弥漫性子宫平滑肌瘤病影像学特征及临床诊治分析

Imaging features and clinical analysis of diffuse uterine leiomyomatosis cases

目的探讨弥漫性子宫平滑肌瘤病(DUL)影像学特征及临床诊治要点。方法回顾性分析2009年8月至2019年9月北京协和医院妇产科诊治的6例弥漫性子宫平滑肌瘤病患者影像学及临床资料,评估手术治疗及术后随诊情况,探讨DUL适宜诊治方案。结果6例患者初次诊断DUL年龄为(27±3)岁。所有患者主诉月经量增多,3例伴有中重度贫血,3例不孕。术前超声及磁共振成像(MRI)均提示子宫对称性增大,肌层弥漫分布大小不等肌瘤结节,瘤体间几乎无正常肌层组织。4例患者术前应用促性腺激素释放激素激动剂(GnRH-a)治疗。5例既往共行6次肌瘤剔除术,此次3例行开腹肌瘤剔除手术,剔除大小不等肌瘤188~300个,出血1200~2500 ml。2例分别于术后第51个月、第40个月复发,1例术后应用西罗莫司至今20个月暂无复发。3例行全子宫切除术,其中1例因盆腔粘连严重行部分小肠切除吻合术及部分大网膜切除术,术中出血2000 ml。结论盆腔影像检查尤其是MRI有助于DUL早期识别及术前评估。保留生育功能是DUL治疗难点,子宫肌瘤剔除术后复发风险高,全子宫切除是目前治愈DUL的最后方法。

Objective To discuss the imaging, clinical features and management of diffuse uterine leiomyomatosis (DUL).Methods Six cases of DUL confirmed in Peking Union Medical College Hospital from August 2009 to September 2019 were reviewed on their image and clinical data. Retrospective analysis was conducted on their perioperative and postoperative follow-up data.Results The average age of the first diagnosis of DUL was (27±3) years old. All of the patients complained menorrhagia and three patients suffered moderate to severe anemia. Three patients were diagnosed infertility. Pelvic ultrasound and MRI showed symmetrical enlarged uterus with complete replacement of the myometrium by innumerable, confluent leiomyomas.Four patients were treated with GnRH-a before operation to reduce the volume of myoma and correct anemia. Among the six patients, five had undergone myomectomy because of DUL before visiting Peking Union Medical College Hospital. Three patients underwent open myomectomy. The number of resected myoma was 188-300 and the bleeding volume was 1 200-2 500 ml. Two of them suffered recurrence at 51 and 40 months after operation. One received sirolimus for 20 months without recurrence until now. Other three patients underwent hysterectomy. One patient underwent partial small bowel resection and partial omentum resection because of severe pelvic adhesion during hysterectomy, and the blood loss was 2 000 ml.Conclusions Pelvic imaging especially MRI is helpful for early recognition and preoperative evaluation for DUL. Fertility preservation is a great challenge for DUL patients. The risk of recurrence after myomectomy is high. Hysterectomy is the last choice to completely cure DUL at present.