Dataset Information

Subxiphoid completion thymectomy for refractory non-thymomatous myasthenia gravis.

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.

SUBMITTER: Ambrogi V

PROVIDER: S-EPMC7330301 | biostudies-literature | 2020 May

REPOSITORIES: biostudies-literature

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Publications

Subxiphoid completion thymectomy for refractory non-thymomatous myasthenia gravis.

Ambrogi Vincenzo V Tacconi Federico F Sellitri Francesco F Tamburrini Alessandro A Perroni Gianluca G Carlea Federica F La Rocca Eleonora E Vanni Gianluca G Schillaci Orazio O Mineo Tommaso Claudio TC

Journal of thoracic disease 20200501 5

<h4>Background</h4>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.<h4>Methods</h4>Between July 2010 and December 2017, 15 consecutive patients with refractory non-thymomatous myasthenia, 8 women and 7 m ...[more]

PMID: 32642144

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Project description:BackgroundThymectomy has been a mainstay in the treatment of myasthenia gravis, but there is no conclusive evidence of its benefit. We conducted a multicenter, randomized trial comparing thymectomy plus prednisone with prednisone alone.MethodsWe compared extended transsternal thymectomy plus alternate-day prednisone with alternate-day prednisone alone. Patients 18 to 65 years of age who had generalized nonthymomatous myasthenia gravis with a disease duration of less than 5 years were included if they had Myasthenia Gravis Foundation of America clinical class II to IV disease (on a scale from I to V, with higher classes indicating more severe disease) and elevated circulating concentrations of acetylcholine-receptor antibody. The primary outcomes were the time-weighted average Quantitative Myasthenia Gravis score (on a scale from 0 to 39, with higher scores indicating more severe disease) over a 3-year period, as assessed by means of blinded rating, and the time-weighted average required dose of prednisone over a 3-year period.ResultsA total of 126 patients underwent randomization between 2006 and 2012 at 36 sites. Patients who underwent thymectomy had a lower time-weighted average Quantitative Myasthenia Gravis score over a 3-year period than those who received prednisone alone (6.15 vs. 8.99, P<0.001); patients in the thymectomy group also had a lower average requirement for alternate-day prednisone (44 mg vs. 60 mg, P<0.001). Fewer patients in the thymectomy group than in the prednisone-only group required immunosuppression with azathioprine (17% vs. 48%, P<0.001) or were hospitalized for exacerbations (9% vs. 37%, P<0.001). The number of patients with treatment-associated complications did not differ significantly between groups (P=0.73), but patients in the thymectomy group had fewer treatment-associated symptoms related to immunosuppressive medications (P<0.001) and lower distress levels related to symptoms (P=0.003).ConclusionsThymectomy improved clinical outcomes over a 3-year period in patients with nonthymomatous myasthenia gravis. (Funded by the National Institute of Neurological Disorders and Stroke and others; MGTX ClinicalTrials.gov number, NCT00294658.).

