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Transcervical-subxiphoid-videothoracoscopic "maximal" thymectomy--operative technique and early results.

BACKGROUND: The operative technique of a transcervical-subxiphoid-videothoracoscopic "maximal" thymectomy without sternotomy is described and the early results of the follow-up of patients operated on are analyzed. METHODS: One-hundred "maximal" transcervical-subxiphoid-videothoracoscopic thymectomies were performed for nonthymomatous myasthenia gravis during a recent 32-month period (from September 1, 2000 to May 8, 2003). Patient characteristics, complications, pathologic findings, and the results of follow-up were analyzed. RESULTS: The study group included 83 women and 17 men. The mean age was 29.8 years (range, 10-69 years). The mean preoperative duration of myasthenia was 2.73 years (range, 3 months to 17 years). The preoperative Osserman score was I-III, 27 patients were taking steroids preoperatively. Eleven operations were performed by two teams working simultaneously and 89 operations were performed by one surgeon including four combined thymectomy-thyroid operations in patients with myasthenia and thyroid nodules. The mean operative time for two-team approach thymectomies was 159.09 minutes (range, 140-170 minutes) and the mean operative time for the thymectomy performed by one surgeon was 199.41 minutes (range, 150-270 minutes) (p = 0.0004). There was a 15.0% (15 out of 100) postoperative morbidity and no mortality. Foci of ectopic thymic tissue were found in 71.0% of the patients and were most prevalent in the perithymic fat (37.0%) and in the aorta-pulmonary window (33.0%). The mean weight of the specimen was 78.4 g (range, 14.5-253.0 g). In 48 patients followed-up for 12 months, the improvement rate was 83.3%, the no improvement rate was 14.6%, and 1 patient died during the follow-up period. Complete remission rates were 18.8% and 32.0% after 1 and 2 years of follow-up, respectively. CONCLUSIONS: We conclude that the "maximal" transcervical-subxiphoid-videothoracoscopic thymectomy is a safe operative technique, avoiding a sternotomy, performed partly in an open fashion with the extensiveness comparable with the transsternal extended and "maximal" thymectomies. The two-team approach helps to reduce the operative time. However, because of the limited time of follow-up it is too early for the final assessment of the long-term results of this method in the treatment of myasthenia gravis.

Adipose Tissue↗

Thymectomy in the treatment of myasthenia.

Thymectomy has been performed in 69 patients, 58 females and 11 males, age range 14 to 70 years, with a maximum in females between 20 to 30 years. Twenty-seven percent of the patients were older than 40 years. Considerable improvement resulting from remission and partial remission occurred in 89% of females and in about 50% of males, the figure for thymomatous myasthenia was about 65% in the first year after thymectomy. In the following years, improvements increased partially and the number of remissions rose as well. By chance the duration of the disease was longer than 6 years in 20% of our patients and in these a benefit from thymectomy was also found. There were 7 early deaths and 4 late deaths. Thymectomy cannot be called a cure but is an essential part of the whole treatment procedure of myasthenia comprising the administration of anticholinesterase and especially immunosuppressive agents. It was found difficult to estimate the effect of any single measure in this retrospective study by the major benefit of thymectomy is seen in initiating remissions or at least considerable improvements. Indication for operation is thought to be present in progressive myasthenia, acute or chronic, and thymoma, provided that the patient has reached an optimal condition preoperatively by adequate anticholinesterase adjustment, neurophysiological control and immunosuppressive treatment. Thymectomy is not an emergency operation and should be carefully planned. Since relapses after thymectomy are not rare, a careful neurological long-term follow-up appears obligatory.

Adolescent↗

Levels of recent thymic emigrant cells decrease in children undergoing partial thymectomy during cardiac surgery.

The human thymus is required for establishment of a T-cell pool in fetal life, but postnatal thymectomy is not known to cause immunodeficiency. T-cell emigration from thymus (thymic recent emigrants [TRECs]) is a continuous thymic-dependent process. We studied TREC levels pre- and post-partial thymectomy in children undergoing cardiac surgery. TRECs were quantitated by real-time PCR in peripheral blood lymphocytes of 24 children (0 to 12 years). TREC values were 47916 +/- 9271 pre-partial thymectomy and 33157 +/- 8479 post-partial thymectomy in 11 paired patients (P = 0.014). Interval between pre- and post-partial thymectomy was 8.8 days +/- 5.8 days. Another group of 8 children had 30384 +/- 9748 TRECs 16 days to 6 years post-partial thymectomy. There was a significant drop in TREC values post-partial thymectomy in the immediate postoperative period compared to prethymectomy TREC levels. While decreased thymic output may persist, the long-term implications were not evaluated in this patient population.

