A CASE OF ACUTE ZONAL OCCULT OUTER RETINOPATHY UNDER IMMUNOMODULATORY TREATMENT

To describe the clinical course and treatment response of a case of acute zonal occult outer retinopathy.

This is an observational case report. The examinations included ophthalmic examination, longitudinal multimodal imaging, visual field testing, electrophysiologic recordings, serologic analyses, and whole genome sequencing.

In this report, the authors present the case of an otherwise healthy 33-year-old woman with bilateral acute zonal occult outer retinopathy manifestation. Other possible causes of the observed retinal lesions were ruled out by extensive diagnostic workup. Treatment with oral prednisolone therapy led to temporal disease control but progression was observed after prednisolone discontinuation. A tapered oral prednisolone therapy in combination with adalimumab initiation prevented further progression for at least 21 months.

Diagnosis of acute zonal occult outer retinopathy is frequently complicated by the unspecific symptoms of the disease and significant number of differential diagnoses. Complete diagnostic workup is important to rule out other etiologies. Owing to the lack of randomized controlled trials, therapeutic decisions obligatorily rely on empiric treatment decisions in combination with frequent follow-up examinations.