Skip to main content
Compare/E88.4 vs H49.81

E88.4 vs H49.81

E88.4 (Mitochondrial metabolism disorders) compared with H49.81 (Kearns-Sayre syndrome), from the official CMS tabular data. Do not report these codes together: the official tabular list marks them Excludes1.

Can these codes be reported together?

No — do not report together. E88.4 carries an Excludes1 note covering H49.81: “Kearns-Sayre syndrome (H49.81)”

Sole exception: when the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

Side by side

E88.4Mitochondrial metabolism disordersH49.81Kearns-Sayre syndrome
Billing statusNon-billable header

Report instead: E88.40, E88.41, E88.42, E88.43, E88.49

Non-billable header

Report instead: H49.811, H49.812, H49.813, H49.819

ClassificationE00-E89 — Endocrine, Nutritional and Metabolic Diseases (E00-E89)H00-H59 — Diseases of the Eye and Adnexa (H00-H59)
DefinitionMitochondrial metabolism disorders is a non-billable ICD-10-CM category code (E88.4).Kearns-Sayre syndrome is a non-billable ICD-10-CM category code (H49.81).
Includes
Progressive external ophthalmoplegia with pigmentary retinopathy
Use additional code
codes for associated conditions

Derived from the official CMS ICD-10-CM tabular data (FY2026). Not billing advice; verify sequencing rules for the encounter. All data sources