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Myasthenia Gravis with Thymoma Results of Treatment
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There continues to be a debate regarding the effectiveness of thymectomy in the treatment of nonthymomatous myasthenia gravis (MG) and, when undertaken, which thymectomy technique is the procedure of choice. The debate persists primarily because of the lack of controlled prospective studies. Analysis has been complicated by the absence, until very...
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Citations
... The benefit of thymectomy is not immediate and appears in the following 1-2 years. 56 The current belief is that the benefits of thymectomy are most significant if performed within the first three years of initial symptoms. 57,58 One study in Iran showed that early thymectomy (less than one year after MG diagnosis) is beneficial. ...
Myasthenia gravis (MG) is an immune-mediated potentially treatable disease in which rapid diagnosis and proper treatment can control symptoms. Treatment should be individualized in each patient according to distribution (ocular or generalized) and severity of the weakness, antibody status, thymus pathology, patient comorbidities, and preferences. A group of Iranian neuromuscular specialists have written these recommendations to treat MG based on national conditions. Four of the authors performed an extensive literature review, including PubMed, EMBASE, and Google Scholar, from 1932 to 2020 before the central meeting to define headings and subheadings. The experts held a 2-day session where the primary drafts were discussed point by point. Primary algorithms for the management of MG patients were prepared in the panel discussion. After the panel, the discussions continued in virtual group discussions, and the prepared guideline was finalized after agreement and concordance between the panel members. Finally, a total of 71 expert recommendations were included. We attempted to develop a guideline based on Iran's local requirements. We hope that these guidelines help healthcare professionals in proper treatment and follow-up of patients with MG.
... El primer reporte que existe sobre la timectomía es de hace 75 años, aproximadamente, y, a partir de entonces, se han desarrollado múltiples estudios en los que se sugiere que, entre mayor cantidad de tejido tímico se retire, mejores son los resultados clínicos de la intervención 8,9 . Se han descrito diferentes técnicas quirúrgicas para la timectomía; se puede practicar mediante abordaje abierto, como la esternotomía, o con intervenciones menos invasivas, como la toracoscopia bilateral o la unilateral 7,10 . No existe evidencia clínica clara que indique el mejor tipo de abordaje quirúrgico en estos pacientes. ...
Introducción. La miastenia grave es una enfermedad autoinmunitaria con una prevalencia mundial de 150 a 250 casos por 1´000.000 de habitantes. El tratamiento recomendado para la miastenia grave sin timoma es la timectomía total, la cual es la única alternativa de curación.
Métodos. Se llevó a cabo un estudio descriptivo y retrospectivo de una serie de casos de pacientes adultos con miastenia grave sin timoma sometidos a timectomía, durante el periodo de 2010 a 2017. En el análisis estadístico descriptivo, se utilizaron frecuencias absolutas y porcentajes para las variables cualitativas y, para las variables cuantitativas, se utilizaron la mediana y el rango intercuartílico.
Resultados. Veintiocho pacientes con miastenia grave sin timoma se sometieron a timectomía desde el año 2010 hasta el 2017. Se categorizaron según la clasificación del estado posterior a la intervención de la Myasthenia Gravis Foundation of America y se evidenció que 4 (14,3 %) pacientes presentaban remisión completa y el grado 3 de manifestaciones clínicas mínimas fue el más frecuente en 19 (67,9 %); 26 (92,9 %) tuvieron mejoría con respecto al cambio del estado clínico, en 2 (7,1 %) no se documentaron cambios y en ningún paciente hubo empeoramiento, exacerbación o muerte secundaria a la enfermedad.
Conclusiones. A lo largo de siete años se practicó timectomía a 28 pacientes con diagnóstico de miastenia grave sin timoma, aproximadamente, en el 15 % de los pacientes hubo remisión completa, el grado 3 de manifestaciones mínimas fue el más frecuente y el 93 % presentó mejoría de su estatus clínico.
... Thymectomy is most commonly performed for thymoma and other anterior mediastinal masses, and in cases of medication-refractory myasthenia gravis with or without associated thymoma (1)(2)(3)(4)(5)(6). While median sternotomy has been the longstanding approach to thymectomy and anterior mediastinal resections, numerous minimally invasive approaches have emerged in the last several decades (7)(8)(9). ...
