E00.0

HCC Category Mapping

What This Code Means

A birth defect caused by severe iodine deficiency during pregnancy, resulting in intellectual disability, growth problems, and neurological damage in the newborn.

Coding Tips

• •This is a congenital condition; document maternal iodine deficiency history and neonatal screening results
• •Differentiate from other congenital hypothyroidism codes; this specifically indicates iodine deficiency etiology

Clinical Significance

Congenital iodine-deficiency syndrome, neurological type (neurological cretinism), results from severe iodine deficiency during fetal development, causing irreversible neurological damage including intellectual disability, spastic diplegia, deaf-mutism, and motor impairment. This condition is virtually eliminated in countries with adequate iodine supplementation programs but remains a concern in endemic iodine-deficient regions. The neurological damage is permanent and occurs primarily during the first and second trimesters.

Documentation Requirements

• ✓Documentation must establish the diagnosis of congenital iodine-deficiency syndrome with neurological manifestations, including intellectual disability, motor deficits, or hearing impairment attributable to prenatal iodine deficiency.
• ✓History of maternal iodine deficiency and thyroid function testing should be documented.
• ✓The neurological type is distinguished from the myxedematous type by the predominance of neurological over thyroid findings.

Commonly Confused Codes