Cardiac Surgical Procedures↗

Video-assisted thoracoscopic thymectomy for myasthenia gravis.

Video-assisted thoracoscopic surgery (VATS) provides a new approach to thymectomy. From June 1993 to December 1994, we performed a total of eight thymectomies for myasthenia gravis (MG). There were four male and four female patients with ages ranging from 9 to 76 years. Three of the eight patients had associated thymoma. We believe that complete thymectomy was accomplished in all cases by examination of the thymic bed and resected specimen. There was no mortality or intraoperative complications. The median postoperative hospital stay was 5 days (range, 2 to 37 days). One patient required ventilatory support postoperatively. Clinical improvement was observed in all patients after a mean follow-up of 10 months (range, 2 to 21 months). Compared with a comparable historical group of patients with MG who underwent transsternal thymectomy, the VATS group was associated with significantly less analgesic requirement and shortened hospital stay. We conclude that VAT thymectomy is technically feasible and is associated with a favorable postoperative course compared with the transsternal approach. We believe that complete thymectomy can be achieved by this approach. Further investigation with long-term follow-up is needed to further clarify the role of VAT thymectomy in thoracic surgery.

Adult↗

[Video-assisted thoracoscopic thymectomy--preliminary results].

Video-assisted thoracoscopic surgery (VATS) provides a new approach to thymectomy. From April 1999 to December 1999, we performed a total of 10 video-assisted thoracoscopic thymectomies for myasthenia gravis (MG). There were one male and nine female patients with ages ranging from 8 to 59 years. Thymoma was present in one of the ten patients. We considered that complete thymectomy was accomplished in all cases by examination of the thymic bed and of the resected specimen. There was no mortality or intraoperative complications. The median postoperative hospital stay was 4 days. Clinical improvement was observed in all patients after this short follow-up. Compared with a similar historical group of patients with MG who underwent transsternal thymectomy, the VATS group was associated with significantly less analgesic requirement and shortened hospital stay. We conclude that VAT thymectomy is technically feasible and is associated with a favorable postoperative course compared with the transsternal approach. We believe that complete thymectomy can be achieved by this approach. Further investigation with long-term follow-up is needed to further clarify the role of VAT thymectomy in thoracic surgery.

Adult↗

Anesthetic implications for video assisted thoracoscopic thymectomy in myasthenia gravis.

Thymectomy is an established therapy in the management of generalized myasthenia gravis (MG). However, the optimal surgical approach to thymectomy has remained controversial. There are advocates for transternal, transcervical approaches for "maximal" thymectomy. Video-assisted thoracoscopic thymectomy (VATT) presents new approach to thymectomy. By minimizing chest wall trauma, VATT not only causes less postoperative pain, shortens hospital stay, gives better cosmetic results but also leads to wider acceptance by patients for earlier surgery. Anesthesia for thymectomy in MG is challenging. Currently we are using non-muscle relaxant technique (NMRT) which we adopted in 1994, for maximal thymectomy. In this paper, we present our limited experience with two cases of VATT using two different NMRTs. Two cases of MG underwent VATT under general anesthesia (GA) and one lung ventilation (OLV) using double lumen tube (DLT). In both cases NMRT was used which encompass, light GA plus thoracic epidural analgesia (TEA) in one case and without TEA in the other case. We believe that the use of NMRT provides good operative and postoperative conditions. In this report we have described two different NMRTs, one with TEA and the other without. Further studies are needed on large number of cases to establish an anesthetic protocol for VATT.

Adult↗

[Thymic lymphoid hyperplasia of myasthenia gravis patients: correlation with clinical features and efficacy of thymectomy].

