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Topcon 3D OCT SD Similar comparisons in the left eye. The apparent irregular thickening and hyper-reflective ELM on the OCT is perhaps due to degeneration of adjacent layers with cellular debris accumulating on the ELM. The ELM, thought to be formed by the footplates of the Muller cells, is structural in
Topcon 3D OCT OS Comparison of the 3 siblings with color fundus photos, AF images and SD OCT in the right eye. In contrast to their brother above who has a thin macula and loss of outer retinal structures in the macula, the two sisters have all layers preserved but an abnormally thickened
Case 1: Topcon 3D OCT OS Similar findings are revealed in the left eye of the 4 yo, both in grayscale and in color.
Case 3 (4 yo daughter): Topcon 3D OCT OD With parental consent, chocolate was used to bribe the 4 yo. Cooperation improved for the OCT images. As revealed in her 5 yo sister, the PIL is somewhat thin and ELM is thickened, irregular and hyper-reflective here in the right eye.
Case 3 (4 yo daughter): Fundus Photography KEY: Color Optomap Auto Fluorescence No marked abnormality is obvious in the color fundus photos or in the AF images in the 4 yo daughter.
Case 3 (4 yo daughter): Optomap FAF Image OD and OS The AF images fail to reveal any obvious abnormality in the 4 yo daughter. Cooperation was poor.
Case 3 (4 yo daughter): Topcon Fundus Photos OD and OS Case 3(4 yo daughter): Above are the fundus photos from the 4 year old daughter. Best corrected VA 20/25+ in each eye. As previously noted, the ILM reflections are normal in youngsters and are nearly always found in both eyes.
Case 2 (5 yo daughter) : Topcon 3D OCT OS The ELM is abnormal in both grey scale and in color.
Case2(5 daughter): Topcon 3D OCT OD In contrast to her brother, the 5 yo sister has all retinal layers, although the PIL is somewhat attenuated. Remarkedly, the external limiting membrane (ELM) is thickened, irregular and grossly hyper-reflective. In normals, the ELM is very subtle and perhaps a fifth to a tenth as thick and
Case 2 (5 yo daughter): Fundus Photography KEY: Color Optomap Auto Fluorescence Although the fundus photos appear normal, the AF images are not. Symmetry of the AF images suggest an inherited retinal degeneration.
