ICD-10-CM 2027
ICD-10 code for AADC deficiency
Aromatic L-amino acid decarboxylase deficiency
From the official ICD-10-CM alphabetic index entry “Deficiency, deficient › AADC (aromatic L-amino acid decarboxylase)”. Page updated September 29, 2026.
About coding AADC deficiency
The ICD-10-CM code for AADC deficiency is E70.81 (Aromatic L-amino acid decarboxylase deficiency).
Within E70.8 (other disorders of aromatic amino-acid metabolism), E70.81 is specifically for aromatic L-amino acid decarboxylase deficiency. Related codes cover other disorders of aromatic amino-acid metabolism (E70.89).
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.
Common questions about AADC deficiency ICD-10 codes
What is the ICD-10 code for AADC deficiency?
- E70.81 — Aromatic L-amino acid decarboxylase deficiency.
Is E70.81 billable?
- Yes. E70.81 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E70.81 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E70.81 as a principal diagnosis.
Is E70.81 a CC or MCC?
- E70.81 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.