E71.510 ICD-10-CM Code: Zellweger syndrome
E71.510 is not a CMS-HCC payment code. MEAT criteria · RAF calculator · HCC Buddy coding tools
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E71.510
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceZellweger syndrome
A severe inherited metabolic disorder present from birth where peroxisomes (cellular structures) fail to develop properly, causing multiple organ system problems including developmental delays and facial abnormalities.
Buddy Insight
Zellweger syndrome (cerebrohepatorenal syndrome) is the most severe form of peroxisome biogenesis disorder, characterized by complete absence of functional peroxisomes leading to devastating multi-system disease presenting at birth.
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 E71.510 in this effective period.
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for E71.510 in this effective period.
Excludes 1
Official- Refsum's disease (G60.1)
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for E71.510 in this effective period.
Use Additional
OfficialICD-10-CM does not list Use Additional Code instructions for E71.510 in this effective period.
Code Also
OfficialICD-10-CM does not list Code Also instructions for E71.510 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 E71.510 an HCC code?
Yes. E71.510 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.
- Code
- E71.510
- Description
- Zellweger 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 E71.510 in the Code Book — tabular path, V28 RAF, and MEAT checklist →
MEAT Criteria for E71.510
For E71.510 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 E71.510 during that encounter, not just copy-forwarded from a problem list.
Coder workflow notes
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What This Code Means
E71.510 is the ICD-10-CM diagnosis code for zellweger syndrome. A severe inherited metabolic disorder present from birth where peroxisomes (cellular structures) fail to develop properly, causing multiple organ system problems including developmental delays and facial abnormalities. E71.510 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, E71.510 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.
This is typically diagnosed in the neonatal period; document age of onset and clinical findings (hypotonia, seizures, facial dysmorphism). Because E71.510 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 E71.510 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •This is typically diagnosed in the neonatal period; document age of onset and clinical findings (hypotonia, seizures, facial dysmorphism)
- •Zellweger syndrome is the most severe form of peroxisomal biogenesis disorders; prognosis is poor with most infants not surviving past infancy
Clinical Significance
Zellweger syndrome (cerebrohepatorenal syndrome) is the most severe form of peroxisome biogenesis disorder, characterized by complete absence of functional peroxisomes leading to devastating multi-system disease presenting at birth. Affected infants display severe hypotonia, seizures, hepatomegaly, renal cysts, characteristic facial features, and profound developmental impairment. Prognosis is poor with most patients not surviving beyond the first year of life.
Documentation Requirements
- ✓Document neonatal presentation including hypotonia, seizures, craniofacial dysmorphism, hepatomegaly, very long chain fatty acid levels (markedly elevated), plasmalogen levels (severely decreased), and genetic testing confirming PEX gene mutations.
- ✓Record supportive care measures and multisystem involvement.