Comparative data on SD-OCT for the retinal nerve fiber layer and retinal macular thickness in a large cohort with Marfan syndrome

Wan Wan Xu, Sudhi P. Kurup, Amani A. Fawzi, Mary K. Durbin, Irene H. Maumenee, Marilyn B. Mets

Research output: Contribution to journalArticle

Abstract

Purpose: To report the distribution of macular and optic nerve topography in the eyes of individuals with Marfan syndrome aged 8–56 years using spectral domain optical coherence tomography (SD-OCT). Methods: Thirty-three patients with Marfan syndrome underwent a full eye examination including slit-lamp biomicroscopy, indirect ophthalmoscopy, and axial length measurement; and SD-OCT measurements of the retinal nerve fiber layer (RNFL) and macular thickness. Results: For patients between the ages of 8 and 12 years, the average RNFL thickness is 98 ± 9 μm, the vertical cup to disc (C:D) ratio is 0.50 ± 0.10, the central subfield thickness (CST) is 274 ± 38 μm, and the macular volume is 10.3 ± 0.6 mm3. For patients between the ages of 13 and 17 years, the average RNFL is 86 ± 16 μm, the vertical C:D ratio is 0.35 ± 0.20, the CST is 259 ± 15 μm, and the macular volume is 10.1 ± 0.5 mm3. For patients 18 years or older, the average RNFL is 89 ± 12 μm, the vertical C:D ratio is 0.46 ± 0.18, the CST is 262 ± 20 μm, and the macular volume is 10.2 ± 0.4 mm3. When the average RNFL data are compared to a normative, age-adjusted database, 6 of 33 (18%) were thinner than the 5% limit. Conclusion: This study reports the distribution of SD-OCT data for patients with Marfan syndrome. Compared to a normative database, 18% of eyes with Marfan syndrome had RNFL thickness < 5% of the population.

Original languageEnglish (US)
Pages (from-to)34-38
Number of pages5
JournalOphthalmic Genetics
Volume38
Issue number1
DOIs
StatePublished - Jan 2 2017
Externally publishedYes

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Marfan Syndrome
Optical Coherence Tomography
Nerve Fibers
Databases
Ophthalmoscopy
Optic Nerve
Population

Keywords

  • Connective tissue disease
  • Marfan syndrome
  • OCT

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Ophthalmology
  • Genetics(clinical)

Cite this

Comparative data on SD-OCT for the retinal nerve fiber layer and retinal macular thickness in a large cohort with Marfan syndrome. / Xu, Wan Wan; Kurup, Sudhi P.; Fawzi, Amani A.; Durbin, Mary K.; Maumenee, Irene H.; Mets, Marilyn B.

In: Ophthalmic Genetics, Vol. 38, No. 1, 02.01.2017, p. 34-38.

Research output: Contribution to journalArticle

Xu, Wan Wan ; Kurup, Sudhi P. ; Fawzi, Amani A. ; Durbin, Mary K. ; Maumenee, Irene H. ; Mets, Marilyn B. / Comparative data on SD-OCT for the retinal nerve fiber layer and retinal macular thickness in a large cohort with Marfan syndrome. In: Ophthalmic Genetics. 2017 ; Vol. 38, No. 1. pp. 34-38.
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abstract = "Purpose: To report the distribution of macular and optic nerve topography in the eyes of individuals with Marfan syndrome aged 8–56 years using spectral domain optical coherence tomography (SD-OCT). Methods: Thirty-three patients with Marfan syndrome underwent a full eye examination including slit-lamp biomicroscopy, indirect ophthalmoscopy, and axial length measurement; and SD-OCT measurements of the retinal nerve fiber layer (RNFL) and macular thickness. Results: For patients between the ages of 8 and 12 years, the average RNFL thickness is 98 ± 9 μm, the vertical cup to disc (C:D) ratio is 0.50 ± 0.10, the central subfield thickness (CST) is 274 ± 38 μm, and the macular volume is 10.3 ± 0.6 mm3. For patients between the ages of 13 and 17 years, the average RNFL is 86 ± 16 μm, the vertical C:D ratio is 0.35 ± 0.20, the CST is 259 ± 15 μm, and the macular volume is 10.1 ± 0.5 mm3. For patients 18 years or older, the average RNFL is 89 ± 12 μm, the vertical C:D ratio is 0.46 ± 0.18, the CST is 262 ± 20 μm, and the macular volume is 10.2 ± 0.4 mm3. When the average RNFL data are compared to a normative, age-adjusted database, 6 of 33 (18{\%}) were thinner than the 5{\%} limit. Conclusion: This study reports the distribution of SD-OCT data for patients with Marfan syndrome. Compared to a normative database, 18{\%} of eyes with Marfan syndrome had RNFL thickness < 5{\%} of the population.",
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AU - Maumenee, Irene H.

