Ocular findings in children with severe clinical symptoms of homozygous sickle cell anaemia in Kaduna, Nigeria

Sickle retinopathy reflects disease-related vascular injury of the eye, which can potentially result in visual loss from vitreous hemorrhage or retinal detachment. Here we review sickle retinopathy among children with sickle cell disease, describe the epidemiology, pediatric risk factors, pathophysiology, ocular findings and treatment. Newer, more sensitive ophthalmological imaging modalities are available for retinal imaging, including ultra-widefield fluorescein angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography. Optical coherence tomography angiography provides a non-invasive view of retinal vascular layers that could previously not be imaged and can be quantified for comparative or prospective analyses. Ultra-widefield fluorescein angiography provides a more comprehensive view of the peripheral retina than traditional imaging techniques. Screening for retinopathy by standard fundoscopic imaging modalities detects a prevalence of approximately 10%. In contrast, these more sensitive methods allow for more sensitive examination that includes the retina perimeter where sickle retinopathy is often first detectable. Use of these new imaging modalities may detect a higher prevalence of early sickle pathology among children than has previously been reported. Earlier detection may help in better understanding the pathogenesis of sickle retinopathy and guide future screening and treatment paradigms.

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“…[75] Other recommendations include biannual or annual examinations as early as age 9. [11, 16, 76, 77]…”

Section: Resultsmentioning

confidence: 99%

New Ways to Detect Pediatric Sickle Cell Retinopathy: A Comprehensive Review

Pahl

Green²,

Bhatia³

et al. 2017

Journal of Pediatric Hematology/Oncology

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“…18 The youngest patient in the literature documented to have HgbSC and PSR was 7 years old. 6 In our cohort, the youngest patient who had NPSR was 6 years old, which confirmed that children younger than 10 years of age can develop SR. 6,8,19,20 Our youngest patient with HgbSC and PSR was 11 years old; the youngest with HgbSS who had PSR was 13 years of age.…”

Section: Discussionmentioning

confidence: 98%

Pediatric sickle cell retinopathy: Correlation with clinical factors

Rosenberg

Hutcheson

2011

Journal of American Association for Pediatric Ophthalmology and

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“…Most studies assessing sickle retinopathy in pediatric populations utilize FA only when indicated on clinical exam skewing results towards a lower prevalence of retinopathy than in fact exists. 10–12, 23, 24 One retrospective analysis of 258 adolescents with SCD aged 10–18 conducted over a 10 year period found a 20.9% prevalence of SR, 11 while another analysis in children with SCD aged 1 to 18 found an 18.7% prevalence of any retinopathy. 12 Our findings of Goldberg Stage I or II retinopathy in the eyes of all adolescents with SCD aged 10–19 are more consistent with Talbot et al which utilized FA studies in all patients and demonstrated peripheral arteriolar closure (Goldberg Stage I) in 90% of HbSS and HbSC adolescents by age 12.…”

Section: Discussionmentioning

confidence: 99%

Optical Coherence Tomography Angiography and Ultra-widefield Fluorescein Angiography for Early Detection of Adolescent Sickle Retinopathy

Pahl

Green

Bhatia

et al. 2017

American Journal of Ophthalmology

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Purpose Using standard screening techniques, sickle retinopathy reportedly occurs in 10% of adolescents with sickle cell disease (SCD). We performed a prospective, observational clinical study to determine if ultra-widefield fluorescein angiography (UWFA), spectral-domain optical coherence tomography (SD-OCT) and optical coherence tomography angiography (OCT-A) detect more frequent retinopathy in adolescents with SCD. Design Cross-sectional study. Methods Setting Institutional. Subjects Sixteen adolescents with SCD, ages 10–19 years, (mean age 14.9 years) and 5 age-equivalent controls (mean age 17.4 years). Observation Procedures Examinations including acuity, standard slit-lamp biomicroscopy, UWFA, SD-OCT and OCT-A were performed. Main Outcome Measures Sickle retinopathy defined by biomicroscopic changes, Goldberg stages I–V, Penman scale, flow void on OCT-A, or macular thinning on SD-OCT. Results While 22/32 SCD eyes (68.8%) had retinopathy on biomicroscopy, by UWFA 4/24 (16.7%) SCD eyes had peripheral arterial occlusion (Goldberg I), and 20/24 eyes (83.3%) had peripheral arteriovenous anastomoses (Goldberg II) in addition. No patients had Goldberg stages III–V. By SD-OCT and OCT-A, thinning of the macula and flow voids in both the superficial and deep retinal capillary plexus were found in 6/30 (20%) eyes. Conclusions All 24 eyes with adequate UWFA studies demonstrated sickle retinopathy. SD-OCT and OCT-A, which have not been previously reported in the adolescent population, detected abnormal macular thinning and flow abnormalities undetected by biomicroscopy. These findings suggest that pediatric sickle retinopathy may be more prevalent than previously suspected. If these findings are confirmed with larger cross-sectional and prospective analyses, these approaches may enhance early screening for sickle retinopathy.

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Ocular findings in children with severe clinical symptoms of homozygous sickle cell anaemia in Kaduna, Nigeria

Cited by 16 publications

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New Ways to Detect Pediatric Sickle Cell Retinopathy: A Comprehensive Review

New Ways to Detect Pediatric Sickle Cell Retinopathy: A Comprehensive Review

Pediatric sickle cell retinopathy: Correlation with clinical factors

Optical Coherence Tomography Angiography and Ultra-widefield Fluorescein Angiography for Early Detection of Adolescent Sickle Retinopathy

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