Thirty nine thymus tissues from myasthenia gravis patients without thymoma who underwent extended thymectomy were evaluated on thymic lymphoid hyperplasia with special emphasis on clinical features and the efficacy of thymectomy. Of 39 patients, 31 were women, but 3 of 7 patients without thymic lymphoid hyperplasia were men. The hyperplastic index of the thymus correlated largely with serum anti-Ach-R antibody titers before surgery. Age, myasthenic type and preoperative duration of the symptoms were almost unrelated to the hyperplastic change in the thymus. The effect of thymectomy was more remarkable in the patients with the hyperplastic thymus than that in them without it, who obtained still no remission after surgery, although the remission rate of them with it reached to 25%. Of 7 patients without the hyperplastic thymus, 4 had no detectable antibody to acetylcholine receptor, whereas in 32 patients with it only 2 were seronegative. This negative immunological factor might make adverse response to thymectomy. Although unfavorable response to thymectomy were observed in the patients without the hyperplastic thymus, most of them were significantly improved through a supplementary treatment with steroid after surgery. Thymectomy was a favorable treatment for myasthenia gravis patients with thymic lymphoid hyperplasia, whereas in them without it the result of thymectomy was unsatisfactory.

Adolescent↗

Thymectomy as treatment of autoimmune diseases other than myasthenia gravis.

This paper reviews the different series of thymectomies performed in patients with autoimmune diseases other than myasthenia gravis. It is possible that thymectomy can decrease the activity of T-helper lymphocytes or, alternatively, it may enhance the activity of T-suppressor lymphocytes, whose function is depressed in autoimmune diseases. Thymectomy was performed empirically for systemic lupus erythematosus and rheumatoid arthritis. The therapeutic benefits were questionable. Conflicting results were reported for thymectomy against autoimmune hemolytic anemia. Several trials were conducted to assess the therapeutic value of thymectomy in multiple sclerosis. Benefits were achieved only in relapsing-remitting but not in chronic-progressive multiple sclerosis. The effect of thymectomy in autoimmune diseases associated with myasthenia gravis were also reported. The authors conclude that thymectomy as treatment for autoimmune diseases other than myasthenia gravis is not elective therapeutic choice and it is acceptable only in selected cases.

Anemia, Hemolytic, Autoimmune↗

Influence of age on extended thymectomy as a treatment for myasthenia gravis.

This retrospective study examines the influence of age on extended thymectomy as a treatment for myasthenia gravis. From 1979 to 1997, 58 patients with myasthenia gravis underwent extended thymectomy at Miyazaki Medical College Hospital. We used the onset age of the disease to divide these patients into two groups: Group A included 14 patients >/= 50 years of age; Group B, 44 patients < 50. Using Osserman's classification and the duration of the disease prior to hospitalization, we observed no significant differences between the two groups although thymoma were more prevalent in Group A. All 58 patients underwent extended thymectomy, with no surgical mortalities. The mean duration of tracheal intubation after thymectomy for Group A was 6.25 days; for Group B, 4.66 days without statistically significant differences between both groups. Using Masaoka's criteria, we evaluated the clinical course of myasthenia gravis following extended thymectomy for each of the 58 patients. The remission rates in Groups A and B were 28.6% and 29.5%, respectively; the improvement rates, 71.4% and 79.5%, respectively with no significant differences among groups. These findings suggest that the clinical course of myasthenia gravis following extended thymectomy is not age-dependent and that extended thymectomy is a clinically safe and effective treatment option for myasthenia gravis patients >/= 50 years of age.

Adolescent↗

Repeat thymectomy in chronic refractory myasthenia gravis.

Encouraged by recent results with "extended" thymectomy in the treatment of myasthenia gravis, we carried out repeat thymectomy in six patients with chronic, refractory disease who did not initially have extended thymectomy. All were completely disabled with longstanding myasthenia. Initial thymectomy (four transsternal, one transcervical, and one substernal) was carried out at a mean of 8.9 years previously (range, 5 to 18). There was no residual thymus observed with CT, but at repeat thymectomy, residual thymic tissue was present in five of six patients. Five patients significantly improved and four returned to full-time work. Mean prednisone dose declined from 51 mg to 18 mg/d, and mean pyridostigmine dose fell from 1,290 mg to 415 mg/d. No patient to date has had a complete remission. These results suggest that repeat thymectomy may benefit some patients with chronic disabling myasthenia gravis, especially when it is uncertain from a review of the operative report whether all thymic tissue was removed at the initial thymectomy.

