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Paediatrics: Congenital abnormalities: lung

Congenital cystic adenomatoid malformation (CAM), Congenital lobar emphysema (CLE).

Congenital abnormalities: lung

 

Congenital cystic adenomatoid malformation (CAM)

 

CAM is a congenital malformation of the lung bud characterized by dys-plasia of respiratory epithelium. The majority of CAMs are now diagnosed prenatally by US and high-risk cases will be associated with hydrops. Symptomatic CAMs should be resected.

 

Large CAMs present at birth with respiratory distress that is managed along conventional lines.

 

Small CAMs will be asymptomatic at birth, but may present in early childhood with pulmonary sepsis.

 

Sequestration

 

Pulmonary sequestrations are segments of lung parenchyma with an anomalous blood supply from the aorta. There may be an abnormal bron-chial connection with the foregut or tracheobronchial tree. The majority are detected prenatally and the management is resection.

 

Large sequestrations will present at birth with respiratory distress or with heart failure from high flow through the feeding vessel.

Sequestrations may present in infancy or childhood with pulmonary sepsis.

 

Congenital lobar emphysema (CLE)

 

CLE is an unusual lung bud anomaly characterized by massive air trapping in the emphysematous lobe. This compresses the surrounding normal lung and may result in mediastinal shift.

 

CLE presenting with progressive respiratory distress within the first few weeks or months of life nearly always require lobectomy. In the acute phase positive pressure ventilation may produce rapid worsening of the emphysema.

 

Lobar emphysema identified on CXR in the absence of symptoms will need close follow-up, and surgery may be avoided.

 

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