Macular neurodegenerative and vascular changes on OCTA in sickle cell disease are not related to its ocular and systemic complications.

PURPOSE: To evaluate macular abnormalities in sickle cell disease (SCD) with OCT-Angiography (OCTA) and to determine associations with sickle cell retinopathy (SCR) and clinical and laboratory characteristics. METHODS: Complete ophthalmic examination was performed in consecutive SCD patients (HbSS, HbSC, HbSß0 or HbSß+ genotype), including fundoscopy and macular SD-OCT/OCTA scans. SCR stage was based on fundoscopic examination (without fluorescein angiography) instead of the Goldberg classification, since fluorescein angiography was only used in case of tentative diagnosis. Medical/ophthalmological history and hematologic characteristics were retrieved from medical records. RESULTS: 249 eyes of 137 patients were analyzed. The mean age was 33.3 ± 12.4 years (range 15-70). Non-proliferative SCR was present in 57 eyes (22.9%) and proliferative SCR in 36 eyes (14.5%). Macular thinning was present in 100 eyes (40.2%) and was associated with lower foveal vessel density (VD) of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) and with enlargement of the foveal avascular zone (FAZ) area, perimeter and acircularity index (AI). Age and female sex were associated with lower (para)foveal VD in the SCP and DCP. No associations were found between SCR presence/severity and macular thinning or VD. CONCLUSION: Macular abnormalities were common, but did not result in visual impairment. No relation with SCR presence/severity was found. While OCTA-imaging is suitable for detecting maculopathy, it appears to have no diagnostic value in identifying patients at risk for SCR.

Natural history and rate of progression of retinopathy in adult patients with sickle cell disease: an 11-year follow-up study.

Sickle cell retinopathy (SCR) is a complication of sickle cell disease (SCD). Proliferative SCR (PSCR) can lead to severe visual impairment due to vitreous hemorrhage or retinal detachment. Knowledge of risk factors for progression and complications of SCR is limited. The aim of this study is to describe the natural history of SCR and to identify risk factors for progressive SCR and development of PSCR. We retrospectively analyzed disease progression in 129 patients with SCD with a median follow-up period of 11 years (interquartile range, 8.5-12). Patients were divided in 2 groups. The genotypes hemoglobin SS (HbSS), HbSß0-thalassemia, and HbSß+-thalassemia were grouped together (n = 83; 64.3%), whereas patients with HbSC (n = 46; 35.7%) were grouped separately. Progression of SCR was observed in 28.7% (37 of 129) of patients. Older age (adjusted odds ratio [aOR], 1.073; 95% confidence interval [CI], 1.024-1.125; P = .003), HbSC genotype (aOR, 25.472; 95% CI, 3.788-171.285; P ≤ 0.001), and lower HbF (aOR, 0.786; 95% CI, 0.623-0.993; P = .043) were associated with PSCR at end of follow-up. Lack of any SCR at end of follow-up was associated with female sex (aOR, 2.555; 95% CI, 1.101-5.931; P = .029), HbSS/HbSß0/HbSß+ genotype (aOR, 3.733; 95% CI, 1.131-12.321; P = .031), and higher HbF levels (aOR, 1.119; 95% CI, 1.007-1.243; P = .037). Differentiated strategies for screening and follow-up of SCR could be considered for patients at low or high risk.

Likelihood of spontaneous closure of cavernous sinus dural arteriovenous fistulas based on clinical and radiological findings.

BACKGROUND: Cavernous sinus dural arteriovenous fistula (CSDAVF) is a rare condition that radiologists would encounter in their careers. We aim to describe the clinical and radiological characteristics of this condition, and to provide a management workflow. METHODS: In our retrospective study, we studied 27 patients with CSDAVF from January 2007 to August 2020. Patients with direct cavernous sinus AVFs and patients with incomplete date were excluded. Clinical and radiological data were collected and analyzed. RESULTS: Fourteen patients were conservatively treated with spontaneous resolution while 13 patients had endovascular intervention performed. In the intervention group, seven patients had intra-cranial reflux seen on radiological imaging and six patients had clinical deterioration, hence requiring intervention. Clinically, among our patients, 21 had proptosis, 20 had conjunctiva hyperaemia, 18 had extraocular movement limitation, 13 had raised intraocular pressure, 11 had chemosis, ten had ocular pain, nine had ocular bruit, eight had headache and six had worsening visual acuity. Radiologically, a concurrence was seen between superior ophthalmic vein thrombosis and spontaneous resolution of the CSDAVF, as compared to those who underwent intervention. A paradoxical increase of ocular symptoms was seen despite a decrease of flow or stagnation of contrast in radiological imaging of CSDAVF. CONCLUSIONS: In our study, 52% of CSDAVF closed spontaneously. As deterioration of ocular symptoms in patients with CSDAVF might also reflect spontaneous progressive occlusion, it warrants dynamic vascular imaging to check the status of venous outflow. Patients with CSDAVF with corticovenous reflux or deterioration of visual acuity need more urgent (endovascular) treatment.