Radiographic appearance of developmental anomalies of the lung
- Paul Stark, MD
Paul Stark, MD
- Professor of Radiology
- University of California San Diego
- Section Editor
- Nestor L Muller, MD, PhD
Nestor L Muller, MD, PhD
- Section Editor — Pulmonary Imaging
- Professor of Radiology
- University of British Columbia
- Deputy Editors
- Geraldine Finlay, MD
Geraldine Finlay, MD
- Senior Deputy Editor — UpToDate
- Deputy Editor — Pulmonary, Critical Care, and Sleep Medicine
- Associate Professor
- Tufts University School of Medicine
- Susanna I Lee, MD, PhD
Susanna I Lee, MD, PhD
- Deputy Editor — Radiology
- Associate Professor of Radiology
- Harvard Medical School
- Massachusetts General Hospital
Developmental anomalies of the lung can be categorized as bronchopulmonary anomalies, vascular anomalies, or combined anomalies (ie, hybrid pulmonary malformations) [1-3]. They are primarily due to aberrant intrauterine development; abnormalities during postnatal lung development are uncommon. Recognition of developmental anomalies is important because they are frequently mimicked by more ominous acquired abnormalities and they can cause complications during infancy, early childhood, or adulthood.
The radiographic appearances of the major developmental anomalies of the lung are discussed in this topic review. Other aspects of the developmental anomalies (eg, clinical presentation, diagnosis, treatment) are presented separately. (See "Congenital anomalies of the intrathoracic airways and tracheoesophageal fistula" and "Congenital lobar emphysema" and "Congenital pulmonary airway (cystic adenomatoid) malformation" and "Clinical manifestations and diagnosis of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)" and "Pulmonary arteriovenous malformations: Clinical features and diagnostic evaluation in adults" and "Pulmonary arteriovenous malformations: Epidemiology, etiology, and pathology in adults".)
Bronchopulmonary anomalies are caused by insults to the lung bud, the embryonic structure from which airways and parenchyma develop. Examples of bronchopulmonary anomalies include pulmonary agenesis, pulmonary hypoplasia, congenital bronchial atresia, congenital lobar emphysema, congenital cystic adenomatoid malformation (CCAM, now renamed as congenital pulmonary airway malformation [CPAM]), bronchogenic cyst, tracheal bronchus, tracheal diverticulum, and accessory cardiac bronchus.
Agenesis — Agenesis is explained by the failure of lung or lobe, their bronchi, and their vessels to develop. Radiographically, agenesis of a lung appears nearly identical to a pneumonectomy and agenesis of a lobe mimics lobar collapse or a lobectomy and leads to lung hypoplasia (image 1 and image 2) . Associated anomalies of ribs or the spine are common.
Congenital bronchial atresia — Congenital bronchial atresia occurs with absence of a proximal bronchus, while the distal bronchial tree is preserved. The apicoposterior segment of the left upper lobe bronchus is involved most frequently, followed by segmental bronchi of the right upper lobe, right middle lobe, and right lower lobe . Its radiographic appearance is due to mucoid impaction distal to the occluded bronchus and hyperexpansion of the affected pulmonary segment. Specifically, a perihilar mass is surrounded by a halo of lung that is hyperlucent due to collateral ventilation and gas trapping (image 3A-B and image 4 and image 5A-C). Congenital bronchial atresia is discussed in detail separately. (See "Congenital anomalies of the intrathoracic airways and tracheoesophageal fistula", section on 'Bronchial atresia'.)
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- BRONCHOPULMONARY ANOMALIES
- Congenital bronchial atresia
- Congenital lobar emphysema
- Congenital pulmonary airway malformation (CPAM)
- Bronchogenic cyst
- Tracheal bronchus
- Tracheal diverticulum
- Accessory cardiac bronchus
- VASCULAR ANOMALIES
- Proximal interruption of a central pulmonary artery
- Anomalous origin of the left pulmonary artery
- Anomalous pulmonary venous drainage
- Pulmonary arteriovenous malformations
- Cystic hygroma
- COMBINED ANOMALIES
- Hypogenetic lung syndrome
- Bronchopulmonary sequestration
- SUMMARY AND RECOMMENDATIONS