• It is a benign, nongranulomatous orbital inflammatory process characterized by extraocular orbital and adnexal inflammation with no known local or systemic cause. (wikipedia.org)
  • Its diagnosis is of exclusion once neoplasm, primary infection and systemic disorders have been ruled out. (wikipedia.org)
  • Orbital pseudotumor has also been observed in association with Crohn's disease, systemic lupus erythematosus, rheumatoid arthritis, diabetes mellitus, myasthenia gravis, and ankylosing spondylitis all of which strengthen the basis of IOI being an immune-mediated disease. (wikipedia.org)
  • Occasionally, cavernous sinus syndrome is the first manifestation of a systemic neoplasm (leukemia, lymphoma). (medscape.com)
  • Treatment goals are aimed at controlling the systemic vascular disease (i.e., hypertension, diabetes, and atherosclerosis) or collagen vascular disease that precipitated NAION in hopes of preventing or delaying bilateral involvement. (aetna.com)
  • proposed the term "IgG4-related autoimmune disease" as a systemic disease with multiorgan involvement [ 3 ]. (hindawi.com)
  • Orbital and adnexal lymphoma is associated with systemic lymphoma in 30-35% of cases. (medscape.com)
  • It can range from a diffuse inflammatory process to a more localized inflammation of muscle, lacrimal gland or orbital fat. (wikipedia.org)
  • They include inflammation of the extraocular muscles (myositis) with tendinous involvement, orbital fat stranding, lacrimal gland inflammation and enlargement (dacryoadenitis), involvement of the optic sheath complex, uvea, and sclera, a focal intraorbital mass or even diffuse orbital involvement. (wikipedia.org)
  • Idiopathic orbital inflammatory (IOI) disease refers to a marginated mass-like enhancing soft tissue involving any area of the orbit. (wikipedia.org)
  • Idiopathic orbital inflammatory syndrome, also known as orbital pseudotumor, was first described by Gleason in 1903 and by Busse and Hochheim. (wikipedia.org)
  • Idiopathic orbital inflammation has a varied clinical presentation depending on the involved tissue. (wikipedia.org)
  • Pediatric IOI accounts for about 17% of cases idiopathic orbital inflammation. (wikipedia.org)
  • The histopathology of idiopathic orbital inflammation is described as nondiagnostic and diverse. (wikipedia.org)
  • The best imaging modality for idiopathic orbital inflammatory disease is contrast-enhanced thin section magnetic resonance with fat suppression. (wikipedia.org)
  • Overall, radiographic features for idiopathic orbital inflammatory syndrome vary widely. (wikipedia.org)
  • Pseudotumor cerebri (also known as idiopathic intracranial hypertension) is a syndrome of increased intracranial pressure without a discernable cause. (aetna.com)
  • Depending on the site of involvement, ocular lymphoma can be intraocular, and adnexal. (medscape.com)
  • The median age at presentation for orbital and adnexal lymphoma is older than 60 years. (medscape.com)
  • A differential diagnosis includes lymphoproliferative lesions, thyroid ophthalmopathy, IgG4-related ophthalmic disease, sarcoidosis, granulomatosis with polyangiitis, orbital cellulitis and carotid-cavernous fistula. (wikipedia.org)
  • We strongly recommend considering the diagnosis of GO with elevated IgG4 in patients with an established diagnosis of GD, elevated serum IgG4 levels, and clinical features of ophthalmic disease overlapping with those of IgG4-related orbital disease. (hindawi.com)
  • Several studies have described cases where onset of orbital pseudotumor was seen simultaneously or several weeks after upper respiratory infections. (wikipedia.org)
  • This results in acute onset of massive exophthalmos with orbital, ocular, and conjunctival chemosis, binocular diplopia, and visual loss. (medscape.com)
  • Presentation varies slightly compared to adults with bilateral involvement, uveitis, disc edema and tissue eosinophilia being more common in this population. (wikipedia.org)
  • A magnetic resonance imaging (MRI) scan further revealed an abnormal infiltrating lesion at the orbital apex encasing the optic nerve and involving all four rectus muscles [ Figures 1 and 2 ]. (oaepublish.com)
  • A structured evidence review (Dickersin and Manheimer, 2002) concluded that "[r]esults from the Ischemic Optic Neuropathy Decompression Trial indicate that optic nerve decompression surgery for nonarteritic ischemic optic neuropathy is not effective. (aetna.com)
  • Signs and symptoms relating to orbital metastasis are usually noted late in the disease progression, and treatment generally consists of local radiotherapy to the orbit [ 6 ] in addition to treatment of the primary cancer, which in this case of ductal adenocarcinoma of the breast required hormonal therapy only. (oaepublish.com)
  • 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. (wikipedia.org)
  • The most often reported are: intra- and extracavernous or metastatic neoplasms, intra-cavernous carotid artery aneurysms, carotid-cavernous fistulas (see image below), infections, thrombosis, and Tolosa-Hunt syndrome. (medscape.com)
  • Primary tumors are the most frequent neoplasm responsible for a cavernous sinus syndrome. (medscape.com)
  • It may occur as a complication of spreading infection from the ethmoid, sphenoid, or frontal sinuses or from midfacial, dental, or orbital infections. (medscape.com)
  • A computed tomography (CT) scan revealed increased abnormal soft tissue enhancement in the superior aspect of the orbit, with involvement of superior and medial rectus muscles. (oaepublish.com)
  • In the setting of extensive sclerosis there may be restriction, compression, and destruction of orbital tissue. (wikipedia.org)
  • The fibrotic process invades adjacent structures of the neck and extends beyond the thyroid capsule. (medscape.com)
  • A differential diagnosis includes lymphoproliferative lesions, thyroid ophthalmopathy, IgG4-related ophthalmic disease, sarcoidosis, granulomatosis with polyangiitis, orbital cellulitis and carotid-cavernous fistula. (wikipedia.org)
  • If not treated promptly and aggressively, newborn infants can experience orbital cellulitis (because the orbital septum is formed poorly in infants), brain abscess, meningitis, sepsis, and death. (medscape.com)
  • PMID- 5097501 TI - Chromosome structure and function in man. (nih.gov)