ICD-10 Code E00 – Congenital iodine-deficiency syndrome (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Congenital iodine-deficiency syndrome is E00.
2026 ICD-10-CM Diagnosis Code E00 – Congenital iodine-deficiency syndrome

What it is

E00 identifies congenital iodine-deficiency syndrome, a disorder caused by inadequate iodine during fetal development. It is used for congenital effects linked to iodine deficiency, including thyroid-related developmental impairment.

Clinical signs

Clinical features vary; refer to documentation. Findings may include growth delay, developmental impairment, and signs of congenital hypothyroidism or goiter when documented.

When to use this code

Use E00 when the record states congenital iodine-deficiency syndrome or congenital effects of iodine deficiency. You may also use it when the clinician documents iodine-deficiency-related congenital hypothyroidism or related developmental consequences. Check documentation for the exact manifestation.

Do not use for

Do not use this code for acquired iodine deficiency, nonspecific hypothyroidism, or thyroid disorders without a congenital cause. If the chart does not clearly link the condition to congenital iodine deficiency, check documentation.

Coding tip

Code the documented congenital condition first, and verify whether any associated manifestation needs separate coding.

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