Catastrophic antiphospholipid antibody syndrome and cocaine abuse associated with bilateral retinal vascular occlusions

Purpose: To describe a case of bilateral central retinal vein occlusion and central retinal artery occlusion associated with antiphospholipid antibody syndrome and cocaine abuse. Methods: A single case report of a 44-year-old woman with a history of cocaine abuse, vasculitis, and 3 spontaneous abortions who developed painless complete loss of vision in both eyes concurrently with progressive motor and sensory polyneuropathy. The patient underwent an extensive laboratory, radiographic, ophthalmologic, and pathologic workup. Results: Diagnosis of catastrophic antiphospholipid antibody syndrome was made based on established clinical and laboratory criteria, including a positive Russell's viper venom test, history of three spontaneous abortions and evidence of microvascular and macrovascular venous occlusions, and multiorgan failure. Urine toxicology screen was positive for cocaine metabolites. Her clinical course was remarkable for progressive mononeuritis multiplex and quadriplegia. She had no light perception on presentation, and fundus examination revealed extensive preretinal, intraretinal, and subretinal hemorrhages. Fluorescein angiography revealed complete occlusion of central retinal arteriolar and venular flow and retinal hemorrhages in both eyes. Conclusion: Bilateral central retinal vein occlusion and central retinal artery occlusion have been rarely reported in the literature and are often associated with underlying thrombotic risk factors, such as antiphospholipid antibody syndrome. Workup for underlying hypercoagulability and/or cocaine use should be considered in atypical and bilateral cases of central vein or artery occlusion. In this case, bilateral central retinal artery and vein occlusions developed as manifestations of catastrophic antiphospholipid antibody syndrome, which has not been previously reported.