Adrienne W. Scott, ... Morton F. Goldberg, in Retina (Fifth Edition),2013


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.

Shibo Tang, ... Yan Luo, in Retina (Fifth Edition), 2013

Hereditary neovascular vitreoretinopathies

These conditions are characterized by hereditary peripheral retinal neovascularization with vitreoretinal traction. We discuss here hereditary conditions without primary vitreal degeneration, unaccompanied by systemic clinical manifestations, and incontinentia pigmenti, sickle-cell retinopathy, and other peripheral proliferative retinopathies that have been reviewed previously.151

Autosomal dominant neovascular inflammatory vitreoretinopathy

ADNIV is an apparently rare condition characterized by cataract, cystoid macular edema, peripheral retinal scarring and pigmentation, peripheral arteriolar closure, and neovascularization of the peripheral retina at the ora serrata.152 Young adults are asymptomatic, but have vitreous cell and selective b-wave loss on the ERG. Neovascularization may result in tractional retinal detachment. About half of patients will develop rubeosis or neovascular glaucoma by age 60 or older. The gene was localized to chromosome 11q13.153 Vitreous bands and sheets are not observed and the vitreous was not optically empty, enabling differentiation from classical vitreoretinal degenerations such as Stickler, Wagner, and SVD. The peripheral retinal vessels are initially normal in ADNIV, and dragging of the macular vessels as seen in familial exudative vitreoretinopathy is absent.

Dominantly inherited peripheral retinal neovascularization

Gitter and colleagues described a family with 7 of 15 members affected with early cataract, uveitis, prominence of the vitreous base, lattice degeneration, and severe peripheral retinal neovascularization leading to vitreous hemorrhage and retinal detachment. The syndrome appears similar to ADNIV, but after reviewing photographs of the ADNIV family, the condition was deemed to be distinct.154

Myron Yanoff MD, Joseph W. Sassani MD MHA, in Ocular Pathology (Seventh Edition), 2015

Idiopathic Polypoidal Choroidal Vasculopathy


Idiopathic polypoidal choroidal vasculopathy (IPCV), a subtype of ARMD, has a predilection for members of darkly pigmented races (rarely, it may be associated with sickle-cell retinopathy).


Clinical characteristics


Reddish-orange, spheroidal, polyp-like structures, presumably aneurysmal dilatations of the inner choroidal vascular network with distinct fundus autofluorescence findings


Multiple, recurrent, serosanguineous detachments of the RPE and neural retina, secondary to leakage and bleeding from the peculiar choroidal vascular lesions

IPCV can resemble ARMD. The former, however, is more common in the peripapillary area, usually has no associated drusen, and is most common in nonwhite patients.


Occasional vitreous hemorrhage and relatively minimal fibrous scarring




The network of peripapillary vessels seen on fluorescein angiography and indocyanine green angiography correspond to branches of the short posterior ciliary arteries.


The elevated polypoidal and tubular lesions correspond to large, thin-walled cavernous vascular channels and choroidal. Intra-Bruch's membrane neovascularization is in continuity with the vascular channels.

Neil J. Friedman MD, Peter K. Kaiser MD, in Case Reviews in Ophthalmology (Second Edition), 2018


Rubeosis (iris neovascularization).


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.


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.


Laser photocoagulation for retinal ischemia and possible peripheral cryotherapy. Treatment of increased IOP or glaucoma may be necessary.


Neovascular glaucoma (NVG) and hyphema. If the underlying cause is PDR, then vitreous hemorrhage and traction retinal detachment can occur.


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.


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.


A hyphema that has clotted and appears black or purple owing to impaired aqueous circulation and deoxygenated blood, which prevents resorption.


Anterior chamber washout is performed for corneal bloodstaining, uncontrolled elevated IOP, persistent blood clot, and rebleed.

KATHRYN E. McGOLDRICK M.D., in Decision Making in Anesthesiology (Fourth Edition), 2007

Most patients with retinal detachment (RD) are >55 years old and may have significant coexisting diseases. RDs are classified according to their type: rhegmatogenous or nonrhegmatogenous (traction or exudative). Traction RDs are frequently encountered with proliferative diabetic retinopathy or sickle cell retinopathy. Exudative RDs are seen with a variety of diseases, including tumors (metastases from breast or lung, retinoblastoma, primary melanoma), certain ocular inflammatory conditions, choroidal hemangioma, and severe pediatric renal disease. Rhegmatogenous (traction) RDs, caused by retinal tears, are common in myopic adults and may also occur in adults following cataract surgery or ocular trauma. Rhegmatogenous RDs are uncommon in children, usually secondary to trauma, myopia, aphakia, Marfan's syndrome, or retinopathy of prematurity. Rhegmatogenous RDs are preceded by a tiny hole or tear in the retina, which becomes detached if the vitreous lifts the retina off from the pigment epithelium. Symptoms associated with retinal tears include floaters and flashes. Therapy for tears includes cryotherapy or laser photocoagulation. When retinal tears progress to RD, peripheral visual field defects occur that may progress to include loss of central vision, and scleral buckling is indicated. Occasionally, complex R require vitrectomy with or without scleral buckling to treat giant retinal tears or proliferative vitreoretinopathy.

Read full chapter

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.