Retinopathy in patients with sickle cell trait sciencedirect. Salmonpatch hemorrhages after central retinal occlusion in sickle cell disease. Presence of salmon patch hemorrhages, sunbursts or commashaped conjuctival vessels. Moreover, venous tortuosity, enlargement of the foveal avascular zone, central.
This worsening can create severe hemorrhages in the eye and retinal detachment. Proliferative sickle cell retinopathy is unique from other proliferative vitreoretinal diseases in that the neovascular seafan lesions can autoinfarct in up to 60% of cases, therefore becoming inactive and no longer pose a threat to vision. Fragile vessels result in hemorrhages in one or more layers of the retina. Posterior segment involvement retinal periphlebitis choroidal granuloma. Salmon patch hemorrhage in sickle cell retinopathy. Figure 2 salmonpatch hemorrhage in sickle cell retinopathy. Sickle retinopathy in a person with hemoglobin snew york. Common sequelae of nonproliferative sickle cell retinopathy include salmon patch retinal hemorrhages, refractile or iridescent spots, and black sunburst lesions fig.
Salmonpatch hemorrhages after central retinal artery occlusion in. Gagliano da, goldberg mf 1989 the evolution of salmonpatch hemorrhages in sickle cell retinopathy. Nia j, lam wc, kleinman dm, kirby m, liu es, et al. All 7 patients with sickle cell trait and vasoproliferative retinopathy documented in their study had evidence of associated systemic diseases such as diabetes, syphilis, tuberculosis, or sarcoidosis. Salmon patch hemorrhages intraretinal hemorrhages following peripheral retinal arteriolar occlusion. The evolution of salmonpatch hemorrhages in sickle cell. A complete blood count cbc is used to evaluate a wide range of hyperviscosityrelated disorders, including anemia, infection and leukemia. What is the pathophysiology of hemoglobinopathy retinopathy. Venous tortuosity of the peripheral vessels, intraretinal salmonpatch hemorrhages, iridescent spots, black sunburst, angioid streaks leads to choroidal neovascular membrane and discrete patches of the midperipheral darker retina associated with sickle cell disease, sickling maculopathy. Laser therapy for retinopathy in sickle cell disease. The patient, a 48yearold male, presented with a one week history of poor vision from the left eye. Sickle cell disease is the most common genetic disease worldwide. The hemorrhagic defect then appears as bright yellow dots at several levels of.
In one patient, the salmonpatch hemorrhages evolved into atrophic schisis cavities. During the subsequent days, multiple salmonpatch hemorrhages developed in the distribution of these occluded arterioles. Salmon patch hemorrhages orangepinkcolored intraretinal hemorrhage seafan retinopathy peripheral neo w subsequent vit. Iridescent spots, representing a collection of hemosiderinladen macrophages, also may be noted. Sickle cell retinopathy scr results from the microvascular occlusions induced by sickleshaped erythrocytes in the retina 4. Sickle cell disease is caused by a small or point mutation in the hemoglobin molecule that is found in red blood cells rbcs. It is considered as the major ocular manifestation of scd to. Salmonpatch hemorrhages after central retinal artery occlusion in sickle cell disease. It occurs at the junction of perfused and nonperfused retina, most commonly found in the superotemporal followed by the inferotemporal quadrants. Occlusion of retinal vasculature first appears in the peripheral retina as salmon patches, which represent retinal hemorrhage from superficial. Deeper retinal hemorrhages have a black sunburst appearance.
The hemoglobin c hb c variant is characterized by a point mutation at the sixth position of the hbb gene, wherein a. Management and therapy of eye disorders in sickle cell disease. Sickle cell proliferative retinopathy columbia ophthalmology. Sickle cell disease scd is characterized by the presence of the hemoglobin s hb s variant due to a single nucleotide change g a g g t g in the. Pain crisis, male gender, and splenic sequestration were clinical risk factors that were associated with sickle cell retinopathy in these pediatric patients. In sickle cell hemaglobinopathy, rbc sickling causes peripheral retinal arteriolar occlusion, leading to ischemic necrosis and weakening of the vessel walls. The recently blocked vessel can be occluded permanently and recanalized with or without the occurrence of salmon patch hemorrhage.
