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1.
BMC Neurol ; 24(1): 188, 2024 Jun 05.
Article in English | MEDLINE | ID: mdl-38840065

ABSTRACT

BACKGROUND: Myasthenia gravis (MG) is a long-term autoimmune disorder that affects the neuromuscular junction, causing muscle weakness and fatigue as its primary clinical features. Vitamin D is crucial for both the autoimmune response and skeletal muscle function. CASE PRESENTATION: Here, we presented a case report documenting the substantial improvement in symptoms experienced by a patient who underwent subtotal gastrectomy for gastric cancer following high-dose Vitamin D supplementation. The patient developed generalized MG two months after the surgery and did not respond adequately to pyridostigmine therapy, experiencing a progressive deterioration of the condition. A significant reduction in vitamin D concentration was observed following subtotal gastrectomy. In response, high-dose vitamin D supplementation was administered to the patient. Within one week of treatment, swallowing symptoms improved, enabling the consumption of a small amount of liquid food. By the second week, substantial swallowing and neck function improvements were evident. After one month, the patient regained the ability to straighten the neck while walking and consumed a regular diet despite persistent difficulties chewing hard food. CONCLUSIONS: This case underscores the therapeutic potential of vitamin D in alleviating MG symptoms, particularly in individuals with compromised vitamin D levels following gastrectomy. The observed improvements present a new perspective on the possible involvement of vitamin D supplementation in the management of postoperative MG cases.


Subject(s)
Gastrectomy , Myasthenia Gravis , Vitamin D , Humans , Gastrectomy/adverse effects , Myasthenia Gravis/surgery , Myasthenia Gravis/drug therapy , Vitamin D/therapeutic use , Vitamin D/administration & dosage , Stomach Neoplasms/surgery , Male , Female , Aged , Middle Aged , Dietary Supplements
2.
Port J Card Thorac Vasc Surg ; 31(1): 23-28, 2024 May 13.
Article in English | MEDLINE | ID: mdl-38743523

ABSTRACT

INTRODUCTION: Myasthenia gravis (MG) is an autoimmune, neurologic disease that causes a wide range of symptoms. While the transsternal, transcervical and thoracotomy approaches are accepted as effective, there is still debate regarding the VATS approach. MATERIALS AND METHODS: We analyzed our center's surgical experience with thymectomy for myasthenia gravis, comparing the results of patients operated on using VATS and more invasive approaches, over a period of 10 years. A search of the department's surgical database for myasthenia gravis cases between January 2010 and January 2021, revealed a total of 40 cases. Twenty-four patients were included in the final analysis and were distributed into two groups: the VATS procedure group (group A) and the open procedure group (group B). The latter included sternotomy, thoracotomy, transcervical and hemiclamshell approaches. Only radical thymectomies were included. The established outcomes were clinical improvement defined as asymptomatic remission, reduction, or discontinuation of the medication necessary to achieve optimal symptom control. RESULTS: The median follow-up time was 27 months (ranging from 4 to 75 months). Videothoracoscopy radical thymectomy was performed on 12 patients. Complete remission with no medication was achieved in 1 case (8.3%), while 2 patients (16.7%) became asymptomatic with reduced medication. An improvement (reduced symptoms or decreased medication) was observed in 8 cases (66.6%). No change in clinical outcome was noted in 1 patient (8.3%). None of the patients reported worsening symptoms. Open thymectomy was performed on 12 patients. Complete remission with no medication was achieved in 1 case (8.3%), while 2 patients (16.7%) became asymptomatic with reduced medication. An improvement was noted in 6 cases (50%). No change in clinical outcome was observed in 3 patients (25%) whereas 2 of them (16.7%) experienced slightly better symptom control but with a significant increase in medication. One patient (8.3%) described the clinical results as without any significant change. None of the patients reported worsening symptoms. CONCLUSION: The videotoracoscopic approach in the treatment of myasthenia gravis is non-inferior compared to the open approach and effective in a long-term follow-up, offering all the additional benefits of less invasive surgery.


