Acute idiopathic blind spot enlargement syndrome - A review of 27 new cases

Objective: To describe the clinical findings in patients with acute idiopat hic blind spot enlargement (AIBSE). Methods? Medical record review of 27 patients with AIBSE (without sufficien t optic nerve head swelling to cause blind spot enlargement) seen in 2 acad emic neuroophthalmology units. Results: All patients were women aged between 19 and 53 years. Twenty-three patients reported positive visual phenomena. Visual acuity was normal in 1 6 patients. All patients had enlarged blind spots of variable size and dens ity Dyschromatopsia and afferent pupil defects were prevalent. Ophthalmosco pic features included uveitis, mild optic nerve swelling, granularity of ma cular pigment, subretinal white dots, and peripapillary pigment disturbance s. Twelve of the 13 patients who underwent fluorescein angiography had opti c disc staining and 5 had retinal pigment epithelial lesions with late stai ning. Fullfield electroretinogram results were normal in 8 of 9 patients, a lthough focal electroretinogram results were abnormal in 8 of 9 patients. P hotopsia always decreased but visual fields did not improve. Six patients e xperienced recurrence. Conclusions: The clinical features of AIBSE include photopsia, visual field defects, abnormal findings from fundoscopic and fluorescein angiography, a nd abnormal results of focal electroretinography. The disease affects the p eripapillary retina and may cause an afferent pupillary defect. The strikin g predilection for the peripapillary retina suggests a local etiologic fact or and distinguishes AIBSE from the multiple evanescent white dot syndrome. Unlike patients with multiple evanescent white dot syndrome, recovery of v isual field did not occur in patients with AIBSE.