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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.

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INTRODUCTION: Juvenile myasthenia gravis (JMG) is an autoimmune disease affecting the neuromuscular junction that appears before 19 years of age with varying degrees of weakness of different muscle groups. The main treatment is pharmacological, but thymectomy has also demonstrated to improve remission rates. OBJECTIVE: To describe the clinical characteristics and postoperative course of pediatric patients with JMG who underwent video-assisted thoracoscopic (VATS) thymectomy. Clinical Serie: Six pa tients who underwent VATS thymectomy between March 2011 and June 2019. The age range at diag nosis was between 2 and 14 years and the average age at surgery was 7 years. All patients were under treatment with pyridostigmine bromide associated with immunosuppression with corticosteroids before surgery. The interval between diagnosis and thymectomy was 21.5 months on average. VATS was performed by left approach, and there was no perioperative morbidity or mortality. The average hospital stay was 2 days. Three patients remain with no symptoms and without corticotherapy. Two patients were on corticosteroids, but in smaller doses than previous to surgery. One patient presented a crisis requiring hospitalization and ventilatory support during follow-up. CONCLUSION: VATS thy mectomy is part of the treatment for JMG. In this series, it appears as a safe approach and its results were favorable.

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Myasthenia Gravis (MG) is an autoimmune disease characterized by weakness and fatigability of skeletal muscles, with improvement following rest. It is a disease of great significance to the anesthesiologist because it affects the neuromuscular junction. Robotic thymectomy has come up in recent times due to the minimally invasive nature and its advantages. This presents a new set of challenges for the anesthesia team, and here we present the various anesthesia considerations and perioperative management in a series of 20 patients who underwent robotic thymectomy. As it is a recent upcoming procedure, there is a paucity of literature on this topic, and most of the available literature talks about One-Lung Ventilation (OLV) and thoracic epidurals. To our notice, this is the first literature without the use of OLV and thoracic epidural for the management of robotic thymectomy.

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Abstract Myasthenia Gravis (MG) is an autoimmune disease characterized by weakness and fatigability of skeletal muscles, with improvement following rest. It is a disease of great significance to the anesthesiologist because it affects the neuromuscular junction. Robotic thymectomy has come up in recent times due to the minimally invasive nature and its advantages. This presents a new set of challenges for the anesthesia team, and here we present the various anesthesia considerations and perioperative management in a series of 20 patients who underwent robotic thymectomy. As it is a recent upcoming procedure, there is a paucity of literature on this topic, and most of the available literature talks about One-Lung Ventilation (OLV) and thoracic epidurals. To our notice, this is the first literature without the use of OLV and thoracic epidural for the management of robotic thymectomy.

Resumo Miastenia Gravis (MG) é uma doença autoimune que se caracteriza por fraqueza e fadiga da musculatura esquelética, com melhora após o repouso. É uma doença de grande interesse para o anestesiologista, pois compromete a junção neuromuscular. Recentemente, a timectomia robótica tem sido empregada por apresentar as vantagens da abordagem minimamente invasiva. O procedimento introduz uma série de novos desafios para a equipe de anestesia. Relatamos aqui as várias considerações anestésicas e o cuidado perioperatório em uma série de 20 pacientes submetidos a timectomia robótica. Sendo um procedimento recente, há limitada literatura discutindo esse tópico e, além disso, a maior parte da literatura disponível concentra a atenção na Ventilação Monopulmonar (VMP) e na peridural torácica. A nosso ver, este é a primeiro relato na literatura sem o emprego de VMP e peridural torácica para o manejo da timectomia robótica.

