Sickle cell retinopathy: A literature review

Abstract

Hemoglobinopathies are a group of hereditary diseases that cause quantitative or qualitative changes in the shape, function or synthesis of hemoglobin. One of the most common is sickle cell anemia, which, due to sickling of erythrocytes, causes vaso-occlusive phenomena. Among the possible ocular manifestations, the most representative is retinopathy, which can lead to blindness if left untreated. Therefore, periodic ophthalmologic monitoring of these patients is important for early diagnosis and adequate therapeutic management, which can be done localy by treating the lesions in the eyes, or systemically.

Atypical Retro Internal Limiting Membrane Haemorrhage in Homozygous Sickle Cell Disease

“…Spontaneous haemorrhages are well described in SCR, but usually take the appearance of salmon patches. In the acute phase of bleeding, the colour of this haemorrhage is usually orange red and becomes yellow and then white in the chronic phase [3]. In our case, the retinal haemorrhage showed a yellow-white appearance at presentation.…”

“…However, acute symptomatic retinal vascular complications could occur and typically take the appearance of salmon patches, small intra-retinal haemorrhages, possibly due to ischemic vessel wall necrosis. They usually disappear without trace, but may sometimes leave a hyperpigmented lesion, called black sunburst [3]. In addition, the proangiogenic state of patients affected by SCD may also, more rarely, lead to dramatic central retinal artery occlusion or paracentral acute middle maculopathy [4].…”

Retinopatia em pacientes com doença falciforme: uma revisão de literatura

“…Dos 13 artigos analisados, 12 autores descrevem a Retinopatia Falciforme como uma doença com início silencioso e assintomático, que pode evoluir gradativamente para Research, Society and Development, v. 9, n. 10, e2839108602, 2020 (CC BY 4. 2017afirmam que a taxa de prevalência da Retinopatia em indivíduos afetados com Doença Falciforme é em torno de 10 a 20% no geral. Das pessoas acometidas, aproximadamente de 42%, podem apresentar complicações com manifestação de perda visual decorrente da retinopatia de forma branda a partir da segunda década de vida que se agrava com o aumento da idade dos pacientes (Melo, 2014;Ribeiro et al, 2017).…”

“…Ainda que a retinopatia proliferativa tenha, praticamente, a mesma origem da não proliferativa, seu percurso diferencia. Para tanto, Ribeiro et al, (2017) Assim sendo, Oliveira, Carvalho, Nascimento, Villas Bôas, Castro & Gonçalves (2014) descrevem que através de estudos realizados puderam perceber que, a partir da visão microscópica, ambos os grupos SS e SC apresentavam retinopatia não proliferativa, tais como: lesões de black sunburst e tortuosidade vascular (representados na Figura 1), sendo a primeira alteração mais comum no grupo SC e a última mais frequente no tipo SS, respectivamente. Além disso, as lesões de salmon patch puderam ser observadas em ambos subtipos da doença falciforme, sendo mais recorrente no grupo SC.…”

Awareness of Retinopathy among Sickle Cell Patients

“…Sickle cell disease (SCD) is the most prevalent type of haemoglobinopathy around the world [1]. It arises from mutations within the normal βglobin gene and is inherited in an autosomal recessive pattern [2] According to World Health Organization (WHO), hemoglobinopathies are prevalent in more than 5 percent of the world population [3] SCD is mostly prevalent in sub-Saharan Africa, India, Saudi Arabia and Mediterranean countries [4] In Saudi Arabia, the prevalence of the sickle cell gene in the adult population was measured to be 4.2% for sickle cell trait and 0.26% for SCD, with the highest incidence recorded in the Eastern Province (around 17% for sickle-cell trait and around 1% for SCD) [5]. The high frequency and prevalence of sickle cell anemia (SCA) in Saudi Arabia is linked to consanguineous marriage, which accounts for more than 50% of marriages, with 40-50% being first cousin marriages [1].…”