Background:
Thoracoscopic approaches to thymectomy and anterior mediastinal mass resection has become increasingly common due to the potential for decreased blood loss and hospital length of stay. However, contralateral mediastinal and phrenic nerve visualization if often difficult from these unilateral approaches, which may affect the ability to achieve a full phrenic to phrenic dissection Herein, we present our early experience of robotic assisted minimally invasive thymectomy (RAMIT) with simultaneous bilateral thoracoscopy and contralateral phrenic nerve visualization.
Methods:
This was a retrospective review of all sequential patients undergoing RAMIT with simultaneous bilateral thoracoscopy from January 2015 to May 2016. This study was approved by our Institutional Review Board (PRO15080367). Individual patient consent was waived.
Results:
Twenty-six patients [median age 58 (range, 29-76) years] were included in this study. Sixteen operations were performed for anterior mediastinal mass, 7 for non-thymomatous myasthenia gravis, and 3 for concurrent myasthenia gravis and thymoma. Median blood loss and hospital stay were 25 mL (range, 3-150 mL) and 3 days (range, 2-8 days), respectively. Twenty-one (80.8%) patients experienced an uncomplicated hospital course. The highest graded complication by Clavien Dindo Classification was a grade III due to pleural effusion requiring drainage via pleural catheter. One patient experienced asymptomatic hemidiaphram palsy postoperatively. There were no 90-day postoperative deaths.
Conclusions:
RAMIT with simultaneous bilateral thoracoscopy is a feasible approach that may allow for enhanced visualization and more complete thymic resection compared to existing unilateral minimally invasive operations. Comparative studies and long-term follow up are needed to adequately assess the potential benefits of RAMIT.
... 20 Primary epithelial tumors of the thymus are found in approximately 50% of all anterior mediastinal masses, of which thymoma is foremost common. 21 Thymectomy is an appropriate therapy in the great care of MG and in the undetermined anterior mediastinal lesion. 22 Minimal access thymectomy can be performed in all patients of thymic neoplasm who will tolerate single lung ventilation. ...
... Furthermore, some studies do support that changes in acetylcholine receptor antibody titers parallel with the clinical changes after thymectomy (56,57), although others do not (58,59). Second, "the more complete the resection, the better clinical outcome" has become an international consensus of thymectomy (60,61). On the other hand, ectopic thymic tissue was also found in more than a half of residual thymus after reoperations, which can relieve the persistent symptoms after the first partial resection (20). ...
Extended thymectomy has been considered the goal of surgery for myasthenia gravis (MG) mainly due to the existence of ectopic thymic tissue. Recently, ectopic thymic tissue has attracted increasing attention in patients with MG following thymectomy. However, the specific role of ectopic thymic tissue in patients with MG is still under debate. A systematic search of the literature was performed on PubMed and Medline according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISM) statement. Studies evaluating the rate of ectopic thymic tissue in patients with MG with or without thymoma were included. Extraction was performed for all eligible studies and the rate of ectopic thymic tissue at common locations was calculated. Eighteen out of fifty-nine studies were eligible for inclusion, of which ten studies reported the common locations of ectopic thymic tissue in mediastinal fat. Of these ten studies, the presence of ectopic thymic tissue was investigated in different anatomical locations in 882 patients, of whom, 509 patients (58%) have at least one positive location with the most common ones being anterior mediastinal fat, pericardiophrenic angles, aortopulmonary window, cervical region (pretracheal fat) and lateral to phrenic nerves. On the other hand, nine studies analyzed the influence of the presence of ectopic thymic tissue on the clinical outcomes of MG patients. Of these, six found that the presence of ectopic thymic tissue in MG patients is a significant predictor of poor outcome after thymectomy, however, the other three did not find a significance. Altogether, ectopic thymic tissue is likely to present in more than a half of patients undergoing thymectomy for MG. Besides, MG patients who have ectopic thymic tissue after thymectomy do not seem to have as good outcome as those who have not.
... Todavía se discute cuál es el tratamiento más eficaz para los pacientes con esta enfermedad. El tratamiento ideal debería ser eficaz, tener efectos secundarios mínimos, ser de fácil administración y de bajo costo (5,7) . De acuerdo con la clínica del paciente, la severidad de los síntomas, incluyendo los músculos comprometidos por la enfermedad, se dan medicamentos que tratan los síntomas y no cambian el curso de la enfermedad (anticolinesterasicos, corticoides, inmunosupresores, inmunoglobulinas, plasmaferesis); otros que frenan la respuesta inmunológica y finalmente la timectomía, que cambia el curso de la enfermedad (4,5) . ...
