Idiopathic recurrent branch retinal arterial occlusion. Natural history and laboratory evaluation.

PURPOSE: To investigate the long-term visual and systemic prognosis of patients with idiopathic recurrent branch retinal artery occlusions, and to test recent hypotheses regarding possible causes of this syndrome.

METHODS: The authors retrospectively reviewed the medical and photographic records of 16 eligible patients. Each of 15 living patients was interviewed by one of the authors, then underwent follow-up ophthalmic examination, formal visual field testing, and a battery of clinical laboratory tests.

RESULTS: Over a mean follow-up of 9 years, only three eyes (9%) lost visual acuity from foveal ischemia, although nine eyes (28%) had central and/or extensive peripheral visual field loss at final examination. Ocular neovascular complications developed in eight eyes (25%). Eight patients (50%) had associated vestibuloauditory and/or transient sensorimotor symptoms, but serious permanent neurologic deficits or recurrent systemic thromboembolic events did not develop. Although most patients had one or more vaso-occlusive risk factors, extensive laboratory testing failed to define the etiology of the arterial occlusions.

CONCLUSIONS: On long-term follow-up, the visual, neurologic, and systemic prognosis for most patients with idiopathic recurrent branch retinal arterial occlusions remains favorable. Although it is probable that such patients are etiologically heterogeneous, the authors theorize that many have mild or partial manifestations of the microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions.