Congential Lung Malformations

Congenital means something is present “at birth.” Congenital lung disorders, also known as cystic lung disease or congenital lung malformations, occur while a baby is still in its mother’s womb. Most congenital lung disorders are discovered during prenatal ultrasounds. About 10 percent of congenital lung disorders are diagnosed at birth, while another 14 percent show up by age 15.

There are four general types of congenital lung disorders:

1. Bronchogenic cysts:

Bronchogenic cysts usually develop on the esophagus or trachea but can also sometimes be found on the lower lobes of the lung. They can compromise airways if they become infected or grow too large.

2. Congenital cystic Adenomatoid Malformation (CCAM)

Congenital cystic adenomatoid malformations (CCAMs) form when a baby’s lung tissue grows more than normal. Babies may have just a single CCAM or several. They can cause cysts that fill with fluid or solid masses in the lungs. CCAMs can also prevent the alveoli (air sacs in the lungs) from developing normally. CCAMs usually form in one lobe of the lung. (CCAMs are sometimes referred to as congenital pulmonary airway malformations or CPAMs.)

3. Congenital Lobar emphysema (CLE)

Lobar emphysema is a rare, serious condition that can interfere with an infant’s airways by trapping airflow during breathing. It can result in overinflation of the lobes of the lung.

4. Pulmonary sequestrations:

Pulmonary sequestrations are solid masses of lung tissue. Pulmonary sequestrations don’t connect to any of the lungs’ airways or blood supply. They can be inside of the lungs (intralobular) or outside (extralobular) in either the chest or stomach.

Signs and Symptoms:

Babies with congenital lung diseases can have fast breathing, chest retractions, recurrent pnuemonias, apneas or when baby stops breathing for few seconds, bluish discouloration of lips, fingers, toes.
These symptoms can be present at birth or may develop later as the chid grows older.

Diagnosis:

is almost always done by radiological methods. In-utero diagnosis is possible during fetal ultrasounds. In newborns and older children, if the pediatric pulmonologist suspects a congenital lung malformation , a Chest X-ray or in some cases a CT scan of chest is done.

Treatment:

Congenital Lung malformations are treated by surgical removal. If the lesion is detected in-utero, and it is non-lethal, pregnancy is continued till term and baby is assessed after birth.