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Reversed-T upper mini-sternotomy for extended thymectomy in myasthenic patients.

A minimally invasive approach for extended thymectomy in myasthenic patients is described. Through an 8- to 10-cm midline skin incision with a reversed-T upper mini-sternotomy, an extended thymectomy was performed. The mediastinal fat was removed beginning from the diaphragm up to the thyroid gland, and to each phrenic nerve, laterally. Extended thymectomy through a reversed-T upper mini-sternotomy warrants complete excision of thymic tissue while allowing a short hospitalization and good cosmetic result.

Adult↗

Pulmonary function after thoracoscopic thymectomy versus median sternotomy for myasthenia gravis.

BACKGROUND: Impaired pulmonary function due to myasthenia gravis (MG) is further compromised by thymectomy, which is necessary in most cases. Thoracoscopic thymectomy (tThx) can achieve the same resection and functional improvement of MG as median sternotomy (sThx). The possible advantage of tThx in maintaining better perioperative lung function was quantified. METHODS: In a prospective trial, 20 patients with MG were randomly allocated to undergo tThx (n = 10) by three-trocar left-sided approach or sThx (n = 10) performed as an extended procedure. Complete pulmonary function was measured at 12-hour intervals, beginning 6 hours postoperatively. Effective postoperative pain control in both groups was achieved by patient-controlled analgesia with morphine sulfate assessed by a visual analogue scale. Statistical analysis for comparison of tThx and sThx was performed using the Mann-Whitney U test. RESULTS: Postoperative vital capacity, forced vital capacity, forced expiratory volume per second, and peak expiratory flow, measured as a percentage of the individual preoperative capacity, were significantly better with tThx compared with sThx. Immediate postoperative lung function was reduced to 35% and 65% after tThx and sThx, respectively. By the third postoperative day, recovery of pulmonary function was complete after tThx but only 55% after sThx. CONCLUSIONS: Less pronounced impairment and faster recovery of pulmonary function after tThx characterize this new approach for thymectomy as minimally invasive. These results could make tThx the preferred surgical treatment of MG, which was improved to the same extent as after sThx.

Adolescent↗

Thymectomy by partial sternotomy for the treatment of myasthenia gravis.

BACKGROUND: Myasthenia gravis is an autoimmune disease characterized by weakness and fatigue of voluntary muscles. Surgical treatment of choice for myasthenia gravis has been thymectomy. However, thymectomy indications and surgical approach are still controversial. The purpose of this study is to evaluate the efficacy of partial median sternotomy approach to the thymus. METHODS: From 1973 to 1999, 478 patients with myasthenia gravis underwent thymectomy through a partial median sternotomy. RESULTS: Sixty-one patients (12.7%) had complete remission of symptoms, 299 (62.5%) had a significant improvement, and 83 (17.4%), a mild improvement; whereas 35 patients (7.4%) had no improvement of clinical symptoms. CONCLUSIONS: Partial median sternotomy has shown to be a useful surgical approach to the thymus, as demonstrated by the good functional and aesthetic results, associated with low morbidity and no mortality.

Adult↗

Median sternotomy T incision for thymectomy in myasthenia gravis.

Controversy exists over the surgical approach for thymectomy for myasthenia gravis. Opinion is divided between transcervical thymectomy and transsternal thymectomy performed through a median sternotomy. A technique that combines the advantages of a small, cosmetic T incision with good visualization through a partial sternotomy is presented. We have utilized this technique in more than 35 patients during the past ten years with excellent results.

Humans↗

A rational approach to total thymectomy in the treatment of myasthenia gravis.

Thymectomy is important in the treatment of myasthenia gravis. Total removal of the gland is considered indicated. Although median sternotomy has been the accepted surgical procedure, the transcervical approach has been advocated as a safer method of achieving total thymectomy. A surgical-anatomical study of the thymus was made in 22 patients. A high incidence of surgically important variations in thymic anatomy was found in the neck and in the mediastinum. We believe wide exposure by way of median sternotomy with direct vision is required to remove all of the extracapsular mediastinal thymus in many patients, and good cervical exposure is required to remove the anomalous tissue in the neck. If a total thymectomy is to be achieved, we recommend a median sternotomy and a cervical incision, using the meticulous dissection described.

