Título

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN SICKLE-CELL MACULOPATHY

Objetivo

To report alterations in Optical Coherence Tomography Angiography in sickle- cell maculopathy.

Relato do Caso

44-year-old female with sickle-cell disease presented with progressive vision loss in the left eye (OS). Ophthalmological examination showed best corrected visual acuity worse than 20/200 in the OD and 20/60 in the OS. Biomicroscopy revealed mature cataract in the OD, OE was pseudophacic. Fundoscopy was not possible in the OD and revealed middle periphery temporal retinal neovascularization and black sunburst spots in the OS, macula was unremarkable. Structural Optical Coherence Tomography (OCT) showed temporal foveal thinning in the OS. OCT- Angiography (OCT-A) demonstrated areas of reduced flow within the deep and choriocapillary plexuses of temporal fovea in the OS (figure 1). Retinal panfotocoagulation was indicated to the OS and facoemulsification to the OD.

Conclusão

In the past, fluorescein angiography was the only available method to evaluate macular abnormalities in sickle-cell disease. With the advent of OCT-A, many studies have shown great correlation with conventional angiography features - with the advantage of allowing discrimination of different vascular plexuses. This recent technology may allow detection of alterations even on childhood, preceding typical findings such as salmon patch and black sunburst lesions. Studies show significantly higher prevalence of mean and temporal capillary density loss in sickle cell disease – mainly committing the deep retinal plexus. Correlation of these findings with retinal thinning is reported as well. Therefore, OCT-A comes up as an important new tool on diagnosis and follow up of sickle-cell disease– in our case clarifying the cause of the vision loss.