Q00-Q07
Medium Complexity

Congenital malformations of the nervous system

Primary Specialty: Pediatric Neurology
Last Updated: 2025-09-09

ICD-10 Codes (49)

44 billable
0 category headers
Q01
Encephalocele
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Q01.0
Billable
Frontal encephalocele
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Q01.1
Billable
Nasofrontal encephalocele
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Q01.2
Billable
Occipital encephalocele
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Q01.8
Billable
Encephalocele of other sites
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Q01.9
Billable
Encephalocele, unspecified
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Q02
Billable
Microcephaly
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Q03
Congenital hydrocephalus
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Q03.0
Billable
Malformations of aqueduct of Sylvius
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Q03.1
Billable
Atresia of foramina of Magendie and Luschka
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Q03.8
Billable
Other congenital hydrocephalus
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Q03.9
Billable
Congenital hydrocephalus, unspecified
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Q04
Other congenital malformations of brain
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Q04.0
Billable
Congenital malformations of corpus callosum
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Q04.1
Billable
Arhinencephaly
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Q04.2
Billable
Holoprosencephaly
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Q04.3
Billable
Other reduction deformities of brain
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Q04.4
Billable
Septo-optic dysplasia of brain
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Q04.5
Billable
Megalencephaly
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Q04.6
Billable
Congenital cerebral cysts
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Q04.8
Billable
Other specified congenital malformations of brain
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Q04.9
Billable
Congenital malformation of brain, unspecified
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Q05
Billable
Spina bifida
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Q05.0
Billable
Cervical spina bifida with hydrocephalus
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Q05.1
Billable
Thoracic spina bifida with hydrocephalus
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Q05.2
Billable
Lumbar spina bifida with hydrocephalus
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Q05.3
Billable
Sacral spina bifida with hydrocephalus
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Q05.4
Billable
Unspecified spina bifida with hydrocephalus
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Q05.5
Billable
Cervical spina bifida without hydrocephalus
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Q05.6
Billable
Thoracic spina bifida without hydrocephalus
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Q05.7
Billable
Lumbar spina bifida without hydrocephalus
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Q05.8
Billable
Sacral spina bifida without hydrocephalus
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Q05.9
Billable
Spina bifida, unspecified
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Q06
Other congenital malformations of spinal cord
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Q06.0
Billable
Amyelia
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Q06.1
Billable
Hypoplasia and dysplasia of spinal cord
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Q06.2
Billable
Diastematomyelia
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Q06.3
Billable
Other congenital cauda equina malformations
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Q06.4
Billable
Hydromyelia
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Q06.8
Billable
Other specified congenital malformations of spinal cord
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Q06.9
Billable
Congenital malformation of spinal cord, unspecified
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Q07
Other congenital malformations of nervous system
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Q07.0
Billable
Arnold-Chiari syndrome
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Q07.00
Billable
Arnold-Chiari syndrome without spina bifida or hydrocephalus
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Q07.01
Billable
Arnold-Chiari syndrome with spina bifida
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Q07.02
Billable
Arnold-Chiari syndrome with hydrocephalus
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Q07.03
Billable
Arnold-Chiari syndrome with spina bifida and hydrocephalus
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Q07.8
Billable
Other specified congenital malformations of nervous system
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Q07.9
Billable
Congenital malformation of nervous system, unspecified
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Updates & Changes

FY 2026 Updates

Current Year

New Codes (1)

Q23.4
Hypoplastic left heart syndrome with mitral atresia

Revised Codes (1)

Q21.0
Ventricular septal defect - updated to include hemodynamic significance when known

Deleted Codes

No codes deleted in this range for FY 2026

Historical Changes

  • •FY 2025: Routine maintenance updates with minor terminology clarifications
  • •FY 2024: Enhanced specificity requirements for certain code ranges
  • •FY 2023: Updated documentation guidelines for improved clarity

Upcoming Changes

  • •Proposed updates pending review by Coordination and Maintenance Committee
  • •Under consideration: Enhanced digital health integration codes

Implementation Guidance

  • •Review all FY 2026 updates for Q00-Q07 codes before implementation
  • •Always verify the most current codes in the ICD-10-CM manual
  • •Ensure clinical documentation supports the selected diagnosis codes
  • +3 more guidance items...

