Fact-sheet: Congenital lobar overinflation
- Congenital lobar emphysema
Updated on 07/16/2021 at 9:03 AM
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Definition
Congenital lobar hyperinflation, formerly known as congenital lobar emphysema, is a congenital pulmonary anomaly resulting in progressive overinflation of one or more lung lobes in a newborn.
Clinical features
Patients typically present with respiratory distress, most often during the neonatal period, and generally within the first six months of life.
May be associated with an anomalous left pulmonary artery, as well as with congenital heart defects:
- ventricular septal defect.
- patent ductus arteriosus (PDA)
- tetralogy of Fallot (ToF)
Radiography
In the immediate postpartum period:
The affected lobe tends to appear opaque and homogeneous due to retained fetal lung fluid, or it may show a diffuse reticular pattern representing distended lymphatic channels filled with fetal lung fluid.
Late findings:
The affected lobe appears as an area of hyperlucency with oligemia (i.e., paucity of vessels), mass effect with mediastinal shift, and hemidiaphragmatic depression.
On lateral decubitus, little to no change in lung volume.
Marked predilection for certain lobes:
- left upper lobe: most common, 40-45%
- right middle lobe: 30%
- right upper lobe: 20%
CT
Generally performed to confirm the diagnosis, assess mediastinal vascular structures, and exclude other abnormalities.
Generally shows the radiographic features in greater detail, with attenuation of vascular structures in the affected lobe, and may also show compressive atelectasis of adjacent lobes.
Management
In mildly symptomatic forms, simple follow-up is generally proposed.
Surgical resection or lobectomy is considered in severe cases.
Differential diagnosis
- Bronchial atresia: the parenchyma distal to the atretic segment may show air trapping
- Congenital pulmonary airway malformation (CPAM)
- Pulmonary arterial hypoplasia
- Pulmonary hypoplasia
- Swyer-James syndrome
- Filamin A mutation