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Ann Saudi MedAnn Saudi MedAnnals of Saudi Medicine0256-49470975-4466King Faisal Specialist Hospital and Research Centre 10.5144/0256-4947.2006.76asm-1-76What”s Your Diagnosis?Diagnosis: Orbital Compression Syndrome in Sickle Cell Disease Al-Suleiman Ahmad M. Frayha Husn Jan-Feb 2006 26 1 76 76 Copyright © 2006, Annals of Saudi Medicine2006This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
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Figure 1 shows the left eye proptosis. Figure 2 is a CT scan showing soft tissue opacity in the left orbit, with a density of 86 Hounsfield units, which is close to fresh blood density, and displacement of the left globe downward. Figure 3 is a peripheral blood film showing sickle cells, anisocytosis, and evidence of hyposplenism (target cells, Howell-Jolly bodies).

Discussion
Sickle cell disease is a genetic disorder of hemoglobin production characterized clinically by anemia, recurrent painful crisis, splenic and hepatic sequestration, acute chest syndrome, stroke and bacterial sepsis. Orbital involvement in sickle cell disease is rare.1,2,3 Only a few cases are reported from Saudi Arabia.4

The orbit is a concave-shaped space comprised of seven bones (frontal, greater and lesser wings of the sphenoid, zygoma, maxilla, lacrimal, palatine and ethmoid).5 Orbital swelling in sickle cell disease occurs with orbital bone infarction, cellulitis, orbital abscesses, and orbital compression syndrome. Orbital compression syndrome is characterized by frontal headache, eyelid edema, proptosis, and fever.3,6 The clinical presentation in our patient is compatible with the diagnosis of orbital compression syndrome. This syndrome is believed to be due to hematoma adjacent to the orbital bones and appears to result from bone marrow infarction. Bilateral involvement occurs in over half of the cases.6 Orbital compression syndrome ranges from mild, which can be resolved with conservative treatment to sight-threatening, which needs surgical exploration and evacuation of the hematoma to prevent loss of vision.7

Figure 1 Photograph of the left eye showing proptosis (downward and outward).

Figure 2 CT scan of the orbits of the eye.

Figure 3 Peripheral blood smear of the patient showing sickle cells, anisocytosis and evidence of hyposplenism (target cells, Howell-Jolly bodies).
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References
1 Wolff MH  Sty JR   Orbital infarction in sickle cell disease Pediatric Radiol 1985 15 1 50 2 Abstract 
2 Naran AD  Fontana L   SCD with orbital infarction and epidural haematoma Pediatric Radiol 2001 31 4 257 9 Abstract 
3 Greer John P  Foerster John  Lukens John  Rodgers George M  Paraskevas Frixos  Glader Bertil   Wintrobe’s Clinical Hematology 11th edition Philadelphia Lippincott Williams & Wilkins 1999 
4 Mallouh AA  Young M  Hanmadan J  Salamah MM   Proptosis, skull infarction and retro-orbital and epidural haematoma in a child with SCD Clin pediatric (phila) 1987 26 10 536 8 
5 Snell RS   Second Edition clinical anatomy for Medical Students 698 699 
6 Serjeant Graham R  Serjeant Beryl E   Sickle cell disease 3rd ed New York Oxford University Press 2001 
7 Kikkawa DO  Pornpanich K  Cruz RC  Levi L  Granet DB   Graded orbital decompression based on severity of proptosis Ophthalmology 2002 109 7 1219 2 Abstract 12093642