Congenital lobar overinflation
Updates to Article Attributes
Congenital lobar overinflation (CLO), previously called congenital lobar emphysema, is a congenital lung abnormality that results in progressive overinflation of one or more lobes of a neonate's lung.
On imaging, it classically presents on chest radiographs as a hyperlucent lung segment with overinflation and contralateral mediastinal shift.
Epidemiology
Congenital lobar overinflation is more common in males (M: F = 3:1) 5.
Clinical presentation
Patients typically present with respiratory distress, most commonly in the neonatal period, and usually within the first six months of life 5.
Pathology
In congenital lobar overinflation, a lobe (or more) becomebecomes distended and may or may not have more an overabundance of alveoli. There are many presumed mechanisms for progressive overdistension of a lobe, including obstruction, cartilage deficiency, dysplasia, and immaturity 2. Most cases are idiopathic.
Associations
CLOCongenital lobar overinflation may be associated an aberrant left pulmonary artery 7 and also with congenital heart defects:
Radiographic features
Interestingly, there is quite a pronounced predilection for somecertain lobes:
- left upper lobe: most common, 40-45%
- right middle lobe: 30%
- right upper lobe: 20%
-
may involveinvolving more than a single lobe: 5% - much rarer in the lower lobes
Therefore despite, although the left upper lobe is most commonly affected, the right hemithorax is more commonly affected than the most common side to be affectedleft 6.
Plain radiograph
Immediate postpartum period
The affected lobe tends to appear opaque and homogeneous because of fetal lung fluid or it may show a diffuse reticular pattern that represents distended lymphatic channels filled with fetal lung fluid.
Later findings
- appears as an area of hyperlucency in the lung with
a paucityoligaemia (i.e. paucity of vessels) - mass effect with mediastinal shift and hemidiaphragmatic depression
-
lateral decubitus
filmsfilm with the patient lying on the affected side will show little or no change in lung volume - lateral film may
demonstrateshow posterior displacement of the heart
CT
CT is usually performed to confirm the diagnosis, evaluate the mediastinal vascular structures, and to rule out other abnormalities.
- shows above features in greater detail
- attenuation of vascular structures in affected lobe 4
- may also show compressive atelectasis of
neighbouringneighboring lobes
Treatment and prognosis
Mildly symptomatic patients are usually followed up. A lobectomy can be considered in severe cases 3.
Differential diagnosis
General imaging differential considerations include:
- bronchial atresia: the parenchyma distal to the atretic segment can have air trapping
- congenital
cystic adenomatoid malformation (CCAM) / congenitalpulmonary airway malformation (CPAM) - pulmonary arterial hypoplasia
- pulmonary hypoplasia
- Swyer-James syndrome
See also
- differential for unilateral transradiant hemithorax
-<p><strong>Congenital lobar overinfl</strong><strong>ation (CLO), </strong>previously called <strong>congenital lobar emphysema</strong>, is a <a href="/articles/congenital-lung-abnormality">congenital lung abnormality</a> that results in progressive overinflation of one or more lobes of a neonate's lung. </p><p>On imaging, it classically presents on chest radiographs as a hyperlucent lung segment with overinflation and contralateral mediastinal shift. </p><h4>Epidemiology</h4><p>Congenital lobar overinflation is more common in males (M: F = 3:1) <sup>5</sup>.</p><h4>Clinical presentation</h4><p>Patients typically present with respiratory distress, most commonly in the neonatal period, and usually within the first six months of life <sup>5</sup>.</p><h4>Pathology</h4><p>In congenital lobar overinflation, a lobe (or more) become distended and may or may not have more alveoli. There are many presumed mechanisms for progressive overdistension of a lobe including obstruction, cartilage deficiency, dysplasia, and immaturity <sup>2</sup>. Most cases are idiopathic.