RESUMEN
Modern guidelines have identified thoracoscopic thymectomy as a preferable option for myasthenia gravis and non-invasive thymoma. In the era of the new coronavirus infection, it is relevant to develop protocols for anesthetic and perioperative support of patients undergoing thymectomy for myasthenia gravis after COVID-associated pneumonia (CAP). We present the results of thoracoscopic thymectomies in patients after CAP. Multidisciplinary team should determine therapeutic support, the need for plasmapheresis and thymectomy. Plasmapheresis and glucocorticosteroids are effective in addition to anticholinesterase therapy at the stages of perioperative support for correction of neurological status in patients with myasthenia combined with chronic obstructive pulmonary disease and pulmonary hypertension. Outpatient direct anticoagulants are advisable considering the need for prolonged postoperative prevention of thrombotic events.
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COVID-19 , Miastenia Gravis , SARS-CoV-2 , Timectomía , Timoma , Neoplasias del Timo , Humanos , Miastenia Gravis/cirugía , Miastenia Gravis/diagnóstico , COVID-19/complicaciones , Timectomía/métodos , Masculino , Timoma/cirugía , Timoma/complicaciones , Femenino , Persona de Mediana Edad , Neoplasias del Timo/cirugía , Neoplasias del Timo/complicaciones , Toracoscopía/métodos , Resultado del Tratamiento , Cirugía Torácica Asistida por Video/métodos , Plasmaféresis/métodos , AdultoRESUMEN
The critical importance of the thymus for generating new naive T cells that protect against novel infections and are tolerant to self-antigens has led to a recent revival of interest in monitoring thymic function in species other than humans and mice. Nonhuman primates such as rhesus macaques (Macaca mulatta) provide particularly useful animal models for translational research in immunology. In this study, we tested the performance of a 15-marker multicolor Ab panel for flow cytometric phenotyping of lymphocyte subsets directly from rhesus whole blood, with validation by thymectomy and T cell depletion. Immunohistochemical and multiplex RNA expression analysis of thymus tissue biopsies and molecular assays on PBMCs were used to further validate thymus function. Results identify Ab panels that can accurately classify rhesus naive T cells (CD3+CD45RA+CD197+ or CD3+CD28+CD95-) and recent thymic emigrants (CD8+CD28+CD95-CD103+CD197+) using just 100 µl of whole blood and commercially available fluorescent Abs. An immunohistochemical panel reactive with pan-cytokeratin (CK), CK14, CD3, Ki-67, CCL21, and TdT provides histologic evidence of thymopoiesis from formalin-fixed, paraffin-embedded thymus tissues. Identification of mRNAs characteristic of both functioning thymic epithelial cells and developing thymocytes and/or molecular detection of products of TCR gene rearrangement provide additional complementary methods to evaluate thymopoiesis, without requiring specific Abs. Combinations of multiparameter flow cytometry, immunohistochemistry, multiplex gene expression, and TCR excision circle assays can comprehensively evaluate thymus function in rhesus macaques while requiring only minimal amounts of peripheral blood or biopsied thymus tissue.
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Citometría de Flujo , Macaca mulatta , Timo , Animales , Timo/inmunología , Timo/metabolismo , Timo/citología , Inmunohistoquímica , Inmunofenotipificación , Masculino , Femenino , Subgrupos de Linfocitos T/inmunología , Subgrupos de Linfocitos T/metabolismo , Linfocitos T/inmunología , Linfocitos T/metabolismo , TimectomíaRESUMEN
BACKGROUND: Myasthenia gravis (MG) is the most common paraneoplastic disorder associated with thymic neoplasms. MG can develop after thymectomy, and this condition is referred to post-thymectomy myasthenia gravis (PTMG). Diffuse panbronchiolitis (DPB), is a rare form of bronchiolitis and is largely restricted to East Asia, has been reported in association with thymic neoplasms. Only three cases of combined MG and DPB have been reported in the literature. CASE PRESENTATION: A 45-year-old Taiwanese woman presented to our hospital with productive cough, rhinorrhea, anosmia, ear fullness, shortness of breath, and weight loss. She had a history of thymoma, and she underwent thymectomy with adjuvant radiotherapy 7 years ago. Chest computed tomography scan revealed diffuse bronchitis and bronchiolitis. DPB was confirmed after video-assisted thoracoscopic surgery lung biopsy, and repeated sputum cultures grew Pseudomonas aeruginosa. She has been on long-term oral azithromycin therapy thereafter. Intravenous antipseudomonal antibiotics, inhaled amikacin, as well as oral levofloxacin were administered. Three months after DPB diagnosis, she developed ptosis, muscle weakness, and hypercapnia requiring the use of noninvasive positive pressure ventilation. MG was diagnosed based on the acetylcholine receptor antibody and repetitive stimulation test results. Her muscle weakness gradually improved after pyridostigmine and corticosteroid therapies. Oral corticosteroids could be tapered off ten months after the diagnosis of MG. She is currently maintained on azithromycin, pyridostigmine, and inhaled amikacin therapies, with intravenous antibiotics administered occasionally during hospitalizations for respiratory infections. CONCLUSIONS: To our knowledge, this might be the first case report of sequential development of DPB followed by PTMG. The coexistence of these two disorders poses a therapeutic challenge for balancing infection control for DPB and immunosuppressant therapies for MG.
