Congenital iodine-deficiency syndrome (E00)
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.
Endocrine, nutritional and metabolic diseases (E00–E89)
Disorders of thyroid gland (E00-E07)
E00 Congenital iodine-deficiency syndrome
- E00.0 Congenital iodine-deficiency syndrome, neurological type
- E00.1 Congenital iodine-deficiency syndrome, myxedematous type
- E00.2 Congenital iodine-deficiency syndrome, mixed type
- E00.9 Congenital iodine-deficiency syndrome, unspecified
Congenital iodine-deficiency syndrome (E00)
Instructional Notations
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
Clinical Terms
The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.
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.
