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Optical coherence tomography (OCT) and OCT angiography allow early identification of sickle cell maculopathy in children and correlate it with systemic risk factors

  • Pediatrics
  • Published:
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Abstract

Purpose

To determine the presence of sickle cell retinopathy and maculopathy and to identify associations between markers of hemolysis and systemic and ocular manifestations in children affected by sickle cell disease.

Methods

Eighteen children with sickle cell disease, aged 5–16 years, underwent complete eye examination including best-corrected visual acuity, slit-lamp biomicroscopy, ophthalmoscopy after pharmacological mydriasis, spectral-domain optical coherence tomography (SD-OCT), and optical coherence tomography angiography (OCTA). Blood test results and clinical history information were collected for each child, including fetal hemoglobin (HbF), hemoglobin (Hb), hematocrit (Htc), mean corpuscular volume (MCV), mean corpuscular hemoglobin concentration (MCHC), reticulocytes percentage (%ret), lactic dehydrogenase (LDH), total and direct bilirubin, glomerular filtration rate, number of painful crises, acute chest syndromes, and splenic sequestration. Therapeutic regimen and transfusion therapy were also evaluated.

Results

Sixteen of 36 eyes (44.4%) had non-proliferative sickle cell retinopathy on ophthalmoscopic evaluation. No patients had proliferative sickle cell retinopathy. In 13 of 36 eyes (36.1%), SD-OCT and OCTA detected signs of sickle cell maculopathy. Nine eyes (25%) presented sickle cell retinopathy and maculopathy, 7 eyes (19.4%) sickle cell retinopathy alone, and 4 eyes (11.1%) sickle cell maculopathy alone. A statistically significant association was found between sickle cell retinopathy; lower levels of HbF, Hb, and Htc; and higher MCV and percentage of reticulocytes. Sickle cell maculopathy was associated with lower values of H and Htc and higher levels of reticulocytes and total bilirubin.

Conclusions

We identified early signs of sickle cell retinopathy and maculopathy in a pediatric population with SD-OCT and OCTA. These two retinal complications were more frequent in children with higher hemolytic rates.

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Author information

Authors and Affiliations

  1. Department of Medicine- Ophthalmology, University of Udine, Udine, Italy

    L. Grego, S. Pignatto, F. Alfier, N. Rassu, F. Samassa, R. Prosperi & P. Lanzetta

  2. Department of Medicine- Paediatrics, University of Udine, Udine, Italy

    M. Arigliani & P. Cogo

  3. Department of Ophthalmology, Hospital of Pordenone, Pordenone, Italy

    F. Rizzetto

  4. Department of Paediatrics, Hospital of Pordenone, Pordenone, Italy

    F. Barbieri & R. Dall’Amico

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  1. L. Grego
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Contributions

Study conception and design were performed by S. Pignatto, M. Arigliani, and L. Grego. All authors contribute to material preparation and data collection and analysis. The first draft of the manuscript was written by L. Grego, and all authors commented and reviewed the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to P. Lanzetta.

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Conflict of interest

PL is a consultant for Bayer, CenterVue, Novartis Pharma AG, outside the submitted work. All authors, except PL, certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge, or beliefs) in the subject matter or materials discussed in this manuscript.

Ethical approval

All procedures performed in the study involving human participants were in accordance with the ethical standards of the regional ethics committee (CEUR) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from parents and tutors of all individual participants included in the study.

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Grego, L., Pignatto, S., Alfier, F. et al. Optical coherence tomography (OCT) and OCT angiography allow early identification of sickle cell maculopathy in children and correlate it with systemic risk factors. Graefes Arch Clin Exp Ophthalmol 258, 2551–2561 (2020). https://doi.org/10.1007/s00417-020-04764-y

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  • DOI: https://doi.org/10.1007/s00417-020-04764-y

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