Adult↗

Effect of pregnancy and birth on the course of myasthenia gravis before or after transsternal radical thymectomy.

OBJECTIVE: Myasthenia gravis (MG) affects women at childbearing age. Therefore, the question arises if these patients should become pregnant and if thymectomy has a positive effect on the course of MG in pregnant patients. METHODS: Fifteen pregnancies had been followed retrospectively. All patients underwent transsternal radical thymectomy for MG. The course of MG in the period before, during, and after the pregnancy was scored according to Ossermann's classification. The effect of thymectomy on delivery and on the newborns was evaluated. RESULTS: Patients were divided in two groups: pregnancies before (group I, n=8) and after (group II, n=7) thymectomy. During pregnancy, in group I, one deterioration was observed and in seven patients the disease was unchanged. In group II, one deterioration, five unchanged courses, and one improvement were observed. In the postpartum period, in group I, seven patients did not change and one improved. In group II, two deteriorations, three unchanged courses, and two improvements were observed. Before pregnancy, group II patients were in a better Ossermann stage in comparison with those in group I. Eight of the 12 deliveries were spontaneous (three abortus). Myasthenic symptoms were observed in two newborns in group I. CONCLUSION: Our data suggest that MG is not prohibitive to have children. The course of MG after transsternal radical thymectomy is often ameliorated. A better MG-stage, reached after thymectomy, before pregnancy seems to be correlated with a better course during pregnancy.

Adult↗

Results of video-assisted thymectomy in patients with myasthenia gravis.

OBJECTIVE: The efficacy of video-assisted thoracic surgery for thymectomy with myasthenia gravis has not been examined. METHODS: Thirty-three consecutive patients underwent total thymectomy by video-assisted techniques between 1992 and 1995. There were 13 male and 20 female patients with a mean age of 38.42 +/- 16.88 years (range 9 to 84 years). The procedures were performed by either a right (n = 11) or left (n = 22) thoracoscopic approach and all anterior mediastinal tissue was removed. RESULTS: There was no perioperative mortality or long-term morbidity. One patient required conversion of the video-assisted technique to a lateral thoracotomy. All patients except one were extubated immediately. The mean hospital stay was 4.12 +/- 6.07 days (range 1 to 37 days) with a median of 3 days. Mean follow-up is 23.39 +/- 11.72 months (range 4 to 47 months). Clinical improvement was seen in 87.9% (29/33): one of two patients (50%) in stage I, 17 of 19 (89.4%) in stage IIA, eight of nine (88.8%) in stage IIB, and three of three (100%) in stage III. Metaanalysis of these results compared with results in nine published series in which other techniques were used showed no difference in clinical improvement after thymectomy between series. CONCLUSION: We conclude that video-assisted thymectomy is as effective as the traditional open surgical approaches for performance of thymectomy in the management of patients with myasthenia gravis. In addition, the improved cosmesis of the video-assisted approach ideally will lead to earlier thymectomy in patients with myasthenia gravis.

Adolescent↗

Thymectomy in myasthenia gravis: response, complications, and associated conditions.

BACKGROUND: Thymectomy is considered the most effective treatment for achieving sustained improvement as well as remission in patients with myasthenia gravis (MG), and most neurologists favor the use of this procedure. The main focus of many current studies is to determine response-predicting factors. METHODS: Clinical records of 152 patients with an established diagnosis of MG who underwent thymectomy at our institution were reviewed. The purpose was to evaluate outcome of surgical management for MG and prognostic factors that influence that outcome. RESULTS: The majority of patients were women (119 of 152); mean age was 32.10 +/- 14.42 years, while time elapsed from diagnosis to surgery was 20.67 +/- 19.7 months. Transsternal thymectomy was performed on 113 patients and transcervical on 39. Forty percent of patients achieved remission and 28% showed improvement; with this, a good response to thymectomy was seen in 68% of patients (n = 103). The most important variables associated with remission were <60 years of age, <2 years of preoperative symptoms, and use of pyridostigmine at low doses. Factors related with poor response were >60 years of age, preoperative Osserman stage other than II, use of high doses of pyridostigmine, use of corticosteroids, and presence of thymic atrophy or thymoma in histopathologic analyses. There was no mortality, although 20 patients (13%) presented complications. CONCLUSIONS: Mexican patients with MG undergoing thymectomies show improvement and remission rates similar to those reported by other studies. Age, length of symptoms, thymic pathology, and medications appear to be predictors of response to thymectomy for MG.

