Exposición a fármacos asociados a agravamiento de síntomas en pacientes con miastenia grave / Drug exposure associated with exacerbation of symptoms in patients with myasthenia gravis

INTRODUCCIÓN: Numerosos fármacos se han relacionado con el agravamiento de síntomas en pacientes con miastenia grave, pero hasta la fecha no existen estudios sobre la exposición a fármacos en estos pacientes. OBJETIVOS: Describir el consumo de fármacos y calcular la tasa anual de episodios de exacerbación en una cohorte de pacientes con miastenia grave, y explorar posibles factores de riesgo de exacerbaciones graves. PACIENTES Y MÉTODOS: Estudio observacional longitudinal retrospectivo que incluye a pacientes adultos con miastenia grave seguidos en consulta. Cálculo de frecuencias, tasas y construcción de modelo de eventos repetidos. RESULTADOS: De 91 pacientes incluidos, el 94,51% estuvo expuesto al menos a un fármaco durante el período de estudio (siete años y un mes). De ellos, 51 tuvieron al menos una prescripción de un fármaco contraindicado en la ficha técnica (56,04%). Se contabilizaron 145 exacerbaciones en 50 pacientes. La tasa anual de incidencia fue de 0,35 exacerbaciones por paciente y año. De estas exacerbaciones, 48 fueron graves (en 18 pacientes), con una tasa anual de incidencia de 0,12. Se halló una posible asociación entre diagnóstico de miastenia grave generalizada y timectomía, con un aumento del riesgo de episodios de exacerbación graves. CONCLUSIONES: En esta cohorte se encontró una amplia exposición a fármacos, pero no asociación con el riesgo de episodios de exacerbación graves. Algo más de la mitad de pacientes tuvo al menos un episodio de exacerbación durante el período de estudio, la mayoría leves. Son necesarios estudios que corroboren estas conclusiones y puedan estudiar posibles correlaciones entre fármacos y el riesgo de episodios de exacerbación

INTRODUCTION: Numerous drugs have been related to exacerbation of myasthenia gravis. So far there are no studies examining the extent of use of drugs related to exacerbation of myasthenia gravis. AIMS: We sought to assess the extent of use of drugs related to exacerbations and the annual incidence rate of exacerbations in a cohort of myasthenia gravis patients. We explored possible risk factors of severe exacerbations. PATIENTS AND METHODS: We performed a retrospective cohort study. We included adult patients followed in neurology department. We estimated frequencies, rates and built a recurrent events model. RESULTS: We included 91 patients. 94.51% of patients had at least one prescription of a drug. 51 patients had at least one prescription of a drug contraindicated according to its drug label. 145 exacerbation episodes were reported in 50 patients. The annual incidence rate of exacerbation episodes was 0.35. 48 exacerbations were severe (in 18 patients). The annual incidence rate of severe exacerbation episodes was 0.12. Generalized myasthenia gravis and thymectomy were associated with a higher risk of severe exacerbation episodes. CONCLUSIONS: Our patients were extensive and widespread exposed to drugs during the follow-up period but we did not find and association with severe exacerbation episodes. Just over half of the patients had at least one exacerbation episode during the study period, most of them were mild. Further studies with larger sample sizes are necessary to corroborate these conclusions and to study possible correlations between the use of drugs and the risk of exacerbation episodes

Risk of incident autoimmune diseases in patients with thymectomy.

OBJECTIVES: The data concerning the association between Tx and ADs remain unclear and are scarce. This study was undertaken to investigate whether people with Tx are more likely to develop ADs, compared to those without Tx. METHODS: Individuals who received Tx between 2002 and 2015 were identified and matched on age and sex with individuals without Tx. We performed multivariate and stratified analysis using the Kaplan-Meier method and Cox proportional hazards models in order to estimate the association between Tx and the risk of developing ADs. RESULTS: A total of 2550 thymectomized (Txd) patients and 24,664.941 non-Txd comparison subjects were selected from NHIRD. Tx-MG (myasthenia gravis) as compared with general population (nonTx-nonMG), adjusted hazard ratio (aHR) were higher for incident Addison disease (aHR = 10.40, 95% CI 1.01-107), autoimmune hemolytic anemia (aHR = 21.54, 95% CI 2.06-14.8), Hashmoto thyroiditis (aHR = 5.52, 95% CI 1.34-34.7), ankylosing spondylitis (aHR = 2.73, 95% CI 1.09-6.84), rheumatoid arthritis (aHR = 5.25, 95% CI 1.79-15.47), primary Sjogren syndrome (pSS) (aHR = 3.77, 95% CI 1.30-11.0), and systemic lupus erythemtoasus (aHR = 10.40). Tx-nonMG as compared with general population, aHR were higher for incident autoimmune hemolytic anemia (aHR = 25.50), Hashmoto thyroiditis (aHR = 6.75) and systemic lupus erythematosus (SLE) (aHR = 13.38). NonTx-MG as compared with general population, aHR were higher for incident Hashmoto thyroiditis (aHR = 6.57), pSS (aHR = 4.50), SLE (aHR = 17.29), and systemic vasculitis (aHR = 25.86). INTERPRETATION: In conclusion, based on a retrospective cohort study throughout Taiwan, patients with Tx have a higher risk of new onset ADs than patients without Tx.

