From A… to… Z(OOR): The Clinical Spectrum of Acute Zonal Occult Outer Retinopathy

Wang, Margaret; Sadaka, Ama; Prager, Thomas C.; Lee, Andrew G.; Pellegrini, F.; Cirone, Daniele; Simone, Luca De; Cimino, Luca

doi:10.1080/01658107.2017.1400076

Cited by 9 publications

(16 citation statements)

References 16 publications

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“…Although the clinical appearance of AZOOR varies depending on disease duration and distribution of lesions, there are some typical features including the demarcating line of progression between the involved and uninvolved retina, the trizonal pattern of sequential involvement of the outer retina, retinal pigment epithelium, and choroid, as well as frequent zonal progression [11][12][13][14]. In our patient, the development of symptoms, distribution, and morphology of abnormalities mimicked those in previously reported cases [9,10,15].…”

Section: Discussionsupporting

confidence: 80%

“…Another key finding in AZOOR is zonal visual field defect [1]. Among the multiple patterns of visual field loss, the most prevalent is an enlarged blind spot with central scotoma [1,10]. Some related abnormalities, such as inflammation in the vitreous, perivascular exudates, cystoid macular edema, changes in the retinal pigment epithelium (RPE), narrowing of the retinal vessels, and optic nerve edema have also been reported [1,9].…”

Section: Discussionmentioning

confidence: 99%

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Acute Zonal Occult Outer Retinopathy in a Patient Suffering from Epilepsy: Five-Year Follow-Up

et al. 2021

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We report an unprecedented case of a young patient with epilepsy coexisting with acute zonal occult outer retinopathy (AZOOR), a rare white dot syndrome of unknown etiology, associated with damage to the large zones of the outer retina. Recently, it has been established that epileptic episodes contribute to an inflammatory response both in the brain and the retina. A 13-year-old male patient with epilepsy was referred by a neurologist for an ophthalmologic consultation due to a sudden deterioration of visual acuity in the left eye. The examination, with a key role of multimodal imaging including color fundus photography, fluorescein angiography, indocyanine green angiography (ICGA), fundus autofluorescence (FAF), swept-source optical coherence tomography (SS-OCT) with visual field assessment, and electroretinography indicated AZOOR as the underlying entity. Findings at the first admission included enlargement of the blind spot in visual field examination along a typical trizonal pattern, which was revealed by FAF, ICGA, and SS-OCT in the left eye. The right eye exhibited no abnormalities. Seminal follow-up revealed no changes in best corrected visual acuity, and multimodal imaging findings remain unaltered. Thus, no medical intervention is required. Our case and recent laboratory findings suggest a causative link between epilepsy and retinal disorders, although this issue requires further research.

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Section: Discussionsupporting

confidence: 80%

Section: Discussionmentioning

confidence: 99%

Acute Zonal Occult Outer Retinopathy in a Patient Suffering from Epilepsy: Five-Year Follow-Up

et al. 2021

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“…It is not clear whether they only share a similar etiology or rather if AZOOR should be considered part of this spectrum of diseases. The debate about AZOOR and other similar occult or non-occult retinopathies centres on whether one is a “lumper” or a “splitter.” Recently, Wang et al 6 argumented that lumpers make more sense than splitters and they propose that there is a chronic equivalent of acute idiopathic blind spot enlargement syndromes and chronic idiopathic blind spot enlargement syndromes which may be on the spectrum of AZOOR.…”

Section: Discussionmentioning

confidence: 99%

Acute zonal occult outer retinopathy complex disease: Lessons learned about choroid, photoreceptors, and retinal function

Pellegrini

Cirone²,

Simone

et al. 2021

European Journal of Ophthalmology

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Purpose: Retinal photoreceptors layer integrity is considered essential to visual function. We report a case of acute zonal occult outer retinopathy (AZOOR) complex disease (namely AIBSE: acute idiopathic blind spot enlargement) in which apparently a full anatomic regeneration is not needed for a complete functional recovery. Methods: Case report with multimodal imaging. Reports: Visual field recovery in the presence of photoreceptors layer disruption studied by means of Optical Coherence Tomography. Choroid and photoreceptors layer thickness thinned progressively during recovery. Conclusion: This case suggests that anatomical retinal integrity as shown by OCT does not always correspond to visual function. Our case highlights that a complete visual recovery can occur even when structural abnormalities are still observable.

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“…Besonders AIBSES und AZOOR weisen Ähnlichkeiten auf [31]. Bei beiden Erkrankungen finden sich Veränderungen auf der Ebene der äußeren Netzhautbanden im OCT mit klinisch Photopsien, Gesichtsfeldausfällen und vermeintlich unauffälligem Fundoskopiebefund.…”

Section: Klassifikationunclassified

“…Über den Einsatz von systemischen Steroiden wurde in Einzelfallbeschreibungen berichtet [3,4,6,7,10,17,21]. Zwölf der eingeschlossenen behandelten Patienten erhielten eine systemische Therapie mit Prednison in einer initialen Dosierung zwischen 30 mg und 100 mg, in der Folge ausschleichend über mehrere Wochen [3,4,6,7,10,15,24,26,31,35]. Der Zeitraum zwischen Symptomauftreten und Therapiebeginn lag dabei zwischen 1 und 4 Wochen.…”

Section: äTiologieunclassified

Acute Idiopathic Blind Spot Enlargement Syndrome (AIBSES) – Nutzen der OCT: Übersichtsarbeit

Zimmermann,

Biermann

2023

Klin Monbl Augenheilkd

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ZusammenfassungLediglich rund 100 Fallberichte existieren zum Acute Idiopathic Blind Spot Enlargement Syndrome (AIBSES). Es ist charakterisiert durch den namensgebenden Gesichtsfeldausfall im Bereich des blinden Flecks, akut einsetzende Photopsien und funduskopisch geringe oder keine Veränderungen der Sehnervenkopfumgebung bei auffälligen äußeren Netzhautbanden in der optischen Kohärenztomografie (OCT). Typisch ist das einseitige Auftreten. Betroffen sind vorwiegend junge Frauen. Während bisherige Übersichtsarbeiten zum AIBSES entweder vor der Einführung der OCT entstanden sind oder den Fokus auf die Abgrenzung gegenüber möglicherweise verwandten Krankheitsbildern der äußeren Netzhaut legten (z. B. Multiple Evanescent White Dot Syndrome und Acute Zonal Occult Outer Retinopathy), arbeiten wir hier die aktuelle Perspektive und bislang erfolgte Behandlungsstrategien auf und möchten zur Steigerung des Bekanntheitsgrades beitragen. Die Einführung der OCT hat seit der Erstbeschreibung des AIBSES in den späten 1980er-Jahren die Diagnosestellung und Charakterisierung von AIBSES als Erkrankung der äußeren Netzhaut vereinfacht. Trotzdem kommt es weiterhin häufig zu Fehldiagnosen im Spektrum der Sehnervenentzündungen, da AIBSES nicht differenzialdiagnostisch erwogen wird.

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From A… to… Z(OOR): The Clinical Spectrum of Acute Zonal Occult Outer Retinopathy

Cited by 9 publications

References 16 publications

Acute Zonal Occult Outer Retinopathy in a Patient Suffering from Epilepsy: Five-Year Follow-Up

Acute Zonal Occult Outer Retinopathy in a Patient Suffering from Epilepsy: Five-Year Follow-Up

Acute zonal occult outer retinopathy complex disease: Lessons learned about choroid, photoreceptors, and retinal function

Acute Idiopathic Blind Spot Enlargement Syndrome (AIBSES) – Nutzen der OCT: Übersichtsarbeit

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