E88.49Other mitochondrial metabolism disorders

E88.49 is the ICD-10-CM diagnosis code for other mitochondrial metabolism disorders. It belongs to category E88 (other and unspecified metabolic disorders), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within E88.4, choose E88.49 (Other mitochondrial metabolism disorders) only when documentation doesn't support a more specific option: mitochondrial metabolism disorder, unspecified (E88.40), MELAS syndrome (E88.41), MERRF syndrome (E88.42) and disorders of mitochondrial tRNA synthetases (E88.43).

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.

In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Before ICD-10, this condition was coded in ICD-9-CM as 277.87 (disorders of mitochondrial metabolism).