Abstract
Purpose: :
To determine the association in sickle hemoglobinopathy patients between focal retinal thinning, as detected by spectral domain optical coherence tomography (SD-OCT), and systemic sickle cell hemoglobinopathy complications.
Methods: :
The medical records of patients with various hemoglobin sickle genotypes were reviewed for the presence of sickle hemoglobinopathy related complications. Findings were analyzed to generate odds ratios for association with focal retinal thinning as detected on SD-OCT by patient and by eye. Association for gender and age with focal retinal thinning was also evaluated.
Results: :
Focal retinal thinning in hemoglobinopathy patients was significantly associated with increased age (CI 0.08-6.85, p<0.05). Mean age of patients with focal retinal thinning was 39.1 yrs (±13.0) versus 35.7 yrs (±11.3) in patients without focal retinal thinning. All hemoglobinopathy patients in this study of SC, SS, and SThal patients were 4.4 times more likely to be associated with pulmonary hypertension than patients without (CI 1.2-16.5, p=0.02). For each individual genotype, SC or SThal patients with focal retinal thinning were not associated with any systemic complications of hemoglobinopathy whereas SS patients with focal retinal thinning were 4.7 times more likely to be associated with pulmonary hypertension than patients without retinal thinning (CI 1.2-18.7, p=0.04).
Conclusions: :
Focal retinal thinning on SD-OCT is commonly found among sickle hemoglobinopathy patients and is significantly associated with increased age. Detection of focal retinal thinning suggests presence of other systemic complications in the individual such as pulmonary hypertension in SC, SS, and SThal sickle hemoglobinopathy patients with significantly greater association within the SS genotype group.
Keywords: vascular occlusion/vascular occlusive disease • imaging/image analysis: clinical • clinical (human) or epidemiologic studies: risk factor assessment