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Aim: This nationwide study evaluated the clinical impact that an early thymectomy, during congenital heart defect (CHD) surgery, had on the health of children and adolescents. Methods: The subjects were patients aged 1–15 years who had undergone CHD surgery at the University Children's Hospital, Helsinki, where all CHD surgery in Finland is carried out, from 2006 to 2018. The parents or the cases and population‐based controls, matched for sex, age and hospital district, completed electronic questionnaires. We excluded those with low birth weights or a known immunodeficiency. Adjusted odds ratios (aOR) and 95% confidence intervals (CI) were calculated for prespecified outcomes. Results: We received responses relating to 260/450 (58%) cases and 1403/4500 (31%) controls and excluded 73 cases with persistent cardiac or respiratory complaints after surgery. The CHD group reported more recurrent hospitalisations due to infections (aOR 6.3, 95% CI 3.0–13) than the controls and more pneumonia episodes (aOR 3.5, 95% CI 2.1–5.6), asthma (aOR 2.5, 95% CI 1.5–4.1) and wheezing (aOR 2.1, 95% CI 1.5–2.9). Conclusion: Hospitalisation due to infections, pneumonia, wheezing and asthma was more common in children after a thymectomy due to open‐heart surgery than population‐based controls, underlining the importance of immunological follow‐ups. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

Purpose: Surgical patients with thymoma and myasthenia gravis (MG) must have their MG status and oncological outcomes critically monitored. We aimed to identify clinicopathological predictors of the postoperative MG status. Methods: We conducted a retrospective review of 40 consecutive surgical patients with MG-related thymomas between 2002 and 2020. The quantitative myasthenia gravis score (QMGS) and Myasthenia Gravis Foundation of America post-intervention status (MGFA-PIS) were used to evaluate postoperative MG status. Results: All patients underwent extended total thymectomy. The most common WHO type was type B2 (32%), while 65% of patients had type B1–B3 and 35% had type A–AB thymomas. Eleven patients (28%) achieved controlled MG status in MGFA-PIS 6 months after surgery. This controlled status was observed more frequently in type A–AB than in B1–B3 (57% vs. 12%, p = 0.007). In a multivariate analysis, WHO type (A–AB or B1–B3) was an independent predictor of worsening episodes of MG based on the QMGS (Type B1–B3, hazard ratio: 3.23, 95% confidence interval: 1.12–9.25). At the last follow-up, 23 patients (58%) achieved controlled MG status. The 5-year overall survival rate of all patients was 93.7%. Conclusion: The WHO type of thymoma is an informative predictor of postoperative MG status in patients with MG-related thymoma. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Kabuki Syndrome (KS) is a multisystemic genetic disorder. A portion of patients has immunological manifestations characterized by increased susceptibility to infections and autoimmunity. Aiming to describe the clinical and laboratory immunological aspects of KS, we conducted a retrospective multicenter observational study on patients with KS treated in centers affiliated to the Italian Primary Immunodeficiency Network. Thirty-nine patients were enrolled, with a median age at evaluation of 10 years (range: 3 m–21y). All individuals had organ malformations of variable severity. Congenital heart defect (CHD) was present in 19/39 patients (49%) and required surgical correction in 9/39 (23%), with associated thymectomy in 7/39 (18%). Autoimmune cytopenia occurred in 6/39 patients (15%) and was significantly correlated with thymectomy (p < 0.002), but not CHD. Individuals with cytopenia treated with mycophenolate as long-term immunomodulatory treatment (n = 4) showed complete response. Increased susceptibility to infections was observed in 22/32 patients (69%). IgG, IgA, and IgM were low in 13/29 (45%), 13/30 (43%) and 4/29 (14%) patients, respectively. Immunoglobulin substitution was required in three patients. Lymphocyte subsets were normal in all patients except for reduced naïve T-cells in 3/15 patients (20%) and reduced memory switched B-cells in 3/17 patients (18%). Elevated CD3 + TCRαβ + CD4-CD8-T-cells were present in 5/17 individuals (23%) and were correlated with hematological and overall autoimmunity (p < 0.05). In conclusion, immunological manifestations of KS in our cohort include susceptibility to infections, antibody deficiency, and autoimmunity. Autoimmune cytopenia is correlated with thymectomy and elevated CD3 + TCRαβ + CD4-CD8-T-cells, and benefits from treatment with mycophenolate. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Recommendations for the treatment of myasthenia gravis (MG) have been difficult to develop because of limited evidence from large randomized controlled trials. New drugs and treatment approaches have recently been shown to be effective in phase 3 studies in seropositive generalized (g) MG. One such drug is efgartigimod, a human-Fc-fragment of IgG1, with a high affinity for the endosomal FcRn. We conducted a multicenter study to evaluate the real-world clinical and safety effects of efgartigimod in 22 gMG patients. We evaluated the strategies for the timing of re-treatment with it. The participants received a total of 59 efgartigimod -treatment cycles. The median number of cycles was 2 (range 1–6). Twenty patients (86.3%) improved by at least 2 MG-ADL points after the first treatment cycle. The median MG-ADL score at baseline was 6.5 (range: 3–17) and 2.5 (range: 0–9) post-treatment (p < 0.001). A consistent improvement of at least 2 points in the MG-ADL score after each cycle occurs in 18 patients. The effect duration of the treatment was usually between 4 and 12 weeks. Two major clinical patterns of treatment response were found. Treatment with efgartigimod was also associated with significant reductions of prednisone doses Overall, the treatment was safe and associated with only minor adverse events. The single fatality was apparently due tosevere respiratory failure. We found that efgartigimod is clinically effective, may be used as a steroid sparing agent and is generally safe for gMG patients. We recommend a personalized preventive treatment approach until clinical stabilization, followed by discontinuation and periodic evaluations. [ABSTRACT FROM AUTHOR]

Patients with neuromuscular diseases are particularly vulnerable in the perioperative period to the development of pulmonary and cardiac complications, or medication side effects. These risks could include hypoventilation, aspiration pneumonia, exacerbation of underlying cardiomyopathy, arrhythmias, adrenal insufficiency, prolonged neuromuscular blockade, issues related to thermoregulation, rhabdomyolysis, malignant hyperthermia, or prolonged mechanical ventilation. Interventions at each of the perioperative stages can be implemented to mitigate these risks. A careful pre-operative evaluation may help identify risk factors so that appropriate interventions are initiated, including cardiology consultation, pulmonary function tests, initiation of noninvasive ventilation, or implementation of preventive measures. Important intraoperative issues include positioning, airway and anesthetic management, and adequate ventilation. The postoperative period may require correction of electrolyte abnormalities, control of secretions with medications, manual or mechanical cough assistance, avoiding the risk of reintubation, judicious pain control, and appropriate medication management. The aim of this review is to increase awareness of the particular surgical challenges in this vulnerable population, and guide the clinician on the various evaluations and interventions that may result in a favorable surgical outcome. [ABSTRACT FROM AUTHOR]

Thymomas are rare tumors originating from thymic tissue and rarely metastasize. They can be diagnosed either incidentally or symptomatically when compressing or invading nearby structure. A 36-year-old man presented with significant high-grade fever, chest pain that worsens upon lying down, and dyspnea. A chest X-Ray and computed tomography followed by biopsy confirmed the diagnosis of thymoma. The management included chemotherapy cycles, followed by surgery. Pericardiectomy was performed with en-bloc thymectomy and partial resection of the infiltrating lung. Venous drainage was restored by 8/16 mm inverted bifurcated brachiocephalic-superior vena cava Dacron bypass. The pericardium was reconstructed by a synthetic Dacron patch, and the right diaphragm metastasis was resected. Neoadjuvant chemotherapy was initiated. After 3 months of follow-up, no recurrence was evidenced by computed tomography. [ABSTRACT FROM AUTHOR]

