Retrobulbar Ischemic Optic Neuropathy Associated with Sickle Cell Disease

A 52-year-old black man with a history of sickle cell SS disease presented with sudden loss of vision in his right eye. Examination of the right eye revealed no light perception and an amaurotic pupil, but an otherwise normal neuro-ophthalmic examination. On initial presentation, there was no evidence of an acute vascular event on funduscopy. Computed tomography failed to demonstrate pathology of the brain or orbit. Repeat fundus examination, 2 weeks later, still failed to demonstrate retinal or optic nerve disease. However, optic nerve pallor and central cupping became evident after 2 months and continued to progress in the ensuing year. Magnetic resonance imaging performed at that time did not disclose the presence of demyelinating disease, or aneurysmal dilation of the carotid or ophthalmic artery. A diagnosis of retrobulbar ischemic optic neuropathy is considered likely in this patient. The association of retrobulbar ischemic optic neuropathy and sickle cell anemia has not been previously reported.