延伸至右侧心腔的静脉内平滑肌瘤病的个性化外科治疗  

Individualized surgical treatment for intravenous leiomyomatosis with extension to the right heart

作　　者：梁湄 苗齐 刘兴荣 马国涛 邵江 成宁海[3] 杜顺达 刘剑州 李晓凤 张超纪 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院心脏外科,北京市100730 [2]中国医学科学院北京协和医学院北京协和医院血管外科,北京市100730 [3]中国医学科学院北京协和医学院北京协和医院妇产科,北京市100730 [4]中国医学科学院北京协和医学院北京协和医院肝脏外科,北京市100730

出　　处：《中国心血管病研究》2017年第10期919-923,共5页Chinese Journal of Cardiovascular Research

摘　　要：目的探讨延伸至右心的静脉内平滑肌瘤病的外科治疗方法、安全性和疗效。本研究旨在报告北京协和医院单中心外科治疗结果。方法回顾2002年11月至2017年3月间在北京协和医院接受延伸至右心的静脉内平滑肌瘤病切除术的41例患者的临床资料。分析临床表现、手术技术、手术效果和随访资料，并将结果与现有文献进行比较。结果41例患者均行延伸至右心的静脉内平滑肌瘤病切除术，平均年龄25-55（44．6±6．2）岁。该病的临床表现多样，包括22例（53．7％）患者呼吸困难、胸闷气短，13例（31．7％）患者下肢水肿，4例（9．8％）患者发生晕厥，4例（9．8％）患者有浆膜腔积液，32例患者（78％）盆腔肿物，4例（9．8％）患者肺栓塞，3例（7．3％）患者肺转移；10例（24．4％）患者无症状。所有患者均有子宫平滑肌瘤病史，其中子宫切除术30例（73．2％）、子宫肌瘤切除术2例。15例（36．6％）肿瘤起自髂静脉，19例（46．3％）肿瘤起自卵巢静脉，6例（14．6％）起自髂静脉与卵巢静脉。在41例病例中，35例（85％）在体外循环下进行肿瘤切除，32例（78％）在深度低温停循环下进行手术。17例（41．5％）进行分期手术切除，24例（58．5％）进行一期手术切除。术式包括单纯经腹手术6例（14．6％）、经胸部切开手术1例（0．3％）、胸腹切口并肝下腔静脉切开20例（48．8％）、胸腹并肝后腔静脉切开术14例（34．1％）。术后石蜡病理诊断证实为静脉内平滑肌瘤病。9例患者有残留肿瘤，其中8例患者肿瘤未进展。术后随访9-172（52．4-36．1）个月，未出现死亡事件。结论我们针对延伸至右侧心腔的静脉内平滑肌瘤病提出具有个体化差异的外科手术治疗策略，为手术方案的选择提供参考。该策略可以为该病患者提供更安全和更有效的手术治疗，但仍需前�Objective Controversy exists regarding surgical treatment for intravenous leiomyomatosis with the right cardiac extension （IVLC）. This study aimed to report the results of management of the tumor at a single center. Methods We reviewed 41 patients with removal of IVLC from November 2002 to March 2017. Clinical features, surgical techniques, operative outcomes and available follow-up data were analyzed, and the results were compared with the available English literature. Statistical analyses were carried out by means of Fisher exact test and Student＇s t test. Results All 41 patients underwent removal of IVLC. The mean age was （44.6±6.2）years （range 25 to 55 years）. The clinical presentations were as follows： 22 （53.7%） cases of dyspnea, chest tightness and shortness of breath, 13（31.7%） of double lower limb edema, 4（9.8%） of syncope, 4（9.8%） of serous cavity effusion, 32（78%） of pelvic mass, 4（9.8%） of pulmonary embolism, 3 （7.3%） of pulmonary metastasis and 10 （24.4%） of lack of symptom. All the patients had history of uterine leiomyoma, 30（73.2% ） patients had previous hysterectomy/myomectomy and 2 patients had previous fibroid by cardiotomy. 15 （36.6%） cases had origin of iliac vein, 19（46.3%） cases origin of ovarian vein and 6（14.6%） cases both origin of both veins. Of the 41 cases with ICVL, 35（85.4%） cases underwent removal of IVCL under CPB, 32（78.0%） cases under DHCA. Staged proce- dures were performed in 14（41.5%） cases, one-stage procedure in 24（51%） cases. Downward extracting by only laparotomy were performed in 6（14.6%） patients, Upward extraction by only sternotomy in 1（0.3%） patients, removal of tumor by sternolaprotomy with infrahepatic in cavotomy 20 （48.8%） patients, and removal of tumor by sternolaprotomy with retrohepatic cavotomy in 14（34.1%） patients. All 41 patients survived with a smooth postoper- ative course. The postoperative histology confirmed IVCL. The residual tumors in 9 cases were no of

关 键 词：静脉内平滑肌瘤病 体外循环 外科治疗 

分 类 号：R654.2[医药卫生—外科学]