Background: Subxiphoid video-assisted thoracoscopic surgery (VATS) is considered a safe and feasible operation for anterior mediastinal mass resection. However, diaphragmatic injury, presented as tearing or puncturing, may occur during subxiphoid VATS despite of low incidence. This study aims to explore risk factors for diaphragmatic injury in subxiphoid VATS, as well as strategies to reduce occurrence of the injury. Methods: We retrospectively reviewed clinical records of 44 consecutive adult patients who underwent subxiphoid VATS. These patients were divided into two groups: diaphragmatic injury group and non-injury group. Perioperative outcomes and anatomic features derived from 3D CT reconstructions were compared between the two groups. Results: Significant differences were observed in operation time (223.25 ± 92.57 vs. 136.28 ± 53.05, P = 0.006), xiphoid length (6.47 ± 0.85 vs. 4.79 ± 1.04, P = 0.001) and length of the xiphoid below the attachment point on the diaphragm (24.86 ± 12.02 vs. 14.61 ± 9.25, P = 0.029). Odds ratio for the length of the xiphoid below the attachment point on the diaphragm was 1.09 (1.001–1.186), P = 0.048 by binary logistic regression analysis. Conclusions: We identified the length of the xiphoid below the attachment point on the diaphragm as an independent risk factor for diaphragm injury during subxiphoid VATS. Prior to subxiphoid VATS, a 3D chest CT reconstruction is recommended to assess the patients' anatomic variations within the xiphoid process. For patients with longer xiphoid process, a higher incision at the middle and upper part of the xiphoid process, and partial xiphoid process resection or xiphoidectomy is preferred. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective Methodology Results Conclusions To compare the safety and effectiveness of different surgical approaches in thymectomy: robotics, subxiphoid, lateral video‐assisted thoracoscopy surgery (LVATS) and open.We retrospectively reviewed 68 cases of thymectomy with a robot‐assisted, subxiphoid, LVATS, open sternotomy or thoracotomy approach for thymic lesions or myasthenia gravis between July 2017 and May 2023 at a single centre. Peri‐operative outcomes (operating time, estimated blood loss, conversion rates, R0 resection, adverse events and length of stay [LOS]) were collected.We observed six conversions to open (from five LVATS and one robot assisted). The median estimated blood loss was lower for LVATS (100.00 [50.0–100.0] mL) compared with open thymectomies (200.0 [150.0–400.0]; P < .001). No intra‐operative adverse events were reported in the robotics, subxiphoid or LVATS groups. In patients with thymic tumours (n = 34), R0 resection was achieved in 100% (2/2) of robotics, 83% of subxiphoid (5/6), 93% (13/14) of LVATS and 75% (n = 9/12) of open cases. The median LOS was shortest for robot assisted (1.0 [interquartile range (IQR) 1.0–3.0]), then subxiphoid (2.0 [IQR 1.0–3.0]), LVATS (2.0 [IQR 1.0–3.0]) then open (5.0 [IQR 4.0–6.0]; P < .001).Our results suggest that with a shorter LOS, robotics, subxiphoid and LVATS thymectomies are safe. Larger size studies are required to compare R0 resection rates between these less invasive surgical approaches. [ABSTRACT FROM AUTHOR]

Introduction: Robotic and thoracoscopic surgery are being increasingly adopted as minimally invasive alternatives to open sternotomy for complete thymectomy. The superior maneuverability range and three-dimensional magnified vision are potential ergonomical advantages of robotic surgery. To compare the ergonomic characteristics of robotic versus thoracoscopic thymectomy, a previously developed scoring system based on impartial findings was employed. The relationship between ergonomic scores and perioperative endpoints was also analyzed. Methods: Perioperative data of patients undergoing robotic or thoracoscopic complete thymectomy between January 2014 and December 2022 at three institutions were retrospectively retrieved. Surgical procedures were divided into four standardized surgical steps: lower-horns, upper-horns, thymic veins and peri-thymic fat dissection. Three ergonomic domains including maneuverability, exposure and instrumentation were scored as excellent(score-3), satisfactory(score-2) and unsatisfactory(score-1) by three independent reviewers. Propensity score matching (2:1) was performed, including anterior mediastinal tumors only. The primary endpoint was the total maneuverability score. Secondary endpoints included the other ergonomic domain scores, intraoperative adverse events, conversion to sternotomy, operative time, post-operative complications and residual disease. Results: A total of 68 robotic and 34 thoracoscopic thymectomies were included after propensity score matching. The robotic group had a higher total maneuverability score (p = 0.039), particularly in the peri-thymic fat dissection (p = 0.003) and peri-thymic fat exposure score (p = 0.027). Moreover, the robotic group had lower intraoperative adverse events (p = 0.02). No differences were found in residual disease. Conclusions: Robotic thymectomy has shown better ergonomic maneuverability compared to thoracoscopy, leading to fewer intraoperative adverse events and comparable early oncological results. [ABSTRACT FROM AUTHOR]

The thymus gland aids in the maturation of the immune system. An overactive or malfunctioning thymus gland, as seen in thymomas, can lead to disrupted immune systems. Thymectomy, the usual treatment, can paradoxically lead to further derangements in the immune system, leading to new autoimmune disorders. Most of these reported disorders are rheumatological. Except preclinical studies, there are no reported cases of autoimmune diabetes post-thymectomy. A 25-year-old woman who had malignant thymoma underwent chemotherapy, followed by thymectomy and radiotherapy. She developed autoimmune diabetes mellitus (AID) approximately 1 year post-thymectomy, evident from raised glycated hemoglobin, anti-glutamic acid decarboxylase (GAD) antibodies, ineffectiveness of oral glucose-lowering agents, and positive response to insulin. AID can occur after thymectomy, as evidenced by animal studies and this case report. Whether these patients would have long-term outcomes and control of diabetes differently than classic type 1 diabetes mellitus (T1D) is uncertain. Further research is needed to prove causality between thymectomy and diabetes. [ABSTRACT FROM AUTHOR]

The thymus is a lymphoid organ involved in the differentiation of T cells, and has a central role in the physiopathogenesis of Myasthenia Gravis (MG). This connection is proved by a series of changes in the level of neuromuscular junctions, which leads to a decrease in the amplitude of the action potential in the post-synaptic membrane. Because of this, the presence of anti-cholinergic receptor antibodies (AChR), characteristic of MG, is found, which causes the progressive regression of the effect of acetylcholine at the level of neuromuscular junctions, with the appearance of muscle weakness. The thymectomy is a surgical variant of drug therapy administered to patients with MG. In the case of patients with nonthymomatous MG, thymectomy has become a therapeutic standard, despite the fact that there is no solid scientific evidence to explain its positive effect. Videothoracoscopic surgery or robotic surgery led to a decrease in the length of hospital stay for these patients. This paper aims to synthesize the information presented in the literature in order to create a background for the perspectives of thymectomy. [ABSTRACT FROM AUTHOR]

Objectives: RAISE (NCT04115293) was a randomized, multicenter, double‐blind, placebo‐controlled phase III study of zilucoplan, a macrocyclic peptide and complement component 5 inhibitor with a dual mechanism of action, in patients with acetylcholine receptor autoantibody‐positive generalized myasthenia gravis (MG). RAISE showed clinically meaningful and statistically significant improvements in MG‐specific outcomes in the overall population. Here, we assess efficacy and safety of zilucoplan in patients with generalized myasthenia gravis in the Japanese patients enrolled in RAISE. Methods: Adults with acetylcholine receptor autoantibody‐positive generalized MG (MGFA disease class II–IV) were randomized 1:1 to daily self‐administered subcutaneous zilucoplan 0.3 mg/kg or placebo injections for 12 weeks. The primary efficacy endpoint was change from baseline to week 12 in the MG Activities of Daily Living score. Safety was assessed by the incidence of treatment‐emergent adverse events. Efficacy and safety outcomes of a Japanese subgroup were prespecified. Results: Overall, 16 Japanese patients were randomized to zilucoplan 0.3 mg/kg (n = 7) or placebo (n = 9). There was a clinically meaningful improvement in the MG Activities of Daily Living score at week 12 for zilucoplan versus placebo in the Japanese population, with least squares mean difference of −4.26 (95% confidence interval −7.40, −1.12), which was comparable with the overall population. The incidence of treatment‐emergent adverse events was similar in both treatment arms, with 57.1% and 55.6% of patients in the zilucoplan and placebo groups, respectively, experiencing treatment‐emergent adverse events. Conclusions: In a subgroup of Japanese patients, zilucoplan showed clinically meaningful improvement in MG‐specific outcomes with a favorable safety profile, consistent with the overall RAISE population. [ABSTRACT FROM AUTHOR]

