Encephalocele

This code describes an encephalocele, a congenital neural tube defect characterized by the protrusion of brain tissue and/or meninges through a defect in the skull. The herniation typically occurs along the midline, most commonly in the occipital region, but can also be frontal or parietal.

Assign this code for documented cases of encephalocele, regardless of its location or the specific contents of the herniated sac (e.g., meningoencephalocele, meningocele). Documentation should clearly identify the presence of this congenital malformation, often diagnosed prenatally or at birth.

• Distinguish from other neural tube defects like spina bifida (741.xx) or anencephaly (740.0).
• Code any associated hydrocephalus separately (e.g., 742.3).
• Ensure documentation specifies if the encephalocele is ruptured or intact, as this may impact treatment but not the primary diagnosis code.