Pediatric Ocular Myasthenia Gravis

  • Kristen Fisher
  • Veeral ShahEmail author
Neurologic Ophthalmology and Otology (R Shin and D Gold, Section Editors)
Part of the following topical collections:
  1. Topical Collection on Neurologic Ophthalmology and Otology


Purpose of review

We present a review of current strategies in the treatment of pediatric ocular myasthenia gravis (OMG). A critical appraisal was performed of the current literature available on OMG and the treatment options available for all age populations. From this data, we present the evidence surrounding therapeutic options for pediatric OMG and discuss treatment outcomes in the pediatric population. We also present gaps in the literature with regard to pediatric OMG and possibilities for future research.

Recent findings

While there is data on the use of steroid-sparing immunosuppressive agents for myasthenia gravis (MG), as a whole it is more specific for the use in generalized juvenile myasthenia gravis (JMG) and more focused toward the adult population. In the currently available literature, there have been reports published on the use of steroid-sparing agents including azathioprine, cyclosporine, and mycophenolate mofetil. A recent open-label trial has provided evidence for the use of tacrolimus to improve symptoms of JMG including OMG that were refractory to prednisone. In addition to this, there has been evidence that thymectomy is effective in controlling pediatric OMG and shows a pattern toward preventing generalization of MG, reducing prednisone dosing, and increasing resolution of disease. There are other treatments used in the pediatric population of MG, including intravenous immunoglobulin (IVIG), plasmapheresis, and rituximab, but currently there are no reports on use in OMG.


In the population of patients with pediatric OMG, a high percentage of patients are able to obtain stabilization of symptoms using only pyridostigmine or pyridostigmine in combination with oral prednisone. Rates of generalization of OMG range from 15 to 35%, with higher rates in the adolescent population, but approximately 25% of pediatric patients with OMG can have complete remission. For pediatric OMG patients with ophthalmologic manifestations that are refractory to pyridostigmine and prednisone, the use of steroid-sparing agents has been practiced more recently. In addition to this, thymectomy has been utilized in this population. In both of these instances, no pediatric randomized control trials have been performed to date.


Pediatric ocular myasthenia gravis Pediatric myasthenia gravis Ptosis Ophthalmoplegia 