... Todos los pacientes fueron timectomizados, la técnica quirúrgica en la mayoría de los pacientes fue la transesternal, esta técnica era la de elección en las timectomias, hasta antes del advenimiento de la cirugía mínima invasiva, actualmente las últimas timectomias las realizamos por VATS con buenos resultados (menos dolor, menor estadía hospitalaria). Algunos estudios han comparado la timectomía VATS con los abordajes abiertos y se ha encontrado que las tasas de remisión y de mejoría con cada técnica son comparables (7,10) . ...
RESUMEN
Introducción:
Los trastornos de la transmisión neuromuscular, son una causa poco frecuente pero importante de debilidad en niños y adultos, se manifiesta por una debilidad quepredomina en ciertos grupos musculares y en forma característica fluctúa en respuesta al esfuerzo y al descanso.Dentro de este grupo de trastornos la de mayor importancia es la Miastenia Gravis.
Objetivo:
Conocer el manejo quirúrgico de la Miastenia Gravis en los servicios de cirugía del Hospital de Clínicas de enero de 2008 a noviembre de 2018.
Material y Metodología:
Estudio observacional, descriptivo, retrospectivo, de corte transversal, del 2008 al 2018.
Resultados:
13 pacientes con diagnóstico de Miastenia Gravis, predominio del sexo femenino, relación observada entre ambos sexos de 2,25/1. El promedio de edad fue de 29,5 años. Las manifestaciones clínicas más frecuentes fueron, ptosis palpebral, diplopía y debilidad muscular generalizada. El subtipo IIB es el más frecuente (Osserman).Todos fueron intervenidos quirúrgicamente, timectomia transesternal en el 84,6% y en el 15,4% timectomia por VATS. Se hallo hiperplasia tímica en el 69,2%.Evolución; el 23,1% está sin medicación, el 46,1% medicación en menor dosis.
Conclusión:
Se observó remisión de la enfermedad y una mejoría clínica postquirúrgica evidente en la gran mayoría de los pacientes.
... Thymectomy is the standard treatment of thymoma and malignant neoplasms of the thymus [1][2][3][4][5], and it has been repeatedly demonstrated to be a safe and effective treatment for myasthenia gravis (MG) in both adult [1,[6][7][8][9] and pediatric patients [10][11][12][13][14]. Traditionally, thymectomies have been performed by either a median sternotomy or transcervical approach in both pediatric and adult patients. Transsternal thymectomy has been historically preferred, as it is thought to allow for a more complete resection of the diseased thymus [3,6]. ...
... Thymectomy is the standard treatment of thymoma and malignant neoplasms of the thymus [1][2][3][4][5], and it has been repeatedly demonstrated to be a safe and effective treatment for myasthenia gravis (MG) in both adult [1,[6][7][8][9] and pediatric patients [10][11][12][13][14]. Traditionally, thymectomies have been performed by either a median sternotomy or transcervical approach in both pediatric and adult patients. Transsternal thymectomy has been historically preferred, as it is thought to allow for a more complete resection of the diseased thymus [3,6]. However, recent advances in technology and surgical technique have allowed surgeons to achieve similar resections and outcomes with minimally invasive approaches [7,15]. ...
Background. Thymectomy in pediatric patients is an effective treatment for myasthenia gravis (MG), thymic neoplasms, and other rarer pathologies. It is an uncommon procedure in children and studies have focused on small, single-institution cohorts. We sought to better characterize its use by utilizing a national database.
Methods. The Kids’ Inpatient Database was used to identify hospital discharge records of patients ≤ 20 years old who underwent thymectomy. A retrospective cross-sectional analysis for 2003, 2006, 2009, and 2012 was performed. Trends in patient characteristics, diagnosis, surgical approach, and short-term outcomes were analyzed. Risk factors were identified using univariate and multivariate analyses.
Results. There were 600 thymectomies identified. MG was the most common indication. Thoracoscopy is being used increasingly for all diagnoses except malignancy. The overall morbidity rate was 14.0%, with respiratory complications representing the largest group. No in-hospital deaths were identified. Private insurance was associated with shorter hospital stays and lower costs. Hispanic race was associated with more complications, longer stays, and higher costs. Thoracoscopic thymectomies had shorter stays than open procedures.
