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Angioid Streaks in a Patient with Sickle Cell Disease in a Patient With Valerie Korb, OD, MBA Academy Case Report Louis Stokes Cleveland VAMC Angioid Streaks in a Patient with Sickle Cell Disease In a patient with sickle cell disease without proliferative retinopathy, the presence of angioid streaks spoking from the optic disc margin is detected during a routine dilated fundus examination. I. Case History • Demographics: 60-year-old African American male • Chief Complaint: Patient lost glasses on the bus a few weeks ago and wants an updated prescription. No visual complaints. • Ocular History: Last eye examination 6 months ago, ocular hypertension OU, mild cataracts OU, refractive error c presbyopia OU, herpes zoster virus x 2011 • Medical History: Sickle cell disease (HbSS), sickle cell anemia, depression, drug and alcohol dependence, hypertension • Medications: Acetaminophen 750mg, Tramadol 50mg, Folic Acid 1mg, Thiamin 100mg, Diphenhydramine 25mg, Mirtazapine 45mg, Venlafaxine 225mg, Omeprazole 20mg, Hydroxyurea 1000mg, Lorazepam 2mg II. Pertinent Findings • Clinical: o BCVA OD: 20/20, OS: 20/20-2 o EOMs: full/no restrictions OD/OS o Pupils: 3mm OD/3 mm OS ERRL, (-) APD OD/OS o Confrontations: FTFC OD/OS o IOP: OD- 19, OS- 18 o Slit Lamp Examination: Mild EBMD OD/OS o Dilated Fundus Examination: ▪ C/D = 0.20; healthy rim tissue; (-) edema/pallor OD/OS ▪ Posterior pole OD: 1 nasal angioid streak spoking from the ONH ▪ Posterior pole OS: 1 superior nasal angioid streak spoking from the ONH ▪ Macula: flat and intact; (-) CNVM OD/OS ▪ Periphery: flat and intact; (-) holes/tears/RDs/hemes/CNVM/sea fan retinopathy OD/OS • Ancillary Testing o Spectralis ONH OCT- OD: (+) RPE disruption, thickened choroid nasal to ONH; OS: (+) RPE disruption, thickened choroid superior nasal to the ONH o Spectralis macula OCT- normal foveal contour, (-) edema/CNVM OD/OS o Spectralis autofluorescence: hypoautofluorescence of the angioid streaks OD/OS o Fundus photos: DFE serves as interpretation OD/OS o Cirrus RNFL OCT- WNL all quadrants OD/OS o Blood work: The patient has abnormally high levels of fetal hemoglobin, likely providing protecting against more severe sickle cell crises and allowing him to outlive the average life span of a sickle cell patient. III. Differential Diagnosis • (PEPSI): Pseudoxanthoma Elasticum, Ehlers-Danlos Syndrome, Paget’s Disease, Sickle Cell Disease, Idiopathic5 • If not angioid streaks, what else could they be? o Lacquer cracks, myopic degeneration, choroidal folds, choroidal rupture5 Valerie Korb, OD, MBA Academy Case Report Louis Stokes Cleveland VAMC IV. Diagnosis and Discussion • The patient, positive for sickle cell anemia and sick cell disease, presented with asymptomatic, non-proliferative angioid streaks. • The patient is asymptomatic, BCVA 20/20 OD/OS, and there is no choroidal neovascularization; therefore, this patient is monitored with DFE every 6 months. He was instructed to return to the clinic stat with any sudden visual changes. • There are 5 different types of hemoglobinopathies. It is crucial to know which one a patient has, as certain forms of the disease affect the eyes more severely. o Sickle cell disease, sickle cell trait, sickle cell C disease, sickle cell thalassemia, hemoglobin C trait2 V. Treatment and Management • While there is no cure for sickle cell disease, treatments are designed to relieve symptoms and treat complications. Goals for treating sickle cell anemia include: pain relief and prevention of infections, organ damage, and stroke.3 • Treatments include hydroxyurea, pain medications, lifestyle changes, antibiotics, blood transfusions, oxygen therapy, gene therapy, bone marrow and stem cell transplant.3 • Due to the risk of developing choroidal neovascularization, patients should be dilated every 6 months, dispensed an Amsler grid for home monitoring and prescribed polycarbonate lenses for additional protection.4 • If neovascularization is present, treat with cryotherapy, photocoagulation or anti-VEGF.1,6 • Treat any retinal detachments if they should occur. VI. Bibliography 1. Bonanomi, M., Lavezzo, M. Sickle cell retinopathy: diagnosis and treatment. Review Article. Brazilian Archives of Ophthalmology. 2013. 2. Bowling, Brad, and Jack J. Kanski. Clinical Ophthalmology: A Systematic Approach 7th ed. S.l., Elsevier, 2011. 3. Claster, S., Vichinsky, E. Managing sickle cell disease. British Medical Journal. 2003;327:1151-1155. 4. Friedbert, M., Rapuano, C. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. Lippincott Williams and Wilkins, 2012. 5. Friedman, N., Kaiser, P., Pineda, R. The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology. W.B. Saunders Company, 1998. 6. Georgalas, I., Papaconstantinou, D., Koutsandrea, C., et al. Angioid streaks, clinical course, complications, and current therapeutic management. Therapeutics and Clinical Risk Management. 2009;5:81-89. VII. Conclusion • Sickle cell disease often leads to sickle cell anemia, which predominately affects the African American population.3 • Carefully look through a patient’s systemic history to know what type of hemoglobinopathy they have, as the severity of ocular findings is dependent upon the type of hemoglobinopathy. • Perform a close examination of the retina to look for non-proliferative signs (angioid streaks, salmon patches, black sunbursts), proliferative signs (sea-fan retinopathy, fibrovascular changes, retinal detachment), and choroidal neovascularization.2 .
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