Pediatric Ocular Myasthenia Gravis
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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.
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.
KeywordsPediatric 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
- 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
- 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
- 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
- 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.•• 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.•• 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