Perioperative challenges and neuromuscular blockade concerns in robotic thymectomy for myasthenia gravis / Desafios perioperatórios e pontos de atenção no bloqueio neuromuscular durante timectomia robótica para miastenia gravis

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.

Miastenia Gravis: reporte de dos casos y revisión de la literatura / Myasthenia Gravis: two case reports and review

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

Tratamiento de la miastenia gravis juvenil / Treatment of juvenile myasthenia gravis

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.

Tratamento cirúrgico da ptose palpebral moderada e grave: análise de resultados / Surgical treatment of moderate and severe ptosis: analysis of results

Introdução: A ptose palpebral é uma afecção comum na prática clínica na qual uma perfeita avaliação torna-se mandatória. Definimos ptose quando a margem palpebral encontra-se abaixo de 2 mm da junção córneo escleral e pode ser classificada em leve, moderada e grave. Existem inúmeras técnicas de reparo e a escolha dependerá da classificação da função do músculo levantador. Métodos: Foram analisados de forma prospectiva, no período de março de 2013 a maio de 2015, quatorze (n = 14) pacientes submetidos ao tratamento cirúrgico de ptose palpebral moderada e grave (n = 21). Inúmeros fatores foram estudados, tais como grau de ptose e função do músculo elevador da pálpebra, tipo de técnica de reparo, complicações imediatas e tardias, etc. Resultados: Quatorze pacientes foram opera-dos, totalizando 21 pálpebras, sendo que, 85% foram de etiologia adquirida e 15% congênita. Com relação ao grau de ptose, 64,3% (n = 9) foram moderadas e 35,7% (n = 5) graves. No que tange à função do músculo levantador, encontramos função boa 28,5% (n = 4), moderada 28,5% (n = 4) e pobre 43% (n = 6). Em relação às com-plicações, 2 casos de hiperemia conjutival e um caso de edema. Obtivemos um alto índice de satisfação com 85,7% (n = 12), com baixas taxas de complicações. Conclusão: A ptose palpebral é uma enfermidade comum na prática clínica e exige por parte do cirurgião um perfeito conhecimento anatômico da delicada estrutura palpebral e também de sua fisiopatologia. Uma perfeita avaliação desse paciente torna-se mandatória para o emprego do tratamento mais adequado.

Introduction: Eyelid ptosis is a common condition in clinical practice for which a complete evaluation is mandatory. Ptosis is defined when the eyelid margin is 2 mm below the corneoscleral junction and can be classified as mild, moderate, and severe. There are numerous repair techniques, and the choice will depend on the classification of the function of the levator muscle. Methods: We evaluated prospectively, from March 2013 to May 2015, 14 patients who underwent surgical treatment of moderate and severe ptosis (n = 21). Several factors were studied, such as degree of ptosis and function of the eyelid levator muscle, type of repair technique, and immediate and late complications. Results: Fourteen patients (21 eyelids) underwent operation. The etiology was acquired in 85% of the cases and congenital in 15%. With respect to the degree of ptosis, 64.3% (n = 9) of the cases were moderate and 35.7% (n = 5) were severe. With respect to the muscle function of the levator, good, moderate, and poor functions were observed in 28.5% (n = 4), 28.5% (n = 4), and 43% (n = 6) of the cases, respectively. With regard to complications, 2 cases of conjunctival hyperemia and one case of edema were observed. We obtained a high satisfaction rate of 85.7% (n = 12), with low complication rates. Conclusion: Eyelid ptosis is a common presentation in clinical practice and requires on the part of the surgeon a detailed anatomical knowledge of the delicate structure of the eyelid and its pathophysiology. A complete evaluation of these patients is mandatory for the employment of the most appropriate treatment.

Miastenia gravis: resultados inmediatos y alejados de la timectomía transesternal extendida / Results of extended transsternal thymectomy for Myasthenia gravis: experience with 58 patients

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.

