Structural changes in the retinal layers of patients with Steinert disease.

PURPOSE: The purpose of this study was to evaluate the different retinal layers of the posterior pole protocol and peripapillary areas in optical coherence tomography (OCT) to identify significant changes attributable to Steinert disease.

METHODS

We analyzed 128 eyes of healthy control subjects and 79 eyes of patients with Steinert disease. We performed the posterior pole and Nsite analytics peripapillary protocols on all subjects and compared thicknesses in the retinal nerve fiber layer (RNFL), ganglion cell layer (GCL), inner plexiform layer (IPL), inner nuclear layer (INL), outer plexiform layer (OPL), outer nuclear layer (ONL), retinal pigment epithelium (RPE), and complete retina (RETINA).

RESULTS

Patients with Steinert disease showed significant alterations in retinal layer thickness compared with age-matched controls. Overall, a generalized thickening of retinal layers was observed; significant thickening of the RNFL was also found in the temporal area of the optic nerve and in the papillomacular bundle, which comprises the retinal ganglion cell axons connecting the macula with the optic nerve.

CONCLUSIONS

Steinert disease is associated with retinal structural alterations in retinal layer structure, OCT may provide potential structural markers of retinal involvement in Steinert disease.