[Thymectomy in thymomatous and non-thymomatous myasthenia gravis: analysis of a cohort of 46 patients]. / Timectomía en miastenia grave timomatosa y no timomatosa: análisis de una cohorte de 46 pacientes.

INTRODUCTION: Factors predicting remission after thymectomy for myasthenia gravis are not well known. AIM: To analyze the clinical evolution of the patients after this intervention and discuss about predictors of response. PATIENTS AND METHODS: We retrospectively reviewed all clinical data of thymectomies in myasthenia gravis patients performed at our hospital between 2006 from 2016. Using the MGFA-PIS classification, «complete stable remission¼, «pharmacologic remission¼, «minimal manifestations¼ and «improved¼ were defined as «good clinical outcome¼, and «unchanged¼, «worse¼, «exacerbation¼ or «died¼, as «poor clinical outcome¼. RESULTS: In 46 consecutive thymectomies for myasthenia gravis, women comprised 71.7%. Median age was 37 years and 10.9% had concomitant autoimmune disorders associated. Thymoma (23.96%) was more frequent in older patients (53 ± 20 vs 33 ± 24 years) and men (54.5% vs 18.8%). A year after thymectomy, 28.2% of patients were in poor clinical outcome group and 54.3% had good clinical outcome. On univariate analysis, thymomatous myasthenia was associated with poor clinical outcome a year after surgical intervention. After ten years of follow-up, 9.8% reached complete stable remission, a total of 32 patients (78%) had a favourable outcome and thymoma was not correlated. CONCLUSION: Thymectomy is considered an effective treatment for myasthenia gravis but the benefit is not immediate. The presence of thymoma may determine a worse initial clinical response following thymectomy in patients with myasthenia gravis.

TITLE: Timectomía en miastenia grave timomatosa y no timomatosa: análisis de una cohorte de 46 pacientes.Introducción. En la actualidad, los factores predictores de remisión de la enfermedad en la miastenia grave tras una timectomía no están claramente establecidos. Objetivo. Analizar la evolución clínica de los pacientes tras esta intervención y abordar los posibles determinantes pronósticos. Pacientes y métodos. Se analizaron retrospectivamente los registros de pacientes con miastenia grave timectomizados en nuestro centro entre 2006 y 2016. Se utilizó la escala Miasthenya Gravis Foundation of America-Post Intervention Status agrupando las categorías «remisión completa estable¼, «remisión farmacológica¼, «manifestaciones mínimas¼ y «mejoría¼ como «buen resultado clínico¼, y las categorías «sin cambios¼, «empeoramiento¼, «exacerbación¼ y «muerte¼, como «mal resultado clínico¼. Resultados. Se analizaron 46 timectomías de pacientes con miastenia grave, un 71,7% mujeres. La mediana de edad era de 37 años y el 10,9% asociaba enfermedades autoinmunes. El timoma (23,9%) fue más frecuentes en los varones (54,5% frente a 18,8%) y a mayor edad (53 ± 20 frente a 33 ± 24 años). Un año después de la timectomía, el 28,2% se encontraba en el grupo de mal resultado clínico, y un 54,3%, en el de buen resultado clínico. En el análisis univariante, el timoma se asoció a peor resultado clínico al año de la intervención. Tras diez años de seguimiento, 32 pacientes (78%) alcanzaron un buen resultado clínico, un 9,8% en remisión completa estable, y el timoma no se correlacionó como factor de mal pronóstico. Conclusión. La timectomía se considera un tratamiento efectivo, pero sin beneficio inmediato. La presencia de timoma podría determinar una respuesta clínica inicial peor tras la realización de una timectomía en pacientes con miastenia grave.

Depression in myasthenia gravis: a heterogeneous and intriguing entity.

