Diverse clinical presentations of orbital sarcoid

Am J Ophthalmol. 2007 Nov;144(5):769-775. doi: 10.1016/j.ajo.2007.07.019. Epub 2007 Sep 17.

Abstract

Purpose: To review the clinical presentation, location, systemic features, management, and natural history of orbital sarcoid.

Design: Retrospective, noncomparative, interventional case series.

Methods: Twenty patients with sarcoid and sarcoidal reactions of the orbit underwent biopsy, excision of localized mass, and systemic and local treatment at a tertiary referral center. Age, gender, onset, symptoms and signs, characterization of disease process, location, systemic disease, associated systemic features, management, and recurrence of the disease were identified.

Results: Of the 20 patients studied, five were male and 15 were female. The mean age was 50.55 +/- 16.43 years (range, 18 to 77 years). The most common symptom was the presence of a palpable mass, followed by eyelid swelling. Review of the computed tomographic scans revealed four main categories of presentation: lacrimal gland infiltration (n = 11; 55%), orbital mass (n = 4; 20%), optic nerve sheath and dural involvement (n = 4; 20%), and extraocular muscle involvement (n = 1; 5%). Concurrent systemic sarcoidosis discovered after the diagnosis of orbital sarcoid was present in 10 cases (50%). The remaining showed no evidence of systemic disease at follow-up. Angiotensin converting enzyme analysis was performed in 10 cases; in only two (20%) was elevated, and in the remaining eight, it was within normal levels.

Conclusions: Orbital sarcoid has a diverse clinical presentation varying from lacrimal gland infiltration, soft tissue orbital mass, intraorbital and extraorbital optic nerve sheath and dural involvement, to extraocular muscle involvement. The orbital site most commonly involved was the lacrimal gland.

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Age of Onset
  • Aged
  • Eyelid Diseases / diagnosis
  • Female
  • Humans
  • Lacrimal Apparatus Diseases / diagnosis
  • Male
  • Middle Aged
  • Oculomotor Nerve Diseases / diagnosis
  • Optic Nerve Diseases / diagnosis
  • Orbital Diseases / diagnosis*
  • Peptidyl-Dipeptidase A / analysis
  • Retrospective Studies
  • Sarcoidosis / diagnosis*
  • Sex Distribution
  • Tomography, X-Ray Computed

Substances

  • Peptidyl-Dipeptidase A