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E00.9

Billable

Congenital iodine-deficiency syndrome, unspecified

HCC Category Mapping

RxHCCHCC 44Thyroid and Parathyroid Disorders
0.000

What This Code Means

A birth defect caused by iodine deficiency during pregnancy where the specific type or presentation is not clearly documented or specified.

Coding Tips

  • Use this code only when the specific type of congenital iodine-deficiency syndrome cannot be determined from documentation
  • Query the provider if more specific information about goiter presence or neurological manifestations is available

Clinical Significance

Congenital iodine-deficiency syndrome, unspecified, is used when a patient has documented congenital iodine-deficiency syndrome but the specific type (neurological, myxedematous, or mixed) has not been determined or documented. This is a less clinically informative code that should prompt further characterization of the patient's clinical presentation. The condition remains extremely rare in regions with adequate iodine supplementation.

Documentation Requirements

  • Minimum documentation requires a confirmed diagnosis of congenital iodine-deficiency syndrome.
  • However, the provider should be queried to specify the type (neurological, myxedematous, or mixed) based on the predominant clinical features.
  • History of iodine-deficient environment, thyroid function tests, and neurodevelopmental assessments should be documented to support type classification.

Commonly Confused Codes

Code Hierarchy

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