Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome

Arch Ophthalmol. 2003 Apr;121(4):491-9. doi: 10.1001/archopht.121.4.491.


Objective: To evaluate the distribution and clinical as well as treatment outcome characteristics of idiopathic orbital inflammation with the aim of delineating a more systematic approach to diagnosis and treatment.

Methods: A 10-year retrospective review of patients with idiopathic orbital inflammation treated at one institution.

Results: Ninety eyes in 65 patients (22 men and 43 women) were studied. Diagnoses were isolated dacryoadenitis (n = 21), isolated myositis (n = 19), concurrent dacryoadenitis and myositis (n = 5), orbital apex syndrome (n = 6), and idiopathic inflammation involving the preseptal region, supraorbital region, sclera, Tenon capsule, orbital fat, or optic nerve (n = 14). The mean age at presentation was 45 years. Pain and periorbital swelling were the most common clinical features and were observed in 45 (69%) and 49 (75%) patients, respectively. Seventeen patients (26%) had bilateral involvement. Biopsy was performed in 19 patients (29%) with atypical presentations or who failed to respond to the initial therapy. Patients were treated with steroids alone (n = 45), steroids and subsequent radiation therapy (n = 8), steroids and nonsteroidal anti-inflammatory agents (n = 6), nonsteroidal anti-inflammatory agents alone in mild cases (n = 2), and, rarely, radiation therapy without steroids (n = 1) or surgical debulking alone (n = 1). Of 65 patients, 41 (63%) represented treatment successes, with complete symptom relief at the time of the last follow-up, and 24 (37%) represented treatment failures, with partial or no relief of symptoms. Treatment failures were often characterized by recurrence of inflammation after a period of quiescence (58%) and unremitting, recalcitrant inflammation (38%); 1 patient ultimately required an exenteration.

Conclusion: Systemic steroid with a slow taper has been the established first-line treatment for idiopathic orbital inflammation, but refractory cases accounted for a significant portion of treatment failures in our study, reflecting the need for a more systematic approach to the study of this multifaceted disease and for therapeutic alternatives to systemic steroids.

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Aged
  • Aged, 80 and over
  • Biopsy
  • Child
  • Child, Preschool
  • Debridement
  • Diagnosis, Differential
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Middle Aged
  • Orbital Pseudotumor* / complications
  • Orbital Pseudotumor* / diagnosis
  • Orbital Pseudotumor* / therapy
  • Radiotherapy, Adjuvant
  • Retrospective Studies
  • Sex Distribution
  • Treatment Failure
  • Treatment Outcome


  • Glucocorticoids