Subject(s)
Myasthenia Gravis , Thoracic Surgery, Video-Assisted , Thymectomy , Humans , Myasthenia Gravis/surgery , Thymectomy/methods , Thoracic Surgery, Video-Assisted/methods , Thoracic Surgery, Video-Assisted/adverse effects , Male , Female , Middle Aged , Adult , Retrospective Studies , Treatment Outcome , Aged , Thoracotomy/methods , Thoracotomy/adverse effects , Young Adult
3.
Rozhl Chir ; 103(2): 40-47, 2024.
Article in English | MEDLINE | ID: mdl-38697812

ABSTRACT

Thoracic surgery is increasingly influenced by the development of minimally invasive approaches which have also influenced surgery in the area of the anterior mediastinum. The previously standard approach to the thymus via partial sternotomy was gradually replaced by the videothoracoscopic approach in most cases. In recent years, robotically assisted surgery has been gaining ground worldwide in this area, as well. The aim of our paper is to provide a comprehensive overview of procedures in the field of the thymus, including their indications, and to share our first experience with robot-assisted thymus surgery. At the 3rd Department of Surgery, since the start of the robot-assisted thymus surgery program, 23 thymectomies have been performed using this approach, of which 17 were performed for thymoma, 3 for myasthenia gravis, and 3 for parathyroid adenoma localized in thymus tissue. From our experience and the available data, it follows that the length of hospitalization, the rate of complications and the resulting effect of robot-assisted procedures is comparable to VTS procedures; however, the robot-assisted surgery also allows for mini-invasive treatment even in significantly obese patients and in patients with advanced thymic tumors who would otherwise be indicated for open thymectomy.


Subject(s)
Myasthenia Gravis , Robotic Surgical Procedures , Thymectomy , Thymoma , Thymus Neoplasms , Humans , Robotic Surgical Procedures/methods , Thymectomy/methods , Thymus Neoplasms/surgery , Thymoma/surgery , Myasthenia Gravis/surgery , Parathyroid Neoplasms/surgery , Thymus Gland/surgery , Male
4.
Neurology ; 102(12): e209482, 2024 Jun 25.
Article in English | MEDLINE | ID: mdl-38781559

ABSTRACT

The role of immunosenescence, particularly the natural process of thymic involution during aging, is increasingly acknowledged as a factor contributing to the development of autoimmune diseases and cancer. Recently, a concern has been raised about deleterious consequences of the surgical removal of thymic tissue, including for patients who undergo thymectomy for myasthenia gravis (MG) or resection of a thymoma. This review adopts a multidisciplinary approach to scrutinize the evidence concerning the long-term risks of cancer and autoimmunity postthymectomy. We conclude that for patients with acetylcholine receptor antibody-positive MG and those diagnosed with thymoma, the removal of the thymus offers prominent benefits that well outweigh the potential risks. However, incidental removal of thymic tissue during other thoracic surgeries should be minimized whenever feasible.


Subject(s)
Myasthenia Gravis , Thymectomy , Thymoma , Thymus Gland , Thymus Neoplasms , Humans , Thymectomy/adverse effects , Thymectomy/methods , Myasthenia Gravis/surgery , Thymus Gland/surgery , Thymus Neoplasms/surgery , Thymus Neoplasms/complications , Thymoma/surgery , Thymoma/complications , Postoperative Complications/etiology , Autoimmune Diseases/surgery
6.
J Clin Neurosci ; 124: 20-26, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38640804

ABSTRACT

OBJECTIVE: To investigate the relationship between short-term changes in quantitative myasthenia gravis score (QMGS) after thymectomy and postoperative recurrence in myasthenia gravis (MG) patients without thymoma. METHODS: A retrospective observational cohort study. The QMGS of 44 patients with non-thymomatous MG were evaluated before and 1 month after thymectomy, and the frequency and time of postoperative recurrence were recorded. The reduction rate of QMGS (rr-QMGS) was defined as (QMGS one week before thymectomy - QMGS one month after thymectomy)/ QMGS one week before thymectomy × 100 %, as an indicator of short-term symptom change after thymectomy. The receiver operating characteristic (ROC) curve was established to determine an appropriate cut-off value of rr-QMGS for distinguishing postoperative recurrence. Multivariate Cox regression analysis was applied to predict postoperative recurrence. RESULTS: Postoperative recurrence occurred in 21 patients (30 times in total) during follow-up. The mean annual recurrence rate was 3.98 times/year preoperatively and 0.30 times/year postoperatively. ROC analysis determined the cut-off value of rr-QMGS was 36.7 % (sensitivity 90.5 %, specificity 52.2 %). Multivariate Cox regression analysis showed that rr-QMGS<36.7 % (hazard rate[HR]6.251, P = 0.014) is positive predictor of postoperative recurrence. Kaplan-Meier analysis showed that postoperative recurrence time was earlier in the low rr-QMGS group than in the high rr-QMGS group (12.62 vs. 36.60 months, p = 0.005). CONCLUSIONS: Low rr-QMGS is associated with early postoperative recurrence. Rr-QMGS can be used to predict postoperative recurrence of non-thymomatous MG.