Caracterización clínica de los pacientes miasténicos timectomizados / Clinical Characterization of Thymectomized Myasthenic Patients

Introducción: la miastenia gravis es una enfermedad crónica de la unión neuromuscular, de naturaleza autoinmune y es reconocido que la timectomía es una modalidad terapéutica efectiva en esta entidad clínica. Objetivos: realizar una caracterización clínica de los pacientes miasténicos timectomizados. Métodos: se realizó un estudio observacional descriptivo retrospectivo, en el Servicio de Neurología del Hospital Clínico Quirúrgico Hermanos Ameijeiras en el periodo comprendido de enero de 1984 a diciembre de 2014. Resultados: se reclutaron 393 pacientes, la mayoría fueron del sexo femenino y del grupo de edad de 30-39 años, predominó el grado II b (65,3 por ciento) de intensidad de la enfermedad. La hiperplasia tímica (66,9 por ciento) fue la alteración histológica del timo más común. El hipertiroidismo fue la enfermedad autoinmune más frecuente. La crisis miasténica representó la complicación posoperatoria principal y apareció esencialmente en pacientes con timoma. La mortalidad fue de 1,3 por ciento. Conclusiones: fue más común el grado II b de intensidad de la enfermedad, el tipo histológico de hiperplasia tímica y la mortalidad baja(AU)

Introduction: Myasthenia gravis is a chronic disease of the neuromuscular junction, it is autoimmune by nature and it is recognized that thymectomy is an effective therapeutic modality in this clinical entity. Objectives: Clinically characterize the myasthenic thymectomized patients. Methods: A retrospective, descriptive, observational study was conducted in the Neurology Service at Hermanos Ameijeiras Surgical Clinical Hospital from January 1984 to December 2014. Results: 393 patients were recruited; the majority was female and aged 30-39 years. 65.3 percent of them had grade II b of disease intensity. Thymic hyperplasia (66.9 percent was the most common histological alteration of the thymus. Hyperthyroidism was the most frequent autoimmune disease. The myasthenic crisis represented the main postoperative complication and it essentially showed in patients with thymoma. Mortality was 1.3 percent. Conclusions: Grade II b disease intensity was more common, the histological type of thymic hyperplasia and mortality was low(AU)

Síntomas de miastenia grave en un paciente con antecedente de timectomía por timoma invasor / Symptoms of myasthenia gravis in a patient with a history of thymectomy for invasive thymoma

Introducción. La miastenia grave es una enfermedad autoinmunitaria mediada por anticuerpos. Entre 10 y 15 % de quienes la padecen tienen timoma y su presencia se asocia con una mayor gravedad de los síntomas, crisis miasténicas y fracaso del tratamiento de primera línea. La timectomía se recomienda en pacientes jóvenes con miastenia grave generalizada y en todos los pacientes con timoma. Caso clínico. Se presenta el caso de una mujer de 43 años que, en el 2005, presentó una primera crisis miasténica asociada con un timoma invasor que se trató con timectomía y radioterapia. Durante los siguientes tres años, presentó síntomas graves y dos crisis más, que obligaron a suministrarle respiración mecánica asistida e inmunoglobulina. Al cabo del tratamiento, no se evidenciaron signos de recurrencia en las tomografías de tórax con contraste. Entre el 2009 y el 2012, la gravedad de los síntomas fue menor. En el 2013, estos se exacerbaron y una resonancia magnética de tórax con contraste reveló una lesión en el mediastino anterior, ya observada en el 2011, sugestiva de tejido residual o fibrosis. Se inició el tratamiento usual con inmunoglobulina y se hizo una tomografía por emisión de positrones cuyos resultados no fueron concluyentes, por lo que se llevó a cabo una nueva resección y se constató que no había recurrencia del tumor. Conclusiones. Los pacientes con miastenia grave y aquellos con timoma asociado, deben someterse a la timectomía como parte del tratamiento. Sin embargo, la exacerbación de los síntomas o su reaparición después del procedimiento no necesariamente implica una nueva alteración en el timo.

