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SICKLE CELL RETINOPATHY
Adrienne W. Scott, ... Morton F. Goldberg, in Retina (Fifth Edition),2013
Imaging
Selected imaging modalities facilitate diagnosis, monitoring, and assessment of treatment response in sickle-cell retinopathy. As impaired choroidal circulation may be present in eyes with SCD, indocyanine green (ICG) angiography has been studied as a way to assess choroidal perfusion, but the utility of ICG in sickle retinopathy is as yet undetermined.120 The use of ultrawide-field imaging is particularly helpful to monitor peripheral lesions and to assess treatment response (Fig. 57.12). Fluorescein angiography remains the gold-standard imaging tool for assessment of retinal perfusion status. A potential limitation of conventional fluorescein angiography is the inability to image the pathology of the far peripheral retina in some eyes with sickle retinopathy.108 Accordingly, ultrawide-field fundus photography and angiography have become useful in the evaluation of the retinal periphery in eyes with sickle-cell retinopathy. As previously mentioned, OCT and especially SD-OCT, with its high resolution, may provide important details about foveal anatomy, which may aid in the diagnosis and management of sickle retinopathy,79,81–83 even in asymptomatic patients.
Neil J. Friedman MD, Peter K. Kaiser MD, in Case Reviews in Ophthalmology (Second Edition), 2018
1.
Rubeosis (iris neovascularization).
2.
Ocular ischemia, most commonly due to proliferative diabetic retinopathy, central retinal vein occlusion, and carotid occlusive disease. Rubeosis is also associated with anterior segment ischemia, chronic retinal detachment, tumors, sickle cell retinopathy, and chronic inflammation.
3.
RAPD, increased IOP, corneal edema, angle neovascularization, retinal neovascularization/hemorrhages, or optic nerve cupping. Fluorescein angiogram may demonstrate retinal nonperfusion and neovascularization. Visual field testing may show glaucomatous defects.
4.
Laser photocoagulation for retinal ischemia and possible peripheral cryotherapy. Treatment of increased IOP or glaucoma may be necessary.
5.
Neovascular glaucoma (NVG) and hyphema. If the underlying cause is PDR, then vitreous hemorrhage and traction retinal detachment can occur.
6.
NVG is a form of secondary angle-closure glaucoma. Neovascularization of the iris and angle results in occlusion of the trabecular meshwork. NVG usually requires a glaucoma drainage implant or cyclodestructive procedure to adequately control IOP.
7.
Topical steroids and cycloplegic, may require treatment of increased IOP (do not use miotic agents or prostaglandin analogues, and avoid carbonic anhydrase inhibitors in patients with sickle cell disease), consider aminocaproic acid. Daily observation for the first 5 days to monitor the IOP and check for a rebleed. The patient should avoid aspirin-containing products, remain at bedrest, sleep with the head of the bed elevated, and protect the eye with a shield. Anterior chamber washout may be required.
8.
A hyphema that has clotted and appears black or purple owing to impaired aqueous circulation and deoxygenated blood, which prevents resorption.
9.
Anterior chamber washout is performed for corneal bloodstaining, uncontrolled elevated IOP, persistent blood clot, and rebleed.
Yogen Saunthararajah, Elliott P. Vichinsky, in Hematology (Seventh Edition), 2018
Ocular Complications221
The retina is particularly vulnerable to vasoocclusion, and annual retinal examination is part of routine health care maintenance for patients with SCD. Superficial retinal hemorrhages have a pink “salmon patch” appearance. Deeper retinal hemorrhages have a “black sunburst” appearance. Other manifestations of sickle cell retinopathy include iridescent spots, retinal neovascularization, and retinal detachment. More subtle signs of sickle cell retinopathy are optic nerve head vascular changes, vascular tortuosity, macular changes (e.g., microaneurysms and vascular loops), and peripheral arteriovenous anastomoses. Other ophthalmologic complications are anterior chamber ischemia, tortuosity of conjunctival vessels, retinal artery occlusion, and angioid streaks. Sickle cell retinopathy is best seen by fluorescein angiography (Fig. 42.14). The earlier onset and greater frequency of proliferative retinopathy in Hb SC disease and sickle cell–β+-thalassemia compared with sickle cell anemia and sickle cell–β°-thalassemia suggest that retinal vessels are more susceptible to occlusion by more viscous blood than by more rigid individual cells. Peripheral sickle retinopathy may require vision-saving therapy with laser photocoagulation. Orbital compression syndrome caused by vasoocclusion of the periorbital marrow space and subperiosteal hemorrhage has been observed to result in headache, fever, and palpebral edema. In this situation, culture, CT scan, and MRI should be used to rule out infectious, neoplastic, and other hemorrhagic etiologies. Conservative therapy, including local measures, analgesia, fluids, transfusion, and careful ophthalmologic surveillance, is recommended unless compression of the optic nerve ensues, in which case surgical decompression should be considered.
Fig. 42.14. FLUORESCEIN ANGIOGRAPHY DEMONSTRATING A “SEA FAN” APPEARANCE OF SICKLE PROLIFERATIVE RETINOPATHY.(Courtesy W.C. Mentzer.)
Sickle Cell Retinopathy