Iniencephaly

This code identifies a severe congenital malformation characterized by extreme retroflexion of the head, a defect in the occipital bone, and an abnormal curvature of the cervical and thoracic spine. The neck is typically absent or very short, with the face looking upwards. It is often associated with other central nervous system anomalies.

Assign this code when documentation clearly indicates a diagnosis of iniencephaly, a rare and usually lethal neural tube defect. This diagnosis is typically made prenatally via ultrasound or postnatally upon physical examination. Supporting documentation will describe the characteristic head and spinal deformities.

• This is a congenital anomaly; ensure documentation specifies it as such.
• Always look for and code any associated congenital anomalies, as iniencephaly rarely occurs in isolation.
• Consider sequencing this code as a principal diagnosis when it is the primary reason for the encounter, such as for diagnostic workup or counseling.