2026 ICD-10-CM Diagnosis Code E75.28Canavan disease

ICD-10-CM Codes›E00–E89›E70-E88›E75

ICD-10-CM E75.28
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E75.28 is a billable ICD-10-CM diagnosis code for canavan disease. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other nervous system disorders (often hereditary or degenerative) and Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E75.28
Billable Status
Yes — Valid for Submission
Code Describes
Canavan disease
Short Description
Canavan disease
Same as the full description in the CMS dataset.
Parent Code
E75.2
Other sphingolipidosis

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE75Disorders of sphingolipid metabolism and other lipid storage disorders
This CodeE75.28Canavan disease

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Canavan disease
    • Disease, diseased
      • Canavan

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR NVS006
Other nervous system disorders (often hereditary or degenerative)
Default principal diagnosis: inpatient No · outpatient No
CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Canavan Disease

    a rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy appearance. aspartoacylase deficiency leads to an accumulation of n-acetylaspartate in astrocytes. inheritance may be autosomal recessive or the illness may occur sporadically. this illness occurs more frequently in individuals of ashkenazic jewish descent. the neonatal form features the onset of hypotonia and lethargy at birth, rapidly progressing to coma and death. the infantile form features developmental delay, dyskinesias, hypotonia, spasticity, blindness, and megalencephaly. the juvenile form is characterized by ataxia; optic atrophy; and dementia. (from adams et al., principles of neurology, 6th ed, p944; am j med genet 1988 feb;29(2):463-71)

Code History & ChangesHistory

Replacement E75.28 replaces the following previously assigned code(s):

  • E75.29 - Other sphingolipidosis
FY 2024AddedAdded to the ICD-10-CM code setEffective October 1, 2023.
FY 2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E75.28Overview

Is E75.28 (Other sphingolipidosis) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report canavan disease on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.