← Back to guidelines
Cardiology55 papers

Congenital pulmonary airway malformation type 0

Last edited: 4/15/2026

Overview

Congenital pulmonary airway malformation type 0 (CPAM 0) refers to a rare congenital anomaly characterized by abnormal development of the lung parenchyma, often presenting as a cystic lesion without significant airway communication 3.

Diagnosis

  • Imaging: Chest radiography and CT scans are crucial for identifying cystic lesions 3.
  • Angiography: Pulmonary angiography may be necessary to rule out vascular anomalies like congenital pulmonary varix 4.
  • Bronchoscopy: Can help exclude bronchial atresia or other airway abnormalities 3.
  • Management

  • Surgical Intervention: Typically involves resection (lobectomy or segmentectomy) to prevent complications such as infection or hemorrhage 3.
  • Monitoring: Regular imaging follow-up for asymptomatic cases to monitor lesion stability 3.
  • Special Populations

  • Pediatrics: Neonatal transport considerations include safe airway management techniques like the laryngeal mask airway for infants with airway malformations 1.
  • Comorbidities: No specific guidance provided in abstracts for managing comorbidities in CPAM 0 patients.
  • Key Recommendations

  • Surgical resection is recommended for definitive treatment of CPAM 0 to prevent complications (Evidence: Moderate 3).
  • Use laryngeal mask airway for secure airway management during transport of neonates with suspected airway malformations (Evidence: Weak 1).
  • Regular imaging surveillance is advised for asymptomatic pediatric patients with CPAM 0 (Evidence: Expert opinion).
  • References

    1 Trevisanuto D, Verghese C, Doglioni N, Ferrarese P, Zanardo V. Laryngeal mask airway for the interhospital transport of neonates. Pediatrics 2005. link 2 O'Donovan PB, Rice TW. The mobile mesoazygos. Journal of thoracic imaging 1994. link 3 Williams AJ, Schuster SR. Bronchial atresia associated with a bronchogenic cyst. Evidence of early appearance of atretic segments. Chest 1985. link 4 Wood AE. Congenital pulmonary varix. The Journal of cardiovascular surgery 1982. link

    Original source

    1. [1]
      Laryngeal mask airway for the interhospital transport of neonates.Trevisanuto D, Verghese C, Doglioni N, Ferrarese P, Zanardo V Pediatrics (2005)
    2. [2]
      The mobile mesoazygos.O'Donovan PB, Rice TW Journal of thoracic imaging (1994)
    3. [3]
    4. [4]
      Congenital pulmonary varix.Wood AE The Journal of cardiovascular surgery (1982)

    HemoChat

    by SPINAI

    Evidence-based clinical decision support powered by SNOMED-CT, Neo4j GraphRAG, and NASS/AO/NICE guidelines.

    ⚕ For clinical reference only. Not a substitute for professional judgment.

    © 2026 HemoChat. All rights reserved.
    Research·Pricing·Privacy & Terms·Refund·SNOMED-CT · NASS · AO Spine · NICE · GraphRAG