pulmonary agenesis

Infant with
respiratory distress. CXR AP and lateral (above) shows complete opacification of the left hemithorax and tracheal and mediastinal shift to the left and hyperexpansion of the right lung. CXR AP taken during a bronchogram (below left) shows absence of the left mainstem bronchus. AP image from a pulmonary angiogram (below right) shows a normal appearing right main pulmonary artery and absence of the left pulmonary artery.The diagnosis was pulmonary agenesis.
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Late
presentation of unilateral lung agenesis in adulthood. CT pulmonary angiogram showing continuation of patent main pulmonary artery into right pulmonary artery with absent left pulmonary artery (A, B). The heart is shifted into left hemithorax, lying flush with the chest wall with increased pericardial fat (C, D)
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Newborn with
complex congenital heart disease on prenatal US. CXR AP (left) shows the right hemithorax to be smaller than the left hemithorax and there is opacity in the right upper and middle lobes and an aerated right lower lobe. There are multiple hemivertebrae and a butterfly vertebra in the thoracic spine causing a scoliosis convex left. Axial CT with contrast of the chest (above right) shows the right pulmonary artery to be absent along with the right upper lobe. Axial CT (below right) shows absence of the right middle lobe of the lung but the right lower lobe of the lung is present and aerated via a small bronchus off of the trachea (not shown) and perfused by collateral vessels arising from the 7 o’clock position off of the aorta.The diagnosis was right pulmonary agenesis with the hypoplastic right lower lobe receiving its arterial supply from collaterals off of the aorta in a patient who has multiple vertebral body anomalies resulting in congenital scoliosis.
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Newborn with
respiratory distress. CXR AP (above) shows complete opacification of the left hemithorax with mediastinal shift to the left. Axial CT without contrast of the chest (below) show hyperexpansion of the right lung with herniation across the midline, mediastinal shift to the left, and non-visualization of the left mainstem bronchus.The diagnosis was left pulmonary agenesis.
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Late
presentation of unilateral lung agenesis in adulthood. Minimum intensity projection CT images in coronal and sagittal planes show solitary right bronchus and lung (A, B). Mild tracheal narrowing is seen in its upper intrathoracic part (B). There is herniation of hypertrophied right lung across the midline into the left upper hemithorax (C)
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Late
presentation of unilateral lung agenesis in adulthood. CT topogram (A) showing homogenous opacity of left hemithorax with non-bifurcation of trachea with mediastinal shift and contralateral herniation of right lung. CT images in coronal lung window (B), coronal mediastinal window (C), and axial lung window settings (D) showing absent left lung with shift of mediastinal structures into left hemithorax
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Agenesis of
right lung. CT shows an opaque right hemithorax with a shift of the mediastinum towards the ipsilateral side. The right main bronchus is not visualised.
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Late
presentation of unilateral lung agenesis in adulthood. Chest radiograph in frontal projection showing homogenous opacity of left hemithorax with mediastinal shift and contralateral herniation of right lung into left upper hemithorax
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Imaging of
congenital lung diseases presenting in the adulthood: a pictorial review. CT images of pulmonary aplasia and pneumonectomy. Rudimentary blind-ending bronchus can be seen on axial CT image in a 27-year-old asymptomatic female with pulmonary aplasia and helps in the differentiation from pulmonary agenesis (a) (arrow). 3D reformatted images (b, c) show the only left airways and compensatory hyperplasia of the left lung into the right side. The left main bronchial stump is observed on axial CT and 3D reformatted images in a 33-year-old patient with right pneumonectomy (d–f) (arrows). Rib deformities and pleural fluid due to pneumonectomy are depicted on axial CT image (d) (arrow)
insightsimaging.springeropen.com • CC-by-4.0
Beyond
bronchitis: a review of the congenital and acquired abnormalities of the bronchus. Congenital pulmonary and bronchial malformation. a Illustrations demonstrating lobar bronchial malformations resulting in agenesis, aplasia, or hypoplasia as compared to normal. b Frontal chest x-ray demonstrating agenesis of the right mainstem bronchus and right lung (*); note the hyperexpanded left lung crossing the midline (triangle)
insightsimaging.springeropen.com • Open Access
Agenesis of
right lung. CT thorax shows hyperinflated left lung, which pushes the heart into the right hemithorax.
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Agenesis of
right lung. CT thorax coronal view shows left main bronchus originating from the trachea. Right main bronchus is not visualised. (This feature differentiates lung agenesis form lung aplasia).
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Agenesis of
right lung. Axial CT in the lung window shows absence of the right lung and a hyperinflation of the left lung parenchyma which extends into the right hemithorax.
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Agenesis of
right lung. Axial CT in the lung window shows a hyperinflation of the left lung parenchyma.
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Agenesis of
right lung. Contrast-enhanced CT of the chest shows a shift of the mediastinum towards the right side. The right bronchus is not visualized and the left main bronchus appears normal.
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Agenesis of
right lung. Contrast-enhanced CT of the chest shows absence of the right lung and compensatory hyperinflation of the left lung.
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Newborn with
respiratory distress. CXR AP and lateral (above) shows the right hemithorax, which is completely opacified, to be smaller in size than the left hemithorax and there is hyperexpansion of the left lung and mediastinal shift to the right. AP image from a vintage bronchogram (below) shows contrast filling the airways of the left upper lobe, lingula and left lower lobe. There is no contrast in the right hemithorax.The diagnosis was pulmonary agenesis of the right lung.
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Radiopaedia • CC-by-nc-sa 3.0 • de

Pulmonary aplasia is a rare congenital pathology in which there is unilateral or bilateral absence of lung tissue. It is distinguished from pulmonary agenesis, although similar, the main difference being that there is a short-blind ending bronchus in aplasia .

It is usually unilateral, as bilateral pulmonary aplasia is not viable. It is frequently associated with other congenital abnormalities, mainly cardiovascular, and has been reported to occur with VACTERL syndrome .

Clinical presentation

Pulmonary aplasia usually presents with neonatal respiratory distress of variable intensity . In rare cases, it may go unnoticed until later in childhood or adolescence .

It can also be discovered during prenatal ultrasound screening, on which it presents as a hyperechoic hemithorax . The diagnosis is frequently hard to make, but mediastinal shift will usually be prominent.

Pathology

Abnormal blood flow in the dorsal aortic arch during the 4th week of gestation had been hypothesized to cause pulmonary agenesis . The contralateral lung may develop as much as twice more alveoli in response to pulmonary aplasia or agenesis .

Radiographic features 

Plain radiograph

On chest x-ray, it can present as a hemithorax white-out or ipsilateral lung volume loss with ipsilateral shift of mediastinal structures. Contralateral lung hyperinflation is usual with herniation of the normal lung into the contralateral hemithorax. A main ipsilateral bronchus is rarely seen, although CT can demonstrate a rudimentary main bronchus .

CT

CT will confirm the absence of lung parenchyma and mediastinal ipsilateral shift. Contralateral lung hyperinflation is usual with herniation of the normal lung into the contralateral hemithorax. Also, there is an ipsilateral absence of pulmonary artery. It may also show other cardiac congenital malformations and ipsilateral bronchus remnant.

Differential diagnosis

The main differential diagnosis is pulmonary hypoplasia and complete lung atelectasis.

For other causes of hemithorax white-out with ipsilateral mediastinal shift, see

See also

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu pulmonale Aplasie:
Lungenhypoplasie
 
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kongenitaler
Perikarddefekt
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