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BACKGROUND: Juvenile myasthenia gravis (JMG) is an antibody mediated autoimmune disorder that manifests as progressive voluntary muscle weakness and fatigue. In medically refractory cases, thymectomy has been shown to abrogate symptoms and reduce glucocorticoid dependence. While transcervical or transsternal incisions have been the traditional approach, adult trends now favor thoracoscopic thymectomy. Little data exist to support this approach in children. METHODS: A retrospective review of all patients younger than 20 years of age who underwent a thymectomy for JMG at two pediatric institutions between 2001 and 2018 was performed. Children were divided into either an open (transcervical or transsternal) or thoracoscopic group and baseline characteristics, perioperative, and postoperative outcomes were compared. RESULTS: Thirty-four thymectomies were performed during the 18-year study period; 18 via an open and 16 via a thoracoscopic approach. The operative time was shorter for open procedures compared thoracoscopic ones (108 ±â€¯49 and 145 ±â€¯43 min, respectively, p = 0.025). Thoracoscopic thymectomy was associated with less intraoperative blood loss (5.5 ±â€¯6.0 vs 55 ±â€¯67 ml, p = 0.007), decreased duration of postoperative intravenous narcotic use (5.0 ±â€¯1.5 vs 20 ±â€¯23 h, p = 0.018), and a shorter length of hospitalization (1.7 ±â€¯1.0 vs 2.7 ±â€¯1.1 days, p = 0.009). No perioperative complication occurred in either group. Clinical improvement was reported in 94% of children in both groups. CONCLUSIONS: Thoracoscopic thymectomy in children is a safe and effective surgical technique for the treatment of JMG. Increased acceptance of this minimally invasive approach by children, families, and referring neurologists may enable earlier surgical intervention. TYPE OF STUDY: Clinical research paper. LEVEL OF EVIDENCE: III.

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Myasthenia gravis is an acquired autoimmune disorder of the neuromuscular junction characterized by fluctuating weakness and fatigability of skeletal muscles. The diagnosis can be established by clinical and serologic testing, with predominance of autoantibodies against the acetylcholine receptor, and Muscle-specific kinase antibodies. We report two cases of Myasthenia gravis, the first one is a 31 year old patient with a debut of the disease, mainly with bulbar symptoms, and the second one is a 29 year old patient diagnosed with generalized Miasthenia Gravis also mainly with bulbar symptoms with worsening of symptomatology. In this report treatments alternatives and management approaches are discused

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Juvenile myasthenia gravis is a rare autoimmune disease, which has made it difficult to collect data from prospective randomized controlled trials to evaluate the efficacy and results of different treatments. Although there are differences between the juvenile myasthenia gravis and that of the adult, the data provided by some researches in adults in the treatment of juvenile myasthenia gravis have been used. The different therapeutic options will be evaluated, with the different evidences that sustain it and a treatment algorithm will be elaborated keeping always in mind that each patient offers us different challenges.

La miastenia gravis juvenil es una enfermedad autoimmune poco frecuente, por lo que ha sido difícil recopilar datos de estudios controlados aleatorizados prospectivos para evaluar la eficacia y los resultados de distintos tratamientos. Si bien hay diferencias entre la miastenia gravis juvenil y la del adulto, se han utilizado los datos aportados por algunas investigaciones en adultos en el tratamiento de la miastenia gravis juvenil. Se evaluarán las distintas opciones terapéuticas, con las distintas evidencias que lo sostienen y se elaborará un algoritmo de tratamiento teniendo siempre presente que cada paciente nos ofrece distintos desafíos.

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La miastenia gravis juvenil es una enfermedad autoimmune poco frecuente, por lo que ha sido difícil recopilar datos de estudios controlados aleatorizados prospectivos para evaluar la eficacia y los resultados de distintos tratamientos. Si bien hay diferencias entre la miastenia gravis juvenil y la del adulto, se han utilizado los datos aportados por algunas investigaciones en adultos en el tratamiento de la miastenia gravis juvenil. Se evaluarán las distintas opciones terapéuticas, con las distintas evidencias que lo sostienen y se elaborará un algoritmo de tratamiento teniendo siempre presente que cada paciente nos ofrece distintos desafíos.