Subject(s)
Myasthenia Gravis , Recurrence , Thymectomy , Humans , Myasthenia Gravis/surgery , Myasthenia Gravis/diagnosis , Thymectomy/adverse effects , Thymectomy/methods , Male , Female , Middle Aged , Retrospective Studies , Adult , Aged , Young Adult , ROC Curve , Follow-Up Studies
7.
Eur J Surg Oncol ; 50(6): 108349, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38640605

ABSTRACT

BACKGROUD: The standard resection for early-stage thymoma is total thymectomy and complete tumour excision with or without myasthenia gravis but the optimal surgery mode for patients with early-stage non-myasthenic thymoma is debatable. This study analysed the oncological outcomes for non-myasthenic patients with early-stage thymoma treated by thymectomy or limited resection in the long term. METHODS: Patients who had resections of thymic neoplasms at Taipei Veteran General Hospital, Taiwan between December 1997 and March 2013 were recruited, exclusive of those combined clinical evidence of myasthenia gravis were reviewed. A total of 113 patients were retrospectively reviewed with pathologic early stage (Masaoka stage I and II) thymoma who underwent limited resection or extended thymectomy to compare their long-term oncologic and surgical outcomes. RESULTS: The median observation time was 134.1 months [interquartile range (IQR) 90.7-176.1 months]. In our cohort, 52 patients underwent extended thymectomy and 61 patients underwent limited resection. Shorter duration of surgery (p < 0.001) and length of stay (p = 0.006) were demonstrated in limited resection group. Six patients experienced thymoma recurrence, two of which had combined myasthenia gravis development after recurrence. There was no significant difference (p = 0.851) in freedom-from-recurrence, with similar 10-year freedom-from-recurrence rates between the limited resection group (96.2 %) and the thymectomy group (93.2 %). Tumour-related survival was also not significantly different between groups (p = 0.726).result CONCLUSION: Patients with early-stage non-myasthenic thymoma who underwent limited resection without complete excision of the thymus achieved similar oncologic outcomes during the long-term follow-up and better peri-operative results compared to those who underwent thymectomy.


Subject(s)
Neoplasm Staging , Thymectomy , Thymoma , Thymus Neoplasms , Humans , Thymectomy/methods , Thymoma/surgery , Thymoma/pathology , Thymoma/complications , Male , Thymus Neoplasms/surgery , Thymus Neoplasms/pathology , Thymus Neoplasms/complications , Female , Middle Aged , Follow-Up Studies , Retrospective Studies , Adult , Aged , Myasthenia Gravis/surgery , Survival Rate , Neoplasm Recurrence, Local , Operative Time , Length of Stay , Taiwan/epidemiology , Treatment Outcome
8.
Port J Card Thorac Vasc Surg ; 30(4): 31-38, 2024 Feb 09.
Article in English | MEDLINE | ID: mdl-38345885

ABSTRACT

INTRODUCTION: Thymectomy remains a mainstay of treatment in Thymomatous (T) and Nonthymomatous (nT) Myasthenia Gravis (MG), with improved clinical outcomes and reduced need for medical treatment, however, there is little research regarding long-term follow-up. We aim to assess the impact of surgery on the long-term outcome of patients with MG at our center. METHODS: Retrospective analyses of MG patients submitted to thymectomy between 2007 and 2017 at the thoracic surgery department of CHUC. Clinical assessment was performed according to the MG Foundation of America (MGFA) Clinical Classification (cMGFA). The follow-up was categorized according to the MGFA Post-intervention Status (MGFA-PIS) and cMGFA. Statistical analysis was performed with SPSS, to a significance level of 5%. RESULTS: Thirty-seven patients underwent extended thymectomy and 67.6% were female. Median age at diagnosis was 46.68±19.2 years. Most patients (83.8%) had anti-acetylcholine receptor antibodies and 81.1% had generalized forms of MG. Many patients (67.6%) had surgery less than 12 months after the clinical diagnosis. TMG was present in 19 (51.4%) patients. Compared to nTMG, these patients were older (54.06±17.9 vs 40.17±19.4 years) and most were men (52.9% vs 16.7%). We obtained a good outcome in most patients in the first (81.1%), second (86.1%), and fifth (84.8%) year of follow-up. There was a shift towards better prognosis categories in the good outcome group: 9.1% CSR, 3.0% PR, and 66,7% MM in the fifth year. Preoperative medical treatment did not influence the long-term follow-up outcome. A shorter time to surgery (< 12 months) correlated with better outcomes at year 5 (p=0.016). CONCLUSION: Thymectomy led to a sustained clinical improvement in our cohort, allowing for a reduced need for medication. A shorter time to surgery seems to have a positive influence on long-term prognosis. We expect that an extended follow-up would improve our results.