BACKGROUND: Depressive symptoms in myasthenia gravis (MG) are common, may mimic other disease features, and contribute to misdiagnosis and diagnostic delay. Nevertheless, the clinical determinants of depressive symptoms in MG remain poorly studied, in particular their overlap with fatigue. Moreover, studies in MG have rarely looked at distinct depression phenotypes. METHODS: In 68 consecutive MG patients, we ascertained cognitive-affective and somatic depression with the Beck Depression Inventory (BDI), and also assessed age at disease onset, education, marital state, work ability, sleepiness, fatigue, and treatment modalities. Disease severity was graded according to the Myasthenia Gravis Foundation of America (MGFA) classification. RESULTS: The prevalence of moderate-severe depression was 20.5%. While depression and fatigue showed large overlap (n = 37, 54.4%), only fatigue increased with disease severity, while BDI scores did not. Thymectomy was independently associated with lower BDI scores, but had no impact on fatigue. Total BDI scores were similar in patients with predominantly cognitive-affective and with predominantly somatic depression. However, ESS correlated only with cognitive-affective BDI, and younger age was independently associated with cognitive-affective BDI. Conversely, female sex and thymectomy were independently associated with somatic BDI. CONCLUSIONS: Depression and fatigue are highly prevalent and largely overlapping comorbidities in MG, but only fatigue increased with disease severity, and only depression was milder in thymectomized patients. Comparative use of BDI subscales in MG reveals distinct depression phenotypes with distinct correlations to other disease features.

[Clinical course of myasthenia gravis at the department of neurology in Antananarivo Madagascar]. / Evolution clinique de la myasthénie auto-immune en Neurologie à Antananarivo, Madagascar.

INTRODUCTION: myasthenia gravis (MG) is a rare disease affecting the neuromuscular junction. It can lead to a life-threatening condition, especially when it is associated with respiratory failure. Full remission is possible with treatment. Our aims are to describe the clinical course of patients with MG with under treatment, in order to further improve management and to implement a database of patients living in Madagascar. METHODS: we conducted a descriptive retrospective study in the Department of Neurology at the Befelatanana Antananarivo University Hospital Center between January 2010 and December 2017. This study involved all patients diagnosed with MG based on positive prostigmin test. RESULTS: among the 5814 hospitalized patients, 25 (0,42%) were included. Only 16 patients were on follow-up (64%) of whom 14 were receiving medical treatment. Three of them had undergone thymectomy. The mean follow-up period was 24 months. Among treated patients, 8 improved and 2 died. Among thymectomized patients, 2 improved and 1 died. CONCLUSION: myasthenia gravis is a rare but serious disease. Improvement is possible with treatment even in the absence of adequate technical equipment for its management in Madagascar. Currently, a campaign in partnership with Myasthenia Gravis Association in Madagascar is underway for breathing devices useful especially when myasthenic crises occur.

Epidemiology, symptomatology and treatment of patients with myasthenia gravis in an Australian hospital.

Myasthenia gravis (MG) is a disorder affecting neuromuscular transmission with heterogeneous manifestations and treatments. This study describes clinical features and management of MG patients at a metropolitan hospital in Australia. Overall findings were consistent with previously published data. However, frequency of intravenous immuno-globulin use was higher, reasons for which are explored. Management is best conducted through specialist clinics with necessary expertise and standardised treatment protocols.

Refractory myasthenia gravis: Characteristics of a portuguese cohort.

INTRODUCTION: Some myasthenia gravis (MG) patients are refractory to conventional treatments. METHODS: To describe the clinical features of refractory MG (RMG) and explore the association with human leukocyte antigen HLA-DRB1 alleles, a cohort study of 114 consecutive MG patients was performed. Patients were classified as RMG based on predefined criteria. RESULTS: Twenty-two patients were found to have RMG (19.3%). There were no differences between non-RMG and RMG patients with respect to sex, age of onset, abnormal 3-Hz repetitive nerve stimulation, anti-acetylcholine receptor antibody positivity, thymectomy, thymoma or thymic hyperplasia, and polyautoimmunity. HLA-DRB1*03 was more frequent in the non-RMG vs. control population (P = 3 × 10-6 ). The HLA-DRB1*13 allele was less frequent in non-RMG patients compared with controls (P = 0.002), and less frequent in the non-RMG group compared with the RMG group (P = 0.003). DISCUSSION: HLA-DRB1*03 was more common in non-RMG, and the HLA-DRB1*13 allele appeared to have a protective role, as reported previously in other autoimmune disorders. Muscle Nerve 60: 188-191, 2019.

Effect of clinicopathologic features on survival of patients with thymic carcinomas and thymic neuroendocrine tumors: A population-based analysis.