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    Phillips LH, Torner JC, Anderson MS, Cox GM. The epidemiology of myasthenia gravis in central and western Virginia. Neurology. 1992;42(10):1888–93.CrossRefGoogle Scholar
  2. 2.
    Andrews PI. Autoimmune myasthenia gravis in childhood. Semin Neurol. 2004;24(1):101–10.CrossRefGoogle Scholar
  3. 3.
    Vanderpluym J, Vajsar J, Jacob FD, Mah JK, Grenier D, Kolski H. Clinical characteristics of pediatric myasthenia: a surveillance study. Pediatrics. 2013;132(4):e939–44.CrossRefGoogle Scholar
  4. 4.
    Kim JH, Hwang JM, Hwang YS, Kim KJ, Chae J. Childhood ocular myasthenia gravis. Ophthalmology. 2003;110(7):1458–62.CrossRefGoogle Scholar
  5. 5.
    Pineles SL, Avery RA, Moss HE, et al. Visual and systemic outcomes in pediatric ocular myasthenia gravis. Am J Ophthalmol. 2010;150(4):453–459.e3.CrossRefGoogle Scholar
  6. 6.
    •• Vanikieti K, Lowwongngam K, Padungkiatsagul T, Visudtibhan A, Poonyathalang A. Juvenile ocular myasthenia gravis: presentation and outcome of a large cohort. Pediatr Neurol. 2018;87:36–41 A recent large cohort of juvenile patients with OMG is reported in this article, discussing symptom presentation and treatment outcomes.CrossRefGoogle Scholar
  7. 7.
    Liu Z, Feng H, Yeung SC, et al. Extended transsternal thymectomy for the treatment of ocular myasthenia gravis. Ann Thorac Surg. 2011;92(6):1993–9.CrossRefGoogle Scholar
  8. 8.
    Motobayashi M, Inaba Y, Nishimura T, Kobayashi N, Nakazawa Y, Koike K. An increase in circulating B cell-activating factor in childhood-onset ocular myasthenia gravis. Pediatr Neurol. 2015;52(4):404–9.CrossRefGoogle Scholar
  9. 9.
    •• Liu C, Gui M, Cao Y, et al. Tacrolimus improves symptoms of children with myasthenia gravis refractory to prednisone. Pediatr Neurol. 2017;77:42–7 Recent study published on the use of tacrolimus in 14 children with MG and its effectiveness.CrossRefGoogle Scholar
  10. 10.
    • Smith SV, Lee AG. Update on ocular myasthenia gravis. Neurol Clin. 2017;35(1):115–23 Recent relevant review on OMG, diagnostic modalities, and treatment options.CrossRefGoogle Scholar
  11. 11.
    Mullaney P, Vajsar J, Smith R, Buncic JR. The natural history and ophthalmic involvement in childhood myasthenia gravis at the hospital for sick children. Ophthalmology. 2000;107(3):504–10.CrossRefGoogle Scholar
  12. 12.
    Lee HN, Kang HC, Lee JS, Kim HD, Shin HY, Kim SM, et al. Juvenile myasthenia gravis in Korea: subgroup analysis according to sex and onset age. J Child Neurol. 2016;31(14):1561–8.CrossRefGoogle Scholar
  13. 13.
    Mittal MK, Barohn RJ, Pasnoor M, McVey A, Herbelin L, Whittaker T, et al. Ocular myasthenia gravis in an academic neuro-ophthalmology clinic: clinical features and therapeutic response. J Clin Neuromuscul Dis. 2011;13(1):46–52.CrossRefGoogle Scholar
  14. 14.
    Ortiz S, Borchert M. Long-term outcomes of pediatric ocular myasthenia gravis. Ophthalmology. 2008;115(7):1245–1248.e1.CrossRefGoogle Scholar
  15. 15.
    Peragallo JH, Velez FG, Demer JL, Pineles SL. Long-term follow-up of strabismus surgery for patients with ocular myasthenia gravis. J Neuroophthalmol. 2013;33(1):40–4.CrossRefGoogle Scholar
  16. 16.
    Park KA, Oh SY. Treatment for diplopia in patients with myasthenia gravis. Graefes Arch Clin Exp Ophthalmol. 2013;251(3):895–901.CrossRefGoogle Scholar
  17. 17.
    Bradley EA, Bartley GB, Chapman KL, Waller RR. Surgical correction of blepharoptosis in patients with myasthenia gravis. Ophthalmic Plast Reconstr Surg. 2001;17(2):103–10.CrossRefGoogle Scholar
  18. 18.
    • Brogan K, Farrugia ME, Crofts K. Ptosis surgery in patients with myasthenia gravis: a useful adjunct to medical therapy. Semin Ophthalmol. 2018;33(3):429–34 A report of patients with persistent ptosis in MG who have undergone surgical correction and discussion of the benefits and outcome of the procedure.CrossRefGoogle Scholar
  19. 19.
    • Belliveau MJ, Oestreicher JH. Ptosis repair in ocular myasthenia gravis. Semin Ophthalmol. 2017;32(5):564-8 Additional case series of patients with OMG and persistent ptosis who have undergone surgical repair and the outcomes related to the procedure.CrossRefGoogle Scholar
