Congenital lobar emphysema (also known as congenital lobar overinflation) is an anomaly or malformation of fetal lung development. During lung growth in the fetal stage in the womb there is obstruction to the baby’s airway which leads to an enlarged or an overinflated lobe of a lung. The overinflation could be mild to severe. The overinflated lobe is not functional and does not help in breathing. In addition, the overinflated lobe compresses the other normal portion of the lung.
What are the signs and symptoms of Congenital Lobar Emphysema?
The signs and symptoms of congenital lobar emphysema are variable and depend on the amount of the lung which is affected. In mild cases babies might be having no symptoms at all. They are usually detected when a child gets an XRAY for some other reason. In moderate cases babies have fast breathing, trouble breathing, chest in-drawing and poor growth. Some children with severe problems can get sick; they turn blue (cyanosis), extreme difficulty in breathing and are unable to feed.
What are the other diagnoses that need to be considered in children suspected to have Congenital Lobar Emphysema?
There can be various other lung problems that might be confused with Congenital Lobar Emphysema. Lung collapse and air leak or pneumothorax due to infection are usually confused with Congenital Lobar Emphysema. Other other congenital lung problems like lung hypoplasia, congenital diaphragmatic hernia and congenital cystic adenomatoid malformation (CCAM) can also mimic Congenital Lobar Emphysema.
What are the investigations required for diagnosis of Congenital Lobar Emphysema?
Congenital Lobar Emphysema is usually suspected in babies with respiratory distress and XRAY suggesting a portion of the lung which is expanded (appears more black on the XRAY film). As sometimes diagnosis can be confused with other congenital lung problems and lung infections a CT scan of the chest is required to make a proper diagnosis. Other investigations like an ECHO cardiography is done to look at the heart of the baby.
How is Congenital Lobar Emphysema treated?
There is no medical treatment for Congenital Lobar Emphysema. Surgery is the only treatment option for babies with Congenital Lobar Emphysema. The affected lobe needs to be removed. The timing of the surgery depends on the condition of the baby. In babies with respiratory problems early surgery is recommended. This would improve the baby’s symptoms immediately and also allow the other normal lobes of the lungs to expand. Early surgery also avoids further lung infections. Most babies with Congenital Lobar Emphysema do extremely well and have a normal lung function.
