The Henle Fiber Layer in Albinism

Comparison to Normal and Relationship to Outer Nuclear Layer Thickness and Foveal Cone Density

Daniel J. Lee, Erica N. Woertz, Alexis Visotcky, Melissa A. Wilk, Heather Heitkotter, Rachel E. Linderman, Sergey Tarima, C. Gail Summers, Brian P. Brooks, Murray H. Brilliant, Bhavna J. Antony, Brandon Lujan, Joseph Carroll

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Purpose: Directional optical coherence tomography (D-OCT) allows the visualization of the Henle fiber layer (HFL) in vivo. Here, we used D-OCT to characterize the HFL and outer nuclear layer (ONL) in albinism and examine the relationship between true foveal ONL and peak cone density. Methods: Horizontal D-OCT B-scans were acquired, registered, and averaged for 12 subjects with oculocutaneous albinism and 26 control subjects. Averaged images were manually segmented to extract HFL and ONL thickness. Adaptive optics scanning light ophthalmoscopy was used to acquire images of the foveal cone mosaic in 10 subjects with albinism, from which peak cone density was assessed. Results: Across the foveal region, the HFL topography was different between subjects with albinism and normal controls. In particular, foveal HFL thickness was thicker in albinism than in normal controls (P < 0.0001), whereas foveal ONL thickness was thinner in albinism than in normal controls (P < 0.0001). The total HFL and ONL thickness was not significantly different between albinism and controls (P = 0.3169). Foveal ONL thickness was positively correlated with peak cone density in subjects with albinism (r = 0.8061, P = 0.0072). Conclusions: Foveal HFL and ONL topography are significantly altered in albinism relative to normal controls. Our data suggest that increased foveal cone packing drives the formation of Henle fibers, more so than the lateral displacement of inner retinal neurons (which is reduced in albinism). The ability to quantify foveal ONL and HFL may help further stratify grading schemes used to assess foveal hypoplasia.

Original languageEnglish (US)
Pages (from-to)5336-5348
Number of pages13
JournalInvestigative ophthalmology & visual science
Volume59
Issue number13
DOIs
StatePublished - Nov 1 2018

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Albinism
Optical Coherence Tomography
Oculocutaneous Albinism
Retinal Neurons
Ophthalmoscopy

ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems
  • Cellular and Molecular Neuroscience

Cite this

The Henle Fiber Layer in Albinism : Comparison to Normal and Relationship to Outer Nuclear Layer Thickness and Foveal Cone Density. / Lee, Daniel J.; Woertz, Erica N.; Visotcky, Alexis; Wilk, Melissa A.; Heitkotter, Heather; Linderman, Rachel E.; Tarima, Sergey; Summers, C. Gail; Brooks, Brian P.; Brilliant, Murray H.; Antony, Bhavna J.; Lujan, Brandon; Carroll, Joseph.

In: Investigative ophthalmology & visual science, Vol. 59, No. 13, 01.11.2018, p. 5336-5348.

Research output: Contribution to journalArticle

Lee, DJ, Woertz, EN, Visotcky, A, Wilk, MA, Heitkotter, H, Linderman, RE, Tarima, S, Summers, CG, Brooks, BP, Brilliant, MH, Antony, BJ, Lujan, B & Carroll, J 2018, 'The Henle Fiber Layer in Albinism: Comparison to Normal and Relationship to Outer Nuclear Layer Thickness and Foveal Cone Density', Investigative ophthalmology & visual science, vol. 59, no. 13, pp. 5336-5348. https://doi.org/10.1167/iovs.18-24145
Lee, Daniel J. ; Woertz, Erica N. ; Visotcky, Alexis ; Wilk, Melissa A. ; Heitkotter, Heather ; Linderman, Rachel E. ; Tarima, Sergey ; Summers, C. Gail ; Brooks, Brian P. ; Brilliant, Murray H. ; Antony, Bhavna J. ; Lujan, Brandon ; Carroll, Joseph. / The Henle Fiber Layer in Albinism : Comparison to Normal and Relationship to Outer Nuclear Layer Thickness and Foveal Cone Density. In: Investigative ophthalmology & visual science. 2018 ; Vol. 59, No. 13. pp. 5336-5348.
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abstract = "Purpose: Directional optical coherence tomography (D-OCT) allows the visualization of the Henle fiber layer (HFL) in vivo. Here, we used D-OCT to characterize the HFL and outer nuclear layer (ONL) in albinism and examine the relationship between true foveal ONL and peak cone density. Methods: Horizontal D-OCT B-scans were acquired, registered, and averaged for 12 subjects with oculocutaneous albinism and 26 control subjects. Averaged images were manually segmented to extract HFL and ONL thickness. Adaptive optics scanning light ophthalmoscopy was used to acquire images of the foveal cone mosaic in 10 subjects with albinism, from which peak cone density was assessed. Results: Across the foveal region, the HFL topography was different between subjects with albinism and normal controls. In particular, foveal HFL thickness was thicker in albinism than in normal controls (P < 0.0001), whereas foveal ONL thickness was thinner in albinism than in normal controls (P < 0.0001). The total HFL and ONL thickness was not significantly different between albinism and controls (P = 0.3169). Foveal ONL thickness was positively correlated with peak cone density in subjects with albinism (r = 0.8061, P = 0.0072). Conclusions: Foveal HFL and ONL topography are significantly altered in albinism relative to normal controls. Our data suggest that increased foveal cone packing drives the formation of Henle fibers, more so than the lateral displacement of inner retinal neurons (which is reduced in albinism). The ability to quantify foveal ONL and HFL may help further stratify grading schemes used to assess foveal hypoplasia.",
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AU - Visotcky, Alexis

AU - Wilk, Melissa A.

AU - Heitkotter, Heather

AU - Linderman, Rachel E.

AU - Tarima, Sergey

AU - Summers, C. Gail

AU - Brooks, Brian P.

AU - Brilliant, Murray H.

AU - Antony, Bhavna J.

AU - Lujan, Brandon

AU - Carroll, Joseph

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