【摘要】 部分先天性子宫阴道缺如综合征（Mayer-Rokitansky-Kuster-Hauser syndrome,MRKH综合征）患者存在发育不良的子宫，其中无功能性内膜的始基子宫极少并发子宫腺肌病引发盆腔疼痛。报告1例MRKH综合征合并子宫腺肌病的病例，患者49岁时出现周期性腹痛合并盆腔实性肿物，腹腔镜手术探查考虑双侧始基子宫，左侧子宫腺肌病，行双侧始基子宫切除术，组织病理学检查明确诊断。术后患者顺利出院，随访腹痛缓解无复发。同时进行了文献综述，讨论MRKH综合征合并子宫腺肌病相关的诊断和治疗问题。更多还原

【Abstract】 Some patients with Mayer-Rokitansky-Kuster-Hauser(MRKH) syndrome have underdeveloped uterus.Among them, adenomyosis in a rudimentary uterus without functional endometrium rarely caused pelvic pain. We reported a case of MRKH syndrome complicated with adenomyosis. The 49-year-old patient presented with periodic abdominal pain and a pelvic solid mass. Laparoscopic exploration suggested bilateral rudimentary uterus with adenomyosis in the left uterus.Bilateral rudimentary hysterectomy was performed, and the diagnosis was confirmed by histopathological examination. The patient was discharged smoothly after the operation, and follow-up showed relief of abdominal pain without recurrence.Meanwhile, a literature review was conducted to discuss the diagnosis and treatment of MRKH syndrome complicated with adenomyosis.更多还原