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Bronquiolitis , Miastenia Gravis , Timectomía , Neoplasias del Timo , Humanos , Femenino , Miastenia Gravis/etiología , Persona de Mediana Edad , Bronquiolitis/etiología , Timectomía/efectos adversos , Neoplasias del Timo/cirugía , Neoplasias del Timo/complicaciones , Tomografía Computarizada por Rayos X , Infecciones por Haemophilus/etiología , Infecciones por Haemophilus/diagnóstico , Timoma/cirugía , Antibacterianos/uso terapéutico , TaiwánRESUMEN
Myasthenia gravis (MG) is an autoimmune disease characterized by muscle fatigability due to acetylcholine receptor (AChR) autoantibodies. To better characterize juvenile MG (JMG), we analyzed 85 pre- and 132 post-pubescent JMG (with a cutoff age of 13) compared to 721 adult MG patients under 40 years old using a French database. Clinical data, anti-AChR antibody titers, thymectomy, and thymic histology were analyzed. The proportion of females was higher in each subgroup. No significant difference in the anti-AChR titers was observed. Interestingly, the proportion of AChR+ MG patients was notably lower among adult MG patients aged between 30 and 40 years, at 69.7%, compared to over 82.4% in the other subgroups. Thymic histological data were examined in patients who underwent thymectomy during the year of MG onset. Notably, in pre-JMG, the percentage of thymectomized patients was significantly lower (32.9% compared to more than 42.5% in other subgroups), and the delay to thymectomy was twice as long. We found a positive correlation between anti-AChR antibodies and germinal center grade across patient categories. Additionally, only females, particularly post-JMG patients, exhibited the highest rates of lymphofollicular hyperplasia (95% of cases) and germinal center grade. These findings reveal distinct patterns in JMG patients, particularly regarding thymic follicular hyperplasia, which appears to be exacerbated in females after puberty.
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Autoanticuerpos , Miastenia Gravis , Receptores Colinérgicos , Timectomía , Timo , Humanos , Miastenia Gravis/patología , Miastenia Gravis/epidemiología , Femenino , Masculino , Adulto , Francia/epidemiología , Timo/patología , Timo/cirugía , Adolescente , Autoanticuerpos/inmunología , Autoanticuerpos/sangre , Receptores Colinérgicos/inmunología , Adulto Joven , Niño , Estudios de Cohortes , Centro Germinal/patología , Centro Germinal/inmunologíaRESUMEN
Myasthenia gravis is a well-understood autoimmune disease of the neuromuscular synapse that is medicinally treatable with favorable results and therefore should not be overlooked in the differential diagnostic evaluation of vertical diplopia. Myasthenia is primarily a clinical diagnosis. Positive indications include double vision of fluctuating severity, diurnal variations, double vision after lengthy gaze fixation on a distant object and in the primary position as well as diplopia in various visual directions, often associated with a varying extent of ptosis. Clinical tests are the Simpson test, the ice on eyes test and the probatory administration of pyridostigmine. Positive results corroborate this diagnosis but negative results do not exclude myasthenia. The same applies for the determination of specific autoantibodies. In addition to ocular symptoms it is important to search for generalized symptoms and bulbopharyngeal symptoms in particular should prompt immediate neurological diagnostics. In addition to symptomatic treatment a wide range of immunotherapeutic agents are available. Thymectomy is also used for immunomodulatory indications according to the 2023 revised guidelines. Patient-centered treatment goals, patient education and comprehensive information, also via the self-help organization German Myasthenia Society, are essential components of successful treatment of myasthenia.
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Diplopía , Miastenia Gravis , Humanos , Diplopía/etiología , Diplopía/diagnóstico , Miastenia Gravis/diagnóstico , Miastenia Gravis/terapia , Miastenia Gravis/complicaciones , Anciano , Diagnóstico Diferencial , Anciano de 80 o más Años , Timectomía , Femenino , MasculinoRESUMEN
We report a unique case of a 66-year-old man who was incidentally identified to have a mass in the thymus region by computerized tomography scan. CT revealed a well-defined 1.6 × 1 × 0.9 cm thymus mass with moderate uniform enhancement. Thoracoscopic thymectomy was performed, and the pathological diagnosis was primary glomus tumor of the thymus. There were no atypia or malignant histological features, and no primary tumors in other sites. To our knowledge, this is the first case of primary thymic glomus tumor reported in the literature.
