Short answer · Medically reviewed summary · Last updated: 2026-05-08

The primary ICD-10-CM code for Encephalocele is Q01, which encompasses various specific anatomical locations such as frontal, nasofrontal, and occipital encephalocele. In the older ICD-9-CM classification system, Encephalocele was categorized under code 742.0. What is the clinical classification of Encephalocele? Encephalocele is a rare neural tube defect characterized by a sac-like protrusion of the brain and its surrounding membranes through an opening in the skull.

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ICD10 code of Encephalocele and ICD9 code

ICD-10 and ICD-9 codes for Encephalocele, with classification details for clinicians, coders and patients.

ICD9 and ICD10 codes of Encephalocele

The primary ICD-10-CM code for Encephalocele is Q01, which encompasses various specific anatomical locations such as frontal, nasofrontal, and occipital encephalocele. In the older ICD-9-CM classification system, Encephalocele was categorized under code 742.0.



What is the clinical classification of Encephalocele?


Encephalocele is a rare neural tube defect characterized by a sac-like protrusion of the brain and its surrounding membranes through an opening in the skull. Clinically, Encephalocele is classified based on the location of the defect, with occipital encephaloceles being the most common form in Western countries. Understanding these codes is essential for proper medical record documentation, insurance billing, and tracking the prevalence of Encephalocele within clinical registries.



How is Encephalocele categorized for medical coding?


Medical coding for Encephalocele requires specificity to ensure accurate diagnostic tracking. The ICD-10 system provides a granular breakdown to reflect the exact presentation of the condition:



  • Q01.0: Frontal encephalocele

  • Q01.1: Nasofrontal encephalocele

  • Q01.2: Encephalocele of nasopharynx

  • Q01.8: Encephalocele of other sites (e.g., parietal or occipital)

  • Q01.9: Encephalocele, unspecified



Is there support for families living with Encephalocele?


Navigating a diagnosis of Encephalocele can be emotionally challenging for families. At DiseaseMaps.org, 27 community members have shared their experiences, providing a space for peer support and shared knowledge. Connecting with others who understand the complexities of Encephalocele can help mitigate the isolation often felt by caregivers.



Next steps



  • Consult with a pediatric neurosurgeon to discuss the specific anatomical presentation of the Encephalocele.

  • Request a referral to a clinical geneticist to explore potential underlying causes.

  • Join the DiseaseMaps.org community to connect with other families affected by this condition.

  • Keep a detailed file of all diagnostic imaging and ICD-10 coded reports for your medical records.



Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment.



References



  • NIH Genetic and Rare Diseases Information Center (GARD): Encephalocele

  • Orphanet: Neural tube defect (ORPHA:647)

  • ICD-10-CM Browser (CDC/NCHS): Congenital malformations of the nervous system (Q00-Q07)

  • OMIM (Online Mendelian Inheritance in Man): Encephalocele entry #218500

Medical disclaimer: This information does not substitute professional medical advice. Always consult your doctor before making health decisions.
Source: DiseaseMaps.org
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ICD9 and ICD10 codes of Encephalocele

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