Myasthenia gravis (MG) is a neuromuscular autoimmune disorder which clinically presents as muscular weakness and fatigue due to autoantibody formation against acetylcholine receptors (AChR), leading to their subsequent destruction. Due to the neuromuscular implications of MG, certain considerations must be taken into account when providing anesthesia to MG patients. In the following case report, we have outlined procedural considerations for the anesthetic management of a patient with MG undergoing deep sedation for an elective oral surgery in an outpatient setting, as well as a discussion of relevant literature.
Anesthesia ; Deep Sedation ; Fatigue ; Humans ; Muscle Weakness ; Myasthenia Gravis ; Outpatients ; Receptors, Cholinergic ; Surgery, Oral

BackgroundThe co-existence of myasthenia gravis (MG) and thymoma makes the surgical treatment more complicated and adjuvant radiation more controversial. The aim of this study was to investigate adjuvant radiotherapy for thymoma with MG after extended thymectomy.

MethodsA total of 181 patients with both MG and thymoma were recruited between 2003 and 2014 at Tongren Hospital, China. Among all the patients, 157 patients received radiation therapy after surgery (Group A); whereas the other 24 patients did not receive radiation therapy (Group B). According to the time that patients started mediastinal radiation therapy, we subdivided the 157 patients in Group A into subgroups (1-month subgroup, n = 98; 2-month subgroup, n = 7; and 3-month subgroup, n = 52). We then compared the effect of the mediastinal radiation therapy across these different groups using the survival rate, the rate of postoperative myasthenic crisis, and the complete stable remission (CSR) rate as the primary endpoints.

ResultsThere was a significant difference in the occurrence of postoperative myasthenic crisis between 1-month subgroup and Group B (χ = 4.631, P = 0.031). The rates of reaching CSR were 32.6% in 1-month subgroup, 25% in 3-month subgroup, and 22.7% in Group B, respectively. The overall survival rates of 1-month subgroup, 3-month subgroup, and Group B were 88.8%, 83.3%, and 77.3%, respectively. Analysis on the Kaplan-Meier survival curves demonstrated that within 8 years after surgery, there was no significant difference in aspects of overall survival and disease-free survival between 1-month subgroup and Group B, and between 3-month subgroup and Group B; over 8 years after surgery, the disease-free survival rates in 1-month subgroup, 3-month subgroup and Group B were 79.4%, 70.6%, and 55.3%, respectively.

ConclusionsAdjuvant radiation within 1 month after extended thymectomy may be helpful in controlling postoperative MG, such as decreasing the possibility of postoperative myasthenic crisis, and raising cumulative probabilities of reaching CSR.

Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Myasthenia Gravis ; surgery ; therapy ; Postoperative Period ; Radiotherapy, Adjuvant ; methods ; Thymectomy ; methods ; Thymoma ; surgery ; therapy ; Thymus Neoplasms ; surgery ; therapy ; Treatment Outcome

BACKGROUND: Thymoma is frequently associated with myasthenia gravis (MG). However, whether MG is a factor for the outcome of patients with thymoma following complete thymectomy remains unknown. The aim of this study is to investigate the effect of thymoma with MG prognostic factors. METHODS: A retrospective analysis of The Chinese Alliance for Research in Thymomas (ChART) database within 1992-2012 complete cases 875 cases, 20 years follow-up data analysis thymic tumor tissue type credits and MG, Masaoka staging and prognosis, postoperative adjuvant therapy and relationship with the prognosis of surgical removal of the way. RESULTS: Thymic tumor tissue type credit has correlation with MG, difference was statistically significant (χ²=24.908, P<0.001). MG: incidence of B2 type (58/178, 32.58%) > B3 type (65/239, 27.20%) > B1 (27/132, 20.45%) > AB (43/267, 16.10%) > type A, 10.17% (6/59), Masaoka stage has no correlation with MG (χ²=0.365, P=1.365). Survival analysis showed that the WHO classification, Masaoka stage associated with prognosis (P<0.05), and whether the merger MG (χ²=0.113, P=0.736), postoperative adjuvant radiotherapy (χ²=0.380, P=0.538) has nothing to do with the prognosis, postoperative adjuvant chemotherapy is associated with poor prognosis (χ²=14.417, P<0.001). Whether has nothing to do with the prognosis of the thymus resection (χ²=1.548, P=1.548), whether the whole correlated with the curative effect of thymus excision with MG (χ²=24.695, P<0.001). CONCLUSIONS Thymoma patients with MG and extended thymectomy have no correlation with prognosis. Extended thymectomy can improve the effect of MG patients.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; complications ; diagnosis ; surgery ; Prognosis ; Retrospective Studies ; Survival Analysis ; Thymoma ; complications ; Young Adult

