CASE 39

Clinical Presentation

A 6-month-old presents with focal wheezing and mild respiratory distress.

Figure 39A

Radiologic Findings

Frontal (Fig. 39A1) and lateral (Fig. 39A2) conventional chest radiographs demonstrate focal lucency and overinflation of the left upper lobe with mild shift of mediastinal structures to the right.

Figure 39B Axial CT images of the child in Fig. 39A demonstrate left upper lobe lucency, attenuated vascularity, and overinflation, consistent with CLE.

Diagnosis

Congenital lobar emphysema (CLE)

Differential Diagnosis

• Bronchial atresia
  
• Foreign body
  
• Focal bronchomalacia/stenosis
  
• Focal extrinsic compression on left upper lobe bronchus, for example, bronchogenic cyst, vascular anomaly
  
• Endobronchial lesion, mucous plug

Discussion

Background

The definition of CLE is pathologic overdistention of a lobe on a congenital basis. It is one of the most common congenital pulmonary abnormalities. Despite its name, there is no true emphysema seen at pathology; that is, there is no alveolar wall destruction. Many have opted, therefore, to call this entity by the name of “congenital lobar overinflation.”

Etiology

CLE may occur secondary to a variety of causes. In approximately half of cases, no demonstrable cause can be found.

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