Introduction: Myasthenia gravis is an antibody-mediated autoimmune disease. Approximately 10-15% of patients present with a thymoma, the presence of which is associated with greater severity of symptoms, myasthenic crisis, and irresponsiveness to front-line therapy. A thymectomy is recommended in young patients with generalized myasthenia gravis and in all patients presenting with thymoma. Clinical case: The patient was a 43-year-old woman, who first showed symptoms of myasthenic crisis in 2005 and presented with invasive thymoma managed with thymectomy and radiotherapy. In the subsequent three years, the patient presented with severe symptoms and two myasthenic crises that required mechanical ventilation and immunoglobulin treatment. Contrast chest computed tomography examinations showed no recurrence. Between 2009 and 2012, the patient experienced decreased symptom severity. In 2013, the patient presented with an exacerbation of symptoms; a contrast chest magnetic resonance scan showed a lesion in the anterior mediastinum, previously observed in 2011, suggestive of residual tissue as opposed to fibrosis. Regular management was started with immunoglobulins; a positron emission tomography scan was inconclusive, requiring a new resection, which showed no evidence of tumor recurrence. Conclusions: Patients with myasthenia gravis and those with myasthenia-related thymoma both share thymectomy as an element of treatment. However, following the procedure, exacerbation or reappearance of symptoms does not necessarily represent new alterations in the thymus.

Myasthenia gravis and thymus: long-term follow-up screening of thymectomized and non-thymectomized patients / Miastenia gravis e timo: investigacao no seguimento de longo prazo em pacientes timectomizados e nao timectomizados

Thymoma screening is recommended at the onset of myasthenia gravis (MG) or when patients with MG present with clinical deterioration or a progressive increase of anti-acetylcholine receptor antibody. However, it is unknown if it is necessary to repeat the screening of thymoma at fixed intervals, even in the absence of MG deterioration, when the initial screening is negative. We analyzed the recurrence rate and incidence of new thymoma in a series of patients with well-controlled MG. The sample consisted of 53 patients, aged 17 to 72 years, and the follow-up varied between 75 and 472 months. The chest computerized tomography detected thymus abnormalities in eight patients at the initial screening and no abnormalities in all patients at a second screening after five years. The findings of this study support the classical opinion that screening for thymoma should be recommended only if there is clinical deterioration due to the disease.

A investigação de timoma é recomendada em pacientes com miastenia gravis (MG) no início da doença, em caso de haver piora clínica ou aumento dos níveis do anticorpo antirreceptor de acetilcolina. Contudo, não foi estabelecido se é necessário repetir a investigação de timoma em intervalos fixos, na ausência de piora clínica, quando a investigação inicial foi negativa. A taxa de recorrência e a incidência de novo timoma foram analisadas em uma série de pacientes com MG bem controlada. A amostra consiste de 53 pacientes, idade entre 17 e 72 anos, com tempo de acompanhamento variando entre 75 e 472 meses. A primeira tomografia computadorizada de tórax detectou anormalidades no timo em oito pacientes durante a investigação inicial da doença e nenhuma anormalidade no segundo exame, após cinco anos de doença, em todos os pacientes. Os achados desse estudo corroboram a clássica opinião de que a investigação de timoma deveria ser recomendada somente se houver piora clínica da doença.

Resultado de la timectomía en los tumores epiteliales / Result of thymectomy in epithelial tumors

Introducción: el timoma describe las neoplasias que no presentan atipia manifiesta del componente epitelial, cuando se exhibe claramente atipia citológica se le conoce como carcinoma tímico, y la cirugía es el tratamiento de elección. Nuestro objetivo es evaluar la eficacia de la timectomía en los enfermos con tumores epiteliales del timo (timoma, carcinoma tímico). Métodos: se estudiaron 26 enfermos entre enero 2007 a enero 2012 que, con este diagnostico, fueron operados en nuestro centro. Resultados: la miastenia gravis estuvo presente en 16 (61,5 por ciento) pacientes, de ellos en el posoperatorio se extubaron 11 (68,7 por ciento) después de 12 horas. La esternotomía total fue el abordaje principal 13 (50 por ciento), cuando la lesión era superior a los 7 centímetros fue más probable la ampliación a un hemitórax. Cuando se necesitó resección de pulmón, pericardio o ambos, el tiempo quirúrgico fue superior a los 120 minutos. Se complicaron 7 (29,6 por ciento), de ellos 5 (71,4 por ciento) de causa respiratoria. En 19 (73,9 por ciento) los tumores se clasificaron como estadio I de Masaoka y en 6 (23,1 por ciento) hubo carcinoma tímico.Tuvimos 1 fallecido (3,8 por ciento). Todos los miasténicos tuvieron remisión completa o farmacológica, en 2 hubo recidiva local y en ninguno fallecimiento durante el seguimiento. Conclusiones: la timectomía transesternal es el tratamiento de elección, y se necesita ampliar a un hemitórax cuando la lesión es más de 7 cm con resección de pericardio, pulmón o ambos. En este caso, se obtendrán resultados favorables en los miasténicos y en el control del tumor durante el seguimiento(AU)

