky-Küster-Hauser syndrome: Sexuality, psychological effects, and quality of life. Journal of Pediatric and Adolescent Gynecology22: 339–346.
4.
BeisertMJSzymańska-PytlińskaMKapczukK, et al. (2015) Sexual activity of women with Mayer-Rokitansky-Küster-Hauser Syndrome (MRKHS): Preliminary study. Ginekologia Polska86: 648–652.
5.
BombardDSMousaSA (2014) Mayer-Rokitansky-Kuster-Hauser syndrome: Complications, diagnosis and possible treatment options: A review. Gynecological Endocrinology30: 618–623.
6.
BourdelNChauvetPBilloneV, et al. (2019) Systematic review of quality of life measures in patients with endometriosis. PLoS ONE14: e0208464.
7.
BrunnerFFliegnerMKruppK, et al. (2016) Gender role, gender identity and sexual orientation in CAIS (“XY-women”) compared with subfertile and infertile 46,XX women. The Journal of Sex Research53: 109–124.
8.
CanziEAccordiniMFacchinF (2019) “Is blood thicker than water?” Donor conceived offspring’s subjective experiences of the donor: A systematic narrative review. Reproductive Biomedicine Online38: 797–807.
9.
CarrardCChevret-MeassonMLunelA, et al. (2012) Sexuality after sigmoid vaginoplasty in patients with Mayer-Rokitansky-Küster-Hauser syndrome. Fertility and Sterility97: 691–696.
10.
CheikhelardABidetMBaptisteA, et al. (2018) Surgery is not superior to dilation for the management of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome: A multicenter comparative observational study in 131 patients. American Journal of Obstetrics & Gynecology219: 281.e1–281.e9.
11.
CulleyLLawCHudsonN, et al. (2013) The social and psychological impact of endometriosis on women’s lives: A critical narrative review. Human Reproduction Update19: 625–639.
12.
DabaghiSZandiMIlkhaniM (2019) Sexual satisfaction in patients with Mayer-Rokitansky-Küster-Hauser syndrome after surgical and non-surgical techniques: A systematic review. International Urogynecology Journal30: 353–362.
13.
DearJCreightonSMConwayGS, et al. (2019) Sexual experience before treatment for vaginal agenesis: A retrospective review of 137 women. Journal of Pediatric and Adolescent Gynecology32: 300–304.
14.
Dixon-WoodsMAgarwalSJonesD, et al. (2005) Synthesising qualitative and quantitative evidence: A review of possible methods. Journal of Health Services and Research Policy10: 45–53.
15.
EjzenbergDAndrausWBaratelli Carelli MendesLR, et al. (2019) Livebirth after uterus transplantation from a deceased donor in a recipient with uterine infertility. Lancet392: 2697–2704.
16.
ErnstMESandbergDEKeeganC, et al. (2016) The lived experience of MRKH: Sharing health information with peers. Journal of Pediatric and Adolescent Gynecology29: 154–158.
17.
FedeleLFrontinoGMottaF, et al. (2010) Creation of a neovagina in Rokitansky patients with a pelvic kidney: Comparison of long-term results of the modified Vecchietti and McIndoe techniques. Fertility and Sterility93: 1280–12805.
18.
FliegnerMKruppKBrunnerF, et al. (2014) Sexual life and sexual wellness in individuals with Complete Androgen Insensitivity Syndrome (CAIS) and Mayer-Rokitansky-Küster-Hauser Syndrome (MRKHS). The Journal of Sexual Medicine11: 729–742.
19.
FliegnerMRichter-AppeltHKruppK, et al. (2018) Living with permanent infertility: A German study on attitudes toward motherhood in individuals with Complete Androgen Insensitivity Syndrome (CAIS) and Mayer-Rokitansky-Küster-Hauser Syndrome (MRKHS). Health Care for Women International39: 1295–1315.
20.
GattiCDel RossiCLombardiL, et al. (2010) Sexuality and psychosocial functioning in young women after colovaginoplasty. The Journal of Urology184: 1799–1803.
