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

• DOI: 10.1080/01676830.2024.2448813
• Publication Date: 2025-02-24T15:05:10Z
• Authors: Mikayla J. Baker; Alisha Kamboj; Amanda C. Maltry; Raymond G. Areaux; Andrew R. Harrison; Ali Mokhtarzadeh
• Keywords: Granulomatosis with polyangiitis; Churg-Strauss Syndrome

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