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.
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http://dx.doi.org/10.3390/children9040484 | DOI Listing |
J Pediatr Adolesc Gynecol
December 2024
Queen's University, Department of Psychology. Electronic address:
Study Objective: Mayer Rokitansky Küster Hauser syndrome (MRKH) is characterized by the underdevelopment or absence of the uterus and upper vagina. Navigating healthcare interactions may present unique challenges for those with MRKH as there appears to be sparse clinical education and research available to healthcare providers. Additionally, limited research exists on the healthcare experiences of people with MRKH.
View Article and Find Full Text PDFPak J Med Sci
December 2024
Riffat Jaleel, FCPS, Department of Obstetrics and Gynaecology Unit II, D. Ruth K. M. Pfau Civil Hospital Karachi and, Dow University of Health Sciences, Karachi, Pakistan.
Objective: We aimed to determine frequency, clinical presentation, etiology and management in patients presenting with primary amenorrhea in tertiary care setting.
Method: This was a case series conducted in outpatient Department of Gynecology and Obstetrics unit II, Dr. Ruth K.
Cureus
November 2024
School of Medicine, Hospital Militar Escuela "Dr. Alejandro Dávila Bolaños", Managua, NIC.
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder affecting the female reproductive system, primarily characterized by the absence or underdevelopment of the uterus and upper two-thirds of the vagina, with preserved ovarian function and normal secondary sexual characteristics. It is a rare disease though prevalence may vary based on genetic and environmental factors. This report details a case of a 26-year-old female patient with a history of smoking, alcohol use, and prior inguinal hernioplasty, presenting with primary amenorrhea and inability to engage in vaginal intercourse.
View Article and Find Full Text PDFJ Clin Med
November 2024
Division of Gynaecology, Poznan University of Medical Sciences, Polna 33, 60-535 Poznan, Poland.
Women with an aplastic uterus (ESHRE/ESGE classification) or Müllerian agenesis (ASRM MAC 2021) might present with functional uterine remnants. Our study aimed to report the clinical course of symptomatic uterine rudiments in adolescents and adults with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS). This study involved 20 patients with MRKHS who, between 2012 and 2023, underwent surgery for symptomatic uterine horns at the mean age of 25.
View Article and Find Full Text PDFAust N Z J Obstet Gynaecol
November 2024
School of Clinical Medicine, University of New South Wales, Randwick, New South Wales, Australia.
Background: Vaginal aplasia or hypoplasia often requires the creation of a neovagina using vaginal dilation therapy, vaginoplasty surgery, or a combination of both. However, the absence of validated guidelines and the controversy surrounding vaginoplasty surgery have limited our understanding of the long-term outcomes and impact on quality of life and psychosexual functioning for women with a short or absent vagina. This study provides valuable insights into the Australian context, reflecting the treatment approaches and long-term psychosocial outcomes for this patient group.
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