The impact of Mayer–Rokitansky–Küster–Hauser Syndrome on Psychology, Quality of Life, and Sexual Life of Patients: A Systematic Review

Abstract

Background: Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is a rare congenital syndrome characterized by uterovaginal agenesis. Most patients are diagnosed during adolescence, when body image and sexual identity are shaped. Our main objective was to investigate how MRKH syndrome affects psychology, quality of life (QoL), and the sexual life of patients compared with non-affected individuals. Methods: Original peer-reviewed research papers examining psychological outcomes, QoL, and sexual function of MRKH patients were searched in PubMed. Titles, abstracts, and full text from potentially eligible records were reviewed by two independent reviewers. Case reports and papers published not in English were excluded. Results: Our search identified 63 records, of which 20 were included: 10 examined psychological and psychosocial outcomes, 14 examined sexual function outcomes, and 6 examined QoL outcomes. Results may be affected by selection bias and confounding due to differences between MRKH patients and controls. Conclusions: MRKH could be associated with a higher prevalence of anxiety and depression symptoms and social insecurity compared with women of a similar age without the condition. MRKH could also be associated with greater pain and discomfort during sexual intercourse and limitations in arousal, lubrication, and orgasm. MRKH patients more commonly experience impairment of mental-health-related QoL, but physical-health-related QoL is not affected.

Functional genomics analysis identifies loss ofHNF1Bfunction as a cause of Mayer–Rokitansky–Küster–Hauser syndrome

“…Like other Müllerian anomalies, this congenital condition is characterized by variable presentations and a complex, undefined genetics ( 58 ). MRKH syndrome denies affected women the possibility of conceiving and bearing children, and its almost invariably unexpected diagnosis can result in distress, confusion, depression and shame ( 7 ). The situation is exacerbated by the current inability of healthcare professionals to provide a molecular diagnosis and hence an explanation for how the condition arose in any given affected woman.…”

“…Because women with MRKH syndrome develop otherwise typical female secondary sex characteristics, most are not diagnosed until adolescence due to primary amenorrhea. The diagnosis typically causes significant psychological distress for these women and their families ( 7 ).…”

An Investigation of Depressive Symptoms in Patients with Congenital Cervical and Vaginal Aplasia: A Cross-Sectional Study

“…Patients were asked to complete the items on a scale of 0 to 3. The score is divided into five categories: no (0-4), mild (5-9), moderate (10)(11)(12)(13)(14), moderately severe (15)(16)(17)(18)(19), and severe (20 or above). The test for depressive symptoms was based on a cut-off score of 10.…”

“…Therefore, depressive symptom screening in MRKH patients is of great importance. 9,10 We believe that patients with congenital cervical and vaginal aplasia have more serious problems than those with MRKH, such as cyclic abdominal pain and endometriosis. If patients have depression and other psychological problems, it may affect the postoperative management.…”

The Impact of COVID-19 on the Motivations of Women Seeking a Uterus Transplant

“…Patients with MRKH often present in puberty around the age of 16 years with amenorrhoea in the presence of normal secondary pubertal characteristics. This diagnosis often impacts on their sense of wellbeing and quality of life [ 6 ]. The discovery of the loss of childbearing at this early stage of their life has a profound impact on their psychological wellbeing and remains so as they progress through into adulthood [ 7 , 8 ].…”