Myasthenia gravis (MG) is a common antibody mediated, cell-mediated, and complement dependent neuromuscular junction immune disease. The treatment mainly includes drug therapy (symptomatic therapy, non-specific immunosuppressive therapy, targeted immunotherapy), immune regulation (intravenous injection of human immunoglobulin and plasma exchange), and thymectomy. With the continuous deepening of research on MG treatment, targeted immune regulation of B cells, complement system, and neonatal Fc receptors has become a current research hotspot in the treatment of MG. Compared with traditional immunosuppressants, MG patients have better tolerance to new biological agents. This article elaborates on the research of MG targeted therapy related drugs and summarizes their efficacy and safety in MG treatment, aiming to find more treatment options.
ObjectiveTo investigate the perioperative safety of patients with myasthenia gravis who take high doses of oral corticosteroids. MethodsA retrospective analysis was conducted on the clinical data of patients with myasthenia gravis who received oral corticosteroids and underwent thoracoscopic thymectomy at the Department of Thoracic Surgery, the University of Hong Kong-Shenzhen Hospital from April 2013 to October 2019. Patients were divided into a high-dose steroid group and a medium-to-low dose steroid group based on the dosage of oral steroids, and the clinical data of the two groups were compared. ResultsA total of 102 patients were included, including 19 (18.62%) males and 83 (81.37%) females, with an average age of (32.25±9.83) years. All patients in both groups successfully completed the surgery without major intraoperative bleeding, conversion to open chest surgery, delayed extubation, severe infection, or perioperative death. The daily oral steroid dose for the high-dose steroid group was (35.81±4.29) mg, and for the medium-to-low dose steroid group it was (15.29±2.17) mg. There was no statistical difference in the operation time [(124.69±23.51) min vs. (117.89±21.46) min, P=0.172] and intraoperative blood loss [(21.19±3.48) mL vs. (20.56±3.41) mL, P=0.419] between the two groups. Postoperatively, 12 (11.76%) patients developed complications: one patient of myasthenic crisis (medium-to-low dose steroid group), which was improved after short-term respiratory support and intravenous immunoglobulin treatment; 11 patients of respiratory/swallowing difficulties (9 in the low-dose steroid group and 2 in the high-dose steroid group), which were improved after anticholinergic treatment to reduce oral secretions and sputum suction, and the patients were discharged smoothly. There was no statistical difference in the incidence of postoperative complications between the two groups (P=0.637). ConclusionOn the basis of good perioperative management, it is safe and feasible for patients with myasthenia gravis who take high dose of oral steroids to undergo thymectomy, and they have the same perioperative safety as patients with medium-to-low dose steroids.
Objective To present the preliminary clinical experience of robot assisted trans-subxiphoid (extended) thymectomy in patients with thymic neoplasms or myasthenia gravis. Methods A total of 62 patients (34 males and 28 females at an average age of 38±11 years) suffering from thymic neoplasms or myasthenia gravis who underwent robotic (extended) thymectomy via subxiphoid approach were included in our department between August 2016 and August 2017. All of the operation were completed through 4 ports. In details, the observation hole was created just below the xiphoid process, two ports for arm 1 and arm 2 were created below bilateral subcostal arch at the midclavicular line, and trocar for arm 3 was placed in the 5th or 6th intercostal space at the anterior axillary line, respectively. Patients with thymic neoplasms received thymectomy. Patients with myasthenia gravis received extended thymectomy. Results All the patients experienced uneventful operations. The mean operative time was 116.0±34.0 min. The mean intraoperative blood loss was 5.6±4.3 ml. The mean postoperative hospital stay was 4.0±2.2 days. There was no intra-operational massive hemorrhage, mortality, conversion or postoperative complication during the postoperative and follow-up period. Conclusion Robotic trans-subxiphoid thymectomy is safe and feasible, which is a promising technique for extensive application.
