Título / Title

THREE-YEARS FOLLOW UP IN A PATIENT PRESENTING ACUTE SICKLE CELL ISCHEMIC MACULOPATHY

Introdução / Purpose

To describe a three-year follow up in a patient presenting acute sickle cell ischemic maculopathy

Material e Método / Methods

Case report

Resultados / Results

Twenty-seven years old male presenting acute painless decreased visual acuity in the left eye (OS). He had medical history of sickle cell anemia and regular use of folic acid. Best corrected visual acuity (BCVA) was 20/20 in the right eye and counting fingers in the OS. In the initial examination, retina of the OD presented increased vascular tortuosity, and the OS presented increased vascular tortuosity, white patchy areas within the horizontal raphe in the macula and a temporal salmon patch hemorrhage. Fundus autofluorescence (FAF) presents normal in the OD and a hyperatuofluorescence in the macular areas of the OS. Fluorescein angiography revealed foveal avascular zone irregularity in the OD and central and peripheral ischemia in the OS (Goldberg grade 2 retinopathy in OU). Photocoagulation was performed. Swept-source optical coherence tomography (SS-OCT) was normal in the OD and presented a paracentral acute middle maculopathy (PAMM) in the OS. Optical Coherence tomography angiography (OCTA) of the OD showed flow reduction areas temporal and nasal to the fovea in both superficial capillary plexus and deep capilary plexus (DCP). Three years after, the BCVA was 20/20 in the right eye and 20/200 in the OS. Fundoscopic evaluation of the OS presented a regular fundus color, FAF was normal in OU and SD-OCT presented macular thickness reduction due to middle layers atrophy.

Discussão e Conclusões / Conclusion

In SCD patients, macular infarts especially in the temporal area are consider to be due to the watershed zone in the macular the horizontal raphe area and the occurrence of PAMM is thought to be due to the high consumption of oxygen in the photorreceptors area, leadind to oclusions in the DCP. Despite all new assembled information, there are still questions that remains in sickle cell retinopathy

Palavras Chave

Sickle Cell, retinopathy

Area

CLINICAL RETINA