Abstract
Orbital compression syndrome is caused by disorders of varying etiologies involving the orbit and presents with ocular and extraocular dysfunction. Ocular involvement of sickle cell disease is uncommon. A 17-year-old male presented with low backache and proptosis of both eyes of 5 days duration without past history of pain crisis or transfusion. Examination revealed pallor, icterus, bilateral proptosis, conjuctival chemosis, and symmetrical restriction of ocular movements with preserved visual acuity. He was drowsy with no other focal deficits. The fundus showed macular edema, venous engorgement, and no hemorrhage. His peripheral smear showed presence of sickle cells. Computed tomography (CT) scan of the orbit revealed orbital subperiosteal hematomas. CT head images showed epidural hematoma in the frontal lobe. High-performance liquid chromatography (HPLC) and mutation studies revealed sickle beta-thalassemia in the patient. He was managed with supportive care, transfusions to keep hemoglobin above 10 g/dl, and hydroxyurea. The patient recovered fully and remained well during follow-up of 12 months. Our case was unique for presenting as orbital compression syndrome without any history of vaso-occlusive crisis.
Similar content being viewed by others
References
Nagpal KC, Goldberg MF, Rabb MF (1977) Ocular manifestations of sickle hemoglobinopathies. Surv Ophthalmol 21:391–411
Curren EL, Fleming JC, Rice K, Wang WC (1997) Orbital compression syndrome in sickle cell disease. Ophthalmology 104:1610–1615
Sidman JD, Brownlee RE, Smith WC, Fry TL (1990) Orbital complications of sickle cell disease. Int J Pediatr Otorhinolaryngol 119:181–184
Ganesh A, William RR, Mitra S, Yanamadala S, Hussein SS, Al-Kindi S, Zakariah M, Al-Lamki Z, Knox-Macaulay H (2001) Orbital involvement in sickle cell disease: a report of five cases and review literature. Eye 15:774–780
Al-Rashid RA (1979) Orbital apex syndrome secondary to sickle cell anemia. J Pediatr 95:426–427
Buchanan GR, Glader BE (1978) Leucocyte counts in children with sickle cell disease. Comparative values in the steady state, vaso-occlusive crisis and bacterial infection. Am J Dis Child 132:396–398
Rebsamen SL, Bilaniuk LT, Granet D, Foster J, Low J, Heyman S, Katowitz J (1993) Orbital wall infarction in sickle cell disease: MR evaluation. AJNR Am J Neuroradiol 14:777–779
Mankad VN, Williams JP, Harpen MD (1990) Magnetic resonance imaging of bone marrow in sickle cell disease: clinical, hematologic and pathological correlations. Blood 75:274–283
Blank JP, Gill FM (1981) Orbital infarctions in sickle cell disease. Pediatrics 67:879–881
Seeler RA (1983) Exophthalmos in haemoglobin SC disease. J Pediatr 102:90–91
Garty I, Koren A, Garzozi H (1984) Frontal and orbital bone infarctions causing periorbital swelling in patients with sickle cell anemia. Arch Ophthalmol 102:1486–1488
Wolff MH, Sty JR (1985) Orbital infarction in sickle cell disease. Pediatr Radiol 15:50–52
Ozsoylu S, Jama H, Erturk G, Tokatli A (1986) Orbital apex syndrome due to sickle cell anaemia. Pediatr Hematol Oncol 3:183–185
Seibert RW, Seibert JJ, Frazier EA (1987) Infarction of orbit and paranasal sinuses in sickle cell disease. South Med J 80:1569–1571
Mallouh AA, Young M, Hamdan J, Salamah MM (1987) Proptosis, skull infarction and retroorbital and epidural haematomas in a child with sickle cell disease. Clin Pediatr 26:536–538
Karacostas D, Artemis N, Papadopoulu M Christakis J (1991) Case report: epidural and bilateral retroorbital hematomas complicating sickle cell anemia. Am J Med Sci 302:107–109
Nath J Jin DK, Rahman R, Ballas SK, Batuman OA (1998) Successful medical management of orbital abscess in a patient with sickle cell anaemia. Ophthalmic Surg Lasers 29:860–863
Tony J, Subramanya G, Kallur KG, Chalapathy AV, Sheshadri S, Lakhkar B (1995) Proptosis, skull infarction and epidural haematomas in sickle thalassemia. Postgrad Med J 71:445
Naran A, Fontana L (2001) Sickle cell disease with orbital infarction and epidural haematoma. Pediatr Radiol 31:257–259
Royal JE Harris VJSansi PK (1988) Facial bone infarcts in sickle cell syndromes. Radiology 169:529–531
Mallouh A, Hamdan J (1986) Intracranial hemorrhage in patients with sickle cell disease (letter). Am J Dis Child 6:505–506
Resar LM, Oliva MM, Casella JF (1996) Skull infarction and epidural hematomas in a patient with sickle cell anemia. J Pediatr Hematol Oncol 18:413–415
Davies SC (1991) The vaso-occlusive crisis of sickle cell disease. BMJ 302:1551–1552
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Dixit, A., Chatterjee, T.C., Papneja, M. et al. Sickle beta-thalassemia presenting as orbital compression syndrome. Ann Hematol 83, 536–540 (2004). https://doi.org/10.1007/s00277-004-0851-1
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00277-004-0851-1