Objectives: This comprehensive review aimed to compile cases of patients with thymoma diagnosed with both autoimmune encephalitis (AE) and myasthenia gravis (MG), and describe their clinical characteristics. Methods: Clinical records of 3 AE patients in the first affiliated hospital of Sun Yat‐sen University were reviewed. All of them were diagnosed with AE between 1 November 2021 and 1 March 2022, and clinical evidence about thymoma and MG was found. All published case reports were searched for comprehensive literature from January 1990 to June 2022. Results: A total of 18 cases diagnosed with thymoma‐associated autoimmune encephalitis (TAAE) and thymoma‐associated myasthenia gravis (TAMG) were included in this complication, wherein 3 cases were in the first affiliated hospital of Sun Yat‐sen University and the other 15 were published case reports. 5/18 patients had alpha‐amino‐3‐hydroxy‐5‐methyl‐4‐isoxazolepropionic acid receptor antibody (AMPAR‐Ab) in their serum and cerebrospinal fluid (CSF). All of them had positive anti‐acetylcholine receptor antibody (AChR‐Ab). And 12/18 patients showed a positive response to thymectomy and immunotherapy. Besides, thymoma recurrences were detected because of AE onset. And the shortest interval between operation and AE onset was 2 years in patients with thymoma recurrence. Conclusions: There was no significant difference in the clinical manifestations between these patients and others with only TAMG or TAAE. TAAE was commonly associated with AMPAR2‐Ab. Significantly, AE more commonly heralded thymoma recurrences than MG onset. And the intervals of thymectomy and MG or AE onset had different meanings for thymoma recurrence and prognoses of patients. [ABSTRACT FROM AUTHOR]

Background and purpose: Germinal centers (GCs) can be observed in the thymic tissues of patients with thymoma‐associated myasthenia gravis (MG). Although an association between thymic GCs and MG has been suggested, it is unknown whether the presence of GCs could predict the development of MG after the resection of thymoma, known as postthymectomy MG. Methods: We conducted a retrospective analysis of previously nonmyasthenic patients who underwent surgical removal of the thymoma. All available thymic tissue slides were rereviewed by a pathologist to assess for GCs. Patients were classified into GC‐positive and GC‐negative groups based on the presence of GCs. The incidence of postthymectomy MG was compared between the two groups, and the risk factors for postthymectomy MG were assessed. Results: Of the 196 previously nonmyasthenic patients who underwent thymoma resection, 21 were GC‐positive, whereas 175 were GC‐negative. Postthymectomy MG developed in 11 (5.6%) patients and showed a higher incidence in the GC‐positive group than in the GC‐negative group (33.3% vs. 2.3%, p < 0.001). No postoperative radiotherapy and the presence of GCs were risk factors for postthymectomy MG in the univariate analysis. In multivariate analysis, invasive thymoma (hazard ratio [HR] = 9.835, 95% confidence interval [CI] = 1.358–105.372), postoperative radiotherapy (HR = 0.160, 95% CI = 0.029–0.893), and presence of GCs (HR = 15.834, 95% CI = 3.742–67.000) were significantly associated with postthymectomy MG. Conclusions: Thymic GCs may be a significant risk factor for postthymectomy MG. Even in patients with thymoma who do not show clinical symptoms of MG, postthymectomy MG should be considered, especially if thymic GCs are observed. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective: The efficacy and safety of subxiphoid thoracoscopic thymectomy (SVATS) for early thymoma are unknown. The purposes of this meta-analysis were to evaluate the effectiveness and safety of SVATS for early thymoma, to compare it with unilateral intercostal approach video thoracoscopic surgery (IVATS) thymectomy, and to investigate the clinical efficacy of modified subxiphoid thoracoscopic thymectomy (MSVATS) for early anterior mediastinal thymoma. Methods: Original articles describing subxiphoid and unilateral intercostal approaches for thoracoscopic thymectomy to treat early thymoma published up to March 2023 were searched from PubMed, Embase, and the Cochrane Library. Standardized mean differences (SMDs) and 95% confidence intervals (CIs) were calculated and analyzed for heterogeneity. Clinical data were retrospectively collected from all Masaoka stage I and II thymoma patients who underwent modified subxiphoid and unilateral intercostal approach thoracoscopic thymectomies between September 2020 and March 2023. The operative time, intraoperative bleeding, postoperative drainage, extubation time, postoperative hospital stay, postoperative visual analog pain score (VAS), and postoperative complications were compared, and the clinical advantages of the modified subxiphoid approach for early-stage anterior mediastinal thymoma were analyzed. Results: A total of 1607 cases were included in the seven studies in this paper. Of these, 591 cases underwent SVATS thymectomies, and 1016 cases underwent IVATS thymectomies. SVATS thymectomy was compared with IVATS thymectomy in terms of age (SMD = − 0.09, 95% CI: −0.20 to − 0.03, I2 = 20%, p = 0.13), body mass index (BMI; SMD = − 0.10, 95% CI: −0.21 to − 0.01, I2 = 0%, p = 0.08), thymoma size (SMD = − 0.01, 95% CI: −0.01, I2 = 0%, p = 0.08), operative time (SMD = − 0.70, 95% CI: −1.43–0.03, I2 = 97%, p = 0.06), intraoperative bleeding (SMD = − 0.30. 95% CI: −0.66–0.06, I2 = 89%, p = 0.10), time to extubation (SMD = − 0.34, 95%CI: −0.73–0.05, I2 = 91%, p = 0.09), postoperative hospital stay (SMD = − 0.40, 95% CI: −0.93–0.12, I2 = 93%, p = 0.13), and postoperative complications (odds ratio [OR] = 0.94, 95% CI: 0.42–2.12, I2 = 57%, p = 0.88), which were not statistically significantly different between the SVATS and IVATS groups. However, the postoperative drainage in the SVATS group was less than that in the IVATS group (SMD = − 0.43, 95%CI: −0.84 to − 0.02, I2 = 88%, p = 0.04), and the difference was statistically significant. More importantly, the postoperative VAS was lower in the SVATS group on days 1 (SMD = − 1.73, 95%CI: −2.27 to − 1.19, I2 = 93%, p < 0.00001), 3 (SMD = − 1.88, 95%CI: −2.84 to − 0.81, I2 = 97%, p = 0.0005), and 7 (SMD = − 1.18, 95%CI: −2.28 to − 0.08, I2 = 97%, p = 0.04) than in the IVATS group, and these differences were statistically significant. A total of 117 patients undergoing thoracoscopic thymectomy for early thymoma in the Department of Thoracic Surgery of the Second Hospital of Jilin University were retrospectively collected and included in the analysis, for which a modified subxiphoid approach was used in 42 cases and a unilateral intercostal approach was used in 75 cases. The differences between the two groups (MSVATS vs. IVATS) in general clinical characteristics such as age, sex, tumor diameter, Masaoka stage, Word Health Organization (WHO) stage, and intraoperative and postoperative conditions, including operative time, postoperative drainage, extubation time, postoperative hospital stay, and postoperative complication rates, were not statistically significant (p > 0.05), while BMI, intraoperative bleeding, and VAS on postoperative days 1, 3, and 7 were all statistically significant (p < 0.05) in the MSVATS group compared with the IVATS group. Conclusion: The meta-analysis showed that the conventional subxiphoid approach was superior in terms of postoperative drainage and postoperative VAS pain scores compared with the unilateral intercostal approach. Moreover, the modified subxiphoid approach had significant advantages in intraoperative bleeding and postoperative VAS pain scores compared with the unilateral intercostal approach. These results indicate that MSVATS can provide more convenient operation conditions, a better pleural cavity view, and a more complete thymectomy in the treatment of early thymoma, indicating that is a safe and feasible minimally invasive surgical method. [ABSTRACT FROM AUTHOR]

