ICD-10-CM 2027
ICD-10 code for mitochondrial metabolism disorder
Mitochondrial metabolism disorder, unspecified
From the official ICD-10-CM alphabetic index entry “Disorder (of) › mitochondrial metabolism”. Page updated September 29, 2026.
About coding mitochondrial metabolism disorder
The ICD-10-CM code for mitochondrial metabolism disorder is E88.40 (Mitochondrial metabolism disorder, unspecified).
Within E88.4, choose E88.40 (Mitochondrial metabolism disorder, unspecified) only when documentation doesn't support a more specific option: MELAS syndrome (E88.41), MERRF syndrome (E88.42), disorders of mitochondrial tRNA synthetases (E88.43) and other mitochondrial metabolism disorders (E88.49).
Use an additional code for: codes for associated conditions.
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.
Common questions about mitochondrial metabolism disorder ICD-10 codes
What is the ICD-10 code for mitochondrial metabolism disorder?
- E88.40 — Mitochondrial metabolism disorder, unspecified.
Is E88.40 billable?
- Yes. E88.40 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E88.40 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E88.40 as a principal diagnosis.
Is E88.40 a CC or MCC?
- E88.40 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.