Neurology & Pain Management Coding Alert

Question: What is encephalocele, and how do I choose an ICD-10 code for this condition?

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Answer: According to the Centers for Disease Control and Prevention (CDC) “encephalocele is a sac-like protrusion or projection of the brain and the membranes that cover it through an opening in the skull. Encephalocele happens when the neural tube does not close completely during pregnancy.”

There are several different types of encephalocele diagnoses, which we’ll discuss below:

Initial Dx: The ICD-10 journey starts with Q01.- (Encephalocele), the parent code for the condition.

4thcharacter options: You’ll next need to choose a 4th character to complete the encephalocele

diagnosis. Chose from these options based on the specifics of the patient’s condition:

• Q01.0 (Frontal encephalocele)
• Q01.1 (Nasofrontal encephalocele)
• Q01.2 (Occipital encephalocele)
• Q01.8 (Encephalocele of other sites)
• Q01.9 (Encephalocele, unspecified)

Also known as …: There are several synonyms for encephalocele listed under the Q01.- descriptor. If you see

one of the following terms marking a patient’s diagnosis, code it as encephalocele:

• Arnold-Chiari syndrome, type III
• Encephalocystocele
• Encephalomyelocele
• Hydroencephalocele
• Hydromeningocele, cranial
• Meningocele, cerebral
• Meningoencephalocele

Example: Encounter notes indicate that the provider treated a patient with “cerebral meningocele, occipital.” On the claim, you’d report Q01.2 for the patient’s condition.