J Korean Ophthalmol Soc.  2016 Mar;57(3):413-419. 10.3341/jkos.2016.57.3.413.

Clinical Characteristics of Acute Zonal Occult Outer Retinopathy

• Bae KW
• Park KH
• Woo SJ
• Hwang JM

• KMID: 2213242
• DOI: http://doi.org/10.3341/jkos.2016.57.3.413

Abstract

PURPOSE
To investigate the clinical characteristics of patients with acute zonal occult outer retinopathy (AZOOR).
METHODS
Medical records of 13 patients who visited Seoul National University Bundang Hospital from May, 2003 to May, 2015 and diagnosed with AZOOR were retrospectively reviewed.
RESULTS
Thirteen patients (11 women and 2 men), with a mean age of 28.5 ± 11.4 years were followed for 42.8 ± 30.3 months. Visual field loss, photopsia, and blurred vision were common complaints. Initially, four patients had bilateral disease and seven patients showed bilateral involvement at the last visit. Mean best corrected visual acuity of involved eyes (BCVA) was 0.75 ± 0.32 (log MAR). Among 20 eyes with AZOOR, BCVA was 20/40 or better in 17 eyes (85.0%). The mean spherical equivalent was -4.59 ± 3.23 diopters (D), and 15 eyes (75.0%) had myopia less than -2.00 D. Nine eyes of seven patients (34.6%) had abnormal fundus findings. All patients underwent full field electroretinogram (ERG) or multifocal ERG and a visual field test. Thirteen patients (100.0%) showed a decreased response in ERG and visual field defects presented in every patient. With respect to the visual field test, 10 eyes (50.0%) showed improvement, 6 eyes (30.0%) had stationary status, and the progression of the visual field defect was observed in 4 eyes (20.0%). Among 13 patients, 4 (30.8%) patients showed flu-like symptom, 4 (30.8%) patients had fatigue, 2 (15.4%) patients had nausea, and 2 (15.4%) patients showed headache.
CONCLUSIONS
AZOOR should be considered as one of the differential diagnoses, especially in female patients with myopia who show photopsia or visual field defects. ERG and visual field tests are necessary to confirm a decrease in retinal function and visual field loss. Central vision is preserved in most cases and recovery of visual field defect occurs often.

Keyword

Acute zonal occult outer retinopathy; Photopsia; Visual field loss

MeSH Terms

Diagnosis, Differential
Fatigue
Female
Fluconazole*
Headache
Humans
Medical Records
Myopia
Nausea
Retinaldehyde
Retrospective Studies
Seoul
Visual Acuity
Visual Field Tests
Visual Fields
Fluconazole
Retinaldehyde

Figure

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  Figure 1. Fundus photography and spectral-domain optical coherence tomography examination. Fundus photograph (A) of the left eye shows normal posterior pole. Corresponding image of spectral-domain optical coherence tomography (B) shows disruption (arrowheads) of the internal segment-outer segment junction and cone outer segment tip line at temporal peripapillary area.

• 

  Figure 2. Fundus photography showing retinal degeneration. Fundus photograph of the left eye shows normal appearance one month after presentation (A). Seven months later after then, there is mild hypopigmentation of the retinal pigmented epithelium (RPE) around peripapillary area (B). At 69 months from the onset, it is observed that generalized severe RPE atrophy and depigmentation (C).

• 

  Figure 3. Visual field test and electroretinogram. Goldmann perimetry (target I4) shows central scotoma of the left eye (A). Decreased amplitudes (circles with dotted line) corresponding visual field defect is observed in multifocal electroretinogram (ERG) (B). Standard full-field ERG reveals slightly decreased rod and maximal combined response in left eye compared to those of the right eye (C). Rt = right; Lt = left; Diff = difference.

Reference

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