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Clinical Rheumatology Jul 2020The thymus plays an integral role in immune system regulation, modulating the development, diversity, and selection of T lymphocytes, a critical feature for the... (Review)
Review
The thymus plays an integral role in immune system regulation, modulating the development, diversity, and selection of T lymphocytes, a critical feature for the prevention of T cell-mediated autoimmunity. Thymoma is a rare tumor of the thymus. Autoimmune diseases and paraneoplastic syndromes such as myasthenia gravis, pure red blood cell aplasia, and systemic lupus erythematosus, although relatively uncommon, have been described in association with thymomas. Rare cases of post-thymectomy autoimmune related diseases, including systemic lupus erythematosus and pure red cell aplasia, have been reported in the literature. Here, we present the case of a 65-year-old male who developed systemic lupus erythematosus 2 years after thymectomy in the setting of thymoma-associated pure red cell aplasia.
Topics: Aged; Autoimmunity; Humans; Lupus Erythematosus, Systemic; Male; Radiography; Red-Cell Aplasia, Pure; T-Lymphocytes; Thymectomy; Thymoma; Thymus Gland; Thymus Neoplasms; Tomography, X-Ray Computed
PubMed: 32277368
DOI: 10.1007/s10067-020-05061-z -
Interactive Cardiovascular and Thoracic... Mar 2022Our goal was to evaluate the effect of thymectomy on the progression of thymolipomatous myasthenia gravis.
OBJECTIVES
Our goal was to evaluate the effect of thymectomy on the progression of thymolipomatous myasthenia gravis.
METHODS
An electronic search performed across PubMed, MEDLINE and Web of Science databases included all article types. We included 15 series comprising 36 cases that met specific criteria, including case reports or case series related to thymolipoma with a myasthenia gravis association, where thymectomy was cited as the primary intervention with postoperative reporting of the prognosis and articles written in the English language.
RESULTS
Our study included 17 men (47.2%) and 19 women (52.8%). Tumour sizes varied between 34 × 18 × 7 cm and 2.8 × 2.3 × 1.9 cm; the weight of the tumours ranged between 38 and 1780 g (mean 190, standard deviation 341). The surgical approaches were a median sternotomy in 29 patients (80.6%), a thoracotomy in 1 patient (2.8%), video-assisted thoracoscopic surgery in 2 patients (5.6%) and unreported approaches in 4 (11.1%) patients. The disease was entirely resolved with complete, stable remission in 5 patients (13.9%); symptoms were improved in 19 (52.8%) and stable in 10 patients (27.7%). We identified 2 groups of patients according to their improvement post-thymectomy (improved group and group with no change).
CONCLUSIONS
Although the cases were uncontrolled and did not demonstrate strong associations, they do support some hypotheses. We found a significant statistical difference between the 2 groups in terms of age, because younger patients tended to improve to a greater degree post-thymectomy. Also, we found that female patients with thymoma visible on the imaging scans were significantly associated with post-thymectomy myasthenia gravis improvement.
REGISTRATION NUMBER IN PROSPERO
CRD42020173229.
Topics: Female; Humans; Male; Myasthenia Gravis; Thymectomy; Thymoma; Thymus Neoplasms; Treatment Outcome
PubMed: 35362060
DOI: 10.1093/icvts/ivab295 -
The Annals of Thoracic Surgery Dec 2011
Topics: Female; Humans; Male; Thoracic Surgery, Video-Assisted; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 22115210
DOI: 10.1016/j.athoracsur.2011.08.004 -
Pediatric Surgery International May 2019A randomized controlled trial of thymectomy in myasthenia gravis demonstrated improved clinical outcomes in adults, but data surrounding juvenile cases, especially those...
PURPOSE
A randomized controlled trial of thymectomy in myasthenia gravis demonstrated improved clinical outcomes in adults, but data surrounding juvenile cases, especially those treated with minimally invasive approaches, are limited. Here, we review our experience with thoracoscopic thymectomy for juvenile myasthenia gravis (JMG) in the largest cohort to date.
METHODS
All cases of thymectomy for JMG in a single tertiary referral center between 2007 and 2018 were reviewed (N = 50). Patients underwent left thoracoscopic approach with extended dissection and without use of monopolar energy. Demographics, diagnostic criteria, and clinical classification, as well as surgical data were collected. Clinical status and medications were reviewed in follow-up.
RESULTS
The mean age at surgery was 10.5 ± 0.8 years. Ocular disease and generalized disease each comprised half of the cohort. No patients suffered complications or increased risk of morbidity or mortality with thymectomy. At any interval of follow-up through 3.5 years, 49.8% of patients were improved compared to their pre-operative presentation, and there was a significant trend towards decreased steroid use.
CONCLUSION
Thoracoscopic thymectomy is a safe treatment for juvenile myasthenia gravis in pediatric patients over a wide range of ages, body masses, and symptoms. Our experience adds evidence that pediatric patients likely benefit from thymectomy with improved clinical status and reduced medications.
