A patient with sickle cell disease may infarct orbital and periorbital tissues.


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


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