ICD-10 Code E75.28 – Canavan disease (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.28 – Canavan disease
What it is
Canavan disease is an inherited metabolic disorder that affects the brain’s white matter. Use this code for documented Canavan disease, also called spongiform leukodystrophy.
Clinical signs
Clinical features vary; refer to documentation. Common findings include developmental delay, hypotonia, macrocephaly, and progressive neurologic impairment in affected children.
When to use this code
Use E75.28 when the provider documents Canavan disease as the confirmed diagnosis. It may appear in neurology, genetics, or pediatric records after clinical evaluation, imaging, or genetic testing supports the condition.
Code it for active disease management, specialist assessment, or a history that is still clinically relevant in the encounter. Check documentation if the note only mentions family history or carrier status.
Do not use for
Do not use this code for nonspecific developmental delay, leukodystrophy without a named diagnosis, or family history alone. Check documentation if the chart does not clearly confirm Canavan disease.
Coding tip
Code E75.28 only when the record explicitly names Canavan disease; otherwise query for the specific diagnosis.