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胸腔镜下胸腺切除术治疗青少年重症肌无力

Thoracoscopic thymectomy for juvenile myasthenia gravis.

作者信息

Kim Aimee G, Upah Sydney A, Brandsema John F, Yum Sabrina W, Blinman Thane A

机构信息

Division of Pediatric General, Thoracic and Fetal Surgery, Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA, 19104, USA.

Division of Neurology, Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA, 19104, USA.

出版信息

Pediatr Surg Int. 2019 May;35(5):603-610. doi: 10.1007/s00383-019-04441-0. Epub 2019 Feb 7.

Abstract

PURPOSE

A randomized controlled trial of thymectomy in myasthenia gravis demonstrated improved clinical outcomes in adults, but data surrounding juvenile cases, especially those treated with minimally invasive approaches, are limited. Here, we review our experience with thoracoscopic thymectomy for juvenile myasthenia gravis (JMG) in the largest cohort to date.

METHODS

All cases of thymectomy for JMG in a single tertiary referral center between 2007 and 2018 were reviewed (N = 50). Patients underwent left thoracoscopic approach with extended dissection and without use of monopolar energy. Demographics, diagnostic criteria, and clinical classification, as well as surgical data were collected. Clinical status and medications were reviewed in follow-up.

RESULTS

The mean age at surgery was 10.5 ± 0.8 years. Ocular disease and generalized disease each comprised half of the cohort. No patients suffered complications or increased risk of morbidity or mortality with thymectomy. At any interval of follow-up through 3.5 years, 49.8% of patients were improved compared to their pre-operative presentation, and there was a significant trend towards decreased steroid use.

CONCLUSION

Thoracoscopic thymectomy is a safe treatment for juvenile myasthenia gravis in pediatric patients over a wide range of ages, body masses, and symptoms. Our experience adds evidence that pediatric patients likely benefit from thymectomy with improved clinical status and reduced medications.

摘要

目的

一项重症肌无力胸腺切除术的随机对照试验表明,该手术可改善成人患者的临床结局,但有关青少年病例的数据,尤其是采用微创方法治疗的病例,却很有限。在此,我们回顾了迄今为止最大队列中青少年重症肌无力(JMG)胸腔镜胸腺切除术的经验。

方法

回顾了2007年至2018年间在单一三级转诊中心进行的所有JMG胸腺切除术病例(N = 50)。患者接受左胸腔镜手术,进行广泛解剖,不使用单极能量。收集了人口统计学、诊断标准、临床分类以及手术数据。随访时复查临床状况和用药情况。

结果

手术平均年龄为10.5±0.8岁。眼肌型疾病和全身型疾病各占队列的一半。没有患者因胸腺切除术而出现并发症或发病率及死亡率增加的风险。在长达3.5年的任何随访期间,49.8%的患者与术前相比病情有所改善,且类固醇使用量有显著下降趋势。

结论

胸腔镜胸腺切除术对于不同年龄、体重和症状的儿童患者的青少年重症肌无力是一种安全的治疗方法。我们的经验进一步证明,儿童患者可能从胸腺切除术中获益,临床状况改善且用药减少。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b42/6456483/b8a1a515179b/383_2019_4441_Fig1_HTML.jpg

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