Journal List > J Korean Ophthalmol Soc > v.57(3) > 1010527

Bae, Park, Woo, and Hwang: Clinical Characteristics of Acute Zonal Occult Outer Retinopathy

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.

References

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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.
jkos-57-413f1.tif
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).
jkos-57-413f2.tif
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.
jkos-57-413f3.tif
Table 1.
Clinical characteristics of patients with AZOOR
Clinical factors Result
Gender
 Male 15.4% (2/13)
 Female 84.6% (11/13)
Age at first visit (years) 28.5 ± 11.4 (12.4-51.5)
Follow up period (months) 42.8 ± 30.3 (8.7-116.2)
Duration from the onset to diagnosis (weeks) 62.1 ± 93.1 (1.4-313.1)
Clinical symptoms (multiple answers)
 Scotoma 61.5% (8/13)
 Photopsia 61.5% (8/13)
 Blurred vision 23.1% (3/13)
Associated generalized symptom (multiple answers)
 Flu-like symptom 30.8% (4/13)
 Fatique 30.8% (4/13)
 Nausea 15.4% (2/13)
 Headache 15.4% (2/13)
Bilaterality
 Unilateral involvement at presentation 69.2% (9/13)
 Bilateral involvement at presentation 30.8% (4/13)
 Unilateral involvement during follow up 46.2% (6/13)
 Bilateral involvement during follow up 53.8% (7/13)
Mean BCVA (log MAR)
 Eyes with AZOOR at first exam (17 eyes) 0.23 ± 0.33
 Fellow eyes at first exam (9 eyes) 0.01 ± 0.03
 Eyes with AZOOR at last visit (20 eyes) 0.28 ± 0.56
 Fellow eyes at last visit (6 eyes) 0.00 ± 0.00
Refractive errors −4.59 ± 3.23 (-0.50∼-9.25)
 Hyperopic (SE > +0.25 D) 0.0% (0/20)
 Emmetropic (SE: +0.25 to −0.25 D) 0.0% (0/20)
 Myopic (SE < −0.25 D) 100.0% (20/20)
  SE: −0.50 to −1.75 D 25.0% (5/20)
  SE: less than −2.00 D 75.0% (15/20)
Treatment
 Observation 69.2% (9/13)
 Medical treatment (steroid and/or immunosuppressants) 30.8% (4/13)

Values are presented as mean ± SD unless otherwise indicated.

AZOOR = acute zonal occult outer retinopathy; BCVA = best corrected visual acuity; log MAR = logarithm of the minimum angle of resolution; SE = spherical equivalent.

Table 2.
Ancillary study results and follow up data of patients with AZOOR
Laboratory tests Result
Number of patients (total 13 patients) Number of eyes (total 26 eyes)
Fundus examination
 Initial examination
  Normal fundi 53.8% (7/13) 69.2% (18/26)
  Localized RPE atrophy or depigmentation 46.2% (6/13) 30.8% (8/26)
 Final examination
  Normal fundi 46.2% (6/13) 65.4% (17/26)
  Localized RPE atrophy or depigmentation 53.8% (7/13) 34.6% (9/26)
Full field ERG/multifocal ERG
 Decreased cone response alone 30.8% (4/13)
 Decreased rod response alone 7.7% (1/13)
 Decreased cone and rod function 30.8% (4/13)
 Locally decreased reseponse in mfERG 46.2% (6/13)
Visual field findings (multiple answers)
 Initial examination
  BSE 47.1% (8/17*)
  Central scotoma 35.3% (6/17)
  One-quadrant scotoma 11.8% (2/17)
  Two-quadrant scotoma 35.3% (6/17)
  Three-quadrant scotoma 5.9% (1/17)
  Four-quadrant scotoma 5.9% (1/17)
 Final examination
  BSE 50.0% (7/14*)
  Central scotoma 42.9% (6/14)
  One-quadrant scotoma 7.1% (1/14)
  Two-quadrant scotoma 7.1% (1/14)
  Three-quadrant scotoma 7.1% (1/14)
  Four-quadrant scotoma 14.3% (2/14)
Course of visual field results
 Improvement 50.0% (10/20*)
 Stable disease 30.0% (6/20)
 Progression 20.0% (4/20)

AZOOR = acute zonal occult outer retinopathy; RPE = retinal pigment epithelium; ERG = electroretinography; mfERG = multifocal electroretinography; BSE = blind spot enlargement.

* Initially, 17 eyes showed visual field defect and totally 20 eyes presented visual field loss during follow up. On last visit, 6 eyes had normal visual field results and scotomas remained in 14 eyes.

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