Coding and coverage · ICD-10-CM

E71 › E71.3

E71.31 Disorders of fatty-acid oxidation

Not billable (header)Chapter 4

Report one of the more specific codes below.

Check coverage with this diagnosis

Codes under this one

  • E71.310 Long chain/very long chain acyl CoA dehydrogenase deficiency
  • E71.311 Medium chain acyl CoA dehydrogenase deficiency
  • E71.312 Short chain acyl CoA dehydrogenase deficiency
  • E71.313 Glutaric aciduria type II
  • E71.314 Muscle carnitine palmitoyltransferase deficiency
  • E71.318 Other disorders of fatty-acid oxidation

Instructional notes

Chapter 4

Excludes1Not coded here: never report with this code, except when the two conditions are unrelated.
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
Notes
  • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.

Section E70-E88

Excludes1Not coded here: never report with this code, except when the two conditions are unrelated.
  • 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)
Excludes2Not included here: the patient may have both; report both when both are present.
  • Ehlers-Danlos syndromes (Q79.6-)

E71.3

Excludes1Not coded here: never report with this code, except when the two conditions are unrelated.
  • peroxisomal disorders (E71.5)
  • Refsum's disease (G60.1)
  • Schilder's disease (G37.0)
Excludes2Not included here: the patient may have both; report both when both are present.
  • carnitine deficiency due to inborn error of metabolism (E71.42)

Chapter 4: Endocrine, nutritional and metabolic diseases · E70-E88 Metabolic disorders (E70-E88)

Source: ICD-10-CM FY 2027 code descriptions and tabular list (CMS and CDC National Center for Health Statistics), effective 2026-10-01 through 2027-09-30 · CMS ICD-10 files. HCC mapping: CMS-HCC V28, payment year 2027. As of 2026-11-04.

Coverage and payment depend on the claim details; confirm with the Medicare Administrative Contractor.