Q40-Q44
Medium Complexity

Congenital malformations of the respiratory system

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

ICD-10 Codes (36)

34 billable
0 category headers
Q41
Congenital absence, atresia and stenosis of small intestine
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Q41.0
Billable
Congenital absence, atresia and stenosis of duodenum
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Q41.1
Billable
Congenital absence, atresia and stenosis of jejunum
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Q41.2
Billable
Congenital absence, atresia and stenosis of ileum
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Q41.8
Billable
Congenital absence, atresia and stenosis of other specified parts of small intestine
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Q41.9
Billable
Congenital absence, atresia and stenosis of small intestine, part unspecified
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Q42
Billable
Congenital absence, atresia and stenosis of large intestine
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Q42.0
Billable
Congenital absence, atresia and stenosis of rectum with fistula
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Q42.1
Billable
Congenital absence, atresia and stenosis of rectum without fistula
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Q42.2
Billable
Congenital absence, atresia and stenosis of anus with fistula
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Q42.3
Billable
Congenital absence, atresia and stenosis of anus without fistula
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Q42.8
Billable
Congenital absence, atresia and stenosis of other parts of large intestine
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Q42.9
Billable
Congenital absence, atresia and stenosis of large intestine, part unspecified
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Q43
Billable
Other congenital malformations of intestine
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Q43.0
Billable
Meckel's diverticulum (displaced) (hypertrophic)
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Q43.1
Billable
Hirschsprung's disease
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Q43.2
Billable
Other congenital functional disorders of colon
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Q43.3
Billable
Congenital malformations of intestinal fixation
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Q43.4
Billable
Duplication of intestine
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Q43.5
Billable
Ectopic anus
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Q43.6
Billable
Congenital fistula of rectum and anus
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Q43.7
Billable
Persistent cloaca
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Q43.8
Billable
Other specified congenital malformations of intestine
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Q43.9
Billable
Congenital malformation of intestine, unspecified
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Q44
Congenital malformations of gallbladder, bile ducts and liver
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Q44.0
Billable
Agenesis, aplasia and hypoplasia of gallbladder
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Q44.1
Billable
Other congenital malformations of gallbladder
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Q44.2
Billable
Atresia of bile ducts
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Q44.3
Billable
Congenital stenosis and stricture of bile ducts
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Q44.4
Billable
Choledochal cyst
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Q44.5
Billable
Other congenital malformations of bile ducts
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Q44.6
Billable
Cystic disease of liver
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Q44.7
Billable
Other congenital malformations of liver
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Q44.70
Billable
Other congenital malformation of liver, unspecified
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Q44.71
Billable
Alagille syndrome
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Q44.79
Billable
Other congenital malformations of liver
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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 Q40-Q44 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 Q40-Q44 is dedicated to congenital malformations of the respiratory system. These codes are used to document conditions present at birth that affect the structure and function of the respiratory system. This range includes codes for anomalies like congenital bronchiectasis, congenital malformation of larynx, trachea, bronchus, lung, and other congenital malformations of the respiratory system.

Key Usage Points:

  • Always code to the highest level of specificity within the Q40-Q44 range.
  • Use additional codes to identify any associated conditions or complications.
  • Remember that these codes are for congenital conditions, not acquired ones.
  • Codes in this range can be used across all healthcare settings.
  • Always verify the code in the Tabular List before assigning it.

Coding Guidelines

When to Use:

  • When a patient presents with a congenital malformation of the respiratory system.
  • When a patient's congenital respiratory condition impacts their current health status.
  • When a patient is undergoing treatment for a congenital respiratory malformation.
  • When a patient's congenital respiratory condition is relevant to the care they are receiving.

When NOT to Use:

  • When the condition is not congenital.
  • When the condition is not of the respiratory system.
  • When the condition is an acquired defect of the respiratory system.
  • When the condition has been corrected and no longer impacts the patient's health.

Code Exclusions

Always verify exclusions in the Tabular List before assigning a code.

Documentation Requirements

Proper documentation for codes in the Q40-Q44 range should include a clear statement of the congenital malformation, its location within the respiratory system, and any associated conditions or complications. The documentation should also indicate how the condition impacts the patient's current health status and treatment plan.

Clinical Information:

  • Specific type of congenital malformation
  • Location of the malformation in the respiratory system
  • Impact of the malformation on the patient's health
  • Any associated conditions or complications
  • Treatment plan for the malformation

Supporting Evidence:

  • Medical history records
  • Diagnostic imaging reports
  • Operative reports
  • Clinical notes
Good Documentation Example:

Patient has congenital bronchiectasis affecting both lungs, causing recurrent respiratory infections. Treatment includes physiotherapy and antibiotics.

Poor Documentation Example:

Patient has lung condition.

Common Documentation Errors:

  • Not documenting the specific type of malformation
  • Not indicating the location of the malformation
  • Not documenting associated conditions or complications
  • Not indicating how the malformation impacts the patient's health

Range Statistics

5
Total Codes
34
Billable
Complexity:
Medium
Primary Use:Clinical Documentation
Chapter:17

Coding Complexity

Medium
Complexity Rating

Coding for congenital malformations of the respiratory system can be complex due to the need to accurately identify the specific type and location of the malformation, as well as any associated conditions or complications. However, with careful review of the documentation and a good understanding of the respiratory system, these codes can be assigned accurately.

Key Factors:
  • Determining the specific type of malformation
  • Identifying the location of the malformation
  • Understanding the impact of the malformation on the patient's health
  • Coding associated conditions or complications

Specialty Focus

These codes are primarily used by pediatricians, pulmonologists, and thoracic surgeons. They are also relevant for any healthcare provider treating a patient with a congenital respiratory malformation.

Primary Specialties:
Pediatrics
40%
Pulmonology
30%
Thoracic Surgery
30%
Clinical Scenarios:
  • A newborn diagnosed with congenital laryngomalacia
  • A child with congenital tracheomalacia requiring surgical intervention
  • An adult with a history of congenital bronchiectasis presenting with a respiratory infection
  • A patient with congenital lung hypoplasia undergoing a lung transplant evaluation

Resources & References

Resources for coding congenital malformations of the respiratory system include the ICD-10-CM Official Guidelines for Coding and Reporting, the American Academy of Pediatrics guidelines, and various clinical textbooks and journals.

Official Guidelines:

  • ICD-10-CM Official Guidelines for Coding and Reporting
  • American Academy of Pediatrics guidelines
  • World Health Organization ICD-10 guidelines

Clinical References:

  • Pediatric Pulmonology textbook
  • Thoracic Surgery textbook

Educational Materials:

  • AAPC ICD-10 training
  • AHIMA ICD-10 training

Frequently Asked Questions

Can I use a Q40-Q44 code for an acquired respiratory malformation?

No, these codes are specifically for congenital malformations. For acquired malformations, use the appropriate codes in the J98.2-J98.9 range.