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PubMed2001

Thymectomy by thoracoscopic approach in myasthenia gravis.

BACKGROUND: A series of 25 thoracoscopic thymectomies performed in the Department of General Surgery and Liver Transplantation of the Fundeni Clinical Institute between April 1999 and April 2000 is analyzed. METHODS: Thoroscopic thymectomies were performed on 4 male patients (16%) and 21 female patients (84%), aged between 8 and 60 years. RESULTS: The mean operative time was 90 (+/-15) min. There were no conversions to open thymectomy. Mortality was nil, and morbidity consisted of one minor postoperative right pneumothorax probably related to a injury to right mediastinal pleura that was not observed intraoperatively. Hospital stay ranged from 2 to 4 days, with a mean of 2.28 days. The patients were transferred to the neurological department and they were usually discharged after 1 more day. CONCLUSIONS: Postoperatively, all patients had clinical improvement of their disease both in symptoms and medication requirements, but a longer follow-up is necessary. The results are edifying regarding the very low morbidity, the lack of mortality, the acceptance of the patients, and the short hospitalization.

Adolescent↗
PubMed1979

Myasthenia gravis, thymectomy and serum thymic hormone activity.

Serum thymic hormone activity was measured in 36 patients with myasthenia gravis and in 10 control subjects from each age decade. In all 25 patients under 50 years of age results were within, or close to, the normal range. Activity at levels considered normal for juveniles was detected in 10 of the 11 older patients whereas levels normally decline in older subjects. One week after thymectomy, 13 of 17 patients (76 per cent) had no demonstrable serum thymic hormone activity. However, 10 months or longer after thymectomy only five patients (30 per cent) lacked thymic hormone activity in the serum. There was a significant correlation between clinial improvement and sustained lowering of serum thymic hormone activity after thymectomy.

Adolescent↗
PubMed1980

The current role of thymectomy for myasthenia gravis.

Myasthenia gravis is an autoimmune disease characterized by muscle weakness and fatigability due to a reduction in available acetylcholine receptors at the neuromuscular junction. Treatment with anticholinesterase drugs and corticosteroids has improved the prognosis for patients with this disease. However, controversy continues concerning the indications for thymectomy. During a 9 year period, 27 patients who underwent thymectomy by median sternotomy were reviewed. Eighty-one percent of these patients benefited from the procedure. Clinical improvement did not correlate with age, sex, duration of symptoms, severity of disease or thymic histology, This suggests that the indications for thymectomy should be liberalized to include most patients with generalized myasthenia who fail to respond readily to conventional medical therapy. The importance of immunosuppression nd plasmapheresis in the therapy of myasthenia gravis awaits further delineation of the immune defect associated with the disease.

Adolescent↗
PubMed1982

Transcervical thymectomy for thymoma in myasthenia gravis.

The results of thymectomy performed through a transcervical approach in 37 myasthenic patients with thymomas is reported and compared with results in 97 patients who had thymomas removed through a transsternal approach. In 29 of the former patients the thymomas were unsuspected and found at the time of thymectomy, and in 8 a preoperative chest roentgenogram was suspicious for the presence of a tumor. In the transcervical group there were only 4 invasive thymomas, while in the transsternal group there were 32. In the transcervical group there was no evidence of recurrence in the patients with unsuspected thymomas, and one recurrence in the group with suspected thymomas. In the transsternal group eight patients had known recurrence or persistent disease. The transcervical approach seems appropriate for the removal of small thymomas discovered at the time of thymectomy or suspected from the preoperative work-up.

Adult↗
PubMed1994

Thymectomy for the myasthenia gravis patient: factors influencing outcome.

Thymectomy is a therapeutic option for patients with myasthenia gravis with moderate to severe disability. To document the efficacy of thymectomy coupled with medical therapy to treat this disease and to identify clinical factors that influence outcome, the clinical courses of all 46 patients (12 male and 34 female; mean age, 30 +/- 16 years) with myasthenia gravis who underwent thymectomy through a median sternotomy at a single institution over a 21-year period were reviewed. Clinical staging was determined preoperatively, at 1 month, 6 months, and 12 months postoperatively, and at last follow-up (mean time, 75 months postoperatively) using the Oosterhuis classification. Changes in severity of illness were graded as "deteriorated," "unchanged," "improved," or "much improved." Preoperative Oosterhuis classification was 3.3 +/- 1.1 and at last follow-up, 1.4 +/- 1.2 (p = 0.022). At last follow-up, 40 patients (87%) were in the improved or much improved category, and 6 patients were in the deteriorated or unchanged category. Status at 1 month, 6 months, and 12 months after operation predicted outcome at last follow-up visit (p = 0.007, p = 0.005, and p = 0.001, respectively). Clinical factors that positively influenced outcome were age less than 45 years (p = 0.004), female sex (p = 0.0309), and preoperative stage (p = 0.021).