BACKGROUND: Thymic carcinomas (TCs) and thymic neuroendocrine tumors (TNETs) are aggressive cancers with poor survival outcome and limited investigation. This study is to investigate clinicopathologic features on TC and TNET patients' prognosis of a large cohort. MATERIALS AND METHODS: The Surveillance, Epidemiology, and End Results database were used to identify a total of 362 TC and TNET patients with documented clinicopathologic features we investigated. The characteristics and overall survival of the TC and TNET patients were studied. RESULTS: Two hundred and forty TC and 122 TNET patients were identified. For the entire cohort of TC and TNET, histologic type (P < 0.001), tumor size (P = 0.015), Masaoka-Koga stage (P = 0.008), regional node positive (P = 0.004), surgery of primary site (P < 0.001), lymph node surgery (P = 0.013), and chemotherapy (P = 0.001) were considered as significant clinicopathologic features that could affect prognosis of TC and TNET patients in univariate analysis. More importantly, histologic type (P < 0.001), regional nodes positive (P = 0.03) and surgery of primary site (P < 0.001) were able to independently predict overall survival of those patients. In addition, for the cohort of TC, we found that regional nodes positive (P = 0.034) and surgery of primary site (P = 0.001) could be independent predictors of TC patients' survival. CONCLUSION: Regional nodes detection is essential for TC and TNET patients. Surgery of primary site is the preferred primary treatment for those patients.

The devil is in the details: Assessing treatment and outcomes of 6,795 patients undergoing remedial parathyroidectomy in the Collaborative Endocrine Surgery Quality Improvement Program.

BACKGROUND: Multi-institutional data describing remedial parathyroidectomy compared with index parathyroidectomy are scarce. METHODS: Using data in the Collaborative Endocrine Surgery Quality Improvement Program (2014-2017), baseline characteristics and outcomes of patients undergoing remedial parathyroidectomy versus index parathyroidectomy were examined using bivariate and multivariate methods. Rates of hypercalcemia and hypocalcemia at ≥ 180 days were assessed. RESULTS: Among 6,795 patients, 367 (5.4%) underwent remedial parathyroidectomy. A single localization study was done in 24.8% versus 26.9% of remedial parathyroidectomy versus index parathyroidectomy (P = .37). Patients undergoing remedial parathyroidectomy had higher rates of preoperative laryngoscopy (45.5% versus 6.2%, P < .001), intraoperative nerve monitoring (57.5% versus 34.5%, P < .001), and < 50% drop in hyperparathyroidism than those undergoing index parathyroidectomy (9.6% versus 3.3%, P < .001). Among patients with ≥ 180 days follow-up, none of the remedial parathyroidectomy versus three index parathyroidectomy patients (0.3%) had vocal cord dysfunction. Hypercalcemia rates for remedial parathyroidectomy and index parathyroidectomy were 10.5% versus 5.0 (P = .07), and hypocalcemia rates were 10.5% versus 2.4% (P < .001). After multivariate adjustment, failure to cure was 4.0 times more likely in remedial parathyroidectomy than index parathyroidectomy (P < .001). CONCLUSION: This is the first multi-institutional examination of remedial parathyroidectomy outcomes in the Collaborative Endocrine Surgery Quality Improvement Program. Nerve injury rates are low; high rates of hypercalcemia and hypocalcemia suggest potential opportunities to refine the preoperative and intraoperative management of patients undergoing remedial parathyroidectomy.

Comparing robotic and trans-sternal thymectomy for early-stage thymoma: a propensity score-matching study.

OBJECTIVES: Minimally invasive techniques seem to be promising alternatives to open approaches in the surgical treatment of early-stage thymoma, although there are controversies because of lack of data on long-term results. The aim of the study was to evaluate the surgical and oncological results after robotic thymectomy for early-stage thymoma compared to median sternotomy. METHODS: Between 1982 and 2017, 164 patients with early-stage thymoma (Masaoka I and II) were operated on by median sternotomy (108 patients) or the robotic approach (56 patients). Duration of surgery, amount of blood loss, complications, duration of chest drainage, postoperative hospital stay, oncological results and total costs were retrospectively evaluated. Data were analysed also after propensity score matching. RESULTS: Compared to the trans-sternal group, robotic thymectomy had significantly longer average operative times (P < 0.001) but less intraoperative blood loss (P = 0.01), less perioperative complications (P = 0.03), shorter time to chest drainage removal and hospital discharge (P < 0.001). The median expense for the trans-sternal approach was significantly higher than the cost of the robotic procedure (P < 0.001), mainly due to longer hospitalization. From an oncological point of view, there were no differences in thymoma recurrence, although follow-up of the trans-sternal group was significantly longer (P < 0.001). Data were confirmed after propensity score matching. CONCLUSIONS: Robotic thymectomy for early-stage thymoma is a technically safe and feasible procedure with low complication rate and shorter hospital stay compared to the trans-sternal approach. Cost analysis revealed lower expenses for the robotic procedure due to the reduced hospital stay. The oncological outcomes seemed comparable, but longer follow-up is needed.