Introduction: Thymomea describes the neoplasias that do not present evident atypia of the epithelial component; the clear cytological atypia indicated thymic carcinoma and surgery is the treatment of choice. The objective of this paper was to evaluate the efficacy of thymectomy aimed at patients with epithelial tumors in the thymus (thymoma, thymic carcinoma). Methods: Twenty six patients under study from January 2007 to January 2012, they were operated on after this diagnosis. Results: Myasthenia gravis was present in 16 patients (61.5 percent), 11 of them (68.7 percent) were removed their intubation after 12 hours of surgery. Total sternotomy was the main approach in 13 patients (50 percent); in case of an over 7cm long injure, the approach was extended to hemithorax. Resection of lung, pericardium or both required more than 120min surgical time. Seven patients (29.6 percent) suffered complications, 5 of them (71.4 percent) respiratory complications. Nineteen patients (73.9 percent) had Masaoka's staging I tumors whereas 6 (23.1 percent) had thymic carcinoma. One patient died (3.8 percent). All these patients with myasthenia gravis showed complete or pharmacological remission, 2 experienced local relapse and no patient died in the follow-up period. Conclusions: Transsternal thymectomy is the treatment of choice. It is required to extend it to hemithorax when there is an over 7 cm long injure, with resection of the pericardium, the lung or both. In this case, the favorable results will be achieved in myasthenic patients and in the tumor control during the follow-up phase(AU)

Resultados de la cirugía torácica mínimamente invasiva (vídeo-asistida) en el tratamiento de la Miastenia Gravis / Results of minimally invasive thoracic surgery (video-assisted) in the treatment of Myasthenia Gravis

Introduction: Myasthenia Gravis (MG) is the most common primary disorder of neuromuscular transmission. Treatment is multidisciplinary medical and surgical. Thymectomy is now part of standard treatment with clinical improvement in approximately 85 percent of cases. The best approach has not yet been defined. Objective: To evaluate the results of thymectomy for minimally invasive surgery (video-assisted) in patients with MG, with or without thymoma, analyzing the results in the literature to date. Material and Methods: We conducted a systematic review of literature in the PubMed database. Results: We found 66 publications related to the search, 20 studies were selected for review. We included those that evaluated the results of thymectomy in MG patients with or without thymoma. Discussion: Minimally invasive surgery (video-assisted) is a safe way, with low morbidity, shorter hospitalization, better cosmetic result and complete remission rates or improvement at least equivalent to conventional surgical approaches. These results should be evaluated with caution because there isn't enough evidence to ensure that minimally invasive surgery is the best approach.

Introducción: La Miastenia Gravis (MG) es el desorden primario más común de la transmisión neuro-muscular. Su tratamiento es multidisciplinario, médico y quirúrgico. La timectomía es en la actualidad parte del estándar de tratamiento con mejorías clínicas en aproximadamente el 85 por ciento de los casos. El mejor abordaje aún no se ha definido. Objetivo: Evaluar los resultados de la timectomía por cirugía mínimamente invasiva (video-asistida) en pacientes con MG, con o sin timoma, analizando los resultados en la literatura disponible hasta la actualidad. Material y Métodos: Se realizó una revisión sistemática de la literatura en la base de datos PubMed. Resultados: Se encontró 66 publicaciones relacionadas con la búsqueda, de los cuales se seleccionó 20 estudios para revisión. Se incluyó aquellos en los que se evaluó los resultados de la timectomía en pacientes con MG, con o sin timoma. Discusión: La cirugía mínimamente invasiva (video-asistida) es una vía segura, con tasas de morbilidad bajas, menor tiempo de hospitalización, buen resultado estético y tasas de remisión completa o de mejoría al parecer equivalentes a las vías de abordaje clásicas. Estos resultados deben evaluarse con cautela ya que no existe suficiente evidencia para asegurar que la cirugía mínimamente invasiva es el mejor abordaje.