Range Overview

high priority

The ICD-10 code range Q00-Q07 pertains to congenital malformations of the nervous system. These codes are used to classify and document birth defects affecting the brain, spinal cord, and other parts of the nervous system. The scope of these codes includes conditions like spina bifida, anencephaly, and congenital hydrocephalus. The usage of these codes is critical in pediatric neurology, neurosurgery, and neonatology for accurate documentation, research, and reimbursement purposes.

Key Usage Points:

  • •Always code to the highest level of specificity.
  • •Include all documented conditions that coexist at the time of the encounter.
  • •Use additional codes to identify any associated conditions.
  • •Do not use these codes for conditions acquired after birth.
  • •Use additional code from category Z38 for multiple births.

Coding Guidelines

When to Use:

  • ✓When a newborn is diagnosed with a congenital malformation of the nervous system.
  • ✓When a patient has a known history of a congenital nervous system malformation and it impacts their current health status.
  • ✓When a patient is being treated for complications related to a congenital nervous system malformation.
  • ✓When a patient is undergoing surgery to correct a congenital nervous system malformation.

When NOT to Use:

  • ✗When a patient has an acquired neurological condition.
  • ✗When a patient has a nervous system disorder not present at birth.
  • ✗When the condition has been corrected and no longer affects the patient's health status.
  • ✗When the condition is not documented by a healthcare provider.

Code Exclusions

Always verify exclusions in the ICD-10 manual to ensure accurate coding.

Documentation Requirements

Documentation for congenital malformations of the nervous system should be comprehensive and include the specific type of malformation, its location, any associated conditions, and the impact on the patient's health status. Any surgical procedures performed or planned should also be documented.

Clinical Information:

  • •Specific type of congenital malformation.
  • •Location of the malformation.
  • •Any associated conditions or complications.
  • •Impact on patient's health status.
  • •Any surgical procedures performed or planned.

Supporting Evidence:

  • •Medical history records.
  • •Clinical examination findings.
  • •Imaging reports.
  • •Surgical reports.
Good Documentation Example:

Patient diagnosed with congenital hydrocephalus, confirmed by MRI. Shunt placement surgery planned.

Poor Documentation Example:

Patient has a brain condition.

Common Documentation Errors:

  • âš Not documenting the specific type of malformation.
  • âš Not including associated conditions or complications.
  • âš Not documenting the impact on the patient's health status.
  • âš Not documenting surgical procedures.

Range Statistics

8
Total Codes
44
Billable
Complexity:
Medium
Primary Use:Clinical Documentation
Chapter:17

Coding Complexity

Medium
Complexity Rating

Coding for congenital malformations of the nervous system can be complex due to the need to understand the specific type of malformation, identify associated conditions or complications, and know when to use additional codes. However, with careful review of the documentation and a solid understanding of the ICD-10 guidelines, these codes can be applied accurately.

Key Factors:
  • â–¸Understanding the specific type of malformation.
  • â–¸Identifying associated conditions or complications.
  • â–¸Knowing when to use additional codes.
  • â–¸Understanding when not to use these codes.

Specialty Focus

These codes are primarily used in pediatric neurology, neurosurgery, and neonatology. They are critical for documenting congenital malformations, planning treatment, tracking outcomes, and conducting research.

Primary Specialties:
Pediatric Neurology
40%
Neurosurgery
30%
Neonatology
30%
Clinical Scenarios:
  • • A newborn diagnosed with spina bifida.
  • • A child undergoing surgery for congenital hydrocephalus.
  • • A patient with a history of anencephaly impacting current health status.
  • • A patient being treated for complications related to a congenital nervous system malformation.

Resources & References

Several resources are available for coding congenital malformations of the nervous system, including the official ICD-10-CM guidelines, clinical reference books, and educational materials from professional coding organizations.

Official Guidelines:

  • ICD-10-CM Official Guidelines for Coding and Reporting
  • American Health Information Management Association (AHIMA) Coding Guidelines
  • Centers for Medicare & Medicaid Services (CMS) ICD-10 Provider Resources

Clinical References:

  • Pediatric Neurology textbook
  • Neurosurgery textbook

Educational Materials:

  • American Academy of Professional Coders (AAPC) ICD-10 Training
  • AHIMA ICD-10 Training

Frequently Asked Questions

Can I use a Q00-Q07 code for a condition acquired after birth?

No, these codes are specifically for congenital malformations, which are present at birth. Acquired conditions should be coded with the appropriate codes from other sections of the ICD-10.