Schwaab T, Bryand A
Service de chirurgie gynécologique, pôle de gynécologie-obstétrique, centre médico-chirurgical obstétrique (CMCO), CHRU de Strasbourg, 19, rue Louis-Pasteur, 67300 Schiltigheim, France.
Service de chirurgie gynécologique, pôle de gynécologie-obstétrique, centre médico-chirurgical obstétrique (CMCO), CHRU de Strasbourg, 19, rue Louis-Pasteur, 67300 Schiltigheim, France.
Gynecol Obstet Fertil Senol. 2019 Nov;47(11):783-789. doi: 10.1016/j.gofs.2019.07.002. Epub 2019 Jul 5.
Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is the most common cause of congenital absence or severe hypoplasia of structures derived from Muller's canals including the upper vagina, uterus and fallopian tubes. The definition of this syndrome is the presence of normal female secondary sexual development criteria relating to the presence of functional ovaries associated with vaginal agenesis and uterine anomalies ranging from a rudimentary uterus to the total absence of uterus. The main clinical sign of MRKH is primary amenorrhea. Confirmation of diagnosis and identification of associated abnormalities are based primarily on imaging and Magnetic Resonance Imaging (MRI) is currently the gold standard in the comprehensive evaluation of MRKH syndrome. Therefore, this study evaluated the place of ultrasound in the diagnostic and therapeutic management of patients treated for MRKH syndrome.
This retrospective, single-center, observational study collected all patients in charge of diagnosis or treatment of MRKH Syndrome between January 2000 and June 2017 within the University Hospital Gynecology and Obstetrics Department of Strasbourg. The analysis of the medical files allowed the evaluation of ultrasound in the different stages of the patient's care.
Twenty-one patients were included and 81% get an ultrasound, 38% of them had a referred ultrasound performed by a certified radiologist. Forty-eight percent of the patients had an MRI and every ultrasound provided a correct diagnosis. Sixteen patients received therapeutic management and only 50% of patients had preoperative MRI.
The role of medical imaging is to define the extent of uterovaginal abnormalities for accurate diagnosis, describe any coexisting abnormalities, and provide a roadmap for surgical planning. The first-line examination is transabdominal ultrasound, a simple, non-invasive procedure. The use of MRI in our series did not bring any diagnostic surplus value. Despite the faster and easier access to MRI; ultrasound remains an indispensable tool in the diagnostic and therapeutic management of MRKH patients.
迈耶-罗基坦斯基-库斯特-豪泽综合征（MRKH）是先天性缺失或源自苗勒管的结构严重发育不全的最常见原因，这些结构包括上阴道、子宫和输卵管。该综合征的定义是存在与功能性卵巢相关的正常女性第二性征发育标准，同时伴有阴道闭锁以及子宫异常，从始基子宫到子宫完全缺失不等。MRKH的主要临床体征是原发性闭经。诊断的确立以及相关异常情况的识别主要基于影像学检查，而磁共振成像（MRI）目前是MRKH综合征综合评估的金标准。因此，本研究评估了超声在接受MRKH综合征治疗患者的诊断和治疗管理中的作用。
这项回顾性、单中心观察性研究收集了2000年1月至2017年6月期间在斯特拉斯堡大学医院妇产科负责诊断或治疗MRKH综合征的所有患者。通过分析病历评估超声在患者治疗不同阶段的作用。
纳入21例患者，81%接受了超声检查，其中38%由认证放射科医生进行了转诊超声检查。48%的患者进行了MRI检查，每次超声检查均做出了正确诊断。16例患者接受了治疗管理，只有50%的患者术前进行了MRI检查。
医学成像的作用是确定子宫阴道异常的程度以进行准确诊断，描述任何并存的异常情况，并为手术规划提供路线图。一线检查是经腹超声，这是一种简单、无创的检查方法。在我们的研究系列中，MRI的使用并未带来任何额外的诊断价值。尽管MRI获取更快、更便捷，但超声仍然是MRKH患者诊断和治疗管理中不可或缺的工具。