Acetylcholine↗
PubMed1996

One hundred consecutive thymectomies for myasthenia gravis.

BACKGROUND: Between June 1997 and November 1993, 100 consecutive thymectomies for myasthenia gravis were performed at University of North Carolina Hospitals in Chapel Hill. METHODS: A consistent, planned protocol involving preoperative, intraoperative, and postoperative care was followed. All thymectomies were performed through a median sternotomy with removal of all visible thymus and perithymic fat in the anterior mediastinum. RESULTS: There was no perioperative mortality or longterm morbidity. Mean postoperative hospital stay was 6.3 days (range, 3 to 18 days). Ninety-six percent of the patients were extubated the day of the operation, and all patients were extubated within 24 hours. Mean postoperative intensive care unit stay was 1.2 days (range, 1 to 4 days). After a mean follow-up of 65 months (range, 1 to 199 months), 78% of all patients are improved by at least one modified Osserman classification when their current status is compared with their worst preoperative disease severity. In fact, 69% of patients with mild disease preoperatively (class I, II, or III maximal severity) are in pharmacologic remission (asymptomatic without regular medication), whereas 29% of patients with severe disease (class IV or V) are in remission (p = 0.0001). CONCLUSIONS: Our programmatic approach to thymectomy through a sternotomy has shown minimal morbidity and mortality. It is beneficial to myasthenics at both ends of the age and severity spectrum.

Adult↗
PubMed1986

Asymptomatic oligoclonal CSF IgG and progressive increase of intrathecal IgG synthesis in a patient with myasthenia gravis treated with thymectomy--a 4-years follow-up.

A young woman is described who had myasthenia gravis which was favourably influenced by thymectomy. Although there was no evidence for another neurological disease over 7.5 years of clinical observation and computed tomography as well as visual evoked response were normal, CSF studies over 0.5 years prior to and 3.5 years after thymectomy revealed persistent mononuclear pleocytosis, high CSF IgG index, oligoclonal IgG bands in CSF, and increasing IgG synthesis rate within the CNS, reflecting a continuous local humoral immune response. CSF/serum ratios of antibodies to acetylcholine receptors (AChR) were continuously lower than CSF/serum IgG ratios, contradicting intrathecal AChR antibody production. Proportion of total T cells was slightly higher in CSF than peripheral blood, while active T cells were lower in CSF. CSF lymphocytes did not proliferate on PHA stimulation but responded in allogeneic mixed lymphocyte culture. B cells were 4% in CSF and 4.5% in peripheral blood, but 227 IgG, 0 IgA and 0 IgM producing cells were detected among 20 X 10(3) CSF lymphocytes, compared to 5, 4 and 0 in 20 X 10(3) peripheral blood lymphocytes. This patient represents an in vivo 'experiment' regarding influence of thymectomy on CSF compartment constituents. The present study also shows that an individual can be clinically healthy despite continuous and pronounced intrathecal immune response.

Adult↗
PubMed1983

Allograft rejection in Xenopus laevis following larval thymectomy.

Xenopus laevis thymectomized at stages 41 through 49 accept first set allografts, while animals thymectomized at stage 51 or older reject allografts in times similar to intact animals. However, thymectomy at progressively earlier stages results in a greater proportion of animals unable to reject second set grafts. In some animals, the allograft response remains deficient even after multiple challenges. The results indicate that alloreactive cells are thymus dependent, and suggest that the thymus processes precursor thymocytes starting upon its formation at around stage 41. The processed cells, competent to respond to alloantigens, are released to the periphery almost immediately. While an increasing pool of processed T cells accumulates during stages 41-49, the persistent defective allograft response displayed by animals thymectomized during these stages suggests that early thymectomy may leave a population of alloreactive cells qualitatively defective in some subpopulation necessary for normal allograft responses, or that any residual cells processed prior to thymectomy are capable of only limited clonal expansion.

Animals↗
PubMed1986

Effects of X-irradiation and thymectomy on the immune response of the marine teleost, Sebastiscus marmoratus.

