It is the most common painful orbital mass in the adult population, and is associated with proptosis, cranial nerve palsy (Tolosa–Hunt syndrome), uveitis, and retinal detachment.
It can range from a diffuse inflammatory process to a more localized inflammation of muscle, lacrimal gland or orbital fat.
Presentation varies slightly compared to adults with bilateral involvement, uveitis, disc edema and tissue eosinophilia being more common in this population.
[8] Another study by Wirostko et al. proposes that organisms resembling Mollicutes cause orbital inflammation by destroying the cytoplasmic organelles of parasitized cells.
However, one study by Mottow-Lippe, Jakobiec, and Smith [12] suggests that the release of circulating antigens caused by local vascular permeability triggers an inflammatory cascade in the affected tissues.
[8] It includes diverse polymorphous infiltrate, atypical granulomatous inflammation, tissue eosinophilia, and infiltrative sclerosis[7][8][13][14][15][16] Although several classification schemes have been postulated, none have been definitively accepted due to the absence of distinct differences among the histopathological types as to the signs, symptoms, clinical course, and outcome.
[8] A differential diagnosis includes lymphoproliferative lesions, thyroid ophthalmopathy, IgG4-related ophthalmic disease, sarcoidosis, granulomatosis with polyangiitis, orbital cellulitis and carotid-cavernous fistula.
[9] The best imaging modality for idiopathic orbital inflammatory disease is contrast-enhanced thin section magnetic resonance with fat suppression.
The best diagnostic clue is a poorly marginated, mass-like enhancing soft tissue involving any area of the orbit.
There is also decreased signal intensity compared to most orbital lesions due to cellular infiltrate and fibrosis.
[citation needed] Ultrasonographic findings On grayscale ultrasound there is reduced reflectivity, regular internal echoes, and weak attenuation, in a way, similar to lymphoproliferative lesions.
Among the available options there is: surgery, alternative corticosteroid delivery, radiation therapy, non-steroidal anti-inflammatory drugs, cytotoxic agents (chlorambucil, cyclophosphamide), corticosteroid sparing immunosuppressants (methotrexate, cyclosporine, azathioprine), IV immune-globin, plasmapheresis, and biologic treatments (such as TNF-α inhibitors).