Topics: Adolescent; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Length of Stay; Male; Myasthenia Gravis; Retrospective Studies; Tertiary Care Centers; Thoracoscopy; Thymectomy; Treatment Outcome
PubMed: 30729982
DOI: 10.1007/s00383-019-04441-0 -
Multimedia Manual of Cardiothoracic... Jul 2020Bleeding during thoracoscopic thymectomy is a potentially critical complication that necessitates either immediate effective thoracoscopic control or conversion to an...
Bleeding during thoracoscopic thymectomy is a potentially critical complication that necessitates either immediate effective thoracoscopic control or conversion to an open approach. It can be difficult to manage and the surgeon must respond quickly in order to keep the patient safe . In this video tutorial we present various forms of intraoperative bleeding that can occur during thoracoscopic thymectomy and discuss how to manage them in patients with myasthenia gravis. Our management techniques include compression, clipping, stitching, and safe conversion to an open approach.
Topics: Adult; Blood Loss, Surgical; Female; Hemostasis, Surgical; Humans; Intraoperative Complications; Male; Myasthenia Gravis; Thoracic Surgery, Video-Assisted; Thymectomy; Thymus Gland; Treatment Outcome
PubMed: 32820874
DOI: 10.1510/mmcts.2020.044 -
BMJ Case Reports Apr 2022Myasthenia gravis (MG) is the most common autoimmune disorder affecting the neuromuscular junction in the USA. It is not uncommon for these patients to have concomitant...
Myasthenia gravis (MG) is the most common autoimmune disorder affecting the neuromuscular junction in the USA. It is not uncommon for these patients to have concomitant autoimmune diseases including autoimmune thyroid disease. We describe here our method of performing a reproducible robotically assisted one-stage thymectomy and thyroidectomy. An African-American woman presented to our institution with a medical history of hypertension, morbid obesity, type 2 diabetes mellitus, symptomatic MG and symptomatic non-toxic substernal multinodular goitre. A one-stage minimally invasive right-sided robotic radical thymectomy and a transcervical total thyroidectomy with excision of the substernal goitre was successfully performed. The treatment of thyroid and thymus pathologies varies drastically from medical observation to surgery. This combined approach surgery clearly benefits the patient by offering similar operative time, fewer operative and postoperative recovery experiences, decreased anaesthesia risks associated with MG patients through fewer intubations, and a faster return to baseline function.
Topics: Diabetes Mellitus, Type 2; Female; Humans; Myasthenia Gravis; Robotic Surgical Procedures; Thymectomy; Thyroidectomy; Treatment Outcome
PubMed: 35459655
DOI: 10.1136/bcr-2022-249292 -
Indian Journal of Pathology &... 2022Myasthenia gravis (MG) is a prototypic T-cell-dependent antibody-mediated autoimmune disease that leads to ocular or generalized muscular weakness. The disease is most...
BACKGROUND
Myasthenia gravis (MG) is a prototypic T-cell-dependent antibody-mediated autoimmune disease that leads to ocular or generalized muscular weakness. The disease is most commonly caused by antibodies to the acetylcholine receptors, often with underlying thymic pathology.
AIMS
This study is aimed at analyzing the pathological spectrum of the excised thymuses in patients with myasthenia.
MATERIALS AND METHODS
This was a retrospective 10-year study of 68 thymectomy specimens performed as a part of the treatment of patients with MG.
STATISTICAL ANALYSIS
Nil.
RESULTS
There were 47 males and 21 females (male to female ratio of 2.2:1) with a mean age of 41 years. Only three patients presented with ocular myasthenia. The thymus was normal in 9 patients (13.2%) and atrophic in 17 patients (25%). Follicular hyperplasia and thymomas were seen in 6 and 36 patients, respectively.
CONCLUSION
The thymectomies performed in patients of MG had a fairly variable spectrum on histology; the thymic tumors were predominantly of the cortical phenotype.
Topics: Adolescent; Adult; Aged; Female; Histological Techniques; Humans; Male; Middle Aged; Myasthenia Gravis; Retrospective Studies; Thymectomy; Thymoma; Young Adult
PubMed: 35074977
DOI: 10.4103/IJPM.IJPM_935_20 -
Surgery Oct 2020We compared the clinical outcomes and cost-efficiency of surgical approaches (sternotomy-open, video assisted thoracoscopic surgery, and robotic assisted thoracic...
BACKGROUND
We compared the clinical outcomes and cost-efficiency of surgical approaches (sternotomy-open, video assisted thoracoscopic surgery, and robotic assisted thoracic surgery) for thymectomy.
METHODS
This is a retrospective review of 220 consecutive patients who underwent thymectomy between January 1, 2007, and January 31, 2017. Surgical approach was determined by the surgeon, but we only included cases that could be resected using any of the 3 approaches.
