Hemoglobinopathies: ocular manifestations in children and adolescents

Hemoglobinopathies are genetic disorders that lead to abnormal structure of the hemoglobin molecule. Sickle cell disease, the most common inherited blood disorder, is characterized by defective oxygen transport. Almost every part of the eye can be affected by sickle cell disease; however, proliferat...

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Main Authors: Omar Moussa, Royce W.S. Chen
Format: Article
Language:English
Published: SAGE Publishing 2021-06-01
Series:Therapeutic Advances in Ophthalmology
Online Access:https://doi.org/10.1177/25158414211022882
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author Omar Moussa
Royce W.S. Chen
author_facet Omar Moussa
Royce W.S. Chen
author_sort Omar Moussa
collection DOAJ
description Hemoglobinopathies are genetic disorders that lead to abnormal structure of the hemoglobin molecule. Sickle cell disease, the most common inherited blood disorder, is characterized by defective oxygen transport. Almost every part of the eye can be affected by sickle cell disease; however, proliferative sickle cell retinopathy is the primary cause of vision loss, either from vitreous hemorrhage or retinal detachment. Here we review the various manifestations of hemoglobinopathies on the eyes of children and adolescents, with a specific focus on sickle cell disease and its different phenotypes. Newer, more sensitive ophthalmological imaging modalities, including ultra-widefield fluorescein angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography, are available. These sensitive modalities allow for a more thorough examination of the retinal periphery where sickle cell retinopathy is often present. Utilization of such modalities will help with the early detection of the disease in children, which provide a better understanding of the pathogenesis of the disease and guide future screening and treatment regimens.
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spelling doaj.art-6d739cc1028747e8b3a9c94ba91aa2512022-12-21T18:21:41ZengSAGE PublishingTherapeutic Advances in Ophthalmology2515-84142021-06-011310.1177/25158414211022882Hemoglobinopathies: ocular manifestations in children and adolescentsOmar MoussaRoyce W.S. ChenHemoglobinopathies are genetic disorders that lead to abnormal structure of the hemoglobin molecule. Sickle cell disease, the most common inherited blood disorder, is characterized by defective oxygen transport. Almost every part of the eye can be affected by sickle cell disease; however, proliferative sickle cell retinopathy is the primary cause of vision loss, either from vitreous hemorrhage or retinal detachment. Here we review the various manifestations of hemoglobinopathies on the eyes of children and adolescents, with a specific focus on sickle cell disease and its different phenotypes. Newer, more sensitive ophthalmological imaging modalities, including ultra-widefield fluorescein angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography, are available. These sensitive modalities allow for a more thorough examination of the retinal periphery where sickle cell retinopathy is often present. Utilization of such modalities will help with the early detection of the disease in children, which provide a better understanding of the pathogenesis of the disease and guide future screening and treatment regimens.https://doi.org/10.1177/25158414211022882
spellingShingle Omar Moussa
Royce W.S. Chen
Hemoglobinopathies: ocular manifestations in children and adolescents
Therapeutic Advances in Ophthalmology
title Hemoglobinopathies: ocular manifestations in children and adolescents
title_full Hemoglobinopathies: ocular manifestations in children and adolescents
title_fullStr Hemoglobinopathies: ocular manifestations in children and adolescents
title_full_unstemmed Hemoglobinopathies: ocular manifestations in children and adolescents
title_short Hemoglobinopathies: ocular manifestations in children and adolescents
title_sort hemoglobinopathies ocular manifestations in children and adolescents
url https://doi.org/10.1177/25158414211022882
work_keys_str_mv AT omarmoussa hemoglobinopathiesocularmanifestationsinchildrenandadolescents
AT roycewschen hemoglobinopathiesocularmanifestationsinchildrenandadolescents