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But not all achromats have the "pathognomonic" sign Cirrus™ HD-OCT Horizontal Scan Images Note in Case 5 several of retinal layers are missing and the overall retinal thickness appears thin. Note in Case 6 the horizontal OCT sections appear essentially normal. Interestingly, Case 6 is the sister of the initial patient presented with
Note the sharply delineated rectangular hyporeflective space in the horizontal OCT sections. 6 of 8 achromats examined presented the "pathognomonic" OCT sign. (Note only 6 of the achromatopsia cases were presented in this issue).
Case 2: Cirrus™ HD-OCT Horizontal Scan Images OS The OCT section in the left eye also appear normal.
Case 6: Cirrus'™ HD-OCT Horizontal Scan Images OD A horizontal OCT section through the fovea reveals a retina that appears essentially normal. There may be loss of some tissue in the outer nuclear layer (see page 14). The PIL is intact under the fovea and no hyporefiective space is apparent.
Case 6: Zeiss FF450plus Autofluorescence Fundus Camera* Image of Left Eye * VISUPAC 481 ZK5 and Pike 145The characteristic dark oval zone is not apparent in the FAF image of the right eye.
Case 6: Zeiss FF450plus Autofluorescence Fundus Camera* Image of Right Eye * VISUPAC 481 ZK5 and Pike 145 The characteristic dark oval zone is not apparent in the FAF image of the right eye.
Case 6: Zeiss FF450plus Color Fundus Camera* Image of Left Eye * VISUPAC 481 ZK5 and Pike 145 Note there are no obvious abnormalities in the color fundus image of the left or right eye (see previous page).
Case 6: Zeiss FF450plus Color Fundus Camera* Image of Right Eye * VISUPAC 481 ZK5 and Pike 145Case 6: A 23-year-old female diagnosed with achromatopsia. BCVA measured 20/200 OU.
Case 5: Flash Electroretinogram (ERG) Results The flash electroretinogram (ERG) is the single best test of overall retina function. The patient had a normal scotopic, but flat photopic ERG as anticipated.
Case 5: Cirrus™ HD-OCT Horizontal Scan Images OS Although there has been no obvious progression in vision loss, the OCT pattern presents more like a cone dystrophy than achromatopsia. This case does not show the characteristic OCT pattern found in other achromats and could be a very slow progressing form of cone
