Abstract
Purpose:
To analyze the morphometric retinal and choroidal changes in cases of Alport syndrome by optical coherence tomography (OCT).
Methods:
Patients with retinal changes secondary to Alport syndrome were included. A comprehensive ophthalmologic evaluation was performed including visual acuity (VA) and axial length (AL) measurement. The whole macular area was scanned by swept source OCT. The automated segmentation performed by the software was used to analyze the thickness of the retina and the choroid displayed in an ETDRS grid; and also the thickness of the ganglion cell layer (GCL), and the ganglion cell complex layer (GCC), both of them displayed in a 6 sector grid. All these measurements were compared to an age, gender and AL matched control group.
Results:
Six eyes of three patients (2 men and 1 woman; mean age: 34.88) diagnosed of Alport syndrome were included; 18 eyes of 9 patients (6 men and 3 woman; mean age: 34.33 (p=0.893) were used as control eyes. The mean VA was 0.41+/- 0.17, and the mean AL was 24.11 +/-0.47 mm (control group: 23.75 +/-0.86 mm; p=0.243). The mean retinal foveal thickness was 209+/-47.38 microns (μm) (control group: 239.67 +/-17.83 μm; p=0.151) and the mean macular retinal thickness was 255.96 +/-14.97 μm (control group: 283.76 +/-80.89 μm; p=0.001). The mean subfoveal choroidal thickness was 294.57 +/-36.24 μm (control group: 345.72 +/-80.89 μm; p=0.033) and the mean choroidal retinal thickness was 291.70+/-46.06 μm (control group: 324.32+/-66.29 μm; p=0.142). The mean thickness of the GC layer was 64.11 +/-3.96 μm (control group: 73.16 +/-3.94 μm; p=0.001); the mean GCC layer thickness was 101.58 +/-7.19 μm (control group: 109.58 +/-8.36 μm; p=0.015). The analysis by quadrants showed that the temporal macular area was the most affected showing significant differences in the thickness of the retina, the GC layer and the GCC (p=0.001).
Conclusions:
In our study patients with Alport syndrome showed a significant thinning in the retinal thickness, affecting predominantly the temporal area of the GCL and GCC thickness compared with a control group. We evidenced a significant thinning in the foveal choroidal thickness whereas the mean macular choroidal thickness was not affected.
Keywords: 531 ganglion cells •
696 retinal degenerations: hereditary •
550 imaging/image analysis: clinical