title: Orbital infarction in sickle cell disease.
abstract: 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.
authors.affiliation: Department of Ophthalmology, Sultan Qaboos University Hospital, Muscat, Oman. gananu@gmail.com
authors.forename: Anuradha
authors.initials: A
authors.lastname: Ganesh
authors.forename: Sana
authors.initials: S
authors.lastname: Al-Zuhaibi
authors.forename: Anil
authors.initials: A
authors.lastname: Pathare
authors.forename: Ranjan
authors.initials: R
authors.lastname: William
authors.forename: Rana
authors.initials: R
authors.lastname: Al-Senawi
authors.forename: Abdullah
authors.initials: A
authors.lastname: Al-Mujaini
authors.forename: Samir
authors.initials: S
authors.lastname: Hussain
authors.forename: Yasser
authors.initials: Y
authors.lastname: Wali
authors.forename: Salam
authors.initials: S
authors.lastname: Alkindi
authors.forename: Mathew
authors.initials: M
authors.lastname: Zachariah
authors.forename: Huxley
authors.initials: H
authors.lastname: Knox-Macaulay
mesh_terms: 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