The actual incidence of multiple thymoma is unknown and rarely reported because it remains controversial whether the cases represent a disease of multicentric origin or a disease resulting from intrathymic metastasis. In this case, a patient underwent total thymectomy for multiple thymoma with myasthenia gravis via bilateral video-assisted thoracic surgery. A well-encapsulated multinodular cystic mass, measuring 57 mm×50 mm×22 mm in the right lobe of the thymus, and a well-encapsulated mass, measuring 32 mm×15 mm×14 mm in the left lobe, were found. Both tumors were type B2 thymoma. Few cases of multiple thymoma with myasthenia gravis have ever been reported in the literature. We report a case of synchronous multiple thymoma associated with myasthenia gravis.
Humans ; Incidence ; Myasthenia Gravis* ; Neoplasm Metastasis ; Thoracic Surgery, Video-Assisted ; Thymectomy ; Thymoma* ; Thymus Gland

OBJECTIVETo explore the role of TNFα induced protein 3 interacting protein 1 (TNIP1) in the pathogenesis of myasthenia gravis (MG) among patients with thymoma.

METHODSFrom December 2014 to March 2015, 11 patients with MG associated thymoma (MGT) and 11 non-MG thymoma (NMGT) patients receiving thymectomy were selected. Thymus specimens were obtained during surgery, and peripheral venous blood samples were obtained before the surgery. For the MGT cohorts, peripheral venous blood samples were also collected at 3-6 months after the surgery. TNIP1 mRNA was determined with quantitative real-time PCR (qPCR), and its protein expression was evaluated by Western blotting.

RESULTSFor both thymus specimen and peripheral blood samples, the levels of TNIP1 mRNA and protein in the MGT group were both significantly lower than those of the NMGT patients (P<0.05). For the MGT group, the levels of TNIP1 mRNA and protein of the peripheral blood samples have increased following thymus resection compared with before the surgery.

CONCLUSIONReduced TNIP1 expression may have a role in the pathogenesis of MG for patients with thymoma. Thymectomy may help to recover the expression of TNIP1 among such patients.

Blotting, Western ; DNA-Binding Proteins ; genetics ; metabolism ; Female ; Gene Expression ; Genetic Predisposition to Disease ; genetics ; Humans ; Male ; Myasthenia Gravis ; complications ; genetics ; Reverse Transcriptase Polymerase Chain Reaction ; Thymectomy ; Thymoma ; complications ; surgery ; Thymus Neoplasms ; complications ; surgery

OBJECTIVETo evaluate the different pathological and clinical characteristics of thymomas with and without myasthenia gravis (MG) and to determine whether the presence of MG influences the prognosis in thymoma patients.

METHODSThe clinical data from 228 consecutive patients (median sternotomy were used in 153, video-assisted thoracoscopic themectomy were used in 75) operated on from January 1992 to December 2007 was analyzed retrospectively. These thymoma patients had been subdivided into two groups: thymoma with MG (n = 125) and thymoma without MG (n = 103). All thymic epithelial tumors were classified according to the WHO histologic classification and the Masaoka clinical staging system. The result was evaluated according to the Myasthenia Gravis Foundation of America's criterion. The clinical features of the 2 test was compared between the two groups by χ² test, and the survival were compared between the two groups by Cox analysis.

