ICD-10-CM 2027
ICD-10 code for Menkes' disease or syndrome
From the official ICD-10-CM alphabetic index entry “Menkes' disease or syndrome”. Page updated September 29, 2026.
About coding Menkes' disease or syndrome
The ICD-10-CM code for Menkes' disease or syndrome is E83.09 (Other disorders of copper metabolism). The official index lists 1 more specific code, so check the documentation for details such as type, cause, site or severity before settling on E83.09.
Within E83.0, choose E83.09 (Other disorders of copper metabolism) only when documentation doesn't support a more specific option: disorder of copper metabolism, unspecified (E83.00) and Wilson's disease (E83.01).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
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.
More specific Menkes' disease or syndrome codes (official index)
- meaning maple-syrup-urine disease E71.0 — Maple-syrup-urine disease
Common questions about Menkes' disease or syndrome ICD-10 codes
What is the ICD-10 code for Menkes' disease or syndrome?
- E83.09 — Other disorders of copper metabolism. More specific codes apply when the documentation supports them: E71.0.
What is the ICD-10 code for meaning maple-syrup-urine disease menkes' disease or syndrome?
- E71.0 — Maple-syrup-urine disease.
Is E83.09 billable?
- Yes. E83.09 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E83.09 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E83.09 as a principal diagnosis.
Is E83.09 a CC or MCC?
- No. E83.09 is neither a CC nor an MCC under MS-DRG v44.0.