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Mayer-Rokitansky-Küster-Hauser综合征的磁共振成像与临床特征:来自专业专科中心的10年回顾

Magnetic resonance imaging and clinical features of Mayer-Rokitansky-Küster-Hauser syndrome: A 10-year review from a dedicated specialist centre.

作者信息

Cooper Nina, Al-Memar Maya, Linton-Reid Kristofer, Edmonds Keith, Rose Gillian, Dixon Nuala, McNamara Cillian, Fotopoulou Christina, Ree Katherine Van, Bharwani Nishat

机构信息

Department of Disorders for Reproductive Development, Queen Charlotte's & Chelsea Hospital, Imperial College Healthcare NHS Trust, London, UK.

Department of Metabolism, Digestion and Reproduction, Faculty of Medicine, Imperial College London, London, UK.

出版信息

BJOG. 2025 Jan;132(1):64-71. doi: 10.1111/1471-0528.17928. Epub 2024 Aug 12.

Abstract

OBJECTIVE

To correlate the clinical history with imaging findings of women with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome.

DESIGN

Retrospective cohort study.

SETTING

A UK IOTA and ESGO-certified tertiary referral centre for disorders of reproductive development.

POPULATION

All patients with a diagnosis of MRKH and who had undergone an MRI pelvis between 1 January 2011 and 31 April 2021 were included.

METHODS

MRI images were analysed by specialist gynaecological radiologists. Clinical data was extracted from an electronic patient record system. Statistical analysis was computed in R (version 4.1.2), R base stats package and ggstatsplot (v0.5.0).

MAIN OUTCOME MEASURES

Clinical history and predefined imaging features.

RESULTS

One hundred and thirty-four patients were included. Median age at MRI was 18 years (10-64 years). Half (48.2%) of women presenting had a history of pain, most often abdominal (84.6%) or vaginal (9.2%). Remnants were identified in 91.8% of women (n = 123). 4.5% of women had imaging features of endometriosis (n = 6). Women with a functional remnants were significantly more likely to experience pain (p < 0.001). Pain history was not strongly associated with ectopic ovarian position. Common gynaecological pathology such as endometriosis, ovarian cysts and fibroids were also identified.

CONCLUSIONS

We identify that majority of women with MRKH will have uterine remnants with a connecting fibrous band, and an ectopic ovarian position 44.0% of cases. Abdominal pain was significantly associated with functional remnants on MRI. Further work is required to identify how other gynaecological pathology impacts women with MRKH.

摘要

目的

将 Mayer-Rokitansky-Küster-Hauser(MRKH)综合征女性的临床病史与影像学表现相关联。

设计

回顾性队列研究。

设置

一家英国 IOTA 和 ESGO 认证的生殖发育障碍三级转诊中心。

研究对象

纳入所有诊断为 MRKH 且在 2011 年 1 月 1 日至 2021 年 4 月 31 日期间接受过盆腔 MRI 检查的患者。

方法

由专业妇科放射科医生分析 MRI 图像。从电子病历系统中提取临床数据。使用 R(版本 4.1.2)、R 基础统计包和 ggstatsplot(v0.5.0)进行统计分析。

主要观察指标

临床病史和预定义的影像学特征。

结果

共纳入 134 例患者。MRI 检查时的中位年龄为 18 岁(10 - 64 岁)。半数(48.2%)就诊女性有疼痛病史,最常见的是腹痛(84.6%)或阴道痛(9.2%)。91.8%的女性(n = 123)发现有残余物。4.5%的女性有子宫内膜异位症的影像学特征(n = 6)。有功能性残余物的女性更易出现疼痛(p < 0.001)。疼痛病史与异位卵巢位置无强关联。还发现了常见的妇科病理情况,如子宫内膜异位症、卵巢囊肿和子宫肌瘤。

结论

我们发现大多数 MRKH 综合征女性会有带连接纤维带的子宫残余物,44.0%的病例有异位卵巢位置。腹痛与 MRI 上的功能性残余物显著相关。需要进一步研究以确定其他妇科病理情况如何影响 MRKH 综合征女性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c31c/11612609/8251748fa609/BJO-132-64-g001.jpg

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