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Paramacular temporal atrophy in sickle cell disease occurs early in childhood

Gilles C Martin1,2, Eliane Albuisson3,4,5, Valentine Brousse6

  • 1Ophthalmology Department and Rare Ophthalmological Diseases Reference Centre, Necker-Enfants Malades University Hospital, APHP, Paris, France gilles.martin@aphp.fr.

The British Journal of Ophthalmology
|August 23, 2018
PubMed

Insights

Paramacular atrophy, a retinal condition, affects over half of children with sickle cell disease (SCD). This condition appears early and may have different causes than peripheral retinopathy in SCD patients.

Area of Science:

  • Ophthalmology
  • Hematology
  • Retinal Imaging

Background:

  • Paramacular atrophy, initially observed in a few sickle cell disease (SCD) patients, is now recognized in a significant percentage of adult eyes with SS-SCD (48%) and SC-SCD (35%).
  • This condition involves atrophic areas of the retina temporal to the macula.

Purpose of the Study:

  • To describe the characteristics of paramacular atrophy in children diagnosed with sickle cell disease (SCD).
  • To investigate the prevalence and patterns of retinal atrophy in pediatric SCD patients.

Main Methods:

  • Retrospective review of spectral-domain optical coherence tomography (SD-OCT) images from 81 children with SCD.
  • Utilized specific imaging patterns, including one targeting the retina temporal to the macula, to detect retinal atrophy.
  • Assessed fundus examination data for the presence and severity of SCD peripheral retinopathy.

Main Results:

  • Retinal atrophy was detected in 53% of eyes using a specific temporal imaging pattern, affecting 64% of the children studied.
  • Prevalence did not significantly differ between HbSS and HbSC genotypes (p=0.92) or show a correlation with age.
  • Peripheral retinopathy was identified in 11% of children, with a notable association between HbSC genotype and retinopathy severity (p=0.003).

Conclusions:

  • Paramacular temporal atrophy is an early-onset finding in sickle cell disease (SCD).
  • The early occurrence suggests distinct underlying mechanisms compared to peripheral retinopathy in SCD.
Abstract

Keywords:
child health (paediatrics)macularetina

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