A CASE OF ACUTE ZONAL OCCULT OUTER RETINOPATHY UNDER IMMUNOMODULATORY TREATMENT

Abstract

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

Methods: 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.

Results: 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.

Conclusion: 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.

Keywords: AZOOR; acute outer; acute zonal occult outer retinopathy; adalimumab; degeneration; retina.

Fig. 1.

FAF, color fundus photography, and visual field examinations at initial presentation. A, B. Note the hyperautofluorescent, granular lesions with narrow nongranular, hyperautoflourescent borders extending from the optic disk toward the superior and temporal fundus in the right eye and toward the superior fundus in the left eye on FAF imaging. C, D. Color fundus photographs showing mild signs of retinal attenuation in areas corresponding to the lesions seen on FAF imaging. The lower border of the lesion in the right eye showed a clearly visible orange line (white arrows in C). No such orange line was observable in the left eye (D). E. Static 30° perimetry at initial presentation to our clinic showing homonymous inferocentral scotomas that primarily affected the lower left quadrants but violated the vertical meridian (the left panel represents the left eye and the right panel the right eye).

Fig. 2.

Fundus autofluorescence and fundus photography time course. The left column shows FAF images and color fundus photographs of the right eye, the right column shows corresponding images of the left eye. After initial presentation to our clinic (month 0), a new satellite lesion nasal to the optic disk developed in the right eye (month 2, white arrow). We initiated a prednisolone therapy (upper red box) under which the lesions remained stable for 4 months. With prednisolone tapering, another satellite lesion in the right eye developed superior to the optic disk (month 8, white arrow). We initiated another prednisolone therapy, followed by the initiation of adalimumab (lower red and green box; months 8–11). After discontinuation of prednisolone and with adalimumab monotherapy, the lesions remained stable until the most recent follow-up (month 32). Over time, the lesions adopted a rather hypoautofluorescent phenotype in contrast to the initial hyperautofluorescence.