E88.4 – Mitochondrial metabolism disorders

ICD-10-CM 2027 diagnosis code · Metabolic disorders

Non-billable header

ICD-10-CM code E88.4

Code: E88.4 (claims format: E884)
Description: Mitochondrial metabolism disorders
Billable: No – use a more specific code below
Valid for: Dates of service October 1, 2026 – September 30, 2027 (FY2027)
Chapter: 4. Endocrine, nutritional and metabolic diseases (E00-E89)
Block: E70-E88 Metabolic disorders
Parent codes: E88

Billable codes under E88.4

E88.4 is a header code and cannot be used on claims. Choose the most specific code:

E88.40 – Mitochondrial metabolism disorder, unspecifiedBillable
E88.41 – MELAS syndromeBillable
E88.42 – MERRF syndromeBillable
E88.43 – Disorders of mitochondrial tRNA synthetasesBillable
E88.49 – Other mitochondrial metabolism disordersBillable

Notes for E88.4

Excludes1 (not coded here):

disorders of pyruvate metabolism (E74.4)
Kearns-Sayre syndrome (H49.81)
Leber's disease (H47.22)
Leigh's encephalopathy (G31.82)
Mitochondrial myopathy, NEC (G71.3)
Reye's syndrome (G93.7)

Notes inherited from parent codes

Instructional notes at a category or block level apply to every code below it.

E88 – Other and unspecified metabolic disorders

Excludes1 (not coded here):

histiocytosis X (chronic) (C96.6)

Use additional code:

codes for associated conditions

Block E70-E88

Excludes1 (not coded here):

androgen insensitivity syndrome (E34.5-)
congenital adrenal hyperplasia (E25.0)
hemolytic anemias attributable to enzyme disorders (D55.-)
Marfan syndrome (Q87.4-)
5-alpha-reductase deficiency (E29.1)

Excludes2 (not included here):

Ehlers-Danlos syndromes (Q79.6-)

Related codes in E88

Frequently asked questions

What is ICD-10 code E88.4?

E88.4 is the ICD-10-CM code for mitochondrial metabolism disorders, in the block E70-E88 (Metabolic disorders).

Is E88.4 a billable code?

No. E88.4 is a header code with more specific child codes; report one of the billable codes listed above.

What category does E88.4 belong to?

It belongs to category E88 – Other and unspecified metabolic disorders.

Source: CDC/NCHS ICD-10-CM FY2027 code files, tabular list and index. Reference only – code assignment must follow the official ICD-10-CM guidelines and documentation in the medical record.