Sickle Cell Maculopathy: Microstructural Analysis Using OCTA and Identification of Genetic, Systemic, and Biological Risk Factors

• Published in: American journal of ophthalmology Vol. 224; pp. 7 - 17
• Main Authors: Fares, Selim, Hajjar, Sophie, Romana, Marc, Connes, Philippe, Acomat, Malik, Zorobabel, Coralie, Zuber, Kevin, Boulanger-Scemama, Elise, Etienne-Julan, Maryse, David, Thierry, Beral, Laurence
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.04.2021; Elsevier Limited; Elsevier Masson
• Subjects: Adolescent; Adult; Age; Anemia, Sickle Cell - diagnosis; Anemia, Sickle Cell - epidemiology; Anemia, Sickle Cell - genetics; Anticoagulants; Blood Coagulation Disorders; Cross-Sectional Studies; Female; Fluorescein Angiography; Genotype; Genotype & phenotype; Health risk assessment; Hemoglobin; Hemoglobin C - genetics; Hemoglobin, Sickle - genetics; Hemoglobins, Abnormal - genetics; Hemolysis; Human health and pathology; Humans; Ischemia; Life Sciences; Male; Middle Aged; Multimodal Imaging; Neutrophils; Retinal Diseases - diagnosis; Retinal Diseases - epidemiology; Retinal Diseases - genetics; Retinal Vessels - pathology; Risk Factors; Sensory Organs; Sickle cell disease; Software; Tomography; Tomography, Optical Coherence; Visual Acuity; Young Adult; Guadeloupe
• ISSN: 0002-9394; 1879-1891
• DOI: 10.1016/j.ajo.2020.11.019

To identify genetic, systemic, and biological factors associated with the occurrence of sickle cell maculopathy (SCM). To evaluate microvascular macular alterations using optical coherence tomography angiography (OCTA) in sickle cell disease (SCD). Cross-sectional study. One hundred fifty-one eyes of 78 adult SCD patients (43 HbSS, 30 HbSC, 4 S/β+, and 1 HbS Lepore) and 40 eyes of 20 healthy controls underwent spectral-domain optical coherence tomography (SDOCT) and OCTA using Spectralis HRA+OCT (Heidelberg Engineering, Heidelberg, Germany). We analyzed the occurrence of SCM, the foveal avascular zone (FAZ) area, and the severity of macular ischemia and studied their relationships with genetic, systemic, and biological parameters using multivariate logistic regression analysis. Maculopathy occurred in 66 eyes (44%), and more frequently in HbSS patients (71%, P = .004). Multivariate analysis identified HbSS genotype and lower prothrombin ratio (PR) as independently associated with SCM (P = .01). Proliferative sickle cell retinopathy was also associated with SCM (P = .02). FAZ enlargement was associated with higher lactate dehydrogenase level (P = .02). Macular ischemia was more severe in patients with lower hemoglobin level (P = .004) and lower PR (P = .01). No flow areas were identified with OCTA even in eyes with no macular thinning (36 eyes, 42%) and appeared more frequently in the temporal superior subfield (36%). HbSS genotype, abnormal coagulation and hemolysis increase the risk of SCM. OCTA provides valuable criteria to identify potential risk factors of SCM. OCTA also improves detection of early microvascular changes before the onset of macular thinning.