Paeds · respiratory-sleep-and-airway
Congenital lung and airway malformations
Also known as Congenital lung malformations · Congenital thoracic malformations · Congenital pulmonary airway malformation (CPAM/CCAM) · Bronchopulmonary sequestration · Congenital lobar emphysema · Bronchogenic cyst
Fellowship guide to congenital lung and airway malformations — the CPAM, sequestration, lobar emphysema, bronchogenic cyst and bronchial atresia spectrum, how antenatal detection has rewritten the epidemiology, the two questions that drive management (is it causing trouble now, and does an asymptomatic lesion need surgery or surveillance?), and the malignancy risk that keeps the resection debate alive.
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Target exams
Red flags
- A fetus with a large lung lesion, mediastinal shift and developing hydrops is heading for fetal demise and needs urgent tertiary fetal-medicine referral for steroids, drainage or fetal surgery
- A neonate with respiratory distress and a hyperlucent thorax may have congenital lobar emphysema or a tension cyst — positive-pressure ventilation can rapidly over-distend the lesion and precipitate cardiorespiratory collapse
- A congenital diaphragmatic hernia can look identical to a cystic lung lesion on the antenatal scan, so the diaphragm must be positively confirmed before a lesion is dismissed as benign lung
- Recurrent pneumonia striking the same lobe is a congenital malformation until a chest CT proves otherwise, not a run of bad luck to be met with another antibiotic course
- A cystic lung lesion can harbour or evolve into pleuropulmonary blastoma, so an atypical or family-clustered lesion mandates DICER1 consideration and pathological review rather than complacent watching
- Never accept a normal chest radiograph as excluding a congenital lung malformation — many lesions are invisible on plain film and are only defined by CT
Life stages
Care settings
Clinical exam formats
Board mappings
- Respiratory Medicine
- Neonatal and Perinatal Medicine
- Congenital lung and airway malformation recognition
- Paediatric respiratory and sleep medicine competencies
- Diagnosis and multidisciplinary management of congenital thoracic malformations
- Current PREP curriculum — antenatal-to-postnatal continuity of care
- Clinical Applications
- Interpreting antenatal and postnatal imaging of congenital lung lesions
- Long and Short Cases
- Respiratory scenarios and the child with a congenital lung lesion
- 1. Good clinical care: Managing complexity and uncertainty
- Respiratory: Evaluates and manages the child with a congenital lung malformation
- Foundation of Practice (FOP)
- Applied Knowledge in Practice (AKP)
- Respiratory and neonatal medicine
- Clinical
- Respiratory assessment and management
- Communication and management planning with families
- General Pediatrics Content Outline — Respiratory system
- Congenital malformations of the lung and airway
- General Pediatrics EPA: Counsel families on an antenatally detected anomaly
- Patient Care: Evaluation of the child with a congenital lung lesion
- Medical Knowledge: Paediatric congenital thoracic and airway malformations
- Systems-Based Practice: coordinated fetal, neonatal and surgical care
- Medical Expert
- Paediatric respiratory assessment and congenital malformation management
- Pediatrics Core EPA — Assessing and coordinating care for a congenital anomaly
Overview & Definition
A healthy-looking newborn is reviewed on the postnatal ward because the twenty-week scan showed a bright patch in one lung. The baby feeds well, breathes comfortably, and looks entirely normal, yet the parents have spent half the pregnancy fearing the worst. The paediatric task is to explain what the lesion is, decide what imaging and follow-up it needs, and give a straight answer about whether an operation is coming — and this scenario, the well baby with an antenatal finding, is now the commonest way these lesions present. [1] [8]
Congenital lung and airway malformations are abnormalities of lung or large-airway development that arise before birth and range from a tiny incidental cyst to a life-threatening mass that fills the chest. The named lesions overlap in their embryology and imaging, and hybrid forms that combine features of two lesions are common, so it is more useful to treat them as one spectrum with shared principles than as rigidly separate diseases. [1] [2]
What has genuinely changed the topic is antenatal ultrasound. A generation ago most lesions declared themselves through neonatal distress or recurrent childhood infection; today the majority are detected in an asymptomatic fetus and followed to a planned, calm postnatal pathway. This shift explains why so much of the modern literature and so many of the hardest questions are about the child who has no symptoms at all. [8] [1]
References12ShowHide
- [1]Pederiva F, Rothenberg SS, Hall N, et al Congenital lung malformations. Nat Rev Dis Primers, 2023.PMID 37919294
- [2]Dehner LP, Schultz KAP, Hill DA Congenital Pulmonary Airway Malformations With a Reconsideration and Current Perspective on the Stocker Classification. Pediatr Dev Pathol, 2023.PMID 37334833
- [3]Thorburn C, Kattini C, El Hafid M, et al The safety of conservative management of asymptomatic congenital pulmonary airway malformations (CPAMs) in children: A systematic review. J Pediatr Surg, 2026.PMID 41643769
- [4]Lambrecht S, Elrod J, Thater G, et al Long-term outcomes after operative versus conservative management of congenital thoracic malformations: a propensity-matched cohort study. Pediatr Surg Int, 2026.PMID 42043561
- [5]Mohamed SF, Abouegla M, Abouzeid M, et al Efficacy of lobectomy versus segmentectomy for congenital lung malformations: a systematic review and meta-analysis. Pediatr Surg Int, 2026.PMID 41879907
- [6]Muntean A, Marsland L, Sikdar O, et al Neonatal Surgery for Congenital Lung Malformations: Indications, Outcomes and Association With Malignancy. J Pediatr Surg, 2025.PMID 40031114
- [7]Peranteau WH, Boelig MM, Khalek N, et al Effect of single and multiple courses of maternal betamethasone on prenatal congenital lung lesion growth and fetal survival. J Pediatr Surg, 2016.PMID 26526208
- [8]Bush A Prenatal presentation and postnatal management of congenital thoracic malformations. Early Hum Dev, 2009.PMID 19758773
- [9]Kontopoulos E, Quintero LF, Quintero Kontopoulos A, et al Mathematical reappraisal of the congenital cystic adenomatoid malformation volume ratio. J Matern Fetal Neonatal Med, 2026.PMID 42010266
- [10]Wagner ML, Peiro JL, Rymeski BA, et al Long-term outcomes of congenital high airway obstruction syndrome (CHAOS) at a single comprehensive fetal center. Fetal Diagn Ther, 2026.PMID 42213638
- [11]Hong F, Cai W, Li J, et al Pulmonary sequestration with a rare renal artery subtype: a comprehensive clinicopathological analysis of 20 pediatric cases. BMC Pulm Med, 2026.PMID 42046053
- [12]Abboud F, Muhammad AA, Ali S, et al Congenital Lobar Emphysema in an Infant Presenting With Persistent Cough and Progressive Respiratory Symptoms: A Case Report. Clin Case Rep, 2026.PMID 42063629