Hemiretinal Artery Occlusion in an 11-Year-Old Child with Dextrocardia

Purpose. To report a case of hemiretinal artery occlusion in a child with dextrocardia, visceral heterotaxia, and secondary polycythemia. Methods. Complete clinical examination, fundus photography, and retinal fluorescein angiography were performed. Laboratory testing included complete blood cell count, homocysteine, protein c, protein s, activated protein s, methyltetrahydrofolate and homocysteine activator genes, factor leiden V gene, antithrombin III, and activated protein c resistance. In addition, transthoracic and transesophageal echocardiogram and cardiac catheterism were performed. Results. We report an 11-year-old boy with a sudden, painless visual loss in his right eye. His past medical history is remarkable for a congenital cardiac disease. He presented with vision of light perception in the right eye and a relative afferent pupillary defect. Fundus findings included a macular cherry-red spot and inferior hemiretinal whitening consistent with hemiretinal artery occlusion. Laboratory testing showed increased red blood cell (RBC) count, hemoglobin, and hematocrit. The patient was treated with four phlebotomies with improvement of RBC count and after one month reperfusion of the retina and a visual acuity of 20/200 were observed. Thrombophilia and cardiac screening were negative, except for secondary polycythemia. Conclusion. Hemiretinal artery occlusion is extremely rare in children and is often associated with congenital cardiac disease and hypercoagulative states.