Título

ACUTE SICKLE CELL ISCHEMIC MACULOPATHY: A 3-YEAR FOLLOW-UP

Objetivo

To describe a 3-year follow up in a single patient presenting acute sickle cell ischemic maculopathy.

Métodos

Case report

Resultados

27-year-old male presenting acute painless decreased visual acuity in the left eye with medical history of sickle cell disease. Best corrected visual acuity was 20/20 in the right eye and counting fingers in the OS.
In the initial examination, retina of the both eyes presented increased vascular tortuosity and the OS also revealed white patchy areas within the horizontal raphe in the macula as well as a temporal “salmon patch" hemorrhage.
Fundus autofluorescence presented normality in the OD and a hyperautofluorescence in the macular area of the OS. Fluorescein angiography revealed irregularity of foveal avascular zone in the OD, central and peripheral ischemia in the OS (Goldberg grade 2 retinopathy).
SS-OCT presented a paracentral acute middle maculopathy (PAMM) in the OS. OCT-A of the OD showed flow reduction areas temporal and nasal to the fovea in both superficial and deep capilary plexus (DCP).
Three years later, vision was 20/20 in the right eye and 20/200 in the left. Fundoscopic evaluation of the left eye presented a regular fundus color, normal autofluorescence and SS-OCT presented macular thickness reduction due to middle layers atrophy, as a result of ischemic impairment.

Discussão e Conclusões

Microvasculature occlusion is the common mechanism underlying both peripheral proliferative sickle cell retinopathy (SCR) and thinning of the macular inner retinal layers. Maculopathy consists of atrophic ischaemic changes and has been described on OCT as macular splaying and thinning. It is usually asymptomatic until a substantial central involvement occurs, hence the importance of a multimodal retinal analysis to get through a precocious diagnostic. Macular infarctions occur especially in the temporal area, in the watershed zone, where capillaries are most narrow. PAMM develops in such location suggesting that ischaemic changes follow vascular events occurring in DCP.

Palavras Chave

SICKLE CELL, MACULOPATHY, ISCHEMIA

Área

Clínica