Bilateral orbital inflammation in a pediatric patient with eosinophilic granulomatosis with polyangiitis.

Abstract

A 5-year-old female presented to the ophthalmology clinic with one month of left upper eyelid ptosis and three months of intermittent wheezing and coughing. MRI of the brain and orbits revealed infiltrative enhancement involving both orbits, including the retro-maxillary space, maxillary sinus walls, greater wing of the sphenoid, and possibly the left cavernous sinus. She experienced acute respiratory decompensation in the setting of pansinusitis and tree-in-bud nodular pulmonary opacifications. Laboratory workup revealed anemia, thrombocytosis, and elevated inflammatory markers. An orbitotomy with biopsy demonstrated an eosinophil-rich granulomatous infiltrate, consistent with eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome. The patient was placed on high-dose steroids and mepolizumab. To our knowledge, this patient represents the youngest case of EGPA with orbital involvement. Further, this case illustrates the importance of prompt orbital biopsy to provide a timely, unified diagnosis, enabling specialists to initiate appropriate disease management to reduce morbidity and mortality.