Researchers found thymectomy to be beneficial for patients with childhood-onset myasthenia gravis (MG) and may be used as a therapeutic tool, as published in the European Journal of Pediatric Surgery.

They conducted an observational retrospective study in China that included 32 patients of both sexes previously diagnosed with MG at 14 years old or younger. At the time of surgery, the median age was 13.77±3.35, and follow-up was conducted in the short-term at 1 year and the long-term at 5 years.

The prognosis after thymectomy was classified into either complete stable remission, pharmacological remission, minor manifestation, improvement, unchanged, worse, exacerbation, or death, as it is specified by the Myasthenia Gravis Foundation of America.

The authors defined a good outcome if improvement or better status was achieved. In 1 year following surgery, 65.63% had a good outcome, 53.12% in 3 years, and 62.50% in 5 years. Quantified MG and MG Activities of Daily Living scores were also assessed and shown to decrease progressively, especially in patients with generalized MG (GMG) in comparison with ocular MG.

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Furthermore, longer preoperative terms, males, and GMG constituted risk factors for worse short-term results. Conversely, a shorter time from the diagnosis to surgery and the MG subgroup were predicted as long-term improvements.

Moreover, a minimally invasive approach vs an open thymectomy had similar success rates, indicating that the thoracoscopy is not superior. Finally, postsurgical treatment with immunomodulators did not modify the prognosis. The use of thymectomy as a treatment for childhood-onset MG is questioned and not quite accepted, yielding a high relevance to these findings.

“Furthermore, there is insufficient evidence to determine the optimum timing for thymectomy in children. Our data showed that a long preoperative duration in patients with [childhood-onset MG] was related to the poor prognosis of thymectomy, so early surgical treatment is recommended,” the authors said.

“The association between the long duration and the poor prognosis is probably due to the prolonged and cumulative damage at the neuromuscular plate,” the authors concluded and said that although a small sample size, lack of randomization, and single-centered data constituted limitations of this publication, the promising results call for further studies.

Reference

Zhang Q, Cao Y, Bi Z, et al. Childhood-onset myasthenia gravis patients benefited from thymectomy in a long-term follow-up observation. Eur J Pediatr Surg. Published online March 9, 2022. doi:10.1055/s-0042-1744150