Temporal retinal thinning and increased foveal avascular zone blood vessel density in alport syndrome: a case report.

Multifocal electroretinogram, Alport syndrome, optical coherence tomography, optical coherence tomography angiography, temporal retinal thinning Summary table of the most common ocular findings in X-linked Alport syndrome and reported percentage of cases that involve those ocular findings (total vs. males vs. females).[1],[5],[6],[9] HT <table><thead><tr><td>Ocular findings in X-linked Alport syndrome</td><td>% of total cases</td><td>% Males</td><td>% Females</td></tr></thead><tbody><tr><td>Temporal macular thinning</td><td>81%</td><td>89-95%</td><td>67-75%</td></tr><tr><td>Dot-and-fleck retinopathy (central & peripheral)</td><td>13-70%</td><td>60-73%</td><td>27-45%</td></tr><tr><td>Lozenge sign/dull macular reflex</td><td>19%</td><td>47%</td><td>18%</td></tr><tr><td>Lenticonus</td><td>2-50%</td><td>37%</td><td>0%/Rare</td></tr></tbody></table> ht Additional diagnostic testing can include electroretinography (ERG), OCT/OCT-A, and fundus photography/autofluorescence.[2],[[7], [9]] ERG testing (i.e. full-field ERG, multifocal ERG) is typically normal in Alport syndrome, but can be abnormal also.[1],[10] Our patient showed a mildly depressed multifocal ERG signal temporally in the patient's left eye consistent with his temporal retinal thinning. [Extracted from the article]