ICD-10 Code E00.1 – Congenital iodine-deficiency syndrome, myxedematous type (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E00.1 – Congenital iodine-deficiency syndrome, myxedematous type
What it is
E00.1 identifies congenital iodine-deficiency syndrome with a myxedematous type, a condition caused by iodine deficiency before birth. It reflects chronic developmental effects from inadequate maternal and fetal iodine supply.
Clinical signs
Typical findings include growth and developmental delay, hypothyroidism-related features, and myxedematous appearance. Clinical features vary; refer to documentation for the specific congenital manifestations present.
When to use this code
Use E00.1 when the provider documents congenital iodine-deficiency syndrome, myxedematous type, or equivalent wording. It is appropriate for confirmed congenital cases tied to iodine deficiency rather than acquired thyroid disease. Check documentation if the type is not clearly stated.
Do not use for
Do not use this code for acquired hypothyroidism, nonspecific thyroid enlargement, or iodine deficiency without congenital syndrome documentation. If the record does not specify the myxedematous type, check documentation.
Coding tip
Code the documented congenital type exactly as stated; do not infer it from hypothyroidism alone.