Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Obstetrical and Gynecological Diseases, Beijing, China.
Biomed Res Int. 2020 Nov 24;2020:6808409. doi: 10.1155/2020/6808409. eCollection 2020.
To elucidate the characteristics of symptomatic attack of rudimentary uteri in MRKH syndrome and highlight the rare and unexpected possibilities.
A cohort of 202 Chinese MRKH syndrome patients admitted to the Peking Union Medical College Hospital from Jan 2009 to Dec 2016 was analyzed retrospectively. Based on the symptoms of abdominal pain before vaginoplasty, the patients were categorized into the asymptomatic and symptomatic groups.
Totally, 21 patients had their uteri removed due to obstructive bleeding, 19 of them had symptoms of abdominal pain before vaginoplasty, the mean duration of abdominal pain before artificial vaginoplasty was 5.0 years (range, 0.5-10 years), and the mean age at first onset of recurrent abdominal pain was 14.3 years old (range 11-18). Two special cases showed unusual long incubation periods up to 23 years. Ultrasound detected endometrioid echo in four asymptomatic patients. Among the symptomatic group, 7 patients had no imaging evidence for endometrial cavities despite clinical pain. Two of them developed severe symptoms over the next two or four years and eventually had their uteri removed. Two patients reported persistent abdominal pain with a visual analog scale (VAS) score of 4-5, still under observation. Three patients were lost to follow-up.
More than 10% of the patients with MRKH syndrome had surgical indication to remove the rudimentary uteri. The discrepancy between clinical symptoms and imaging calls for the vigilance for prophylactic surgery or prolonged follow-up.
阐明 MRKH 综合征中残角子宫症状性发作的特征，并强调罕见且意外的可能性。
回顾性分析 2009 年 1 月至 2016 年 12 月期间在北京协和医院就诊的 202 例中国 MRKH 综合征患者。根据阴道成形术前腹痛症状，将患者分为无症状组和有症状组。
共有 21 例患者因梗阻性出血切除子宫，其中 19 例患者在阴道成形术前有腹痛症状，人工阴道成形术前腹痛的平均持续时间为 5.0 年（0.5-10 年），首次反复发作腹痛的平均年龄为 14.3 岁（11-18 岁）。有 2 例特殊病例表现出异常长的潜伏期，长达 23 年。4 例无症状患者的超声检查显示有子宫内膜回声。在有症状组中，7 例患者尽管有临床疼痛，但影像学检查未见子宫内膜腔的证据。其中 2 例在接下来的 2 或 4 年内出现严重症状，最终切除了子宫。2 例患者报告持续腹痛，视觉模拟评分（VAS）为 4-5，仍在观察中。3 例患者失访。
超过 10%的 MRKH 综合征患者有手术切除残角子宫的指征。临床症状与影像学表现之间的差异需要警惕预防性手术或延长随访。