[Which surgery for mediastinum tumor: Experience of the Department of thoracic surgery of CHU Hassan II of Fès]. / Quelle chirurgie pour quelle tumeur du médiastin : expérience du service de chirurgie thoracique de CHU Hassan II de Fès.

INTRODUCTION: Tumors of the mediastinum are a heterogeneous group of dysembryoplatic and neoplastic diseases essentially with different prognoses and therapeutic. These tumors develop slowly and remain long asymptomatic in 40-50% of cases. The purpose of our work is to bring the result of surgical management in diagnostic and therapeutic of principal mediastinum tumors framework. PATIENTS AND METHOD: We reviewed retrospectively the records of 68 patients in our training, between January 2009 and December 2013, for tumor of the mediastinum in the diagnostic framework and or therapy. RESULTS: There were 37 men and 31 women with a mean age of 37 years with extremes ranging from 11 to 73 years and 77.94% had an age between 11 and 50. In 39 patients, surgery had a diagnostic purpose (2 benign tumors and 37 malignancies including 27 cases of lymphomas). Curative surgery was performed in 34 patients, dominated by the tumors of thymic origin in 15 cases. Conventional surgery had involved 32 patients. The surgical approach was a total vertical sternotomy in 14 patients, in 17 patients was posterolateral thoracotomy and a left anterior thoracotomy in 1 patient. Video assisted thoracic surgery had been done in 3 patients under resection of a pleuropericardique cyst. Overall mortality was 4.41 percent. It is a death at D17 of the postoperative (thymoma with myasthenia) following a myasthenic crisis requiring a tracheotomy. A patient operated on for invasive thymoma developed myopathy and died at D44 of the postoperative following a difficulty of weaning. Another patient had a thymoma B3 benefited from 6 courses of neoadjuvant chemotherapy and then a thymectomy had presented a respiratory distress with bilateral pleural effusion, death at D10 of the postoperative by septic shock following a nosocomial infection. CONCLUSION: Tumors of the mediastinum are infrequent, discovered more often by chance. The main prognostic factor is the completeness of tumor resection without taking the break. Conventional surgery always keeps a place in our context, despite the advent of minimally invasive surgery.

Video-Assisted Thoracoscopic Versus Robotic-Assisted Thoracoscopic Thymectomy: Systematic Review and Meta-analysis.

OBJECTIVE: Minimally invasive thoracic surgical procedures, performed with or without the assistance of a robot, have gained popularity over the last decade. They have increasingly become the choice of intervention for a number of thoracic surgical operations. Minimally invasive surgery decreases postoperative pain, hospital stay and leads to a faster recovery in comparison with conventional open methods. Minimally invasive techniques to perform a thymectomy include video-assisted thoracoscopic surgery (VATS) or robotic-assisted thoracoscopic surgery (RATS). In this study, we aim to systematically review and interrogate the literature on minimally invasive thymectomy and draw a meta-analysis on the outcomes between the two approaches. METHODS: An extensive electronic health database search was performed on all articles published from inception to May 2015 for studies describing outcomes in VATS and RATS thymectomy. RESULTS: A total of 350 patients were included in this study, for which 182 and 168 patients underwent RATS and VATS thymectomy, respectively. There were no recorded in-hospital deaths for either procedure. There was no statistical difference in conversion to open, length of hospital stay, or postoperative pneumonia. Operational times for RATS thymectomy were longer. CONCLUSIONS: The VATS and RATS thymectomy offer good and safe operative and perioperative outcomes. There is little difference between the two groups. However, there is poor evidence basis for the long-term outcomes in minimally invasive procedures for thymectomy. It is imperative that future studies evaluate oncological outcomes both short and long term as well as those related to safety.

Thymectomy is safe for myasthenia gravis patients: Analysis of the NSQIP database.

INTRODUCTION: The aim of this study was to determine thymectomy-associated morbidity and mortality outcomes among myasthenia gravis (MG) patients. METHODS: Patients undergoing thymectomy were identified from the National Surgical Quality Improvement Program database from 2005 to 2012 using Current Procedural Terminology codes. Patient demographics, clinical characteristics, and postoperative outcomes were stratified by MG diagnosis. Logistic regression was used to identify predictors for morbidity outcomes. The chi-square test was used to examine the association between MG diagnosis and surgical approach. RESULTS: A total of 1,148 subjects underwent thymectomy, and 108 had MG. Compared with control subjects, MG patients had a more severe American Society of Anesthesiologists physical classification and frailty index score, greater corticosteroid usage, and a higher rate of reintubation. There were no deaths among MG patients. The majority of MG patients underwent transsternal thymectomy. CONCLUSION: Although patients with MG have a greater preoperative morbidity and a higher frequency of reintubation, thymectomy was found to be a safe procedure overall.