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Tumor Glómico , Neoplasias del Timo , Tomografía Computarizada por Rayos X , Humanos , Masculino , Anciano , Tumor Glómico/cirugía , Tumor Glómico/patología , Tumor Glómico/diagnóstico , Tumor Glómico/diagnóstico por imagen , Neoplasias del Timo/cirugía , Neoplasias del Timo/patología , Neoplasias del Timo/diagnóstico , Neoplasias del Timo/diagnóstico por imagen , Timectomía , Timo/patología , ToracoscopíaRESUMEN
BACKGROUND: Thymic mucosa-associated lymphoid tissue (MALT) lymphoma is rare and is known to be associated with Sjögren's syndrome (SjS). SjS is rarely accompanied by serositis. Here, we describe the first case of postoperative cardiac tamponade and acute pleuritis in a patient with thymic MALT lymphoma associated with SjS. CASE PRESENTATION: A 33-year-old woman with SjS presented with an anterior mediastinal mass on chest computed tomography, which was performed for further examination of the condition. Suspecting a thymic MALT lymphoma or thymic epithelial tumor, total thymectomy was performed. The mediastinal mass was histopathologically diagnosed as a thymic MALT lymphoma. The patient was discharged with a good postoperative course but visited the hospital 30 days after surgery for dyspnea. Cardiac tamponade was observed and drainage was performed. Four days after pericardial drainage, chest radiography revealed massive left pleural effusion, and thoracic drainage was performed. The patient was diagnosed with serositis associated with SjS and treated with methylprednisolone, which relieved cardiac tamponade and pleuritis. CONCLUSIONS: Surgical invasion of thymic MALT lymphomas associated with SjS may cause serositis. Postoperative follow-up should be conducted, considering the possibility of cardiac tamponade or acute pleuritis due to serositis as postoperative complications.
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Taponamiento Cardíaco , Linfoma de Células B de la Zona Marginal , Pleuresia , Complicaciones Posoperatorias , Síndrome de Sjögren , Neoplasias del Timo , Humanos , Linfoma de Células B de la Zona Marginal/complicaciones , Linfoma de Células B de la Zona Marginal/cirugía , Linfoma de Células B de la Zona Marginal/patología , Femenino , Adulto , Taponamiento Cardíaco/etiología , Taponamiento Cardíaco/cirugía , Taponamiento Cardíaco/diagnóstico , Síndrome de Sjögren/complicaciones , Pleuresia/etiología , Neoplasias del Timo/cirugía , Neoplasias del Timo/complicaciones , Neoplasias del Timo/patología , Complicaciones Posoperatorias/etiología , Timectomía/efectos adversos , Pronóstico , Tomografía Computarizada por Rayos X , Enfermedad AgudaRESUMEN
BACKGROUND: Partial or complete thymectomy is routinely performed in paediatric open-heart surgeries when treating congenital heart defects. Whether or not thymectomised children require systematic immunological monitoring later in life is unknown. The objective of this study was to investigate the effects of preoperatively and postoperatively used antibiotics, hospitalisation and surgical complications on self-reported immunological vulnerability in paediatric patients with early thymectomy to better recognise the patients who could benefit from immunological follow-up in the future. METHODS: We conducted a retrospective cohort study, including 98 children and adolescents aged 1-15 years, who had undergone an open-heart surgery and thymectomy in infancy and who had previously answered a survey regarding different immune-mediated symptoms and diagnoses. We performed a comprehensive chart review of preoperative and postoperative factors from 1 year preceding and 1 year following the open-heart surgery and compared the participants who had self-reported symptoms of immunological vulnerability to those who had not. RESULTS: The median age at primary open-heart surgery and thymectomy was 19.5 days in the overall study population (60% men, n=56) and thymectomies mainly partial (80%, n=78). Broad-spectrum antibiotics were more frequently used preoperatively in participants with self-reported immunological vulnerability (OR=3.05; 95% CI 1.01 to 9.23). This group also had greater overall use of antibiotics postoperatively (OR=3.21; 95% CI 1.33 to 7.76). These findings were more pronounced in the subgroup of neonatally operated children. There was no statistically significant difference in the duration of intensive care unit stay, hospitalisation time, prevalence of severe infections, surgical complications or glucocorticoid use between the main study groups. CONCLUSION: Antimicrobial agents were more frequently used both preoperatively and postoperatively in thymectomised children with self-reported immunological vulnerability after thymectomy. Substantial use of antimicrobial agents early in life should be considered a potential risk factor for increased immunological vulnerability when evaluating the significance of immune-mediated symptom occurrence in thymectomised paediatric patients.
