Orbital infarction in sickle cell disease.
OBJECTIVE
To determine the role of hematological and genetic factors in the development of orbital infarction in sickle cell disease.


METHODS
Retrospective, noncomparative case series.


METHODS
Fourteen sickle cell disease patients were diagnosed with orbital infarction during a vaso-occlusive crisis. Clinical and radiological findings were reviewed retrospectively. Sickle cell disease patients without orbital infarction were recruited as controls after matching for disease severity. Sickle haplotypes were determined for all patients. Differences between groups were evaluated statistically.


RESULTS
Patients with orbital infarction in sickle cell disease presented with acute periorbital pain and swelling with or without proptosis, ophthalmoplegia, and visual impairment during a vaso-occlusive crisis. Radiological findings included orbital soft tissue swelling (100%), hematoma (orbital, 36%; intracranial, 21%), and abnormal bone marrow intensities. Severity of orbital involvement was unrelated to that of the systemic disease (Pearson correlation coefficient, -0.1567). Affected patients predominantly had the Benin haplotype (P < .00782).


CONCLUSIONS
Orbital infarction is a potential threat to vision in sickle cell disease patients. Magnetic resonance imaging is more specific than computed tomography or nuclear scintigraphy in the evaluation of orbital changes. The degree of severity of the orbital manifestations appears unrelated to the severity of sickle cell disease. Patients with the Benin haplotype are more likely to develop orbital infarction during vaso-occlusive crises.
Ganesh
Anuradha
A
Department of Ophthalmology, Sultan Qaboos University Hospital, Muscat, Oman. gananu@gmail.com
Al-Zuhaibi
Sana
S
Pathare
Anil
A
William
Ranjan
R
Al-Senawi
Rana
R
Al-Mujaini
Abdullah
A
Hussain
Samir
S
Wali
Yasser
Y
Alkindi
Salam
S
Zachariah
Mathew
M
Knox-Macaulay
Huxley
H
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000175:diagnosis; D002648:Child; D004487:Edema / Q000209:etiology; D005094:Exophthalmos / Q000209:etiology; D005260:Female; D006239:Haplotypes; D006801:Humans; D007223:Infant; D007238:Infarction / Q000175:diagnosis / Q000209:etiology*; D008279:Magnetic Resonance Imaging; D008297:Male; D009886:Ophthalmoplegia / Q000209:etiology; D009915:Orbit / Q000098:blood supply* / Q000473:pathology; D010146:Pain / Q000209:etiology; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014786:Vision Disorders / Q000209:etiology