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Gland Surgery Apr 2024Myasthenic crisis (MC) may occur after thymectomy in patients with myasthenia gravis (MG), but effective preventive interventions can reduce the occurrence of this... (Review)
Review
BACKGROUND
Myasthenic crisis (MC) may occur after thymectomy in patients with myasthenia gravis (MG), but effective preventive interventions can reduce the occurrence of this complication. Previous research on MC focused on risk factors, emergency treatment, etc., which was relatively scattered and did not form a comprehensive management framework. This study sought to retrieve and summarize the relevant evidence on the prevention and management of postoperative MC to provide a theoretical reference for clinical medical staff.
METHODS
According to the evidence pyramid model, relevant articles were retrieved from UpToDate, British Medical Journal (BMJ) Best Practice, World Health Organization (WHO), Scottish Intercollegiate Guidelines Network (SIGN), Guidelines International Network (GIN), Australian Joanna Briggs Institute (JBI) Healthcare Database, Medlive, PubMed, Cochrane Library, Embase, Web of Science, China National Knowledge Infrastructure (CNKI), and Wanfang. The types of evidence included clinical guidelines, expert consensus articles, clinical decisions, systematic reviews, and randomized controlled trials (RCTs). The quality evaluations were conducted using the Appraisal of Guidelines for Research and Evaluation II (AGREE II) evaluation tool for guidelines, the Australian JBI Evidence-Based Healthcare Center evaluation tool for expert consensus articles, the Critical Appraisal for Summaries of Evidence (CASE) evaluation tool for clinical decisions, the Assessment of Multiple Systematic Reviews (AMSTAR) evaluation tool for systematic reviews, and the Cochrane risk-of-bias tool for RCTs.
RESULTS
A total of 12 articles were included in this study, including three clinical guidelines, three expert consensus articles, three clinical decisions, two systematic reviews, and one RCT. From these articles, we summarized 39 pieces of evidence on the prevention and management of postoperative MC.
CONCLUSIONS
This study summarized the best evidence on the prevention and management of postoperative MC and provided to clinical staffs evidence-based clinical approaches to help reduce the incidence of this complication.
PubMed: 38720682
DOI: 10.21037/gs-24-90 -
Journal of Surgical Case Reports May 2024Thymomas are rare tumors originating from thymic tissue and rarely metastasize. They can be diagnosed either incidentally or symptomatically when compressing or invading...
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.
PubMed: 38706480
DOI: 10.1093/jscr/rjae263 -
EJNMMI Research May 2024Thymic cysts are a rare benign disease that needs to be distinguished from low-risk thymoma. [F]fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed...
BACKGROUND
Thymic cysts are a rare benign disease that needs to be distinguished from low-risk thymoma. [F]fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) is a non-invasive imaging technique used in the differential diagnosis of thymic epithelial tumours, but its usefulness for thymic cysts remains unclear. Our study evaluated the utility of visual findings and quantitative parameters of [F]FDG PET/CT for differentiating between thymic cysts and low-risk thymomas.
METHODS
Patients who underwent preoperative [F]FDG PET/CT followed by thymectomy for a thymic mass were retrospectively analyzed. The visual [F]FDG PET/CT findings evaluated were PET visual grade, PET central metabolic defect, and CT shape. The quantitative [F]FDG PET/CT parameters evaluated were PET maximum standardized uptake value (SUVmax), CT diameter (cm), and CT attenuation in Hounsfield units (HU). Findings and parameters for differentiating thymic cysts from low-risk thymomas were assessed using Pearson's chi-square test, the Mann-Whitney U-test, and receiver operating characteristics (ROC) curve analysis.
RESULTS
Seventy patients (18 thymic cysts and 52 low-risk thymomas) were finally included. Visual findings of PET visual grade (P < 0.001) and PET central metabolic defect (P < 0.001) showed significant differences between thymic cysts and low-risk thymomas, but CT shape did not. Among the quantitative parameters, PET SUVmax (P < 0.001), CT diameter (P < 0.001), and CT HU (P = 0.004) showed significant differences. In ROC analysis, PET SUVmax demonstrated the highest area under the curve (AUC) of 0.996 (P < 0.001), with a cut-off of equal to or less than 2.1 having a sensitivity of 100.0% and specificity of 94.2%. The AUC of PET SUVmax was significantly larger than that of CT diameter (P = 0.009) and CT HU (P = 0.004).
CONCLUSIONS
Among the [F]FDG PET/CT parameters examined, low FDG uptake (SUVmax ≤ 2.1, equal to or less than the mediastinum) is a strong diagnostic marker for a thymic cyst. PET visual grade and central metabolic defect are easily accessible findings.
PubMed: 38702532
DOI: 10.1186/s13550-024-01108-3 -
Therapeutic Advances in Neurological... 2024There are limited data on the real-world healthcare resource use (HCRU) and management costs of myasthenia gravis (MG) in England.
