Poster 17

by in  Poster Session 1

Extraocular Movement Deficits Secondary to Granulomatosis with Polyangiitis Lesions in Children 

Andre N. Ali-Ridha, MD; Majd Mustafa, MD; Christian El-Hadad, MD; Jia Yue You, BSc; Ayesha Khan, MD
McGill University
Montreal, Canada

 

Introduction: To describe a small series of patients presenting with extraocular movement deficits secondary to rare orbital lesions that were biopsy proven positive for granulomatosis with polyangiitis (GPA).

Methods: The study is an observational case series of three children who presented with symptoms associated with GPA and were found to have variable degrees of binocular diplopia and extraocular motor deficits on initial examination.

Results: Specific characteristics of the patients included one child who had binocular diplopia and a very mild (-1) restriction in elevation and abduction with ptosis and erythema of the upper eyelid with a small, firm, non-mobile mass on palpation. The second patient also had binocular diplopia with a mild to moderate (-2) restriction and pain in upgaze with ptosis and swelling of the upper eyelid. The last patient had very mild restriction in upgaze (-0.5) with proptosis and enlargement of the lacrimal gland. All three children had full resolution of their extraocular motor deficits and diplopia following treatment for their GPA lesions.

Discussion: Orbital lesions that are less commonly encountered in children including granulomatosis with polyangiitis can cause differing degrees of impairment in extraocular movements with variable symptoms that often resolve with proper treatment of the underlying pathology.

Conclusion: Due to the transient nature of extraocular motor deficits in vasculitis-related orbital lesions in children, these patients should be treated by addressing the primary cause of their lesions based on histopathological diagnosis which often leads to rapid resolution of any impairments without the need for further unnecessary medical or invasive surgical management.

References: Grisaru S, Yuen GWH, Miettunen PM, Hamiwka LA. Incidence of Wegener’s granulomatosis in children. J Rheumatol 2010;37:440-2.
Levi M, Kodsi SR, Rubin SE, Lyons C, Golden R, Olitsky SE. Ocular involvement as the initial manifestation of Wegener’s granulomatosis in children. J Am Assoc Pediatr Ophthalmol Strabismus 2008 ;12:94-6.
Haynes BF, Fishman ML, Fauci AS, Wolff SM. The ocular manifestations of Wegener’s granulomatosis. Fifteen years experience and review of the literature. Am J Med 1977;63:131-41.

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