21.
Heller-BoersmaJGSchmidtUHEdmondsDK (2007) A randomized controlled trial of a cognitive-behavioural group intervention versus waiting-list control for women with uterovaginal agenesis (Mayer-Rokitansky-Küster-Hauser syndrome: MRKH). Human Reproduction22: 2296–2301.
22.
Heller-BoersmaJGSchmidtUHEdmondsDK (2009) Psychological distress in women with uterovaginal agenesis (Mayer-Rokitansky-Küster-Hauser syndrome, MRKH). Psychosomatics50: 277–281.
23.
HerlinMBjørnAMRasmussenM, et al. (2016) Prevalence and patient characteristics of Mayer-Rokitansky-Küster-Hauser syndrome: A nationwide registry-based study. Human Reproduction31: 2384–2390.
24.
KimberleyNHutsonJMSouthwellBR, et al. (2011) Well-being and sexual function outcomes in women with vaginal agenesis. Fertility and Sterility95: 238–241.
25.
KruppKFliegnerMBrunnerF, et al. (2012) How do individuals with Complete Androgen Insensitivity Syndrome, Mayer-Rokitansky-Küster-Hauser syndrome or polycystic ovary syndrome experience contact to other affected persons?Geburtshilfe und Frauenheilkunde72: 1009–1017.
26.
LabusLDDjordjevicMLStanojevicDS, et al. (2011) Rectosigmoid vaginoplasty in patients with vaginal agenesis: Sexual and psychosocial outcomes. Sexual Health8: 427–430.
27.
LaggariVDiaremeSChristogiorgosS, et al. (2009) Anxiety and depression in adolescents with polycystic ovary syndrome and Mayer-Rokitansky-Küster-Hauser syndrome. Journal of Psychosomatic Obstetrics & Gynecology30: 83–88.
28.
LeithnerKNadererAHartungD, et al. (2015) Sexual and psychosocial functioning in women with MRKHS after neovaginoplasty according to Wharton-Sheares-George: A case control study. PLoS ONE10: e0124604.
29.
LiaoLMConwayGSIsmail-PrattI, et al. (2011) Emotional and sexual wellness and quality of life in women with Rokitansky syndrome. American Journal of Obstetrics & Gynecology205: 117.e1–e6.
30.
MoherDLiberatiATetzlaffJ, et al. (2010) Preferred Reporting Items for Systematic Reviews and Meta-Analyses: The PRISMA statement. International Journal of Surgery8: 336–341.
31.
PastorZFroněkJNováčkováM, et al. (2017) Sexual life of women with Mayer-Rokitansky-Küster-Hauser syndrome after laparoscopic Vecchietti vaginoplasty. Sexual Medicine5: e106–e113.
32.
PattersonCJCrawfordRJahodaA (2016) Exploring the psychological impact of Mayer-Rokitansky-Küster-Hauser syndrome on young women: An interpretative phenomenological analysis. Journal of Health Psychology21: 1228–1240.
33.
SabatucciIPalaiaIMarcheseC, et al. (2019) Treatment of the Mayer-Rokitansky-Küster-Hauser syndrome with autologous in vitro cultured vaginal tissue: Descriptive study of long-term results and patient outcomes. BJOG: An International Journal of Obstetrics & Gynaecology126: 123–127.
34.
ShepherdJHardenAReesR, et al. (2006) Young people and healthy eating: A systematic review of research on barriers and facilitators. Health Education Research21: 239–257.
35.
WatanabeKKobayashiYBannoK, et al. (2017) Recent advances in the molecular mechanisms of Mayer-Rokitansky-Küster-Hauser syndrome. Biomedical Reports7: 123–127.
36.
WeijenborgPTMKluiversKBDessensAB, et al. (2019) Sexual functioning, sexual esteem, genital self-image and psychological and relational functioning in women with Mayer-Rokitansky-Küster-Hauser syndrome: A case-control study. Human Reproduction34: 1661–1673.
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