Orbital
Pseudotumor
is defined as a benign, non infective, nonspecific
inflammatory conditions of the orbit without
identifiable local or systemic causes.
Idiopathic orbital inflammation has highly
variable clinical features, from a diffuse to
very focal process targeting specific orbital
tissues, such as the lacrimal gland, extraocular
muscles, optic nerve and orbital fat.
Presentations vary according to the specific
location and the degree of inflammation,
fibrosis, and mass effect. Ptosis, chemosis,
motility dysfunction, and optic neuropathy may
also be found. Unilateral presentation is
typical, but bilateral presentations are not
uncommon.
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Idiopathic
orbital inflammatory Syndrome
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Radiological imaging studies allow tissue
characterization and localization without
surgical intervention and thereby have become
invaluable diagnostic tools. Computed tomography
is the preferred mode of imaging. Idiopathic
orbital inflammation is typically seen on CT
scans as a focal or diffuse mass, usually poorly
demarcated and enhancing with contrast. Common
CT findings include enhancement with contrast
medium, infiltration of retrobulbar fat,
proptosis, extraocular muscle enlargement,
muscle tendon or sheath enlargement, apical fat
edema, optic nerve thickening, uveal-scleral
thickening, edema of the Tenon capsule, and
lacrimal gland infiltration. Tendons of the
extraocular muscles may be involved or spared.
The histopathological spectrum of idiopathic
orbital inflammation is typically non
diagnostic, wide, and diverse, ranging from the
typical diffuse polymorphous infiltrate to the
atypical granulomatous inflammation, tissue
eosinophilia, and infiltrative sclerosis. In the
absence of systemic fibro inflammatory,
granulomatous, and vasculitic disease.
Idiopathic
orbital inflammation
respond rapidly to high dose steroid
therapy in tapering doses but recurrences are
common. In such cases, chemotherapy (e.g.
methotrexate, cyclosporine) and low-dose
radiation (e.g. 1500-2500 cGy EBRT) may be
needed to control the inflammation.