Congenital Lung Cysts in Infants

Congenital lung cysts in babies form as abnormal air-filled spaces in the lung tissue during development in the womb. The most common type is congenital cystic adenomatoid malformation (CCAM), which may affect respiratory functions and may require intervention immediately after birth.

The question of how congenital lung cysts are diagnosed is critical for early diagnosis. These cysts are usually detected by prenatal ultrasound and detailed with fetal MRI. After birth, computed tomography (CT) and chest X-ray are used to confirm the diagnosis.

The symptoms of congenital lung cysts in babies vary according to the size of the lesion. Large cysts may cause respiratory distress immediately after birth, while small ones may remain asymptomatic. In some cases, infection, air leakage, or mediastinal compression may develop.

The treatment process is planned according to the size of the cyst and the clinical picture. While the cyst is surgically removed in symptomatic cases, regular follow-up may be preferred in asymptomatic cases. Early surgery is effective in reducing the risks of infection and malignancy and generally provides a good prognosis.

What Are Congenital Lung Cysts in Babies?

Congenital Lung Cysts in Babies are cystic lesions that may contain air or fluid and form during the development of lung tissue in the womb. They are most commonly seen as congenital pulmonary airway malformation and bronchogenic cyst. After birth, they may cause respiratory distress, bruising, or recurrent infections. Diagnosis is made by prenatal ultrasonography and postnatal imaging. Treatment may include surgical removal according to the type of lesion and clinical findings.

How can the symptoms of congenital lung cysts in my baby be recognized?

One of the greatest blessings that technology offers us is that we can now detect most of these cases before the baby is even born. During the detailed ultrasonography performed in your pregnancy follow-up, our perinatology specialists may detect an image in the baby’s chest that looks different from normal, a brightness, or a cystic structure. This early diagnosis allows us to be prepared for the postnatal period.

However, sometimes the cysts may be very small or may escape the eye of the ultrasound. Then, the symptoms after birth guide us. Some babies draw an obvious picture as if saying “I’m here” immediately after birth, while others may remain hidden for months or even years without showing any symptoms. This “silent” period may sometimes mislead parents. You may think, “My baby looks perfectly fine, why should I take them to the doctor?” However, the structural disorder inside may be silently preparing to grow or cause problems.

The situations you should pay attention to and that make us suspicious are as follows:

• Rapid breathing
• Retractions in the chest while breathing
• Getting tired quickly during feeding
• Bluish discoloration around the lips
• Frequent recurrent lung infections
• Persistent cough
• Slow weight gain

If your baby has one or more of these symptoms, or if a suspicious condition was seen in the womb, advanced examination is definitely required. While a standard chest X-ray can give us a rough idea here, the method that draws the real map is Computed Tomography (CT). Tomography shows us millimetrically where the cyst is located, its size, and its relationship with neighboring organs.

What risks do untreated congenital lung cysts carry?

The decision moment that families struggle with most is making the decision for surgery for their baby who appears to have no problems at all and is sleeping peacefully. I understand this feeling very well. Questions such as “Should we wait, will it go away?” or “Will it disappear on its own as they grow?” may be on your mind. However, medical facts and the experience of years tell us this: in this group of diseases, waiting usually does nothing except increase the risk.

There are three major categories of risk for the body in leaving these cysts there as they are. As surgeons, we recommend “early and definitive treatment,” namely surgery, to protect your baby from these risks. Our aim is not only to save today but also to secure your child’s adult life.

The risks we may face if left untreated are as follows:

• Recurrent pneumonia attacks
• Formation of a lung abscess
• Infection of the cysts
• Airway obstructions
• Sudden lung collapse
• Severe chest pains
• Respiratory failure
• Development of a malignant tumor

The risk of infection in particular is very high. Since the cysts are not in full connection with the airways, they cannot clear the secretions inside them. This stagnant environment is a perfect pool for bacteria to multiply. The child becomes ill continuously and has to use antibiotics all the time. Each infection causes adhesions in the lung, and this makes the surgery that will later be necessary technically more difficult.

More importantly, although it is a low possibility, there is a risk of cancerous transformation (malignancy) in these cysts at older ages. We know that some rare tumors such as pleuropulmonary blastoma may originate from these cysts. For us surgeons, even a one-in-a-thousand chance that a tissue carries the potential for malignancy is a sufficient and strong reason for it to be removed from the body.

Why is vascular mapping of vital importance in pulmonary sequestration surgery?

Among congenital lung cysts, the type we call “Pulmonary Sequestration” requires completely separate care and attention in terms of surgical planning. There is a very critical anatomical difference that distinguishes this condition from the others, and this difference directly determines the safety of the surgery.

Normally, lung tissue receives blood from the vessel coming from the right side of the heart, which we call the “pulmonary artery.” The pressure in this vessel is relatively low. However, in cases of Pulmonary Sequestration, that diseased piece of lung tissue is disconnected from the normal circulatory system. It meets its blood requirement from an abnormal vessel coming from the “Aorta,” the body’s largest and highest-pressure vessel, or from its branches inside the abdomen.

You can imagine it like this: it is like a house that is not connected to the normal city network and has drawn an illegal line directly from the main dam line. This “illegal line,” that is, the abnormal artery, carries extremely high-pressure blood. Not being aware of the presence of this vessel during surgery or not knowing its location precisely may lead to serious unwanted bleeding. For this reason, in each of our babies with suspected sequestration, we definitely perform “vascular mapping” before the operation.

