ICNC Abstracts, ICNC 2018

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Long term Outcome of Thymectomy in Pediatric Myasthenia Gravis
leeha Singh, Leeha Singh, Shilpa Kulkarni, Anaita Hegde, K. N. Shah

Last modified: 2018-09-09

Abstract


Introduction:

The importance of thymectomy in the treatment of Pediatric myasthenia gravis (JMG) is poorly defined.  Thymectomy for the treatment of myasthenia gravis is based on several lines of evidence that support a central role of the thymus in the pathogenesis of the disease. This study aims to highlight its role in Pediatric  myasthenia gravis by describing a cohort of patient who underwent thymectomy.

Methods:

In a retrospective study patients with pediatric myasthenia gravis who underwent thymectomy in the pediatric neurology department from 2005 to 2017 were included in the analysis. Long term outcome of thymectomy was studied.

Results:

Total of 18 patients were diagnosed as acquired MG. Four patients (2 Females, 2 males) underwent thymectomy. Average age of presentation was 3.5 years. All patients progressed to develop generalized myasthenia. According to MFGA clinical classification 3 patients had IIIa grading and one had grading of V. All patients failed long term steroid therapy along with high dose pyridostigmine. One patient went on to develop myasthenic crisis. All patients received pulse steroids and IVIg before surgery. Average age of thymectomy was 7 years. Average follow up period was 4 years. On histology all 4 patients had thymic hyperplasia. Three patients (75%) had Anti-acetyl cholinesterase antibodies positive.  Two patient (50%) attained remission with gradually decreasing requirement of pyridostigmine. One (25%) had transient benefit and one (25%) died due to complications of thymectomy.

Conclusion:

Thymectomy is a viable treatment option in pediatric myasthenia gravis. Role of thymectomy and patient selection needs to better defined.


Keywords


Myasthenia Gravis, thymectomy, outcome

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