Effects of X-irradiation and thymectomy on the immune response of the rock fish, S. marmoratus were studied. Animals were thymectomized and irradiated at the dose of 2000 R. In non-thymectomized and irradiated fish, injected with SRBC one week after irradiation, antibody production was completely suppressed and they required twice the time for rejection of scales allografted three days after irradiation. On the other hand, fish which were irradiated four days after thymectomy and injected one week after irradiation, showed a fairly high level of antibody, although, in the allograft rejection, no significant difference was observed when compared to the irradiated fish. Furthermore, animals thymectomized, irradiated and autoimplanted showed higher production of antibody when immunized three months after irradiation compared to matched controls. In the same manner, in the allograft rejection, a slight restoration was found in fish reconstituted with a non-irradiated thymus. From these results, it is considered that, as in mammals, the adult thymus of fish plays an important role in recovery from the damage to the immune system caused by irradiation. Furthermore, a combination of X-irradiation and thymectomy suggests that suppressor T-cells which are resistant to X-rays exist in the fish thymus.

Animals↗
PubMed1991

Anti-acetylcholine receptor antibodies decrease after thymectomy in patients with myasthenia gravis. Clinical correlations.

Clinical course and changes in serum levels of antibodies to the acetylcholine receptor (a-AChR) were followed in 82 patients with myasthenia gravis during a period of 1-8 years after thymectomy. Decrease in a-AChR immediately after thymectomy was influenced by changes in a total IgG. Immunosuppressive medication affected serum a-AChR at all points of time. In a subgroup of 41 patients without thymoma who had no immunosuppressive drugs, there was a steady decrease in a-AChR concomitant with clinical improvement from 6 weeks after thymectomy.

Adolescent↗
PubMed1994

Thymectomy and malignancy.

Three hundred ninety patients who underwent thymectomy for myasthenia gravis (MG) were followed up to investigate the development of associated malignancies. There were 102 patients with thymoma and 288 without thymoma. Malignant neoplasms were detected in ten patients, four of whom already had the tumor at the time MG was diagnosed. Thus, malignancy developed after thymectomy in six patients. Malignant fibrous histiocytoma (MFH) developed in three patients, as well as gastric cancer, gastric leiomyosarcoma, rectal cancer, liver cancer, lung cancer, breast cancer, and thymic carcinoid in one patient each. Nine of the ten malignancies developed in the thymoma group, and only one in the non-thymoma group. The predicted number of patients with malignancy was 2.63 in the thymoma group and 2.65 in the non-thymoma group. Our findings suggest that the presence of thymoma facilitates the occurrence of extrathymic malignancy, and that thymectomy never enhances the occurrence of malignancy but possibly inhibits it.

Adolescent↗
PubMed2004

Experience with programmed steroid treatment with thymectomy in nonthymomatous myasthenia gravis.

BACKGROUND: The benefit of thymectomy in myasthenia gravis management is recognized but the perioperative course can fluctuate. The goal of this study was to assess the feasibility and clinical benefit of dose-escalated steroid therapy with thymectomy for nonthymomatous myasthenia gravis. METHODS: We reviewed the records of 69 myasthenia gravis patients who were followed up after undergoing transsternal thymectomy with extended anterior mediastinal dissection in our hospital between 1976-2000. Forty-eight patients in the programmed treatment group who had dose-escalated and de-escalated steroid therapy during the perioperative period comprised 17 patients with ocular myasthenia gravis and 31 patients with generalized myasthenia gravis. Clinical benefits and clinical remission, which was diagnosed when the patients were symptom-free without medications for at least 1 year, were compared with those of 21 patients in the occasional treatment group who received medications occasionally over the perioperative period. RESULTS: Postoperative respiratory failure and myasthenic crisis did not occur in the programmed treatment group but did occur in 6 patients in the occasional treatment group. Remission rates in the programmed treatment group (mean follow-up, 6.4 years) were 30% at 3 years, 38% at 5 years, and 46% at 10 years; rates in the occasional treatment group (mean follow-up, 9.6 years) were 25% at 3 years, 25% at 5 years, and 45% at 10 years. CONCLUSIONS: Programmed steroid therapy in patients with nonthymomatous myasthenia gravis is feasible and it provides clinical benefit when fluctuating symptoms occur during the perioperative period.

Adult↗
PubMed2006

Transsternal thymectomy for myasthenia gravis: surgical outcome.

