Q20-Q28
Medium Complexity

Congenital malformations of eye, ear, face and neck

Primary Specialty: Pediatrics
Last Updated: 2025-09-10

ICD-10 Codes (108)

101 billable
0 category headers
Q21
Congenital malformations of cardiac septa
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Q21.0
Billable
Ventricular septal defect
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Q21.1
Billable
Atrial septal defect
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Q21.10
Billable
Atrial septal defect, unspecified
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Q21.11
Billable
Secundum atrial septal defect
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Q21.12
Billable
Patent foramen ovale
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Q21.13
Billable
Coronary sinus atrial septal defect
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Q21.14
Billable
Superior sinus venosus atrial septal defect
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Q21.15
Billable
Inferior sinus venosus atrial septal defect
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Q21.16
Billable
Sinus venosus atrial septal defect, unspecified
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Q21.19
Billable
Other specified atrial septal defect
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Q21.2
Billable
Atrioventricular septal defect
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Q21.20
Billable
Atrioventricular septal defect, unspecified as to partial or complete
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Q21.21
Billable
Partial atrioventricular septal defect
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Q21.22
Billable
Transitional atrioventricular septal defect
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Q21.23
Billable
Complete atrioventricular septal defect
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Q21.3
Billable
Tetralogy of Fallot
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Q21.4
Billable
Aortopulmonary septal defect
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Q21.8
Billable
Other congenital malformations of cardiac septa
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Q21.9
Billable
Congenital malformation of cardiac septum, unspecified
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Q22
Congenital malformations of pulmonary and tricuspid valves
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Q22.0
Billable
Pulmonary valve atresia
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Q22.1
Billable
Congenital pulmonary valve stenosis
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Q22.2
Billable
Congenital pulmonary valve insufficiency
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Q22.3
Billable
Other congenital malformations of pulmonary valve
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Q22.4
Billable
Congenital tricuspid stenosis
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Q22.5
Billable
Ebstein's anomaly
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Q22.6
Billable
Hypoplastic right heart syndrome
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Q22.8
Billable
Other congenital malformations of tricuspid valve
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Q22.9
Billable
Congenital malformation of tricuspid valve, unspecified
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Q23
Congenital malformations of aortic and mitral valves
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Q23.0
Billable
Congenital stenosis of aortic valve
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Q23.1
Billable
Congenital insufficiency of aortic valve
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Q23.2
Billable
Congenital mitral stenosis
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Q23.3
Billable
Congenital mitral insufficiency
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Q23.4
Billable
Hypoplastic left heart syndrome
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Q23.8
Billable
Other congenital malformations of aortic and mitral valves
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Q23.81
Billable
Bicuspid aortic valve
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Q23.82
Billable
Congenital mitral valve cleft leaflet
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Q23.88
Billable
Other congenital malformations of aortic and mitral valves
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Q23.9
Billable
Congenital malformation of aortic and mitral valves, unspecified
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Q24
Other congenital malformations of heart
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Q24.0
Billable
Dextrocardia
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Q24.1
Billable
Levocardia
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Q24.2
Billable
Cor triatriatum
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Q24.3
Billable
Pulmonary infundibular stenosis
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Q24.4
Billable
Congenital subaortic stenosis
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Q24.5
Billable
Malformation of coronary vessels
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Q24.6
Billable
Congenital heart block
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Q24.8
Billable
Other specified congenital malformations of heart
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Q24.9
Billable
Congenital malformation of heart, unspecified
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Q25
Congenital malformations of great arteries
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Q25.0
Billable
Patent ductus arteriosus
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Q25.1
Billable
Coarctation of aorta
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Q25.2
Billable
Atresia of aorta
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Q25.21
Billable
Interruption of aortic arch
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Q25.29
Billable
Other atresia of aorta
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Q25.3
Billable
Supravalvular aortic stenosis
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Q25.4
Billable
Other congenital malformations of aorta
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Q25.40
Billable
Congenital malformation of aorta unspecified
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Q25.41
Billable
Absence and aplasia of aorta
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Q25.42
Billable
Hypoplasia of aorta
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Q25.43
Billable
Congenital aneurysm of aorta
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Q25.44
Billable
Congenital dilation of aorta
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Q25.45
Billable
Double aortic arch
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Q25.46
Billable
Tortuous aortic arch
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Q25.47
Billable
Right aortic arch
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Q25.48
Billable
Anomalous origin of subclavian artery
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Q25.49
Billable
Other congenital malformations of aorta
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Q25.5
Billable
Atresia of pulmonary artery
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Q25.6
Billable
Stenosis of pulmonary artery
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Q25.7
Billable
Other congenital malformations of pulmonary artery
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Q25.71
Billable
Coarctation of pulmonary artery
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Q25.72
Billable
Congenital pulmonary arteriovenous malformation
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Q25.79
Billable
Other congenital malformations of pulmonary artery
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Q25.8
Billable
Other congenital malformations of other great arteries
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Q25.9
Billable
Congenital malformation of great arteries, unspecified
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Q26
Congenital malformations of great veins
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Q26.0
Billable
Congenital stenosis of vena cava
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Q26.1
Billable
Persistent left superior vena cava
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Q26.2
Billable
Total anomalous pulmonary venous connection
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Q26.3
Billable
Partial anomalous pulmonary venous connection
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Q26.4
Billable
Anomalous pulmonary venous connection, unspecified
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Q26.5
Billable
Anomalous portal venous connection
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Q26.6
Billable
Portal vein-hepatic artery fistula
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Q26.8
Billable
Other congenital malformations of great veins
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Q26.9
Billable
Congenital malformation of great vein, unspecified
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Q27
Billable
Other congenital malformations of peripheral vascular system
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Q27.0
Billable
Congenital absence and hypoplasia of umbilical artery
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Q27.1
Billable
Congenital renal artery stenosis
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Q27.2
Billable
Other congenital malformations of renal artery
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Q27.3
Billable
Arteriovenous malformation (peripheral)
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Q27.30
Billable
Arteriovenous malformation, site unspecified
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Q27.31
Billable
Arteriovenous malformation of vessel of upper limb
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Q27.32
Billable
Arteriovenous malformation of vessel of lower limb
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Q27.33
Billable
Arteriovenous malformation of digestive system vessel
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Q27.34
Billable
Arteriovenous malformation of renal vessel
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Q27.39
Billable
Arteriovenous malformation, other site
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Q27.4
Billable
Congenital phlebectasia
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Q27.8
Billable
Other specified congenital malformations of peripheral vascular system
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Q27.9
Billable
Congenital malformation of peripheral vascular system, unspecified
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Q28
Other congenital malformations of circulatory system
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Q28.0
Billable
Arteriovenous malformation of precerebral vessels
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Q28.1
Billable
Other malformations of precerebral vessels
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Q28.2
Billable
Arteriovenous malformation of cerebral vessels
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Q28.3
Billable
Other malformations of cerebral vessels
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Q28.8
Billable
Other specified congenital malformations of circulatory system
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Q28.9
Billable
Congenital malformation of circulatory 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 Q20-Q28 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 category Q20-Q28 encompasses congenital malformations of the eye, ear, face, and neck. These codes are used to document congenital conditions, which are present from birth. This range includes a variety of conditions such as congenital malformations of eyelid, lacrimal apparatus and orbit, congenital malformations of ear causing hearing impairment, cleft lip and cleft palate, and other congenital malformations of face and neck.