Subject(s)
Myasthenia Gravis , Thymectomy , Male , Humans , Female , Adult , Middle Aged , Aged , Thymectomy/methods , Treatment Outcome , Retrospective Studies , Myasthenia Gravis/surgery , Prognosis
9.
J Cardiothorac Surg ; 19(1): 37, 2024 Jan 31.
Article in English | MEDLINE | ID: mdl-38297367

ABSTRACT

BACKGROUND: Thymoma and myasthenia gravis (MG) interact with each other. This study aimed to evaluate the effects of thymoma on neurological outcome of MG patients after thymectomy using the propensity score matching (PSM) method. METHODS: Consecutive patients with MG who underwent thymectomy at Beijing Hospital between January 2012 and August 2021 were retrospectively enrolled. Clinical and follow-up data were collected. Statistical analysis was performed using SPSS 23.0 software. PSM was performed to eliminate selection bias. RESULTS: A total of 456 patients were included in this study. Thymoma was present in 138 (30.3%) patients. The median follow-up time was 72 (range, 12-135) months. At the last follow-up, a lower proportion of thymomatous MG patients achieved complete stable remission (CSR) compared with non-thymomatous MG patients (P = 0.011), and the effective rate [CSR + pharmatologic remission (PR) + minimal manifestations (MM)] of thymomatous MG patients was also lower (P = 0.037). Considering time to CSR, Kaplan-Meier analysis showed thymomatous MG patients had lower cumulative CSR rate than non-thymomatous MG patients (log-rank, P = 0.019). After PSM, 105 pairs of patients were matched successfully. For the matched patients, thymomatous MG patients had a lower CSR rate and a lower effective rate (P = 0.002, 0.039, respectively), and K-M analysis still showed thymomatous MG patients had lower cumulative CSR rate (log-rank, P = 0.048). Multivariate Cox analysis demonstrated that thymoma (HR: 0.592, 95% CI 0.389-0.900, P = 0.014), older age at the time of surgery (HR: 0.971, 95% CI 0.953-0.990, P = 0.003), and preoperative course of MG > 12 months (HR: 0.474, 95% CI 0.317-0.708, P = 0.000) were negative predictive factors for CSR. CONCLUSIONS: Thymoma had a negative effect on the neurological outcome of MG after thymectomy. MG patients with old age and a preoperative course of longer than one year had a lower probability of achieving CSR.


Subject(s)
Myasthenia Gravis , Thymoma , Thymus Neoplasms , Humans , Thymoma/complications , Thymoma/surgery , Thymectomy/methods , Propensity Score , Retrospective Studies , Thymus Neoplasms/complications , Thymus Neoplasms/surgery , Myasthenia Gravis/complications , Myasthenia Gravis/surgery , Pathologic Complete Response , Treatment Outcome
10.
Tohoku J Exp Med ; 262(1): 29-31, 2024 Jan 23.
Article in English | MEDLINE | ID: mdl-37967941

ABSTRACT

Juvenile myasthenia gravis (JMG) exhibits a more favorable response to glucocorticoids and has a better prognosis than adult myasthenia gravis. However, no established treatment exists for refractory JMG. Although thymectomy has been performed in several patients with refractory systemic JMG, there are few detailed clinical descriptions of patients who underwent thymectomy. Here, we present the case of a 10-year-old boy with refractory systemic JMG who was successfully treated with thymectomy. The patient developed symptoms, including dysphagia, malaise, diurnal ptosis, and weakness in the trunk muscles, and he was diagnosed with generalized JMG. Despite undergoing various treatments, including steroids, tacrolimus, steroid pulse therapy, intravenous immunoglobulin, azathioprine (AZT), and rituximab, his symptoms did not improve. Therefore, he underwent a thoracoscopic thymectomy 24 months after disease onset. Thymectomy led to remission, as demonstrated by a significant reduction in the quantitative myasthenia gravis score and anti-acetylcholine receptor antibody levels, which persisted for 43 months after surgery. Our case demonstrates the effectiveness of thymectomy in systemic JMG patients with positive anti-acetylcholine receptor antibodies, despite therapeutic failure with AZT and rituximab, within 2 years of disease onset.


