静脉内平滑肌瘤延伸至右侧心腔的诊断及治疗

Diagnosis and Surgical Treatment of Leiomyomatosis With Intravenous Extension Into the Right Heart

目的 :探讨静脉内平滑肌瘤延伸至右侧心腔的诊断与治疗。方法 :1991年 10月至 2 0 0 1年 5月我院收治静脉内平滑肌瘤延伸至右侧心腔患者 6例 ,第 1例患者术前拟诊为右心房粘液瘤 ,术中发现右心房肿物系下腔静脉肿物的延伸 ,仅切除了右心房内肿物 ,未进行二期手术。其余 5例术前应用超声心动图及腹部B超检查明确诊断 ,选择分期手术 ,一期胸腹联合切口 ,体外循环深低温暂停循环下切除心腔、下腔静脉及髂静脉肿瘤 ,3例二期切除子宫病变 ,行盆腔清扫术 ,2例未再接受妇科手术。结果 :第 1例患者术后 1年 6个月后死亡 ,其余 5例随访至今良好。结论 :对多发性子宫肌瘤或右心房肿物患者应警惕该病 ,结合超声心动图及腹部B超检查可确诊。一期胸腹联合切口切除右侧心腔、下腔静脉及髂静脉内肿瘤 ,二期切除子宫病变组织进行盆腔清扫术 ,可获满意的效果。

Objective: To review the experience in diagnosis and surgical treatment of leiomyomatosis with intravenous extension into the right heart. Methods:From October 1991 to May 2001, 6 patients with leiomyomatosis and intravenous extension into the right heart were treated in our hospital. The mean age was (33.2±5) years (range 31 to 42), 5 patients had a history of subtotal hysterectomy and one was admitted to our hospital because of uteruis myoma.Pre-operative diagnosis of the first case was right atrial myxoma.Surgery was performed by cardiopulmonary bypass. It was identified as myxoma associated with inferior vena cava myxoma during operation,and only right atrial myxoma was resected.Reoperation was not carried out. The other 5 cases were correctly diagnosed by echocardiography and brightness mode ultrasonagraphy on of abdominal cavity. They received the staged operation.At first stage tumors in right heart, inferior vena cava were resected through abdominal-thoracic incision. Three patients received hysterectomy at second stage.Two patients refused to receive operation. Results: No death at operation.The mean fellow-up was (34.1±8.6) months (range 18 months to 8 years).The first patient died one year and seven months after operation.The other 5 patients had an uneventful recovery and no recurrence. Conclusion: Echocardiography and brightness mode ultrasonagraphy on abdominal cavity are the main diagnostic method for intravenous leiomyomatosis extending into the right heart. Surgery should start without delay as long as the diagnosis is established.At first stage tumors in the right heart and inferior cave vein should be resected.At second stage hysterectomy should be carried out. The long term result is satisfactory.