evolution of the quality of life and experience of young women with utero-vaginal aplasia (mrkhpsy)

Conditions: Mrkh Syndrome

Summary: this study aims to evaluate the global evolution of the quality of life on patients diagnosed with mrkh syndrome (mayer-rokitansky-kuster-hauser), 8 to 10 years after the first research.

between 2012 and 2015, the french national reference center for rare gynecological pathologies (pgr) has conducted a study with 2 axis: a medical and a psychological section (clinical trial number nct01911884). the medical axis's aim was to compare the global and sexual quality of life of mrkh patients according to the mode of care (surgical or non-surgical) for vaginal aplasia. a total of 131 patients, with a mean age of 26.5 (±5.5) years old, were recruited for the study. the quality of life was assessed considering 3 quality of life scales: global (whoqol-bref) and sexual (fsfi and fsds-r). quality of life scores and vaginal depth were compared and revealed that the surgery was not superior to self-dilation, whether in terms of quality of life or anatomical results. in addition, surgery was associated with complications in half of the cases. it should be mentioned that the overall quality of life of all 131 patients was similar to that of the general population, but significantly impaired in the psychosocial dimension in the younger patients. notably, the quality of sexual life was not impaired with fsfi scores similar to those of the general population, but there was a profound sexual distress in nearly 71% of patients according to fsds-r scores. the psychological component of this initial research (called t1) focused specifically on the psychological, and more widely on the psychosocial, impact of the diagnosis of utero-vaginal aplasia in adolescent girls. 40 patients out of the 131 in the medical component participated (the first 40 contacted to participate in the medical component research). the results revealed a real experience of disability, and unexpectedly, addictive reactionary disorders (anorexia/bulimia or sport addiction), and an overrepresentation of heterosexual orientation compared to the general population. the absence of a uterus led to severe moral suffering regarding projections of access to biological motherhood. this new research (called t2) involves the same population as t1 with the object of making each patient her own witness. the purpose of the current study is to assess the evolution of this suffering, 8 to 10 years after the t1 study, using the same methodology as the previous study. the investigators hope to find an improvement in quality of life, a disappearance of addictive disorders and possibly a more vivid, but painful expression due to age, of the desire for motherhood, even if the uterus transplant is a source of hope. finding predictive factors of psychosocial difficulties would allow teh investigators to improve care practices, mainly psychological, which could also benefit other women with a rare disease affecting their sexuality and/or fertility.

Eligibility criteria:
inclusion criteria: * patient aged of 18 years old or older * patient who participated in the initial t1 psychological component of the previous study (mrkh protocol) exclusion criteria - refusal to participate in the study

Keywords: mayer-rokitansky-kuster-hauser syndrome quality of life utero-vaginal aplasia