Clinical and Longitudinal Heterogeneity Within the Acute Outer Retinopathy Spectrum: A Three-Case Series

To describe three distinct presentations within the acute outer retinopathy (AOR) spectrum, emphasizing differences in stage, longitudinal evolution, and structure-function relationships.

Retrospective case series.

The first, a 14-year-old asymptomatic male, had bilateral sectoral and spot-like outer retinal lesions in the subacute-chronic stage. Optical coherence tomography (OCT) demonstrated outer nuclear layer thinning with ellipsoid and interdigitation zone disruption and focal retinal pigment epithelium (RPE) attenuation, without the angular sign of Henle fiber layer hyperreflectivity (ASHH). Fundus autofluorescence (FAF) revealed lesions with a hypoautofluorescent core and hyperautofluorescent border, while fluorescein angiography (FA) showed window-defect hyperfluorescence and focal venular wall staining. The second, a 20-year-old male, developed severe visual field loss after a viral illness. FAF showed perivenular outer retinal involvement, and OCT revealed perivenular disruption with ASHH documented at 11 months. Visual field deficits were severe and partially correlated with structural abnormalities, with incomplete recovery over 3 years. Pattern electroretinography showed borderline reduction, whereas full-field electroretinography was within the lower limits of normal. The third, a 43-year-old female, presented with acute visual symptoms and mild intraocular inflammation. FAF showed sectoral peripapillary hyperautofluorescence, with corresponding outer retinal disruption on OCT. FA demonstrated venular wall staining and optic disc hyperfluorescence without diffuse vascular leakage. Perimetry showed bilateral diffuse sensitivity loss, while electrophysiology demonstrated generalized cone-predominant photoreceptor dysfunction.

These cases illustrate clinical and longitudinal heterogeneity within established AOR patterns, including asymptomatic subacute-chronic disease, perivenular disease with structure-function dissociation and late documentation of ASHH, and symptomatic sectoral disease with diffuse photoreceptor dysfunction.