Adolescent↗

Long-term clinical outcome after transcervical thymectomy for myasthenia gravis.

BACKGROUND: We reviewed the long-term clinical outcome after transcervical thymectomy for generalized myasthenia gravis without thymoma in 52 patients who had this procedure at The Toronto Hospital between 1977 and 1986, and compared the results with those reported after more radical surgical approaches. METHODS: Preoperative and postoperative patient evaluations were based on a modified Osserman classification. We defined complete remission as asymptomatic with normal strength and without medications for myasthenia gravis. The complete remission rate was selected as the best measure for comparison between different surgical approaches. RESULTS: The 52 patients were followed up for a mean of 8.4 years (+/-6.1 years [standard deviation]). The preoperative mean Osserman grade was 2.7 compared with 0.4 at final follow-up. Complete remission occurred in 44.2% of patients. Similar results are reported after transsternal thymectomy. CONCLUSIONS: Comparable results after transcervical and transsternal thymectomy favor the use of the less radical approach.

Adolescent↗

In myasthenia gravis, clinical and immunological improvement post-thymectomy segregate with results of in vitro antibody secretion by immunocytes.

In myasthenia gravis (MG), presence of pathogenic acetylcholine receptor antibodies (AchRAb) and thymic pathology are related. Our study assesses the long-term clinical outcome of thymectomy as a function of thymic pathologies, AchRAb levels in blood and the ability of immunocytes to secrete AchRAb ex vivo. We found that those patients who have thymocytes that secrete AchRAb in vitro have a greater decrease in their need for Mestinon over the 60 months of follow-up than the non-secretors (p = 0.04). Those patients whose cultured peripheral blood mononuclear cells (PBMC) secreted AchRAb also had a greater decrease in Mestinon requirements over the 60 months of follow-up than the non-secretors (p = 0.05), however, differences between the two groups at each individual assessment were not statistically significant. Serial post-op AchRAb measurements in sera of thymocyte and PBMC non-secretors showed no change from pre-op levels (< 10 nmol/l), while secretors had a reduction of more than 80% from pre-op levels, starting 6 months post-op. We also found that results of PBMC culture at time of thymectomy is a better predictor of response to thymectomy than serum AchRAb levels but inferior to results of thymocytes cultures. Patients with thymoma had the highest proportion of individuals secreting AchRAb from thymocytes in culture (78%), the shortest duration of disease (9 months) and showed the greatest trend toward improvement in clinical signs and symptoms post-op. We have thus demonstrated the long held suspicion that removal of actively secreting cells from the thymus at an early time in the disease confers the greatest benefit.

Adult↗

Effect of chronic treatment with piracetam and tacrine on some changes caused by thymectomy in the rat brain.

Thymectomized rats, 5 weeks after surgery, showed a significant impairment in learning and memory as shown by deficits in passive avoidance and in the Morris water maze test. The behaviour of the thymectomized rats in the "open field" apparatus was largely unchanged. Following treatment for 20 days with either piracetam (500 mg/kg) or tacrine (3.0 mg/kg), the deficit in passive avoidance learning was largely reversed. Chronic treatment with tacrine also reversed the deficit in the behaviour of the thymectomized rats in the Morris water maze. The effects of thymectomy on the biogenic amines and some of their metabolites in the amygdaloid cortex, hypothalamus, striatum and olfactory bulbs were also determined. Relative to the sham-operated controls, thymectomy resulted in a reduction in the noradrenaline concentration in the amygdala, hypothalamus, and olfactory bulbs. This effect was reversed by chronic piracetam and tacrine treatments. The concentration of dopamine was also reduced in the olfactory bulbs after thymectomy whereas in the striatum the concentration of 5-hydroxytryptamine (5-HT; serotonin) was increased. The concentration of gamma amino butyric acid (GABA) was determined in amygdaloid cortex and hippocampus only. The only significant change occurred following chronic treatment of thymectomized rats with tacrine, when a significant elevation of GABA was found. Neither piracetam nor tacrine produced any change in the amines of their metabolites in the sham-operated controls. Tacrine, however, elevated the dopamine and reduced the 5-HT content of the hypothalamus and increased the 3,4-dihydroxylphenylacetic acid concentration of the striatum of thymectomized rats. Examination of the differential white blood cell count of the thymectomized rats showed that the percentage of lymphocytes was decreased, and the percentage of neutrophils increased, relative to the sham-operated controls. Chronic lacrine, but not piracetam, treatment reversed the lesion-induced changes.

