Jacob Stallbaumer – Memphis VA Early Detection and Treatment of Sickle Cell with Neovascularization Abstract 65 year old African American female with sickle cell anemia presents with complaint of ocular irritation. Ocular examination reveals microvascular changes OD. Subsequent revealed neovascularization that underwent successful treatment with Pascal laser. Case History 65 year old African American female presents to clinic with complaints of ocular irritation. Systemic history includes sickle cell anemia and pulmonary sarcoidosis, as well as gastroesophageal reflux disease. Patient is currently taking Sumatriptan and Topirimate. Ocular history includes dry eye that is being treated with carboxymethylcellulose 0.5% and Celluvisc 1%; and a single occurrence of bilateral anterior in 2000 that was successfully treated. Clinical findings BCVA’s were 20/20 OD and OS, were equal, round and reactive to light. EOM’s were full and confrontational visual fields were full to finger count. SLE showed moderate lash debris, pingueculas OU, nasal corneal pannus with thinning OS as well as anterior cortical and nuclear sclerosis OD and OS. DFE revealed 0.15 DCR’s, dominant drusen outside vascular arcades OU and 1.5 DD in size area of microvascular changes in the mid-peripherial OD. IVFA revealed scattered micro aneurysms and an area of hyperflourescence consistent with neovascularization in the area of previously noted microvascular changes. Differential Diagnosis Primary: Stage 3 sickle cell retinopathy. Others: Proliferative , branch retinal vein occlusion, , Eales disease1. Diagnosis and Discussion Sickle cell disease results from a single amino acid point mutation leading to formation of hemoglobin S. This mutation causes sickling of red blood cells which leads to hypoxic events due to mechanical obstruction of blood vessels. Sickle cell disease is the most common inherited blood disorder and has an autosomal recessive inheritance pattern8. One in 400 African Americans is born with sickle cell anemia, while 1 in 1000 has sickle cell disease1. Systemic signs include cerebral vascular accidents, acute chest syndrome, pulmonary hypertension, priapism, pneumonia and leg ulcers, among others. Ocular complications of sickle cell anemia are traditionally classified using the Goldberg stages. Stage 1 involves peripheral vascular occlusions and silver wiring of the arterioles. Stage 2 is defined by arteriovenous anastomoses at the border of perfused and nonperfused retina. There is no leakage in this stage. In stage 3, neovascularization develops, usually in the shape of a "sea-fan", which is considered to be a pathognomonic sign. The development of signifies stage 4 and stage 5 is the progression to retinal detachment5. Stages 4 and 5 account for the majority of vision loss in patients with sickle cell disease6,7. Stages 1 and 2 of sickle cell retinopathy are not generally treated. Treatment for stage 3 includes intravitreal anti-VegF injection or scatter laser photocoagulation to the ischemic area. This reduces oxygen demand and prevents further neovascularization. However, 30-40% of proliferative sickle cell retinopathy lesions auto infarct and involute spontaneously. The goal of stage 3 sickle cell retinopathy treatment is to prevent stages 4 and 52,4,5,6,7. In cases of stage 4 or 5 sickle cell retinopathy, a can be performed. In this case, the patient was referred to where they successfully treated the neovascularization with a Pascal laser. Conclusion IVFA is an important tool in diagnosing proliferative retinal disease. The diagnoses of PSR with IVFA is not a definitive indication for treatment, as 30-40% of PSR lesions involute spontaneously. However, the risk of vision loss associated with PSR strongly suggests treatment. Bibliography 1. Cao, J., et al. “Angiogenic Factors in Human Proliferative Sickle Cell Retinopathy.” British Journal of Ophthalmology, vol. 83, no. 7, Jan. 1999, pp. 838–846. 2. Cohen, SB, et al. “Diagnosis and Management of Ocular Complications of Sickle Hemoglobinopathies: Part V.” Ophthalmic Surgery , vol. 17, no. 6, June 1986, pp. 369– 374. 3. Condon, P. I., and G. R. Serjeant. “Photocoagulation in Proliferative Sickle Retinopathy: Results of a 5-Year Study.” British Journal of Ophthalmology, vol. 64, no. 11, Jan. 1980, pp. 832–840. 4. Goldberg, Morton. “Retinal Neovascularization in Sickle Cell Retinopathy.” American Academy of Ophthalmology and Otolaryngology, vol. 83, no. 3, 1 May 1977, pp. 409– 431 5. Mitropoulos, Panagiotis G., et al. “Intravitreal Ranibizumab for Stage IV Proliferative Sickle Cell Retinopathy: A First Case Report.” Case Reports in Ophthalmological Medicine, vol. 2014, 2014, pp. 1–6. 6. Penman, Alan D., and Graham R. Serjeant. “Recent Advances in the Treatment of Proliferative Sickle Cell Retinopathy.” Current Opinion in Ophthalmology, vol. 3, no. 3, 1992, pp. 379–388. 7. Rednam, Krishna Rao V., et al. “Scatter Retinal Photocoagulation for Proliferative Sickle Cell Retinopathy.” American Journal of Ophthalmology, vol. 93, no. 5, 1982, pp. 594– 599. 8. Saidkasimova, Shohista, et al. “Risk Factors for in Patients with Sickle Cell Disease in London.” European Journal of Ophthalmology, vol. 26, no. 5, 2016, pp. 431–435.