Orbital eosinophilic angiocentric fibrosis: Case report and review of the literature

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.