Congenital lung malformations happen during the early stages of lung development in utero. They are characterized by cystic lesions that are usually found in just one of the lobes of your baby’s lungs.
Some are caused by blockage of the airway to a lobe and can overinflate, squashing and moving the normal lung tissue next to it. These are called lobular overinflation or pulmonary sequestrations.
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In babies with wheezing, a high-pitched noise is heard when the air passes through the chest. It is a sign that the airways are irritated or blocked, which can decrease oxygen levels in the blood. Wheezing may also be caused by infections, allergies, gastroesophageal reflux disease (GERD), or a lung disorder such as asthma.
Most congenital lung anomalies develop while a baby is in the womb. If they show up on an ultrasound scan, doctors will monitor the pregnancy and often arrange to deliver the baby at a specialist center. They will check the baby’s progress after birth to see if the symptoms are getting better or worse.
The treatment for most of these conditions depends on the size and location of the lung lesion. For example, if a cyst is compressing a child’s airway, doctors will remove it using surgery called thoracoscopy. Smaller cysts may have no effect, while larger ones can be life-threatening and need to be removed early. Children who have these conditions treated as infants tend to do well and experience rapid compensatory lung growth.
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The most common lung malformations that can cause symptoms are cysts and masses that develop on the lungs while your baby is in the womb. These include congenital lobar emphysema, congenital cystic adenomatoid malformations (CCAM or CPAM), and pulmonary sequestrations.
These usually show up during routine prenatal ultrasound scans. But they can also be found after birth, such as when a lung X-ray is taken for another reason or when a child has recurrent infections.
Doctors aren’t sure what causes these types of lung problems. They’re not linked to a particular gene or chromosome and they don’t run in families.
Some lung malformations don’t have any effect on breathing, while others cause a variety of problems. The problem depends on how big the lesion is, where it is in the lungs, and whether or not it connects to any airways or blood vessels. This is why the problems associated with CPAM and other CCAMs differ from those of pulmonary sequestrations, which are usually small, do not touch any airways or blood vessels, and occur on the outside (extralobular) or inside (intralobular) the lungs.
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If a baby has a lung malformation, coughing can be an important symptom. The condition can cause a lot of coughing, which can irritate the lungs and lead to more breathing problems. It can also cause other symptoms, such as leaking of fluid from the lungs; sleeplessness; headaches; dizziness or fainting; leaking of urine; vomiting; and broken ribs.
Most babies with lung lesions are diagnosed before birth during a routine ultrasound. If the lesion is large, other tests may be done. These include a fetal MRI and a fetal echocardiogram, which is a special heart ultrasound.
Lesions in the lungs happen when part of the lung grows abnormally. They can be cystic or solid. Lesions can affect one or more lobes of the lung, and they can be in the lungs’ airways or in the blood supply to the lungs. Some types of lesions include bronchopulmonary sequestration, congenital cystic adenomatoid malformation (CCAM), pulmonary hyperlucent lobes, pulmonary overinflation and foregut duplication cyst. It’s not clear what causes these lesions, but they are not related to any gene or chromosomal abnormality and do not run in families.
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Swallowing involves many nerves and muscles in your mouth, throat area, and food pipe (esophagus). Any disorder or disease that impacts the muscle or nerve cells can make it hard to swallow. It’s called dysphagia. It can cause pain when you swallow (odynophagia) or the sensation that food or liquid is stuck in your throat (globus). Swallowing problems occur in two stages: the oropharyngeal stage and the pharyngo-esophageal stage.
Problems in the oropharyngeal phase include trouble latching on to feed, poor positioning of the nasopharynx during swallowing, delayed initiation of the pharyngo-esophageal swallow, and airway symptoms such as penetration and silent aspiration. In the esophageal stage, problems include acid reflux (GERD), achalasia, or spasms of the lower esophagus.
Treatment depends on the cause of the swallowing problems and how severe they are. Medications may help reduce stomach acid and relieve achalasia. A feeding tube might be needed to provide liquid nutrients if your child can’t chew and swallow. Surgery or radiation might be needed if cancer is causing the swallowing problems. These treatments may require your child to be under anesthesia.
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The type of symptoms you might have depends on what caused the lung abnormality. Some types cause a combination of symptoms. Others happen alone.
One type, called congenital pulmonary airway malformations (CPAM), causes cysts (abnormal closed sacs in the body that contain fluid or gas). This type of lung problem usually affects only one side of the lung. The cysts can be solid masses of extra lung tissue, or they may be made up of fluid-filled spaces (a condition called emphysema).
Another cause of CPA is an open heart defect. These defects force blue blood to mix with red blood in the heart channels that carry oxygen to the rest of the body. Examples include transposition of the great arteries, truncus arteriosus and ventricular septal defect.
Some types of heart problems cause cyanosis. This means the blood isn’t getting enough oxygen. It can be due to problems with the pulmonary valve or the aorta (the main artery that carries blood from your heart). The aorta can be too narrow or have an opening that’s too big (aortic coarctation) or it can have a valve that opens and closes to regulate blood flow (bicuspid aortic valve). The pulmonary valve may be obstructed or leaky.