ICD-10 code for Canavan disease

The ICD-10-CM code for Canavan disease is E75.28 (Canavan disease).

Within E75.2 (other sphingolipidosis), E75.28 is specifically for Canavan disease. Related codes cover Fabry (-Anderson) disease (E75.21), Gaucher disease (E75.22), Krabbe disease (E75.23), Niemann-Pick disease (E75.24), Metachromatic leukodystrophy (E75.25), Sulfatase deficiency (E75.26), Pelizaeus-Merzbacher disease (E75.27) and other sphingolipidosis (E75.29).

As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.

In MS-DRG v44.0, it is part of the grouping logic for DRG 056 (Degenerative Nervous System Disorders with MCC, relative weight 2.2655) and DRG 057 (Degenerative Nervous System Disorders without MCC, relative weight 1.2838), in MDC 01 (Diseases and Disorders of the Nervous System), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.