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Antibacterianos , Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Hospitalización , Complicaciones Posoperatorias , Autoinforme , Timectomía , Humanos , Masculino , Estudios Retrospectivos , Timectomía/efectos adversos , Femenino , Niño , Lactante , Preescolar , Adolescente , Antibacterianos/uso terapéutico , Antibacterianos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Complicaciones Posoperatorias/epidemiología , Cardiopatías Congénitas/cirugía , Hospitalización/estadística & datos numéricos , Recién NacidoRESUMEN
INTRODUCTION: Myasthenia gravis (MG) is an autoimmune, neurologic disease that causes a wide range of symptoms. While the transsternal, transcervical and thoracotomy approaches are accepted as effective, there is still debate regarding the VATS approach. MATERIALS AND METHODS: We analyzed our center's surgical experience with thymectomy for myasthenia gravis, comparing the results of patients operated on using VATS and more invasive approaches, over a period of 10 years. A search of the department's surgical database for myasthenia gravis cases between January 2010 and January 2021, revealed a total of 40 cases. Twenty-four patients were included in the final analysis and were distributed into two groups: the VATS procedure group (group A) and the open procedure group (group B). The latter included sternotomy, thoracotomy, transcervical and hemiclamshell approaches. Only radical thymectomies were included. The established outcomes were clinical improvement defined as asymptomatic remission, reduction, or discontinuation of the medication necessary to achieve optimal symptom control. RESULTS: The median follow-up time was 27 months (ranging from 4 to 75 months). Videothoracoscopy radical thymectomy was performed on 12 patients. Complete remission with no medication was achieved in 1 case (8.3%), while 2 patients (16.7%) became asymptomatic with reduced medication. An improvement (reduced symptoms or decreased medication) was observed in 8 cases (66.6%). No change in clinical outcome was noted in 1 patient (8.3%). None of the patients reported worsening symptoms. Open thymectomy was performed on 12 patients. Complete remission with no medication was achieved in 1 case (8.3%), while 2 patients (16.7%) became asymptomatic with reduced medication. An improvement was noted in 6 cases (50%). No change in clinical outcome was observed in 3 patients (25%) whereas 2 of them (16.7%) experienced slightly better symptom control but with a significant increase in medication. One patient (8.3%) described the clinical results as without any significant change. None of the patients reported worsening symptoms. CONCLUSION: The videotoracoscopic approach in the treatment of myasthenia gravis is non-inferior compared to the open approach and effective in a long-term follow-up, offering all the additional benefits of less invasive surgery.
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Miastenia Gravis , Cirugía Torácica Asistida por Video , Timectomía , Humanos , Miastenia Gravis/cirugía , Timectomía/métodos , Cirugía Torácica Asistida por Video/métodos , Cirugía Torácica Asistida por Video/efectos adversos , Masculino , Femenino , Persona de Mediana Edad , Adulto , Estudios Retrospectivos , Resultado del Tratamiento , Anciano , Toracotomía/métodos , Toracotomía/efectos adversos , Adulto JovenRESUMEN
The role of immunosenescence, particularly the natural process of thymic involution during aging, is increasingly acknowledged as a factor contributing to the development of autoimmune diseases and cancer. Recently, a concern has been raised about deleterious consequences of the surgical removal of thymic tissue, including for patients who undergo thymectomy for myasthenia gravis (MG) or resection of a thymoma. This review adopts a multidisciplinary approach to scrutinize the evidence concerning the long-term risks of cancer and autoimmunity postthymectomy. We conclude that for patients with acetylcholine receptor antibody-positive MG and those diagnosed with thymoma, the removal of the thymus offers prominent benefits that well outweigh the potential risks. However, incidental removal of thymic tissue during other thoracic surgeries should be minimized whenever feasible.