Conclusion. Thymectomy in the pediatric population is being performed safely, with low morbidity and no identified mortalities. Thoracoscopy results in reduced length of stay and is being used increasingly. Of note, socioeconomic and racial factors impact outcomes.
... Although thymectomy and CABG are very common procedures but a combination of these two in a myasthenia gravis patient is very rare in our setting. No special surgical technique is required to perform these procedures simultaneously as both thymectomy and CABG can be performed via median sternotomy [2]. Both the phrenic nerves should be taken care of while doing thymectomy as phrenic nerve palsy can result in diaphragm dysfunction which can compromise the respiratory reserve of the patient [3]. ...
The role of thymus in the pathogenesis of myasthenia gravis is not entirely clear, but most patients with myasthenia gravis are found to have some degree of thymus abnormality. The thymus is hypothesized to be the site of autoantibody formation and therefore thymectomy has been proposed as a first line therapy. This is especially true if a thymoma is present, as thymectomy has been reported to significantly improve the clinical condition. Here we report a case of off pump coronary artery bypass grafting surgery who underwent thymectomy at the same sitting.
... Extended thymectomy (ET) is widely accepted as efficacious for the treatment of non-thymomatous myasthenia gravis (NTMG), especially after the recent randomized controlled trial findings published in the New England Journal of Medicine provided evidence of the benefit of thymectomy plus medication over medication alone [1,2]. Long-term outcomes following ET are variable, with reported complete remission and improvement rate increasing to 88% and approximately 100%, respectively [3,4]. ...
Objectives:
To evaluate the predictive value of the intraoperative thymofatty specimen weight (TFSW) index on predicting the prognosis of extended thymectomy (ET) for non-thymomatous myasthenia gravis.
Methods:
This is a prospective non-interventional study in which patients who underwent ET between January 2012 and June 2015 were enrolled. Resected thymus and surrounding adipose tissues were weighed using an electronic scale intraoperatively and adjusted to the body surface area (BSA) to calculate the TFSW index. The primary end-point was defined as complete stable remission (CSR) according to the Myasthenia Gravis Foundation of America (MGFA) guidelines.
Results:
One hundred and eighteen patients who completed postoperative follow-up were included in this study. After a mean follow-up period of 44 months, 68 (57.6%) patients reached clinical CSR. The MGFA class, histopathology and TFSW index were associated with a postoperative CSR in univariate analysis. When the Cox hazard multiple regression model was used, the TFSW index was found to be an independent predictor for CSR (hazard ratio 2.056; 95% confidence interval 1.182-3.576). Based on ROC analysis, an optimal TFSW index cut-off value (35.9 g/m2) with the highest sensitivity and specificity was determined.
Conclusions:
The TFSW index is an important independent predictor for mid-term CSR after ET in non-thymomatous myasthenia gravis patients. During the ET surgery, every effort should be made to take a tissue specimen with a TFSW index more than 35.9 g/m2.
... Remission rates in the first year are less than 20%. However, the remission rates have increased up to 50% over 7-10 years [7,9,23]. ...
... Median sternotomy (extended transsternal thymectomy or combined transcervical-transsternal thymectomy) is preferred by many chest surgeons and neurologists [9,23]. This approach provides a broad exploration area from mediastinum to neck, allowing complete resection of all thymic and associated fat tissues. ...
... The simple transcervical approach is rarely performed. Surgical excursion of the thymus is inadequate, and residual thymus tissue remains in most patients [23,29]. ...
In recent years, thymectomy has become a widespread procedure in the treatment of myasthenia gravis (MG). Likelihood of remission was highest in preoperative mild disease classification (Osserman classification 1, 2A). In absence of thymoma or hyperplasia, there was no relationship between age and gender in remission with thymectomy. In MG treatment, randomized trials that compare conservative treatment with thymectomy have started, recently. As with non-randomized trials, remission with thymectomy in MG treatment was better than conservative treatment with only medication. There are four major methods for the surgical approach: transcervical, minimally invasive, transsternal, and combined transcervical transsternal thymectomy. Transsternal approach with thymectomy is the accepted standard surgical approach for many years. In recent years, the incidence of thymectomy has been increasing with minimally invasive techniques using thoracoscopic and robotic methods. There are not any randomized, controlled studies which are comparing surgical techniques. However, when comparing non-randomized trials, it is seen that minimally invasive thymectomy approaches give similar results to more aggressive approaches.