Simple Summary: Extended thymectomy is the gold standard in the treatment of patients with thymic neoplasm and affected by myasthenia gravis. For a long time, the traditional approach has been sternotomy, though the application of minimally invasive techniques has spread in recent decades. Several authors have demonstrated the safety and feasibility of minimally invasive thymectomy. This multicenter study aims to compare the outcomes of robotic and open thymectomy in myasthenic patients affected by thymic tumors. Short-term and long-term results were presented, showing how the robotic approach can be considered comparable to open surgery in terms of oncological radicality and the improvement of myasthenic symptomatology, with associated faster recovery. Thymectomy is the gold standard in the treatment of thymic neoplasm and plays a key role in the therapeutic path of myasthenia gravis. For years, sternotomy has been the traditional approach for removing anterior mediastinal lesions, although the robotic thymectomy is now widely performed. The literature is still lacking in papers comparing the two approaches and evaluating long-term oncological and neurological outcomes. This study aims to analyze the postoperative results of open and robotic thymectomy for thymic neoplasms in myasthenic patients. Surgical, oncological and neurological data of myasthenic patients affected by thymic neoplasms and surgically treated with extended thymectomy, both with the open and the robotic approach, in six Italian Thoracic Centers between 2011 and 2021 were evaluated. A total of 213 patients were enrolled in the study: 110 (51.6%) were treated with the open approach, and 103 (48.4%) were treated with robotic surgery. The open surgery, compared with the robotic, presented a shorter operating time (p < 0.001), a higher number of postoperative complications (p = 0.038) and longer postoperative hospitalization (p = 0.006). No other differences were observed in terms of surgical, oncological or neurological outcomes. The robotic approach can be considered safe and feasible, comparable to the open technique, in terms of surgical, oncological and neurological outcomes. [ABSTRACT FROM AUTHOR]

This article discusses the clinical and immunological consequences of early-life thymectomy in children with congenital heart disease (CHD). Thymectomy, the removal of the thymus, is often performed during cardiovascular surgery in these children. The study found that thymectomized children commonly exhibit lymphopenia and an altered T cell profile, including a decrease in naïve CD4 and CD8 cells and an increase in memory T cells. The research also suggests that thymectomy may lead to premature immune aging, reduced thymic output, and an increased risk of autoimmune development later in life. The findings highlight the importance of monitoring the long-term effects of early-life thymectomy. [Extracted from the article]

This article discusses the impact of congenital heart disease (CHD) on thymus function in infants. The study found that infants with highly complex CHD experience thymic atrophy, resulting in reduced thymic output. This compromised thymic function may contribute to the increased susceptibility to infections and immune-mediated diseases observed in these patients. The researchers suggest that preserving the thymus during cardiovascular surgery could improve immune function and the long-term health of CHD patients. [Extracted from the article]

Background and Objectives: Preoperative respiratory muscle training (RMT) for patients undergoing surgical treatment for myasthenia gravis (MG) has not been shown to improve postoperative complications. The purpose of this research was to analyse the effects of RMT and aerobic exercise on respiration, physical activity, and hospital stay in patients with MG prior to surgery. Methods: Ninety patients with MG who were having a prolonged thymectomy were split into two groups at random. In the SG, 45 participants underwent preoperative moderate-to-intense RMT in addition to aerobic exercise and respiratory physiotherapy, while the CG received only chest physiotherapy. Patients' vital capacities (VC, FVC, FEV1, FEV1/FVC, and PEF) and exercise capacities (6-minute walk test [6 MWT]) were assessed prior to surgery, during recovery, and immediately prior to discharge. Both length of hospital stay and "activities of daily living" (ADL) scores were calculated. Result: Vital capacity, exercise capacity, and demographic and surgical characteristics were similar across the two groups before surgery. While the FEV1/FVC ratio did not change significantly from pre- to post-op in the CG, all measures of lung function (VC, FVC, FEV1, PEF, and 6MWT) were significantly worse after surgery (p=0.001). After surgery, the SG had significantly better VC, FVC, FEV1, and PEF than the CG did (p=0.012, 0.030, 0.014, and 0.035 respectively), while having similar 6MWT results. On day 5 post-op, SG ADL was significantly greater than CG ADL (p=0.001). Conclusion: Recovery from surgery for people with MG can speed up with the help of respiratory muscle training (RMT) and aerobic exercise. [ABSTRACT FROM AUTHOR]

Purpose: Clinical feasibility nomograms were developed to facilitate the differentiation between thymic epithelial tumors (TETs) and mediastinal lymphomas (MLs), aiming to minimize the occurrence of non-therapeutic thymectomy. Methods: A total of 255 patients diagnosed with TETs or MLs underwent pre-treatment 18F-FDG PET/CT. Comprehensive clinical and imaging data were collected, including age, gender, lactate dehydrogenase (LDH) level, pathological results, presence of myasthenia gravis symptoms, B symptoms, mass size, location, morphology, margins, density, and metabolic parameters derived from PET/CT. Radiomic features were extracted from the region of interest (ROI) of the primary lesion. Feature selection techniques were employed to identify the most discriminative subset of features. Machine learning methods were utilized to build candidate models, which were subsequently evaluated based on their area under the curve (AUC). Finally, nomograms were constructed using the optimal model to provide a clinical tool for improved diagnostic accuracy. The performance of the radiomic models was evaluated by their calibration, discrimination, and clinical utility. Results: Several independent risk factors were identified for distinguishing TETs from MLs, including average standardized uptake value (SUVavg), LDH, age, mass size, and radiomic score (rad-score). Significance was observed in differentiating the two types of tumors based on these factors. The best clinical efficacy was demonstrated by the combined model, with an impressive AUC of 0.954. Decision curve analysis and calibration curves indicated that the combined model was clinically advantageous for discriminating TETs from MLs. Besides, the results of external validation showed a sensitivity of 0.8 and a specificity of 0.78. Conclusion: Preoperatively, the differentiation of TETs from MLs can be facilitated by the utilization of the combined clinical information and radiomics model. This approach holds promise in minimizing the occurrence of unnecessary anterior mediastinal surgeries. [ABSTRACT FROM AUTHOR]

According to the different classifications now in use, thymic tumours are staged by the extent of local invasiveness, and tumour size is not included as a major determinant for the T category. The aim of this double-site retrospective study is to analyse the correlation between tumour dimension and overall survival (OS) in patients who underwent surgical treatment. From January 2000 to December 2020, patients with thymic epithelial tumours who underwent surgical resection were included in this study. Data from a total of 332 patients were analysed. Five- and ten-year overall survival (5–10 YOS) was 89.26% and 87.08%, respectively, while five- and ten-year disease-free survival (DFS) was 88.12% and 84.2%, respectively. Univariate analysis showed a significant correlation between male sex (p-value 0.02), older age (p-value < 0.01), absence of myasthenia gravis (p-value < 0.01), increase in pTNM (pathological Tumor Node Metastasis) (p-value 0.03) and increase in the number of infiltrated organs (p-value 0.02) with an increase in tumour dimension. Tumour dimension alone was not effective in the prediction of DFS and OS, both when considered as a continuous variable and when considered with a cut-off of 3 and 5 cm. However, with multivariate analysis, it was effective in predicting OS in the aforementioned conditions (p-value < 0.01). Moreover, multivariate analysis was also used in the thymoma and Masaoka I subgroups. In our experience, the role of tumour dimension as a descriptor of the T parameter of the TNM (Tumor Node Metastasis) staging system seemed to be useful in improving this system. [ABSTRACT FROM AUTHOR]

Objective: To evaluate effectivity of thymectomy as an alternative treatment of MG in our center, between 2007 and 2019 and to know how it has impacted in life quality and pharmacological management. Material and Method: cohort study patients treated with thymectomy by Thoracic Surgical team from Gustavo Fricke Hospital between 2007 and 2019. The study variables were medical treatment and anticholinesterases doses before and after the thymectomy, and life quality measured through MG-QOL15 survey. Standard deviation measures and statistics comparisons were used for the analysis of these variables, considering statistically significant a p < 0.05. Results: total of 20 patients, mainly young women, thymectomy through a Trans-sternal approach. Anticholinesterase doses, showed a statistically significant decrease from 5.05 to 3.06 before and after thymectomy (p < 0.05). Discussion: It has been demonstrated that thymectomy plays an important role on the management of MG, giving a mastery against medical exclusive treatment, The American Foundation for Myasthenia Gravis recommends the use of MG-QOL15 survey as an important tool to evaluate life quality. There is a limited amount of national literature related to this topic. Conclusion: Thymectomy is a life quality changing procedure for MG patients and it helps to significantly reduce the drug doses used. [ABSTRACT FROM AUTHOR]

