Central Macular Splaying and Outer Retinal Thinning in Asymptomatic Sickle Cell Patients by Spectral-Domain Optical Coherence Tomography

doi:10.1016/j.ajo.2010.12.010

American Journal of Ophthalmology

Volume 151, Issue 6, June 2011, Pages 990-994.e1

https://doi.org/10.1016/j.ajo.2010.12.010 Get rights and content

Purpose

To investigate the prevalence and degree of macular thinning on optical coherence tomography (SDOCT) in African-American female patients with asymptomatic sickle cell disease.

Design

Prospective comparative case series.

Methods

Twenty-one sickle cell patients (42 eyes) without other systemic or ocular diseases and 18 healthy control patients (33 eyes) underwent SD-OCT. Images were manually segmented to measure inner retinal thickness (IRT) and outer retinal thickness (ORT). Central macular (central 1 mm), parafoveal (0.5–1.5 mm eccentricity), and perifoveal (1.5–3 mm eccentricity) thickness measurements were obtained in sickle cell patients and age/gender/race-matched healthy control subjects.

Results

Central macular total thickness (CMT) in sickle cell patients was 220 ± 3 μm (mean ± SEM), which was significantly lower (P < .05) than controls (228 ± 3 μm). Parafoveal regions had thickness measurements of 314 ± 5 μm (nasal) and 304 ± 2 μm (temporal), which were significantly lower than controls (327 ± 2 μm and 311 ± 2 μm nasally and temporally, respectively) (P < .03, P < .043). There was also no significant difference in IRT in central macula, parafoveal, and perifoveal regions. Central macular ORT was 175 ± 2 μm vs 185 ± 1 μm in controls (P < .0002). ORT in temporal parafoveal and perifoveal regions was 142 ± 2 μm and 120 ± 1 μm, respectively, vs 150 ± 1 μm and 122 ± 1 μm in controls (P < .001 and P = .16, respectively).

Conclusions

Manual segmentation of SD-OCT images revealed significant total retinal thinning in the central macula and splaying in asymptomatic sickle cell patients. Retinal thinning was predominately in outer retinal layers in central macula and parafoveal regions.

Section snippets

Patient Selection

Twenty-one African-American female sickle cell patients (42 eyes) without history of ocular disease, aside from refractive error or sickle cell retinopathy, and 18 African-American female healthy control subjects (33 eyes) without clinical evidence of maculopathy by ophthalmic examination participated in the study. The ages and diagnoses of patients are listed in the Table. The average age of sickle cell patients (30 ± 2 years) was comparable to the control subjects (34 ± 2 years) (P = .20).

Results

A comparison of mean central total macular thickness (CMT, central 1 mm) in sickle cell patients and control subjects is shown in Figure 3. CMT was 220 ± 3 μm (mean ± standard error of the mean [SEM]), significantly lower than controls (228 ± 3 μm) (P < .05). Parafoveal regions (0.5 to 1.5 mm eccentricity) had thickness measurements of 314 ± 5 μm and 304 ± 2 μm at nasal and temporal regions, respectively, which were significantly lower than controls (327 ± 2 μm [nasal] and 311 ± 2 μm

Discussion

Patients with SCD suffer from manifestations of vascular occlusions in retinal tissue. Sickle cell retinopathy is known to cause retinal pathologies predominantly in the retinal periphery, but can also cause macular infarctions.¹¹ These changes may cause changes in the thickness of retinal layers in the macula and reduce vision. To our knowledge, segmentation analysis of the alteration in the macular thickness in SCD retinopathy has not been reported. In the current study, thickness of the

Quan (Donny) Hoang, is a graduate of Northwestern University with majors in chemistry, biology and integrated science. He received his PhD and MD in 2006 from the University of Illinois at Chicago. His graduate research focused on the ionic and signal transduction mechanisms of sleep and arousal. He completed ophthalmology residency at the Illinois Eye and Ear Infirmary and is currently a Vitreo-Retinal Surgery fellow at Columbia University/Vitreous-Retina-Macula Consultants of NY in New York,

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Citation Excerpt :
Other findings suggest the outer retina and choroid may not be entirely spared from ischemic insult, but any quantitative changes may not be clinically significant. This was demonstrated in a review of OCT findings in 21 asymptomatic patients with SCD who were noted to have central macular outer retinal thickness 10 μm thinner than controls [27]. In addition, choroidal thickness has been noted to be significantly thinner in patients with SCD than age-matched healthy controls suggesting outer retinal damage that remains subclinical [28].
Sickle Cell Maculopathy: Microstructural Analysis Using OCTA and Identification of Genetic, Systemic, and Biological Risk Factors
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To identify genetic, systemic, and biological factors associated with the occurrence of sickle cell maculopathy (SCM). To evaluate microvascular macular alterations using optical coherence tomography angiography (OCTA) in sickle cell disease (SCD).
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One hundred fifty-one eyes of 78 adult SCD patients (43 HbSS, 30 HbSC, 4 S/β⁺, and 1 HbS Lepore) and 40 eyes of 20 healthy controls underwent spectral-domain optical coherence tomography (SDOCT) and OCTA using Spectralis HRA+OCT (Heidelberg Engineering, Heidelberg, Germany). We analyzed the occurrence of SCM, the foveal avascular zone (FAZ) area, and the severity of macular ischemia and studied their relationships with genetic, systemic, and biological parameters using multivariate logistic regression analysis.
Maculopathy occurred in 66 eyes (44%), and more frequently in HbSS patients (71%, P = .004). Multivariate analysis identified HbSS genotype and lower prothrombin ratio (PR) as independently associated with SCM (P = .01). Proliferative sickle cell retinopathy was also associated with SCM (P = .02). FAZ enlargement was associated with higher lactate dehydrogenase level (P = .02). Macular ischemia was more severe in patients with lower hemoglobin level (P = .004) and lower PR (P = .01). No flow areas were identified with OCTA even in eyes with no macular thinning (36 eyes, 42%) and appeared more frequently in the temporal superior subfield (36%).
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Original articleCentral Macular Splaying and Outer Retinal Thinning in Asymptomatic Sickle Cell Patients by Spectral-Domain Optical Coherence Tomography

Purpose

Design

Methods

Results

Conclusions

Section snippets

Patient Selection

Results

Discussion

Surv Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Am J Ophthalmol

Original article
Central Macular Splaying and Outer Retinal Thinning in Asymptomatic Sickle Cell Patients by Spectral-Domain Optical Coherence Tomography