From the Departments of Imaging (M.A.H.C., S.H.P., A.P.K.) and Women's Health (C.E.W., S.M.C.), University College London Hospital, 235 Euston Rd, London NW1 2BU, England.
Radiology. 2013 Dec;269(3):787-92. doi: 10.1148/radiol.13130211. Epub 2013 Oct 28.
To evaluate the diverse magnetic resonance (MR) imaging findings of the pelvis in women with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome.
This retrospective review had institutional review board approval with waiver of informed consent. Between 2001 and 2011, 215 female patients with MRKH syndrome attended clinics, and 66 underwent pelvic MR imaging (age range, 14-40 years; median age, 19 years). One reviewer reviewed MR images for presence, site, volumes, and differentiation into layers (myometrium, junctional zone, and endometrium) of uterine remnants. Ovarian volumes and positions were assessed. Vaginal length was measured.
Rudimentary uteri were found in 61 patients (92%); 54 were bilateral, and seven were unilateral. All uterine buds were located laterally in the pelvis and had a constant caudal relationship with their paired ovary. Mean uterine volume was 6.4 mL (range, 0.4-80.2 mL), and 18 uteri had a volume greater than 10 mL. Twenty-four uterine buds (21%) showed differentiation into more than one layer. Two uteri contained intraluminal blood, and two showed signs of adenomyosis, indicating functioning endometrial tissue; these patients had cyclical pain. Bilateral ovaries were present in 54 patients; ovaries were ectopic in 27 patients. Twenty-two patients had no discernible vagina (dimple or less). Of the 44 patients with a vagina, the mean length was 2.0 cm (range, 1.0-6.5 cm).
Rudimentary uteri are common in patients with MRKH syndrome. They can be relatively large and have functioning endometrium, which can be associated with pain. Uteri have a constant caudal relationship to ovaries. Ovaries are commonly ectopic, and this must be recognized in patients undergoing fertility treatment. Online supplemental material is available for this article.
评估 Mayer-Rokitansky-Kuster-Hauser（MRKH）综合征女性患者的骨盆多种磁共振（MR）成像表现。
本回顾性研究经机构审查委员会批准豁免知情同意。2001 年至 2011 年间，215 例 MRKH 综合征女性患者就诊于诊所，其中 66 例行盆腔 MR 成像检查（年龄 14-40 岁，中位年龄 19 岁）。一位评估者评估了子宫残迹的存在、部位、体积和分层（子宫肌层、交界区和子宫内膜）分化。评估了卵巢体积和位置。测量了阴道长度。
61 例（92%）患者发现有原始子宫；54 例为双侧，7 例为单侧。所有子宫芽均位于骨盆外侧，与对侧卵巢具有恒定的尾侧关系。子宫平均体积为 6.4 毫升（范围，0.4-80.2 毫升），18 个子宫体积大于 10 毫升。24 个子宫芽（21%）显示出多于一层的分化。两个子宫内有腔内血液，两个显示出子宫腺肌病的迹象，表明存在功能性子宫内膜组织；这些患者有周期性疼痛。54 例患者双侧卵巢存在，27 例患者卵巢异位。22 例患者无明显阴道（凹痕或更小）。在 44 例有阴道的患者中，平均长度为 2.0 厘米（范围，1.0-6.5 厘米）。
原始子宫在 MRKH 综合征患者中很常见。它们可以相对较大，并具有功能性子宫内膜，这可能与疼痛有关。子宫与卵巢具有恒定的尾侧关系。卵巢常异位，这在接受生育治疗的患者中必须得到识别。本文提供了在线补充材料。