High rate of unnecessary thymectomy and its cause. Can computed tomography distinguish thymoma, lymphoma, thymic hyperplasia, and thymic cysts?

PURPOSE: To determine the non-therapeutic thymectomy rate in a recent six-year consecutive thymectomy cohort, the etiology of these unnecessary thymectomies, and the differentiating CT features of thymoma, lymphoma, thymic hyperplasia, and thymic cysts. MATERIALS AND METHODS: Electronic data base query of all thymectomies performed at the Massachusetts General Hospital from 2006 to 2012 yielded 160 thymectomy cases, 124 of which had available imaging. The non-therapeutic thymectomy rate (includes thymectomy for lymphoma and benign disease) was calculated. Preoperative clinical and CT imaging features were assessed by review of the in-house electronic medical record by 2 thoracic surgeons and 2 pathology-blinded radiologists, respectively. RESULTS: The non-therapeutic thymectomy rate of 43.8% (70/160) was largely secondary to concern for thymoma and was comprised of lymphoma (54.3%, 38/70), thymic bed cysts (24.3%, 17/70), thymic hyperplasia (17.1%, 12/70), and reactive or atrophic tissue (4.3%, 3/70). Among these four lesions, there were significant differences in location with respect to midline, morphology, circumscription, homogeneity of attenuation, fatty intercalation, coexistent lymphadenopathy, overt pericardial invasion, and mass effect (p<0.001). True thymic cysts ranged in attenuation from -20 to 58Hounsfield units (HU), with a mean attenuation of 23HU. CONCLUSION: The high rate of unnecessary thymectomy was due to misinterpretation of thymic cysts, thymic hyperplasia, and lymphoma as thymoma on chest CT. This study demonstrates differentiating features between thymoma, lymphoma, thymic hyperplasia, and thymic cysts on chest CT which may help triage more patients away from thymectomy toward less invasive and non-invasive means of diagnosis and thereby lower the non-therapeutic thymectomy rate.

Extended transcervical thymectomy with partial upper sternotomy: results in non-thymomatous patients with myasthenia gravis.

OBJECTIVES: Thymectomy is a recognized treatment for myasthenia gravis (MG), but the optimal surgical approach is yet to be determined. This study analysed the results in non-thymomatous MG patients treated at our institution using an extended transcervical access with partial upper sternotomy (TC-US), in order to describe cumulative incidence of remission and its predictors. METHODS: In the period 1988-2012, 215 non-thymomatous MG patients underwent thymectomy using the TC-US approach. There were 61 males and 154 females (median age: 33 years). Primary end points were complete stable remission (CSR) and pharmacological remission (PR). Clinico-pathological predictors of CSR/PR were analysed including age, gender, preoperative MG symptom duration, preoperative immunosuppression therapy and disease severity. RESULTS: The median follow-up period was 127 months. The median preoperative duration of MG symptoms was 9 months (interquartile range 4-13). The median operative time was 65 min (range: 45-135). There was no postoperative death. Morbidity rate was 7% (14 patients, no major complication). Ten patients died at the follow-up (3 of MG). MG symptoms improved in 85% (150/176) of the patients. CSR rate was 34%, PR rate was 4%. Cumulative incidence of CSR/PR was 27, 37 and 46% at 5, 10 and 15 years, respectively. Independent predictors of increased CSR/PR rate were age (P = 0.028) and MG symptom duration <6 months (P = 0.013). CONCLUSIONS: Our data suggest that in patients with non-thymomatous MG, thymectomy by TC-US has a remission rate not inferior to those reported after trans-sternal or video-assisted thoracic surgery techniques. The short duration of MG symptoms before thymectomy is a predictor of remission. The technique strikes a reasonable balance between the extent of thymic resection, operative and anaesthesia time, patient acceptance, neurological outcome and costs.

Early and mid-term outcomes of trans-sternal and video-assisted thoracoscopic surgery for thymoma.

