Current understanding of acute zonal occult outer retinopathy (AZOOR)

Abstract

Acute Zonal Occult Outer Retinopathy (AZOOR) is characterized by sudden visual impairment, often presenting with scotoma and photopsia in young to middle-aged adults, showing a female predominance. This condition, distinct from genetic disorders like retinitis pigmentosa, lacks a hereditary basis and exhibits unique fundus changes and imaging features indicative of outer retinal dysfunction. Recent advancements have broadened our understanding, identifying variants like Acute Annular Outer Retinopathy (AAOR) and Multizonal outer retinopathy and retinal pigment epitheliopathy (MORR), each with specific clinical presentations and imaging characteristics. The diagnosis of AZOOR and its variants primarily relies on excluding other conditions through comprehensive evaluation, including imaging and serological testing. Treatment approaches, including the use of corticosteroids and immunosuppressives, remain debated, with some evidence suggesting benefits in the early stages. The prognosis of AZOOR varies, with most patients experiencing stabilization, although complications like choroidal neovascularization may occur, requiring targeted therapy. This manuscript elucidates the complexity of AZOOR, emphasizing the necessity of high clinical suspicion and the role of advanced imaging in diagnosis and management.

Figure 1

Image characteristics of a case of acute zonal occult outer retinopathy in a 45-year-old woman who presented with gradual onset vision loss for the past 1 year, scotoma, and nyctalopia. She had no visual complaints before this event and no family history of night blindness. Color fundus photograph of (a) right eye and (b) left eye showing pale disk and diffuse retinal pigment epithelial alteration at the posterior pole and along the vascular arcades. Blue fundus autofluorescence of (c) right eye and (d) left eye showing normal autofluorescence in the area outside of the demarcating line (zone 1—orange star), speckled hyperautofluorescence within the lesion (zone 2—blue star), and hypoautofluorescent areas that corresponded to the retinal pigment epithelium atrophy (zone 3—yellow star)

Figure 2

Infrared reflectance of (a) right eye and (b) left eye with structural OCT scan of the green line (c and d) showing multifocal hyper-reflective material present inside the demarcating line (blue arrow) and loss of external limiting membrane and ellipsoid zone corresponding to atrophic areas (orange arrow)