Changes in quality of life and intimate experience after 10 years in young women with mayer-rokitansky-kuster-hauser syndrome

Karinne Gueniche; Alejandra Marquez; Ammar Redha Ould; Magali Viaud; Costa Sabrina Da; Iphigenie Cavadias; Chloe Ouallouche; Michel Polak; Caroline Elie; Alaa Cheikhelard

doi:10.1530/endoabs.110.P1015

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Endocrine Abstracts (2025) 110 P1015 | DOI: 10.1530/endoabs.110.P1015

ECEESPE2025 Poster Presentations Reproductive and Developmental Endocrinology (93 abstracts)

Changes in quality of life and intimate experience after 10 years in young women with mayer-rokitansky-kuster-hauser syndrome

Karinne Gueniche ^1,2,3 , Alejandra Marquez ² , Redha Ould Ammar ^4,5 , Magali Viaud ¹ , Sabrina Da Costa ¹ , Iphigénie Cavadias ¹ , Chloe Ouallouche ¹ , Michel Polak ^1,6 , Caroline Elie ^4,5 & Alaa Cheikhelard ⁷

Author affiliations

¹Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, GHU Paris Centre, Centre de Référence des Pathologies Gynécologiques Rares, Service d’endocrinologie, diabétologie et gynécologie pédiatriques, Paris, France; ²Université Paris Cité, UFR Psychologie - Site Boulogne, ED 261-3CH, Boulogne, France; ³UMR S 1123 ECEVE Université Paris Cité, Paris, France; ⁴Université Paris Cité, UMR 1343 Pharmacologie et évaluation des thérapeutiques chez l’enfant et la femme enceinte, Paris, France; ⁵Hôpital Universitaire Necker Enfants-malades, Assistance Publique - Hôpitaux de Paris, GHU Paris Centre, Unité de Recherche Clinique de l’Hôpital Necker, Paris, France; ⁶Université Paris Cité, Faculté de Médecine, Paris, France; ⁷Hôpital Universitaire Necker Enfants Malades, Assistance Publique - Hôpitaux de Paris, GHU Paris Centre, Centre de Référence des Pathologies Gynécologiques Rares, Service de chirurgie pédiatrique viscérale et urologique / Service d’endocrinologie, diabétologie et gynécologie pédiatriques, Paris, France

JOINT428

Backgroud: MRKH syndrome is a cataclysmic diagnosis with a heavy psychological burden. In a previous national study (T1, n = 131, mean age 26.5y), we reported similar global and sexual quality of life to the general population (GP), except for lower psychosocial health and social relationship scores in younger patients, alongside high sexual distress. In an additional qualitative psychological analysis (n = 40), we reported traumatic handicap experience, unexpected eating/addictive disorders, and fertility distress. All self-defined heterosexual (T1).

Objective: To study their psychological evolution ten years later (T2), using the same methodology.

Study design: 25 patients were included; 8 lost to follow-up, 7 declined. All completed WhoQol-bref (general QoL), FSFI, and FSDS-R (sexual QoL) scales. Qualitative psychological functioning was assessed using clinical interviews, Rorschach and TAT tests. T2 results were compared to previous data T1, after a median 9.6-year interval [IQR 9.2;10].

Results: Median age was 35.7y [32-38.3], (25-35y:n = 11, 35-45y:n = 14). All were sexually active, two self-defined bisexual. Quality of life decreased only in the physical dimension (T1:78,6 – T2:75, difference -7.1 [-14.3;0], P = 0.004) and remained lower than that of the GP (P = 0.008), which was not the case at T1. Eleven patients had new health issues between T1 and T2 (only 2 related to MRKH). Psychological health was much lower than in the other dimensions (scores 66.7 vs 75 to 78.1). Besides, compared to GP, the psychosocial score was significantly lower only in the younger group (62.5 vs 70.8 P = 0.036), similarly to T1. Sexual quality of life and sexual distress were not different between T1 and T2 (FSFI score: T1:26.2 – T2:27.7, difference 1.2 [-0.9;4], P = 0.13), (FSDS-R score: T1:16 – T2:13, difference -2 [-14;4], P = 0.14)). Fourteen (56%) had dyspareunia, which was significantly associated to lower WhoQol psychological scores at T2 (60.4 [58.3;69.8] vs 79.2 [64.6;79.2], P = 0.044), 14(56%) had cyclic pelvic pain. All patients expressed at Rorschach tests perseverant and erroneous ideations on internal genitals including ovaries as a missing organ. Eating disorders disappeared in 2/5 patients who had them at T1. Sports addiction disappeared in 4 patients who had them at T1. Five patients (20%) had children (3 adopted, 2 surrogated). Motivation for parentality declined from 88% (n = 22) to 44% (n = 11) and considering uterine transplantation dropped [T1:36% (n = 8/22) T2:18% (n = 2/11)].

Conclusions: Quality of life remained good after 10 years, except in the psychological domain, especially among younger individuals. Qualitative assessment revealed a higher complex psychosocial impact of uterovaginal aplasia. Dyspareunia was frequent and associated to poor psychological health.