Sickle Cell Retinopathy

Sickle Cell Retinopathy
SYMPTOMS Non-proliferative: Typically asymptomatic but may report Central vision loss and Metamorphopsia in the presence of cystoid macular edema, Central vision loss in the presence of macular ischemia and thinning, Central vision loss and Peripheral vision loss in the presence of optic neuropathy and retinal artery occlusions
Proliferative: Central vision loss and Metamorphopsia in the presence of cystoid macular edema, Central vision loss in the presence of macular ischemia and thinning, Central vision loss and Peripheral vision loss in the presence of optic neuropathy, retinal artery occlusions, subhyaloid/preretinal/intravitreal hemorrhages, tractional retinal detachments, and neovascular glaucoma
SIGNS Typically bilateral
Non-proliferative signs: Conjunctival sickling sign, Iris atrophy, Dot/blot hemorrhages, Flame hemorrhages, Cotton wool spots, Exudates, Roth spots, Venous/venule tortuosity, Cystoid macular edema, Optic neuropathy, Retinal capillary nonperfusion, Salmon patch hemorrhages, Iridescent spots, Angioid streaks, Black sunburst spots, Macula ischemia/thinning (typically temporal to the fovea), Retinal artery occlusions
Proliferative signs: All of the signs seen with non-proliferative sickle cell retinopathy and NVI, NVA, Neovascular glaucoma, Hyphema (high risk of rebleed), Peripheral arteriolar occlusions, Arteriolar-venular anastomosis, Seafan peripheral retinal neovascularization, Subhyaloid hemorrhages, Preretinal hemorrhages, Intravitreal hemorrhages, Tractional retinal detachments
WORK-UP Pupils, EOMs, Full eye exam with dilated retinal exam (look closely at the pupillary ruff for NVI especially before dilating), Gonioscopy, OCT analysis of the macula (signs of cystoid macular edema are best seen with an OCT), OCT analysis of the optic nerve, OCT-Angiography, Fluorescein Angiography, Indocyanine Green Angiography, Fundus photos, Infrared photos, B-scan ultrasound (if unable to view the retina), Electrodiagnostic testing (multifocal ERG), Watzke-Allen test, Macular photostress test, Amsler grid
TREATMENT Give take home Amsler grid in order to monitor for change
Patient needs to follow up with their primary care provider in order to diagnose, evaluate, and treat the sickle cell anemia
Patient needs to use caution with any strenuous exercise or activities in the presence of proliferative sickle cell retinopathy
Patient should sleep with their head elevated in presence of a preretinal, subhyaloid, and/or intravitreal hemorrhage
Non-proliferative sickle cell retinopathy: Needs to be monitored closely. There is no ocular treatment
Non-proliferative sickle cell retinopathy with cystoid macular edema: Refer to retinal specialist ASAP for treatment
Proliferative sickle cell retinopathy with or without cystoid macular edema: Refer to retinal specialist ASAP for treatment
Proliferative sickle cell retinopathy with a tractional retinal detachment: Refer to retinal specialist ASAP for treatment
Sickle cell retinopathy with a choroidal neovascular membrane secondary to angioid streaks: Refer to retinal specialist ASAP for treatment
NVI and/or NVA with normal IOP and no evidence of glaucomatous optic nerve damage: Refer to retinal specialist within 48 hours for anti-VEGF treatment and/or PRP
NVI and/or NVA with elevated IOPs but no evidence of glaucomatous optic nerve damage or with associated secondary open angle glaucoma: Begin treatment with topical and oral glaucoma medications (use caution with glaucoma medications in patients with sickle cell anemia). Refer to retinal specialist within 48 hours for anti-VEGF treatment and/or PRP
NVI and/or NVA with secondary angle closure with or without glaucoma: The goal is to lower the IOP as quickly as possible in office (use caution with glaucoma medications in patients with sickle cell anemia) and then refer to a retinal specialist ASAP for anti-VEGF treatment and/or PRP. A glaucoma specialist will most likely be involved as well as patient will need a trabeculectomy and/or shunt
FOLLOW-UP If there is no evidence of retinopathy, patient should be seen back in 6-12 months
If there is evidence of non-proliferative sickle cell retinopathy, patient should be seen back in 3-6 months
If macula is stable (in a patient with non-proliferative sickle cell retinopathy) following treatment by retinal specialist, patient should be seen back in 3-4 months
Once proliferative sickle cell retinopathy becomes involutional or quiescent and the retina and macula is stable following treatment by the retinal specialist, patient should be seen back every 4-6 months
Neovascular glaucoma: Patient will most likely continue care with a retinal specialist and/or glaucoma specialist. If condition is stable and patient is no longer seeing a retinal specialist and/or glaucoma specialist, the patient should be seen every 2-4 months
ADDITIONAL LAB | TESTS The patient needs to follow-up with their PCP for additional testing if not already done which includes the following: CBC with differential, Sickledex, Hemoglobin electrophoresis
ETIOLOGY In patients with sickle cells anemia, red blood cells are rigid and irregular in shape due to abnormal hemoglobin, especially in low oxygen environments. This leads to poor transport of oxygen as well as occlusion of retinal vasculature (especially capillaries) due to these sickle-shaped red blood cells.
DIFFERENTIAL DX Diabetic retinopathy, Hypertensive retinopathy, Anemia retinopathy, HIV retinopathy, Leukemia retinopathy
NOTES The sickle cells variants most commonly associated with ocular complications are S-thal and SC
Sickle Cell Retinopathy: Fundus photo demonstrating a salmon patch hemorrhage https://eyewiki.aao.org/File:Deepak_Fig_1.jpeg
Posted in Ocular Conditions, Retina-Vascular