This is uncommon and is difficult to diagnose because of the absence of signs in the anterior segment. It is most often associated with rheumatoid arthritis and should be suspected in any patient whom presents with pain, proptosis, papilloedema and exudative retinal detachment.
The condition is unilateral and is associated with severe pain, a decrease in visual acuity, diplopia and a limitation of ocular movements.
Recurrences may lead to extreme thinning of the sclera and result in posterior staphyloma or perfusion.
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