2027 ICD-10-CM Diagnosis Code E00Congenital iodine-deficiency syndrome

ICD-10-CM Codes›E00–E89›E00-E07›E00

ICD-10-CM E00
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

E00 is a non-billable ICD-10-CM category code for congenital iodine-deficiency syndrome, so it cannot be submitted on claims. Use a more specific code from this category instead, such as E00.0, E00.1, E00.2, and E00.9.

Code Identity

ICD-10-CM Code
E00
Billable Status
No — Non-Billable Category
Code Describes
Congenital iodine-deficiency syndrome
Short Description
Congenital iodine-deficiency syndrome
Same as the full description in the CMS dataset.
Chapter
E00-E07
Disorders of thyroid gland

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE00-E07Disorders of thyroid gland
CategoryE00Congenital iodine-deficiency syndrome
This CodeE00Congenital iodine-deficiency syndrome

Specific Coding for Congenital iodine-deficiency syndromeOverview

Non-specific codes like E00 require more characters. Use one of these billable codes instead:

  • Use E00.0 for Congenital iodine-deficiency syndrome, neurological type

  • Use E00.1 for Congenital iodine-deficiency syndrome, myxedematous type

  • Use E00.2 for Congenital iodine-deficiency syndrome, mixed type

  • Use E00.9 for Congenital iodine-deficiency syndrome, unspecified

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to E00: its own notes plus those printed at Chapter 4. A note printed at a category, block or chapter applies to every code under it.

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

On E00 itself
  • subclinical iodine-deficiency hypothyroidism (E02)
From Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases applies to 973 codes
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Use Additional Code

Add a second code, after this one, to fully describe the condition.

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

Notes

General instructions on how these codes are used.

From Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases applies to 973 codes
  • 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.

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name E00, its category, or a range that includes it.

Applicable To 1

These notes mention E00.

  • Z83.4 Family history of other endocrine, nutritional and metabolic diseases
    Conditions classifiable to E00-E07, E15-E88

Excludes1 4

These codes carry an Excludes1 note naming E00: they are not reported together with it, unless the two conditions are unrelated.

  • E01 Iodine-deficiency related thyroid disorders and allied conditions
    congenital iodine-deficiency syndrome (E00.-)
  • E03 Other hypothyroidism
    iodine-deficiency related hypothyroidism (E00-E02)
  • E04 Other nontoxic goiter
    iodine-deficiency related goiter (E00-E02)
  • E61 Deficiency of other nutrient elements
    iodine deficiency related thyroid disorders (E00-E02)

Excludes2 1

These codes carry an Excludes2 note naming E00: its condition is not part of those codes, and both may be reported when the patient has both.

  • Q86 Congenital malformation syndromes due to known exogenous causes, not elsewhere classified
    iodine-deficiency-related hypothyroidism (E00-E02)

Code First 3

These codes say to code first a condition named in the note, and E00 is one of the conditions named.

  • F02 Dementia in other diseases classified elsewhere
    hypothyroidism, acquired (E00-E03.-)
  • G63 Polyneuropathy in diseases classified elsewhere
    endocrine disease, except diabetes (E00-E07, E15-E16, E20-E34)
  • M14.8 Arthropathies in other specified diseases classified elsewhere
    hypothyroidism (E00-E03)

Code Also 1

These codes say E00 may also be needed to fully describe the condition. The note gives no sequencing direction.

  • I27.29 Other secondary pulmonary hypertension
    hypothyroidism (E00-E03)

Clinical InformationClinical

  • Congenital Iodine Deficiency Syndrome

    a condition attributed to iodine deficiency during gestation. it is characterized by a spectrum of mental and physical disabilities.

Patient EducationClinical

Thyroid Diseases

Your thyroid is a small, butterfly-shaped gland in the front of your neck. It makes hormones that control the way the body uses energy. These hormones affect nearly every organ in your body and control many of your body's most important functions. For example, they affect your breathing, heart rate, weight, digestion, and moods.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About E00Overview

What is the ICD-10 code for congenital iodine-deficiency syndrome?

E00 is the ICD-10-CM category for congenital iodine-deficiency syndrome, but it is a non-billable header: claims need a more specific code from this category, listed on this page.

Is E00 (Congenital iodine-deficiency syndrome) a billable code?

No. This is a category header that groups the codes for congenital iodine-deficiency syndrome, and headers cannot be submitted on claims. Claims for congenital iodine-deficiency syndrome need a more specific code from this category, such as E00.0, E00.1, and E00.2.

Can E00 be reported with E02?

Not as a rule. The Excludes1 note on E00 lists "subclinical iodine-deficiency hypothyroidism (E02)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).

Does E00 need an additional code?

When the condition applies, yes. The Use Additional Code note reads: code (F70-F79) to identify associated intellectual disabilities.. The additional code is reported after E00.