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Thymectomy for myasthenia gravis.

Thymectomy was performed on 31 patients with myasthenia gravis. Four of them had thymoma, 17 hyperplasia and 9 had normal thymic histology on microscopy. Discontinuation of anticholinergic medication together with tracheostomy, artificial ventilation and intensive observation were essential in the postoperative management. All the patients survived operation and were discharged from hospital. Sixty-eight per cent of the patients were in remission or improved after an average follow-up of 2.5 years. If the patients with thymoma were excluded, 74% were improved and, if only the patients with thymic hyperplasia were included in the series, 89% were improved after thymectomy. The severity of disease and duration of symptoms pre-operatively seemed to be of minor importance for the results of operation, since good results were obtained also in patients with a long history and in clinical classes III and IV. Two patients developed tracheal stenosis as a complication of intubation with a tracheostomy tube. Tracheal resection was performed in one of them and endobronchial removal of granulation tissue producing the stenosis in the other. Both recovered uneventfully. One patient, who was asymptomatic and without medication for myasthenia, died suddenly of acute cerebral bleeding 3 years after thymectomy.

Adolescent↗

Transcervical thymectomy in patients with nonthymomatous myasthenia gravis.

Transcervical thymectomy was performed during a 13-year period in 21 patients with nonthymomatous myasthenia gravis. Follow-up showed that only eight patients benefited from the operation. These eight were among the younger patients in the series. This observation is discussed, and also the theoretic background for early thymectomy. The transsternal approach is recommended for thymectomy in elderly patients, but the transcervical may be considered for younger patients, although transsternal reexploration is advocated if this first operation should fail.

Adolescent↗

Very severe aplastic anemia appearing after thymectomy.

Aplastic anemia is a rare complication of thymoma and is extremely infrequent after thymectomy. We present a case of a 60-year-old woman with very severe aplastic anemia appearing sixteen months after thymectomy for a thymoma. She underwent thymectomy for a thymoma in April 2000. Preoperative examination revealed no hematologic abnormality. About sixteen months after the operation, she was readmitted because of pancytopenia with cough and fever. Bone marrow aspiration revealed a very severe hypoplasia in all the three cell lines with over 80% fatty tissue, and chest CT revealed no recurrence of thymoma. Her aplastic anemia had responded to cyclosporine A and granulocyte-colony stimulating factor (G-CSF).

Anemia, Aplastic↗

[Video-assisted extensive thymectomy in the treatment of autoimmune myasthenia].

In the surgical treatment of autoimmune myasthenia, complete thymectomy is considered indispensable to ensure maximum clinical results. From May 1995 through June 1997, we carried out video-assisted extended thymectomy via sole left access in 16 patients. There were no deaths and no major complications. At the intermediate-term follow-up, remission and improvement rates were 19% and 56% respectively. These results were similar to those achieved on the last 16 patients who had extended thymectomy via median sternotomy. We believe that the advantages of the video-assisted approach render it a reliable option for the surgical management of autoimmune myasthenia.

Adult↗

[A case of limb-girdle type myasthenia gravis in whom rheumatoid arthritis appeared immediately after thymectomy].

We report a 48-year-old female who presented limb-girdle type myasthenia gravis with inflammatory lung lesions and rheumatoid arthritis. She demonstrated a rapidly progressive muscle weakness of extremities. Neurological examination revealed facial muscle weakness, and proximal dominant limb muscle atrophy and weakness. Ptosis, ophthalmoplegia, and bulbar palsy were not observed. The edrophonium test and serum anti-acetylcholine receptor antibody were positive. The repetitive nerve stimulation showed 55% waning in the thenar muscles. From these findings, she was diagnosed as having myasthenia gravis. Plain chest X-P and body CT showed tumor-like lesions in the lung. Lung biopsy revealed the infiltration of lymphocytes. These lesions decreased in size after thymectomy and corticosteroid administration. Immediately after thymectomy, she began to have morning stiffness with pain and swelling of the finger and knee joints. RAHA test, which was negative before thymectomy, became highly positive. These findings were consistent with rheumatoid arthritis. In this patient, thymus probably played a role to suppress the development of rheumatoid arthritis.

Arthritis, Rheumatoid↗

Extended thymectomy for myasthenia gravis in an octogenarian. A case report.

