When OCT Speaks Before the Fundus Does: Early ASHH Sign Revealing Acute Outer Retinopathy

PURPOSE: Within the acute zonal occult outer retinopathy (AZOOR) complex, acute outer retinopathy (AOR) has recently been proposed as an entity with rapid progression which remains under study. The present report aims to illustrate the clinical presentation, multimodal imaging findings, and disease course of AOR and to emphasize its role within the AZOOR spectrum. METHODS: We report a 19-year-old female presenting with acute photopsia and nasal scotoma in her left eye with a subtle whitish area in the fundus examination and the ASHH on OCT. Her visual acuity declined from 20/30 to 20/200 and to hand movements (HM), despite initiation of systemic corticosteroids. Partial structural recovery occurred over the following months without functional improvement. RESULTS: This case highlights the potentially aggressive course of AOR and supports the role of the ASHH sign as an early marker of complete photoreceptor disruption. The rapid progression and the dissociation between structural recovery and persistent visual loss suggest that the underlying pathophysiological mechanisms remain incompletely understood. CONCLUSION: AOR should be suspected in patients with typical AZOOR symptoms and the ASHH sign on OCT despite minimal fundus findings. Multimodal imaging, particularly OCT, is essential for early diagnosis and monitoring of disease progression.