Treatment of patients with mayer-rokitanskyküster-hauser syndrome in a tertiary hospital

Abstract

Introduction: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is an uncommon congenital malformation characterised by agenesis or hypoplasia of the vagina and uterus. Here, we describe the treatment of patients with MRKH syndrome in a tertiary hospital. Methods: This retrospective study included patients with MRKH syndrome attending the Paediatric and Adolescent Gynaecology Clinic in a tertiary hospital. Their clinical manifestations, examinations, and methods for neovagina creation were recorded. Among patients who underwent vaginal dilation (VD), therapy duration, vaginal width and length at baseline and after VD, complications, and sexual activity and dyspareunia outcomes were evaluated. Results: Forty nine patients with MRKH syndrome were identified. Their mean age at presentation was 17.9 years; 69.4% and 24.5% of patients presented for primary amenorrhoea treatment and vaginoplasty, respectively. Forty eight patients had normal renal imaging findings and 46 XX karyotypes. Seventeen (34.7%) patients underwent VD as first-line therapy; three did not complete the therapy. Two had surgical vaginoplasty, whereas five achieved adequate vaginal length by sexual intercourse alone; 25 had not yet requested VD. The mean duration of VD was 16±10.2 (range, 4-35) weeks. The widths and lengths of the vagina at baseline and after VD were 1.1±0.28 cm and 1.3±0.7 cm, and 3.1±0.5 cm and 6.9±0.9 cm, respectively. The overall success rate of VD was 92.3%. Vaginal spotting was the most common complication (21%); only one patient reported dyspareunia. Conclusions: Mayer-Rokitansky-Küster-Hauser syndrome is an uncommon condition that requires multidisciplinary specialist care. Vaginal dilation is an effective first-line approach for neovagina creation.