An 86-year-old woman with myasthenia gravis successfully underwent an extended thymectomy. The patient had a 2-year history of generalized myasthenia and had limited response to anticholinesterases and steroids. An extended thymectomy was successfully performed in the usual fashion. The postoperative course was uneventful except for an episode of psychosis probably due to postoperative steroid therapy. The steroid dosage was gradually reduced to 5 mg/day over seven months after the operation, during which she became fully asymptomatic. No symptoms due to myasthenia have surfaced for over 2 years after surgery. Although elderly patients are usually considered to be less responsive to an operation, thymectomy may sometimes be the treatment of choice for myasthenia gravis even in octogenarians.

Aged↗

Normalization of elevated CD4-/CD8- (double-negative) T cells after thymectomy parallels clinical remission in myasthenia gravis associated with thymic hyperplasia but not thymoma.

T-cell-dependent B-cell help is likely to be of major importance in the pathogenesis of myasthenia gravis, but mechanisms provoking a pathological anti-acetylcholine receptor (AChR) response are poorly understood. We report on the dysregulation of recently identified CD4-/CD8- (double-negative) T cells (DN T cells), which have been shown to participate in immunoregulation and antibody augmentation. Compared with healthy controls, significantly increased frequencies of DN T cells were found in the blood of myasthenia gravis patients with lymphofollicular hyperplasia. After thymectomy, however, normalization in the number of these cells was seen in parallel with clinical improvement and reduction in anti-AChR antibody titers. The effect of thymectomy was observed irrespective of adjuvant treatment and held true for up to 4 years of follow-up. In marked contrast, frequencies similar to control values were found in myasthenia gravis patients with thymoma, with thymectomy having no further reducing effect. These data indicate that CD4-/CD8- T cells not only participate in the pathogenesis of myasthenia gravis but also correlate with disease activity and histological findings.

Adult↗

Sevoflurane anesthesia in a myasthenic patient undergoing transsternal thymectomy.

Myasthenia gravis (MG) is an autoimmune disease resulting from the production of antibodies against the acetylcholine receptors of the neuromuscular synapse. The thymus gland is involved in the autosensitization process, and there is a consensus that all adults with generalized MG should have a thymectomy. Removal of a much thymic tissue as possible via the transsternal approach in the logic goal of thymectomy in the treatment of MG. Because of the unpredictable response to succinylcholine and the marked sensitivity to nondepolarizing muscle relaxants in the MG patients, some anesthesiologists avoid the use of muscle relaxants in the myasthenic patients, and depend on deep inhalation anesthesia, such as halothane, isoflurane or sevoflurane. In the present report, we used sevoflurane 4%, without supplementation by muscle relaxants, for the induction and maintenance of anesthesia in a MG patient undergoing transsternal thymectomy. The report reviews the anesthetic technique, and describes the electromyographic (EMG) changes following sevoflurane.

Adult↗

[Thymectomy in myasthenia gravis. A series of 68 patients].

Since January 1988 to December 1998, by the Department of Thoracic Surgery, Haut-Lévêque Hospital, University of Bordeaux, 68 consecutive patients were operated for myasthenia gravis. The aim of our study was to evaluate the results obtained in a group of patients who underwent a thymectomy for myasthenia gravis, in order to contribute for the determination of the prognostic factors which can influence the post-operative course. This series consists on 68 patients. Females were predominant, 41 patients (60.2%) versus 27 males (39.7%). The age extended between 15 and 80 years, average of 45.2 years. The follow-up concerned 52 patients (76.4%); out of there, 8 are in complete remission (15.3%), 31 (59.6%) are in a phase of clinical improvement, 11 patients did not benefit from thymectomy (21.1%), 1 patient died immediately after the surgical operation, I patient died some years after the surgical operation for another reason. In conclusion the thymectomy is a beneficial procedure for myasthenia gravis patients.

Adolescent↗

Transcervical thymectomy for myasthenia gravis.

The authors of this article contend that the transcervical approach for thymectomy allows the safe and complete removal of the thymus and [table: see text] provides equivalent benefit to the patients with regard to opportunity for clinical remission or freedom from progression of the symptoms of MG. The low morbidity and short hospitalization after transcervical thymectomy represent minimal barriers and allow increased willingness of the neurologist to refer a patient for surgical therapy and increased acceptance of the patient towards the recommendations. The authors believe that an early, safe, and complete thymectomy offers all the benefits of surgical removal of the thymus to a patient with MG with minimal risk for morbidity and postoperative pain.

Humans↗

Video-assisted thoracoscopy thymectomy for myasthenia gravis.

