ICD-10 Code H35.54 – Dystrophies primarily involving the retinal pigment epithelium (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code H35.54 – Dystrophies primarily involving the retinal pigment epithelium
What it is
This code identifies inherited or degenerative retinal disorders that primarily affect the retinal pigment epithelium, the cell layer that supports the retina. Use it when the chart documents a dystrophy centered on this tissue.
Clinical signs
Findings may include progressive visual disturbance, abnormal fundus appearance, and retinal pigmentary changes. Clinical features vary; refer to documentation for the specific dystrophy named by the provider.
When to use this code
Use H35.54 when the provider documents a dystrophy primarily involving the retinal pigment epithelium, such as a retinal pigment epithelium dystrophy or a related inherited retinal disorder. Code the condition as described in the record and avoid substituting a broader retinal diagnosis unless documented.
Do not use for
Do not use this code for retinal conditions that primarily affect other layers, such as the photoreceptors or macula, unless the provider specifically links them to retinal pigment epithelium dystrophy. Check documentation when the diagnosis is unclear.
Coding tip
Match the code to the exact wording in the assessment and problem list, and query if the record only says “retinal dystrophy” without specifying retinal pigment epithelium involvement.