Encephalocele of other sites

This diagnosis code signifies a congenital neural tube defect where brain tissue and meninges protrude through a skull defect at a location other than the occipital, frontal, or parietal regions. It represents a herniation of intracranial contents, often covered by skin or a thin membrane, occurring due to incomplete closure of the cranial vault during fetal development.

Assign this code for encephaloceles documented as occurring in atypical or unspecified cranial locations, such as the sphenoidal, ethmoidal, or nasofrontal regions, when a more specific site code is not applicable. Documentation should clearly indicate the presence of an encephalocele and specify that its location is not one of the commonly coded sites.

• Do not use this code if the encephalocele is specifically documented as occipital (Q01.0), frontal (Q01.1), or parietal (Q01.2); use the more specific code.
• Ensure the medical record clearly distinguishes an encephalocele from a meningocele (Q01.9 for unspecified site), which involves only meninges without brain tissue.
• Consider sequencing this code as a primary diagnosis when it is the reason for the encounter, or as a secondary diagnosis when it is a co-morbidity or complication of another condition.