Congenital Surgical Diseases of the Respiratory System

In this article, we will discuss the most common surgical diseases of the respiratory tract in infants and children. These pathologies can be grouped under two headings.

Respiratory Anomalies

• Choanal Atresia
• Tracheal agenesis and atresia
• Tracheomalacia
• Tracheal stenosis and ring
• Laryngo-Trachea-Bronchial cleft
• Bronchial atresia
• Bronchogenic cyst

Lung Anomalies

• Lung agenesis and atresia
• Accessory lobes
• Pulmonary sequestration
• Congenital lobar emphysema
• Lung cysts
• Congenital cystic malformation

Choanal Atresia:

It is a congenital pathology in which the back part of the nose, which is the first part of the respiratory system, is blocked and connects to the nasopharynx, which is the second part. 60% of the cases are unilateral and on the right, while unilateral cases are seen in approximately one in 8000-10,000 live births, bilateral cases are seen in approximately one in 80,000 live births. Approximately 20% of the blockage is cartilage-based, while the rest is bone-based.

The clinical picture varies depending on whether it is unilateral or bilateral. Unilateral blockages may not usually give a clinical picture. In bilateral cases, the baby is normally seen breathing through the mouth and it is noticed that he suddenly turns purple while feeding. Because the baby cannot breathe and turns purple because his nose is blocked while sucking.

A definitive diagnosis is made by first inserting a hard catheter into the nose and then performing an endoscopy. The pathology is surgically corrected.

Congenital Bronchogenic Cysts:

A congenital cystic anomaly of the bronchi. It constitutes approximately 10% of chest cavity lesions seen in children, and is more common in men. It is usually not large, so it does not cause a clinical picture and is asymptomatic. However, when cysts become infected or press on the organs in the chest cavity, especially the lungs, they cause clinical findings. The main clinical findings are cough, purulent sputum, fever and hemoptysis, and if the cyst ruptures and pneumothorax occurs, respiratory distress and compression symptoms occur. The diagnosis is made by history and physical examination, and confirmed by imaging methods. A round or oval lesion with fluid density and smooth edges is detected on chest radiographs, and the presence of an air-fluid level suggests that the cyst is infected. In endemic countries such as Turkey, it must be distinguished from pulmonary hydatid cysts and lung abscesses. Differential diagnosis is made with Computerized Tomography and Blood tests.

If left untreated, it can cause recurrent lung infection, air leakage and respiratory failure if it bursts, and lung cancer in later stages of life.

Treatment; very small cysts can be followed if they do not show clinical findings. However, the general approach is surgical excision for the reasons mentioned above. The principle is to completely remove the cyst, if it is not completely removed, it can recur. It can be done with open or thoracoscopic methods.

 

Picture; Chest X-ray and computer tomography images of air-filled cystic lesions in the middle and lower zones of the right lung, respectively

Congenital Lobar Emphysema

Congenital lobar emphysema (CLA) is a massive distension of normal lung tissue. It can involve the entire lung, one lobe, or one or several segments.

Pathology is caused by an abnormality in the cartilage structures of the bronchi, air actively enters the alveoli but cannot exit due to pathology and is trapped inside. The lung swells and creates pressure on the normal lung and thoracic anatomical structures.

It is seen in one in every 20,000-30,000 births. 1/3 of the cases are noticed at birth, most in the first six months. It is more common in males. It is most commonly seen in the left upper lobe, middle lobe, right upper and lower lobes, respectively.

Its etiology has not been clearly explained, and it has been argued that various mechanisms play a role in the etiology. These are infection damaging the alveoli, thick secretions obstructing the lobar bronchus, absence of cartilage, bronchomalacia and polyalveolar lobe.

Clinical findings occur in 30% of cases following birth and in others during childhood. Dyspnea, intercostal and supraclavicular retractions, wheezing, progressive cyanosis and feeding difficulties are typical.

Diagnosis is made with history and physical examination, and confirmed with imaging methods. The findings described above are detected in the history, On physical examination, the baby's chest wall may be swollen on one side, and respiratory sounds are decreased or cannot be heard on that side upon auscultation. Echogenicity and/or cystic appearance in the lungs are increased on prenatal USG.

