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    OpenPayerBilling CodesCongenital malformations of the nervous systemICD-10-CM q00-anencephaly-and-similar-malformations
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    ICD-10-CM Code For Anencephaly And Similar Malformations: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q00 includes congenital malformations such as anencephaly and closely related neural tube defects characterized by absent or severely underdeveloped cranial structures and brain tissue. These ICD-10-CM diagnosis codes are used to document congenital central nervous system malformations identified prenatally or at birth, capturing the presence and extent of neural tube defects and associated organ involvement. Accurate coding within the ICD-10-CM Q00 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM Q00: Anencephaly And Similar Malformations Overview

    The Q00 group comprises neural tube defects characterized by absence or severe underdevelopment of major portions of the brain and skull, most notably anencephaly and related malformations. These congenital malformations affect the central nervous system and are typically identified prenatally or at birth. Accurate coding is important for proper clinical documentation and for reimbursement processes tied to congenital anomaly care.

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    When to Use

    Use a code from this group when documenting a confirmed diagnosis of anencephaly or a closely related neural tube defect diagnosed prenatally or at delivery. Select the code that best describes the specific anomaly and laterality or associated findings as documented in the medical record.

    ICD-10-CM Codes

    3 rows

    ICD-10-CM CodeDescription
    Q00.0Anencephaly
    Q00.1Craniorachischisis
    Q00.2Iniencephaly

    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 neonate is born at term with obvious absence of cranial vault and scalp tissue on delivery exam, with brain tissue largely absent on bedside inspection and confirmed by immediate neonatal head ultrasound. The delivery was by spontaneous vaginal birth after an uncomplicated pregnancy until prenatal ultrasound late in gestation suggested a severe cranial abnormality; the infant is evaluated by neonatology for stabilization and counseling. A diagnosis from this group is assigned because the physical findings and imaging demonstrate congenital absence of major portions of the brain and skull consistent with anencephaly. Typical codes: Q00.0
    • A pregnant patient presents for prenatal genetic counseling after a second-trimester anatomy ultrasound shows acrania and absence of fetal cranial vault; the obstetric team discusses prognosis and options and orders fetal MRI for further characterization. The diagnosis code from this group is assigned on the maternal and fetal records to document the fetal neural tube defect detected antenatally and guide perinatal planning and reporting. Typical codes: Q00.1, Q00.0
    • A termination of pregnancy is performed for a fetus diagnosed earlier with a cranial neural tube defect and associated multiple anomalies; the procedure encounter and pathology report document the congenital malformation as the primary reason for the termination. A code from this group is used on the termination and pathology documentation to indicate the fetal congenital absence of significant cranial structures contributing to the decision for pregnancy termination. Typical codes: Q00.2, Q00.0

    Related Codes

    Q01-ENCEPHALOCELE

    ICD-10-CM Code For Encephalocele: 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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