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Abnormal foveal morphology in ocular albinism imaged with spectral-domain optical coherence tomography.
Arch Ophthalmol. 2009 Jan; 127(1):37-44.AO

Abstract

OBJECTIVES

To evaluate the spectrum of foveal architecture in pediatric albinism and to assess the utility of spectral-domain optical coherence tomography (OCT) in ocular imaging of children with nystagmus.

METHODS

Spectral-domain OCT imaging was performed on study subjects in 3 groups: subjects with ocular albinism (OA) or suspected OA with foveal hypoplasia, with nystagmus, and with or without iris transillumination; a subject with oculocutaneous albinism and Hermansky-Pudlak syndrome; and control subjects. Dense volumetric scans of each fovea were captured using standard and handheld spectral-domain OCT devices. Images were postprocessed and scored for the presence and configuration of each retinal layer across the fovea.

RESULTS

High-quality spectral-domain OCT images obtained from each subject revealed a range of abnormalities in subjects with OA or suspected OA and the subject with oculocutaneous albinism and Hermansky-Pudlak syndrome: persistence of an abnormal, highly reflective band across the fovea, multiple inner retinal layers normally absent at the center of the fovea, and loss of the normally thickened photoreceptor nuclear layer at the fovea when compared with that in control subjects. The optic nerve was elevated in multiple eyes of subjects with OA or suspected OA and the subject with oculocutaneous albinism and Hermansky-Pudlak syndrome.

CONCLUSIONS

A spectrum of foveal morphological abnormalities is seen in subjects with OA or suspected OA, which in some cases contrasted with previous studies using time-domain OCT. These OCT findings clarify the morphology of foveal hypoplasia seen clinically. This imaging modality may be useful in evaluating children.

Authors+Show Affiliations

Department of Ophthalmology, Duke University Medical Center, Durham, North Carolina 27710, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

19139336

Citation

Chong, Gabriel T., et al. "Abnormal Foveal Morphology in Ocular Albinism Imaged With Spectral-domain Optical Coherence Tomography." Archives of Ophthalmology (Chicago, Ill. : 1960), vol. 127, no. 1, 2009, pp. 37-44.
Chong GT, Farsiu S, Freedman SF, et al. Abnormal foveal morphology in ocular albinism imaged with spectral-domain optical coherence tomography. Arch Ophthalmol. 2009;127(1):37-44.
Chong, G. T., Farsiu, S., Freedman, S. F., Sarin, N., Koreishi, A. F., Izatt, J. A., & Toth, C. A. (2009). Abnormal foveal morphology in ocular albinism imaged with spectral-domain optical coherence tomography. Archives of Ophthalmology (Chicago, Ill. : 1960), 127(1), 37-44. https://doi.org/10.1001/archophthalmol.2008.550
Chong GT, et al. Abnormal Foveal Morphology in Ocular Albinism Imaged With Spectral-domain Optical Coherence Tomography. Arch Ophthalmol. 2009;127(1):37-44. PubMed PMID: 19139336.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Abnormal foveal morphology in ocular albinism imaged with spectral-domain optical coherence tomography. AU - Chong,Gabriel T, AU - Farsiu,Sina, AU - Freedman,Sharon F, AU - Sarin,Neeru, AU - Koreishi,Anjum F, AU - Izatt,Joseph A, AU - Toth,Cynthia A, PY - 2009/1/14/entrez PY - 2009/1/14/pubmed PY - 2009/1/31/medline SP - 37 EP - 44 JF - Archives of ophthalmology (Chicago, Ill. : 1960) JO - Arch Ophthalmol VL - 127 IS - 1 N2 - OBJECTIVES: To evaluate the spectrum of foveal architecture in pediatric albinism and to assess the utility of spectral-domain optical coherence tomography (OCT) in ocular imaging of children with nystagmus. METHODS: Spectral-domain OCT imaging was performed on study subjects in 3 groups: subjects with ocular albinism (OA) or suspected OA with foveal hypoplasia, with nystagmus, and with or without iris transillumination; a subject with oculocutaneous albinism and Hermansky-Pudlak syndrome; and control subjects. Dense volumetric scans of each fovea were captured using standard and handheld spectral-domain OCT devices. Images were postprocessed and scored for the presence and configuration of each retinal layer across the fovea. RESULTS: High-quality spectral-domain OCT images obtained from each subject revealed a range of abnormalities in subjects with OA or suspected OA and the subject with oculocutaneous albinism and Hermansky-Pudlak syndrome: persistence of an abnormal, highly reflective band across the fovea, multiple inner retinal layers normally absent at the center of the fovea, and loss of the normally thickened photoreceptor nuclear layer at the fovea when compared with that in control subjects. The optic nerve was elevated in multiple eyes of subjects with OA or suspected OA and the subject with oculocutaneous albinism and Hermansky-Pudlak syndrome. CONCLUSIONS: A spectrum of foveal morphological abnormalities is seen in subjects with OA or suspected OA, which in some cases contrasted with previous studies using time-domain OCT. These OCT findings clarify the morphology of foveal hypoplasia seen clinically. This imaging modality may be useful in evaluating children. SN - 1538-3601 UR - https://www.unboundmedicine.com/medline/citation/19139336/Abnormal_foveal_morphology_in_ocular_albinism_imaged_with_spectral_domain_optical_coherence_tomography_ L2 - https://jamanetwork.com/journals/jamaophthalmology/fullarticle/10.1001/archophthalmol.2008.550 DB - PRIME DP - Unbound Medicine ER -