Dystrophies primarily w the retinal pigment epithelium

This code signifies a group of inherited or acquired disorders characterized by progressive degeneration of the retinal pigment epithelium (RPE), the layer of cells supporting the photoreceptors in the retina. This RPE dysfunction leads to impaired visual function, often manifesting as central or peripheral vision loss.

Use this code for diagnoses such as Best's vitelliform macular dystrophy, adult-onset foveomacular vitelliform dystrophy, or other specific RPE dystrophies. Documentation should clearly state the diagnosis of an RPE dystrophy, often supported by ophthalmoscopy, electroretinography (ERG), or optical coherence tomography (OCT) findings.

• Differentiate from other retinal dystrophies not primarily involving the RPE, such as those affecting photoreceptors directly.
• Ensure the documentation specifies "dystrophy" and not just "degeneration" if a more specific dystrophy is identified.
• Consider laterality if specified in the clinical documentation (e.g., H35.541 for right eye, H35.542 for left eye, H35.543 for bilateral).