Abstract
PurposeTo report the clinical course and multimodal imaging features of acute zonal occult outer retinopathy (AZOOR) complicated by choroidal neovascularization (CNV) treated with anti-vascular endothelial growth factor (VEGF) treatment or photodynamic therapy (PDT).MethodsObservational case series. Retrospective analysis of patients presenting to different institutions with evidence of AZOOR and neovascular lesions. Diagnosis of AZOOR was made on the basis of clinical presentation and multimodal imaging. All patients underwent a comprehensive ophthalmic evaluation and multimodal retinal imaging, including color fundus photos, fundus autofluorescence, fundus fluorescein angiography and spectral-domain optical coherence tomography.ResultsFour patients (three males, mean age 53.5 years) were included in the study. Mean follow-up was 5.1 years. Presentation of AZOOR was unilateral in two patients and bilateral in the remainder two patients. One of the patients presenting with unilateral AZOOR developed zonal lesions in the fellow eye during follow-up. All patients presented with unilateral type 2 (subretinal) CNV. Three patients underwent intravitreal anti-VEGF injections and one patient underwent a single PDT. Multimodal retinal imaging showed zonal or multizonal progression during treatment. After treatment, visual acuity and CNV stabilization was observed in all patients.ConclusionsThe presence of CNV expands the clinical spectrum of AZOOR. CNV complicating AZOOR may be effectively treated with intravitreal injections of anti-VEGF, despite progression of the zonal lesions. Further studies are required to define the role of treatment in the progression of the zonal lesions.
Highlights
As originally defined in 1992 by Gass in his Donder’s Lecture [1], acute zonal occult outer retinopathy (AZOOR) is a rare, condition of unknown etiology, characterized by an acute loss of one or more zones of outer retinal function
A common feature of the disease is the acute zonal lesion, or lesions, delineated by a grayish ring on funduscopy or a demarcation line of hyperautofluorescence seen on fundus autofluorescence imaging, which has been referred to as acute annular outer retinopathy (AAOR) [2]
The purpose of this study is to report in details the clinical course and the multimodal retinal imaging of patients presenting with choroidal neovascularization (CNV) and AZOOR
Summary
As originally defined in 1992 by Gass in his Donder’s Lecture [1], acute zonal occult outer retinopathy (AZOOR) is a rare, condition of unknown etiology, characterized by an acute loss of one or more zones of outer retinal function. AZOOR became a general diagnostic term for chorioretinal diseases with visual loss of uncertain origin. Introini et al Int J Retin Vitr (2018) 4:32 on multimodal retinal imaging findings, including a demarcating line of progression between the involved and uninvolved retina and a trizonal pattern of chorioretinal degeneration, has been recently proposed [3, 4]. Levison et al [6] recently reported a case of CNV in AZOOR but long-term follow-up was not included.
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