  20. 20.
    Pruitt JA, Ilsen PF. On the frontline: what an optometrist needs to know about myasthenia gravis. Optometry. 2010;81(9):454–60.CrossRefGoogle Scholar
  21. 21.
    Han J, Han SY, Han SH, Lee JB. Strabismus surgery and long-term visual outcomes in patients with preadolescent onset ocular myasthenia gravis. Graefes Arch Clin Exp Ophthalmol. 2015;253(1):157–63.CrossRefGoogle Scholar
  22. 22.
    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.CrossRefGoogle Scholar
  23. 23.
    Sanders DB, Evoli A. Immunosuppressive therapies in myasthenia gravis. Autoimmunity. 2010;43(5–6):428–35.CrossRefGoogle Scholar
  24. 24.
    Ionita CM, Acsadi G. Management of juvenile myasthenia gravis. Pediatr Neurol. 2013;48(2):95–104.CrossRefGoogle Scholar
  25. 25.
    Ishigaki K, Shishikura K, Murakami T, Suzuki H, Hirayama Y, Osawa M. Benefits of FK 506 for refractory eye symptoms in a young child with ocular myasthenia gravis. Brain Dev. 2009;31(8):634–7.CrossRefGoogle Scholar
  26. 26.
    Ware TL, Ryan MM, Kornberg AJ. Autoimmune myasthenia gravis, immunotherapy and thymectomy in children. Neuromuscul Disord. 2012;22(2):118–21.CrossRefGoogle Scholar
  27. 27.
    • Ashfaq A, Bernes SM, Weidler EM, Notrica DM. Outcomes of thoracoscopic thymectomy in patients with juvenile myasthenia gravis. J Pediatr Surg. 2016;51(7):1078–83 A relevant case series on outcomes of patients with MG who have undergone thymectomy and discussion of efficacy of thymectomy.CrossRefGoogle Scholar
  28. 28.
    Hennessey IA, Long AM, Hughes I, Humphrey G. Thymectomy for inducing remission in juvenile myasthenia gravis. Pediatr Surg Int. 2011;27(6):591–4.CrossRefGoogle Scholar
  29. 29.
    •• Kim AG, Upah SA, Brandsema JF, Yum SW, Blinman TA. Thoracoscopic thymectomy for juvenile myasthenia gravis. Pediatr Surg Int. 2019;35(5):603–10 Most recent and largest cohort of juvenile patients who have undergone thymectomy, discussion of outcomes and evidence relating to efficacy of the procedure.CrossRefGoogle Scholar
  30. 30.
    •• Zhu K, Li J, Huang X, et al. Thymectomy is a beneficial therapy for patients with non-thymomatous ocular myasthenia gravis: a systematic review and meta-analysis. Neurol Sci. 2017;38(10):1753–60 This article specifically discusses the outcomes of patients with OMG who have undergone thymectomy, review of literature, and review of patients.CrossRefGoogle Scholar
  31. 31.
    •• Catalano MA, Mullan CW, Rich BS, Glick RD. Pediatric thymectomy: a study of national trends in demographics, short-term outcomes, and cost. Pediatr Surg Int. 2019;35(7):749–57.A cross-sectional analysis looking at thymectomy in the pediatric population, safety, and outcomes with the procedure.CrossRefGoogle Scholar
  32. 32.
    Kaba E, Cosgun T, Ayalp K, Toker A. Robotic thymectomy for myasthenia gravis. Ann Cardiothorac Surg. 2019;8(2):288–91.CrossRefGoogle Scholar
  33. 33.
    Selcen D, Dabrowski ER, Michon AM, Nigro MA. High-dose intravenous immunoglobulin therapy in juvenile myasthenia gravis. Pediatr Neurol. 2000;22(1):40–3.CrossRefGoogle Scholar
  34. 34.
    Wylam ME, Anderson PM, Kuntz NL, Rodriguez V. Successful treatment of refractory myasthenia gravis using rituximab: a pediatric case report. J Pediatr. 2003;143(5):674–7.CrossRefGoogle Scholar
  35. 35.
    Tzaribachev N, Koetter I, Kuemmerle-deschner JB, Schedel J. Rituximab for the treatment of refractory pediatric autoimmune diseases: a case series. Cases J. 2009;2:6609.CrossRefGoogle Scholar
  36. 36.
    Zebardast N, Patwa HS, Novella SP, Goldstein JM. Rituximab in the management of refractory myasthenia gravis. Muscle Nerve. 2010;41(3):375–8.CrossRefGoogle Scholar
  37. 37.
    Dhillon S. Eculizumab: a review in generalized myasthenia gravis. Drugs. 2018;78(3):367–76.CrossRefGoogle Scholar
  38. 38.
    Edmundson C, Guidon AC. Eculizumab: a complementary addition to existing long-term therapies for myasthenia gravis. Muscle Nerve. 2019;60(1):7–9.PubMedGoogle Scholar

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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Ophthalmology, Texas Children’s HospitalBaylor College of MedicineHoustonUSA

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