Project description:BACKGROUND:The Thymectomy Trial in Non-Thymomatous Myasthenia Gravis Patients Receiving Prednisone (MGTX) showed that thymectomy combined with prednisone was superior to prednisone alone in improving clinical status as measured by the Quantitative Myasthenia Gravis (QMG) score in patients with generalised non-thymomatous myasthenia gravis at 3 years. We investigated the long-term effects of thymectomy up to 5 years on clinical status, medication requirements, and adverse events. METHODS:We did a rater-blinded 2-year extension study at 36 centres in 15 countries for all patients who completed the randomised controlled MGTX and were willing to participate. MGTX patients were aged 18 to 65 years at enrolment, had generalised non-thymomatous myasthenia gravis of less than 5 years' duration, had acetylcholine receptor antibody titres of 1·00 nmol/L or higher (or concentrations of 0·50-0·99 nmol/L if diagnosis was confirmed by positive edrophonium or abnormal repetitive nerve stimulation, or abnormal single fibre electromyography), had Myasthenia Gravis Foundation of America Clinical Classification Class II-IV disease, and were on optimal anticholinesterase therapy with or without oral corticosteroids. In MGTX, patients were randomly assigned (1:1) to either thymectomy plus prednisone or prednisone alone. All patients in both groups received oral prednisone at doses titrated up to 100 mg on alternate days until they achieved minimal manifestation status. The primary endpoints of the extension phase were the time-weighted means of the QMG score and alternate-day prednisone dose from month 0 to month 60. Analyses were by intention to treat. The trial is registered with ClinicalTrials.gov, number NCT00294658. It is closed to new participants, with follow-up completed. FINDINGS:Of the 111 patients who completed the 3-year MGTX, 68 (61%) entered the extension study between Sept 1, 2009, and Aug 26, 2015 (33 in the prednisone alone group and 35 in the prednisone plus thymectomy group). 50 (74%) patients completed the 60-month assessment, 24 in the prednisone alone group and 26 in the prednisone plus thymectomy group. At 5 years, patients in the thymectomy plus prednisone group had significantly lower time-weighted mean QMG scores (5·47 [SD 3·87] vs 9·34 [5·08]; p=0·0007) and mean alternate-day prednisone doses (24 mg [SD 21] vs 48 mg [29]; p=0·0002) than did those in the prednisone alone group. 14 (42%) of 33 patients in the prednisone group, and 12 (34%) of 35 in the thymectomy plus prednisone group, had at least one adverse event by month 60. No treatment-related deaths were reported during the extension phase. INTERPRETATION:At 5 years, thymectomy plus prednisone continues to confer benefits in patients with generalised non-thymomatous myasthenia gravis compared with prednisone alone. Although caution is appropriate when generalising our findings because of the small sample size of our study, they nevertheless provide further support for the benefits of thymectomy in patients with generalised non-thymomatous myasthenia gravis. FUNDING:National Institutes of Health, National Institute of Neurological Disorders and Stroke.

Project description:BackgroundApproaches of thoracoscopic thymectomy for myasthenia gravis (MG) are debatable. The subxiphoid approach is widely utilized recent years for its better visualization of the anterior mediastinum. In the present study, we compared perioperative outcomes and mid-term effects of the extended thymectomy for MG between the subxiphoid approach and the routine right-thoracic approach.MethodsOne hundred and thirty-one MG patients treated with thoracoscopic extended thymectomy were analyzed. Among them, 68 patients were operated on via the subxiphoid approach and the other 63 via the conventional right-side unilateral approach. The patient outcomes were retrospectively reviewed and evaluated. Mid-term clinical outcome was assessed according to the classification system proposed by the Myasthenia Gravis Foundation of America (MGFA). Clinical efficacy and variables influencing outcome were evaluated by the Kaplan-Meier method and Cox proportional hazards regression analysis.ResultsCompared with the right thoracic approach, the duration of the procedure via the subxiphoid approach was significantly shorter (P=0.035), the rates of total thymectomy were higher (P=0.028), and the pain scores on postoperative days 1, 3, and 7 were significantly lower (P<0.001, P<0.001, and P=0.03, respectively). A total of 112 patients with MG were followed up. The subxiphoid approach group reported higher rates of complete stable remission (CSR) and effective treatment of MG, although these differences were not statistically significant (Z=-0.484, P=0.627). By multivariate Cox proportional hazards modes analysis, the chance of CSR was significantly increased when age <40 (OR: 2.623, 95% CI: 1.150-5.983, P=0.022), non-thymomatous MG (OR: 1.078, 95% CI: 1.101-3.316, P=0.021) and MGFA clinical classification (OR: 2.024, 95%:1.164-3.523, P=0.013).ConclusionsThe subxiphoid approach has shorter operation time, higher rates of total thymectomy and better quality of life compared with the lateral thoracoscopic approach. Preoperative age, pathological diagnoses and MGFA Clinical Classification are the independent risk factors for non-complete stable remission (NCSR) after thymectomy.

Dataset Information

Subxiphoid completion thymectomy for refractory non-thymomatous myasthenia gravis.

Background

Methods

Results

Conclusions

Publications

Subxiphoid completion thymectomy for refractory non-thymomatous myasthenia gravis.

Similar Datasets

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