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    v1.0.28
    OpenPayerBilling CodesCongenital malformations of the nervous systemICD-10-CM q01-encephalocele
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    ICD-10-CM Code For Encephalocele: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q01 includes congenital encephaloceles, which are defects with herniation of brain tissue or meninges through a cranial defect and related anatomic subtypes such as occipital, frontal, and basal encephaloceles. These ICD-10-CM diagnosis codes are used to document clinical presentations involving neural tube closure defects, anatomic site of the lesion, and associated central nervous system involvement or complications. Accurate coding within the ICD-10-CM Q01 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM Q01: Encephalocele Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM Q01) encephalocele group represents congenital herniation of intracranial contents through a skull defect affecting the central nervous system. These diagnoses target developmental malformations of the neural tube and surrounding cranial structures. Accurate coding matters for appropriate clinical documentation and reimbursement because it captures anatomic site, presence of brain tissue or meninges in the sac, and any associated anomalies that impact care needs.

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    When to Use

    Use a code from this group when a congenital cranial defect with herniation of intracranial contents has been diagnosed, documented by imaging or surgical findings. Select the code that reflects the specific anatomic site and any documented involvement of brain tissue or meninges.

    ICD-10-CM Codes

    5 rows

    ICD-10-CM CodeDescription
    Q01.0Frontal encephalocele
    Q01.1Nasofrontal encephalocele
    Q01.2Occipital encephalocele
    Q01.8Encephalocele of other sites
    Q01.9Encephalocele, unspecified

    DRG Codes

    5 rows

    DRG CodeDescription
    091Other Disorders of Nervous System with MCC
    092Other Disorders of Nervous System with CC
    093Other Disorders of Nervous System without CC/MCC
    791Prematurity with Major Problems
    793Full Term Neonate with Major Problems

    Typical Clinical Scenarios

    • A newborn delivered at term with a visibly protruding sac at the occipital region containing neural tissue and cerebrospinal fluid is evaluated by neonatology and pediatric neurosurgery for defect characterization and initial management. The birth presentation prompts imaging (head ultrasound/CT/MRI) confirming a meningocele or encephalocele involving the occipital skull defect, and the diagnosis from this group is assigned to capture the congenital neural tube defect. Typical codes: Q01.0, Q01.9
    • An infant presents to clinic with a midline nasal mass that enlarges with crying and is associated with intermittent CSF rhinorrhea and possible meningitis; ENT and neurosurgical teams perform imaging showing a frontoethmoidal encephalocele extending into the nasal cavity. Because this is a congenital skull and brain tissue herniation through a defect causing functional impairment and infection risk, a code from this group is used to describe the specific anatomic encephalocele. Typical codes: Q01.1, Q01.8
    • An adult with a history of repaired occipital encephalocele in childhood presents with seizures and new focal neurologic deficits; current evaluation documents residual or recurrent encephalocele-associated cortical malformation contributing to chronic epilepsy and neurologic comorbidity. In this chronic complication/comorbidity setting, an appropriate congenital encephalocele code is assigned to reflect the underlying structural cause relevant to ongoing care and coding. Typical codes: Q01.2, Q01.9

    Related Codes

    Q00-ANENCEPHALY-AND-SIMILAR-MALFORMATIONS

    ICD-10-CM Code For Anencephaly And Similar Malformations: Group Overview

    Q02-MICROCEPHALY

    ICD-10-CM Code For Microcephaly: Group Overview

    Q03-CONGENITAL-HYDROCEPHALUS

    ICD-10-CM Code For Congenital Hydrocephalus: Group Overview

    Q04-OTHER-CONGENITAL-MALFORMATIONS-OF-BRAIN

    ICD-10-CM Code For Other Congenital Malformations Of Brain: Group Overview

    Q05-SPINA-BIFIDA

    ICD-10-CM Code For Spina Bifida: Group Overview

    Q06-OTHER-CONGENITAL-MALFORMATIONS-OF-SPINAL-CORD

    ICD-10-CM Code For Other Congenital Malformations Of Spinal Cord: Group Overview

    Q07-OTHER-CONGENITAL-MALFORMATIONS-NERVOUS-SYSTEM

    ICD-10-CM Code For Other Congenital Malformations Of Nervous System: Group Overview

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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