Congenital lung malformations

J Bras Pneumol. 2011 Mar-Apr;37(2):259-71. doi: 10.1590/s1806-37132011000200017.
[Article in English, Portuguese]


Congenital lung malformations are rare and vary widely in their clinical presentation and severity, depending mostly on the degree of lung involvement and their location in the thoracic cavity. They can manifest at any age and can be the source of significant morbidity and mortality in infants and children. Individuals with congenital lung malformations can present with respiratory symptoms at birth or can remain asymptomatic for long periods. Recently, there has been an increase in the early diagnosis of these malformations, a change that is attributable to the routine use of prenatal ultrasound. The clinical manifestation of these malformations varies from respiratory distress in the immediate postnatal period to an incidental finding on chest X-rays. Early diagnosis and prompt treatment offer the possibility of absolutely normal lung development. The treatment of asymptomatic patients with lung malformations is controversial, because the prognosis of these diseases is unpredictable. The management of these lesions depends on the type of malformation and symptoms. Because of the risk of complications, most authors recommend resection of the lesion at the time of diagnosis. Lobectomy is the procedure of choice and yields excellent long-term results. This article describes the principal congenital lung malformations, their diagnosis, and the controversies regarding treatment.

Publication types

  • Review

MeSH terms

  • Arteriovenous Malformations / diagnosis
  • Bronchopulmonary Sequestration / diagnosis
  • Child
  • Cystic Adenomatoid Malformation of Lung, Congenital / diagnosis
  • Early Diagnosis
  • Humans
  • Infant
  • Lung / abnormalities*
  • Lung / blood supply
  • Lung Diseases / congenital
  • Lung Diseases / diagnosis*
  • Pulmonary Emphysema / congenital
  • Pulmonary Emphysema / diagnosis