Adolescent↗

Clinical, electrophysiological and immunological remissions after thymectomy in myasthenia gravis.

OBJECTIVES: Approximately 50% of patients treated with thymectomy have a chance for symptom-free life. However, immunological and neurophysiological abnormalities may be detected in patients with clinical remission. Although improvement usually parallels decrease in acetylcholine receptor antibody (AChRAb) levels and jitter values, there is a question what factors influence immunological and electrophysiological remission in a population of myasthenia gravis (MG) patients. METHODS: We analyzed retrospectively clinical data of 32 MG patients operated for generalized MG, followed-up at our department for 17.2 (4-31) years. They were in clinical remission for 12.8 (2-25) years. All of them had single fiber electromyograhy (SFEMG) of extensor digitorum communis muscle (EDC) muscle and estimation of AChRAb level at the end of follow-up. Their age at onset of MG was 17 years (6-48) and at thymectomy 19 (6.4-58) years. Tensilon test was positive in 30, repetitive nerve stimulation in 29 cases. RESULTS: Clinical remission was reached on average 4.2 years after thymectomy. SFEMG jitter value normalized in 60% of cases. AChRAb were negative only in 34% of patients. Jitter values correlated with AChRAb levels (P=0.006, r=0.5) but were not related to clinical factors. Only time to thymectomy correlated with time from thymectomy to clinical remission (P=0.001, r=0.5). CONCLUSIONS: Clinical remission is not always accompanied by normalization of SFEMG and AChRAb. Although normalization of neuromuscular transmission in patients with remission of MG is individual, short duration of MG before thymectomy increases the chance of early remission.

Adolescent↗

Thoracoscopic thymectomy in juvenile myasthenia gravis.

BACKGROUND: Although transsternal thymectomy is an effective method in the treatment of juvenile myasthenia gravis (JMG) it is traumatic in pediatric patients. Thoracoscopic thymectomy offers an effective and less traumatic approach with respect to cosmesis and postoperative recovery. METHODS: A retrospective analysis of 6 consecutive patients treated with thoracoscopic thymectomy was performed. Perioperative parameters and cost analysis were compared with those of 6 consecutive open procedures performed before the study. RESULTS: Thoracoscopic thymectomy can be performed in patients as young as 1.6 years. There was no conversion to open procedure and no perioperative morbidity and mortality. The length of operating time and the surgical cost of thoracoscopic procedure were not significantly different from those of open procedure. The length of hospitalization, however, was significantly shorter with thoracoscopic procedure, and hence the overall cost was significantly reduced (P < .05). An intermediate follow-up shows that outcome after thoracoscopic procedure is equally as effective as open procedure. CONCLUSIONS: Thoracoscopic thymectomy offers an equally effective but cosmetically more acceptable approach than sternotomy. It has a quicker recovery period and appears to be a less costly alternative to transsternal thymectomy.

Adolescent↗

Effects of thymectomy in myasthenia gravis.

Factors influencing onset of remission in myasthenia gravis were evaluated in 2062 patients, of whom 962 had had thymectomy. Multivariate analysis showed that appearance of early remissions among all patients was significantly and independently influenced by thymectomy, by milder disease, and by absence of coexisting thymomas. Patients with mild generalized symptoms treated with thymectomy reached remission more frequently, even when compared with those with ocular myasthenia treated without surgery. Short duration of disease before thymectomy in mild cases was another factor associated with earlier remissions. Mortality for all patients was significantly and independently influenced by severity of symptoms, age, associated thymomas, and failure to remove the thymus. Patients without thymectomy and with thymomas had, in addition, earlier onset of extrathymic neoplasms. Morbidity after the transcervical approach was minimal. This study demonstrates that early thymectomy by the transcervical approach, when technically feasible, has significant clinical advantages over the transthoracic approach and should be advocated for all patients with myasthenia gravis, including those with ocular disease.

