What are Congenital Lung Diseases?
Congenital lung disorders, also known as cystic lung disease or congenital lung malformations, occur prenatally.
We diagnose congenital lung conditions on prenatal ultrasounds. We diagnose about 10 percent of congenital lung disorders at birth, while 14 percent develop by age 15.
Congenital Lung Diseases in Adults
Congenital lung anomalies are also present in adults because they are often symptomatic in childhood or younger age. They have unique symptoms that often mimic other thoracic pathologies and may present acutely and require urgent evaluation.
Misdiagnosis is common. Congenital diseases such as cystic adenomatoid malformation, sequestration, congenital lobar emphysema, and bronchogenic cysts can be misdiagnosed. It is essential to evaluate these diseases for differential diagnosis in adult thoracic pathology and to understand their presentation, potential complications, and management strategies.
Radiological diagnosis is made reliably by CT and MR imaging. The malignant potential is an important consideration. The management of these asymptomatic lesions has always been controversial. However, early removal of these lesions is generally recommended to avoid the potential for later complications. We recommend complete resection.
We recommend the removal of these lesions by thoracoscopic surgery; nonetheless, these congenital lung malformations require well-organized collaboration between pediatric and adult pulmonologists to ensure smooth development from childhood to adulthood.
What are the Different Congenital Lung Disorders?
There are four general types of congenital lung disorders:
Bronchogenic cysts
Bronchogenic cysts usually develop in the bronchus or trachea. Still, they can present in the lower lobes of the lung. They can compromise the respiratory tract if they become infected or get too large.
Congenital cyst
Congenital cystic adenomatoid malformations (CCAMs) occur when a baby's lung tissue grows larger than usual. Babies may have only a single cyst or multiple. They can cause cysts filled with fluid or solid masses in the lungs. Cysts can also impair the alveoli (air sacs in the lungs) functions. Cysts are usually located in one lobe of the lung. (CCAMs are also called congenital pulmonary airway malformations or CPAMs.)
Lobar emphysema
Lobar emphysema is a rare and severe condition that disrupts airflow during breathing. It can cause over-inflation of the lung lobes.
Pulmonary sequestrations
Pulmonary sequestrations are masses of solid lung tissue. Pulmonary sequestrations do not connect to any airway or bloodstream of the lungs. They can be inside the rib cage, inside (intralobular), or outside (extralobular) the lungs.
What are the signs and symptoms of Congenital Lung Diseases?
Children with congenital lung disorders may have the following symptoms.
- Rapid breathing
- Painful or difficult breathing
- Shortness of breath or wheezing,
- Recurrent pneumonia
Problems with congenital lung conditions depend on the malformations' severity and location. Smaller cysts may have no effect, while larger cysts may be fatal.
How Are Congenital Lung Diseases Diagnosed?
We can see congenital lung disorders on regular prenatal ultrasounds. If it is not fatal, we monitor the condition prenatally with ultrasounds, MRIs, or echocardiograms.
Bronchogenic cysts are usually revealed on fetal ultrasound if your fetus has signs of airway obstruction. Chest X-rays detect the condition after birth in newborns.
Lobar emphysema is usually discovered on a chest X-ray after your baby is born. Symptoms such as painful or difficult breathing usually appear soon after birth. The malformation can be seen on an X-ray.
<strong>Pulmonary sequestrations</strong> are noticed late.
Congenital Lung Diseases Treatments
About 10 percent of fetuses with cysts may develop a condition known as fetal hydrops. Fetal hydrops can cause fluid accumulation in the lungs and heart failure.
Doctors use ultrasound to monitor babies with cysts for signs of this condition. The mother may be prescribed corticosteroids to help prevent fetal hydrops. Doctors may perform fetal surgery to fix the problem if steroids don't work.
Most often, babies with congenital lung disorders are asymptomatic at birth.
Congenital lung disease treatment is surgery.
A bronchogenic cyst can compress the baby's airway, so it must be carefully removed. Depending on the location of the cyst and the child's age, the doctor may remove it using a minimally invasive surgery called a thoracoscopy.
Cysts may need surgical aspiration (suction of fluid from the lesion) or surgical mass removal. In lobar emphysema and pulmonary sequestration cases, lobectomy is almost always performed.
Surgeons remove the affected lobes to prevent further damage to the lung. Most doctors recommend waiting until the baby is six months and one year old.
Surgical Technique
We generally prefer closed surgery methods (Thoracoscopic Surgery/VATS) in Pediatric lung diseases. With the development of technology, we prefer closed surgery methods even for children aged 8-12 months. The closed surgery method uses one or two incisions, as in open surgery, by placing an intercostal retractor. One of these incisions is 1 cm, and the other is a two or 3-cm incision. Surgical instruments are advanced through these incisions. The diseased area is removed. Closed surgery; has many advantages, such as less pain, shorter recovery time, and more aesthetics. Even a tiny incision looks very big in open surgery. Patients generally stay in the intensive care unit for one day. Patients are discharged in 3-4 days.
These closed surgeries in children require special high-tech devices and need a highly experienced center.
Frequently Asked Questions About Congenital Lung Diseases
If babies are born with congenital lung disease, treatment is possible only with surgery, not medication. Therefore, good observation and treatment plan is essential after birth.
It is crucial because congenital lung diseases undiagnosed in childhood will form the infrastructure of different conditions later. Therefore, as in every disease, early diagnosis and patient history from childhood to adulthood are vital in congenital lung diseases.
The choice of the hospital is critical in delivering babies with congenital lung diseases. The delivery must be in hospitals with neonatal intensive care units. We recommend hospitalization in the newborn intensive care unit in case of possible problems to prevent possible complications.
Yes, it is operable because they cause problems, especially in the respiratory tract. In this sense, you may need an operation, depending on your doctor's decision. We generally prefer the closed surgery method/thoracoscopic surgery/VATS. Bronchogenic cysts usually develop in the trachea but sometimes also in the lower lobes of the lung, which may affect the surgery method.
Congenital lung diseases can be fatal if left untreated. As with any disease, early diagnosis is critical in congenital lung diseases.
We generally prefer closed surgery methods (Thoracoscopic Surgery/VATS) in Pediatric lung diseases. With the development of technology, we prefer closed surgery methods even for children aged 8-12 months. The closed surgery method uses one or two incisions, as in open surgery, by placing an intercostal retractor. One of these incisions is 1 cm, and the other is a two or 3-cm incision. Surgical instruments are advanced through these incisions. The diseased area is removed. Closed surgery; has many advantages, such as less pain, shorter recovery time, and more aesthetics. Even a tiny incision looks very big in open surgery. Patients generally stay in the intensive care unit for one day. Patients are discharged in 3-4 days.
These closed surgeries in children require special high-tech devices and need a highly experienced center.