Iniencephaly

This code represents iniencephaly, a severe congenital malformation characterized by a defect in the occipital bone, a fixed retroflexion of the head, and a short, malformed spine. The head is hyperextended and fused to the cervical and thoracic vertebrae, often accompanied by anencephaly or encephalocele. This condition is typically incompatible with life.

Use this code when documentation confirms a diagnosis of iniencephaly, a rare and severe neural tube defect. This diagnosis is usually made prenatally via ultrasound or postnatally upon physical examination. Documentation should clearly describe the characteristic features, including the retroflexed head, spinal defects, and occipital bone abnormalities.

• Do not confuse this with other neural tube defects like anencephaly (Q00.0) or encephalocele (Q01.-) unless they co-occur and are separately documented.
• Always review the medical record for associated congenital anomalies, which should be coded separately if present.
• This code is typically assigned for live births with the condition or for fetal diagnoses documented in maternal records.