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V.C. Fan, T.G. L. Hunter, D. Darbari, K. Williams, E. Kidwell, L.S. Jones; Orbital Complications of Sickle Cell Disease: Case Series and Literature Review . Invest. Ophthalmol. Vis. Sci. 2005;46(13):5712.
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To present two cases of orbital complications associated with sickle cell anemia at Howard University Hospital and present a literature review of the orbital complications of sickle cell disease.
A retrospective review was performed of inpatients from Howard University Hospital treated for sickle cell disease and periorbital swelling or exophthalmos between 1984 and 2004. An English language Medline search was also performed for all cases in the literature of orbital complications of sickle cell anemia. A total of two cases and 20 articles were reviewed for ocular complaints, history, ophthalmic findings, imaging, hospital course, medical and surgical management, and visual outcomes. An analysis of the two cases presenting at our hospital and those in the literature was performed.
Of the two patients, one had orbital swelling with presumed orbital infarction simliar to the majority of cases in the literature. This patient's findings resolved with conservative management. The other case was diagnosed with unilateral orbital cellulitis extending from sinus disease. This patient developed orbital compression syndrome with light perception vision in his right eye and responded to medial wall orbital decompression and drainage of subperiosteal abscess with full visual recovery. There were 26 reported cases identified in the literature.
There are few reports of orbital involvement in sickle cell disease. The majority of these are associated with vaso–occlusive crisis and orbital wall infarctions, and conservative management is usually adequate to prevent sight–threatening orbital compression. Orbital abscess is a much rarer cause of orbital involvement in sickle cell disease. Prompt imaging is necessary to determine the etiology of orbital compression to insure timely surgical intervention for patients with orbital infection.
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