Anencephaly

This code represents anencephaly, a severe congenital neural tube defect characterized by the absence of a major portion of the brain, skull, and scalp. It results from the failure of the neural tube to close at the cranial end during early embryonic development. Infants with anencephaly are typically stillborn or die shortly after birth.

Use this code for a definitive diagnosis of anencephaly, typically identified prenatally via ultrasound or confirmed postnatally. Documentation should clearly state the diagnosis of anencephaly, often including findings from imaging studies or a pathology report. This code is appropriate for cases of stillbirth or live birth with this condition.

• Do not confuse with other neural tube defects like spina bifida (Q05.-) or encephalocele (Q01.-).
• Always sequence this code as the primary diagnosis when it is the reason for the encounter or the underlying cause of death.
• Ensure documentation specifies "anencephaly" and not just a general "neural tube defect" to support this specific code.