Summary: | Congenital lung malformations rarely occur but could represent an important cause of respiratory distress in the newborn. As the development of the respiratory tract occurs according to a strict timetable, abnormalities present at birth may be accurately dated to disturbances of intercellular relationships that have occurred at fixed points in intrauterine life. Major structural abnormalities of the respiratory tract are usually fatal or frequently diagnosed before birth or early in postnatal life. Other developmental anomalies may not manifest themselves until much later in life. Antenatal diagnosis by ultrasound scan permits early recognition and thus adequate management. After birth, thoracic-computed tomography is the most useful diagnostic tool. Management of the lesions is based on the characteristics of the lesion and the clinical status of the patient. The pathogenesis, clinical presentation, diagnostic tools, and management options of the important congenital lung malformations are briefly reviewed.
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