ICD-10 Code E76.22 – Sanfilippo mucopolysaccharidoses (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E76.22 – Sanfilippo mucopolysaccharidoses
What it is
E76.22 identifies Sanfilippo mucopolysaccharidoses, a group of inherited lysosomal storage disorders. These conditions cause abnormal buildup of heparan sulfate, leading to progressive neurologic disease.
Clinical signs
Clinical features vary; refer to documentation. Common findings include developmental delay, behavioral problems, progressive cognitive decline, and speech impairment, with possible coarse facial features or organ enlargement.
When to use this code
Use this code when the clinician documents Sanfilippo syndrome, mucopolysaccharidosis type III, or a specific subtype included under that diagnosis. It is appropriate for confirmed hereditary disease and for encounters focused on evaluation or management of the disorder. Check documentation if the record names a different mucopolysaccharidosis type.
Do not use for
Do not use this code for other mucopolysaccharidoses, such as Hurler or Hunter syndrome, unless Sanfilippo disease is specifically documented. Do not assign it for nonspecific developmental delay without a confirmed diagnosis.
Coding tip
Look for the exact syndrome name or MPS III wording in the chart before coding.