Thymectomy is a treatment for pediatric myasthenia gravis, but the efficacy over time is unknown. Multi-institutional data are also lacking. Therefore, the objective of this study was to determine the efficacy of thymectomy for pediatric myasthenia gravis using medication burden and health care utilization as proxies for disease severity. This was a cross-sectional study of the Pediatric Health Information System database among children who underwent thymectomy at one of 49 children's hospitals from 2004 to 2022. Differences in annual median number of doses of myasthenia-related medications, admissions, and health care costs in the year before thymectomy to three years after were compared. A comparison cohort that did not undergo thymectomy was utilized. Medians were compared using the Wilcoxon signed-rank test. Generalized linear regression estimated the effect of surgical approach on outcomes. A total of451 patients (238 patients who underwent thymectomy and 213 nonthymectomy patients) were identified. Following thymectomy, the decrease in annual median total number of myasthenia-related doses was 12.0 (interquartile range: 6 to 31) (P < 0.001). The decrease in number of annual admissions was 2.0 (1 to 4) (P < 0.001), which represented a cost difference of $5292 ($3533 to $8681) (P < 0.001). No differences were observed in the control cohort. In a generalized linear regression model, surgical approach was not associated with the efficacy of thymectomy (P = 0.55). Thymectomy is an effective treatment for pediatric myasthenia gravis, evidenced by the decreased medication burden and health care utilization after surgery. Surgical approach did not influence the success of surgery. Thymectomy should be considered earlier in the treatment algorithm. [ABSTRACT FROM AUTHOR]

Introduction: Retrosternal goitre was first described by Albrecht von Haller in 1749, as the extension of the thyroid tissue below the upper opening of the chest. Retrosternal goitre is most commonly defined as one that either descends below the thoracic inlet, or has more than 50% of its volume below this level. Retrosternal Goitres can be classified as either primary or secondary. Most retrosternal goitres can be removed through a cervical approach, while a partial or total sternotomy should be performed only in a minority of patients, ranging between 1-11%. Aims & Objectives: Retrosternal goiter may cause symptoms of airway obstruction and dysphagia, but often it is asymptomatic and is increasingly detected incidentally with imaging investigations. Consensus has been reached that sternotomy is not necessary in most cases, as a collar incision normally suffices. Our retrospective study was to analyse personal experience in the surgical management of retrosternal goitres, defining, in particular, the features requiring sternotomy. Materials and Methods: This is a Retrospective study comprising of 45 patients who underwent thyroidectomies between period of 2015-2022 in department of surgical oncology NIMS HYD. The patients with were analyzed further, with regard to demographics, presentation, indications, and outcome of surgical treatment and HPE reports. Results: A total of 45 patients with 11 male & 34 female patients with Mean age 50 yrs (Age range 22-75). Most common presentation was neck swelling 68%, followed by respiratory symptoms. Surgical procedure predominantly done was near total thyroidectomy. Tracheostomy was done in 7 cases. In all cases recurrent laryngeal nerve identified and preserved. In 43 cases retrosternal goitre was removed by transcervical approach & sternotomy was needed in only 2 cases. Final histological diagnosis revealed malignancy in 6 cases (4 Papillary & 2 follicular ca). No intra or perioperative deaths occurred. Discussion: All patients who presented to department of surgical oncology NIMS Hyd. with thyroid swelling were clinically examined and patients in whom lower border of swelling was not palpable were considered to have retrosternal extension and were further evaluated using CXR, HRUS AND CT. Conclusion: In our experience surgical approach for retrosternal goitre should be determined by type of retrosternal extension (ectopic) and cytomorphology. Transcervical approach is gold standard in exploring the retrosternal extent of goitre. meticulous digital dissection should be carried out by the experienced surgeon. In cases where goitre extension in to thoracic cavity is bilateral and in malignancies sternotomy should be considered for excellent exposure and for reducing complications. [ABSTRACT FROM AUTHOR]

Background: Myasthenia gravis (MG) patients interact unpredictably with anesthetic agents, including neuromuscular blocking agents. Here, we investigate the effectiveness of general anesthesia without muscle relaxants using either propofol via target-controlled infusion systems (TCI) or sevoflurane in MG patients undergoing thoracoscopic thymectomy. Methods: This prospective, open-label, observational study was conducted in a university hospital. We included 90 myasthenic patients undergoing thoracoscopic thymectomy with general anesthesia. Patients received induction and maintenance anesthesia with propofol TCI (group P, n = 45) or induction with propofol 2–3 mg.kg−1 and maintenance anesthesia with sevoflurane (group S, n = 45). In both groups, the procedure was performed under the guidance of entropy with sufentanil but not a muscle relaxant. Intubation conditions, hemodynamic changes, respiratory function, neuromuscular transmission, arterial blood gas, and complications were evaluated. Results: All patients achieved good intubation conditions. Hemodynamic instability was more frequent in group S than in group P, mostly in the induction stage, and was controllable. The reduction in the intraoperative train-of-four ratio from baseline at 30 min, 60 min, and 90 min in group S was 10.3%, 14.2%, and 14.3%, respectively, significantly higher than that in group P (6.8%, 7.2%, and 8.4%, respectively), which completely recovered at the end of the surgery. All patients were extubated in the operating room without complications. No other significant differences between the groups were observed. Conclusions: Anesthesia with propofol TCI or sevoflurane without muscle relaxants in MG patients offered safe and effective conditions for thoracoscopic thymectomy. Sevoflurane achieved higher levels of intraoperative muscular relaxation than propofol TCI. Postoperative neuromuscular function was not affected by these anesthetics. [ABSTRACT FROM AUTHOR]

Extended thymectomy is performed to excise the ectopic thymic tissue around the thymus; however, the distribution of the ectopic thymic tissue in the adipose tissue remains unclear. This study investigated the distribution of ectopic thymic tissue in extended thymectomy. This study included 31 patients who underwent extended thymectomy. Pathological specimens were reviewed to examine the distribution of ectopic thymic tissues in six locations: the right and left outer sides of the thymus tissue and the upper and lower ends of the resected tissue in extended thymectomy. The frequency of ectopic thymic tissue and the ratio to the surrounding adipose tissue were estimated. In our study, all patients, including patients >60 years, had thymic tissue in the surrounding adipose tissue removed during extended thymectomy. The probability of an ectopic thymus existing near the diaphragm, i.e., at the caudal end of the resection, was 13–19%, and the ratio of the area of the ectopic thymus to that of adipose tissue was <1% in almost all cases. In three patients with myasthenia gravis, an ectopic thymus was found in the adipose tissue around the diaphragm at a rate of >1%; there were no cases exceeding 10%. This value is extremely low compared with those of the other excision areas of extended thymectomy. Ectopic thymic tissues are far less likely to be present in adipose tissue on the diaphragm compared with that of other areas. This finding may help surgeons during the excision of adipose tissue around the thymus. [ABSTRACT FROM AUTHOR]

We present 2 cases referred for evaluation of Graves disease (GD) associated with an incidental mediastinal mass. Chest computed tomography (CT) scans showed a 1.2 × 2.4 × 4.3 cm and a 5.7 × 2.6 × 7 cm thymic enlargement, respectively, consistent with thymic hyperplasia (TH) in the 2 patients. Patient 1 had been assessed by thoracic surgery for the mediastinal mass, and thymectomy had been performed to exclude thymoma, with an anatomopathological diagnosis consistent with thymic hyperplasia. Patient 2 was treated with methimazole. CT scan was repeated after he maintained a euthyroid state, which revealed total regression of the mass. There is a well-documented association between these 2 entities, but it is often underdiagnosed and unrecognized in routine clinal practice. The benign evolution, as evidenced by regression of thymic hyperplasia after resolution of the hyperthyroidism, is characteristic. These cases highlight the importance of recognizing the association of GD and TH and warrant a conservative approach, preventing unnecessary thymic evaluation and surgery. [ABSTRACT FROM AUTHOR]