ObjectiveTo analyze the research hotspots and progress of surgical treatment of myasthenia gravis. MethodsThe top 100 most cited articles on surgical treatment of myasthenia gravis were identified by searching the Web of Science database, and a bibliometric analysis was conducted. ResultsThe publication year of the top 100 most cited articles ranged from 1939 to 2021, and the number of citations ranged from 55 to 850 per article. Most of the included articles were original research articles (75/100), which were mainly retrospective studies (64/75). The United States was the country with the most published articles and most citations, and Annals of Thoracic Surgery was the most sourced journal (n=20). Through VOSviewer analysis, high-density keywords were thymectomy, maximal thymectomy, extended thymectomy, transcervical thymectomy, thymoma, and autoantibodies. ConclusionThe scope of surgical resection, surgical approach and pathogenesis are the current hotspots in the field of surgical treatment of myasthenia gravis. It is hoped that this paper can provide references for future researches in this field.
Objective To summarize experiences of surgical treatment and long-term results of myasthenia gravis (MG). Methods Two hundred thirty-six patients underwent thymectomy for MG in our department from Jan.1978 to Dec. 2002. The perioperative management, relative factors of postoperative crisis and long-term results were analysed. Results In 236 patients postoperative crisis took place in 44 cases accounted for 18.6%. The occurrence of postoperative crisis was related to preoperative management, modified Osserman clinical classification and combination with thymoma. Three cases died in the postoperative periods. Among them, one died of acute respiratory distress syndrome induced by aspiration and the other died of crisis. The effective rate in 1, 3, 5 years was 84.6%, 91.0% and 89.0% respectively. Conclusions Thymectomy for MG is safe and effective. Delayed extubation could decrease the needs of tracheotomy in patients with high risk factors for postoperative crisis. The partial sternotomy approach is less traumatic but the long-term effects of surgery are identical to those reported by the most authors.
Objective To investigate application values and techniques of robot-assisted extended thymectomy for the treatment of typeⅠmyasthenia gravis （MG） using Da Vinci S system. Methods We retrospectively analyzed clinical data of 3 patients with MG who underwent robot-assisted extended thymectomy in General Hospital of Shenyang Military Command from March 2012 to September 2012. All the patients were ocular MG （typeⅠ） including 2 men （33 years and 66 years old respectively） and 1 woman （21 years old）. Surgical outcomes were analyzed. Results All the 3 patients successfully received robot-assisted extended thymectomy without accessorial incisions. None of the patients required converting to open sternotomy or postoperative reexploration for bleeding. Intraoperative blood loss was 5-10 ml．Overall operation time was95-138 minutes, and thymoma dissection time was 26-80 minutes. No myasthenic crisis or other major postoperative complic-ation occurred. Postoperative chest drainage duration was 3-9 days and postoperative hospital stay was 10-15 days. Two patientswere followed up for 6-12 months after discharge without MG recurrence. Conclusion Robot-assisted extended thymectomy is safe and feasible for the treatment of typeⅠMG with satisfactory results．
ObjectiveTo analyze the risk factors for post-thymectomy myasthenic crisis (PTMC) and prolonged mechanical ventilation, in myasthenia gravis patients who underwent extended thymectomy. MethodsWe retrospectively analyzed the clinical data of 79 patients including 38 males and 41 females who experienced PTMC and required mechanical ventilation in Daping Hospital between June 2008 and November 2014. Single factor analysis and multivariate analysis were conducted. ResultsMorbidity of PTMC was 20.6% (79/384). Result of single-factor analysis showed that postoperative pneumonia was one of the main reasons of prolonged mechanical ventilation (P < 0.05). Result of multiple-factor analysis showed that the operation time was positively correlated with mechanical ventilation time (P < 0.05). The risk factor of prolonged mechanical ventilation time in PTMC was not associated with sex, age, disease history, myasthenic crisis history, Osserman classification, dosage of pyridostigmine before and after the operation, surgical approach, bleeding volume, other therapies besides mechanical ventilation (P > 0.05). ConclusionMechanical ventilation is one the main therapy of PTMC, operation time, and postoperative pneumonia are the main factors to prolong mechanical ventilation time. In order to decrease morbidity of PTMC and shorten mechanical ventilation time, the operation time should be controlled and pulmonary infection should be avoided.
