Craniorachischisis

This code represents a severe neural tube defect characterized by a complete failure of closure of the neural tube along the entire length of the brain and spinal cord. It results in an open defect involving both the cranium and the vertebral column, exposing the brain and spinal cord tissue.

Assign this code for cases of congenital malformation where there is a documented complete non-closure of the neural tube affecting both the skull and the spine. Documentation should clearly indicate the presence of both anencephaly/exencephaly and a severe spina bifida defect, often with exposed neural tissue.

• This is a rare and severe congenital anomaly; ensure documentation explicitly confirms the combined cranial and spinal defect.
• Do not confuse with isolated anencephaly (Q00.0) or isolated spina bifida (Q05.- series) without cranial involvement.
• Code additional associated congenital anomalies separately, if documented.