E00.1 Congenital iodine-deficiency syndrome, myxedematous type

Billable ICD-10-CM code, FY2026.

Applicable to

• Endemic hypothyroid cretinism
• Endemic cretinism, myxedematous type

Applies from E00 Congenital iodine-deficiency syndrome

Excludes1 (never code together)

• subclinical iodine-deficiency hypothyroidism (E02)

Use additional code

• code (F70-F79) to identify associated intellectual disabilities.

Risk adjustment (HCC)

• RxHCC V08: HCC 44 — Thyroid Disorders

MS-DRG index

Defines the logic of DRG(s) 643-645 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 243 Congenital hypothyroidism (approximate)

Code annotations containing back-references to E00.1

Back-references to E00.1

• Code First: D63.8 Anemia in other chronic diseases classified elsewhere ("hypothyroidism (E00.0-E03.9)")
• Code First: G13.2 Systemic atrophy primarily affecting the central nervous system in myxedema ("myxedematous congenital iodine deficiency (E00.1)")
• Code First: G32.8 Other specified degenerative disorders of nervous system in diseases classified elsewhere ("cerebral degeneration (due to) hypothyroidism (E00.0-E03.9)")

Back-references to E00

• Type 1 Excludes: E01 Iodine-deficiency related thyroid disorders and allied conditions ("congenital iodine-deficiency syndrome (E00.-)")
• Type 1 Excludes: E03 Other hypothyroidism ("iodine-deficiency related hypothyroidism (E00-E02)")
• Type 1 Excludes: E04 Other nontoxic goiter ("iodine-deficiency related goiter (E00-E02)")
• Type 1 Excludes: E61 Deficiency of other nutrient elements ("iodine deficiency related thyroid disorders (E00-E02)")
• Code First: F02 Dementia in other diseases classified elsewhere ("hypothyroidism, acquired (E00-E03.-)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("endocrine disease, except diabetes (E00-E07, E15-E16, E20-E34)")
• Code Also: I27.29 Other secondary pulmonary hypertension ("hypothyroidism (E00-E03)")
• Code First: M14.8 Arthropathies in other specified diseases classified elsewhere ("hypothyroidism (E00-E03)")
• Type 2 Excludes: Q86 Congenital malformation syndromes due to known exogenous causes, not elsewhere classified ("iodine-deficiency-related hypothyroidism (E00-E02)")

Diagnosis Index entries for E00.1

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

Nearby codes

• E00 Congenital iodine-deficiency syndrome
• E00.0 Congenital iodine-deficiency syndrome, neurological type
• E00.1 Congenital iodine-deficiency syndrome, myxedematous type (this code)
• E00.2 Congenital iodine-deficiency syndrome, mixed type
• E00.9 Congenital iodine-deficiency syndrome, unspecified