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
- Postseptal cellulitis
- Thyroid Eye Disease
- Others:
- Systemic diseases: Granulomatosis with polyangiitis, Polyarteritis nodosa, Waldenström’s macroglobulinemia involving the orbit, Sarcoidosis
- Malignant (lymphoproliferative) orbital tumors
- Ruptured dermoid cyst
- Carotid cavernous fistula
- Trauma/foreign body
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)