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重症肌无力的“最大程度”胸腺切除术。结果

"Maximal" thymectomy for myasthenia gravis. Results.

作者信息

Jaretzki A, Penn A S, Younger D S, Wolff M, Olarte M R, Lovelace R E, Rowland L P

机构信息

Department of Surgery, Columbia-Presbyterian Medical Center, New York, NY 10032.

出版信息

J Thorac Cardiovasc Surg. 1988 May;95(5):747-57.

PMID:3361927
Abstract

Thymectomy has been shown to be effective in the treatment of myasthenia gravis. The logical goal of operation has been complete removal of the thymus, but there has been controversy about the surgical technique and its relation to results. Surgical-anatomic studies have shown gross and microscopic thymus widely distributed in the neck and mediastinum. We believe that an en bloc transcervical-transsternal "maximal" thymectomy is required to remove all thymic tissue predictably. Ninety-five patients with generalized myasthenia gravis underwent "maximal" thymectomy consecutively between 1977 and 1985 and were evaluated 6 months to 89 months after operation. In Group A (N = 72), myasthenia gravis without thymoma, the uncorrected data revealed that 96% (69) had benefited from operation: 79% (57) had no symptoms; 46% (33) were in remission; 33% (24) were symptom free when receiving minimal doses of pyridostigmine; and none were worse. Life table analysis yielded a remission rate of 81% at 89 months. In group B (N = 8), myasthenia gravis without thymoma for which patients underwent reexploration for incapacitating weakness after earlier transcervical or transsternal operations, residual thymus was found in all. One patient was in remission, two were symptom free when receiving medication, one was unchanged, and none were worse. In group C (N 15), myasthenia gravis and thymoma, two patients were in remission and nine were symptom free when receiving medication. Two patients in this group died 2 and 4 years postoperatively in crisis. Response to thymectomy in group A was greater in patients with mild myasthenia gravis and may have been better in patients who had symptoms for less than 60 months preoperatively, but the response did not depend on age, sex, presence or absence of thymic hyperplasia or involution, or titers of acetylcholine receptor antibodies. The response to thymectomy in group B was striking but slower than in group A, perhaps because symptoms were more severe and of longer duration. The response in group C was also less good than in group A and proportionately fewer benefited. These results support the recommendation for thymectomy in the treatment of patients with generalized myasthenia gravis and indicate the desirability of a maximal procedure. For persistent or recurrent severe symptoms after previous transcervical or submaximal transsternal resections, reoperation by this technique is also recommended.

摘要

胸腺切除术已被证明对重症肌无力的治疗有效。手术的合理目标是完全切除胸腺,但手术技术及其与治疗效果的关系一直存在争议。外科解剖学研究表明,大体及显微镜下可见的胸腺广泛分布于颈部和纵隔。我们认为,需要采用经颈部 - 经胸骨整块“最大限度”胸腺切除术,才能可预测地切除所有胸腺组织。1977年至1985年间,95例全身型重症肌无力患者连续接受了“最大限度”胸腺切除术,并在术后6个月至89个月进行了评估。A组(n = 72)为无胸腺瘤的重症肌无力患者,未经校正的数据显示,96%(69例)患者从手术中获益:79%(57例)无症状;46%(33例)缓解;33%(24例)在接受最小剂量吡啶斯的明时无症状;且无一例病情恶化。寿命表分析显示,89个月时缓解率为81%。B组(n = 8)为无胸腺瘤的重症肌无力患者,这些患者在早期经颈部或经胸骨手术后因肌无力失能而接受再次探查,结果发现均有残留胸腺。1例患者缓解,2例在接受药物治疗时无症状,1例病情无变化,且无一例病情恶化。C组(n = 15)为重症肌无力合并胸腺瘤患者,2例缓解,9例在接受药物治疗时无症状。该组2例患者术后2年和4年因危象死亡。A组中,轻症重症肌无力患者对胸腺切除术的反应更好,术前症状出现时间少于60个月的患者反应可能更佳,但反应并不取决于年龄、性别、胸腺增生或萎缩的有无,或乙酰胆碱受体抗体滴度。B组对胸腺切除术的反应显著,但比A组慢,可能是因为症状更严重且持续时间更长。C组的反应也不如A组好,受益患者比例相对较少。这些结果支持在全身型重症肌无力患者治疗中推荐胸腺切除术,并表明采用最大限度手术的可取性。对于先前经颈部或次全经胸骨切除术后持续或复发的严重症状,也建议采用该技术再次手术。

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