Most patients have evidence of a systemic vaso-occlusive crisis with:
(1) fever
(2) malaise
(3) bone pain (long bones, vertebrae, other) with overlying tenderness
Clinical findings may include:
(1) acute orbital and/or periorbital pain
(2) acute orbital and/or periorbital swelling
(3) variable visual impairment
(4) mild to moderate periorbital tenderness
(5) chemosis and conjunctival hyperemia
(6) proptosis
(7) variable exposure keratitis and/or corneal hypesthesia
(8) variable disorder of ocular motility (ophthalmoplegia)
Imaging studies of the orbit show:
(1) soft tissue swelling
(2) hematoma(s), which may be subperiosteal or epidural)
(3) signals over bone suggesting marrow infarction
Features suggesting another diagnosis (usually infection):
(1) severe periorbital tenderness
(2) systemic toxicity
(3) positive blood culture