Key Usage Points:

  • •Always code to the highest level of specificity.
  • •Use additional codes to identify any associated conditions.
  • •If a patient has multiple congenital malformations, each should be coded separately.
  • •For newborns, also use a code from Z38, Liveborn infants, to identify birth status.
  • •Remember to code also any associated syndromes.

Coding Guidelines

When to Use:

  • ✓When a patient presents with a congenital malformation of the eye, ear, face, or neck.
  • ✓When a patient has a history of a congenital malformation that impacts their current health status.
  • ✓When a patient's congenital malformation is being surgically corrected.
  • ✓When a patient's congenital malformation is causing other health complications.

When NOT to Use:

  • ✗When a patient's condition is acquired, not congenital.
  • ✗When a patient's condition is not related to the eye, ear, face, or neck.
  • ✗When a patient's condition is not a malformation, but a different type of disorder.
  • ✗When the condition is not present, but there is a family history of it.

Code Exclusions

Always check the ICD-10 manual for the latest exclusions as they can change with each update.

Documentation Requirements

Proper documentation for congenital malformations of the eye, ear, face, and neck should include specific details about the condition. This includes the exact malformation, its location, any related conditions or complications, and any surgical procedures performed or planned.

Clinical Information:

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

Supporting Evidence:

  • •Medical history and physical examination findings
  • •Imaging studies
  • •Surgical reports
  • •Consultation notes
Good Documentation Example:

Patient has a congenital malformation of the right ear causing hearing impairment. The malformation is a microtia. No related conditions or complications at this time. Surgical correction is planned.

Poor Documentation Example:

Patient has a malformed ear.

Common Documentation Errors:

  • âš Not specifying the exact type of malformation
  • âš Not indicating the location of the malformation
  • âš Not documenting related conditions or complications
  • âš Not documenting surgical procedures

Range Statistics

9
Total Codes
101
Billable
Complexity:
Medium
Primary Use:Clinical Documentation
Chapter:17

Coding Complexity

Medium
Complexity Rating

Coding for congenital malformations of the eye, ear, face, and neck can be moderately complex. It requires a detailed understanding of the specific malformations, related conditions, and any surgical procedures. Additionally, guidelines for these codes can change, requiring coders to stay updated.

Key Factors:
  • â–¸Understanding the specific type of malformation
  • â–¸Identifying any related conditions or complications
  • â–¸Coding any surgical procedures
  • â–¸Keeping up with changes in coding guidelines

Specialty Focus

These codes are primarily used in pediatrics, otolaryngology (ENT), and ophthalmology. They are also relevant for plastic surgeons who perform corrective surgeries.

Primary Specialties:
Pediatrics
40%
Otolaryngology
30%
Ophthalmology
20%
Plastic Surgery
10%
Clinical Scenarios:
  • • A newborn diagnosed with cleft lip and palate.
  • • A child with congenital glaucoma.
  • • An adult seeking correction for a congenital ear malformation causing hearing loss.
  • • A patient with Goldenhar syndrome, which includes several face and neck malformations.

Resources & References

Several resources are available for coding congenital malformations of the eye, ear, face, and neck. These include the official ICD-10 guidelines, clinical references, and educational materials.

Official Guidelines:

  • ICD-10-CM Official Guidelines for Coding and Reporting
  • American Health Information Management Association (AHIMA) guidelines
  • Centers for Disease Control and Prevention (CDC) guidelines

Clinical References:

  • American Academy of Pediatrics guidelines
  • American Academy of Ophthalmology guidelines
  • American Academy of Otolaryngology–Head and Neck Surgery guidelines

Educational Materials:

  • ICD-10-CM Coding Handbook
  • Medical Coding Pro ICD-10 resources
  • AAPC ICD-10 training materials

Frequently Asked Questions

Can I use these codes for acquired malformations?

No, these codes are specifically for congenital malformations, which are present from birth. Acquired malformations should be coded differently.