Juvenile myasthenia gravis is a rare autoimmune disease, which has made it difficult to collect data from prospective randomized controlled trials to evaluate the efficacy and results of different treatments. Although there are differences between the juvenile myasthenia gravis and that of the adult, the data provided by some researches in adults in the treatment of juvenile myasthenia gravis have been used. The different therapeutic options will be evaluated, with the different evidences that sustain it and a treatment algorithm will be elaborated keeping always in mind that each patient offers us different challenges.

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OBJECTIVES: Our goal was to evaluate, through a systematic review, the efficacy of plasmapheresis in the preoperative preparation of the patient for a thymectomy for the treatment of myasthenia gravis. METHODS: MEDLINE, Embase, LILACS, Scopus and CENTRAL databases were searched. The following outcomes were evaluated: myasthenic crisis, mortality, pneumonia, bleeding, use of mechanical ventilation, length of hospital stay and intensive care unit (ICU) stay. RevMan 5.3 software provided by the Cochrane Collaboration was used for the meta-analysis. RESULTS: The total number of patients evaluated in the 7 included studies was 360. Plasmapheresis during the preoperative period did not decrease the myasthenic crisis [risk ratio (RR) 0.36, 95% confidence interval (CI) 0.08-1.66; I2 = 44%; 5 studies, 243 patients]. There was also no change in the mortality rate (RR 0.7, 95% CI 0.11-4.62; I2 = 0%; 3 studies, 172 patients) or pneumonia cases (RR 0.28, 95% CI 0.07-1.09; I2 = 27%; 5 studies, 272 patients). Bleeding was greater in patients who underwent plasmapheresis (mean difference 34.34 ml; 95% CI 24.93-43.75; I2 = 0%). We evaluated the following outcomes: need for mechanical ventilation, hospital stay, ICU stay and mechanical ventilation, but these outcomes were not adequate to perform the meta-analysis due to the high heterogeneity among the studies. Subgroup analysis showed that plasmapheresis performed during the preoperative period in patients with severe disease (Osserman III and IV) decreased the myasthenic crisis postoperatively (RR 0.12, 95% CI 0.02-0.65; I2 = 63%). CONCLUSIONS: Plasmapheresis may reduce the myasthenic crisis during the postoperative period in patients with severe disease but may produce little or no difference in patients with mild clinical expression of the disease.

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BACKGROUND: Thymectomy improves clinical outcomes and decreases the need for medical treatment in patients with myasthenia gravis (MG). AIM: To describe the immediate and long-term results of extended transsternal thymectomy (ETT) in patients with MG. MATERIAL AND METHODS: A review of databases, surgical protocols, clinical records and interviews of patients subjected to extended transsternal thymectomy for MG between 1990 and 2016. Perioperative clinical characteristics, anticholinesterase treatment, immediate and remote surgical results were analyzed and patients were followed from one to 10years. RESULTS: We studied 58 patients aged 35 ± 14years (72%) women. In the preoperative period, according to Osserman classification, nine patients (15,5%) were in grade I, eight (13,8%) in grade IIA 8 and 40 (69%) in grade IIB. The pathological study of the surgical piece showed thymic hyperplasia in 39 cases (67,2%). Four patients had postoperative complications but none died. In the Follow-up at 1, 3, 5, 8 and 10years the Masaoka palliation rate was 71.7, 77.5, 67.7, 70.0 and 70,6% respectively. The figures for remission rate were 13.0, 15.0,19.4, 35.0 and 35,3% respectively. The figures for Zielinski positive results were 79.6, 87.5, 87.1, 90.0 and 82,4% respectively. The DeFilippi score improved by 80.4, 87.5, 87.1, 90.0 and 82.4% respectively. The Myasthenia Gravis Foundation of America Post-Intervention State improved by 67.4, 77.5, 77.5, 75.0 and 70,6% respectively. Mean Myasthenia Gravis Activities of daily living (MGADL) and Myasthenia Gravis Quality of life scale 15 (MGQOL 15) were 1.65 and 6.31 respectively. CONCLUSIONS: In selected patients with MG, extended transsternal thymectomy in MG has good immediate and long-term results.

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