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Author:
Christopher M Oermann, MD
Literature review current through: Feb 2019. | This topic last updated: Aug 28, 2017.
Males appear to be affected more than females, in a ratio of 3:1 [1]. The reason for
the male predominance is unknown [1,3,5].
overdistention of one or more lobes of the lung [12]. This leads to compression of the
remaining lung tissue and herniation of the affected lobe across the anterior
mediastinum into the opposite chest, causing displacement of the mediastinum.
The different pulmonary lobes are variably affected by CLE. The left upper lobe is
affected most often (40 to 50 percent of cases) [1,3,13,14]. The distribution in right
middle, right upper, and lower lobes is 25 to 35, 20, and 2 to 10 percent, respectively.
CLE affecting multiple lobes is rare.
Progressive respiratory distress develops rapidly in some infants, while others have a
more gradual, insidious onset [1,3-5,7,8,15,16], and some may remain asymptomatic
for years [17,18]. The severity depends upon the size of the affected lobe, the
compression of surrounding lung tissue, and the extent of mediastinal shift.
Infants typically have tachypnea and increased work of breathing, and often have
cyanosis [1,3,4,7,8]. Recurrent pneumonia or poor feeding with failure to thrive are
less frequent presentations that may occur in milder forms.
If the chest radiograph is obtained immediately after birth, the affected lobe may
appear opacified due to retained fetal lung fluid. As the fluid is absorbed and the lobe
becomes filled with air, progressive hyperinflation develops.
Computed tomography (CT) of the chest or magnetic resonance imaging may help
establish the diagnosis of CLE in atypical cases and may demonstrate an intrinsic or
extrinsic source of airway obstruction [16,19,20]. Studies with contrast may also
demonstrate vascular causes of airway compression and resultant emphysematous
changes. Alternatively, echocardiography may identify vascular structures causing
airway compression.
present. The vast majority of lesions are symptomatic and require early resection. For
asymptomatic patients, conservative management usually is appropriate because
they are unlikely to develop complications. By contrast, the management of
asymptomatic patients with other types of congenital abnormalities of the lower
airway is controversial, with arguments made for and against surgical resection
[39,40]. This is because these other lesions may be associated with risks for infection
or malignancy, which is not the case in CLE. (See "Congenital pulmonary airway
(cystic adenomatoid) malformation", section on 'Asymptomatic patients'.)
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