Subject(s)
Myasthenia Gravis , Thymectomy , Child , Humans , Male , Autoantibodies , Disease Progression , Glucocorticoids/therapeutic use , Myasthenia Gravis/drug therapy , Myasthenia Gravis/surgery , Rituximab , Treatment Outcome
11.
BMC Anesthesiol ; 23(1): 349, 2023 10 21.
Article in English | MEDLINE | ID: mdl-37865733

ABSTRACT

BACKGROUND: Myasthenia gravis (MG) patients interact unpredictably with anesthetic agents, including neuromuscular blocking agents. Here, we investigate the effectiveness of general anesthesia without muscle relaxants using either propofol via target-controlled infusion systems (TCI) or sevoflurane in MG patients undergoing thoracoscopic thymectomy. METHODS: This prospective, open-label, observational study was conducted in a university hospital. We included 90 myasthenic patients undergoing thoracoscopic thymectomy with general anesthesia. Patients received induction and maintenance anesthesia with propofol TCI (group P, n = 45) or induction with propofol 2-3 mg.kg-1 and maintenance anesthesia with sevoflurane (group S, n = 45). In both groups, the procedure was performed under the guidance of entropy with sufentanil but not a muscle relaxant. Intubation conditions, hemodynamic changes, respiratory function, neuromuscular transmission, arterial blood gas, and complications were evaluated. RESULTS: All patients achieved good intubation conditions. Hemodynamic instability was more frequent in group S than in group P, mostly in the induction stage, and was controllable. The reduction in the intraoperative train-of-four ratio from baseline at 30 min, 60 min, and 90 min in group S was 10.3%, 14.2%, and 14.3%, respectively, significantly higher than that in group P (6.8%, 7.2%, and 8.4%, respectively), which completely recovered at the end of the surgery. All patients were extubated in the operating room without complications. No other significant differences between the groups were observed. CONCLUSIONS: Anesthesia with propofol TCI or sevoflurane without muscle relaxants in MG patients offered safe and effective conditions for thoracoscopic thymectomy. Sevoflurane achieved higher levels of intraoperative muscular relaxation than propofol TCI. Postoperative neuromuscular function was not affected by these anesthetics.


Subject(s)
Methyl Ethers , Myasthenia Gravis , Propofol , Humans , Propofol/pharmacology , Sevoflurane , Thymectomy/methods , Prospective Studies , Myasthenia Gravis/surgery , Anesthesia, General , Muscles , Anesthetics, Intravenous
12.
Curr Opin Neurol ; 36(5): 416-423, 2023 10 01.
Article in English | MEDLINE | ID: mdl-37639450

ABSTRACT

PURPOSE OF REVIEW: Thymectomy has long been used in the treatment of patients with myasthenia gravis and antibodies against the acetylcholine receptor. However, its effectiveness has only been proven a few years ago in a randomized controlled trial in patients under the age of 65. Here, we review the current literature focusing on patient subgroups, potential biomarkers for outcome prediction and the choice of surgical approach. RECENT FINDINGS: Long-term follow-up studies after thymectomy confirmed that the benefits regarding clinical outcome parameters and a reduced need for immunosuppressive treatment persist. Nevertheless, a substantial proportion of patients in real-world cohorts do not reach complete stable remission after thymectomy indicating that the underlying autoimmune process is sustained in the periphery. Our understanding of the responsible mechanisms has improved with recent studies. Presently, outcome data after thymectomy in several patient subgroups, such as those aged over 50 years, those with juvenile onset or those with purely ocular symptoms are limited and have been the focus of recent research activities. Similarly, biomarkers guiding an appropriate patient selection for thymectomy are under investigation. A number of cohort studies demonstrated that minimal invasive surgical techniques such as extended robotic thymectomy lead to similar positive outcomes as a transsternal approach with potentially fewer short-term adverse effects. SUMMARY: Thymectomy is an effective treatment option in adult patients with early onset acetylcholine-receptor positive myasthenia gravis but uncertainty remains with regard to certain patient subgroups.


Subject(s)
Drug-Related Side Effects and Adverse Reactions , Myasthenia Gravis , Adult , Humans , Middle Aged , Thymectomy , Myasthenia Gravis/surgery , Antibodies , Immunosuppressive Agents , Randomized Controlled Trials as Topic
13.
Pediatr Neurol ; 148: 17-22, 2023 11.
Article in English | MEDLINE | ID: mdl-37651972

ABSTRACT

BACKGROUND: Thymectomy is a treatment for pediatric myasthenia gravis, but the efficacy over time is unknown. Multi-institutional data are also lacking. Therefore, the objective of this study was to determine the efficacy of thymectomy for pediatric myasthenia gravis using medication burden and health care utilization as proxies for disease severity. METHODS: This was a cross-sectional study of the Pediatric Health Information System database among children who underwent thymectomy at one of 49 children's hospitals from 2004 to 2022. Differences in annual median number of doses of myasthenia-related medications, admissions, and health care costs in the year before thymectomy to three years after were compared. A comparison cohort that did not undergo thymectomy was utilized. Medians were compared using the Wilcoxon signed-rank test. Generalized linear regression estimated the effect of surgical approach on outcomes. RESULTS: A total of451 patients (238 patients who underwent thymectomy and 213 nonthymectomy patients) were identified. Following thymectomy, the decrease in annual median total number of myasthenia-related doses was 12.0 (interquartile range: 6 to 31) (P < 0.001). The decrease in number of annual admissions was 2.0 (1 to 4) (P < 0.001), which represented a cost difference of $5292 ($3533 to $8681) (P < 0.001). No differences were observed in the control cohort. In a generalized linear regression model, surgical approach was not associated with the efficacy of thymectomy (P = 0.55). CONCLUSIONS: Thymectomy is an effective treatment for pediatric myasthenia gravis, evidenced by the decreased medication burden and health care utilization after surgery. Surgical approach did not influence the success of surgery. Thymectomy should be considered earlier in the treatment algorithm.


