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Original Investigation |

Concentric Macular Rings Sign in Patients With Foveal Hypoplasia ONLINE FIRST

Kurt Spiteri Cornish, FRCOphth1; Aravind R. Reddy, FRCOphth1; Vikki A. McBain, PhD1
[+] Author Affiliations
1Eye Outpatient Department, Aberdeen Royal Infirmary, Aberdeen, Scotland
JAMA Ophthalmol. Published online June 19, 2014. doi:10.1001/jamaophthalmol.2014.1715
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Importance  We describe a sign that can be used as a rapid and noninvasive adjunct to aid in the diagnosis of foveal hypoplasia.

Objective  To describe a concentric macular rings sign found on infrared reflectance (IRR) images in patients with foveal hypoplasia.

Design, Setting, and Patients  We studied 13 patients with foveal hypoplasia (7 with ocular albinism [OA], 5 with oculocutaneous albinism [OCA], and 1 with aniridia) at a tertiary ophthalmology center with access to electrodiagnostic services from February 18, 2009, through April 9, 2013.

Main Outcomes and Measures  All patients and an age-matched control participant underwent a complete clinical examination, electroretinography (full field and pattern), visual evoked potentials, fundus autofluorescence IRR, and optical coherence tomography (OCT). One patient with OA and the control participant also underwent scanning laser polarimetry with variable corneal compensation (GDx VCC).

Results  Thirteen patients (6 girls and 7 boys), with a mean age of 5.8 years (range, 3-11 years), were included in the study. Seven patients were diagnosed as having OA and had minimal clinical signs (fine nystagmus in 2 patients and subtle iris transillumination in 5 patients). Five patients with OCA and 1 with aniridia were also included. In 12 patients, OA and OCA were confirmed with 5-channel visual evoked potentials (optic nerve misrouting). Whenever OCT was performed, foveal hypoplasia was indicated by the lack of foveal dip. The macula lacked the foveal attenuation normally seen with fundus autofluorescence, and a concentric macular rings reflex was seen with IRR in all 13 patients and with GDx VCC in 1 patient. A normal bowtie reflex was seen with IRR and GDx VCC in the age-matched control participant.

Conclusions and Relevance  Our findings suggest that concentric macular rings seen on IRR or GDx VCC can occur in patients with foveal hypoplasia and can therefore aid in the diagnosis, especially in patients with minimal clinical signs (mild OA) or in cases in which OCT cannot be performed (young patients or patients with high-amplitude nystagmus).

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Figure 1.
Findings From Patient 1

A and B, Optical coherence tomogram shows foveal hypoplasia with complete absence of a foveal dip (arrows) in the right and left eyes, respectively. C, Optical coherence tomogram from an age-matched control participant highlights the foveal dip. D and E, Infrared reflectance image shows the concentric macular rings sign (dashed circles) described in this study in the right and left eyes, respectively. F, Infrared reflectance image in an age-matched control participant shows the bowtie reflex (dashed bowtie pattern). G and H, Fundus autofluorescence image shows loss of normal central attenuation at the fovea in the right and left eyes, respectively. I, Fundus autofluorescence image in an age-matched control participant shows the central attenuated signal due to increased macular pigment. J and K, Scanning laser polarimetry with variable corneal compensation image shows the concentric macular rings sign in the right and left eyes, respectively. L, Scanning laser polarimetry with variable corneal compensation image in an age-matched control participant shows the bowtie reflex.

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Figure 2.
Findings From 6 Patients With Newly Diagnosed Ocular Albinism and 3 Patients With Oculocutaneous Albinism

A-I, Foveal hypoplasia (arrows) was seen on optical coherence tomograms, and the concentric macular rings sign (dashed circles) was seen on infrared reflectance images taken with the Heidelberg retina angiograph 2 in all patients.

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