Thymectomy in Children with Juvenile Myasthenia Gravis: Is It Recommended?

Abstract

Objective: Juvenile myasthenia gravis (JMG) is a rare disease with possible severe forms. Thymectomy is supported by many authors particularly in generalised forms with positive AcetylCholine Receptor Antibody (AChR- Ab). The aim of our study was to discuss the indication and the outcome of thymectomy in five children with JMG. Methods: We performed a retrospective study of the medical files of patients with JMG, who were hospitalised in our pediatric surgery department for thymectomy. For all the children we performed Acetylcholine Receptor Autoantibody (ACHRAB) test-system producer, a chest x-ray, a chest Computed Tomography Scan (CT-scan) and a Magnetic Resonance Imaging (MRI). Thymectomy was indicated on the presence of anomalies on CT-Scan or MRI and realised via either thoracotomy or video-assisted thoracoscopy. The study was conducted with the approval of the ethics committee at our institution. Results: Based on our findings, thymectomy in JMG with imaging anomalies seems to be associated with clinical improvement. In our patients complete remission after surgery was seen in one case, a clinical improvement with a decrease of the medical therapeutic dosage was noted in three other cases. One patient could not be evaluated because of non adherence to medical treatment. Surgery by thoracotomy or video assisted thoracoscopy (VAT) was also well tolerated by all the patients. Conclusion: The benefits of thymectomy in children with JMG are still controversial. However, many researchers have reported a sustained improvement of symptoms in the majority of patients after thymectomy. Our findings seem to encourage thymectomy in cases of JMG and particularly when imaging anomalies are found. VAT thymectomy was well tolerated and it is considered by many researchers to be the standard method for thymectomy in children.