Subject(s)
Myasthenia Gravis , Thymectomy , Humans , Child , Cross-Sectional Studies , Retrospective Studies , Treatment Outcome , Myasthenia Gravis/surgery , Myasthenia Gravis/drug therapy , Tertiary Care Centers
14.
Kyobu Geka ; 76(7): 523-527, 2023 Jul.
Article in Japanese | MEDLINE | ID: mdl-37475095

ABSTRACT

Extended thymectomy is a procedure to remove the thymus gland and surrounding adipose tissue, while the traditional approach via a median sternotomy, minimally invasive approaches such as video-assisted thoracoscopic surgery (VATS) and robot-assisted thoracoscopic surgery (RATS) have been adopted. This report described the technique of bilateral approach for extended thymectomy in patients with myasthenia gravis (MG) by robot-assisted thoracoscopic surgery, and also showed the perioperative outcomes and postoperative exacerbation rates of 11 patients. In most patients, score of MG symptom were reduced and levels of anti-acetylcholine receptor antibodies declined postoperatively. In a small number of cases, the safety and efficacy of a RATS bilateral approach for extended thymectomy were confirmed.


Subject(s)
Myasthenia Gravis , Robotics , Humans , Thymectomy/methods , Treatment Outcome , Thoracic Surgery, Video-Assisted , Myasthenia Gravis/surgery
15.
Kyobu Geka ; 76(7): 571-575, 2023 Jul.
Article in Japanese | MEDLINE | ID: mdl-37475104

ABSTRACT

OBJECTIVES: This study aimed to identify the validity of video-assisted thoracic surgery (VATS) for thymoma, including myasthenia gravis( MG). METHODS: A total of 20 patients who underwent VATS for thymoma at our institution between 2011 and 2019 were included in the study. Preoperative complications and prognosis were analyzed to detect the safety and efficiency of VATS thymectomy. MG improvement after VATS extended thymectomy was evaluated using a quantitative myasthenia gravis score( QMGS) and Myasthenia Gravis Foundation of America post-intervention status( MGFA-PIS). RESULTS: The median tumor size was 2.6 cm (range, 1.0-8.0 cm). All thymomas were classified as TNM stageⅠ. Two patients had type A thymoma, five had type AB, six had type B1, six had type B2, and one had type B3. Postoperative complications were only observed in one patient with pneumonia. The median follow-up period was 5.1 years;no recurrence and disease-related deaths were observed. However, three patients died of other diseases. In patients with MG, postoperative crisis was not observed, and the symptoms improved in all the patients as evaluated by QMGS and MGFA-PIS. CONCLUSIONS: VATS thymectomy and VATS extended thymectomy for patients with thymoma may be effective methods to improve not only prognosis but also MG, provided the range of resections are comparable to that of conventional open surgery.


Subject(s)
Myasthenia Gravis , Thymoma , Thymus Neoplasms , Humans , Thymoma/complications , Thoracic Surgery, Video-Assisted , Retrospective Studies , Thymus Neoplasms/complications , Myasthenia Gravis/surgery , Thymectomy/methods , Treatment Outcome
16.
Updates Surg ; 75(8): 2321-2326, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37368230

ABSTRACT

Myasthenia gravis (MG) is an autoimmune disease (AD), and patients with MG often have other types of ADs. We analyzed the prognosis of patients with MG complicated by AD after thymectomy. A retrospective analysis was performed for patients with MG complicated by ADs treated surgically in our center over the past 22 years, and their general condition and follow-up data were collected and analyzed. 33 patients were included totally. 28 patients displayed improvement or even complete recovery of MG, and 23 of 36 ADs revealed improvement or even complete recovery. The prognosis of MG is significantly correlated with the duration of postoperative follow-up time (p = 0.028), and in patients with thymoma, the larger the tumor diameter, the better the prognosis of MG (p = 0.026). Thymic hyperplasia patients were predominantly female (p = 0.049) and young (p < 0.001). The most common concomitant AD in this study was a thyroid-associated AD, which was associated with thymic hyperplasia (p < 0.001), Osserman type I MG (p < 0.001), and young age (p < 0.001). Thymectomy had a good therapeutic effect on MG complicated by AD, and there was a close correlation between surgery, thymus, MG, and ADs.


