Abstract
Purpose:
Sickle cell retinopathy (SCR) is a hemoglobinopathy that can present with mild to severe proliferative retinal findings. In this abstract we describe features of non-proliferative and proliferative SCR and sight threatening complications of this disorder.
Methods:
Clinical and imaging data were retrospectively collected from patients attending medical retina clinics at Moorfields Eye Hospital. Color fundus photographs, Optical Coherence Tomography (OCT), Fluorescein angiography (FA) and health records were reviewed. Clinical features noted included: abnormalities of the vitreoretinal interface at the macula [vitreomacular traction (VMT), macular hole (MH), epiretinal membrane (ERM)] and features related to the proliferative retinopathy [vitreous hemorrhage (VH), tractional retinal detachment (TRD)]. Final visual outcome was collected.
Results:
260 eyes from 130 patients with SCR were reviewed retrospectively. 24 eyes (9.23%) had no apparent ocular disease. Complications of the vitreoretinal interface were seen in 77 eyes: 29.62% (TRD 18.85%, MH 5.38%, VMT 1.54%, ERM 3.85%). A total of 66 eyes (25.4%) underwent one or more vitreoretinal procedures (12.7% for TRD, 3.5% for MH, 8.1% for VH). The final visual outcome was as follows: 80% had a visual acuity (VA) equal or better than 6/12, while 20% had a final VA equal or worse than 6/18 [No light perception in 4 eyes (1.54%), light perception in 4 (1.54%), hand motion in 5 (1.92%), count fingers in 3 (1.15%), and 36 eyes (13.85%) had VA between 6/18 to 6/60].
Conclusions:
SCR, both non-proliferative and proliferative disease, can present with sight threatening complications. A higher than expected number of patients had significant visual loss from abnormalities of the vitreoretinal interface at the macula or related to proliferative disease that ultimately required surgical intervention.
Keywords: 688 retina •
550 imaging/image analysis: clinical