Low-dose spinal anesthesia for urgent laparotomy in severe myasthenia gravis

Myasthenia gravis [MG] is an autoimmune disease with an incidence of 2-10/100,000 cases per year, characterized by muscle weakness secondary to destruction of postsynaptic acetylcholine receptors. In these patients, important perioperative issues remain unresolved, namely, optimal administration of cholinesterase inhibitors, risks of regional anesthesia, and prediction of need of postoperative mechanical ventilation. We describe the use of a low-dose spinal anesthesia in a patient with MG who was submitted for emergence exploratory laparotomy. The utilization of low-dose spinal anesthesia allowed us to perform surgery with no adverse respiratory or cardiovascular events in this patient

Timectomía videotoracoscópica en miastenia gravis: técnica quirúrgica y experiencia inicial / Thymectomy in myasthenia gravis thoracic: surgical technique and initial experience

Introducción: Desde la década del 40 se reconoce el beneficio de la timectomía en el tratamiento de la miastenia gravis. La misma se realizó tradicionalmente por esternotomía o cervicotomía. La videotoracoscopia permite la extirpación tímica con menor agresión quirúrgica y recuperación más temprana. Material y Métodos: En los últimos 4 años se realizó un total de 14 timectomía por videotoracoscopia a pacientes portadores de miastenia gravis. Los pacientes eran 11 mujeres y 3 varones, con una edad media de 34,1 años, con un rango entre 16 y 53 años. En todos los casos se realizó mediante videotoracoscopía de 3 trocares, con acceso por hemitorax derecho. Resultados: Se realizó timectomía total exclusivamente toracoscópica en todos los casos, sin conversiones ni reoperaciones. Un solo paciente requirió ARM posterior menor a 4hs de postoperatorio, en 4 pacientes se extubó en UTI durante las 2 hs de postoperatorio y los 9 restantes salieron sin tubo endotraqueal de quirófano. La mortalidad postoperatoria fue nula. Conclusiones: la timectomía videotoracoscópica es útil en el tratamiento de la miastenia gravis y tiene como ventaja una mejor recuperación del paciente, y una mejor visión quirúrgica, que redunda en menor morbimortalidad quirúrgica.

Introduction: From the 40 recognized the benefit of thymectomy in the treatment of myasthenia gravis. The same was done traditionally by sternotomy or neck incision. VATS allows removal thymic less aggressive surgery and early recovery. Material and Methods: In the last 4 years was a total of 14 video-assisted thymectomy in patients with myasthenia gravis. Patients were 11 women and 3 men, mean age of 34.1 years, ranging between 16 and 53 years. In all cases was performed by video-assisted thoracoscopy of 3 trocars, accessible by right hemithorax. Results: We performed thoracoscopic thymectomy only com­plete in all cases without conversion or reoperation. One patient required subsequent ARM less than 4 hrs after surgery in 4 patients were extubated in the ICU during the 2 hrs after surgery and the remaining 9 endotracheal tube left without surgery. Postoperative mortality was nil. Conclusions: VATS thymectomy is useful in the treatment of myasthenia gravis and has the advantage of better patient recovery, and better surgical view, resulting in less surgical morbidity.

Enfermedad de graves en asociación con miastenia gravis: presentación de un caso y revisión de la literatura / Myasthenia gravis with concomitant hyperthyroidism: report of one case

Myasthenia gravis and Graves disease are autoimmune diseases that can coexist in the same patient. We report a 18 years old female with a history of myasthenia gravis diagnosed at the age of six, treated with neostigmine and prednisone. She starts with palpitations, dyspnea, proximal muscle weakness and exophthalmos appearing seven months after the onset of symptoms. She was admitted to the hospital due to her decompensated hyperthyroidism. After admission, she develops a myasthenic crisis, that requires mechanical ventilation. Afterwards, the patient is subjected to a thymectomy and thyroidectomy with a good postoperative evolution.