The methods we use for this mapping are as follows:

• Computed Tomography Angiography (CTA)
• Magnetic Resonance Angiography (MRA)

Thanks to these advanced imaging techniques, as surgeons, we have a road map in our hands before entering the surgery. We see three-dimensionally where that abnormal vessel comes from and the route it follows to enter the cyst. During the surgery, our first task is not to try to remove the cyst or the lung tissue, but to “defuse this bomb,” that is, to find and safely close that high-pressure vessel. Once the vessel is under control, the rest is a standard procedure. That is why vascular mapping is not a luxury for us but a vital necessity.

Should surgery for congenital lung cysts be performed with the closed method?

Years ago, to perform these operations, we used to make large incisions in the chest cage, separate the ribs with metal instruments, and reach the lung that way. This open method, which we call “Thoracotomy,” of course allowed us to do our job, but the cost was a little heavy. In babies, stretching the ribs increased postoperative pain, prolonged the healing process, and could lead to chest deformities in the future.

However, with the development of medicine and technology, we have now made a giant leap. Today, our standard approach is “Minimally Invasive Surgery,” that is, the closed method. In this technique, known medically as “Video-Assisted Thoracoscopic Surgery” (VATS), we enter the baby’s chest through only 2 or 3 tiny holes of a few millimeters. Through one hole we insert a high-resolution camera, and through the others, very thin and delicate instruments.

The comfort this method provides to our babies compared to open surgery is remarkable. Since there are no large muscle incisions and the ribs are not strained, the “pain” factor is reduced to a minimum.

The advantages provided by the closed method (VATS) are as follows:

• Much less postoperative pain
• Shorter hospital stay
• Almost no visible scar cosmetically
• Fast recovery and return to normal life
• Prevention of muscle and skeletal system damage
• Less blood loss
• Reduced risk of infection

Of course, in surgery, there is no such thing as “never.” In some very complex situations where there are extensive adhesions or a serious risk of bleeding, it may be necessary to switch to the open method for the baby’s safety. The only criterion for your surgeon in making this decision is to protect your baby’s life in the safest possible way at that moment. However, in the vast majority of cases, we are able to complete the operation successfully with the closed method.

How much lung tissue is removed during surgery and is lobectomy necessary?

One of the biggest questions in parents’ minds is, “How much of my child’s lung will be removed?” You may feel a concern as if if one part is missing, your child’s life will remain incomplete. This needs to be clarified. Our lungs consist of sections we call “lobes,” three on the right and two on the left. In the surgical treatment of cysts, we decide how much tissue to remove according to the type and location of the cyst.

Our general rule is this: to clean the disease completely, but while doing so to preserve as much healthy tissue as possible. However, achieving this balance does not always mean simply removing the cyst alone. Especially in conditions such as CPAM and Intralobar Sequestration, the diseased tissue may be completely spread within that lobe or its boundaries may be unclear.

In such cases, the procedure we call “Lobectomy,” that is, removing the entire lobe, is the safest route. Why do we not just remove the cyst? Because the tissue around the cyst is generally unhealthy as well, and trying to peel the cyst away may lead both to air leaks and to diseased tissue being left behind. Even a tiny remaining cyst fragment may create a risk of infection or recurrence later. Therefore, lobectomy means a “radical solution.”

However, in conditions we call “Extralobar Sequestration,” where the disease has its own covering and lies separately from the normal lung, our work is easier. Here, we can remove only the diseased part (segmentectomy or cyst excision) and complete the procedure without touching the healthy lung at all.

The factors that determine our resection strategy are as follows:

• The anatomical location of the cyst
• The size of the lesion
• The type of the disease
• Its relationship with vascular structures
• The condition of healthy tissue

What is the postoperative recovery process and hospital follow-up like?

When the operation is finished, we usually take your baby to the Intensive Care Unit for a while. Do not let the term “intensive care” frighten you. This does not mean that things are going badly. On the contrary, babies are more sensitive than adults during the waking period after anesthesia, and keeping them in the most well-equipped place under the supervision of the most experienced nurses is entirely a safety measure. Most babies return to the regular ward and to their mother’s arms a few hours after the operation or the next morning.

After the operation, you will see a thin tube in the chest cage that we call a “drain.” This tube is there to remove the small amount of blood and fluid that may accumulate inside and at the same time to monitor possible air leaks from the lung. This is an extremely standard practice.

The stages we expect and monitor during the recovery process are as follows:

• Ensuring pain control
• The baby breathing comfortably on their own
• Starting feeding
• Decrease in fluid coming from the drain
• Stopping of air leakage
• Increase in mobility

This is where the beauty of closed surgery (VATS) becomes apparent. Babies usually feel very little pain and begin moving around within 24-48 hours as if they had never had surgery. The drain is usually removed after 2 or 3 days. Once the drain is removed, there is no obstacle left for you to take your baby in your arms and go home. The total hospital stay is generally between 3 and 5 days.

Does surgery for congenital lung cysts affect the child’s future life?

Now we come to the most important and most hopeful part. Having one lobe removed never means that your child will become a “disabled” or “low-capacity” individual in the future. The human body, especially the bodies of babies, has an incredible ability to adapt and renew itself.

Lung development in babies does not end at birth; new air sacs (alveoli) continue to form until approximately the age of 8. When we remove the diseased lobe, the remaining healthy lobes of the lung expand and grow to fill that empty space in the chest cavity. In medicine, we call this “compensatory hypertrophy.” In other words, the remaining healthy tissue makes extra effort to take over the duty of what is gone and increases its capacity.

Long-term scientific studies have shown that children who undergo lobectomy in infancy have respiratory function tests that are normal or very close to normal when they reach adulthood. When these children grow up, they can run, swim, and become professional athletes just like their peers. They do not fall behind in any physical activity.

Frequently Asked Questions