Craniorachischisis

Craniorachischisis is a severe congenital neural tube defect characterized by the complete failure of the neural tube to close, resulting in an open brain and spinal cord. This malformation involves the absence of the skull and vertebral arches, leaving the brain and spinal cord exposed. It is a rare and typically lethal condition.

This code is used for cases of complete craniorachischisis, where both the cranium and spinal column are extensively open. Documentation should clearly indicate the presence of an open neural tube defect affecting both the brain and spinal cord, often noted during prenatal imaging or at birth.

• Differentiate from anencephaly (absence of brain and skull) or spina bifida (open spinal column only).
• Always review imaging reports and pathology findings for definitive diagnosis.
• Consider sequencing with codes for associated congenital anomalies if present and documented.