(Congenital) Lung Diseases Requiring Surgery in Children
There may be some congenital diseases of the lung. These diseases may occur at birth or in infancy, some of them may not be recognized in infancy because they do not show any symptoms, and are only recognized when complications develop due to these diseases at an older age.
What is Congenital Cystic Adenomatoid Malformation?
Congenital cystic adenomatoid malformation (CCAM) is a congenital disorder. It is usually recognized in early infancy. In this disorder, the affected areas of the lung are characterized by cystic and sometimes adenomatoid masses or a combination of both in different proportions.
These areas can be large and put pressure on the intact parts of the lung and can affect the development of the intact lung. There is also the potential for the adenomatoid form to develop into a malignant tumor. When the disease affects a small area of the lung, there are few symptoms and it may be found incidentally on prenatal or postnatal radiologic examinations. When the disease affects larger areas, there may be symptoms including shortness of breath and respiratory distress.
When congenital cystic adenomatoid malformation is diagnosed, surgery is necessary to remove the affected part of the lung. If symptoms are common in the baby, surgery should be performed without waiting for the baby to grow up. In patients without symptoms, surgery can be postponed until the patient grown up.
What is congenital lobar emphysema?
In congenital lobar emphysema, another congenital lung disease, one lobe of the lung has diffuse emphysema and is completely filled with air cysts. This lobe, which is quite large in volume, compresses both the same side intact lung areas and also the contralateral lung by pushing the midline across and prevents the intact lung from functioning normally. The upper lobe of the left lung is most usually affected. The baby may develop shortness of breath at birth.
Today, these diseases can be diagnosed even before birth, in utero, and potentially dangerous conditions can be prevented with rapid intervention after birth, if necessary. Depending on the clinical condition of the baby, emergency surgery may be necessary or, if the baby is stable, can be waited to grow up for a bit. The affected lung lobe is then surgically removed.
What is pulmonary sequestration?
Sequestration is the congenital presence of foreign tissues within the lung tissue or in the chest cavity near the lung, with blood circulation not similar to that of the lungs. Sometimes it can be recognized in infancy (shortness of breath, feeding problems), but it is usually recognized and diagnosed in later periods, either incidentally or in cases such as the development of infection.
The treatment consists of surgical removal of the tissue called sequestration. If the sequestration is inside the lung, it is sufficient to remove it together with the affected part of the lung, and if it is outside but next to the lung, it is sufficient to remove only the sequestration itself.
What is a bronchogenic cyst?
There are mucus-filled cysts next to the airways. Sometimes they grow next to the main bronchi and sometimes they grow into the lung. Although they are congenital, they are usually recognized at older age. They develop infection and give symptoms. They are removed by surgery.
Which type of surgery is performed on whom in congenital lung diseases?
In these diseases, the clinical condition of the patient, severity of the disease, symptoms, etc. are taken into consideration and the decision is made jointly by the pediatrician and the surgeon. In newborn babies, emergency surgery may be necessary, or the baby may be allowed to grow up a little, or in some cases may be followed up.
The method of surgery may be open surgery or videothoracoscopic surgery (VATS) depending on the age of the patient and the condition of the disease.
