Congenital Lobar Emphysema Flashcards
Congenital lobar emphysema
It is characterized by massive distension and overexpansion of the affected lobe of the lung, usually the left upper lobe or the right middle lobe as a result of air trapping
leads to compression of the surrounding lung as well as the contralateral lung which may cause life-threatening respiratory distress, which if not recognized and treated, may be fatal
CLE primarily involves how many lobe of the lung ?
one lobe of the lung
Which lobe is predominantly involved
upper lobes are predominantly involved
Left upper lobe, 40–42 %.
- Right middle lobe, 34–35 %.
- Right upper lobe, 20–21 %.
Associated anomalies are
Congenital heart disease (PDA, VSD) is the commonest.
− Rib cage defects
− Renal anomalies
Clinical features
acute neonatal respiratory distress
recurrent chest infection
mediastinal shift, hyperresonance, and decreased breath sounds on the affected side.
cough, wheezing, respiratory distress, and cyanosis.
Diagnosis
Antenatal ultrasound shows affected lobe as an overinflated, fluid-filled lobe associated with mediastinal herniation.
• In neonates, the affected lobe may be slightly opacified, rather than lucent, because it is still filled with fluids.
Later - XRAY Large, hyperlucent, emphysematous lobe with attenuated but defined vascularity
There is also compression of the remaining lung on that side or collapse , flattened hemidiaphragm, and widened intercostal spaces.
CT SCAN -
Ventilation perfusion scan
CT scan diagnosis of CLE will show
hyperlucent, hyperexpanded lobe with attenuated but intact vascularity pattern. − Midline sub sternal herniation of the affected lobe.
− Compression of the remaining lung.
− The mediastinum is significantly shifted away from the side of the affected lobe. − Substernal lobar herniation and compression of the remaining lung.
− The cause of obstruction especially extrinsic causes.
Treatment
Total lobectomy
