Acute zonal occult outer retinopathy (AZOOR) is a rare disease of unknown etiology characterized by focal photoreceptor abnormalities. Aim of this project was to evaluate how to monitor its clinical activity.


Retrospective analysis of patients with uveitis was conducted at Uveitis Centre in Prague and patients with diagnosis of AZOOR were identified. Clinical activity of AZOOR has been monitored by visual acuity, visual field (VF) testing, fundus autofluorescence imaging (FAF), spectral domain optical coherence tomography (SD-OCT), enhanced depth imaging optical coherence tomography (EDI-OCT), and electroretinogram (ERG).


From 2531 patients with uveitis, white dot syndromes (WDS) were diagnosed in 86 patients (3%), most frequently with punctate inner choroidopathy (PIC). The diagnosis of AZOOR has been established in 7 patients (0,3%). AZOOR was associated with PIC in four patients, with multiple evanescent white dot syndrome in one patient and with systemic autoimmune disease in two patients. VF testing showed various types of scotomata, which were changing with AZOOR activity. SD-OCT imaging revealed changes of ellipsoid zone in the retina, correlating with VF changes. Thickness of choroid has been analyzed by EDI‑OCT, showing changes of thickness with AZOOR activity. ERG was pathological in all tested patients. Visual acuity testing correlated with activity of AZOOR only in patients with central or centrocecal scotoma. FAF correlated more with WDS activity than with AZOOR.


Regarding AZOOR, several clinical examination methods can be used for monitoring. From these the most relevant clinically appears VF testing in correlation with SD-OCT and EDI‑OCT.



1Jarmyla Heissigerova, 2M. Mazná, 1Petra Svozílková, 1Michaela Brichová, 1Bohdan Kousal, 1Zora Dubská, 1Eva Řihová
1Department of Ophthalmology, Charles University and General University Hospital Prague, Czech Republic  2Private Eye Clinic, Faculty of Biomedical Engineering, Czech Technical University Prague, Czech Republic