OBJECTIVES: Video-assisted thoracoscopic surgery (VATS) for thymoma has uncertain safety and effectiveness in comparison with trans-sternal resection. This feasibility study compared short- and mid-term outcomes for patients undergoing these two procedures, highlights weaknesses in current research and makes recommendations for long-term technological evaluations in this field. METHODS: Consecutive thymoma cases between 2004 and 2010 were identified. Patients were divided into two groups according to surgical approach (Group I trans-sternal; Group II VATS) and comparisons were made between groups. The primary outcome was overall survival. Secondary outcomes included operative morbidity and mortality, hospital stay, recurrence rate and disease-free survival. RESULTS: Thirty-nine patients were included (Group I: n = 22 vs Group II: n = 17). There were no differences between groups at baseline for all measured covariates. No deaths occurred within 30 days of surgery. More patients in Group I developed complications (Group I: n = 10 vs Group II: n = 3; P = 0.093), while hospital stay was shorter in Group II (Group I: 6.4 ± 4.6 days vs Group II: 4.4 ± 1.8 days; P = 0.030). Five-year overall survival (Group I: 93.8 ± 6.1% vs Group II: 83.3 ± 11.2%; P = 0.425), 5-year disease-free survival (Group I: 71.0 ± 15.3% vs Group II: 83.3 ± 11.2%; P = 0.827) and recurrence rates at final follow-up (Group I: n = 2 vs Group II: n = 1; P = 0.363) were similar between the groups. CONCLUSION: VATS thymectomy for thymoma is feasible, safe and has comparable mid-term oncological outcomes to trans-sternal thymectomy. Future research is required to evaluate long-term oncological outcomes of VATS thymectomy for thymoma in national registries and randomized, controlled trials.

Thymectomy in myasthenia gravis: proposal for a predictive score of postoperative myasthenic crisis.

OBJECTIVES: Thymectomy plays an important role in patients with myasthenia gravis (MG). This study aimed to explore predictors of postoperative myasthenic crisis (POMC) after thymectomy and to define a predictive score of respiratory failure. METHODS: The clinical data of 177 patients with MG undergoing thymectomy from January 1995 to December 2011 were retrospectively reviewed. The following factors were analysed in relation to the occurrence of myasthenic crisis: gender, age, body mass index (BMI), anti-acetylcholine receptor-antibody level, bulbar symptoms, comorbidities, duration of symptoms, Osserman-stage, Myasthenia Gravis Foundation of America (MGFA) stage, history of myasthenic crisis, use of immoglobulins or plasmapheresis, kind of therapy, spirometric and blood gas parameters, histology, kind of surgery, non-myasthenic complications and duration of intubation. RESULTS: Twenty-two patients experienced postoperative respiratory failure after thymectomy. Univariate analysis revealed a correlation with age >60 years (odds ratio (OR) = 1.79, 95% confidence interval (CI) = 1.04-6.78; P = 0.040); Osserman-stage (IIB- OR = 5.16, 95% CI = 1.10-24.18; P = 0.037, III-IV- OR = 8.75, 95% CI = 1.53-50.05; P = 0.015); bulbar symptoms (OR = 7.42, 95% CI = 1.67-32.84; P = 0.008); BMI >28 (OR = 3.99, 95% CI = 1.58-10.03; P = 0.003); preoperative plasmapheresis (OR = 2.97, 95% CI = 1.18-14.04; P = 0.021); duration of symptoms >2 years (OR = 4.00, 95% CI = 1.09-14.762; P = 0.036); extended surgery (OR = 2.52, 95% CI = 1.02-6.22; P = 0.045); lung (OR = 4.05, 95% CI = 1.44-11.42; P = 0.008), pericardial (OR = 3.78, 95% CI = 1.45-9.82; P = 0.006) or pleural resection (OR = 3.23, 95% CI = 1.30-8.03; P = 0.012); Vital Capacity % <80% (OR = 0.20, 95% CI = 0.05-0.82; P = 0.025) and PaCO2 >40 mmHg (OR = 3.76, 95% CI = 1.12-12.68; P = 0.032). Multivariate logistic regression analysis showed that Osserman-stage (IIB- OR = 5.69, 95% CI = 1.09-29.69; P = 0.039 (III-IV- OR = 11.33, 95% CI = 1.67-76.72; P = 0.013), BMI >28 (OR = 3.65, 95% CI = 1.10-12.15; P = 0.035), history of myasthenic crisis (OR = 24.10, 95% CI = 2.34-248.04; P = 0.007), duration of symptoms >2 years (OR = 5.94, 95% CI = 1.12-31.48; P = 0.036) and lung resection (OR = 8.48, 95% CI = 2.18-32.97; P = 0.002) independently predict POMC. Excluding history of preoperative myasthenic crisis (statistically associated with Osserman-stage), we built a scoring system according to the OR of Osserman-stage (I-IIA, IIB, III-IV), BMI (<28, ≥ 28), duration of symptoms (<1, 1-2, >2 years) and association with a pulmonary resection. This model helped in creating four classes with increasing risk of respiratory failure (Group I, 6%; Group II, 10%; Group III, 25%; Group IV, 50%). CONCLUSIONS: Our model facilitates the stratification of patient risk and prediction of the occurrence of POMC. Moreover, it could help to guide the anaesthesiologist's decision on the duration of intubation. Further studies based on larger series are needed to confirm these preliminary data.