Over the past 8 years, the technique of video-assisted thoracoscopic surgery (VATS) thymectomy has continued to evolve. Although the procedure has become better defined and greater experience has been gained, numerous issues still exist. Whether it is best performed through a left sided, right sided, or as a bilateral approach with or without a cervical incision, is not clear. Equivalence of outcomes compared with more standard approaches to thymectomy is still an issue. Experience still is relatively limited to a few centers, and follow-up still is relatively short. In the author's own experience, it seems that availability of the less invasive approach has allowed thymectomy to be performed earlier in the course of the disease especially in young female patients in whom cosmesis is an important issue.

Adult↗

[Thymectomy for late-onset myasthenia gravis in the elderly (with report of 23 cases)].

OBJECTIVE: To evaluate the effect of thymectomy in the treatment of late-onset myasthenia gravis (MG) in the elderly. METHODS: Twenty-three such cases receiving thymectomy were followed up. RESULTS: MG was more frequent in male patients in the elderly, with generalized MG more prevalent that was often complicated by thymoma and myasthenia crisis. The total effective rate of the surgical intervention was 43.5 %. CONCLUSION: Thymectomy is applicable in late-onset MG in the elderly in spite of the fact that its effect is not as good as that in younger patients.

Age Factors↗

Effect of patient-controlled epidural analgesia for pain relief after thymectomy in patients with myasthenia gravis.

OBJECTIVE: To evaluate the effect of patient-controlled epidural analgesia (PCEA) with ropivacaine or bupivacaine in relieving pain after thymectomy in patients with myasthenia gravis. METHODS: Twenty adult ASA I-II patients with myasthenia gravis were randomized to receive either 0.125% ropivacaine (Group R, n=10) or 0.125% bupivacaine (Group B, n=10) with a PCEA device after transsternal thymectomy. PCEA (continuous infusion at 1 ml/h, bolus dose of 4 ml and lockout time of 30 min) was implemented via an epidural catheter inserted in the T3-4 intervertebral space. The vital signs and visual analogue scale (VAS), together with cumulative consumption (CC) of ropivacaine or bupivacaine were recorded within 48 h postoperatively. RESULTS: The vital signs, including systolic and diastolic blood pressure, heart rate, SpO2, pH and PaCO2, did not show any significant differences between the 2 groups. The CC of the local anesthetic was significantly higher in group R than that in group B at 24 and 48 h postoperatively, but VAS were not significantly different between the 2 groups which was less than 4 in both groups. CONCLUSIONS: PCEA with low concentration of ropivacaine or bupivacaine may provide effective and safe analgesia after transsternal thymectomy.

Adult↗

Thymectomy for myasthenia gravis: evaluation of results in 282 patients.

The results of thymectomy in 282 patients with acquired and generalised myasthenia gravis are presented. The study includes patients submitted to surgical treatment in the period between 1958 and 1990. Remission or marked improvement was obtained in 70% of the patients with follow-up extending from one to 25 years. The total post-operative mortality rate was 8% dying predominantly patients with thymoma. Median sternotomy was made in 278 patients, transcervical thymectomy in two patients and thyroidectomy with concomitant thymectomy in other two. Surgical methods, operatory phases, previous treatment with prednisone and long term evolution with final results are discussed.

Adolescent↗

Role of thymectomy in myasthenia gravis.

Thirteen patients with myasthenia gravis underwent total thymectomy between January, 1988 and December, 1991. The duration of symptoms prior to surgery varied from 2 months to 20 years. In a follow-up ranging from 2 months to 4 years, 11 patients showed a significant improvement with either complete discontinuation of medication or a marked reduction in doses. One patient with a small benign thymoma showed some improvement but subsequently required stepping up of anticholinesterase medication and addition of steroids and immunosuppressants; another patient with atrophic thymic tissue had complete remission after thymectomy but developed myasthenic symptoms six months later requiring medication again. Thymectomy is recommended for all patients with generalised myasthenia gravis with or without thymoma regardless of the duration of disease unless the patient is a very high risk candidate for surgery. It is not recommended for isolated ocular myasthenia gravis.

Adolescent↗

Successful treatment of pure red cell aplasia with cyclosporin A and erythropoietin after thymectomy in a 88-year old woman.

