Primary orbital eosinophilic angiocentric fibrosis with intranasal extension

Head Neck. 2014 Jan;36(1):E8-E11. doi: 10.1002/hed.23396. Epub 2013 Sep 11.


Background: Eosinophilic angiocentric fibrosis is a chronic, idiopathic disorder that usually involves the upper respiratory tract and features progressive submucosal perivascular fibrosis of unknown etiology. To our knowledge, only 5 cases of eosinophilic angiocentric fibrosis with primary orbital involvement have been reported.

Methods and results: We report the case of a 46-year-old man with right proptosis and lateral globe displacement caused by a primary eosinophilic angiocentric fibrosis extending from the orbit into the anterior ethmoid. The nasal extension of the lesion helped in establishing the correct diagnosis.

Conclusion: Physicians involved in the treatment of orbital pathologies should be familiar with this entity, because it may manifest as an intraorbital mass growing primarily or secondly into the orbit. The clinical manifestations of eosinophilic angiocentric fibrosis with orbital involvement often mimic other more common ophthalmological diseases. Biopsies are necessary for diagnosis and treatment planning, although cures are usually of palliative effect.

Keywords: endoscopic sinus surgery; eosinophilic angiocentric fibrosis; onion-skin pattern; orbit; sinonasal tract.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Biopsy, Needle
  • Eosinophilic Granuloma / pathology*
  • Eosinophilic Granuloma / surgery
  • Ethmoid Sinus / pathology
  • Fibrosis / pathology*
  • Fibrosis / surgery
  • Follow-Up Studies
  • Humans
  • Immunohistochemistry
  • Magnetic Resonance Imaging / methods
  • Male
  • Middle Aged
  • Nasal Obstruction / diagnosis
  • Nasal Obstruction / etiology
  • Nose Diseases / pathology*
  • Nose Diseases / surgery
  • Orbital Diseases / pathology*
  • Orbital Diseases / therapy
  • Rare Diseases
  • Tomography, X-Ray Computed / methods
  • Treatment Outcome