Hasegawa Ayumi, Igarashi Hideki, Ohta Tsuyoshi, Kurachi Hirohisa, Takahashi Kazuhiro
Department of Obstetrics and Gynecology, Yamagata University Faculty of Medicine, Yamagata, and Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan.
Obstet Gynecol. 2015 Feb;125(2):393-396. doi: 10.1097/AOG.0000000000000646.
Mayer-Rokitansky-Küster-Hauser syndrome is a rare congenital anomaly characterized by congenital aplasia or hypoplasia of the uterus and vagina. We report a case of Mayer-Rokitansky-Küster-Hauser syndrome with multiple large pelvic masses diagnosed by three-dimensional computed tomography (CT) angiography.
A 40-year-old woman with Mayer-Rokitansky-Küster-Hauser syndrome presented with an abdominal mass that had grown for 3 months. Magnetic resonance imaging (MRI) confirmed several solid masses, and normal bilateral ovaries were detected; three-dimensional CT revealed that these tumors were supplied from the right ovarian and uterine arteries, suggesting that they arose from the uterus. Accordingly, leiomyoma was suspected. Laparoscopic surgery was contraindicated, and the patient therefore underwent laparotomy. The masses were resected with the bilateral rudimentary uteri and fallopian tubes, and pathologic evaluation confirmed leiomyoma.
Combined MRI and three-dimensional CT angiography can accurately evaluate the origin and anatomic properties of leiomyomas in patients with Mayer-Rokitansky-Küster-Hauser syndrome.
迈耶-罗基坦斯基-库斯特-豪泽综合征是一种罕见的先天性异常，其特征为子宫和阴道先天性发育不全或发育不良。我们报告一例通过三维计算机断层扫描（CT）血管造影诊断出的伴有多个盆腔大肿块的迈耶-罗基坦斯基-库斯特-豪泽综合征病例。
一名患有迈耶-罗基坦斯基-库斯特-豪泽综合征的40岁女性因腹部肿块就诊，该肿块已持续增大3个月。磁共振成像（MRI）证实有多个实性肿块，并检测到双侧卵巢正常；三维CT显示这些肿瘤由右侧卵巢动脉和子宫动脉供血，提示肿瘤起源于子宫。因此，怀疑为平滑肌瘤。因腹腔镜手术存在禁忌，故该患者接受了剖腹手术。将肿块与双侧残角子宫及输卵管一并切除，病理评估证实为平滑肌瘤。
MRI与三维CT血管造影相结合能够准确评估迈耶-罗基坦斯基-库斯特-豪泽综合征患者平滑肌瘤的起源及解剖特性。