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Miastenia Gravis , Timectomía , Timoma , Timo , Neoplasias del Timo , Humanos , Timectomía/efectos adversos , Timectomía/métodos , Miastenia Gravis/cirugía , Timo/cirugía , Neoplasias del Timo/cirugía , Neoplasias del Timo/complicaciones , Timoma/cirugía , Timoma/complicaciones , Complicaciones Posoperatorias/etiología , Enfermedades Autoinmunes/cirugíaRESUMEN
Purpose: This study aimed to investigate the impact of ultrasound-guided, bilateral, low level (T8-T9) deep serratus anterior plane (DSAP) blocks on postoperative recovery quality and postoperative analgesia in patients undergoing trans-subxiphoid robotic thymectomy (TRT). Methods: 39 patients undergoing TRT were randomized to receive either low DSAP block under general anesthesia (Group S) or the sham block (Group C) on each side. The primary outcome was the QoR-40 score at postoperative day (POD) 1. Secondary outcomes included numeric rating scale (NRS) scores over time, postoperative 48 hours opioid consumption, QoR-40 scores at POD 2, 30, and 90. Results: The QoR-40 scores on POD1-2 were higher in Group S than in Group C [179.1 (4.9) vs 167.7 (2.8), P < 0.01; 187.7 (4.6) vs 178.1 (3), P < 0.01, respectively]. Pain scores were significantly lower in Group S, both during resting and motion at postoperative 6h, 12h, and 24h (P < 0.05 for each). The total amount of sufentanil consumed in the first 48 h was lower in Group S than in Group C [61.4 (4.9) vs 78.9 (4.6), P < 0.001]. Conclusion: The bilateral low DSAP blocks enhanced the QoR-40 for 2 days postoperatively, relieved postsurgical pain, and reduced opioid consumption during the early postoperative period in patients undergoing TRT.
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Bloqueo Nervioso , Dolor Postoperatorio , Procedimientos Quirúrgicos Robotizados , Timectomía , Humanos , Timectomía/métodos , Femenino , Masculino , Procedimientos Quirúrgicos Robotizados/métodos , Procedimientos Quirúrgicos Robotizados/efectos adversos , Persona de Mediana Edad , Dolor Postoperatorio/etiología , Dolor Postoperatorio/prevención & control , Dolor Postoperatorio/tratamiento farmacológico , Bloqueo Nervioso/métodos , Adulto , Analgésicos Opioides/administración & dosificación , Analgésicos Opioides/uso terapéutico , Dimensión del Dolor , Resultado del Tratamiento , Anestesia General/métodosRESUMEN
Thymic tumors are very rare neoplasms in children and account for less than 1% of mediastinal tumors in pediatric patients. One-third of the pediatric patients present with symptoms related to the compression of the tumor mass on the surrounding anatomic structures, and paraneoplastic syndromes such as myasthenia gravis, pure red cell aplasia, acquired hypogammaglobulinemia, and connective tissue disorders, which rarely occur in children with thymic tumors. Herein, we report a case of thymic carcinoma mimicking the symptoms of a connective tissue disease with symmetrical polyarthritis accompanying myositis, fever, weight loss, and malaise in a 15-year-old male patient. To our knowledge, this is the first case pediatric thymic carcinoma accompany with severe polyarthritis and myopathy, thus we have reviewed the current literature regarding the cases of thymic malignancies coexisting with paraneoplastic syndromes in children.
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Artritis , Miositis , Síndromes Paraneoplásicos , Timoma , Neoplasias del Timo , Humanos , Masculino , Miositis/diagnóstico , Miositis/complicaciones , Síndromes Paraneoplásicos/diagnóstico , Síndromes Paraneoplásicos/etiología , Neoplasias del Timo/complicaciones , Neoplasias del Timo/diagnóstico , Adolescente , Artritis/diagnóstico , Artritis/etiología , Timoma/complicaciones , Timoma/diagnóstico , Resultado del Tratamiento , Timectomía , BiopsiaRESUMEN
Thoracic surgery is increasingly influenced by the development of minimally invasive approaches which have also influenced surgery in the area of the anterior mediastinum. The previously standard approach to the thymus via partial sternotomy was gradually replaced by the videothoracoscopic approach in most cases. In recent years, robotically assisted surgery has been gaining ground worldwide in this area, as well. The aim of our paper is to provide a comprehensive overview of procedures in the field of the thymus, including their indications, and to share our first experience with robot-assisted thymus surgery. At the 3rd Department of Surgery, since the start of the robot-assisted thymus surgery program, 23 thymectomies have been performed using this approach, of which 17 were performed for thymoma, 3 for myasthenia gravis, and 3 for parathyroid adenoma localized in thymus tissue. From our experience and the available data, it follows that the length of hospitalization, the rate of complications and the resulting effect of robot-assisted procedures is comparable to VTS procedures; however, the robot-assisted surgery also allows for mini-invasive treatment even in significantly obese patients and in patients with advanced thymic tumors who would otherwise be indicated for open thymectomy.
