Department of Obstetrics and Gynaecology, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong.
Hong Kong Med J. 2020 Oct;26(5):397-403. doi: 10.12809/hkmj208467. Epub 2020 Oct 16.
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.
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.
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.
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.
Mayer-Rokitansky-Küster-Hauser（MRKH）综合征是一种罕见的先天性畸形，其特征为阴道和子宫的发育不全或发育不良。在这里，我们描述了在一家三级医院中对 MRKH 综合征患者的治疗。
这项回顾性研究纳入了在一家三级医院儿科和青少年妇科诊所就诊的 MRKH 综合征患者。记录了他们的临床表现、检查以及新阴道创建方法。在接受阴道扩张（VD）的患者中，评估了治疗持续时间、VD 前和 VD 后的阴道宽度和长度、并发症以及性活动和性交困难的结局。
确定了 49 例 MRKH 综合征患者。她们的初诊年龄平均为 17.9 岁；69.4%和 24.5%的患者因原发性闭经治疗和阴道成形术就诊。48 例患者的肾脏影像学检查结果正常，核型为 46，XX。17 例（34.7%）患者作为一线治疗接受了 VD；3 例未完成治疗。2 例行手术阴道成形术，5 例仅通过性交获得了足够的阴道长度；25 例尚未要求 VD。VD 的平均持续时间为 16±10.2（范围 4-35）周。VD 前后阴道的宽度和长度分别为 1.1±0.28cm 和 1.3±0.7cm，3.1±0.5cm 和 6.9±0.9cm。VD 的总体成功率为 92.3%。阴道出血是最常见的并发症（21%）；仅有 1 例患者报告性交困难。
Mayer-Rokitansky-Küster-Hauser 综合征是一种罕见的疾病，需要多学科专家的治疗。阴道扩张是创建新阴道的有效一线方法。
