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    OpenPayerBilling CodesCongenital malformations of the respiratory systemICD-10-CM q33-congenital-malformations-of-lung
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    ICD-10-CM Code For Congenital Malformations Of Lung: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q33 includes congenital structural abnormalities of the lung such as pulmonary hypoplasia and congenital cystic lung lesions that present at birth and may affect respiratory function. These ICD-10-CM diagnosis codes are used to document congenital lung anomalies, capturing disease severity, laterality when applicable, and associated respiratory compromise or complications. Accurate coding within the ICD-10-CM Q33 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM Q33: Congenital Malformations Of Lung Overview

    The diagnoses in this group represent congenital structural abnormalities of the lung and lower respiratory tract present at birth, including pulmonary hypoplasia and congenital cystic lung lesions. These conditions target the respiratory system and can affect lung development, airway function, and gas exchange. Accurate coding is important for appropriate clinical documentation and to support reimbursement for services related to neonatal or pediatric respiratory care.

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    When to Use

    Use a code from this group when a congenital lung malformation is diagnosed clinically or identified by imaging at birth or during infancy. Assign codes that reflect the specific malformation, laterality if documented, and any associated respiratory compromise or complications.

    ICD-10-CM Codes

    9 rows

    ICD-10-CM CodeDescription
    Q33.0Congenital cystic lung
    Q33.1Accessory lobe of lung
    Q33.2Sequestration of lung
    Q33.3Agenesis of lung
    Q33.4Congenital bronchiectasis
    Q33.5Ectopic tissue in lung
    Q33.6Congenital hypoplasia and dysplasia of lung
    Q33.8Other congenital malformations of lung
    Q33.9Congenital malformation of lung, unspecified

    DRG Codes

    5 rows

    DRG CodeDescription
    190Chronic Obstructive Pulmonary Disease with MCC
    191Chronic Obstructive Pulmonary Disease with CC
    192Chronic Obstructive Pulmonary Disease without CC/MCC
    205Other Respiratory System Diagnoses with MCC
    206Other Respiratory System Diagnoses without MCC

    Typical Clinical Scenarios

    • A newborn in the neonatal intensive care unit with severe respiratory distress at birth, asymmetric chest expansion, and imaging showing an absent or underdeveloped lung lobe; the infant requires intubation and further evaluation for congenital pulmonary airway malformation or lung agenesis, prompting assignment of a congenital respiratory malformation code. Typical codes: Q33.0, Q33.1
    • A pediatric patient with recurrent focal pneumonia localized to the same area on chest radiographs and CT demonstrating an isolated congenital pulmonary airway malformation (CPAM) or bronchogenic cyst causing chronic airspace problems; the child is referred for surgical evaluation and the chronic congenital lesion is coded. Typical codes: Q33.2, Q33.8
    • An adult presenting for preoperative evaluation who has a known history of unilateral lung hypoplasia discovered incidentally in childhood and now listed as a comorbidity while undergoing unrelated surgery; the documented congenital malformation of the lung is recorded to capture perioperative risk. Typical codes: Q33.6, Q33.9

    Related Codes

    Q30-CONGENITAL-MALFORMATIONS-OF-NOSE

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

    Q31-CONGENITAL-MALFORMATIONS-OF-LARYNX

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

    Q32-CONGENITAL-MALFORMATIONS-TRACHEA-BRONCHUS

    ICD-10-CM Code For Congenital Malformations Of Trachea And Bronchus: Group Overview

    Q34-OTHER-CONGENITAL-RESPIRATORY-MALFORMATIONS

    ICD-10-CM Code For Other Congenital Malformations Of Respiratory 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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