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Miastenia Gravis , Procedimientos Quirúrgicos Robotizados , Timectomía , Timoma , Neoplasias del Timo , Humanos , Procedimientos Quirúrgicos Robotizados/métodos , Timectomía/métodos , Neoplasias del Timo/cirugía , Timoma/cirugía , Miastenia Gravis/cirugía , Neoplasias de las Paratiroides/cirugía , Timo/cirugía , MasculinoRESUMEN
BACKGROUND: Cardiac herniation occurs when there is a residual pericardial defect post thoracic surgery and is recognised as a rare but fatal complication. It confers a high mortality and requires immediate surgical correction upon recognition. We present a case of cardiac herniation occurring post thymectomy and left upper lobectomy. CASE PRESENTATION: Initial presentation: A 48-year-old male, hypertensive smoker presented with progressive breathlessness and was found to have a left upper zone mass confirmed on CT biopsy as carcinoid of unclear origin. PET-CT revealed avidity in a left anterior mediastinal area, left upper lobe (LUL) lung mass, mediastinal lymph nodes, and a right thymic satellite nodule. Intraoperatively: Access via left thoracotomy and sternotomy. The LUL tumour involved the left thymic lobe (LTL), left superior pulmonary vein (LSPV), left phrenic nerve and intervening mediastinal fat and pericardium, which were resected en-masse. The satellite nodule in the right thymic lobe (RTL) was adjacent to the junction between the left innominate vein and superior vena cava (SVC). The pericardium was resected from the SVC to the left atrial appendage. Clinical deterioration: Initially the patient was doing well clinically on day 1, however there was sudden bradycardia, hypotension, clamminess, and oligoanuria, with raised central venous pressures and troponins. ECG: no capture in leads V1-2, but positive deflections seen on posterior leads. Echo: no acoustic windows, but good windows seen posteriorly. CXR: left mediastinal shift. Redo operation: After initial resuscitation and stabilisation on the intensive care unit, on day 2 a redo-sternotomy revealed cardiac herniation into the left thoracic cavity with the left ventricular apex pointing towards the spine, and inferior caval kinking. After reduction and repair of the pericardial defect with a fenestrated GoreTex patch, the patient recovered well with complete resolution of the ECG and CXR. CONCLUSION: Cardiac herniation can even occur following sub-pneumonectomy lung resections and should be considered as a differential when faced with a sudden clinical deterioration, warranting early surgical correction.
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Deterioro Clínico , Cardiopatías , Masculino , Humanos , Persona de Mediana Edad , Timectomía/efectos adversos , Vena Cava Superior/cirugía , Tomografía Computarizada por Tomografía de Emisión de Positrones , Cardiopatías/cirugía , Hernia/etiología , Hernia/complicaciones , Neumonectomía/efectos adversosRESUMEN
BACKGROUND: Juvenile myasthenia gravis (JMG) is a rare autoimmune disease that causes fatigable muscle weakness in children aged <18 years. There is currently no curative treatment or internationally accepted standard of care for JMG. The objective is to investigate relationships between clinical presentation, antibody status, severity of disease onset, electrodiagnostic evaluation, and response to therapy in JMG. METHODS: This study was a retrospective chart review. Congenital myasthenic syndromes were excluded. Data on demographics, treatments, and outcomes were collected. Disease severity was evaluated using Myasthenia Gravis Foundation of America (MGFA) clinical classifications. RESULTS: We identified 84 patients with JMG at Children's Medical Center Dallas between January 2014 and February 2022. It was found that 52% of patients presented with ocular JMG (median onset age 4.5 years) and 48% with generalized JMG (median onset age 11.5 years); 81% tested positive for acetylcholine receptor antibodies. Patients were 17% non-Hispanic white, 29% Hispanic, 39% black, and 12% Asian. There was a significant difference in average MGFA scores between ethnicities (P = 0.047) and age groups (P = 0.004), with postpubertal patients having higher average MGFA scores than prepubertal patients. Seventy-one percent of patients who underwent thymectomy experienced a decrease in MGFA scores postprocedure. CONCLUSIONS: Our study showed that there were significant differences in disease severity between ethnicities and age groups and that most patients who underwent thymectomy showed clinical improvement. These outcomes highlight the need for additional therapies in the treatment of JMG and the importance of extending clinical trials to the pediatric population.
