Idiopathic orbital inflammation
Citation, DOI & article data
Idiopathic orbital inflammation (IOI), also known as orbital pseudotumor and non-specific orbital inflammation (NSOI), 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 immediate retrobulbar fat
myositic pseudotumor (myositis): predominantly involves the extraocular muscles 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:
Imaging demonstrates enlargement of the muscle belly of one (or more) extraocular muscles typically with the involvement of tendinous insertions. Involvement of the tendinous insertion distinguishes the idiopathic orbital inflammation from thyroid-associated orbitopathy (TAO) in which the insertion point is spared. However, sparing of the anterior tendon does not exclude 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 extraocular 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:
Tolosa-Hunt syndrome: related condition with the involvement of the cranial nerves in the cavernous sinus and resulting ophthalmoplegia
- 1. Schaffler G, 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. PMC7976342 - Pubmed
- 2. Kapur R, Sepahdari A, Mafee M 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
- 3. Wolfgang Dähnert. Radiology Review Manual. (2003) ISBN: 9780781738958 - Google Books
- 4. Nugent R, Rootman J, Robertson W, Lapointe J, Harrison P. Acute Orbital Pseudotumors: Classification and CT Features. AJR Am J Roentgenol. 1981;137(5):957-62. doi:10.2214/ajr.137.5.957 - Pubmed
- 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 S, Rothfus W, Slamovits T, Kennerdell J, Curtin H. Computed Tomography of Orbital Myositis. AJR Am J Roentgenol. 1984;143(3):671-4. doi:10.2214/ajr.143.3.671 - Pubmed