AU - Mets, Marilyn B.

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N2 - Purpose: To report the distribution of macular and optic nerve topography in the eyes of individuals with Marfan syndrome aged 8–56 years using spectral domain optical coherence tomography (SD-OCT). Methods: Thirty-three patients with Marfan syndrome underwent a full eye examination including slit-lamp biomicroscopy, indirect ophthalmoscopy, and axial length measurement; and SD-OCT measurements of the retinal nerve fiber layer (RNFL) and macular thickness. Results: For patients between the ages of 8 and 12 years, the average RNFL thickness is 98 ± 9 μm, the vertical cup to disc (C:D) ratio is 0.50 ± 0.10, the central subfield thickness (CST) is 274 ± 38 μm, and the macular volume is 10.3 ± 0.6 mm3. For patients between the ages of 13 and 17 years, the average RNFL is 86 ± 16 μm, the vertical C:D ratio is 0.35 ± 0.20, the CST is 259 ± 15 μm, and the macular volume is 10.1 ± 0.5 mm3. For patients 18 years or older, the average RNFL is 89 ± 12 μm, the vertical C:D ratio is 0.46 ± 0.18, the CST is 262 ± 20 μm, and the macular volume is 10.2 ± 0.4 mm3. When the average RNFL data are compared to a normative, age-adjusted database, 6 of 33 (18%) were thinner than the 5% limit. Conclusion: This study reports the distribution of SD-OCT data for patients with Marfan syndrome. Compared to a normative database, 18% of eyes with Marfan syndrome had RNFL thickness < 5% of the population.

AB - Purpose: To report the distribution of macular and optic nerve topography in the eyes of individuals with Marfan syndrome aged 8–56 years using spectral domain optical coherence tomography (SD-OCT). Methods: Thirty-three patients with Marfan syndrome underwent a full eye examination including slit-lamp biomicroscopy, indirect ophthalmoscopy, and axial length measurement; and SD-OCT measurements of the retinal nerve fiber layer (RNFL) and macular thickness. Results: For patients between the ages of 8 and 12 years, the average RNFL thickness is 98 ± 9 μm, the vertical cup to disc (C:D) ratio is 0.50 ± 0.10, the central subfield thickness (CST) is 274 ± 38 μm, and the macular volume is 10.3 ± 0.6 mm3. For patients between the ages of 13 and 17 years, the average RNFL is 86 ± 16 μm, the vertical C:D ratio is 0.35 ± 0.20, the CST is 259 ± 15 μm, and the macular volume is 10.1 ± 0.5 mm3. For patients 18 years or older, the average RNFL is 89 ± 12 μm, the vertical C:D ratio is 0.46 ± 0.18, the CST is 262 ± 20 μm, and the macular volume is 10.2 ± 0.4 mm3. When the average RNFL data are compared to a normative, age-adjusted database, 6 of 33 (18%) were thinner than the 5% limit. Conclusion: This study reports the distribution of SD-OCT data for patients with Marfan syndrome. Compared to a normative database, 18% of eyes with Marfan syndrome had RNFL thickness < 5% of the population.

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