BACKGROUND: Transsternal thymectomy is well established in the treatment of myasthenia gravis. Surgical strategy and patient selection, however, remain controversial. This paper reports the experience of a supraregional center looking into the influence of different preoperative risk factors on surgical outcome. METHODS: Between 1987 and 1998, 85 consecutive patients (65 female; mean age, 30.5 years) were enrolled. The mean preoperative Myasthenia Gravis Foundation of America stage was 2.3. The preoperative, early, and late follow-up data were analyzed retrospectively. RESULTS: Mean follow-up was 4.5 years (range, 1 to 14; 376 follow-up years). Mean duration of disease before surgery was 31 months. There were no operative or late deaths. Eight patients had major complications. Seventy-two patients were free from any early or late morbidity. Immunosupression therapy patients were more prone to have complications. At their last visit, 15 patients (17%) were in complete remission; 67 reported clinical improvement. Sixty-three were asymptomatic or in stage I on no or minimal treatment. Remission and clinical improvement were not predicted by patient's age, sex, duration of disease prior to surgery, thymic pathology, or antiacetylcholine receptor antibodies titer. Greater severity of symptoms before surgery was associated with greater subsequent improvement. Remission at 1 year predicted remission at the end of follow-up. CONCLUSIONS: Transsternal thymectomy for myasthenia gravis is safe and effective. It benefits most patients, especially those with severe symptoms. The long interval from diagnosis to surgery demonstrates it is never too late for thymectomy.

Adolescent↗
PubMed2006

Outcomes after 151 extended transcervical thymectomies for myasthenia gravis.

BACKGROUND: The ideal operative technique for thymectomy in myasthenia gravis (MG) remains controversial. We present the largest series of extended transcervical thymectomy to provide outcomes data to compare with transsternal procedures. METHODS: A retrospective chart review/interview was made of 164 patients operated upon from 1992 to 2004. Complete remission (CR) was defined as asymptomatic off medication for 6 months or asymptomatic on low-dose single-drug therapy (< or = 10 mg/d prednisone or < or = 150 mg/d azathioprine). A modified Osserman classification based upon the Myasthenia Gravis Foundation of America quantitative disease severity score was employed. RESULTS: The overall complication rate was 7.3%, and nearly all procedures were outpatient. Mean age at surgery was 43 years, and mean preoperative Osserman class was 2.3 (21% class 1; 39% class 2; 28% class 3; 12% class 4). Mean length of follow-up was 53 months. Mean postoperative Osserman class was 1.0. Nineteen percent of patients failed to improve. The crude cumulative CR rate was 37% (n = 58). Kaplan-Meier estimates of CR were 43% and 45% at 3 and 6 years, respectively. On multivariate analysis, only preoperative disease severity was significantly (inversely) associated with Kaplan-Meier CR rates. Longer-term follow-up (83 months) of only the earlier patients shows preserved CR rates (46%). CONCLUSIONS: This largest series of extended transcervical thymectomy for MG confirms that the 5-year Kaplan-Meier CR rate is comparable with that obtained after transsternal procedures. Patients with less severe disease have higher CR rates. Complete responses are durable, as the CR rate remains stable with extended follow-up.

Adolescent↗
PubMed2005

Myasthenia gravis appearing after thymectomy for thymoma.

OBJECTIVE: A few thymoma patients without myasthenia gravis (MG) have been observed to develop MG after total removal of the thymoma (postoperative MG). However, the cause of this is not yet known because of the rarity of postoperative MG patients. This study evaluated the clinical characteristics of the 8 postoperative MG patients. METHODS: We compiled 1089 thymoma patients treated between 1990 and 1994 in 115 institutes in Japan, and found 8 cases of postoperative MG. RESULTS: Postoperative MG was found in 8 (0.97%) of 827 thymoma patients without preoperative MG. The postoperative MG patients included 1 male and 7 females, with a mean age of 50.5+/-15.0 years. The thymoma was completely resected in all cases. The surgical method used was extended thymectomy in 2 cases and thymothymectomy in 6 cases. There were 2 cases (0.7%) of postoperative MG in the extended thymectomy group (n = 275), 6 (1.9%) in the thymothymectomy group (n = 321), and none in the tumor resection group (n = 137). The interval between thymectomy and the onset of postoperative MG varied (6 days-45 months, 19.3+/-16.5 months). The type of MG was ocular in 2 cases and general in 5 cases, according to the modified Osserman classification. The postoperative MG was responsive to anti-cholinesterase compounds and/or steroids. The improvement rate was 86%. CONCLUSIONS: Postoperative MG was present in about 1% of the patients who underwent total thymoma resection. Resection of the thymus gland does not prevent postoperative MG.

Adult↗
PubMed2006

Complete stable remission after extended transsternal thymectomy in myasthenia gravis.