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Miastenia Gravis , Humanos , Miastenia Gravis/terapia , Miastenia Gravis/diagnóstico , Miastenia Gravis/fisiopatología , Niño , Masculino , Femenino , Estudios Retrospectivos , Texas , Adolescente , Preescolar , Resultado del Tratamiento , Timectomía , Índice de Severidad de la Enfermedad , Edad de InicioRESUMEN
OBJECTIVES: Robotic thymectomy has been suggested and considered technically feasible for thymic tumours. However, because of small-sample series and the lack of data on long-term results, controversies still exist on surgical and oncological results with this approach. We performed a large national multicentre study sought to evaluate the early and long-term outcomes after robot-assisted thoracoscopic thymectomy in thymic epithelial tumours. METHODS: All patients with thymic epithelial tumours operated through a robotic thoracoscopic approach between 2002 and 2022 from 15 Italian centres were enrolled. Demographic characteristics, clinical, intraoperative, postoperative, pathological and follow-up data were retrospectively collected and reviewed. RESULTS: There were 669 patients (307 men and 362 women), 312 (46.6%) of whom had associated myasthenia gravis. Complete thymectomy was performed in 657 (98%) cases and in 57 (8.5%) patients resection of other structures was necessary, with a R0 resection in all but 9 patients (98.6%). Twenty-three patients (3.4%) needed open conversion, but no perioperative mortality occurred. Fifty-one patients (7.7%) had postoperative complications. The median diameter of tumour resected was 4 cm (interquartile range 3-5.5 cm), and Masaoka stage was stage I in 39.8% of patients, stage II in 56.1%, stage III in 3.5% and stage IV in 0.6%. Thymoma was observed in 90.2% of patients while thymic carcinoma occurred in 2.8% of cases. At the end of the follow-up, only 2 patients died for tumour-related causes. Five- and ten-year recurrence rates were 7.4% and 8.3%, respectively. CONCLUSIONS: Through the largest collection of robotic thymectomy for thymic epithelial tumours we demonstrated that robot-enhanced thoracoscopic thymectomy is a technically sound and safe procedure with a low complication rate and optimal oncological outcomes.
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Procedimientos Quirúrgicos Robotizados , Timectomía , Neoplasias del Timo , Humanos , Timectomía/métodos , Neoplasias del Timo/cirugía , Masculino , Femenino , Persona de Mediana Edad , Procedimientos Quirúrgicos Robotizados/métodos , Procedimientos Quirúrgicos Robotizados/estadística & datos numéricos , Procedimientos Quirúrgicos Robotizados/efectos adversos , Estudios Retrospectivos , Anciano , Adulto , Resultado del Tratamiento , Complicaciones Posoperatorias/epidemiología , Italia/epidemiología , Neoplasias Glandulares y Epiteliales/cirugía , Neoplasias Glandulares y Epiteliales/patología , Adulto JovenRESUMEN
BACKGROUD: The standard resection for early-stage thymoma is total thymectomy and complete tumour excision with or without myasthenia gravis but the optimal surgery mode for patients with early-stage non-myasthenic thymoma is debatable. This study analysed the oncological outcomes for non-myasthenic patients with early-stage thymoma treated by thymectomy or limited resection in the long term. METHODS: Patients who had resections of thymic neoplasms at Taipei Veteran General Hospital, Taiwan between December 1997 and March 2013 were recruited, exclusive of those combined clinical evidence of myasthenia gravis were reviewed. A total of 113 patients were retrospectively reviewed with pathologic early stage (Masaoka stage I and II) thymoma who underwent limited resection or extended thymectomy to compare their long-term oncologic and surgical outcomes. RESULTS: The median observation time was 134.1 months [interquartile range (IQR) 90.7-176.1 months]. In our cohort, 52 patients underwent extended thymectomy and 61 patients underwent limited resection. Shorter duration of surgery (p < 0.001) and length of stay (p = 0.006) were demonstrated in limited resection group. Six patients experienced thymoma recurrence, two of which had combined myasthenia gravis development after recurrence. There was no significant difference (p = 0.851) in freedom-from-recurrence, with similar 10-year freedom-from-recurrence rates between the limited resection group (96.2 %) and the thymectomy group (93.2 %). Tumour-related survival was also not significantly different between groups (p = 0.726).result CONCLUSION: Patients with early-stage non-myasthenic thymoma who underwent limited resection without complete excision of the thymus achieved similar oncologic outcomes during the long-term follow-up and better peri-operative results compared to those who underwent thymectomy.
