Anencephalus

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 cephalic end during embryonic development. Infants with this condition are typically stillborn or die shortly after birth.

Use this code when documentation clearly indicates a diagnosis of anencephaly in a fetus or newborn. This diagnosis is often made prenatally via ultrasound or postnatally upon examination. Supporting documentation includes fetal anomaly reports, pathology reports from stillbirths, or newborn medical records detailing the congenital malformation.

• This is a congenital anomaly code, typically assigned to the fetus or newborn.
• Do not confuse with other neural tube defects like spina bifida, which have distinct codes.
• Always review the full medical record to ensure the diagnosis of anencephaly is definitively documented.