RESULTS
Open approach was used in 69 patients, whereas minimally invasive technique was used in 151 (97, video assisted thoracoscopic surgery; 54, robotic assisted thoracic surgery). Open surgery was associated with greater total hospital cost ($22,847 ± $20,061 vs $14,504 ± $10,845, P < .001). Open group also revealed longer duration of intensive care unit (1.2 ± 2.8 vs 0.2 ± 1.3 days, P < .001) and hospital stay (4.3 ± 4.0 vs 2.0 ± 2.6 days, P < .001). There were no differences in major adverse clinical outcomes. Long-term recurrence-free survival after resection of thymoma was similar between the groups.
CONCLUSION
Minimally invasive techniques were equally efficacious compared with the open approach in the resection of the thymus. Additionally, their use was associated with decreased hospital duration of stay and reduced cost. Hence the use of minimally invasive approaches should be encouraged in the resection of thymus.
Topics: Adult; Comparative Effectiveness Research; Cost-Benefit Analysis; Disease-Free Survival; Female; Hospital Costs; Humans; Length of Stay; Male; Middle Aged; Postoperative Complications; Retrospective Studies; Robotic Surgical Procedures; Thoracic Surgery, Video-Assisted; Thymectomy; Thymoma; Thymus Neoplasms; Treatment Outcome
PubMed: 32641277
DOI: 10.1016/j.surg.2020.04.037 -
Sultan Qaboos University Medical Journal Aug 2017(MG) is an autoimmune neuromuscular disorder which is twice as common among women, often presenting in the second and third decades of life. Typically, the first... (Review)
Review
(MG) is an autoimmune neuromuscular disorder which is twice as common among women, often presenting in the second and third decades of life. Typically, the first trimester of pregnancy and first month postpartum are considered high-risk periods for MG exacerbations. During pregnancy, treatment for MG is usually individualised, thus improving its management. Plasma exchange and immunoglobulin therapies can be safely used to treat severe manifestations of the disease or myasthaenic crises. However, thymectomies are not recommended because of the delayed beneficial effects and possible risks associated with the surgery. Assisted vaginal delivery-either vacuum-assisted or with forceps-may be required during labour, although a Caesarean section under epidural anaesthesia should be reserved only for standard obstetric indications. Myasthaenic women should not be discouraged from attempting to conceive, provided that they seek comprehensive counselling and ensure that the disease is under good control before the start of the pregnancy.
Topics: Anesthesia, Epidural; Cesarean Section; Contraindications, Procedure; Delivery, Obstetric; Disease Progression; Female; Humans; Immunoglobulins; Myasthenia Gravis; Plasma Exchange; Pregnancy; Pregnancy Complications; Thymectomy
PubMed: 29062547
DOI: 10.18295/squmj.2017.17.03.002 -
Interactive Cardiovascular and Thoracic... Jan 2021This study analysed the patterns of extraction ranges, characteristics, advantages and disadvantages of median sternotomy (MS) and subxiphoid (SX) approaches for...
OBJECTIVES
This study analysed the patterns of extraction ranges, characteristics, advantages and disadvantages of median sternotomy (MS) and subxiphoid (SX) approaches for extended thymectomy.
METHODS
This study included patients with anterior mediastinum tumour and myasthenia gravis who underwent extended thymectomy at our institution between 2015 and 2018. There were 5 MS and 6 SX extended thymectomy surgeries with the VINCENT software. On preoperative computed tomography, the thymus area and fat tissue surrounding the thymus, which were planned for extraction, were traced using VINCENT (Ver. 4.0). We then constructed three-dimensional images and calculated the volumes. Evaluation of the extended thymectomy approach based on the residual fat tissue was required to determine the area of extended thymectomy.
RESULTS
No significant differences in operation time (min) [SX: 197.3 ± 34.0, MS: 206.6 ± 91.4, drainage duration (days), SX: 2.2 ± 1.0, MS: 2.2 ± 0.4, hospital stay (days), SX: 11.8 ± 1.2, MS: 13.4 ± 2.1, residual rate (%), SX: 29.9 ± 17.5, MS: 58.7 ± 18.0 (P = 0.0519)] were observed between the 2 groups. Bleeding was significantly lower for SX than for MS. The residual rate was lower for SX than for MS.
CONCLUSIONS
Considering the amount of the residual fat tissue, the SX approach allows an adequate dissection area for extended thymectomy compared with the MS approach.
Topics: Adult; Disease Progression; Humans; Length of Stay; Male; Mediastinal Neoplasms; Mediastinum; Middle Aged; Myasthenia Gravis; Operative Time; Sternotomy; Thoracic Surgery, Video-Assisted; Thymectomy; Thymoma; Thymus Neoplasms; Young Adult
PubMed: 33236060
DOI: 10.1093/icvts/ivaa242