E00.1 Congenital iodine-deficiency syndrome, myxedematous type

The ICD-10-CM code for Congenital iodine-deficiency syndrome, myxedematous type is E00.1 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Chapter

4: Endocrine, nutritional and metabolic diseases (E00-E89)

Section

E00-E07: Disorders of thyroid gland (E00-E07)

Category E00

5 codes (4 billable)

FY2026 Status

Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to E00.1:

• Myxedema (adult) (idiocy) (infantile) (juvenile) › congenital
• Cretin, cretinism (congenital) (endemic) (nongoitrous) (sporadic) › type › myxedematous
• Cretin, cretinism (congenital) (endemic) (nongoitrous) (sporadic) › type › hypothyroid
• Syndrome › iodine-deficiency, congenital › type › myxedematous

Inclusion Terms

• Endemic hypothyroid cretinism
• Endemic cretinism, myxedematous type

U.S. Hospital Utilization

E00.1 appeared in too few sampled U.S. inpatient stays in 2023 to report a national estimate (AHRQ suppresses counts of 10 or fewer) — a rare inpatient diagnosis.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

Chapter 4 TOC entry

4. Chapter 4: Endocrine, Nutritional, and Metabolic Diseases (E00-E89) ................................. 39

Diabetes mellitus (TOC entry)

a. Diabetes mellitus ................................................................................................................... 39

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool
• AHRQ — HCUP National Inpatient Sample, Frequencies by Diagnosis Code (2016–2023)

Cite This Page

Reference E00.1 with the canonical source and edition:

E00.1 — Congenital iodine-deficiency syndrome, myxedematous type. ICD-10 Coding Pro (ICD-10-CM FY2026). https://icd10codingpro.com/codes/E00.1

Canonical URL: https://icd10codingpro.com/codes/E00.1