Idiopathic orbital inflammation
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Idiopathic orbital inflammation (IOI), also known as orbital pseudotumor and non-specific orbital inflammation, is an idiopathic inflammatory condition that most commonly involves the extraocular muscles. Less commonly there is inflammatory change involving the uvea, sclera, lacrimal gland, and retrobulbar soft tissue.
The exact etiology is not known but an association with many inflammatory/autoimmune diseases is reported.
Many terms are used interchangeably in the literature to refer to idiopathic orbital inflammation including orbital pseudotumor, non-specific orbital inflammation and orbital inflammatory syndrome.
Patients typically present with rapid-onset, usually unilateral (~90% of cases), painful proptosis and diplopia. Idiopathic orbital inflammation is a diagnosis of exclusion; atypical presentation, poor response to treatment with corticosteroid and recurrence should prompt biopsy to exclude other diseases.
Histologically acute lesions demonstrate lymphocytes (which can be mistaken for orbital lymphoma), plasma cells, and giant cell infiltration.
Division into a number of subgroups according to location has been proposed:
- lacrimal pseudotumor (dacryoadenitis)
- anterior pseudotumor: in the fat immediately behind the globe
- posterior pseudotumor: in the fat at the orbital apex; distinguished from Tolosa-Hunt syndrome in that the cavernous sinus is spared
- myositic pseudotumor (myositis): predominantly involve the EOMs and therefore mimic thyroid-associated orbitopathy (TAO) but unlike TAO it also involves the tendons
- optic perineuritis: involvement of the optic nerve sheath
- diffuse pseudotumor: affecting multiple compartments
The condition has been associated with other inflammatory and autoimmune conditions:
- IgG4-related disease, now recognized as a separate entity: IgG4-related orbital disease
- granulomatosis with polyangiitis
- systemic lupus erythematosus (SLE)
- polyarteritis nodosa (PAN)
- rheumatoid arthritis (RA)
- sclerosing cholangitis
- Riedel thyroiditis
- mediastinal fibrosis
Imaging demonstrates enlargement of the muscle belly of one (or more) extraocular muscles typically with the involvement of their tendinous insertions. Involvement of the tendinous insertion distinguishes it from thyroid-associated orbitopathy (TAO) in which the insertion point is spared. However, sparing of the anterior tendon does not preclude the diagnosis of idiopathic orbital myositis 7.
Additional inflammation can be seen in surrounding tissues, including the orbital fat, lacrimal gland, and optic nerve sheath.
It can appear as an infiltrative mass and extends outside of the orbit via superior or inferior orbital fissures. Extension into the cavernous sinus, meninges, and dura can occur. It is most commonly unilateral but can be bilateral in 25% of cases.
Reported signal characteristics include:
- T1: affected region typically isointense (to extra-ocular muscles) 1 but can also be hypointense 1-3
- T2: affected region typically hypointense due to fibrosis and with more progression of fibrosis it becomes more hypointense, but the signal can also be iso- to hyperintense to extra-ocular muscles 2
- T1 C+ (Gd): moderate to a marked diffuse enhancement
Treatment and prognosis
Most cases resolve rapidly with treatment (usually corticosteroids suffice) although in a subset with more chronic progression chemotherapy and radiotherapy may be required. A degree of residual fibrosis can be demonstrated, especially in the more refractory cases.
History and etymology
The disease was first described by Birch-Hirschfeld et al. in 1905 6. They also introduced the term orbital pseudotumor afterward in 1930 7.
One of the main differential diagnoses of idiopathic orbital inflammation is orbital lymphoma. There is considerable overlap between these entities both clinically and radiologically. However, orbital lymphoma usually presents as a progressive orbitopathy rather than acutely, is more often bilateral, shows lower values on ADC, and does not respond to corticosteroid.
Other imaging differential considerations include:
- orbital cellulitis: usually associated with a subperiosteal abscess from adjacent sinusitis or with a previous history of trauma/dental procedure
- thyroid-associated orbitopathy (TAO): spares the tendinous insertions and not usually painful
- Tolosa-Hunt syndrome: related condition with the involvement of the cranial nerves in the cavernous sinus and resulting ophthalmoplegia
- granulomatosis with polyangiitis: bilateral involvement of the paranasal sinuses and orbits associated with osseous destruction
- orbital sarcoidosis
- orbital metastases
- orbital rhabdomyosarcoma
- 1. Schaffler GJ, Simbrunner J, Lechner H et-al. Idiopathic sclerotic inflammation of the orbit with left optic nerve compression in a patient with multifocal fibrosclerosis. AJNR Am J Neuroradiol. 2000;21 (1): 194-7. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 2. Kapur R, Sepahdari AR, Mafee MF et-al. MR imaging of orbital inflammatory syndrome, orbital cellulitis, and orbital lymphoid lesions: the role of diffusion-weighted imaging. AJNR Am J Neuroradiol. 2009;30 (1): 64-70. doi:10.3174/ajnr.A1315 - Pubmed citation
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- 4.. Nugent RA, Rootman J, Robertson WD et-al. Acute orbital pseudotumors: classification and CT features. AJR Am J Roentgenol. 1981;137 (5): 957-62. AJR Am J Roentgenol (abstract) - Pubmed citation
- 5. Birch-Hirschfeld A. Zur Diagnostic and Pathologic der Orbital Tumoren. Bericht uber die Zusammenkunft der Deutschen Ophthalmologischen Gesellschaft 1905;32:127e35.
- 6. Birch-Hirschfeld A. Handbuch der gesamten augenheilkunde, vol. 9. Berlin: Julius Springer. p. 251–253, 1930
- 7. Dresner SC, Rothfus WE, Slamovits TL, Kennerdell JS, Curtin HD. Computed tomography of orbital myositis. (1984) AJR. American journal of roentgenology. 143 (3): 671-4. doi:10.2214/ajr.143.3.671 - Pubmed