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

Abstract

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.

Coarsening of choriocapillaris on optical coherence tomography angiography as a sign of acute idiopathic blind spot enlargement

“…Secondly, a primary inflammation of the photoreceptors also known as “photoreceptoritis” leads to outer segments attrition. 6 …”

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

“…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.…”

“…Some authors have described cases in which restoration of the ellipsoidal zone on OCT was associated with an improvement in vision [3,5,11]. Occasionally, despite persisting irregularities in the outer retinal bands on OCT, there is a complete regression of visual field defects on perimetry [3,35]. 17 of the 36 studies included in this review, representing 113 patients out of a total of 136 published cases, date from the years prior to 2006, when SD-OCT became commercially available, allowing for a more detailed assessment of the outer peripapillary retina [38].…”

“…The use of systemic steroids has been re-ported in individual case descriptions [3,4,6,7,10,17,21]. Out of the treated patients included in this review, 12 received systemic treatment with prednisone at an initial dose of 30 to 100 mg, subsequently tapered over several weeks [3,4,6,7,10,15,24,26,31,35]. The period between the onset of symptoms and the start of therapy ranged from 1 to 4 weeks.…”

“…Einige Autoren haben Fälle beschrieben, in denen die Wiederherstellung der ellipsoiden Zone im OCT mit einer Verbesserung des Sehvermögens einherging [3,5,11]. Mitunter kommt es trotz bestehender Irregularitäten der äußeren Netzhautbanden im OCT zu einem vollständigen Rückgang der Gesichtsfelddefekte in der Perimetrie [3,35]. 17 der 36 in dieser Übersichtsarbeit berücksichtigten Arbeiten mit 113 der insgesamt 136 publizierten Patienten stammen aus den Jahren vor 2006, dem Jahr, in dem die SD-OCT kommerziell verfügbar wurde, welche die detailliertere Beurteilung der äußeren peripapillären Netzhaut erlaubt [38].…”