ObjectiveTo analyze the surgical efficacy and influencing factors of myasthenia gravis (MG) patients with thymic atrophy after thymectomy. MethodsThe clinical data of MG patients with thymic atrophy undergoing thymectomy between October 2014 and May 2018 in Daping Hospital of Army Medical University and Shijiazhuang People Hospital were retrospectively analyzed. ResultsA total of 71 patients were collected, including 40 males and 31 females with a mean age of 45.17±12.42 years. All patients received the surgery successfully. After the surgery, 20 (28.17%) patients were stable remission, 12 (16.90%) patients were minimal manifestation status,19 (26.76%) patients were improved, 5 (7.04%) patients showed no change, 3 (4.23%) patients were worsened, 10 (14.08%) patients were exacerbated and 2 (2.82%) patients were dead. Multivariate logistic regression analysis showed that the preoperative illness duration (OR=4.61, 95%CI 1.13-18.85, P=0.03), and postoperative pyridostigmine combined with immunosuppressive (OR=0.12, 95%CI 0.03-0.45, P=0.00) were independent risk factors for long-term efficacy of thymectomy for MG patients with thymic atrophy. ConclusionEarly surgery after diagnosis of MG and postoperative pyridostigmine combined with immunosuppressive treatment is beneficial to the prognosis of MG patients with thymic atrophy.
ObjectiveTo analyze the risk factors of myasthenia gravis crisis after thymectomy with myasthenia gravis (MG).MethodsSixty-five myasthenia gravis patients who had myasthenia crisis after thymectomy in Xuanwu Hospital, Capital Medical University from June 2006 to June 2019 were retrospectively enrolled, including 31 males and 34 females, aged 15-78 (45.7±17.8) years. The relationship between myasthenia crisis after thymectomy and surgical option, operation time, pathological type, et al. were anylyzed.ResultsOperation time and pathological type were the predictive factors of postoperative myasthenic crisis. The area under receiver operating characteristic curve (AUC) of MG type (Osserman) was 0.676, the cut-off value wasⅡB type, the sensitivity was 37.5%, the specificity was 90.5%, and the Youden’s index was 0.280. The AUC of thymoma stage (Masaoka) was 0.682, cut-off value was stageⅡ, sensitivity was 62.5%, specificity was 66.7%, and Youden’s index was 0.292. The AUC of blood loss was 0.658, the cut-off value was 90 mL, the sensitivity was 87.5%, the specificity was 69.6%, and the Youden’s index was 0.304.ConclusionPreoperative MG classification, pathological type, operation time and blood loss are the risk factors of postoperative myasthenic crisis. Therefore, adequate preoperative preparation, rapid and careful intraoperative operation and active postoperative management can reduce the occurrence of postoperative myasthenic crisis.
Objective To investigate the value of ice test in the diagnosis of ptosis of myasthenia gravis(MG). Methods A total of 32 patients with myasthenic ptosis and 33 with nonmyasthenic ptosis underwent ice and rest test which were performed alternately twice within 1 day on each patient. Besides, neostigmine test was performed on the patients with myasthenic ptosis after ice and rest test . Two observers who didnrsquo;t know the clinical diagnosis were asked to evaluate the improvement of eyelid elevation by measuring the width between the midpoints of upper and lower eyelid with a 20mm steel rule (precision of 0.5 mm). The average of margin of palpebral fissure width after double ice or rest tests subtrac ted from the one before the tests in one patient was the standard of the improve ment of eyelid elevation. Results Ice and rest test improved myasthenic ptosis but not nonmyasthenic ptosis with the specificity of 100% in both of the tests. In addition, ice test improved myasthenic ptosis more effectively with a higher sensitivity of 78%, and it could also improve the palpebral fissure width in pa i tents with complete myasthenic ptosis apparently. Compared with the neostigmine test, ice test had lower sensitivity, cost shorter time, didnt need injection which avoided the discomfort, and had no side effects. Conclusion Ice test is a simple and safe means with high sensitivity and specificity to diagnose myasthenic ptosis, which is valuable in clinical application. (Chin J Ocul Fundus Dis, 2006,22:382-384）