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E71.118 ICD-10-CM Code: Other branched-chain organic acidurias

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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E71.118

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Other branched-chain organic acidurias

A group of rare inherited metabolic disorders involving the breakdown of branched-chain amino acids, resulting in accumulation of organic acids in the body.

Buddy the Bee presenting code insight

Buddy Insight

This code captures branched-chain organic acidurias not specifically classified elsewhere, representing rare inborn errors where toxic organic acids accumulate due to enzyme deficiencies in branched-chain amino acid catabolism.

CMS-HCC V28

N/A

Not mapped

CMS-HCC V24

HCC 23

RAF 0.194

ACA/HHS

HCC 28

Varies by metal level

ESRD/PACE

HCC 23

RAF 0.036

RXHCC

HCC 43

RAF 0.063

Code Book Path

Official
E71.1Other disorders of branched-chain amino-acid metabolism
E71.11Branched-chain organic acidurias
E71.118Other branched-chain organic acidurias

Inclusion Terms

Official

ICD-10-CM does not list inclusion terms for E71.118 in this effective period.

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E71.118 in this effective period.

Related Child Codes

Official
E71.110Isovaleric acidemia
E71.1113-methylglutaconic aciduria

Includes

Official

ICD-10-CM does not list Includes notes for E71.118 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for E71.118 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E71.118 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E71.118 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E71.118 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance
Document the specific organic aciduria type if identifiable, urine organic acid profiles, plasma amino acid levels, genetic testing results, and current treatment regimen including dietary restrictions.
Record any history of metabolic crises and their frequency.

MEAT Support

HCC Buddy guidance
Document the specific organic aciduria type if identifiable, urine organic acid profiles, plasma amino acid levels, genetic testing results, and current treatment regimen including dietary restrictions.
Record any history of metabolic crises and their frequency.

Audit Caution

HCC Buddy guidance
Use this code only when a specific branched-chain organic aciduria is documented but does not have its own dedicated code.
Do not use when the documentation identifies a named condition with a more specific code available.

Common Mistakes

HCC Buddy guidance
E71.111 (3-methylglutaconic aciduria) when that specific subtype is identified
E71.19 (Other disorders of branched-chain amino-acid metabolism) which is broader
E71.2 (Disorder of branched-chain amino-acid metabolism, unspecified) which lacks specificity.

Last updated: FY2026 ICD-10-CM Apr update, Apr 1, 2026 through Sep 30, 2026. CMS-HCC V28 is 100% phased in for payment year 2026.

Is E71.118 an HCC code?

Yes. E71.118 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.

HCC Category Mapping

V24HCC 23, Other Significant Endocrine and Metabolic Disorders
0.194
ESRDHCC 23, Other Significant Endocrine and Metabolic Disorders
0.036
RxHCCHCC 43, Other Significant Endocrine and Metabolic Disorders
0.063

Each model's RAF is its CMS base weight for that model's standard population, so weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.

Work E71.118 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for E71.118

For E71.118to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

  • MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
  • EEvaluate: test results, medication response, or physical findings reviewed by the provider
  • AAssess: explicit mention in the assessment or plan with acknowledgment of status
  • TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E71.118 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E71.118 is the ICD-10-CM diagnosis code for other branched-chain organic acidurias. A group of rare inherited metabolic disorders involving the breakdown of branched-chain amino acids, resulting in accumulation of organic acids in the body. E71.118 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

Under the older CMS-HCC V24 model, E71.118 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.194. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.

Use this code only when the specific type of branched-chain organic aciduria is not identified. Because E71.118 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E71.118 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • Use this code only when the specific type of branched-chain organic aciduria is not identified
  • If a more specific diagnosis is documented, use the appropriate specific code instead

Clinical Significance

This code captures branched-chain organic acidurias not specifically classified elsewhere, representing rare inborn errors where toxic organic acids accumulate due to enzyme deficiencies in branched-chain amino acid catabolism. These conditions can cause metabolic crises with vomiting, lethargy, and metabolic acidosis, particularly during illness or fasting. Lifelong dietary management and metabolic monitoring are essential.

Documentation Requirements

  • Document the specific organic aciduria type if identifiable, urine organic acid profiles, plasma amino acid levels, genetic testing results, and current treatment regimen including dietary restrictions.
  • Record any history of metabolic crises and their frequency.

Commonly Confused Codes

  • E71.111 (3-methylglutaconic aciduria) when that specific subtype is identified
  • E71.19 (Other disorders of branched-chain amino-acid metabolism) which is broader
  • E71.2 (Disorder of branched-chain amino-acid metabolism, unspecified) which lacks specificity.

Child Codes

Code Hierarchy

Because E71.118 maps to a payment HCC, the documentation must also satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's risk adjustment score.

Work E71.118 in HCC Buddy

Open E71.118 in the Code Book for the full Index-to-Tabular path, MEAT checklist, and V28 HCC mapping, or in the Encoder to code from a keyword search. Pro includes 14 days to try everything.