TY - JOUR
T1 - Orbital Infarction in Sickle Cell Disease
AU - Ganesh, Anuradha
AU - Al-Zuhaibi, Sana
AU - Pathare, Anil
AU - William, Ranjan
AU - Al-Senawi, Rana
AU - Al-Mujaini, Abdullah
AU - Hussain, Samir
AU - Wali, Yasser
AU - Alkindi, Salam
AU - Zachariah, Mathew
AU - Knox-Macaulay, Huxley
N1 - Funding Information:
This study was supported by the Medical Research Ethics Committee of the Sultan Qaboos University, Muscat, Oman. The authors indicate no financial conflict of interest. Involved in design and conduct of study (A.G., S.Z., A.M., A.P., R.W., S.H., M.Z., H.K.M.); data collection (A.G., S.Z., R.S., A.M., A.P., R.W., M.Z.); management (S.A.K., Y.W., M.Z.), analysis (A.P.), and interpretation of the data (A.G., S.Z., A.P., R.W., S.H., H.K.M.); and preparation (A.G., A.P., H.K.M.) and review (S.Z., A.M., R.S., R.W., Y.W.) of the manuscript. This study was approved by the Medical Research and Ethics Committee of Sultan Qaboos University, Muscat, Oman.
PY - 2008/10
Y1 - 2008/10
N2 - Purpose: To determine the role of hematological and genetic factors in the development of orbital infarction in sickle cell disease. Design: 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.
AB - Purpose: To determine the role of hematological and genetic factors in the development of orbital infarction in sickle cell disease. Design: 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.
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U2 - 10.1016/j.ajo.2008.05.041
DO - 10.1016/j.ajo.2008.05.041
M3 - Article
C2 - 18662809
AN - SCOPUS:51649115233
SN - 0002-9394
VL - 146
SP - 595-601.e1
JO - American Journal of Ophthalmology
JF - American Journal of Ophthalmology
IS - 4
ER -