Optimizing care for MRKH patients: From malformation screening to uterus transplantation eligibility.

IF 3.5 2区医学 Q1 OBSTETRICS & GYNECOLOGY

Acta Obstetricia et Gynecologica Scandinavica Pub Date : 2024-10-09 DOI:10.1111/aogs.14985

Auriane Cospain, Ludivine Dion, Maud Bidet, Krystel Nyangoh Timoh, Chloé Quelin, Isis Carton, Alinoe Lavillaureix, Karine Morcel, Paul Rollier, Laurent Pasquier, Bénédicte Nouyou, Sylvie Odent, Daniel Guerrier, Erika Launay, Marc-Antoine Belaud Rotureau, Mélanie Fradin, Sylvie Jaillard, Vincent Lavoué

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引用次数: 0

Abstract

Introduction: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with utero-vaginal aplasia is the most severe form of the Müllerian duct anomalies and can be associated with extra-genital abnormalities such as renal or skeletal anomalies, hearing loss, or cardiac defects. The past two decades have witnessed significant advances both in understanding the etiologies of MRKH and in the development of fertility treatments such as uterine transplantation. The present work aimed to determine the rate of women with MRKH syndrome who underwent optimal initial management (after comprehensive malformation assessment) and to establish the rate of patients eligible for uterine transplantation (i.e., those with a vaginal length ≥7 cm without reconstruction using a bowel segment, and an anti-Müllerian hormone level >1.5 ng/mL before 35 years).

Material and methods: Cohort study of 85 women with MRKH syndrome consulting in our tertiary center.

Results: 62.4% of women with MRKH syndrome had an exhaustive malformative evaluation according to the French guidelines (Protocole National de Diagnostic et de Soin [PNDS]), of which 76.5% had associated malformations (MRKH type II). Pedigree, when available, showed a family history of infertility or a urogenital tract spectrum anomaly in 60% of cases. Concerning the uterine transplantation selection criteria, when evaluated, 22.6% of women had an anti-Müllerian hormone level <1.5 ng/mL and 36% a vaginal length <7 cm. On the 21 women with complete evaluation of both primary and secondary outcomes, 14 of them would be eligible for a uterine transplantation program at the time of consultation according to the main inclusion criteria of uterine transplantation program.

Conclusions: Women with MRKH syndrome are often inadequately explored for associated malformations. Early assessment and monitoring of the ovarian reserve is key for fertility preservation, especially in the era of uterine transplantation.

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优化 MRKH 患者的护理：从畸形筛查到子宫移植资格。

简介Mayer-Rokitansky-Küster-Hauser（MRKH）综合征伴子宫阴道再生不良是穆勒氏管异常中最严重的一种，可伴有肾脏或骨骼异常、听力损失或心脏缺陷等先天性以外的异常。过去二十年来，人们在了解MRKH的病因和开发子宫移植等生育治疗方法方面都取得了重大进展。本研究旨在确定MRKH综合征女性患者中接受最佳初始治疗（经过全面畸形评估）的比例，并确定符合子宫移植条件的患者比例（即阴道长度≥7厘米且未使用肠段进行重建，35岁前抗穆勒氏管激素水平>1.5纳克/毫升）：对在我们的三级中心就诊的85名MRKH综合征女性进行队列研究：62.4%的MRKH综合征女性根据法国指南（Protocole National de Diagnostic et de Soin [PNDS]）进行了详尽的畸形评估，其中76.5%伴有畸形（MRKH II型）。在有血缘关系的情况下，60%的病例有不孕或泌尿生殖道谱系异常的家族史。关于子宫移植的选择标准，经评估，22.6%的妇女有抗穆勒氏管激素水平结论：患有 MRKH 综合征的妇女往往没有充分检查相关畸形。早期评估和监测卵巢储备是保留生育能力的关键，尤其是在子宫移植时代。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Acta Obstetricia et Gynecologica Scandinavica 医学-妇产科学

CiteScore

8.00

自引率

4.70%

发文量

180

审稿时长

3-6 weeks

期刊介绍： Published monthly, Acta Obstetricia et Gynecologica Scandinavica is an international journal dedicated to providing the very latest information on the results of both clinical, basic and translational research work related to all aspects of women’s health from around the globe. The journal regularly publishes commentaries, reviews, and original articles on a wide variety of topics including: gynecology, pregnancy, birth, female urology, gynecologic oncology, fertility and reproductive biology.