Objective: To describe the post-surgical clinical evolution of a case series of patients with juvenile myasthenia gravis (JMG) treated with right video-assisted thoracoscopic (TVA) thymectomy. Materials and Methods: Retrospective study that included 13 pediatric patients with JMG who underwent right TVA thymectomy at the Siglo XXI National Medical Center of Mexico between March 2016 and April 2022. Patients were clinically characterized, and the disease was classified according to Osserman’s criteria. Post-surgical evolution was evaluated using the DeFilippi classification to determine the proportion of patients with improvement and complete remission. Results: The included patients were mostly women (84.6%) with a mean age at diagnosis of 11.1 ± 3.1 years. The four MG classifications were included, with the highest proportion of mild generalized MG (38.5%), followed by ocular (23.1%) and moderatesevere generalized (23.1%). The evaluation of post-surgical progression showed that after three months of follow-up, 92.3% presented improvements, including a decrease in the use of medications. Complete remission was only recorded in one of the patients. Patients who underwent surgery before 12 months of evolution of JMG had better results after TVA thymectomy. Conclusion: The usefulness of TVA thymectomy in Mexican pediatric patients with JMG was demonstrated. Our experience adds evidence that pediatric patients benefit from thymectomy by improving their clinical status and decreasing the use of medications and complications of the disease. [ABSTRACT FROM AUTHOR]

The aim of our study is to show the contribution of thymectomies in the treatment of myasthenia gravis. Patients and méthode: this was a descriptive and retrospective study carried out in the thoracic surgery department of the teaching hospital Hassan II in Fez during the period May 2009-May 2018. It concerned all the patients who had undergone a thymectomy by median sternotomy for myasthenia gravis. The results of this surgery were evaluated by a neurologist using the scale of the Myasthenia Gravis Foundation of America (MGFA). Results: this was a series of 34 myasthenic patients who underwent a thymectomy. There were 24 women and 10 men with an average age of 34. Clinical signs were classified according to Osserman’s myasthenic score. Thus, after surgery, a significant decrease in symptoms and drug doses was observed in 68% of cases, while it was unchanged in 18%. In addition, univariate analysis showed a statistically significant correlation (p = 0.02) between female sex and improved patients after surgery. Likewise, the improved patients had B1 and B2 thymoma in 56 and 44% respectively, while those in the “unchanged” group had B1 and B3 thymoma in 50% each. Conclusion: thymectomy provides clinical improvement and better quality of life for myasthenic patients with very low morbidity and mortality. It must be carried out in centers experienced in this task with intensive care units used to this kind of care. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this article is to evaluate the security and effectiveness of subxiphoid and subcostal robot-assisted thoracoscopic thymectomy (S-RATT) and compare it with subxiphoid and subcostal video-assisted thoracoscopic thymectomy (S-VATT) in terms of short-term perioperative results and costs. Methods: A retrospective study was carried out on 62 individuals who had undergone successful complete thymectomy for anterior mediastinal disease using subxiphoid and subcostal arch approaches. Propensity score-matching analysis was utilized between the two groups, and the perioperative outcomes were compared. Results: The S-RATT group exhibited less intraoperative blood loss (20 ± 15.35 versus 69.55 ± 69.54, P < .001), lower levels of C-reactive protein (112.38 ± 68.08 versus 72.58 ± 42.62, P = .027), and lower postoperative pain scores (2.09 ± 1.54 versus 4.27 ± 1.28, P < .001). However, the hospitalization costs of patients in the S-VATT group were found to be lower than those in the S-RATT group (33,802.41 ± 8785.05 versus 49,977.53 ± 20,221.79, P < .001). Conclusions: S-RATT appears to be a viable and secure method for managing anterior mediastinal tumors. [ABSTRACT FROM AUTHOR]

Purpose: Minimally invasive surgery (MIS) for thymic tumors is now accepted widely, in line with improved surgical techniques; however, we occasionally encounter complicated cases of large tumors or of total thymectomy requiring prolonged operative duration or conversion to an open procedure (OP). We reviewed patients registered in a nationwide database to identify the technical feasibility of MIS for thymic epithelial tumors. Methods: Data on patients treated surgically between 2017 and 2019 were extracted from the National Clinical Database of Japan. Clinical factors and operative outcomes were calculated by tumor diameter using trend analyses. Perioperative outcomes of MIS for non-invasive thymoma were investigated using propensity score-matched analyses. Results: MIS was performed in 46.2% of the patients. The operative duration and conversion rate increased with the tumor diameter (p <.001). After propensity score-matching, operative duration and postoperative hospital stay were shorter (p <.001), and the transfusion rate was lower (p =.007) in patients who underwent MIS than in those who underwent OP for thymomas ≥ 5 cm. Among patients who underwent total thymectomy, blood loss was less (p <.001) and the postoperative hospital stay was shorter (p <.001) in those who underwent MIS than in those who underwent OP. There were no significant differences in postoperative complications and mortality. Conclusions: MIS is technically feasible even for large non-invasive thymomas or for total thymectomy, although the operative duration and open conversion rate increase with the tumor diameter. [ABSTRACT FROM AUTHOR]

The case report of a 54 years patient is described, who was admitted to Hermanos Ameijeiras Clinical-Surgical Teaching Hospital in Havana city due to fever, hypotension and diarrhea, to which complicated respiratory difficulty was added during his hospitalization and he required mechanical ventilator support in the Intensive Care Unit. As a radiographic finding, a shade in the left lung was observed that turned out to be a thymoma; while the blood test revealed hipogammaglobulinemia. It was decided to carry out conventional thymectomy by means of midline sternotomy, after which the patient had a favorable clinical course and was discharged from the hospital 10 days later. [ABSTRACT FROM AUTHOR]

This single-center retrospective exploratory analysis evaluated the effects of sugammadex compared with neostigmine on postoperative recovery in patients with myasthenia gravis (MG) who underwent video-assisted thoracoscopic surgery (VATS)–thymectomy. This retrospective study included 180 patients with MG, aged >18 years, who received sugammadex (sugammadex group, n = 83) or neostigmine–glycopyrrolate (neostigmine group, n = 88) after VATS–thymectomy between November 2007 and December 2020. Inverse probability of treatment weighting (IPTW) adjustment was performed to balance the baseline characteristics between the two groups. The primary outcome was the length of postoperative hospital stay, and the secondary outcomes were the incidence of postoperative mortality and complications, as well as the postoperative extubation and reintubation rates, in the operating room after VATS–thymectomy; the outcomes were compared between the two groups. After IPTW adjustment, the sugammadex group showed a significantly shorter median postoperative hospital stay than the neostigmine group (4 (2, 4) vs. 5 (3, 6) days, respectively; p = 0.003). There were no significant differences between the two groups in the incidences of postoperative complications (including postoperative myasthenic crisis, nerve palsy, atelectasis, and pleural effusion). Patients with MG following VATS–thymectomy who received sugammadex showed a significantly shorter postoperative hospital stay than those who received neostigmine. [ABSTRACT FROM AUTHOR]

Abstract Aim Materials and Methods Results Conclusion We aimed to investigate the relationship between the peripheral lymphocyte subset frequency and thymectomy in patients with myasthenia gravis (MG).The frequencies of regulatory B (Breg) and regulatory T (Treg) cells in peripheral blood samples obtained from 69 patients with MG and 10 healthy controls were analyzed using flow cytometry. Serum acetylcholine receptor antibodies (AchR-Ab) were measured. Patients with MG were subdivided into pre-thymectomy, post-thymectomy, and normal thymus control group.The percentage of Breg cells was significantly decreased in both the pre-thymectomy (7.92 ± 1.30%) and post-thymectomy (8.14 ± 1.34%) groups compared to healthy controls (16.02 ± 2.78%) and reduced in the exacerbation and relapse phase compared to the stable maintenance stage. The proportion of cluster of differentiation (CD) 4 + CD25 + T cells and CD4 + CD25 + CD127low/- Treg cells in MG patients were not significantly different than healthy controls. AchR-Ab titers in aggravating or recurrence patients after thymectomy were significantly higher than that of the stable remission patients (11.13 ± 0.70 and 6.03 ± 0.85 nmol/L, respectively; p < 0.001).The frequency of Breg cells may serve as a potential indicator of MG prognosis, while Treg cell frequency did not demonstrate the same prognostic ability. The concentration of AchR-Ab can be used as a dynamic monitoring index of disease severity in patients with MG. [ABSTRACT FROM AUTHOR]