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Estadificación de Neoplasias , Timectomía , Timoma , Neoplasias del Timo , Humanos , Timectomía/métodos , Timoma/cirugía , Timoma/patología , Timoma/complicaciones , Masculino , Neoplasias del Timo/cirugía , Neoplasias del Timo/patología , Neoplasias del Timo/complicaciones , Femenino , Persona de Mediana Edad , Estudios de Seguimiento , Estudios Retrospectivos , Adulto , Anciano , Miastenia Gravis/cirugía , Tasa de Supervivencia , Recurrencia Local de Neoplasia , Tempo Operativo , Tiempo de Internación , Taiwán/epidemiología , Resultado del TratamientoRESUMEN
BACKGROUND: Current practice patterns suggest open rather than minimally invasive (MIS) approaches for thymomas >4 cm. We hypothesized there would be similar perioperative outcomes and overall survival between open and MIS approaches for large (>4 cm) thymoma resection. METHODS: The National Cancer Database was queried for patients who underwent thymectomy from 2010 to 2020. Surgical approach was characterized as either open or MIS. The primary outcome was overall survival and secondary outcomes were margin status, and length of stay (LOS). Differences between approach cohorts were compared after a 1:1 propensity match. RESULTS: Among 4121 thymectomies, 2474 (60%) were open and 1647 (40%) were MIS. Patients undergoing MIS were older, had fewer comorbidities, and had smaller tumors (median; 4.6 vs 6 cm, P < .001). In the unmatched cohort, MIS and open had similar 90-day mortality (1.1% vs 1.8%, P = .158) and rate of positive margin (25.1% vs 27.9%, P = .109). MIS thymectomy was associated with shorter LOS (2 (1-4) vs 4 (3-6) days, P < .001). Propensity matching reduced the bias between the groups. In this cohort, overall survival was similar between the groups by log-rank test (P = .462) and multivariate cox hazard analysis (HR .882, P = .472). Multivariable regression showed shorter LOS with MIS approach (Coef -1.139, P < .001), and similar odds of positive margin (OR 1.130, P = .150). DISCUSSION: MIS has equivalent oncologic benefit to open resection for large thymomas, but is associated with shorter LOS. When clinically appropriate, MIS thymectomy may be considered a safe alternative to open resection for large thymomas.
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Timectomía , Timoma , Neoplasias del Timo , Humanos , Timoma/cirugía , Timoma/mortalidad , Masculino , Femenino , Persona de Mediana Edad , Timectomía/métodos , Neoplasias del Timo/cirugía , Neoplasias del Timo/mortalidad , Neoplasias del Timo/patología , Anciano , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Tiempo de Internación/estadística & datos numéricos , Puntaje de Propensión , Estudios Retrospectivos , Adulto , Márgenes de Escisión , Resultado del TratamientoRESUMEN
OBJECTIVE: To investigate the relationship between short-term changes in quantitative myasthenia gravis score (QMGS) after thymectomy and postoperative recurrence in myasthenia gravis (MG) patients without thymoma. METHODS: A retrospective observational cohort study. The QMGS of 44 patients with non-thymomatous MG were evaluated before and 1 month after thymectomy, and the frequency and time of postoperative recurrence were recorded. The reduction rate of QMGS (rr-QMGS) was defined as (QMGS one week before thymectomy - QMGS one month after thymectomy)/ QMGS one week before thymectomy × 100 %, as an indicator of short-term symptom change after thymectomy. The receiver operating characteristic (ROC) curve was established to determine an appropriate cut-off value of rr-QMGS for distinguishing postoperative recurrence. Multivariate Cox regression analysis was applied to predict postoperative recurrence. RESULTS: Postoperative recurrence occurred in 21 patients (30 times in total) during follow-up. The mean annual recurrence rate was 3.98 times/year preoperatively and 0.30 times/year postoperatively. ROC analysis determined the cut-off value of rr-QMGS was 36.7 % (sensitivity 90.5 %, specificity 52.2 %). Multivariate Cox regression analysis showed that rr-QMGSï¼36.7 % (hazard rate[HR]6.251, P = 0.014) is positive predictor of postoperative recurrence. Kaplan-Meier analysis showed that postoperative recurrence time was earlier in the low rr-QMGS group than in the high rr-QMGS group (12.62 vs. 36.60 months, p = 0.005). CONCLUSIONS: Low rr-QMGS is associated with early postoperative recurrence. Rr-QMGS can be used to predict postoperative recurrence of non-thymomatous MG.
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Miastenia Gravis , Recurrencia , Timectomía , Humanos , Miastenia Gravis/cirugía , Miastenia Gravis/diagnóstico , Timectomía/efectos adversos , Timectomía/métodos , Masculino , Femenino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto , Anciano , Adulto Joven , Curva ROC , Estudios de SeguimientoRESUMEN
The conventional supine position for robotic thymectomy may present challenges in accessing the lower thymic horns, particularly in cases requiring extensive resection of mediastinal fat. To address this issue, the authors advocate for a lateral patient position during the procedure, emphasizing optimized access to the thymic horns and improved procedural efficacy. The lateral approach involves specific trocar placements and port arrangements to minimize conflicts between instruments. This report proposes an innovative approach to robotic thymectomy for patients diagnosed with thymoma or thymic hyperplasia associated with myasthenia gravis.