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Abnormal foveal morphology in ocular albinism imaged with spectral-domain optical coherence tomography.

Publication ,  Journal Article
Chong, GT; Farsiu, S; Freedman, SF; Sarin, N; Koreishi, AF; Izatt, JA; Toth, CA
Published in: Arch Ophthalmol
January 2009

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.

Duke Scholars

Published In

Arch Ophthalmol

DOI

EISSN

1538-3601

Publication Date

January 2009

Volume

127

Issue

1

Start / End Page

37 / 44

Location

United States

Related Subject Headings

  • Tomography, Optical Coherence
  • Ophthalmology & Optometry
  • Nystagmus, Pathologic
  • Male
  • Humans
  • Hermanski-Pudlak Syndrome
  • Fovea Centralis
  • Fourier Analysis
  • Female
  • Child, Preschool
 

Citation

APA
Chicago
ICMJE
MLA
NLM
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. Arch Ophthalmol, 127(1), 37–44. https://doi.org/10.1001/archophthalmol.2008.550
Chong, Gabriel T., Sina Farsiu, Sharon F. Freedman, Neeru Sarin, Anjum F. Koreishi, Joseph A. Izatt, and Cynthia A. Toth. “Abnormal foveal morphology in ocular albinism imaged with spectral-domain optical coherence tomography.Arch Ophthalmol 127, no. 1 (January 2009): 37–44. https://doi.org/10.1001/archophthalmol.2008.550.
Chong GT, Farsiu S, Freedman SF, Sarin N, Koreishi AF, Izatt JA, et al. Abnormal foveal morphology in ocular albinism imaged with spectral-domain optical coherence tomography. Arch Ophthalmol. 2009 Jan;127(1):37–44.
Chong, Gabriel T., et al. “Abnormal foveal morphology in ocular albinism imaged with spectral-domain optical coherence tomography.Arch Ophthalmol, vol. 127, no. 1, Jan. 2009, pp. 37–44. Pubmed, doi:10.1001/archophthalmol.2008.550.
Chong GT, Farsiu S, Freedman SF, Sarin N, Koreishi AF, Izatt JA, Toth CA. Abnormal foveal morphology in ocular albinism imaged with spectral-domain optical coherence tomography. Arch Ophthalmol. 2009 Jan;127(1):37–44.

Published In

Arch Ophthalmol

DOI

EISSN

1538-3601

Publication Date

January 2009

Volume

127

Issue

1

Start / End Page

37 / 44

Location

United States

Related Subject Headings

  • Tomography, Optical Coherence
  • Ophthalmology & Optometry
  • Nystagmus, Pathologic
  • Male
  • Humans
  • Hermanski-Pudlak Syndrome
  • Fovea Centralis
  • Fourier Analysis
  • Female
  • Child, Preschool