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Does Thymectomy Improve Outcomes in Patients with Nonthymomatous Myasthenia Gravis?

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Difficult Decisions in Thoracic Surgery

Part of the book series: Difficult Decisions in Surgery: An Evidence-Based Approach ((DDSURGERY))

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Abstract

Thymectomy in cases of nonthymomatous myasthenia gravis has undergone investigation from multiple angles over the past several decades. Though many of the early, retrospective trials suggested a clinical benefit in patients with myasthenia gravis undergoing thymectomy, the completion of the MGTX trial (a randomized, multi-centered, rater blinded trial) has provided the strongest possible evidence of a multi-dimensional benefit of thymectomy compared to medical therapy alone in acetylcholine-receptor antibody positive myasthenia gravis. This therapeutic benefit not only manifests in clinical parameters (e.g., decreased medication requirements and decreased subsequent hospitalizations), but also in quality-of-life metrics. Furthermore, recent studies have shown the safety and efficacy of thoracoscopic, minimally-invasive thymectomy. To date, high-quality evidence only supports the routine use of thymectomy in patients 18–65 years of age with acetylcholine receptor positive myasthenia. There is no high-quality evidence to suggest this same benefit extends to acetylcholine-receptor antibody positive patients over 65 years of age, patients with ocular myasthenia, or patients with seronegative myasthenia. It is reasonable to consider and discuss thymectomy with patients that fall into one of these subgroups in cases of medically-refractory disease. Currently there is no data to support any benefit of thymectomy in patients with LRP4 or MuSK antibody positive myasthenia, thus, thymectomy is not recommended in these two subgroups.

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References

  1. Wilks S. On cerebritis, hysteria and bulbar paralysis, as illustrative of arrest of function of the cerebrospinal centres. Guys Hosp Rep. 1877;22:7–55.

    Google Scholar 

  2. Cooper JD. History of thymectomy for myasthenia gravis. Thorac Surg Clin. 2019;29(2):151–8.

    Article  Google Scholar 

  3. Weigert C. II. Pathologisch-anatomischer Beitrag zur Erb’schen Krankheit (Myasthenia gravis). 1901.

    Google Scholar 

  4. Bell ET. Tumors of the thymus in myasthenia gravis. J Nerv Ment Dis. 1917;45(130)

    Google Scholar 

  5. Blalock A, Harvey AM, Ford FR, Lilienthal JL. The treatment of myasthenia gravis by removal of the thymus gland: preliminary report. JAMA. 1941;117(18):1529–33.

    Article  Google Scholar 

  6. Perlo VP, Poskanzer DC, Schwab RS, Viets HR, Osserman KE, Genkins G. Myasthenia gravis: evaluation of treatment in 1,355 patients. Neurology. 1966;16(5):431.

    Article  CAS  Google Scholar 

  7. Kirschner PA, Osserman KE, Kark AE. Studies in myasthenia gravis: transcervical total thymectomy. JAMA. 1969;209(6):906–10.

    Article  CAS  Google Scholar 

  8. Buckingham JM, Howard FM Jr, Bernatz PE, Payne WS, Harrison EG Jr, O’Brien PC, Weiland LH. The value of thymectomy in myasthenia gravis: a computer-assisted matched study. Ann Surg. 1976;184(4):453.

    Article  CAS  Google Scholar 

  9. Wolfe GI, Kaminski HJ, Aban IB, Minisman G, Kuo HC, Marx A, et al. Randomized trial of thymectomy in myasthenia gravis. NEJM. 2016;375(6):511–22.

    Article  Google Scholar 

  10. Wolfe GI, Kaminski HJ, Aban IB, Minisman G, Kuo HC, Marx A, et al. Long-term effect of thymectomy plus prednisone versus prednisone alone in patients with non-thymomatous myasthenia gravis: 2-year extension of the MGTX randomised trial. Lancet Neurol. 2019;18(3):259–68.

    Article  CAS  Google Scholar 

  11. Popescu I, Tomulescu V, Ion V, Tulbure D. Thymectomy by thoracoscopic approach in myasthenia gravis. Surg Endosc. 2002;16(4):679–84.

