Optical Coherence Tomography of Animal Models of Retinitis Pigmentosa: From Animal Studies to Clinical Applications.

PURPOSE

The aim of this study was to understand the relationship between the findings of spectral-domain optical coherence tomography (SD-OCT) of previously reported animal models of retinitis pigmentosa (RP) associated with known genetic mutations and their background structural and functional changes.

METHODS

We reviewed previous publications reporting the SD-OCT findings of animal models of RP and summarized the characteristic findings of SD-OCT in nine different animal models ( , P23H, S334ter, , , rp12, (rd1 and rd10), and ) of human RP.

RESULTS

Despite the various abnormal structural changes found in these different animal models, progressive thinning of the outer nuclear layer (ONL) and hyperreflective change in the inner and outer segment (IS-OS) layers of the photoreceptors were commonly observed on SD-OCT. In the rapidly progressive severe photoreceptor degeneration seen in rd10 and mice, the ONL appeared hyperreflective. Electroretinography revealed various degrees of disease severity in these animal models. : SD-OCT is sensitive enough to detect even mild changes in the photoreceptor OS. Conversely, SD-OCT cannot qualitatively differentiate the pathologic and functional differences in the photoreceptors associated with different genetic abnormalities, with the exception of the rapid progression of severe forms of photoreceptor degeneration. These findings can be of value to understand better the clinical findings and the heterogeneous degenerative processes in patients with RP.