From the Department of Obstetrics and Gynaecology University of the West Indies, and the Windward Medical Centre, Kingston, Jamaica.
Obstet Gynecol. 2012 Feb;119(2 Pt 2):483-485. doi: 10.1097/AOG.0b013e318242a9b5.
Müllerian remnant leiomyomas occur in women with Mayer-Rokitansky-Küster Hauser syndrome in which leiomyomas arise in absence of a uterus. This is a rare condition and we present two unusual cases.
In the first case, a woman with Mayer-Rokitansky-Küster-Hauser syndrome diagnosed was found to have a pelvic mass and was scheduled for laparotomy. A few days before the surgery, acute abdominal pain developed. At surgery she was found to have a twisted adnexum including a Müllerian remnant leiomyoma. This was untwisted successfully; the leiomyoma was excised and the ovary was saved. In the second case, one member of a pair of monozygotic twins was found to have leiomyomas at age 33 after having an absent uterus diagnosed at laparoscopy 8 years previously. She had successful removal of the leiomyoma at laparotomy. Her sister had normal development and had two children.
Mayer-Rokitansky-Küster-Hauser syndrome leading to Müllerian remnant leiomyomas is uncommon but should be suspected in women in whom a pelvic mass develops after the confirmation of the Müllerian defect.
Müllerian 残余性平滑肌瘤发生在 Mayer-Rokitansky-Küster Hauser 综合征的女性中，这些平滑肌瘤在没有子宫的情况下出现。这是一种罕见的情况，我们现在介绍两个不寻常的病例。
在第一个病例中，一名被诊断为 Mayer-Rokitansky-Küster-Hauser 综合征的女性被发现盆腔有肿块，计划进行剖腹手术。在手术前几天，她出现了急性腹痛。在手术中，她发现附件扭转，包括 Müllerian 残余性平滑肌瘤。该肌瘤成功解开，平滑肌瘤被切除，卵巢得以保留。在第二个病例中，一对单卵双胞胎中的一个成员在 8 年前腹腔镜检查确认子宫缺失后，在 33 岁时发现了平滑肌瘤。她成功地进行了剖腹手术切除了肌瘤。她的姐姐发育正常，生了两个孩子。
导致 Müllerian 残余性平滑肌瘤的 Mayer-Rokitansky-Küster-Hauser 综合征并不常见，但在确认 Müllerian 缺陷后出现盆腔肿块的女性中应怀疑存在这种情况。
