ACUTE ZONAL OCCULT OUTER RETINOPATHY AFFECTING THE PERIPHERAL RETINA WITH CENTRIPETAL PROGRESSION.

PURPOSE: To describe a variant of acute zonal occult outer retinopathy (AZOOR) that has concentric involvement of the peripheral retina with centripetal progression toward the posterior pole.

METHODS

Three patients with AZOOR were reported to show peripheral concentric zonal involvement with centripetal progression of their disease from the periphery to the posterior fundus.

RESULTS

All three cases involved elderly hyperopic women with a history of autoimmune disease. All six eyes showed bilateral central peripapillary AZOOR lesions that progressed in a centrifugal manner to the periphery. Five of the six eyes showed the presence of concentric peripheral zonal abnormalities that progressed in a centripetal manner to the posterior pole. In one case, the peripheral and central zonal abnormalities became confluent, leaving only a small island of normal retina temporal to the fovea.

CONCLUSION

A variant of AZOOR may involve the peripheral retina, causing concentric zonal atrophy with centripetal progression, with central peripapillary zonal abnormalities that have centrifugal progression. This may eventually lead to widespread atrophic degeneration with severe visual field loss. Wide-field imaging of the peripheral retina and monitoring of the visual fields are important to document this rare atypical presentation of AZOOR and any subsequent disease progression.