E71.541 ICD-10-CM Code: Zellweger-like syndrome
E71.541 is not a CMS-HCC payment code. MEAT criteria · RAF calculator · HCC coding software
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E71.541
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceZellweger-like syndrome
A severe genetic metabolic disorder similar to Zellweger syndrome that affects multiple organ systems including the brain, liver, and kidneys due to peroxisome dysfunction.
Buddy Insight
Zellweger-like syndrome represents milder forms on the Zellweger spectrum of peroxisome biogenesis disorders, with patients retaining some residual peroxisomal function and potentially surviving into childhood or beyond.
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
OfficialICD-10-CM does not list inclusion terms for E71.541 in this effective period.
Excludes 2
OfficialICD-10-CM does not list Excludes 2 notes for E71.541 in this effective period.
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for E71.541 in this effective period.
Excludes 1
OfficialICD-10-CM does not list Excludes 1 notes for E71.541 in this effective period.
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for E71.541 in this effective period.
Use Additional
OfficialICD-10-CM does not list Use Additional Code instructions for E71.541 in this effective period.
Code Also
OfficialICD-10-CM does not list Code Also instructions for E71.541 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 E71.541 an HCC code?
Yes. E71.541 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.
- Code
- E71.541
- Description
- Zellweger-like syndrome
- 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 E71.541 in the Code Book — tabular path, V28 RAF, and MEAT checklist →
MEAT Criteria for E71.541
For E71.541 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 E71.541 during that encounter, not just copy-forwarded from a problem list.
Coder workflow notes
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What This Code Means
E71.541 is the ICD-10-CM diagnosis code for zellweger-like syndrome. A severe genetic metabolic disorder similar to Zellweger syndrome that affects multiple organ systems including the brain, liver, and kidneys due to peroxisome dysfunction. E71.541 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, E71.541 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.
Distinguish between classic Zellweger syndrome (E71.510) and Zellweger-like syndrome; documentation must specify 'like' variant. Because E71.541 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 E71.541 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •Distinguish between classic Zellweger syndrome (E71.510) and Zellweger-like syndrome; documentation must specify 'like' variant
- •Often presents in infancy with severe developmental and neurological complications
Clinical Significance
Zellweger-like syndrome represents milder forms on the Zellweger spectrum of peroxisome biogenesis disorders, with patients retaining some residual peroxisomal function and potentially surviving into childhood or beyond. Clinical features overlap with classic Zellweger syndrome but are generally less severe, including developmental delay, hepatic dysfunction, hearing loss, and variable neurological involvement. Long-term outcomes depend on the degree of residual peroxisomal function.
Documentation Requirements
- ✓Document that the presentation is Zellweger-like rather than classic Zellweger, very long chain fatty acid levels, plasmalogen levels showing partial reduction, PEX gene mutations, clinical severity assessment, and specific organ system involvement.
- ✓Record developmental milestones and any progressive features.
Commonly Confused Codes
- •E71.510 (Zellweger syndrome) for the classic severe form with no peroxisomal function
- •E71.511 (Neonatal adrenoleukodystrophy) which is on the same spectrum but historically classified separately
- •E71.518 (Other disorders of peroxisome biogenesis) for other biogenesis defects not on the Zellweger spectrum.