Orbital Eosinophilic Angiocentric Fibrosis Case Report and Review of the Literature

Abstract

Objectives To report a patient with a rare case of orbital eosinophilic angiocentric fibrosis (EAF) and to review the literature. Design Interventional case report. Methods A 61-year-old man presented with a 6-week history of right periorbital edema and painless proptosis. Examination revealed a nonaxial proptosis, lateral globe displacement, and mild limitation in right eye adduction. Main Outcome Measures Clinical course and radiological and histological findings. Results Orbital imaging revealed a right medial orbital mass with involvement of middle ethmoidal air cells. An orbital biopsy of the mass demonstrated an inflammatory infiltrate with a marked eosinophilic component, onion skinning of vessels, and surrounding fibrosis. The diagnosis of orbital EAF was made. There was no response to a 3-month treatment course with systemic steroids, but the patient did not want any further surgical interventions. Conclusion Although orbital EAF is rare, ophthalmologists need to be aware of this entity, as it may invade the orbit from the sinonasal tract or present as a localized orbital mass. The presence of even minimal sinus involvement and the characteristic histopathology are useful in establishing the correct diagnosis.