Early Detection of Stargardt Disease with OCT and AF – Page 12 of 30

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 reflective as the PIL. In this eye, the ELM is clearly thicker and more reflective than the PIL. The abnormal ELM is most marked in the macula. A normal OCT is included below for comparison.