Overview
This rare condition typically affects young, healthy females. Presenting symptoms invariably include scotoma and photopsia, but may also include metamorphopsia, photophobia and difficulty with night vision.
In the acute phase, the retina appears normal and central visual acuity unaffected. Fundus autofluorescence (FAF) at this stage shows a diffuse patchy hyper-fluorescent signal. On OCT, outer retinal disruption may be seen, however the RPE is typically intact.
Chronic and more advanced cases are associated with mildly reduced visual acuity and a peripapillary area of RPE atrophy. A demarcation line exists between the affected and unaffected retina, best seen on FAF.
In this stage, AZOOR is characterised by a “trizonal” degeneration. FAF and OCT outside the affected area is normal. Inside this area (separated by the demarcation line), FAF shows speckled hyper-fluorescence and the presence of multifocal material deposited on the RPE (similar to subretinal drusenoid deposits). The third (central) zone is characterised by photoreceptor, RPE and choroidal atrophy.
Differential Diagnosis
References
Mrejen S, Khan S, Gallego-Pinazo R, Jampol LM, Yannuzzi LA. (2014) Acute Zonal Occult Outer Retinopathy: A Classification Based on Multimodal Imaging. JAMA Ophthalmol. 132(9):1089–1098.
Tavallali, A., Yannuzzi, LA. (2015) Acute Zonal Occult Outer Retinopathy; Revisited. Journal of Ophthalmic and Vision Research 10(3): 211–213.