Anesthesia for patients undergoing transsternal thymectomy for juvenile myasthenia gravis

Juvenile myasthenia gravis [JMG] is the rare form of myasthenia gravis presenting in childhood and adolescence. When medical management fails, thymectomy is offered for these patients. Complete resection of the thymus is best achieved through transsternal thymectomy. Anesthetic management of patients with JMG is challenging, particularly in regards to the goals of postoperative pain control, respiratory function, and extubation. We retrospectively reviewed the medical records of 13 patients, ranging in age from 6 to 22 years, who underwent transsternal thymectomy for JMG. Information on patient demographics, characteristics of their disease and treatment, anesthetic management, and postoperative course were collected. All patients had undergone multiple treatment modalities and presented for surgery because of inadequate symptom control with medical management. As expected for a pediatric population, anesthesia induction was age dependent. 40% of the patients underwent an inhalation induction and 60% underwent an intravenous induction. Anesthesia was maintained with a low-dose inhalation agent in all patients, supplemented in 84% of patients with a remifentanil infusion, and in 69% of patients with an epidural infusion. Muscle relaxants were avoided in all patients. With this regimen, 92% of patients could be extubated successfully in the operating room. We found that avoidance of muscle relaxants and use of remifentanil with a low-dose hypnotic agent provided a stable intraoperative course, facilitated rapid emergence, and allowed early extubation in patients with JMG undergoing transsternal thymectomy. Epidural analgesia reduced the need for intra- and postoperative intravenous opioids and did not have an adverse effect on respiratory strength

Anesthetic management of patient with myasthenia gravis and uncontrolled hyperthyroidism for thymectomy.

The relationship between myasthenia gravis (MG) and other autoimmune disorders like hyperthyroidism is well known. It may manifest earlier, concurrently orafter the appearance of MG. The effect of treatment of hyperthyroidism on the control of MG is variable. There may be resolution or conversely, deterioration of the symptoms also. We present a patient who was diagnosed to be hyperthyroid two and half years before the appearance of myasthenic symptoms. Pharmacotherapy for three months neither improved the myasthenic symptoms nor the thyroid function tests. Thymectomy resulted in control of MG as well as hyperthyroidism. In conclusion, effective control of hyperthyroidism in the presence of MG may be difficult. The authors opine that careful peri-operative management of thymectomy is possible in a hyperthyroid state.

The cost-effectiveness of open or thoracoscopic thymectomy compared to medical treatment in managing Myasthenia gravis without thymomas / Costo efectividad de timectomía abierta y toracoscópica frente al tratamiento médico, en el manejo de Miastenia gravis sin timoma

Objective Assessing the cost-effectiveness of open or thoracoscopic thymectomy compared to medical therapy in managing myasthenia gravis not associated with thymoma. Methods A Markov model was designed for evaluating three strategies' cost-effectiveness. Transition probabilities were taken from the pertinent literature; the costs were estimated from official tariff manuals. Incremental cost-effectiveness ratios were estimated and probabilistic and deterministic sensitivity analysis was used for clinical variables, costs and the model's assumptions. Results Thoracoscopic thymectomy was the most effective and least costly strategy and dominated the other two alternatives. The cost per life year gained was Col § 1 129 531 without discount and Col § 805 179 with discount. Univariate sensitivity analysis showed that the main variables affecting the results were the effects' discount rate, the cost of a myasthenic crisis and the probability of complete remission. Thoracoscopy thymectomy was the most cost-effective strategy for different thresholds of willingness to pay in probabilistic analysis. Conclusions Thoracoscopic thymectomy is a cost-effective strategy in the treatment of MG without thymoma.

Objetivo Evaluar la costo efectividad de las timectomía abierta y toracoscópica frente a la terapia mé,dica en el manejo de miastenia gravis sin timoma. Mé,todo Se construyó un modelo de Markov para evaluar la costo efectividad de las 3 estrategias. Las probabilidades de transición se obtuvieron de la literatura. Los costos se estimaron a partir de las tarifas oficiales. Se calculó la costo-efectividad incremental. Se realizaron análisis de sensibilidad probabilísticos y determinísticos para las variables clínicas, los costos supuestos del modelo. Resultados La timectomía toracoscópica es la estrategia más efectiva y menos costosa, y domina a las otras dos alternativas. El costo por año de vida ganado fue de § 1 129 531 y § 805 179 pesos colombianos, con y sin descuento. El análisis de sensibilidad univariado mostró que las principales variables que afectan los resultados son la tasa de descuento, el costo de una crisis miasté,nica y la probabilidad de remisión completa. En el análisis de sensibilidad probabilístico, la timectomía toracoscópica es la estrategia costo-efectiva para los diferentes umbrales de disponibilidad a pagar. Conclusiones La timectomía toracoscópica es una estrategia costo-efectiva en el tratamiento de miastenia gravis sin timoma.

