PEDIATRICS Vol. 42 No. 1 July 1968, pp. 175-188
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Maltz, D. L.
Right arrow Articles by Nadas, A. S.
Right arrow Search for Related Content
PubMed
Right arrow Articles by Maltz, D. L.
Right arrow Articles by Nadas, A. S.

AGENESIS OF THE LUNG

Presentation of Eight New Cases and Review of the Literature

David L. Maltz 1 and Alexander S. Nadas M.D.1

1 Department of Pediatrics, Harvard Medical School, and Sharon Cardiovascular Unit of the Children's Hospital Medical Center, Boston

One hundred sixty-four cases of agenesis of the lung are reviewed, including 36 not previously reviewed and 8 not previously reported. Agenesis, affecting both hemithoraces and both sexes almost equally, should be separated for physiologic reasons from hypoplasia by the total absence of lung parenchyma, bronchial tree, as well as supporting vasculature. Genetic, teratogenic, and mechanical factors may have a bearing on etiology. Chest roentgenograms suggest the diagnosis initially, but careful bronchographic and angiocardiographic studies are necessary for confirmation. The electrocardiogram is helpful in the differential diagnosis of the patients with dextrocardia. Severe respiratory infections are common in infancy and may lead to pneumonia and death. The condition, however, is not uniformly fatal; 24 of 36 patients reported since 1954 were alive at the time of reporting. There is no evidence at present that resting pulmonary artery hypertension or emphysema develops in patients with agenesis of the lung and an intact cardiovascular system. Diagnostic studies reveal pulmonary artery hypertension in a number of patients with a combination of systemicpulmonary communication and agenesis of the lung. Coexisting abnormalities may involve the skeletal, cardiovascular, gastrointestinal, and genitourinary systems.

Submitted on November 2, 1967
Accepted on January 23, 1968




This article has been cited by other articles:


Home page
 RadiologyHome page
E. Y. Lee, P. M. Boiselle, and R. H. Cleveland
Multidetector CT Evaluation of Congenital Lung Anomalies
Radiology, June 1, 2008; 247(3): 632 - 648.
[Abstract] [Full Text] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
R. G. Fuster, J. A. Buendia, J. Estornell, and J. A. Montero
Complete myocardial revascularization and Dor technique in a case of left lung agenesis
J. Thorac. Cardiovasc. Surg., February 1, 2006; 131(2): 466 - 467.
[Full Text] [PDF]

Home page
 J Ultrasound MedHome page
J. A. Gabarre, A. Galindo Izquierdo, M. Rasero Ponferrada, C. Orbea Gallardo, J. M. Puente Agueda, and P. de la Fuente Perez
Isolated Unilateral Pulmonary Agenesis: Early Prenatal Diagnosis and Long-term Follow-up
J. Ultrasound Med., June 1, 2005; 24(6): 865 - 868.
[Full Text] [PDF]

Home page
 Ann. Thorac. Surg.Home page
G. S. Musleh, P. Fernandez, P. K. Jha, and R. Hasan
Mitral valve repair in a 55-year-old man with left lung agenesis
Ann. Thorac. Surg., May 1, 2004; 77(5): 1810 - 1811.
[Abstract] [Full Text] [PDF]

Home page
 J Ultrasound MedHome page
I. M. Vettraino, A. Tawil, and C. H. Comstock
Bilateral Pulmonary Agenesis: Prenatal Sonographic Appearance Simulates Diaphragmatic Hernia
J. Ultrasound Med., July 1, 2003; 22(7): 723 - 726.
[Full Text] [PDF]

Home page
 Arch Otolaryngol Head Neck SurgHome page
B. L. Bentsianov, N. A. Goldstein, R. Giuste, and G. Har-El
Unilateral Pulmonary Agenesis Presenting as an Airway Lesion
Arch Otolaryngol Head Neck Surg, November 1, 2000; 126(11): 1386 - 1389.
[Abstract] [Full Text] [PDF]

Home page
 CLIN PEDIATRHome page
R. D. Greenwood
Cardiovascular Malformations Associated with Extracardiac Anomalies and Malformation Syndromes: Patterns for Diagnosis
Clinical Pediatrics, March 1, 1984; 23(3): 145 - 151.
[Abstract] [PDF]