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Congenital Pulmonary Airway Malformations in Children: Beyond the Pulmonary Cystic Lesion Is There Really an Associated Laryngo‐Tracheal Abnormality?

Pediatric Pulmonology·June 24
Respiratory SystemLimited evidenceCongenital Pulmonary Airway MalformationTracheobronchomalaciaTracheoesophageal FistulaTracheomalaciaScoping ReviewPediatric

Summary

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What was studied

This scoping review (JBI methodology, PRISMA-ScR) mapped all available evidence on laryngotracheal anomalies or upper airway dimensional changes in pediatric patients with congenital pulmonary airway malformations (CPAM), searching PubMed, Scopus, and Web of Science without date restrictions through May 2025.

Key findings

Only 6 studies met inclusion criteria (2 case reports, 1 case series, 3 original articles) from 4,559 records. Reported findings—tracheoesophageal fistula, tracheomalacia, tracheobronchomalacia—were mostly incidental. The single case-control prenatal ultrasound study (41 fetuses with thoracic anomalies including 22 with CPAM vs. 127 controls) found significantly smaller upper airway dimensions in affected fetuses: mean tracheal width 1.79 ± 0.31 mm vs. 2.61 ± 0.32 mm at 20–24 weeks. No definitive causal association between CPAM and laryngotracheal anomalies was established.

Study limitations

- Extremely small evidence base (n=6 studies), mostly case reports and small series with incidentally reported airway findings. - High heterogeneity in study design, diagnostic modalities, and outcome measures prevents any pooled estimate of prevalence or risk. - No standardized prenatal or postnatal airway assessment protocol was used across studies; no formal risk-of-bias appraisal was performed (consistent with scoping review methodology).

Clinical implications

Clinicians managing CPAM should be aware that laryngotracheal anomalies (tracheomalacia, tracheobronchomalacia, tracheoesophageal fistula) may coexist, though current evidence is too sparse to recommend routine airway screening. In complex cases—especially those requiring neonatal surgery—careful prenatal and perinatal airway planning is prudent, as tracheobronchial anomalies can complicate intubation and extubation.

Related Questions

Explore related topics

What is the recommended postnatal airway evaluation protocol for infants diagnosed with CPAM prenatally?How do tracheomalacia and bronchomalacia affect surgical outcomes in children with congenital lung malformations?What prenatal ultrasound findings help distinguish CPAM from congenital high airway obstruction sequence (CHAOS)?

Publication Details

Year
2026
Journal
Pediatric Pulmonology
Sample Size
n=6
Source
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