Animals↗

The impact of HLA on long-term outcome after thymectomy for myasthenia gravis.

In a retrospective series of 86 patients with myasthenia gravis, the only factors predictive of improvement in muscular strength after transsternal thymectomy were preoperative severity of myasthenia (90% versus 54%, p = 0.0014) and HLA-B8 (79% versus 50%, p = 0.0060) in bivariable and multivariable analyses. Both factors were not interrelated (p = 0.824). The statistical effect of HLA-B8 was independent from preoperative severity of disease. Typing for HLA-B8 may thus be a valuable adjunct in predicting the benefit of thymectomy in myasthenia. The observation that an MHC class I allele is associated with clinical improvement after thymectomy suggests that the clinical course of myasthenia may be influenced by class I restricted T-cells.

Alleles↗

[Thymectomy in myasthenia gravis].

OBJECTIVE: To analyze outcome after thymectomy in patients with myasthenia gravis (MG). MATERIAL AND METHODS: Thirty-five patients with MG underwent surgery in our service between June 1987 and June 1998. Ten had associated thymomas. Preoperative Osserman classification showed 2 at level I, 20 at level IIA, 11 at level IIB and 2 at level III. Extended thymectomy through a medial sternotomy was performed in all. RESULTS: Postoperative complications developed in three patients (1 medullary aplasia, 1 postoperative reintubation, 1 myasthenic crisis). Mean follow-up was 89 months, with 22.8% achieving complete remission and 97.1% reporting improvements. The results were similar in the 10 patients with thymomas (20% full remission and 90% showing improvement). By DeFilippi classification, 22.8% were in class 1, 22.8% in class 2, 51.4% in class 3 and 2.8% in class 4. By Osserman classification, 9 were in the same category before and after surgery, 12 had improved one level, 10 had improved 2 levels, 3 had improved 3 levels and 1 patient had improved 4 levels. CONCLUSION: Thymectomy is an appropriate therapeutic procedure in the multidisciplinary treatment of patients with MG and it is the approach of choice for patients with associated thymomas. The intra- and post-operative complication rate is low and the rate of clinical improvement is high.

Adolescent↗

Thirteen years follow-up after radical transsternal thymectomy for myasthenia gravis. Do short-term results predict long-term outcome?

OBJECTIVE: Long-term evaluation of efficacy and quality of life after radical surgical approach for myasthenia gravis (MG). Comparison between short-term follow-up and long-term outcome. METHODS: All patients (n=26, 16 men and 10 women, mean age: 40.7 years) underwent total transsternal thymectomy for MG between 1986 and 1989. Prospective analysis of the patients for short-term follow-up (mean 22.4 months) was published in 1991. The same group of patients was reevaluated in 2001 (range of follow-up 11.4-15.2 years) and assessed according to the classification of Osserman and Oosterhuis. RESULTS: Mean follow-up was 13.0 years (range 11.4-15.2 years). Two patients were lost from follow-up and one died 4 years after thymectomy for reasons unrelated to MG (n=23). No early or late postoperative mortality was observed. One sternal osteomyelitis occurred. Late postoperative morbidity included sternal instabilities (n=2), mild residual thoracic pain (n=6), and hypertrophic scars (n=7). Five patients were rehospitalized for aggravating MG and needed plasmapheresis (n=3) and intubation (n=1). Thirteen patients (56.5%) showed objective clinical improvement, including six patients (26.1%) with complete remission. Eleven patients (47.8%) do not take any medication at all. Because some late relapse may occur several years after operation, the rate of improvement decreased slightly, whereas the difference between short and long-term follow-up was not statistically significant (P=0.405). Twenty patients (87%) returned to work, including part-time occupation (n=4). Fourteen patients (61%) are performing sports regularly. CONCLUSIONS: Our data confirm that radical, transsternal thymectomy is an effective and safe therapeutic modality for MG. Short-term results seem to deteriorate over time, therefore long-term studies for minimally invasive approaches have to prove equal results before replacing the standard procedure.

