E71.511 ICD-10-CM Code: Neonatal adrenoleukodystrophy
HCC Buddy Code Card
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E71.511
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceNeonatal adrenoleukodystrophy
A rare inherited metabolic disorder affecting the nervous system and adrenal glands, presenting in newborns with developmental problems and progressive neurological decline.
Buddy Insight
Neonatal adrenoleukodystrophy is an intermediate-severity peroxisome biogenesis disorder on the Zellweger spectrum, presenting in the neonatal period with hypotonia, seizures, and developmental delay, but with some residual peroxisomal function unlike classic Zellweger syndrome.
CMS-HCC V28
N/A—
Not mapped
CMS-HCC V24
MappedHCC 23
RAF 0.194
ACA/HHS
MappedHCC 28
Varies by metal level
ESRD/PACE
MappedHCC 23
RAF 0.036
RXHCC
MappedHCC 43
RAF 0.063
Code Book Path
Inclusion Terms
Official- Group 1 peroxisomal disorders
Excludes 2
OfficialICD-10-CM does not list Excludes 2 notes for E71.511 in this effective period.
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for E71.511 in this effective period.
Excludes 1
Official- X-linked adrenoleukodystrophy (E71.42-)
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for E71.511 in this effective period.
Use Additional
OfficialICD-10-CM does not list Use Additional Code instructions for E71.511 in this effective period.
Code Also
OfficialICD-10-CM does not list Code Also instructions for E71.511 in this effective period.
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →
Is E71.511 an HCC code?
Yes. E71.511 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.
- Code
- E71.511
- Description
- Neonatal adrenoleukodystrophy
- HCC (V28)
- No CMS-HCC V28 mapping
- RAF
- —
- Billable
- Yes
- Payment year
- 2026
HCC Category Mapping
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 E71.511 in the Code Book — tabular path, V28 RAF, and MEAT checklist →
MEAT Criteria for E71.511
For E71.511 to 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 E71.511 during that encounter, not just copy-forwarded from a problem list.
Coder workflow notes
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What This Code Means
E71.511 is the ICD-10-CM diagnosis code for neonatal adrenoleukodystrophy. A rare inherited metabolic disorder affecting the nervous system and adrenal glands, presenting in newborns with developmental problems and progressive neurological decline. E71.511 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, E71.511 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.
Document the age of symptom onset and specific neurological manifestations (seizures, hypotonia, developmental regression). Because E71.511 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 E71.511 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
Clinical Significance
Neonatal adrenoleukodystrophy is an intermediate-severity peroxisome biogenesis disorder on the Zellweger spectrum, presenting in the neonatal period with hypotonia, seizures, and developmental delay, but with some residual peroxisomal function unlike classic Zellweger syndrome. Patients may survive into childhood but typically experience progressive neurological decline, adrenal insufficiency, and sensorineural hearing loss. Early identification of adrenal insufficiency is critical for management.
Documentation Requirements
- ✓Document age of presentation, neurological findings, adrenal function testing results, very long chain fatty acid levels, peroxisomal function studies, and genetic testing for PEX gene mutations.
- ✓Record developmental milestones, hearing assessments, and adrenal replacement therapy if indicated.
Excludes 1, Do NOT code together
- X-linked adrenoleukodystrophy (E71.42-)
Commonly Confused Codes
- •E71.510 (Zellweger syndrome) which is more severe with no residual peroxisomal function
- •E71.520 (Childhood cerebral X-linked adrenoleukodystrophy) which is a single-enzyme defect rather than a biogenesis disorder
- •E71.518 (Other disorders of peroxisome biogenesis) for non-specific biogenesis defects.