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Concentric macular rings sign in patients with foveal hypoplasia.
JAMA Ophthalmol. 2014 Sep; 132(9):1084-8.JO

Abstract

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).

Authors+Show Affiliations

Eye Outpatient Department, Aberdeen Royal Infirmary, Aberdeen, Scotland.Eye Outpatient Department, Aberdeen Royal Infirmary, Aberdeen, Scotland.Eye Outpatient Department, Aberdeen Royal Infirmary, Aberdeen, Scotland.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

24945710

Citation

Cornish, Kurt Spiteri, et al. "Concentric Macular Rings Sign in Patients With Foveal Hypoplasia." JAMA Ophthalmology, vol. 132, no. 9, 2014, pp. 1084-8.
Cornish KS, Reddy AR, McBain VA. Concentric macular rings sign in patients with foveal hypoplasia. JAMA Ophthalmol. 2014;132(9):1084-8.
Cornish, K. S., Reddy, A. R., & McBain, V. A. (2014). Concentric macular rings sign in patients with foveal hypoplasia. JAMA Ophthalmology, 132(9), 1084-8. https://doi.org/10.1001/jamaophthalmol.2014.1715
Cornish KS, Reddy AR, McBain VA. Concentric Macular Rings Sign in Patients With Foveal Hypoplasia. JAMA Ophthalmol. 2014;132(9):1084-8. PubMed PMID: 24945710.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Concentric macular rings sign in patients with foveal hypoplasia. AU - Cornish,Kurt Spiteri, AU - Reddy,Aravind R, AU - McBain,Vikki A, PY - 2014/6/20/entrez PY - 2014/6/20/pubmed PY - 2015/1/13/medline SP - 1084 EP - 8 JF - JAMA ophthalmology JO - JAMA Ophthalmol VL - 132 IS - 9 N2 - 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). SN - 2168-6173 UR - https://www.unboundmedicine.com/medline/citation/24945710/Concentric_macular_rings_sign_in_patients_with_foveal_hypoplasia_ L2 - https://jamanetwork.com/journals/jamaophthalmology/fullarticle/10.1001/jamaophthalmol.2014.1715 DB - PRIME DP - Unbound Medicine ER -