Acute Idiopathic Blind Spot Enlargement Syndrome—New Perspectives in the OCT Era

Abstract

Acute idiopathic blind spot enlargement syndrome (AIBSES) is characterized by unilateral visual field loss in the blind spot area, acute onset photopsia, and funduscopically few or no optic disc changes. AIBSES predominantly affects young adults and is often misdiagnosed as optic neuritis because of low awareness. Optical coherence tomography (OCT) has become the gold standard in diagnosing AIBSES as a disease of the outer retina. In our case series, we present three consecutive patients with AIBSES followed prospectively with and without steroid therapy. The patients, aged 25 to 27 years, presented in our neuroophthalmology department between 2020 and 2021. We report their disease course and management and discuss therapeutic options, as no well-established procedures exist. Common pitfalls and diagnostic errors are analysed. Two women and one man showed unilateral acute-onset photopsia and blind spot enlargement on perimetry without visual acuity reduction. Spectral domain OCT (Heidelberg Engineering, Heidelberg, Germany) revealed marked peripapillary changes in the ellipsoid zone and autofluorescence in all patients, corresponding to faint blurring of the optic disc margin. Characteristically, there was no P100 latency delay in the visual evoked potential in any of the patients. Two patients received weight-adapted oral prednisolone, which was gradually tapered over six to eight weeks. Two patients showed full recovery of their symptoms at six and seven months after onset, while mild defect healing was seen in one treated patient after 12 months. Follow-up OCT showed restoration of the outer retinal layers 6–12 months after disease onset. Careful history taking and an unprejudiced ophthalmological workup helps in diagnosing AIBSES in young adults with unilateral acute visual field defects. While its etiology is still unclear, accurate diagnosis of AIBSES can be made with peripapillary OCT. In our cases, the disease course of AIBSES was much better than its reputation. Early corticosteroid treatment may support outer retinal reorganisation, which can be followed with OCT in accordance with visual field restoration. This should be addressed in a prospective study.

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

“…In der Studie von Volpe et al lag nur in 3 der 27 Fälle eine Vitritis vor [9]. Teilweise finden sich im OCT Hyperreflektivitäten in der Nähe der inneren Netzhautschichten, die aufgrund ihrer Lage der indirekten Funduskopie nicht zugänglich sind [3].…”

“…peripapillär. Fleckige Bereiche mit später Hyperfluoreszenz in der Peripherie, wie bei MEWDS beschrieben, werden i. d. R. nicht gefunden ebenso wenig wie periphere Veränderungen der Fundus-Autofluoreszenz [3].…”

“…Das Vorkommen von vitrealen Zellen und das klinische Ansprechen auf eine Steroidtherapie lassen eine vermutete parainfektiöse Genese zumindest möglich erscheinen [3].…”

“…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.…”

“…In the study by Volpe et al, vitritis was present in only 3 out of 27 cases [9]. In some cases, hyperreflectivity is found on OCT near the inner layers of the retina, which cannot be observed on indirect fundoscopy due to their location [3].…”