Haptoglobin study in myasthenia gravis / Estudo sobre a haptoglobina na miastenia grave

OBJECTIVE: A cross-sectional study of haptoglobin (Hp) in myasthenia gravis (MG) was designed, with the objective to identify its values and correlate them with different disease status. METHOD: 46 patients were enrolled in the study, all having disease severity established according to the quantitative myasthenia gravis strength scores (QMGSS). Based on the functional scale determined by Myasthenia Gravis Foundation of America (MGFA) recommendations, patients were classified as having: complete stable remission (CSR; n=10); minimal manifestations-0 (MM0; n=6), minimal manifestations-1 (MM1; n=4); pharmacological remission (PR; n=6). Two other groups participated: thymomatous patients (T; n=10) and patients without imunosuppression or thymectomy, until the assessment for Hp (WIT; n=10). Hp dosage was done by immunonephelometry, blindly to clinical data. Student's t-test, Anova test and linear regression were employed for statistical analyses. RESULTS: Statistically significant differences occurred between CSR+MM0xWIT groups (86.62x157.57, p<0.001) and PR+MM1xWIT groups (73.93x157.57, p<0.001). Linear regression showed correlation between Hp levels and QMGSS (r=0.759, p<0.001). CONCLUSION: Our results suggest that Hp may be useful in clinical practice as a disease severity marker in MG.

OBJECTIVO: Desenhou-se estudo transversal sobre a haptoglobina (Hp) na miastenia grave (MG) com o objetivo de identificar seus valores e correlacioná-los a diferentes condições na doença. MÉTODO: 46 pacientes foram incluídos, todos tendo a gravidade da doença estabelecida segundo escores internacionais (QMGSS). Os pacientes tiveram seu estado funcional determinado de acordo com a Myasthenia Gravis Foundation of América (MGFA) e classificados em: remissão completa estável (CSR; n=10); mínima manifestação-0 (MM0; n=6), mínima manifestação-1 (MM1; n=4); remissão farmacológica (PR; n=6). Dois outros grupos participaram: pacientes timomatosos (T; n=10) e pacientes sem imunossupressão ou timectomia, até o momento da inclusão no estudo (WIT; n=10). A dosagem de Hp foi realizada por imunonefelometria, de modo cego quanto à clínica. As análises estatísticas incluíram o teste de Student, Anova e regressão linear. RESULTADOS: Observou-se diferença significativa entre os grupos CSR+MM0xWIT (86,62x157,57, p<0,001) e entre PR+MM1xWIT (73,93x157,57, p<0,001). A regressão linear mostrou correlação positiva entre os valores de Hp e os escores QMGSS (r=0,759, p<0,001). CONCLUSÃO: O estudo sugere que valores altos de Hp se correlacionaram a maior gravidade da MG.

Two lung ventilation through single lumen tracheal tube in thoracoscopic thymectomy: a randomized clinical trial of efficacy and safety

Video assisted thoracoscopic surgery [VATS] has been successfully used for thymectomy in myasthenia gravis [MG]. Thoracoscopic thymectomy [TT] is usually done under general anesthesia with double lumen tubes [DLT]. The aim of this study is evaluation of two lung ventilation through single lumen tubes [SLT] during TT. In this prospective randomized study, TT was done in 30 patients with MG from January 2004 to December 2007. Patients were randomly intubated with either a DLT or SLT, 15 patients in each group. All patients were evaluated for hemodynamie stability, oxygen saturation of hemoglobin [Spo[2]], end-tidal Pco[2] [ETPco[2]], times required for intubation and surgery, satisfaction of surgeon about exposure and postoperative complications. in the SLT group, all patients had stable hemodynamic, Spo[2] and ETPco[2]. In the DLT group, hemodynamic instability occurred in one, decrease in Spo[2] in one and increase in ETPco[2] in another patient. One patient in DLT group developed vocal cord granuloma two months later. Time required for surgery and surgeon's opinion about exposure were similar in the two groups. SLT is safe in TT. It provides good surgical exposure and decreases the cost, time and undesirable complications of DLT