2027 ICD-10-CM Diagnosis Code E75.28Canavan disease
ICD-10-CM Codes›E00–E89›E70-E88›E75
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- Chronic Condition
E75.28 is a billable ICD-10-CM diagnosis code for canavan disease. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 56 through 57. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 24 closely related codes. 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.
E75.28 no longer risk-adjusts for Medicare Advantage: it mapped to HCC 52 under the retired CMS-HCC V24 model through payment year 2025 but maps to no category in the live V28 model. It still risk-adjusts in the ESRD (V21) category 52, ESRD (V24) category 52, and RxHCC Part D (V08) category 41 for payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E75.28 no longer risk-adjusts for Medicare Advantage: it maps to no payment category in the live CMS-HCC V28 model, although it still risk-adjusts in the other CMS models shown below.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E75.28: its own notes plus those printed at E75.2, E75, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
- adrenoleukodystrophy [Addison-Schilder] (E71.528)
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E75.28, its category, or a range that includes it.
Excludes1 2
These codes carry an Excludes1 note naming E75.28: they are not reported together with it, unless the two conditions are unrelated.
Code First 2
These codes say to code first a condition named in the note, and E75.28 is one of the conditions named.
Index to Diseases and InjuriesGuidance
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
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
Questions About E75.28Overview
What is the ICD-10 code for canavan disease?
The ICD-10-CM code for canavan disease is E75.28 (sometimes written as E7528). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
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, 2026 through September 30, 2027.
What MS-DRG does E75.28 group to?
When canavan disease is the principal diagnosis on an inpatient stay, it groups to MS-DRG 57, 56, with relative weights from 1.2835 to 2.2649 depending on complications. Higher weights mean higher Medicare reimbursement.
Is E75.28 a CC or MCC?
CMS lists E75.28 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 24 closely related codes in its exclusion list.
Can E75.28 be reported with E71.528?
Not as a rule. The Excludes1 note at E75.2, which applies to E75.28, lists "adrenoleukodystrophy [Addison-Schilder] (E71.528)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
Does E75.28 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage. E75.28 mapped to HCC 52 in the retired CMS-HCC V24 model, which last determined payment in 2025, but it maps to no category in the live V28 model; see all codes that no longer risk-adjust. It still risk-adjusts in the ESRD (V21) category 52 (Dementia Without Complication), ESRD (V24) category 52 (Dementia Without Complication), and RxHCC Part D (V08) category 41 (Lysosomal Storage Disorders).