RESULTSThere were no peri-operative deaths. 19 cases were inoperable (6 in the group with MG, 13 without MG (χ² = 4.52, P = 0.035)). The proportions of type A and thymic carcinoma were 0 in the group with MG, 10.5% (11/103) and 11.6% (12/103) respectively in the group without MG. According to the Masaoka's clinical staging, in the group MG, 24.8% (31/125) patients were stage III and IV; in the group without MG, 33.0% (34/103) patients were stage III and IV. There was a significant difference between hyperplastic paraneoplastic thymus coexisting in 28.8% (36/125) patients with MG and only 5.8% (6/103) in patients without MG (χ² = 20.91, P = 0.000) Microthymoma was identified in the paraneoplastic thymus of 3 patients with MG. There were 198 patients followed up, the rate was 86.8% (198/228). There was no recurrence in patients with type A and a few patients with type AB, B1, B2, B3 thymoma and thymic carcinoma recurred. The actuarial 5- and 10-year survival rates were 89.3% and 81.2% for patients with MG respectively, and 90.0% and 78.9% for patients without MG respectively. Within 5 years postoperatively, 6 of 9 patients with MG died of myasthenia crisis, while 6 out of 7 deaths in patients without MG were attributable to inoperable tumors (stage IV) and thymic carcinoma.

CONCLUSIONSThe existence of myasthenia gravis has little influence on the prognosis of thymomas, but it is good for early diagnosis and treatment. Extended thymectomy should be performed to all patients with thymoma, no matter they have myasthenia gravis or not. The main cause of death is myasthenia crisis for thymoma patients with MG and stage IV and (or) thymic carcinoma for patients without MG.

Humans ; Myasthenia Gravis ; complications ; pathology ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Neoplasms, Glandular and Epithelial ; complications ; pathology ; surgery ; Postoperative Period ; Prognosis ; Retrospective Studies ; Sternotomy ; Survival Rate ; Thoracic Surgery, Video-Assisted ; Thymectomy ; Thymoma ; complications ; pathology ; surgery ; Thymus Neoplasms ; complications ; pathology ; surgery

A mediastinal mass was incidentally found on chest radiography in a 46-yr-old woman who had had myasthenia gravis (MG) for 2 months. Computed tomography revealed a 4-cm in size, well-defined, and lobulating mass with nodular calcification that was located in the thymus. Microscopically, the mass consisted of diffuse amorphous eosinophilic materials. These deposits exhibited apple-green birefringence under polarized light microscopy after Congo red staining. Immunohistochemical analysis revealed that they were positive for both kappa and lambda light chains and negative for amyloid A. A diagnosis of localized primary thymic amyloidosis was finally made. After thymectomy, the symptoms of MG were controlled with reduced corticosteroid requirements. Localized thymic amyloidosis associated with MG has not been reported to date.
Amyloidosis/complications/*radiography/*surgery ; Calcinosis/*radiography/*surgery ; Female ; Humans ; Immunoglobulin kappa-Chains/immunology ; Immunoglobulin lambda-Chains/immunology ; Mediastinum/radiography/surgery ; Middle Aged ; Myasthenia Gravis/*complications ; Radiography, Thoracic ; Thymectomy ; Thymus Gland/radiography/surgery ; Tomography, X-Ray Computed

There is continuous debate regarding the effectiveness of thymectomy in the treatment of non-thymomatous myasthenia gravis (MG). This systematic review was undertaken to determine whether thymectomy was effective in non-thymomatous MG. We retrieved articles published between January 1980 and September 2013. Sixteen cohort studies were included. Given the considerable heterogeneity, we used a descriptive method instead of statistical synthesis. The median relative rates (RRs) and their interquartile ranges were used to estimate the magnitude of benefit. Compared to conservatively treated MG patients, thymectomized patients had higher survival, clinical remission, pharmacologic remission and improvement rates, and RRs were 1.07 (1.01-1.17), 1.83 (0.82-2.99), 1.55 (1.22-1.95) and 1 (1.00-1.09), respectively. Subgroup analyses showed that patients with moderate to severe generalized MG benefited more from thymectomy, with RRs of survival and pharmacologic remission increasing to 1.35 (1.24-1.49) and 2.68 (1.73-4.17), respectively. These results suggested that thymectomy might be an effective procedure in non-thymomatous MG patients. The patients with moderate to severe generalized MG might benefit more. Taking into account the poor methodological quality of present studies, more well-designed prospective randomized controlled trials (RCTs) are still required to reach unequivocal conclusion.
Disease-Free Survival ; Humans ; Myasthenia Gravis ; mortality ; surgery ; Survival Rate ; Thymectomy

