Orbital compression syndrome in sickle cell disease

E. L. Curran, James Fleming, K. Rice, W. C. Wang

Research output: Contribution to journalArticle

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Abstract

Background: Orbital complications are an uncommonly reported finding in sickle cell disease. Methods: The authors review the reported orbital manifestations of sickle cell disease and discuss a patient with hemoglobin sickle β°thalassemia in whom rapidly progressive bilateral orbital compression developed. Results: Computed tomography of the orbits in a patient with fever, headache, orbital swelling, and optic nerve dysfunction displayed bilateral superior subperiosteal cystic masses. Surgical exploration showed bilateral liquefied hematomas, which were evacuated recovery was complete 13 days after surgery. A mild recurrence 14 months later resolved with conservative treatment the literature contains 11 reports of 16 young patients with sickle cell disease (15 sickle cell disease [Hb SS] and 1 hemoglobin sickle cell disease [Hb SC]) with rapidly developing findings ranging from frontal headache, fever, and eyelid edema to bilateral complete orbital compression syndrome. Including our patient, 60% had orbital hemorrhage on computed tomography. Ten of 12 patients tested were found to have orbital bone marrow infarctions. Sixteen of 17 patients had complete recovery; 13 were treated conservatively and 4 surgically. Only 2 of 17 had recurrence. Conclusions: Orbital complications in sickle cell disease are unusual manifestations in which a vaso-occlusive process in the marrow space around the orbit results in frontal headache, fever, eyelid edema, and often orbital compression syndrome. Subperiosteal hematomas are common and appear to result from bone marrow infarctions. Appropriate management requires a thorough evaluation to exclude other hemorrhagic, infectious or neoplastic processes, as well as vigilant ophthalmic monitoring. Supportive care is effective, unless optic nerve dysfunction or large hematomas are present, which would indicate that surgical evacuation is warranted to prevent loss of vision and to speed recovery.

Original languageEnglish (US)
Pages (from-to)1610-1615
Number of pages6
JournalOphthalmology
Volume104
Issue number10
DOIs
StatePublished - Jan 1 1997

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Sickle Cell Anemia
Hematoma
Headache
Sickle Hemoglobin
Fever
Bone Marrow
Orbit
Eyelids
Optic Nerve
Infarction
Edema
Tomography
Neoplastic Processes
Recurrence
Thalassemia
Ambulatory Surgical Procedures
Hemorrhage

All Science Journal Classification (ASJC) codes

  • Ophthalmology

Cite this

Orbital compression syndrome in sickle cell disease. / Curran, E. L.; Fleming, James; Rice, K.; Wang, W. C.

In: Ophthalmology, Vol. 104, No. 10, 01.01.1997, p. 1610-1615.

Research output: Contribution to journalArticle

Curran, E. L. ; Fleming, James ; Rice, K. ; Wang, W. C. / Orbital compression syndrome in sickle cell disease. In: Ophthalmology. 1997 ; Vol. 104, No. 10. pp. 1610-1615.
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