Subject(s)
Myasthenia Gravis , Thymus Hyperplasia , Thymus Neoplasms , Humans , Female , Male , Thymectomy/adverse effects , Thymus Hyperplasia/complications , Thymus Hyperplasia/surgery , Retrospective Studies , Myasthenia Gravis/complications , Myasthenia Gravis/surgery , Thymus Neoplasms/complications , Thymus Neoplasms/surgery , Treatment Outcome
17.
J Clin Neuromuscul Dis ; 24(4): 222-228, 2023 Jun 01.
Article in English | MEDLINE | ID: mdl-37219866

ABSTRACT

OBJECTIVES: To evaluate patient attitudes and beliefs toward thymectomy for myasthenia gravis (MG). METHODS: The Myasthenia Gravis Foundation of America administered a questionnaire to the MG Patient Registry, an ongoing longitudinal survey of adult MG patients. Questions assessed reasons for or against thymectomy and how hypothetical scenarios would have affected their decision. RESULTS: Of 621 respondents, 190 (31%) reported a history of thymectomy. Of those who underwent thymectomy for nonthymomatous MG, 97 (51.6%) ranked symptom improvement as most important and 100 (53.2%) ranked reducing medication as least important. Among 431 nonthymectomy patients, the most frequent reason for not undergoing thymectomy was that their doctor did not discuss it (152 of 431 = 35.2%) and 235 (56.8%) said that they would have considered it more strongly if their doctor spent more time discussing it. CONCLUSIONS: Thymectomies are motivated more by symptoms than by medication, and a lack of neurologist discussion is the most common barrier to thymectomy.


Subject(s)
Health Knowledge, Attitudes, Practice , Myasthenia Gravis , Patients , Registries , Surveys and Questionnaires , Thymectomy , Routinely Collected Health Data , Myasthenia Gravis/epidemiology , Myasthenia Gravis/surgery , Humans , Male , Female , Middle Aged , Aged , Aged, 80 and over , Thymoma/epidemiology , Goals , Receptors, Cholinergic/immunology , Autoantibodies/analysis
18.
Eur J Cardiothorac Surg ; 63(5)2023 05 02.
Article in English | MEDLINE | ID: mdl-37162377

ABSTRACT

OBJECTIVES: Total thymectomy in addition to medical treatment is an accepted standard therapy for myasthenia gravis (MG). Patients with severe generalized MG present life-threatening events, poor prognosis and higher risk of postoperative myasthenic crisis. The aim of our study is to investigate neurological and surgical results in patients with Myasthenia Gravis Foundation of America (MGFA) class IV and V MG following thymectomy. METHODS: Data on 76 MG patients with preoperative MGFA classes IV and V who underwent thymectomy were retrospectively collected. Primary end points included short-term surgical outcomes and long-term neurological results including the achievement of complete stable remission and any improvement as defined by MGFA Post-Intervention Status criteria. RESULTS: There were 27 (35.5%) males and 49 (64.5%) females; 53 (69.7%) were classified as MGFA class IV and 23 (30.3%) as class V. Thymectomy was performed through sternotomy in 25 (32.9%) patients, Video-assisted thoracic surgery (VATS) in 5 (6.6%) and Robot-assisted thoracic surgery (RATS) in 46 (60.5%). The median operative time was 120 (interquartile range: 95; 148) min. In-hospital mortality was observed in 1 (1.3%) patient and postoperative complications in 14 (18.4%) patients. The median postoperative hospital stay was 4 (interquartile range: 3; 6) days. Pathological examination revealed 31 (40.8%) thymic hyperplasia/other benign and 45 (59.2%) thymomas. Cumulative complete stable remission and improvement probabilities were 20.6% and 83.7% at 5 years and 66.9% and 97.6% at 10 years, respectively. A significant improvement rate was found in patients with age at the time of thymectomy of ≤50 years (P = 0.0236), MGFA class V (P = 0.0154) and acetylcholine receptor antibodies positivity (P = 0.0152). CONCLUSIONS: Thymectomy in patients with severe MG yields good perioperative outcomes and satisfactory long-term neurological improvement, especially for patients younger than 50 years, with MGFA class V and anti-AChR+ MG.