PURPOSE: To define the clinical characteristics of acute vertical strabismus in adults strabismus without known ocular and cranial external factors. METHODS: We performed a retrospective study of 72 adult patients who developed acute vertical strabismus without known ocular and cranial external factors such as trauma or operation and were followed up for at least 6 months. RESULTS: Undetermined cause (n = 41, 57%) was the most common etiology of acute vertical strabismus, followed by fourth cranial nerve palsy (n = 15, 20.8%), myasthenia gravis (n = 7, 9.7%), third cranial nerve palsy (n = 6, 8.3%), brain tumor (n = 2, 2.7%), and carotid-cavernous fistula (n = 1, 1.3%). The average vertical deviation at primary position was 7.2 prism diopter at initial visit. Thirty-eight (62.3%) patients recovered to orthophoria and 13 (21.3%) patients showed decreased level of diplopia. The average recovery period was 2.9 months. Ten cases remained as strabismus and 5 underwent surgery upon patient's request. CONCLUSIONS: Unknown cause was the most common diagonosis of adult acute vertical strabismus without known ocular and cranial external factors. In the present study, 62.3% of patients recovered to orthophoria and 83.6% recovered without surgical procedures.
Adult* ; Brain Neoplasms ; Diplopia ; Fistula ; General Surgery ; Humans ; Myasthenia Gravis ; Oculomotor Nerve ; Paralysis ; Retrospective Studies ; Strabismus* ; Trochlear Nerve Diseases ; Wounds and Injuries

BACKGROUNDThymectomy is an established treatment for myasthenia gravis (MG), and video-assisted thoracoscopic surgery (VATS) thymectomy has become an acceptable surgical procedure. This study aimed to compare the results of VATS thymectomy and open thymectomy and to identify the prognostic factors after thymectomy.

METHODSThe clinical data of 187 consecutive thymectomies performed between July 2000 and December 2009 were retrospectively reviewed; 75 open thymectomies and 112 VATS thymectomies. Clinical efficacy and variables influencing outcome were assessed by Kaplan-Meier survival curves and Cox proportional hazards regression analysis.

RESULTSThe operative blood loss in the VATS group was significantly less than that in the open group ((62.14 ± 55.43) ml vs. (137.87 ± 165.25) ml, P < 0.05). The postoperative crisis rate increased with the severity of preoperative MG and the prescription dose of anticholinesterase. Complete follow-up information of patients more than 12 months after the thymectomy was obtained on 151 cases, 89 cases from the VATS group and 62 cases from the open group, with a mean follow-up period of 59.3 months, range from 12 to 117 months. Complete stable remission (CSR) was the end point for evaluation of the treatment results. The overall five-year CSR rate was 57.5%. Two good prognostic factors were identified; preoperative prescription of anticholinesterase alone (P = 0.035) and non-thymomatous MG (P = 0.003). The five-year CSR rate of the ocular type of MG reached a high level of 67.4%.

CONCLUSIONSThymectomy can achieve good long-term CSR in MG, and VATS is an ideal alternative method. High-dose prescription of anticholinesterase and the advanced stage by Myasthenia Gravis Foundation of America (MGFA) classification have higher risks of postoperative crisis. Preoperative prescription of anticholinesterase alone and non-thymomatous MG are good prognostic factors. Thymectomy should also be considered for the ocular type of MG.

Adolescent ; Adult ; Aged ; Child ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; surgery ; Proportional Hazards Models ; Thoracic Surgery, Video-Assisted ; methods ; Thymectomy ; adverse effects ; methods ; Time Factors ; Treatment Outcome