E00.2

HCC Category Mapping

What This Code Means

A birth defect caused by severe iodine deficiency during pregnancy that results in multiple thyroid and developmental problems, including both goiter (enlarged thyroid) and intellectual disability.

Coding Tips

• •This code indicates congenital cretinism with mixed manifestations; document whether goiter is present and severity of developmental delays
• •Verify if this is truly congenital (present at birth) versus acquired iodine deficiency later in life

Clinical Significance

Congenital iodine-deficiency syndrome, mixed type, represents the combination of both neurological and myxedematous features resulting from severe prenatal iodine deficiency. Patients exhibit both the neurological damage (intellectual disability, motor deficits) and the hypothyroid manifestations (growth retardation, myxedema) seen in the individual types. This mixed presentation reflects the dual impact of iodine deficiency on both brain development and thyroid function.

Documentation Requirements

• ✓Documentation must clearly describe features of both neurological damage (intellectual disability, spasticity, hearing loss) and myxedematous hypothyroidism (growth failure, myxedema, delayed skeletal maturation) attributed to iodine deficiency.
• ✓Thyroid function tests, developmental assessments, and history of iodine-deficient environment should be recorded.
• ✓Current treatment including thyroid replacement and rehabilitation services should be documented.

Commonly Confused Codes