Background: Regardless of its rare occurrence, Thymoma remains the most frequently encountered primary tumor of the anterior mediastinum comprising about 50% of all masses in the region. Surgical resection, via thymectomy, remains the mainstay treatment modality. In locally advanced and borderline resectable tumors, neoadjuvant chemotherapy (NACT) may be utilized to increase the chance of R0 resection, raising the question of its efficacy and safety. Methods: Demographic and clinical data from patients who presented to a tertiary cancer center between January 2015–October 2021 with a diagnosis of thymoma and underwent curative surgical resection was collected. Computed tomography scan was used to delineate clinical staging, tumor size and to detect post-therapeutic variations in tumor burden. The response evaluation criteria in solid tumors (RECIST) was used to classify the effect of NACT on tumor burden. The pathological response was determined by measuring the percentage of necrotic tissue. Results: A total of 23 patients were diagnosed with thymoma. Most patients were male with a mean age 46 (± 15) years at diagnosis. The most common clinical stage was stage II with 5 patients (22%). A total of 12 patients had NACT as compared to 11 patients who had upfront surgery. The mean change in tumor volume was 165 cm3 (p = 0.079) and the change in and maximum diameter was 1.53 ± 1.49 cm (p < 0.01). The effect of NACT on tumor burden based on RECIST criteria was minimal as 8 patients had stable disease. Based on pathological findings, the average necrotic portion of the tumor was 39.5% (p = 0.152). The overall survival rate is 95.65%, mean survival was 115 months (4–125). Recurrence occurred in 5 patients. The NACT group had a higher risk for recurrence (4; 33.3%) with a mean survival of 43.8 months compared to 59.6 months in those who did not receive induction therapy. Conclusions: The exact role of induction chemotherapy in locally advanced thymoma patients remains controversial. NACT effect after utilizing radiological and pathological assessment tools was not found to significantly improve oncological outcomes compared to upfront surgery in locally advanced disease, with minimal radiologic and pathologic effect. To further demonstrate the impact of induction chemotherapy, we recommend multicentric collaborative studies. [ABSTRACT FROM AUTHOR]

BACKGROUND The function of the thymus in human adults is unclear, and routine removal of the thymus is performed in a variety of surgical procedures. We hypothesized that the adult thymus is needed to sustain immune competence and overall health. METHODS We evaluated the risk of death, cancer, and autoimmune disease among adult patients who had undergone thymectomy as compared with demographically matched controls who had undergone similar cardiothoracic surgery without thymectomy. T-cell production and plasma cytokine levels were also compared in a subgroup of patients. RESULTS After exclusions, 1420 patients who had undergone thymectomy and 6021 controls were included in the study; 1146 of the patients who had undergone thymectomy had a matched control and were included in the primary cohort. At 5 years after surgery, all-cause mortality was higher in the thymectomy group than in the control group (8.1°/o vs. 2.8%; relative risk, 2.9; 95% confidence interval [CI], 1.7 to 4.8), as was the risk of cancer (7.4% vs. 3.7%; relative risk, 2.0; 95% CI, 1.3 to 3.2). Although the risk of autoimmune disease did not differ substantially between the groups in the overall primary cohort (relative risk, 1.1; 95% CI, 0.8 to 1.4), a difference was found when patients with preoperative infection, cancer, or autoimmune disease were excluded from the analysis (12.3% vs. 7.9%; relative risk, 1.5; 95% CI, 1.02 to 2.2). In an analysis involving all patients with more than 5 years of follow-up (with or without a matched control), all-cause mortality was higher in the thymectomy group than in the general U.S. population (9.0% vs. 5.2%), as was mortality due to cancer (2.3% vs. 1.5%). In the subgroup of patients in whom T-cell production and plasma cytokine levels were measured (22 in the thymectomy group and 19 in the control group; mean follow-up, 14.2 postoperative years), those who had undergone thymectomy had less new production of CD4+ and CD8+ lymphocytes than controls (mean CD4+ signal joint T-cell receptor excision circle [sjTREC] count, 1451 vs. 526 per microgram of DNA [P=0.009]; mean CD8+ sjTREC count, 1466 vs. 447 per microgram of DNA [PcO.001]) and higher levels of proinflammatory cytokines in the blood. CONCLUSIONS In this study, all-cause mortality and the risk of cancer were higher among patients who had undergone thymectomy than among controls. Thymectomy also appeared be associated with an increased risk of autoimmune disease when patients with preoperative infection, cancer, or autoimmune disease were excluded from the analysis. (Funded by the Tracey and Craig A. Huff Harvard Stem Cell Institute Research Support Fund and others). [ABSTRACT FROM AUTHOR]

Introduction: In recent years, robot-assisted thoracoscopic surgery (RATS) and video-assisted thoracoscopic surgery (VATS) has been more frequently preferred in the surgical treatment of mediastinal masses. The number of studies comparing VATS with RATS is limited. In our study comparing the surgical outcomes of RATS and VATS procedures, we tried to determine the ideal treatment method. Methods: Between 2016 and 2022, fifty-two patients who underwent minimally invasive surgical resection (VATS or RATS) for mediastinal mass were retrospectively analyzed. Results: Mediastinal mass resection was performed by RATS (n=29) or VATS (n=23). 57.7% (n=30) of the mediastial masses were localized in the anterior mediastinum. The most common postoperative pathology was thymoma (27%, n=14). There was no surgical mortality. Grade 1 and 2 complications developed in 6 (11.5%) patients according to the Clavien-Dindo classification. Conversion to open surgery was required in a total of 5 patients [VATS group (n=3), 13% versus RATS group (n=2), 6.9%, p=0.644]. The median length of hospital stay was five days [VATS; 4 days interquartile range (IQR): 3-6] versus RATS; 5.5 days (IQR: 4-8), p=0.081]. The median drainage time was four days [VATS; 3 (2-5) versus RATS; 4.5 (3-7), p=0.133], and the mean drainage amount was 110 mL (70-190) (p=0.162). There was no significant difference between the duration of the operation (for VATS; 75.7±18.4 min, for RATS; 73.5±18.0 min, p=0.674). Postoperative pain scores were similar [median 2.19 (1-3) for RATS and 2.20 (1-3) for VATS, p=1.00]. Conclusion: RATS and VATS are reliable procedures offering many advantages in treating mediastinal masses. Both procedures have similar results in terms of the complication rate, the length of the hospital stay, and duration of surgery. [ABSTRACT FROM AUTHOR]

Background: Surgery for thymic cancers is considered the key of curative treatment. Preoperative patients' characteristics and intraoperative features might influence postoperative outcome. We aim to verify short-term outcomes and possible risk factors for complications after thymectomy. Methods: We retrospectively investigated patients undergoing surgery for thymoma or thymic carcinoma in the period between January 1, 2008, and December 31, 2021, in our department. Preoperative features, surgical technique (open, bilateral VATS, RATS), intraoperative characteristics and incidence of postoperative complications (PC) were analyzed. Results: We included in the study 138 patients. Open surgery was performed in 76 patients (55.1%), in 36 VATS (26.1%) and in 26 RATS (36.1%). Resection of one or more adjacent organs due to neoplastic infiltration was required in 25 patients. PC appeared in 25 patients (52% Clavien–Dindo grade I, 12% grade IVa). Open surgery had a higher incidence of PC (p < 0.001), longer postoperative in-hospital stay (p = 0.045) and larger neoplasm (p = 0.006). PC were significant related to pulmonary resection (p = 0.006), phrenic nerve resection (p = 0.029), resection of more than one organ (p = 0.009) and open surgery (p = 0.001), but only extended surgery of more organs was confirmed as independent prognostic factor for PC (p = 0.0013). Patients with preoperative myasthenia symptoms have a trend toward stage IVa complications (p = 0.065). No differences were observed between outcomes of VATS and RATS. Conclusions: Extended resections are related to a higher incidence of PC, while VATS and RATS guarantee a lower incidence of PC and shorter postoperative stay even in patients that require extended resections. Symptomatic myasthenia patients might have a higher risk toward more severe complications. [ABSTRACT FROM AUTHOR]