What is the prevalence of a "nontherapeutic" thymectomy?

BACKGROUND: Thymectomy is often performed on the basis of computed tomography scan findings that are suspicious for thymoma. However, the final diagnosis may be a condition such as thymic hyperplasia or lymphoma for which thymectomy is not therapeutic. The present analysis was undertaken to determine the prevalence of a "nontherapeutic" thymectomy. METHODS: The Nationwide Inpatient Sample from 2000 through 2009 was queried to identify patients who underwent a thymectomy. Only adult patients who underwent a total thymectomy without other associated procedures were analyzed. Patients with a diagnosis of myasthenia were excluded. A nontherapeutic thymectomy was defined as a patient who underwent thymectomy with an International Classification of Diseases, Ninth Revision diagnosis code of lymphoma, thymic hyperplasia, thymic cyst, or other benign diseases of the thymus. RESULTS: A total of 1,306 patients were identified. Overall, 72.2% (n = 943) of thymectomies were therapeutic and 27.8% (n = 363) were nontherapeutic. The most common diagnosis in the nontherapeutic group was thymic hyperplasia (n = 174). Mortality (0.32% versus 0%; p = 0.083) and overall complication rates (25% versus 17%; p < 0.001) were higher in the therapeutic group. Patients in the nontherapeutic group were younger (median age, 41 versus 56 years; p < 0.001) and more likely to undergo a video-assisted thoracoscopic surgery thymectomy (28% versus 19%; p = 0.085). CONCLUSIONS: In this study, 27.8% of thymectomies were nontherapeutic, and most patients underwent an open approach. The most common benign diagnosis was thymic hyperplasia, a condition for which magnetic resonance imaging has a high predictive value. Consequently, further studies are warranted to determine the optimal evaluation of patients undergoing thymectomy for presumed thymoma.

[Thymoma-associated myasthenia gravis: Clinical features and surgical results]. / La myasthénie associée aux thymomes : particularités cliniques et résultats chirurgicaux.

OBJECTIVE: The aim of this study was to compare the characteristics of myasthenic patients with and without thymoma, and the results of thymectomy in both types of patients. MATERIAL AND METHODS: A retrospective study was conducted among 66 patients who underwent thymectomy for myasthenia gravis in our department over a 10-year period (2000-2010). The surgical approach was sternotomy or anterolateral thoracotomy. Patients were divided into two groups according to the presence of thymoma: with (T-MG) and without (NT-MG) thymoma. Complete stable remission (CSR) was the primary endpoint. RESULTS: Median age was 35.09±9.89 years. The NT-MG group had 38 patients (57.57%) and the T-MG group 28 patients (42.43%). There was no difference between the two groups regarding the surgical approach (P=0.52). T-MG patients were older (40.54±15.16 vs. 31.37±9.46) (P=0.008) and predominantly male. There were more generalized forms (P=0.01) and more bulbar involvement (P=0.02) in the T-MG group. The rate of CSR at 5 years was 7% and 17% in the T-MG and NT-MG patients respectively (P=0.70). At 10 years, it was 36% and 94.73% respectively (P=0.03). CONCLUSION: Thymomatous myasthenia gravis is characterized by the severity of its clinical features. Remission rate at 10 years was significantly lower in the myasthenia with thymoma group.

Unsatisfactory outcomes in myasthenia gravis: influence by care providers.

Myasthenia gravis (MG) can be difficult to treat despite an available therapeutic armamentarium. Our aim was to analyze the factors leading to unsatisfactory outcome (UO). To this end we used the Myasthenia Gravis Foundation of America classification system. Forty one patients with autoimmune MG were followed prospectively from January 2003 to December 2007. Outcomes were assessed throughout follow-up and at a final visit. 'Unchanged', 'worse', 'exacerbation' and 'died of MG' post-intervention status were considered UOs. During follow-up, UO rates reached 54% and were related to undertreatment (41%), poor treatment compliance (23%), infections (23%), and adverse drug effects (13%). The UO rate at final study assessment was 20%. UO during follow-up was significantly (P = 0.004) predictive of UOs at final assessment. When care was provided by neuromuscular (NM) specialists, patients had significantly better follow-up scores (P = 0.01). At final assessment UO rates were 7% and significantly better in patients treated by NM specialists, compared to other physicians where UO rates reached 27%. UO was a frequent finding occurring in more than half our patients during follow-up. Nearly two-thirds of the UOs could have been prevented by appropriate therapeutic adjustments and improved compliance. The differential UO rates at follow-up, their dependency on the degree to which the management was specialized and their correlation with final outcomes suggest that specialized MG care improves outcomes.