Congenital lobar emphysema (CLE) is a rare lung malformation that occurs when air become trapped in the lungs. Infants with CLE have one or more lobes of the lung that are hyperinflated.
An obstruction of one of the main airways of the lungs — called the bronchi — can cause CLE in some newborns. However, experts suggest the condition may have a genetic link. The severity of CLE can vary among people, and some may not experience symptoms until adulthood.
Doctors typically diagnose the condition in the first
Read on to learn more about congenital lobar emphysema.
The exact cause of CLE is not always clear — around
Some infants with CLE have issues with cartilage formation in the bronchi, the passageways into the lungs. This can lead to issues with lung development — they may either underdevelop or develop atypically — which may cause air to stay trapped in the lungs.
Other possible causes of CLE include:
- diseases of the parenchyma, the cells responsible for lung function
- a blockage from inside the bronchi
- a blockage from outside the bronchi
Symptoms may include:
- blue or gray tinge to the skin due to a lack of oxygen
- the chest sinking below the neck or breastbone during breathing
- wheezing
- difficulty feeding or breathing
- frequent respiratory tract infections
- chronic cough
Some conditions with similar symptoms to CLE include:
- bronchial asthma
- pneumonia
- respiratory distress syndrome in infants
- cor pulmonale, or altered function in the right side of the heart that can occur following respiratory disease
- alpha-1 antitrypsin deficiency, a genetic condition that affects the liver and lungs
- tension pneumothorax, when air becomes trapped between the lung and chest wall
- other congenital diseases, such as congenital diaphragmatic hernia or congenital cystic adenomatoid malformation
pneumatocele , when air pockets or cysts develop within the lung parenchyma following injury or trauma- lung hypoplasia, another
rare, congenital condition of the lungs
Considering the above when diagnosing CLE helps doctors determine the best treatment plan.
Diagnosing CLE involves a physical exam of the lungs and medical imaging.
Part of the physical exam involves the doctor tapping the chest to hear sounds within the lungs. The doctor may notice hyper-resonance, which involves a low-pitched, booming sound upon examination. This typically indicates that a particular area of the lung is
Doctors regard a lung CT as the best method for imaging and diagnosing CLE. Another imaging technique that can provide valuable information is an MRI. In infants, MRI can provide detailed images that could potentially evaluate and identify abnormalities.
An ultrasound during pregnancy cannot diagnose CLE. In some cases, ultrasound experts may still notice lung areas with an exaggerated response to sound waves with typical blood flow in the area.
Doctors could also confirm a diagnosis before birth with MRI since it can show more detail in the fetal lung.
Differential diagnosis
Before treating CLE, doctors need to rule out other conditions that may be causing similar symptoms. Many different respiratory diseases may resemble CLE, as mentioned in the section above.
A chest X-ray showing hyperinflated lung lobes can suggest several diagnoses:
- pneumothorax, or collapsed lung
pneumatocele - lung hypoplasia
Performing a differential diagnosis is the process doctors use to make a final diagnosis. They collect data, consider the most likely cause, and eliminate other less likely options.
Once a doctor establishes a diagnosis of CLE, they will choose the most appropriate treatment. These strategies can depend on the severity of the disease.
Conservative treatment
For mild to moderate disease, doctors prescribe conservative treatments, such as oxygen and bronchodilators, to help open the airways and improve breathing.
Follow-up appointments with specialists, such as a respirologist, may be beneficial. Overall conservative treatment can provide favorable results for some.
Learn more about how bronchodilators work.
Surgery
In severe CLE, newborns may require surgery to remove the lung lobe with CLE. Doctors refer to the removal of a lung lobe as a lobectomy, which typically provides
With appropriate treatment, the outlook for CLE is
Sometimes, the condition is so severe it can affect the cardiovascular system, which may affect the prognosis. However, treatments may improve both respiratory and cardiac problems.
Congenital lobar emphysema (CLE) is a rare lung disease where air enters the lungs and becomes trapped. It may be challenging to diagnose CLE before birth. Within the first 6 months of life, newborns can present symptoms of the condition, if not earlier. Doctors diagnose CLE with physical exams and medical imaging.
Doctors manage mild and moderate CLE with conservative treatments. For more severe cases, lobectomy can serve as an effective treatment. With appropriate techniques, the outlook is generally favorable.