E00.0 ICD-10-CM Code: Congenital iodine-deficiency syndrome, neurological type
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Disorders of thyroid gland (E00-E07)
E00.0
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceCongenital iodine-deficiency syndrome, neurological type
A birth defect caused by severe iodine deficiency during pregnancy, resulting in intellectual disability, growth problems, and neurological damage in the newborn.
Buddy Insight
Congenital iodine-deficiency syndrome, neurological type (neurological cretinism), results from severe iodine deficiency during fetal development, causing irreversible neurological damage including intellectual disability, spastic diplegia, deaf-mutism, and motor impairment.
CMS-HCC V28
N/A—
Not mapped
CMS-HCC V24
N/A—
Not mapped
ACA/HHS
N/A—
Not mapped
ESRD/PACE
N/A—
Not mapped
RXHCC
MappedHCC 44
RAF 0.070
Code Book Path
Inclusion Terms
Official- Endemic cretinism, neurological type
Excludes 2
OfficialICD-10-CM does not list Excludes 2 notes for E00.0 in this effective period.
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for E00.0 in this effective period.
Excludes 1
Official- subclinical iodine-deficiency hypothyroidism (E02)
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for E00.0 in this effective period.
Use Additional
Official- code (F70-F79) to identify associated intellectual disabilities.
Code Also
OfficialICD-10-CM does not list Code Also instructions for E00.0 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 E00.0 an HCC code?
E00.0 is not in the CMS-HCC V28 or V24 community payment model, but it does map to Thyroid and Parathyroid Disorders under the Part D RxHCC model.
- Code
- E00.0
- Description
- Congenital iodine-deficiency syndrome, neurological type
- 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 E00.0 in the Code Book — tabular path, V28 RAF, and MEAT checklist →
MEAT Criteria for E00.0
For E00.0 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 E00.0 during that encounter, not just copy-forwarded from a problem list.
Coder workflow notes
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What This Code Means
E00.0 is the ICD-10-CM diagnosis code for congenital iodine-deficiency syndrome, neurological type. A birth defect caused by severe iodine deficiency during pregnancy, resulting in intellectual disability, growth problems, and neurological damage in the newborn. E00.0 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering disorders of thyroid gland (e00-e07).
E00.0 is a billable ICD-10-CM code but does not map to a payment HCC under the CMS-HCC V28, V24, ESRD, or RxHCC risk adjustment models. It can be reported on Medicare Advantage encounter data submissions but it does not contribute to a beneficiary's RAF score and therefore does not affect risk-adjusted payments to the plan.
This code does not map to a CMS-HCC V28 payment category. Capture depends on documentation that supports the diagnosis; verify the HCC assignment against the current CMS mapping for the applicable payment year. Coders reviewing E00.0 should check whether additional documentation would support a more specific child code in the same hierarchy that does map to a payment HCC, capturing the correct specificity is the highest-impact RAF improvement available within accurate coding.
HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E00.0 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •This is a congenital condition; document maternal iodine deficiency history and neonatal screening results
- •Differentiate from other congenital hypothyroidism codes; this specifically indicates iodine deficiency etiology
Clinical Significance
Congenital iodine-deficiency syndrome, neurological type (neurological cretinism), results from severe iodine deficiency during fetal development, causing irreversible neurological damage including intellectual disability, spastic diplegia, deaf-mutism, and motor impairment. This condition is virtually eliminated in countries with adequate iodine supplementation programs but remains a concern in endemic iodine-deficient regions. The neurological damage is permanent and occurs primarily during the first and second trimesters.
Documentation Requirements
- ✓Documentation must establish the diagnosis of congenital iodine-deficiency syndrome with neurological manifestations, including intellectual disability, motor deficits, or hearing impairment attributable to prenatal iodine deficiency.
- ✓History of maternal iodine deficiency and thyroid function testing should be documented.
- ✓The neurological type is distinguished from the myxedematous type by the predominance of neurological over thyroid findings.
Commonly Confused Codes
- •E00.1 (Congenital iodine-deficiency syndrome, myxedematous type) presents primarily with hypothyroid features rather than neurological damage.
- •E00.2 (Mixed type) combines features of both.
- •E03.0 (Congenital hypothyroidism with diffuse goiter) and E03.1 (Congenital hypothyroidism without goiter) represent hypothyroidism from other causes.
- •F70-F79 (Intellectual disabilities) may be coded additionally for the neurological sequelae.