An 88-year old Japanese female with pure red cell aplasia was treated safely and effectively by a combination of thymectomy, cyclosporin A, and erythropoietin. The thymoma was histologically classified as lymphocytic type or cortical type, which are uncommon in cases of a thymoma accompanied by pure red cell aplasia. Immunohistochemical analysis of the thymoma and bone marrow revealed a predominance of CD8(+) cells. Thymectomy alone was ineffective, but cyclosporin A treatment subsequent to thymectomy was safe and effective and resulted in the disappearance of a Vbeta12 bearing T-cell clone in the bone marrow. Additional treatment with erythropoietin enhanced the effects of cyclosporin A and restored the patient's hemoglobin to normal levels. The beneficial effect of cyclosporin A may be attributed not to a broad immunomodulatory effect, but to a local effect on a limited T-cell subset.

Aged, 80 and over↗

[A retrospective group study on post-thymectomy myasthenia gravis].

Retrospective group study on post-thymectomy myasthenia gravis (post-Tm MG) was carried out. Five hundred and twenty-seven resected thymoma cases (133 cases with MG and 394 cases without MG) were collected from 9 hospitals. Post-Tmx MG occurred in 18 out of 394 cases -4.6% of thymoma without MG-, and these 18 patients with post-Tmx MG were investigated retrospectively. As to surgical procedures, the mode of operation, either thymectomy or thymo-thymectomy was not thought to be an important factor in the pathogenesis of post-Tmx MG. These 18 patients could be divided into 7 with early onset and 11 with late onset. In the early onset group, post-Tmx MG occurred within 6 months after the operation, and these 7 patients may have had subclinical MG at the time of the initial operation. In the late onset group, post-Tmx MG was noted in 5 patients when recurrence of the tumor was confirmed, the latest being 11 years after operation. The pathogenesis of post-Tmx MG could not be clarified in the other 6 patients (33.3%). For further evaluation of these 6 cases, a prospective studies with large scale are needed.

Adolescent↗

Rat thymectomy effects on leptin receptor and T-bet: erythroid hyperplasia with maturation arrest and suppressed T-cell-mediated hepatotoxicity.

Thymectomy is an inevitable therapy for thymoma. Therefore, determining hemato-immune changes post-thymectomy is important. Twenty-six normal LEW/Sea rats thymectomized (Tx) at the ages of 38 +/- 5 days were followed without any treatment for 4 months (experiments [Exp] A and B). In addition, 16 LEW/Sea rats Tx at the age of 42 days (Exp C and D) and 10 non-Tx control LEW/Sea rats (Exp E) were immunized with syngeneic male liver cells 2 to 3 times and followed 2.7 months after the first immunization. Flow cytometric (FCM) analysis of mesenteric lymph nodes (MLN) and peripheral blood (PB) showed as follows: among the 26 Tx rats (Exp A and B), MLN lymphocyte population at 4 months postthymectomy was characterized by decreased numbers of CD4+ cells (22%-36%) or alphabeta T-cell receptor (TCR)-positive cells (16%-54%) and increased numbers of interleukin 2 receptors (IL-2R) (>90%). In the 16 Tx-immunized rats (Exp C and D), both alphabeta TCR (MLN) and CD45R (PB) expression on lymphocytes was suppressed with rather high numbers of CD4. Bone marrow (BM) and PB hematological studies of the partially and totally Tx rats indicated the following: 8 BM of the 10 totally Tx males (Exp A) and the 7/10 male spleen showed erythroid hyperplasia with maturation arrest at the stage of basophilic erythroblasts and reticulocytopenia in the PB. One of the 10 males, which had both BM myeloid to erythroid (M/E) ratio of 4.3 and spleen myeopoiesis, was in a more advanced stage, a prestage of pure red cell aplasia (PRCA) with 22% of CD4+ MLN cells. Syngeneic liver cell immunization resulted in the Tx rats as follows: hepatotoxicity based on the immunization was weaker in the 16 Tx rats (Exp C and D) than in the 10 non-Tx rats (Exp E). Polymerase chain reaction (PCR) of PB and MLN showed compensatory activated leptin receptor (LR) and T-bet DNA in 19 Tx rats (Exp A and B) with abnormal FCM findings. The ineffective erythropoiesis at 4 months after thymectomy was explained by the erythroblast LR/erythropoietin receptor (EPOR) dysfunction. The low grade hepatotoxicity in the Tx rats (Exp C and D) was explained by the disturbed Th1 reactions (or the disturbed T-bet gene transcription) at postthymectomy.

Animals↗