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Also called inflammatory orbital pseudotumor. Idiopathic inflammatory disorders of the orbit, defined as nonspecific inflammatory conditions for which no identifiable cause or systemic disease can be found. The morphological appearance of pseudotumor includes a variety of forms with related different clinical pictures. A classification based on structures involved distinguishes six different types:
- anterior, when only the anterior orbit and the globe are involved by inflammation;
- diffuse, when all the retrobulbar fat is filled;
- myositic, characterized by the uni- or bilateral enlargement of one or more of the extraocular muscles in particular mono- or bilaterally;
- apical, when infiltration involves selectively the orbital apex;
- dacryoadenitic, when the lacrimal gland is selectively involved; and
- perineuritic, when the inflammation is particularly concentrated around the optic nerve.
Classical clinical signs of pseudotumor include pain, proptosis, lid swelling, painful limitation of eye movement, decreased vision and chemosis, varying according to the area affected. Usually the symptoms and signs are responsive to steroid therapy.
Diagnosis
Diagnosis is based on clinical examination, laboratory tests, and imaging studies including CT Scan, MRI, and sonography. It might be crucial to have several imaging studied at different interval of the disease.
Treatment
Steroid therapy either orally or intravenously, is playing the main role. Biopsy of the lesion is necessary in cases without good response to the steroid and or recurrence of the disease.