Adolescent↗

Appearance of thymoma 15 years after extended thymectomy for myasthenia gravis without thymoma.

Several cases of thymoma recurrence after resection have been reported. However, thymoma appearance following an extended thymectomy for non-thymomatous myasthenia gravis is very rare. We report a case of thymoma in a 48-year-old woman, 15 years after an extended thymectomy for non-thymomatous myasthenia gravis. The importance of a complete dissection of mediastinal adipose tissue during the extended thymectomy as well as careful follow-up for such patients is also noted.

Female↗

Video-assisted thymectomy for myasthenia gravis: an update of a single institution experience.

OBJECTIVE: Video-assisted thymectomy was introduced in 1992 as a minimally invasive alternative for the treatment of myasthenia gravis. As experience with this technique is limited and follow-up short, we present this expanded and updated experience for purposes of validation of the technique. METHODS: Thirty-eight video-assisted thymectomies for myasthenia gravis were performed in our institution between March 1992 and March 2002. Two patients were lost to follow-up. We analyzed clinical results of 36 patients (14 males and 22 females) with a mean age of 41.2 years. Preoperative clinical staging was assessed by the newly recommended Myasthenia Gravis Foundation of America Clinical Classification. Clinical status at follow-up was assessed by the Myasthenia Gravis Foundation of America Postintervention Status classification. RESULTS: There was no perioperative mortality or long-term morbidity. One of 38 (2.6%) patients required conversion to limited thoracotomy for bleeding. The mean length of hospital stay was 1.64 days (range 0-8 days) with a median stay of 1 day. The mean length of follow-up is 53.24 months (range 4-126 months). Overall clinical improvement at follow-up was observed in 30 of 36 (83.0%) patients, with five of 36 (14.0%) patients in complete stable remission. CONCLUSIONS: Video-assisted thymectomy for myasthenia gravis provides acceptable clinical long-term results by a minimally invasive approach comparable to standard surgical approaches to the disease. The presented data is reported in accordance with the new guidelines by Myasthenia Gravis Foundation of America Task Force for valid comparison with future studies.

Adolescent↗

Predictors of clinical outcome following extended thymectomy in myasthenia gravis.

OBJECTIVE: Thymectomy remains as the optimal treatment of choice in patients with myasthenia gravis (MG), however, the selection criteria for surgery remains controversial. METHODS: We examined the data charts of patients with MG underwent extended thymectomy. We investigated the possible correlations between the clinicopathologic features and clinical outcomes, and analyzed the data to clarify the effect of prognostic factors on clinical outcome. RESULTS: A total of 61 patients with a mean age of 35.8 +/- 12.2 years (range, 13-66 years) were analyzed. The overall improvement/remission and clinical worsening rates were 81.9 and 18.1%, respectively. Ossermann stage (P = 0.011) and presence of mediastinal ectopic thymic tissue (P = 0.007) showed a significant correlation with the clinical outcome. Multivariate analysis confirmed Ossermann stage (P = 0.0158), and presence of mediastinal ectopic thymic tissue (P = 0.0100) as independent predictors on clinical outcome. CONCLUSION: Ossermann stage and the presence of mediastinal ectopic thymic tissue are potential predictors on clinical outcome in patients with MG undergoing extended thymectomy.

Adolescent↗

Maximal thymectomy in children with myasthenia gravis.