Evaluation Studies as Topic↗

Extended cervicomediastinal thymectomy in the integrated management of myasthenia gravis.

OBJECTIVE: The authors evaluated the response to extended cervicomediastinal thymectomy as a component of the integrated management of patients with myasthenia gravis in a large series of patients from a single institution. The authors evaluated the response to therapy with respect to a graded, multivariate, ordinal scale chosen to reflect the full range of the disease's manifestations. SUMMARY BACKGROUND DATA: A number of series, of varying sizes, describe the response of patients with myasthenia gravis to thymectomy primarily with respect to the bivariate endpoint of the presence or absence of "remission." These studies fail to describe the full spectrum of postoperative disease severity and have been unable to quantify definitively the influence of putative prognostic variables, nor to assess definitively the statistical significance of apparent improvements over time. METHODS: The authors evaluated 202 consecutive patients who underwent trans-sternal thymectomy for symptomatic myasthenia gravis from 1969 through 1996 at the Johns Hopkins Hospital. The response to surgery, combined with postoperative medical therapy, was evaluated by a standardized scale reflecting the full spectrum of the disease. These data were analyzed by a novel mean multivariate regression analysis, which allowed the quantification of the statistical significance of apparent responses over time and the evaluation of the independent influence of each of nine putative prognostic indicators. RESULTS: There was no perioperative mortality and a 33% perioperative morbidity. There was a marked clinical response at 6 months to 1 year after surgery that was sustained for at least 10 years thereafter. The median increment of improvement was two (of five) classes. Eighty-six percent and 83% of the patients had improved by at least one class at 5 and 10 years, respectively. These changes were statistically significant (p < 0.001). Whereas the use of extended cervicomediastinal thymectomy was associated with a greater than twofold chance of improvement, compared to conventional trans-sternal thymectomy, neither the pathologic diagnosis (presence of thymoma) nor the age at surgery proved to be negative predictors of postoperative response. CONCLUSIONS: Extended cervicomediastinal thymectomy is the procedure of choice as a component of the integrated management of myasthenia gravis, with significant improvement seen in the group as a whole, as well as in subgroups of patients with thymoma and those older than 40 years of age.

Adolescent↗

Results of transcervical thymectomy for myasthenia gravis in 100 consecutive patients.

OBJECTIVE: To review the results of the authors' most recent 100 consecutive cases of transcervical thymectomy for myasthenia gravis (MG) in terms of complications and outcome in comparison with other reported techniques. SUMMARY BACKGROUND DATA: Myasthenia gravis is believed to be an autoimmune disorder characterized by increasing fatigue with exertion. The role of thymectomy in the management of the disease remains unproven, but there is widespread acceptance of the notion that complete thymectomy improves the course of the disease. Complete excision of the thymus is the goal in all cases; however, the best technique to achieve complete thymectomy remains controversial. The authors favor a transcervical approach through a small collar incision aided by a specially designed sternal retractor. Others prefer a transsternal, a combined transcervical and transsternal ("maximal"), or a video-assisted thoracoscopic surgical approach. METHODS: A retrospective review of the authors' most recent 100 consecutive transcervical thymectomies for nonthymoma-associated MG was performed using medical records and telephone interviews. Patients' symptoms were graded before surgery and at the most recent (within the last 6 months) postoperative time point, using the modified Osserman classification: 0 = asymptomatic, 1 = ocular signs and symptoms, 2 = mild generalized weakness, 3 = moderate generalized weakness, bulbar dysfunction, or both, and 4 = severe generalized weakness, respiratory dysfunction, or both. RESULTS: There were 61 female patients and 39 male patients with a mean age of 38 years (range, 14 to 84). The median hospital stay was 1 day. There were no deaths and no significant complications. Seventy-eight patients who had undergone surgery >12 months ago were available for analysis. In these patients, with a mean follow-up time of 5 years (median 5.3; range, 12 months to 10 years), the median preoperative Osserman grade improved from 3.0 (mean 2.73) before surgery to 1.0 after surgery (mean 0.94). CONCLUSIONS: The transcervical approach for thymectomy for the treatment of MG produces results similar to those of other surgical approaches, with the added benefits of shortened hospital stay, decreased complications, reduced cost, and broader physician and patient acceptance of surgical treatment.

Adolescent↗