BACKGROUND
There are limited data on the real-world healthcare resource use (HCRU) and management costs of myasthenia gravis (MG) in England.
OBJECTIVE
This study aims to assess the burden of disease for patients with MG in England.
DESIGN
A retrospective, observational cohort study of adult patients diagnosed with MG, using data from the Hospital Episode Statistics data warehouse.
METHODS
Patients with a first-ever recorded diagnosis of MG between 30 June 2015 and 30 June 2020 were followed up until 30 June 2021 or death, whichever occurred first. Post-diagnosis patient characteristics, treatment patterns, HCRU, and costs were described. Costs were evaluated using National Health Service reference costs.
RESULTS
A total of 9087 patients with a median follow-up time of 2.9 years (range, 1.7-4.3 years) were included. The mean age at diagnosis was 66.5 years and 53% of the patients were male. A large proportion of patients (72.8%) were admitted as inpatients during follow-up with a mean number of 1.3 admissions. Patients hospitalized for MG-related complications spent a mean of 9.7 days per patient-year in the hospital. During follow-up, 599 (6.6% of the total cohort) and 163 (1.8%) patients had a record of rescue therapy with intravenous immunoglobulin (IVIg) and plasma exchange (PLEX), respectively. Rituximab was administered to 81 (0.9%) patients and 268 (2.9%) patients underwent thymectomy. In those patients receiving rescue therapy or rituximab, >10% received at least three cycles of the same treatment. The average annual cost of hospital admissions across all patients treated with IVIg, PLEX, and rituximab were £907,072, £689,979, and £146,726, respectively.
CONCLUSION
A majority of MG patients required hospitalization or accident and emergency attendance, resulting in high HCRU and costs. A subset of patients required rescue therapy (including IVIg and PLEX), rituximab administration, ventilation, or thymectomy.
PubMed: 38634003
DOI: 10.1177/17562864241237495 -
Journal of Cardiothoracic Surgery Apr 2024Cardiac 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...
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.
Topics: Male; Humans; Middle Aged; Thymectomy; Vena Cava, Superior; Positron Emission Tomography Computed Tomography; Clinical Deterioration; Heart Diseases; Hernia; Pneumonectomy
PubMed: 38627781
DOI: 10.1186/s13019-024-02713-y -
Cureus Mar 2024Spontaneous regression (SR) of thymoma is rare. We report a case of a surgically resected thymoma due to cystic changes owing to acute ischemic infarction with an...
Spontaneous regression (SR) of thymoma is rare. We report a case of a surgically resected thymoma due to cystic changes owing to acute ischemic infarction with an increased anti-acetylcholine receptor antibody level. A 61-year-old male underwent a computed tomography (CT) scan, which showed a 4.9 cm anterior mediastinal tumor and slight right pleural effusion. Blood test results indicated an elevated white blood cell count of 13300/mL. One month later, an enhanced CT scan at our hospital showed spontaneous mediastinal tumor regression to 3.7 cm and no pleural effusion. The tumor contained homogeneous low-density areas on enhanced CT, which showed high intensity on T2-weighted magnetic resonance imaging, indicating cystic changes. He had no symptoms of myasthenia; however, his anti-acetylcholine receptor antibody level was slightly elevated (0.4 nmol/L). Suspecting a thymoma, an extended total thymectomy through a median sternotomy was performed. Histopathological analysis confirmed the diagnosis of thymoma type B2 and Masaoka stage I. SR is due to acute intratumoral infarction. At two years postoperatively, no tumor recurrence or development of myasthenia gravis was observed. Thymomas should be included in the differential diagnosis of anterior mediastinal tumors that regress spontaneously with cystic changes, pleural effusion, and an elevated inflammatory response. Mature cystic teratoma rupture should be differentiated, but preoperative biopsy is often challenging owing to necrotic and fibrous tissues; therefore, early surgical resection is required for diagnosis and treatment.
PubMed: 38618410
DOI: 10.7759/cureus.56240 -
Cureus Mar 2024We present the case report of a patient with seronegative myasthenia gravis (MG) who was admitted for metabolic encephalopathy and acute on chronic hypoxic respiratory...