Subject(s)
Myasthenia Gravis , Thymoma , Thymus Neoplasms , Male , Female , Humans , Thymectomy/adverse effects , Thymectomy/methods , Retrospective Studies , Myasthenia Gravis/surgery , Myasthenia Gravis/complications , Thymoma/surgery , Thymoma/complications , Treatment Outcome , Thymus Neoplasms/surgery
19.
Neuromuscul Disord ; 33(5): 417-424, 2023 05.
Article in English | MEDLINE | ID: mdl-37037051

ABSTRACT

The aim of this study was to investigate the surgical and long-term neurological outcomes of patients with acetylcholine-receptor-antibody-associated myasthenia gravis (AChR-MG) who underwent robotic thymectomy (RATS). We retrospectively analyzed the clinical-pathological data of all patients with AChR-MG who underwent RATS using the DaVinci® Robotic System at the MUMC+ between April 2004 and December 2018. Follow-up data were collected from 60 referring Dutch hospitals. In total, 230 myasthenic patients including 76 patients with a thymoma (33.0%) were enrolled in this study. Mean follow-up time, procedure time and hospitalization were, respectively 65.7 ± 43.1 months, 111±52.5 min and 3.3 ± 2.2 days. Thymomatous patients had significantly more frequently and more severe complications than nonthymomatous patients (18.4% vs. 3.9%, p<0.001). Follow up data was available in 71.7% of the included patients. The Myasthenia Gravis Foundation of America postintervention score showed any kind of improvement of MG-symptoms after RATS in 82.4% of the patients. Complete stable remission (CSR) or pharmacological remission (PR) of MG was observed in 8.4% and 39.4% of the patients, respectively. Mean time till CSR/PR remission after thymectomy was 26.2 ± 29.2 months. No statistical difference was found in remission or improvement in MGFA scale between thymomatous and nonthymomatous patients. RATS is safe and feasible in patients with MG. The majority of the patients (82.4%) improved after thymectomy. CSR and PR were observed in 8.4% and 39.4% of the patients, respectively, with a mean of 26.2 months after thymectomy. Thymomatous patients had more frequently and more severe complications compared to nonthymomatous patients.


Subject(s)
Myasthenia Gravis , Robotic Surgical Procedures , Thymus Neoplasms , Humans , Thymectomy , Acetylcholine , Treatment Outcome , Robotic Surgical Procedures/adverse effects , Retrospective Studies , Myasthenia Gravis/surgery , Myasthenia Gravis/complications , Thymus Neoplasms/complications , Receptors, Cholinergic , Autoantibodies
20.
Respir Med Res ; 83: 101009, 2023 Jun.
Article in English | MEDLINE | ID: mdl-37087902

ABSTRACT

BACKGROUND: Thymic malignancies are rare tumors about which data are limited. Our objective here was to evaluate the outcomes and risk factors for complications and death in patients who underwent extended surgery to remove thymic malignancies. METHODS: We retrospectively included patients who underwent extended resection of locally advanced, nonmetastatic thymic malignancies at our institution. Patients were deemed eligible for resection by a multidisciplinary team. During surgery, priority was given to achieving complete resection rather than to sparing organs. RESULTS: The 108 patients had a mean age of 53 ± 15 years (range, 9-83); among them, 91 had thymoma, 12 thymic carcinoma, and 5 neuroendocrine tumor. The Masaoka stage was III or higher in 86 patients; examination of operative specimens resulted in downstaging of 22 patients. Tumor-free resection margins were achieved in 98 patients. Overall 5- and 10-year survival rates were 80% and 68%, respectively. Myasthenia gravis, present in 36 patients, was the only independent significant risk factor for major postoperative complications. Age older than 70 years, thymic carcinoma or neuroendocrine tumor, pT3 or pT4 stage, and R1 or R2 resection margins independently predicted death. The number of resected structures was not associated with survival. Thymic carcinoma or neuroendocrine tumor was independently associated with shorter disease-free survival. CONCLUSION: In an expert center, extended resection targeting complete resection rather than organ preservation provided good outcomes in patients with locally advanced thymic malignancies. The risk/benefit ratio of surgery should be assessed with special care in patients who are elderly or have myasthenia gravis.


Subject(s)
Myasthenia Gravis , Neuroendocrine Tumors , Thymoma , Thymus Neoplasms , Humans , Aged , Adult , Middle Aged , Thymoma/surgery , Thymoma/pathology , Retrospective Studies , Margins of Excision , Neoplasm Staging , Thymus Neoplasms/epidemiology , Thymus Neoplasms/surgery , Myasthenia Gravis/epidemiology , Myasthenia Gravis/surgery , Myasthenia Gravis/pathology , Neuroendocrine Tumors/surgery , Neuroendocrine Tumors/pathology
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