OBJECTIVES: We performed this study to evaluate the benefit of thymectomy in children with myasthenia gravis (MG). METHODS: Over a period of 15 years from 1986 to 2001, we collected data on 30 children with MG and retrospectively reviewed the outcome of maximal thymectomy. RESULTS: There were 23 females and seven males with a mean age of 13.2 years (range 4-16). The mean duration of the disease was 19.3 months (range 2-144). According to Osserman classification, there were 14 children in class II; 12 in class III; and four children in class IV. One child in class IV required postoperative ventilation and one was re-explored to drain a pericardial effusion secondary to central line leak. We found ectopic thymic tissue in 10 cases (33.3%). During a mean follow-up period of 53.5 months (range 9-180), complete remission was noted in 13 children (43.4%) and improvement in 14 (46.6%). The remaining three children (10%) did not improve following surgery. Univariate analysis (P < 0.05) showed that ectopic thymic tissue is a significant prognostic factor for outcome. CONCLUSION: Maximal thymectomy appears to provide a high rate of remission and improvement in children with MG. However, the presence of ectopic thymic tissue has poor prognostic value.

Adolescent↗

Maximal thymectomy for myasthenia gravis.

Maximal thymectomy was carried out in 48 patients with myasthenia gravis (MG). There were 18 males and 30 females. Thymic hyperplasia was found in 38, and atrophic thymus in 8, patients. Two patients had thymoma. In the non-thymomatous myasthenia gravis complete remission was achieved in 16 patients (34.8%) and pharmacological remission in 20 patients (43.5%) thus giving a total remission in 36 (78.3%) patients. Six patients (13%) improved. There was no improvement in four patients. Thus, the overall benefit from thymectomy was 91.4% in this series. We found that sex, age at onset of disease and steroid therapy influenced the outcome of thymectomy. On the other hand, duration of disease, anti-acetylcholine receptor (AntiAchR) antibodies and thymic histology did not have any bearing on the complete remission rate.

Adolescent↗

Response of thymectomy: clinical and pathological characteristics among seronegative and seropositive myasthenia gravis patients.

OBJECTIVE: To identify the response to thymectomy in patients with seronegative and seropositive myasthenia gravis (SPMG). We analyzed the associated diseases, thymus histology, and the severity of symptoms between the two groups. MATERIAL AND METHODS - DESIGN: Descriptive, comparative. STUDY UNITS: Fourteen patients with seronegative myasthenia gravis (SNMG) and 57 patients with SPMG who had a thymectomy between 1987 and 1997, with at least 3 years of follow-up. The patients were divided into four groups; (1) Remission, (2) Improvement, (3) No change and (4) Deterioration. RESULTS: Fourteen patients (20%) were seronegative and 57 were seropositive (80%). In the group of patients with SNMG, three patients were in remission (21%), five with improvement (36%), five with no change (36%) and one with worsening (7%). In the group of patients with SPMG, 12 were in remission (21%), 17 with improvement (30%), 25 with no change (44%) and three (5%) with worsening. The patients with SNMG were older, with less associated diseases and with a lower frequency of thymomas. CONCLUSIONS: The response to thymectomy was similar between the two groups. It has been suggested that seronegative patients have a better prognosis, but our results show no differences.

Adult↗

Malignant thymic carcinoid is not prevented by transcervical thymectomy in multiple endocrine neoplasia type 1.

Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant tumour syndrome. It is characterized by primary hyperparathyroidism, pituitary neoplasia and foregut lineage neuroendocrine neoplasia. Malignant thymic carcinoid tumours are an uncommon but important manifestation of MEN 1. Transcervical thymectomy is often advocated as prophylaxis against thymic carcinoids, although there is a paucity of evidence to support the efficacy of this procedure. This is the first report of a malignant thymic carcinoid occurring in an MEN 1 patient following prior parathyroidectomy and transcervical thymectomy. It is concluded that transcervical thymectomy does not reliably provide prophylaxis against thymic carcinoid.

Adolescent↗