Abstract
BACKGROUND: Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a difference (disorder) of sex development that results from Müllerian duct aplasia in 46,XX females. The diagnosis of MRKHS is usually established in late adolescence. The purpose of the study was to assess the impact of congenital absence of uterus and vagina (CAUV) on a patient's psychosexual functioning. PARTICIPANTS AND PROCEDURE: Thirty-two women with MRKHS (mean age 22.9 years) and 32 matched healthy controls (mean age 24.75 years) completed three study questionnaires: the Sexual Self-Esteem Inventory for Women, the Rosenberg Self-Esteem Scale, and Minnesota Multiphasic Personality Inventory-2 (MMPI-2). Statistical analysis was performed by IBM SPSS Statistics 22. RESULTS: There was no difference in global self-esteem between the two study groups. MRKHS females had lower sexual self-esteem and experienced higher intensity of some psychological functioning characteristics (paranoia, psychasthenia, schizophrenia) than their peers. Correlations between sexual self-esteem and results on depression, psychopathic deviate, schizophrenia, social introversion and anxiety scales were observed in patients with MRKHS. Global self-esteem and schizophrenia results were significant predictors of sexual self-esteem in the clinical group. Higher global self-esteem and lower results in the schizophrenia scale were associated with higher sexual self-esteem in patients with MRKHS. CONCLUSIONS: Psychological and medical counseling of women with MRKHS should address their impaired sexual self-esteem, especially sexual skills and experiences. While the number of diagnostic responses indicating the presence of specific symptoms in MRKHS females is statistically significantly different, the level of scores obtained does not exceed the threshold of clinical pathology.