Clinical Characteristics of Acute Zonal Occult Outer Retinopathy

Ki Woong Bae; Kyu Hyung Park; Se Joon Woo; Jeong Min Hwang

doi:10.3341/jkos.2016.57.3.413

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

Original Article

J Korean Ophthalmol Soc 2016;57(3):413-419.

Published online: 3 September 2016

DOI: https://doi.org/10.3341/jkos.2016.57.3.413

Clinical Characteristics of Acute Zonal Occult Outer Retinopathy

Ki Woong Bae, MD, Kyu Hyung Park, MD, Se Joon Woo, MD, Jeong Min Hwang, MD

Department of Ophthalmology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea

Address reprint requests to Jeong Min Hwang, MD, Department of Ophthalmology, Seoul National University Bundang Hospital, #82 Gumi-ro 173beon-gil, Bundang-gu, Seongnam 13620, Korea, Tel: 82-31-787-7372, Fax: 82-31-787-4057, E-mail: hjm@snu.ac.kr

Received 30 July 2015 Revised 8 September 2015 Accepted 11 November 2015

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Acute zonal occult outer retinopathy, Photopsia, Visual field loss

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.

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.

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
Laboratory tests	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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