The Missing Uterus, the Missed Diagnosis, and the Missing Care. Mayer-Rokitansky-Küster-Hauser Syndrome in the Lives of Women in Malaysia

Abstract

Study objective: To explore the effect of the diagnosis of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome on affected Malaysian women.

Design: Qualitative study with a quantitative component.

Setting: Pediatric and adolescent gynecology unit at Universiti Kebangsaan Malaysia Medical Centre, Malaysia.

Participants: Twelve women with MRKH.

Interventions: Face-to-face interview and short questionnaire.

Main outcome measures: Thematic analysis was used to understand participants' experiences.

Results: There were 7 themes identified: (1) delayed diagnoses; (2) doctors' roles and attitudes; (3) gender identity; (4) family and society's response; (5) reaction toward infertility; (6) managing sexual intimacy; and (7) coping mechanisms. Several participants consulted their physicians regarding their primary amenorrhea at an opportunistic setting. When they were referred to the gynecologists, they were dismayed at the lack of information given. The term, "MRKH" plays an important role to ease information-seeking. Participants felt that the doctors were insensitive toward them. Mental illness is a significant complication of MRKH. All participants acknowledged that infertility was the hardest part of the condition. The importance of blood lineage affects their outlook on childbearing options. Some were afraid of sexual intimacy and worried that they would not be able to satisfy their partners. Participants gained support and bonded with their counterparts in the MRKH support group.

Conclusion: A multidisciplinary approach including medical, psychological, and social support is essential for the management of MRKH. Adequate information and sexual education plays the utmost importance in preventing social-related complications of MRKH.

Keywords: Adolescent; DSD; MRKH; Müllerian agenesis; Uterine transplant.