    Article  CAS  Google Scholar 

  12. Zieliński M, Hauer Ł, Kużdżał J, Sośnicki W, Harazda M, Pankowski J, Nabialek T, Szlubowski A. Technique of the transcervical-subxiphoid-videothoracoscopic maximal thymectomy. J Minim Access Surg. 2007;3(4):168.

    Article  Google Scholar 

  13. Rückert JC, Ismail M, Swierzy M, Sobel H, Rogalla P, Meisel A, Wernecke KD, Rückert RI, Müller JM. Thoracoscopic thymectomy with the da Vinci robotic system for myasthenia gravis. Ann N Y Acad Sci. 2008;1132(1):329–35.

    Article  Google Scholar 

  14. Tomulescu V, Sgarbura O, Stanescu C, Valciu C, Campeanu A, Herlea V, Popescu I. Ten-year results of thoracoscopic unilateral extended thymectomy performed in nonthymomatous myasthenia gravis. Ann Surg. 2011;254(5):761–6.

    Article  Google Scholar 

  15. Rückert JC, Swierzy M, Ismail M. Comparison of robotic and nonrobotic thoracoscopic thymectomy: a cohort study. J Thorac Cardiovasc Surg. 2011;141(3):673–7.

    Article  Google Scholar 

  16. Jaretzki A III, Bethea M, Wolff M, Olarte MR, Lovelace RE, Penn AS, Rowland L. A rational approach to total thymectomy in the treatment of myasthenia gravis. Ann Thorac Surg. 1977;24(2):120–30.

    Article  Google Scholar 

  17. Jaretzki A. Thymectomy for myasthenia gravis: analysis of the controversies regarding technique and results. Neurology. 1997;48(Suppl 5):52S–63S.

    Article  Google Scholar 

  18. Singh G, Glotzbach J, Costa J, Gorenstein L, Ginsburg M, Sonett JR. Minimally invasive radical thymectomy. Ann Cardiothorac Surg. 2016;5(1):59.

    PubMed  PubMed Central  Google Scholar 

  19. Kerty E, Elsais A, Argov Z, Evoli A, Gilhus NE. EFNS/ENS Guidelines for the treatment of ocular myasthenia. Eur J Neurol. 2014;21(5):687–93.

    Article  CAS  Google Scholar 

  20. Gilhus NE. Myasthenia gravis. NEJM. 2016;375(26):2570–81.

    Article  CAS  Google Scholar 

  21. Bacchi S, Kramer P, Chalk C. Autoantibodies to low-density lipoprotein receptor-related protein 4 in double seronegative myasthenia gravis: a systematic review. Can J Neurol Sci. 2018;45(1):62–7.

    Article  Google Scholar 

  22. Guptill JT, Sanders DB, Evoli A. Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts. Muscle Nerve. 2011;44(1):36–40.

    Article  Google Scholar 

  23. Gilhus NE, Verschuuren JJ. Myasthenia gravis: subgroup classification and therapeutic strategies. Lancet Neurol. 2015;14(10):1023–36.

    Article  CAS  Google Scholar 

  24. Sanders DB, Wolfe GI, Benatar M, Evoli A, Gilhus NE, Illa I, Kuntz N, Massey JM, Melms A, Murai H, Nicolle M, Palace J, Richman DP, Verschuuren J, Narayanaswami P. International consensus guidance for management of myasthenia gravis: executive summary. Neurology. 2016;87(4):419–25.

    Article  Google Scholar 

  25. Sonnet JR, Jaretzki A III. Thymectomy for nonthymomatous myasthenia gravis. A critical review. Ann N Y Acad Sci. 2008;1132:315–28.

    Article  Google Scholar 

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Dubois, R., Sonett, J. (2020). Does Thymectomy Improve Outcomes in Patients with Nonthymomatous Myasthenia Gravis?. In: Ferguson, M. (eds) Difficult Decisions in Thoracic Surgery. Difficult Decisions in Surgery: An Evidence-Based Approach. Springer, Cham. https://doi.org/10.1007/978-3-030-47404-1_53

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  • DOI: https://doi.org/10.1007/978-3-030-47404-1_53

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  • Publisher Name: Springer, Cham

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