2021 ICD-10-CM Diagnosis Code E00.1

Congenital iodine-deficiency syndrome, myxedematous type

    2016 2017 2018 2019 2020 2021 Billable/Specific Code
  • E00.1 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2021 edition of ICD-10-CM E00.1 became effective on October 1, 2020.
  • This is the American ICD-10-CM version of E00.1 - other international versions of ICD-10 E00.1 may differ.
Applicable To
  • Endemic hypothyroid cretinism
  • Endemic cretinism, myxedematous type
The following code(s) above E00.1 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E00.1:
  • E00-E89
    2021 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    Note
    • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E00
    ICD-10-CM Diagnosis Code E00

    Congenital iodine-deficiency syndrome

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • subclinical iodine-deficiency hypothyroidism (E02)
    Use Additional
    • code (F70-F79) to identify associated intellectual disabilities.
    Congenital iodine-deficiency syndrome
Approximate Synonyms
  • Congenital iodine deficiency syndrome, myxedematous ty
  • Endemic congenital iodine deficiency syndrome of myxedematous type
Clinical Information
  • Severely reduced physical and mental growth associated with pyramidal and extrapyramidal signs and symptoms, due to dietary iodine deficiency.
ICD-10-CM E00.1 is grouped within Diagnostic Related Group(s) (MS-DRG v38.0):
  • 643 Endocrine disorders with mcc
  • 644 Endocrine disorders with cc
  • 645 Endocrine disorders without cc/mcc

Convert E00.1 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
Code annotations containing back-references to E00.1:
  • Code First: G13.2
    ICD-10-CM Diagnosis Code G13.2

    Systemic atrophy primarily affecting the central nervous system in myxedema

      2016 2017 2018 2019 2020 2021 Billable/Specific Code Manifestation Code
    Code First
    • underlying disease, such as:
    • hypothyroidism (E03.-)
    • myxedematous congenital iodine deficiency (E00.1)

Diagnosis Index entries containing back-references to E00.1:
  • Cretin, cretinism (congenital) (endemic) (nongoitrous) (sporadic) E00.9
    ICD-10-CM Diagnosis Code E00.9

    Congenital iodine-deficiency syndrome, unspecified

      2016 2017 2018 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Congenital iodine-deficiency hypothyroidism NOS
    • Endemic cretinism NOS
    • type
      • hypothyroid E00.1
      • myxedematous E00.1
  • Myxedema (adult) (idiocy) (infantile) (juvenile) E03.9
    - see also Hypothyroidism
    ICD-10-CM Diagnosis Code E03.9

    Hypothyroidism, unspecified

      2016 2017 2018 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Myxedema NOS
    • congenital E00.1
  • Syndrome - see also Disease
    • iodine-deficiency, congenital E00.9
      ICD-10-CM Diagnosis Code E00.9

      Congenital iodine-deficiency syndrome, unspecified

        2016 2017 2018 2019 2020 2021 Billable/Specific Code
      Applicable To
      • Congenital iodine-deficiency hypothyroidism NOS
      • Endemic cretinism NOS
      • type
        • myxedematous E00.1

ICD-10-CM Codes Adjacent To E00.1
D89.831 …… grade 1
D89.832 …… grade 2
D89.833 …… grade 3
D89.834 …… grade 4
D89.835 …… grade 5
D89.839 …… grade unspecified
D89.89 Other specified disorders involving the immune mechanism, not elsewhere classified
D89.9 Disorder involving the immune mechanism, unspecified
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
E01 Iodine-deficiency related thyroid disorders and allied conditions
E01.0 Iodine-deficiency related diffuse (endemic) goiter
E01.1 Iodine-deficiency related multinodular (endemic) goiter
E01.2 Iodine-deficiency related (endemic) goiter, unspecified
E01.8 Other iodine-deficiency related thyroid disorders and allied conditions
E02 Subclinical iodine-deficiency hypothyroidism
E03 Other hypothyroidism
E03.0 Congenital hypothyroidism with diffuse goiter

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.