Nonspecific Orbital Inflammation

Change Language German

Definition

  • Heterogeneous group of diseases with non-specific, non-neoplastic, non-infectious inflammation of orbital tissue
  • Histopathologically pleomorphic, cellular inflammatory infiltration followed by reactive fibrosis
  • Formerly known as orbital pseudotumor

Clinical Findings

  • Highly variable, different forms depending on location
  • Typically acute onset of periorbital redness, swelling, and pain
  • Red eye, conjunctival chemosis
  • Motility disorders, diplopia, pain with eye movement
  • Ptosis, exophthalmos
  • Reduced vision, RAPD, anisocoria
  • Retinal/choroidal changes: signs of vasculitis, exudative retinal detachment, choroidal effusion, choroidal folds , macular edema, optic disc swelling
  • Usually unilateral in adults
    • If bilateral: work-up recommended for systemic vasculitides and lymphoproliferative disorders
  • In children, bilateral in about 1/3 of cases
    • Often associated with headache, fever, vomiting, lethargy
    • Associated with papillitis or iritis
    • Work-up usually not necessary

Subtypes

  • Dacryoadenitis: painful swelling of the temporal upper eyelid
  • Myositis: motility restrictions with diplopia and pain with eye movement
    • redness in the area of affected muscles
    • Tendons may also be affected unlike in thyroid eye disease
  • Diffuse inflammation of orbital fat: exophthalmos
  • Preseptal and supraorbital region: redness and swelling similar to preseptal or postseptal/orbital cellulitis
  • Perineuritis: optic neuropathy
  • Anterior/posterior scleritis
  • Tolosa-Hunt Syndrome:
    • inflammation in the area of the superior orbital fissure and cavernous sinus
    • painful ophthalmoplegia (N.III, N. IV, N. VI), involvement of N. V / VII possible
    • pupil involvement possible
    • vision loss if the optic nerve is involved

Examination

  • Visual acuity, Ishihara, Pelli-Robson
  • Pupil examination (RAPD?)
  • Motility (pain on movement?)
  • Lid position, Hertel exophthalmometer
  • Intraocular pressure (in different gaze directions for DD thyroid eye disease)
  • Slit lamp: Red eye? Anterior chamber flare/cells?
    • Phenylephrine test/ultrasound for suspected anterior/posterior scleritis
  • Fundus examination

Work-up

  • Diagnosis of exclusion!
  • Lab if bilateral (in adults) or atypical
    • Differential blood count, ESR, ANA, ACE, c-ANCA, p-ANCA, LDH, IgG4/IgG levels, urea/creatinine, fasting blood sugar/HbA1c
    • TSH, fT3/fT4, TSI, TPO
    • Syphilis screening test, HIV
    • Serum protein electrophoresis
  • MRI of orbit with contrast incl. coronal or orbital CT (axial, coronal, and parasagittal) with contrast
    • Thickened posterior sclera? Orbital fat? Lacrimal gland, eye muscles/tendons?
  • Consider X-ray thorax (sarcoidosis? tuberculosis?) before starting systemic steroid therapy
  • Consider ultrasound: thickened muscles? Posterior scleritis (T-sign) , vascular lesions?
  • Incisional biopsy if easily accessible (e.g., lacrimal gland)

Course

  • Spontaneous remission after a few weeks without consequences
  • Intermittent flare-ups and eventual remission
  • Severe persistent inflammation -> progressive fibrosis of orbital tissue with ophthalmoplegia, possibly ptosis, and vision reduction if optic nerve is involved

Treatment

  • For mild cases: initially watchful waiting
  • In persistent cases: biopsy for diagnostic confirmation and to rule out neoplasia
    • CAUTION: under steroid therapy, lymphoma can be misdiagnosed
  • NSAIDs often respond well in mild/moderate cases (before considering systemic steroids)
  • Systemic steroids for confirmed diagnosis + lack of response to NSAIDs:
    • Oral prednisone, initially 80 – 100 mg/day (or 1 mg/kg body weight), then tapering
    • CAUTION: long-term treatment needed (3-4 months), otherwise frequent relapses!
  • +/- Low-dose radiation.
  • +/- Antimetabolites (e.g., methotrexate or mycophenolate mofetil).
  • +/- Systemic infliximab

Differential Diagnoses

Sources

  • EyeWiki Nonspecific Orbital Inflammation
  • The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
  • Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)
  • Review of Ophthalmology; Autoren/Verlag: Neil J. Friedman MD, Peter K. Kaiser MD, William B. Trattler MD; Elsevier; 3rd Edition (2017)
  • M. Sahlmüller, J. Schroeter, 2011, Idiopathische entzündliche Orbitopathie. Augenheilkunde up2date 2011; 1(1): 15-30. DOI: 10.1055/s-0031-1280193