T1 the evolution of salmonpatch hemorrhages in sickle cell retinopathy. Superficial retinal hemorrhages have a pink salmon patch appearance. We defined sickle cell retinopathy as any salmon patch hemorrhages, iridescent spots, black sunbursts, retinal neovascularization, or retinal detachment. Long term followup of scleral buckling procedure with sickle cell disease and retinal detachment treated with the use of hyperbaric oxygen. They are usually seen in the mid periphery of the retina adjacent to a retinal. The evolution of salmonpatch hemorrhages in sickle cell retinopathy. Proliferative sicklecell retinopathy is the most visionthreatening complication of sicklecell disease. When sickle cell disease worsens in the eye, it becomes proliferative sickle cell retinopathy. This image was originally published in the asrs retina image bank by larry halperin, md, retina group of. A case illustrating the diagnosis of proliferative sickle cell retinopathy. An eye on sickle cell retinopathy pubmed central pmc.
Other manifestations of sickle cell retinopathy include iridescent spots, retinal neovascularization, and retinal detachment. It is possible for the disease to go undiagnosed for quite some time unless a formal eye exam is performed or until the patient presents with advanced sight threatening complications. Ophthalmologic manifestations of sickle cell disease scd. Cao j, kunz mathews m, mcleod ds, merges c, hjelmeland lm, lutty ga 1999 angiogenic factors in human proliferative sickle cell retinopathy. Because the ocular changes produced by scd can be seen in other diseases, it is important to rule out other causes of occlusion, including central retinal vein occlusion, eales disease, and reti. This image was originally published in the asrs retina image bank by larry halperin, md, retina group of florida. These unusual cases allowed us to document the origin of salmonpatch hemorrhages after peripheral retinal arteriolar occlusions. A retinal specialists goal is to prevent or eliminate retinal neovascularization before it gets to the point of destruction. Please practice handwashing and social distancing, and check out our resources for adapting to these times.
Retinal occlusion as an advanced complication of sickle. Kay thi myint, soumendra sahoo, aung win thein, soe moe, han ni. Sickle cell retinopathy, salmon patch and black sunburst. Salmonpatch hemorrhages are superficial intraretinal hemorrhages. Examination of the peripheral retina revealed the presence of intraretinal hemorrhages salmon patches, arteriovenous anastomoses, seafan. Sep 11, 20 sickle cell retinopathy, salmon patch and black sunburst stay safe and healthy. Alkhaibari ms 216 retinal occlusion as an advanced complication of sickle cell disease ne ront ophthalmol, 216 doi. Venous tortuosity salmonpatch hemorrhage schisis cavity. The ophthalmoscopic signs of nonproliferative sicklecell retinopathy include. The clinical features of sicklecell retinopathy may be either nonproliferative or proliferative. A retrospective analysis of 258 children with scd identified 54 children with sickle cell retinopathy.
The ocular manifestations of sickle cell disease scd result from vascular occlusion, which may occur in the conjunctiva, iris, retina, and choroid. The evaluation also included attempts to identify the more subtle signs of sickle cell retinopathy, such as optic nerve head vascular changes, vascular tortuosity, macular changes e. In the nonproliferative form the most common clinical findings are salmon patch hemorrhages, iridescent spots and black sunbursts, which can be observed in the peripheral retina. Because of the mutation, the hemoglobin makes polymers in the rbcs, which causes them to become rigid. A complete ophthalmologic examination was performed. The development of sickle cell retinopathy is usually insidious with few visual symptoms in the early stages. Sickle cell hemoglobin c sc and sickle cell thalassemia sthal, are milder systemic forms in the sickle cell disease spectrum, but have a 33% and 14% incidence of proliferative sickle cell retinopathy, respectively. Sickle cell retinopathy and hemoglobinopathies springerlink. Nonproliferative or background sickle retinopathy includes the following manifestations. Sickle cell retinopathy focus of expert analysis updating. Sickle cell retinopathy can be classified as nonproliferative or proliferative.
Hemoglobinopathy retinopathy clinical presentation. Sickle cell retinopathy is an ocular manifestation of the spectrum of sickle cell disease, an inherited group of hemoglobinopathies with numerous systemic and ocular presentations. Nonproliferative retinal changes include intraretinal salmonpatch hemorrhages, which may develop into a black sunburst upon resolution figure 5. Salmonpatch hemorrhages after central retinal artery. The evolution of salmon patch hemorrhages in sickle cell retinopathy. Salmon patch hemorrhage sicklecell disease nonproliferative stage 1 sea fan neovascularization sicklecell disease proliferative stage 3. Hemoglobin and the rbcs are important for carrying oxygen throughout the body in blood vessels. How are nonproliferative retinal changes characterized in.