Effectiveness of thymectomy in juvenile myasthenia gravis and clinical characteristics associated with better outcomes.

Abstract

Thymectomy is an established treatment in adult myasthenia gravis, but its exact role in juvenile myasthenia gravis (JMG) is still uncertain. Thymectomy is frequently considered in the treatment of severe, medically refractory JMG. Surgical approaches have evolved from open median sternotomy to the more cosmesis-preserving thoracoscopic approach. This paper reviews current evidence on the effectiveness of thymectomy in JMG and discusses clinical characteristics which may be associated with improved outcomes. 17 studies including 588 patients who underwent thymectomy from 1997 to 2020 were found, which either reported uncontrolled cohorts undergoing thymectomy, or compared cohorts undergoing different surgical approaches. An improvement in clinical status or reduced requirement for medical therapy following thymectomy was seen in 453 patients (77%). Complete remission was seen in 40% (n = 172/430). Thoracoscopic approaches may provide improved outcomes, fewer complications, and better cosmetic outcomes. Better surgical outcomes may be associated with early intervention, intervention after the onset of puberty, being acetylcholine receptor antibody positive, having more severe disease and the presence of hyperplastic thymic tissue. However, analysis remains hindered by the limitations of the currently available retrospective studies of small cohorts. Nonetheless, available literature suggests a role for thymectomy in JMG patients, especially those with certain clinical characteristics.