</p><h5>Associations</h5><p>CLO may be associated an <a href="/articles/aberrant-left-pulmonary-artery-1">aberrant left pulmonary artery</a> <sup>7</sup> and also with <a href="/articles/congenital-cardiovascular-anomalies">congenital heart defects</a>:</p><ul>- +<p><strong>Congenital lobar overinfl</strong><strong>ation (CLO), </strong>previously called <strong>congenital lobar emphysema</strong>, is a <a href="/articles/congenital-lung-abnormality">congenital lung abnormality</a> that results in progressive overinflation of one or more lobes of a neonate's lung. </p><p>On imaging, it classically presents on chest radiographs as a hyperlucent lung segment with overinflation and contralateral mediastinal shift. </p><h4>Epidemiology</h4><p>Congenital lobar overinflation is more common in males (M: F = 3:1) <sup>5</sup>.</p><h4>Clinical presentation</h4><p>Patients typically present with respiratory distress, most commonly in the neonatal period, and usually within the first six months of life <sup>5</sup>.</p><h4>Pathology</h4><p>In congenital lobar overinflation, a lobe (or more) becomes distended and may or may not have an overabundance of <a title="Alveoli" href="/articles/alveoli">alveoli</a>. There are many presumed mechanisms for progressive overdistension of a lobe, including obstruction, cartilage deficiency, dysplasia, and immaturity <sup>2</sup>. Most cases are idiopathic.</p><h5>Associations</h5><p>Congenital lobar overinflation may be associated an <a href="/articles/aberrant-left-pulmonary-artery-1">aberrant left pulmonary artery</a> <sup>7</sup> and also with <a href="/articles/congenital-cardiovascular-anomalies">congenital heart defects</a>:</p><ul>
-</ul><h4>Radiographic features</h4><p>Interestingly there is quite a pronounced predilection for some lobes:</p><ul>-<li>left upper lobe: most common, 40-45%</li>-<li>right middle lobe: 30%</li>-<li>right upper lobe: 20%</li>-<li>may involve more than a single lobe: 5%</li>- +</ul><h4>Radiographic features</h4><p>Interestingly, there is a pronounced predilection for certain lobes:</p><ul>
- +<li>
- +<a href="/articles/left-upper-lobe">left upper lobe</a>: most common, 40-45%</li>
- +<li>
- +<a href="/articles/right-middle-lobe">right middle lobe</a>: 30%</li>
- +<li>
- +<a href="/articles/right-upper-lobe">right upper lobe</a>: 20%</li>
- +<li>involving more than a single lobe: 5%</li>
-</ul><p>Therefore despite the left upper lobe is most commonly affected, the right hemithorax is the most common side to be affected <sup>6</sup>.</p><h5>Plain radiograph</h5><h6>Immediate postpartum period</h6><p>The affected lobe tends to appear opaque and homogeneous because of fetal lung fluid or it may show a diffuse reticular pattern that represents distended lymphatic channels filled with fetal lung fluid.</p><h6>Later findings</h6><ul>-<li>appears as an area of hyperlucency in the lung with a paucity of vessels</li>- +</ul><p>Therefore, although the left upper lobe is most commonly affected, the right hemithorax is more commonly affected than the left <sup>6</sup>.</p><h5>Plain radiograph</h5><h6>Immediate postpartum period</h6><p>The affected lobe tends to appear opaque and homogeneous because of fetal lung fluid or it may show a diffuse reticular pattern that represents distended lymphatic channels filled with fetal lung fluid.</p><h6>Later findings</h6><ul>
- +<li>appears as an area of hyperlucency in the lung with oligaemia (i.e. paucity of vessels)</li>
-<li>decubitus films lying on the affected side will show little or no change in lung volume</li>-<li>lateral film may demonstrate posterior displacement of the heart</li>- +<li>
- +<a href="/articles/chest-lateral-decubitus-view">lateral decubitus</a> film with the patient lying on the affected side will show little or no change in lung volume</li>
- +<li>lateral film may show posterior displacement of the heart</li>
-<li>may also show compressive atelectasis of neighbouring lobes</li>- +<li>may also show compressive <a href="/articles/lobar-collapse-summary">atelectasis</a> of neighboring lobes</li>
-<li><a href="/articles/congenital-cystic-adenomatoid-malformation">congenital cystic adenomatoid malformation (CCAM) / congenital pulmonary airway malformation (CPAM)</a></li>- +<li><a href="/articles/congenital-cystic-adenomatoid-malformation">congenital pulmonary airway malformation (CPAM)</a></li>
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