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Published on: July 24, 2020
Early diagnosis of sickle cell retinopathy by using ocular coherence tomography in pediatric population (7-18 years)
Pooja Soni1, Bhavna Dhingra1, Samendra Karkhur2
1Department of Pediatrics, All India Institute of Medical Sciences (AIIMS), Bhopal, Madhya Pradesh, India.
Insights
Sickle cell retinopathy is common in Indian children but often undetected. Ocular coherence tomography (OCT) revealed retinal thinning in 25.4% of children with sickle cell disease (SCD), highlighting the need for early screening.
Area of Science:
- Ophthalmology
- Hematology
- Medical Imaging
Background:
- Sickle cell disease (SCD) is a common inherited blood disorder.
- Vaso-occlusion in SCD can lead to sight-threatening retinopathy.
- Early diagnosis and management of retinopathy are crucial for preventing vision loss.
Purpose of the Study:
- To diagnose retinopathy in children with SCD using optical coherence tomography (OCT).
- To assess subclinical stages of retinopathy in Indian children with SCD.
- To investigate the prevalence of retinopathy in asymptomatic SCD patients.
Main Methods:
- A cross-sectional, single-center study involving children aged 7-18 with SCD.
- Comprehensive ophthalmological examinations, including macula and optic disc thickness measurements using Cirrus HD-OCT.
- Analysis of OCT results to detect subclinical retinopathy.
Main Results:
- None of the 55 participants had visual impairment.
- Fundoscopy revealed nonproliferative sickle cell retinopathy (NPSR) in 5.4% of patients.
- OCT detected retinal layer thinning in 25.4% of patients, including macula and retinal nerve fiber layer thinning.
Conclusions:
- Sickle cell retinopathy (SCR) is underdiagnosed in SCD patients.
- Retinal thinning occurs even in early, subclinical stages of SCR.
- Regular screening with advanced diagnostic methods like OCT is essential for early detection and prevention of vision-threatening complications.
Background:
Sickle cell disease (SCD) is the commonest inherited blood disorder leading to complications occurring due to vaso-occlusion including sight-threatening retinopathy. Retinopathy can be managed if diagnosed early and vision loss can be prevented. Since, very less data are available from India, hence, this study was conducted in children (7-18 years) with SCD to diagnose retinopathy by using ocular coherence tomography (OCT) in subclinical stages.
Methods:
This cross sectional single-center study was performed in 7-18 years age group children with SCD without any visual symptoms. Enrolled participants underwent complete ophthalmological examination including macula and optic disc thickness measurements using Cirrus HD-OCT and results were analyzed.
Results:
Among 55 participants, none had visual impairment. Significant fundoscopy finding (nonproliferative sickle cell retinopathy/NPSR) was found in three patients (5.4%), thinning of central macula in four patients (7.27%), inner macula thinning in eight patients (14.5%), outer macula thinning in one patient (1.81%), retinal nerve fiber layer thinning in five patients (9%), ganglion cell layer to inner plexiform layer thinning in eight patients (14.54%). Overall NPSR was found in 5.4% patients detected with fundoscopy, whereas retinal layer thinning was found in 14 patients (25.4%) using OCT.
Conclusion:
Despite of the significant prevalence of SCR, it is still underdiagnosed complication, leading to thinning of the retina from early ages; thus, its early diagnosis by regular screening using newer diagnostic methods can prevent progression to sight-threatening complications and provide better quality of life for these patients.
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