We present the case report of a patient with seronegative myasthenia gravis (MG) who was admitted for metabolic encephalopathy and acute on chronic hypoxic respiratory failure secondary to an MG crisis three days after an intravenous immunoglobulin treatment. In the intensive care unit, her MG was managed with intravenous immunoglobulin, plasmapheresis, prednisone, and pyridostigmine. During the course of her visit, she had urosepsis along with a left chest port that had cultured positive for and developed a right upper extremity deep vein thrombosis (UEDVT) and superficial thrombosis in the left upper extremity despite being on heparin therapy. She had a transient drop in platelets to below 150,000 that resolved within a day. We analyzed the variables of this case report and reviewed the literature of similar cases to elucidate the factors that may have led to the development of the UEDVTs. The patient had many factors in her past medical history that could have contributed to her thrombosis including morbid obesity and prior history of pulmonary embolisms. It is hypothesized that MG disturbs the endothelial cell lining through an increased inflammatory state that could also be a causative factor. There is no definitive way we could link MG as a causative factor due to a lack of testing to assess alteration in the integrity or functionality of her endothelium. A case report we reviewed showed a presentation of UEDVT in an MG patient due to a thymoma compressing the subclavian vein. However, this is not the case in this example due to the patient having a history of thymectomy. She was also at risk due to her hospital stay which led to immobility and placement of a central venous catheter. We conclude the formation of the UEDVT was likely a combination of these factors.
PubMed: 38618351
DOI: 10.7759/cureus.56086 -
Journal of Clinical Medicine Mar 2024: Robotic and thoracoscopic surgery are being increasingly adopted as minimally invasive alternatives to open sternotomy for complete thymectomy. The superior...
: 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. : 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. : A total of 68 robotic and 34 thoracoscopic thymectomies were included after propensity score matching. The robotic group had a higher total maneuverability score ( = 0.039), particularly in the peri-thymic fat dissection ( = 0.003) and peri-thymic fat exposure score ( = 0.027). Moreover, the robotic group had lower intraoperative adverse events ( = 0.02). No differences were found in residual disease. : Robotic thymectomy has shown better ergonomic maneuverability compared to thoracoscopy, leading to fewer intraoperative adverse events and comparable early oncological results.
PubMed: 38610606
DOI: 10.3390/jcm13071841 -
Revue Neurologique Apr 2024The European literature has reported high variability in the incidence and prevalence rates of myasthenia gravis (MG), but no specific epidemiological data for France...
BACKGROUND
The European literature has reported high variability in the incidence and prevalence rates of myasthenia gravis (MG), but no specific epidemiological data for France have been published. This study aimed to assess the incidence and prevalence rates of myasthenia gravis in France based on data extracted from the French National Health Insurance Claims Database (the SNIIRAM database).
METHODS
We conducted a retrospective repeated cross-sectional population study from 2008 to 2018 using a representative sample of the French population (Échantillon généraliste des bénéficiaires) covered by health insurance. We calculated the incidence, prevalence, and sex ratio of MG and screened for comorbidities associated with MG (standardized to the general population).
RESULTS
In total, 331 MG patients were identified between 2008 and 2018. The average incidence of MG in France was 50 per million person-years, while the mean prevalence was 465 per million people. The female-to-male ratio was 1.33. The Incidence of MG gradually increased from 40years of age for women and 60 for men. Thymoma was present for 5.1% of MG patients and a thymectomy was performed for 4.7%. Thyroid disease was the most prevalent autoimmune comorbidity, affecting approximately 8.5% of cases. MG patients had an increased cancer risk, with a standardized rate ratio of 2.38 (95% CI: 1.64-3.46).
CONCLUSION
The incidence and prevalence rates of MG are significantly higher than those previously reported in the literature and the incidence increases with age. The excess risk of cancer raises concerns for MG patients, in particular, concerning the management of immunosuppressive drugs.
PubMed: 38582663
DOI: 10.1016/j.neurol.2024.02.392 -
Journal of Cardiothoracic Surgery Apr 2024Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease, is a rare, self-limiting disease that predominantly affects children and young...
BACKGROUND
Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease, is a rare, self-limiting disease that predominantly affects children and young adults. Moreover, the disease is characterized by painless bilateral cervical lymphadenopathy in 95% of the patients. However, few reports are available on the Rosai-Dorfman disease of the thymus.
CASE PRESENTATION
We report a rare case of thymic Rosai-Dorfman disease detected using computed tomography. During a medical examination, a 50-year-old man underwent a chest computed tomography scan, which revealed an anterior mediastinal single mass with fat in the thymus. A thymectomy was performed to completely remove the tumor using a thoracoscopic technique due to a clinical suspicion of thymoma. Furthermore, Rosai-Dorfman disease was confirmed using histological and immunohistochemical analyses.
CONCLUSIONS
To the best of our knowledge, this is the sixth case of thymus-affecting solitary Rosai-Dorfman disease with histological and immunohistochemical evidence. Fat in the thymus, as was present in this case, has never been described in Rosai-Dorfman disease previously. Our results highlight the challenge of diagnosing this uncommon tumor before surgery, and more cases need to be reported to help with the preoperative diagnosis of such a rare tumor.
Topics: Male; Child; Humans; Middle Aged; Histiocytosis, Sinus; Tomography, X-Ray Computed; Mediastinal Diseases; Diagnosis, Differential; Neoplasms
PubMed: 38575975
DOI: 10.1186/s13019-024-02714-x