Congenital iodine-deficiency syndrome (E00) ICD-10-CM
The E00 code range covers congenital iodine-deficiency syndrome with 5 ICD-10-CM diagnosis codes. 4 of them are billable and valid for claim submission in fiscal year 2026, and the category headers group them but cannot themselves be billed.
Use Additional Code
The “use additional code” indicates that a secondary code could be used to further specify the patient’s condition. This note is not mandatory and is only used if enough information is available to assign an additional code.
Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
- subclinical iodine-deficiency hypothyroidism E02
Codes in the E00 Range 5 codes · 4 billable
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the E00 range.
Congenital Hypothyroidism
A condition in infancy or early childhood due to an in-utero deficiency of THYROID HORMONES that can be caused by genetic or environmental factors, such as thyroid dysgenesis or HYPOTHYROIDISM in infants of mothers treated with THIOURACIL during pregnancy. Endemic cretinism is the result of iodine deficiency. Clinical symptoms include severe MENTAL RETARDATION, impaired skeletal development, short stature, and MYXEDEMA.
Myxedema
A condition characterized by a dry, waxy type of swelling (EDEMA) with abnormal deposits of MUCOPOLYSACCHARIDES in the SKIN and other tissues. It is caused by a deficiency of THYROID HORMONES. The skin becomes puffy around the eyes and on the cheeks. The face is dull and expressionless with thickened nose and lips.
Scleromyxedema
A connective tissue disorder characterized by widespread thickening of SKIN with a cobblestone-like appearance. It is caused by proliferation of FIBROBLASTS and deposition of MUCIN in the DERMIS in the absence of thyroid disease. Most scleromyxedema cases are associated with a MONOCLONAL GAMMOPATHY, immunoglobulin IgG-lambda.
About the E00 Code Range
The ICD-10 code E00 and its subcodes classify different forms of congenital iodine-deficiency syndrome, a condition caused by insufficient iodine affecting thyroid function from birth. These codes help identify specific types for accurate diagnosis and treatment.
These codes cover variations such as the E00.0 neurological type, known as endemic cretinism, affecting neurological development; the E00.1 myxedematous type, associated with myxedema and thyroid hormone deficiency; and the E00.2 mixed type, which combines features of both neurological and myxedematous forms. The E00.9 unspecified type is used when the exact form of congenital iodine deficiency syndrome or hypothyroidism due to iodine deficiency is not clearly detailed. Using synonyms like "endemic cretinism," "myxedema," and "congenital hypothyroidism due to iodine deficiency" ensures correct code selection by linking common clinical terms to their precise ICD-10 designations.
Questions About This Page
How many billable codes are in the E00 range?
Of the 5 codes in this range, 4 are billable and valid for claim submission from October 1, 2025 through September 30, 2026. Category header codes group them but cannot be reported on claims.
What does the E00 range classify?
The range classifies congenital iodine-deficiency syndrome. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2026 ICD-10-CM Tabular List and order file, effective October 1, 2025 through September 30, 2026.
