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剑突下全胸腺切除术治疗难治性非胸腺瘤型重症肌无力。

Subxiphoid completion thymectomy for refractory non-thymomatous myasthenia gravis.

作者信息

Ambrogi Vincenzo, Tacconi Federico, Sellitri Francesco, Tamburrini Alessandro, Perroni Gianluca, Carlea Federica, La Rocca Eleonora, Vanni Gianluca, Schillaci Orazio, Mineo Tommaso Claudio

机构信息

Department of Surgery, Division of Thoracic Surgery, Myasthenia Gravis Unit, Policlinico Tor Vergata University, Rome, Italy.

Division of Thoracic Surgery, Southampton General Hospital, Southampton, UK.

出版信息

J Thorac Dis. 2020 May;12(5):2388-2394. doi: 10.21037/jtd.2020.03.81.

Abstract

BACKGROUND

Completion thymectomy may be performed in patients with non-thymomatous refractory myasthenia gravis (MG) to allow a complete and definitive clearance from residual thymic tissue located in the mediastinum or in lower neck. Hereby we present our short- and long-term results of completion thymectomy using subxiphoid video-assisted thoracoscopy.

METHODS

Between July 2010 and December 2017, 15 consecutive patients with refractory non-thymomatous myasthenia, 8 women and 7 men with a median age of 44 [interquartile range (IQR) 38.5-53.5] years, underwent video-thoracoscopic completion thymectomy through a subxiphoid approach.

RESULTS

Positron emission tomography (PET) showed mildly avid areas [standardized uptake value (SUV) more than or equal to 1.8] in 11 instances. Median operative time was 106 (IQR, 77-141) minutes. No operative deaths nor major morbidity occurred. Mean 1-day postoperative Visual Analogue Scale value was 2.53±0.63. Median hospital stay was 2 (IQR, 1-3.5) days. A significant decrease of the anti-acetylcholine receptor antibodies was observed after 1 month [median percentage changes -67% (IQR, -39% to -83%)]. Median follow-up was 45 (IQR, 21-58) months. At the most recent follow-up complete stable remission was achieved in 5 patients. Another 9 patients had significant improvement in bulbar and limb function, requiring lower doses of corticosteroids and anticholinesterase drugs. Only one patient remained clinically stable albeit drug doses were reduced. One-month postoperative drop of anti-acetylcholine receptor antibodies was significantly correlated with complete stable remission (P=0.002).

CONCLUSIONS

This initial experience confirms that removal of ectopic and residual thymus through a subxiphoid approach can reduce anti-acetylcholine receptor antibody titer correlating to good outcome of refractory MG.

摘要

背景

对于非胸腺瘤性难治性重症肌无力(MG)患者,可进行胸腺切除术,以彻底清除位于纵隔或下颈部的残留胸腺组织。在此,我们展示了经剑突下电视辅助胸腔镜进行胸腺切除术的短期和长期结果。

方法

2010年7月至2017年12月期间,15例连续的难治性非胸腺瘤性重症肌无力患者,8例女性和7例男性,中位年龄44岁[四分位间距(IQR)38.5 - 53.5岁],通过剑突下入路接受了电视胸腔镜下胸腺切除术。

结果

正电子发射断层扫描(PET)显示11例中有轻度摄取增高区域[标准化摄取值(SUV)大于或等于1.8]。中位手术时间为106(IQR,77 - 141)分钟。未发生手术死亡或严重并发症。术后1天视觉模拟评分平均值为2.53±0.63。中位住院时间为2(IQR,1 - 3.5)天。1个月后观察到抗乙酰胆碱受体抗体显著下降[中位百分比变化 - 67%(IQR, - 39%至 - 83%)]。中位随访时间为45(IQR,21 - 58)个月。在最近一次随访时,5例患者实现了完全稳定缓解。另外9例患者的延髓和肢体功能有显著改善,需要更低剂量的皮质类固醇和抗胆碱酯酶药物。只有1例患者尽管药物剂量减少但仍保持临床稳定。术后1个月抗乙酰胆碱受体抗体下降与完全稳定缓解显著相关(P = 0.002)。

结论

这一初步经验证实,通过剑突下入路切除异位和残留胸腺可降低抗乙酰胆碱受体抗体滴度,这与难治性MG的良好预后相关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d1db/7330301/d406254b35dc/jtd-12-05-2388-f1.jpg

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