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HANDHELD SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FINDINGS OF X-LINKED RETINOSCHISIS IN EARLY CHILDHOOD.

Publication ,  Journal Article
Ling, KP; Mangalesh, S; Tran-Viet, D; Gunther, R; Toth, CA; Vajzovic, L
Published in: Retina
October 2020

BACKGROUND/PURPOSE: Using handheld spectral domain optical coherence tomography (SDOCT) imaging to investigate in vivo microanatomic retinal changes and their progression over time in young children with juvenile X-linked retinoschisis (XLRS). METHODS: This retrospective analysis was of handheld SD OCT images obtained under a prospective research protocol in children who had established XLRS diagnosis based on genetic testing or clinical history. Three OCT graders performed standardized qualitative and quantitative assessment of retinal volume scans, which were divided into foveal, parafoveal, and extrafoveal regions. Visual acuity data were obtained when possible. RESULTS: Spectral domain OCT images were available of both eyes in 8 pediatric patients with ages 7 months to 10 years. The schisis cavities involved inner nuclear layer in over 90% (15/16) of eyes in all 3 regions. Retinal nerve fiber and ganglion cell layer involvement was present only in the extrafoveal region in 63% (10/16) eyes and outer nuclear and plexiform layer in few others. In 7 children followed over 2 months to 15 months, the location of schisis remained consistent. Central foveal thickness decreased from the baseline to final available visit in 4/6 eyes. Ellipsoid zone disruption seemed to accompany lower visual acuity in 1/4 eyes. CONCLUSION: Early in life, the SD OCT findings in XLRS demonstrate differences in schisis location in fovea-parafoveal versus extrafoveal region, possible association between poor visual acuity and degree of ellipsoid zone disruption and decrease in central foveal thickness over time in this group. Furthermore, they illustrates that the pattern of XLRS in adults is already present in very young children, and unlike in older children and adults, those presenting with earlier disease may have a more aggressive course. Further studies in this early age group may provide more insights into treatment and prevention of progressive visual impairment in children with XLRS.

Duke Scholars

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Published In

Retina

DOI

EISSN

1539-2864

Publication Date

October 2020

Volume

40

Issue

10

Start / End Page

1996 / 2003

Location

United States

Related Subject Headings

  • Tomography, Optical Coherence
  • Retrospective Studies
  • Retinoschisis
  • Retinal Ganglion Cells
  • Ophthalmology & Optometry
  • Nerve Fibers
  • Male
  • Infant
  • Humans
  • Diagnostic Techniques, Ophthalmological
 

Citation

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MLA
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Ling, K. P., Mangalesh, S., Tran-Viet, D., Gunther, R., Toth, C. A., & Vajzovic, L. (2020). HANDHELD SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FINDINGS OF X-LINKED RETINOSCHISIS IN EARLY CHILDHOOD. Retina, 40(10), 1996–2003. https://doi.org/10.1097/IAE.0000000000002688
Ling, Kiet Phang, Shwetha Mangalesh, Du Tran-Viet, Randall Gunther, Cynthia A. Toth, and Lejla Vajzovic. “HANDHELD SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FINDINGS OF X-LINKED RETINOSCHISIS IN EARLY CHILDHOOD.Retina 40, no. 10 (October 2020): 1996–2003. https://doi.org/10.1097/IAE.0000000000002688.
Ling KP, Mangalesh S, Tran-Viet D, Gunther R, Toth CA, Vajzovic L. HANDHELD SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FINDINGS OF X-LINKED RETINOSCHISIS IN EARLY CHILDHOOD. Retina. 2020 Oct;40(10):1996–2003.
Ling, Kiet Phang, et al. “HANDHELD SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FINDINGS OF X-LINKED RETINOSCHISIS IN EARLY CHILDHOOD.Retina, vol. 40, no. 10, Oct. 2020, pp. 1996–2003. Pubmed, doi:10.1097/IAE.0000000000002688.
Ling KP, Mangalesh S, Tran-Viet D, Gunther R, Toth CA, Vajzovic L. HANDHELD SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FINDINGS OF X-LINKED RETINOSCHISIS IN EARLY CHILDHOOD. Retina. 2020 Oct;40(10):1996–2003.

Published In

Retina

DOI

EISSN

1539-2864

Publication Date

October 2020

Volume

40

Issue

10

Start / End Page

1996 / 2003

Location

United States

Related Subject Headings

  • Tomography, Optical Coherence
  • Retrospective Studies
  • Retinoschisis
  • Retinal Ganglion Cells
  • Ophthalmology & Optometry
  • Nerve Fibers
  • Male
  • Infant
  • Humans
  • Diagnostic Techniques, Ophthalmological