OBJECTIVE: This study sought to determine the efficacy and prognostic factors of extended transsternal thymectomy as a treatment for myasthenia gravis (MG). METHODS: Medical records of 147 patients who underwent extended transsternal thymectomy for MG from January 1991 to December 2002 were reviewed retrospectively. The complete stable remission (CSR) rate and prognostic factors for CSR were assessed in 106 female and 41 male patients. RESULTS: The median age was 35 years (range 11-75 years). Ninety-eight patients had non-thymomatous MG and 49 patients had thymomatous MG. The median follow-up time was 89.7 months (range 12-167 months). Both non-thymomatous MG and thymomatous MG exhibited significant differences in population characteristics and CSR rates (29.6% vs 13.3% at 5 years, 45.2% vs 27.7% at 10 years, p = 0.022). Steroid therapy (hazard ratio: 0.234, p = 0.003) was a poor prognostic factor, while early onset (hazard ratio: 3.519, p = 0.048) was a good prognostic factor for CSR in non-thymomatous MG. In contrast, steroid therapy (hazard ratio: 0.061, p = 0.034) was poor prognostic factor for thymomatous MG. CONCLUSIONS: Extended transsternal thymectomy is a good treatment tool to achieve CSR in MG. Thymomatous MG and non-thymomatous MG were significantly different in patient characteristics and prognosis. Prognostic factors were steroid therapy and age of onset in non-thymomatous MG, and steroid therapy in thymomatous MG.

Adolescent↗
PubMed2004

Prognostic factors for myasthenic crisis after transsternal thymectomy in patients with myasthenia gravis.

OBJECTIVE: The purpose of this study was to assess which clinical features of patients with myasthenia gravis predict postoperative respiratory problems due to myasthenic crisis after transsternal thymectomy. METHODS: One hundred twenty-two patients who underwent transsternal thymectomy in our institute were analyzed retrospectively. Fourteen of those experienced myasthenic crisis and required prolonged (48 hours or more) postoperative mechanical ventilation. The following factors were evaluated: sex, age, body mass index, grade of symptom, disease interval, existence of thymoma, history of preoperative crisis, doses of anticholinesterase drugs, steroid use, pulmonary function, serum anti-acetylcholine receptor antibody, history of pulmonary disease, presence of other disease, operation time, and blood loss. RESULTS: Univariate analysis revealed preoperative bulbar symptoms (odds ratio = 14.246, P =.001), history of preoperative myasthenic crisis (7.091,.018), and preoperative serum level of anti-acetylcholine receptor antibody > 100 nmol/L (4.098,.044) were prognostic factors for postoperative myasthenic crisis. On the other hand, multivariate logistic regression analysis revealed preoperative bulbar symptoms (33.333,.004), preoperative serum level of anti-acetylcholine receptor antibody > 100 nmol/L (7.874,.020), and intraoperative blood loss > 1000 mL (18.519,.048) were prognostic factors for postoperative myasthenic crisis. CONCLUSIONS: In this study, postoperative myasthenic crisis after transsternal thymectomy in 122 patients with myasthenia gravis was affected by the existence of preoperative bulbar symptoms, history of preoperative myasthenic crisis, preoperative serum level of anti-acetylcholine receptor antibody > 100 nmol/L, and intraoperative blood loss > 1000 mL. Meticulous preoperative and postoperative care should be carried out to prevent postoperative myasthenic crisis in patients with these prognostic factors.

Adolescent↗
PubMed2000

Thoracoscopic completion thymectomy in refractory nonthymomatous myasthenia.

BACKGROUND: The aim of this study was to assess the efficacy of thoracoscopic completion thymectomy in patients with refractory nonthymomatous myasthenia. METHODS: Eight patients were operated upon after transcervical (n = 6) or transsternal (n = 2) thymectomy. The mean interval between operations was 129 months. Every patient was completely disabled despite treatment with large dosages of prednisone in combination with pyridostigmine (n = 5) or azathioprine (n = 3) and with repeated plasma exchanges. RESULTS: Gross (n = 5) or microscopic (n = 3) residual thymic tissue was found in all patients. There was no mortality, but morbidity included 2 patients with postoperative myasthenic crisis requiring reintubation and mechanical ventilation. The mean hospital stay was 4.75 days. The mean follow-up was 28.3 months. At the last follow-up, 6 patients had achieved symptomatic improvement as expressed by significant change in mean Osserman class (3.37 versus 2.12, p = 0.03), and prednisone dosage (43 versus 20 mg/d, p = 0.03). Conversely, there was no difference in dosage of pyridostigmine and azathioprine or in number of exchange cycles. CONCLUSIONS: Our results suggest that thoracoscopic completion thymectomy may be beneficial for selected patients with refractory nonthymomatous myasthenia.

Adult↗