Background Thoracoscopic thymectomy is increasingly performed for the treatment of early stage thymoma. It is characterized by shorter postoperative hospital stay, decreased intraoperative blood loss, and fewer complications compared with transsternal thymectomy. Unilateral video-assisted thoracic surgery (VATS) thymectomy can be easily performed from either side of the thorax, because thymus is located in the middle of mediastinum. However, the side that provides better outcomes remains controversial. The purpose of this study was to compare the efficacy of right and left approaches in performing unilateral thoracoscopic thymectomy for thymoma. Methods Consecutive patients affected by thymoma who underwent VATS thymectomy on either side between February 2001 and March 2020 were enrolled in the study. Clinicopathologic, surgical, and oncological outcomes were retrospectively analyzed and compared among the two surgical approaches. Results Unilateral VATS approaches were performed on 29 patients: 12 (41%) on the left side and 17 (59%) on the right side. The mean age was 63.1 ± 11.3 years and the female/male ratio was 1.73:1. The mean operative time and the hospital stay for the left-side VATS and right-side VATS groups were, respectively, 168 ± 49.5 versus 171 ± 47.9 minutes (p = 0.9) and 3 ± 1.03 days versus 3.65 ± 1.93 days (p = 0.7). Postoperative complications occurred in one patient (3%) for left-side VATS group and one patient (3%) for right-side VATS. The 5-year disease-free survival was comparable between two groups (p = 0.74). Conclusion Unilateral VATS thymectomy in patients with thymoma can be safely and effectively performed by experienced surgeons in either side of the thorax with equivalent oncological outcomes. [ABSTRACT FROM AUTHOR]

Introduction/Aims: Myasthenia gravis (MG) with muscle‐specific tyrosine kinase (MuSK) antibodies (MMG) is predominantly seen in women of childbearing age. Our objective in this study was to describe the course of MMG during pregnancy and within 6 months postpartum, and to document any effect on fetal health. Methods: A retrospective review was performed of medical records of patients with MMG seen in the Duke Myasthenia Gravis Clinic from 2003 to 2022. MMG patients with onset of MMG symptoms before or during pregnancy as well as within 6 months postpartum were reviewed. Results: A total of 14 pregnancies in 10 patients were included in our study cohort. Initial MG symptoms developed during pregnancy or within 6 months postpartum in six patients. Four patients had two pregnancies, three of whom developed MG during their first pregnancy. In the patients diagnosed before pregnancy, MG symptoms increased in five of eight patients during pregnancy or postpartum. Four patients required rescue therapy with plasma exchange or intravenous immunoglobulin during pregnancy or postpartum. One patient had a cesarean section after prolonged labor due to failure of progression. There were no other complications of pregnancy or delivery, and all infants were healthy at delivery. Discussion: As in non‐MuSK MG, women with MMG may also have worsening or may develop initial MG symptoms during pregnancy or within 6 months postpartum. More aggressive medical therapy may be required for pregnant patients with MMG. Further study is needed to identify the mechanism and risk of worsening of MMG during pregnancy or postpartum. [ABSTRACT FROM AUTHOR]

Background and purpose: This study investigated the postthymectomy outcomes and factors affecting the prognosis of thymomatous generalized myasthenia gravis (TGMG). Methods: Clinical records of 86 patients with TGMG who underwent thymectomy at our institution between 2012 and 2020 were retrospectively reviewed. Predictors of complete stable remission (CSR) and exacerbation were analyzed using multivariate regression analysis. Results: A total of 16 patients achieved CSR, four achieved pharmacological remission, six exhibited deterioration, and eight died of myasthenia gravis (MG; mean follow‐up = 75.1 months). Male sex (p = 0.049) and disease duration < 11.5 weeks before surgery (p = 0.003) were significant positive predictors of CSR. Onset age < 52.8 years and symptoms of ocular and limb muscle weakness had a higher CSR rate than onset age > 52.8 years (p = 0.056) and symptoms of bulbar muscles (p = 0.071). Female patients had a significant higher risk of exacerbation (p = 0.042). Conclusions: Male sex and disease duration < 11.5 weeks were independent predictors of CSR in TGMG postthymectomy. Onset age < 52.8 years and ocular and limb muscle weakness at onset were associated with a higher probability of achieving CSR than onset age > 52.8 years and bulbar muscle weakness. Female sex was an independent predictor of MG symptom exacerbation in TGMG postthymectomy. [ABSTRACT FROM AUTHOR]

This study aims to retrospectively compare the efficacy and safety of subxiphoid and subcostal arch thoracoscopic resection (SR) and the median sternotomy (MS) for thymoma with myasthenia gravis (MG) via propensity-matched analysis. We retrospectively analyzed 502 patients with thymoma and MG in Tangdu Hospital of the Fourth Military Medical University from December 2012 to December 2017. The patients were allocated to SR group (n = 424) and MS group (n = 78). Perioperative outcomes were compared between SR group and MS group by using propensity-matched analysis. All SR and MS operations were accomplished successfully. Most postoperative outcomes between the two groups showed no significant difference such as remission of MG and postoperative complication (P > 0.05). There were statistically significant differences between MS group and SR group in operation time [(116.3 ± 33.7) min versus (52.2 ± 31.3) min], intraoperative blood loss [(145.2 ± 26.7) mL versus (51.2 ± 10.3) mL], chest drainage duration (3.4 d versus 0 d), days of hospital-stay (5.2 d versus 2.7 d), patient satisfaction score (5.9 ± 2.3 versus 8.7 ± 1.2), the incidence of complications and pain scores, with all P values < 0.05. This study suggests that subxiphoid and subcostal arch thoracoscopic resection is a less invasive procedure with good safety and feasibility as compared with median sternotomy for thymoma with myasthenia gravis. [ABSTRACT FROM AUTHOR]

• Robotic thymectomy is safe and feasible in patients with myasthenia gravis. • The majority of the patients (82.4%) improved in MG manifestations after robotic thymectomy. • MG remission was observed in 47.8% of the patients after thymectomy (mean: 26.2 months). • Thymomatous patients had more frequently complications compared to nonthymomatous patients. The aim of this study was to investigate the surgical and long-term neurological outcomes of patients with acetylcholine-receptor-antibody-associated myasthenia gravis (AChR-MG) who underwent robotic thymectomy (RATS). We retrospectively analyzed the clinical-pathological data of all patients with AChR-MG who underwent RATS using the DaVinci® Robotic System at the MUMC+ between April 2004 and December 2018. Follow-up data were collected from 60 referring Dutch hospitals. In total, 230 myasthenic patients including 76 patients with a thymoma (33.0%) were enrolled in this study. Mean follow-up time, procedure time and hospitalization were, respectively 65.7 ± 43.1 months, 111±52.5 min and 3.3 ± 2.2 days. Thymomatous patients had significantly more frequently and more severe complications than nonthymomatous patients (18.4% vs. 3.9%, p <0.001). Follow up data was available in 71.7% of the included patients. The Myasthenia Gravis Foundation of America postintervention score showed any kind of improvement of MG-symptoms after RATS in 82.4% of the patients. Complete stable remission (CSR) or pharmacological remission (PR) of MG was observed in 8.4% and 39.4% of the patients, respectively. Mean time till CSR/PR remission after thymectomy was 26.2 ± 29.2 months. No statistical difference was found in remission or improvement in MGFA scale between thymomatous and nonthymomatous patients. RATS is safe and feasible in patients with MG. The majority of the patients (82.4%) improved after thymectomy. CSR and PR were observed in 8.4% and 39.4% of the patients, respectively, with a mean of 26.2 months after thymectomy. Thymomatous patients had more frequently and more severe complications compared to nonthymomatous patients. [ABSTRACT FROM AUTHOR]