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Foveal Hypoplasia in Patients with Stickler Syndrome

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Presentation on theme: "Foveal Hypoplasia in Patients with Stickler Syndrome"— Presentation transcript:

1 Foveal Hypoplasia in Patients with Stickler Syndrome
Itsuka Matsushita, MD, Tatsuo Nagata, MD, PhD, Takaaki Hayashi, MD, PhD, Kenichi Kimoto, MD, PhD, Toshiaki Kubota, MD, PhD, Masahito Ohji, MD, PhD, Shunji Kusaka, MD, PhD, Hiroyuki Kondo, MD, PhD  Ophthalmology  Volume 124, Issue 6, Pages (June 2017) DOI: /j.ophtha Copyright © 2017 American Academy of Ophthalmology Terms and Conditions

2 Figure 1 Comparisons of the cross-sectional optical coherence tomography (OCT) images of (A) a normal subject and (B) a patient with Stickler syndrome with foveal hypoplasia. A, An OCT image showing a normal fovea with normal architecture of the foveal layers. The inner retinal layer (IRL) extends from the inner aspect of the internal limiting membrane to the outer border of the inner nuclear layer. The outer retinal layer (ORL) extends from the outer aspect of the outer plexiform layer (OPL) to the inner border of the retinal pigment epithelium (RPE). GCL = ganglion cell layer; INL = inner nuclear layer; IPL = inner plexiform layer. B, An OCT image showing foveal hypoplasia in an eye with Stickler syndrome (patient 7). The foveal IRL (fIRL, a) and parafoveal IRL (pIRL, b) are the thicknesses of the IRL at the central fovea and at 1000 μm from the foveal center. The foveal ORL (fORL, c) and parafoveal ORL (pORL, d) are the thicknesses of the ORL at the central fovea and at 1000 μm nasal from the foveal center. The ratio of the thicknesses at the fovea and parafovea was calculated. The ratio was fIRL/pIRL for the IRL, and the ratio was fORL/pORL for the ORL. Note that the image shows a foveal budge at the foveal center. Henle's fiber layer can be seen as a hyperreflective band within the thickened OPL in the temporal parafoveal area (asterisk). Ophthalmology  , DOI: ( /j.ophtha ) Copyright © 2017 American Academy of Ophthalmology Terms and Conditions

3 Figure 2 Optical coherence tomography (OCT) images of eyes with Stickler syndrome and a normal eye. The left, middle, and right columns are the cross-sectional OCT image, OCT angiography (OCTA) images of the superficial capillary plexus, and the OCTA en face images at the level of the internal limiting membrane, respectively. A, B, C, D, and E are from patient 23 (left eye), patient 8 (right eye), patient 21 (right eye), patient 21 (left eye), and normal control (unaffected sibling of patient 4), respectively. The fovea versus parafovea calculated for the inner retinal layers (fIRL/pIRL) is the ratio of the thicknesses at the fovea relative to that at the parafovea for the inner retinal layer. A–D, Optical coherence tomography images showing mild to strong persistence of the inner retinal layers, and OCTA showing small or no foveal avascular zone (FAZ). A, Foveal hypoplasia of grade 2, and B–D, foveal hypoplasia grade 1 based on the grading of foveal hypoplasia reported by Thomas et al.14 E, An OCT image showing a normal macula and OCTA showing a normal FAZ. Right panels: en face OCT images of all eyes (A–E) showing absence of epiretinal membrane-like folds. FP-IRL = foveal versus parafoveal inner retinal layer. Ophthalmology  , DOI: ( /j.ophtha ) Copyright © 2017 American Academy of Ophthalmology Terms and Conditions

4 Figure 3 Correlation between the visual acuity in logarithm of the minimum angle of resolution (logMAR) units and ratios of thickness at the fovea versus parafovea calculated for the inner retinal layers (fIRL/pIRL ratio). The correlation between the visual acuity and fIRL/pIRL ratio was not significant. R=−0.07; P = 0.67, by Spearman's rank correlation coefficient. Ophthalmology  , DOI: ( /j.ophtha ) Copyright © 2017 American Academy of Ophthalmology Terms and Conditions

5 Figure 4 Correlation between the refractive error and the fovea versus parafovea calculated for the inner retinal layers (fIRL/pIRL) ratio. The correlation was not significant. R=−0.14, P = 0.41, by Spearman's rank correlation coefficient. D = diopters. Ophthalmology  , DOI: ( /j.ophtha ) Copyright © 2017 American Academy of Ophthalmology Terms and Conditions


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