Orbital eosinophilic angiocentric fibrosis case report and review of the literature

Ophthalmology. 2006 Jan;113(1):148-52. doi: 10.1016/j.ophtha.2005.09.035. Epub 2005 Dec 1.


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.

Publication types

  • Case Reports

MeSH terms

  • Eosinophilic Granuloma / diagnostic imaging
  • Eosinophilic Granuloma / drug therapy
  • Eosinophilic Granuloma / pathology*
  • Eosinophils / pathology*
  • Fibrosis
  • Glucocorticoids / therapeutic use
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Orbital Diseases / diagnostic imaging
  • Orbital Diseases / drug therapy
  • Orbital Diseases / pathology*
  • Prednisolone / therapeutic use
  • Tomography, X-Ray Computed


  • Glucocorticoids
  • Prednisolone