In the chest X-ray, the emphysematous lobe presses on the neighboring lobes, causing atelectasis (deflating of healthy lung structures, i.e. failure to ventilate), the diaphragm (respiratory muscle) being pushed down and flattened on the pathological side, and the mediastinum shifting to the healthy side are typical.

Giant bulla, pneumothorax and congenital diaphragmatic hernia should be kept in mind in the differential diagnosis. When the diagnosis cannot be confirmed with direct chest X-ray, thorax CT and ventilation perfusion scintigraphy can be performed.

Despite medical and conservative treatment attempts, the definitive treatment is surgical removal of the affected lung section due to some complications.

 

Images: Lung X-ray, Tomography and Surgical image of Lobar Emphysema

Congenital Cystic Adenomatoid Malformation

Congenital cystic adenomatoid malformation (CCAM) is one of the congenital cystic diseases of the lung. CCAM is a rare developmental anomaly of the lower respiratory system, but it is the most common congenital lung lesion. Its incidence is 1 in 8300-35,000 live births. CCAM is a hamartomatous lesion that contains cystic and adenomatous elements originating from the trachea, bronchus, bronchioles or alveolar tissue. Large lesions can compress adjacent normal tissues and impair alveolar growth and development. The bronchus is usually connected to the tracheobronchial tree, although not normally. It is seen equally in both lungs and can occur in all lobes. Lesions are usually limited to one lobe, but rarely can be seen in more than one lobe.

There are 4 clinical and pathological types defined

Type 1: It is the most common form, with a rate of 60-70%. The cysts have thin walls and a diameter of 2-10 cm. The cysts are single but multiloculated (i.e., they consist of several separate pockets)

Type 2: It is seen in 15-20%. It consists of multiple cysts with a diameter of 0.52 cm. Since it is usually small, it does not have a mass effect on the adjacent lung. In 60% of cases, other congenital anomalies accompany it. These include tracheoesophageal fistula, bilateral renal agenesis or dysgenesis, esophageal atresia, intestinal atresia, diaphragm, heart, central nervous system and bone anomalies. It is not at risk for malignancy.

Type 3: It is seen in 5-10%. It is usually very large, can involve an entire lobe or several lobes. It contains many small-diameter cysts. In affected infants, intrauterine or birth death is observed, and it causes severe respiratory distress in the neonatal period.

Type 4: It is seen in 10-15%. Cysts have a maximum diameter of 7 cm, and can usually occur with tension pneumothorax or infection, at birth or during childhood. However, it can also be detected incidentally in asymptomatic patients.

Diagnosis of the disease: It is established by history, physical examination and confirmed by imaging methods. Prenatal in utero disease can be detected in the history. After birth, the clinic depends on the size of the cyst. It can cause respiratory distress due to compression of the lung, pushing the mediastinum to the opposite side and causing flattening of the diaphragm. Small cysts can be diagnosed months or years after birth as an incidental lesion or a focus of infection.

CCAM can occur with respiratory distress in the neonatal period, or it can remain asymptomatic for years. The severity of respiratory distress is proportional to the size of the lesion. Typical findings are tachypnea, groaning and retractions, increased respiratory effort and cyanosis. Type 2 cases are diagnosed shortly after birth due to other congenital anomalies. Type 3 is the most severe form. The lesions are large and can involve the entire lung, fetal hydrops and pulmonary hypoplasia are typical. Babies may be stillborn or develop severe, progressive respiratory distress, cyanosis and respiratory failure soon after birth. Almost all sick babies are male. Type 4 may occur with pneumothorax in the neonatal period. One-third of the cases are diagnosed in childhood. These are types 1 and 4 cases. Recurrent pneumonia, cough, dyspnea and cyanosis are frequently seen.

The disease can cause serious complications if left untreated. Depending on the type of pathology, it can result in respiratory distress, recurrent lung infection, lung cancer in the later stages of life and death.

The treatment of the disease is the surgical removal of the affected lung section. This procedure can be performed under general anesthesia with open or thoracoscopic methods. If the cysts are small, single or multiple and do not cause clinical complaints, a few months can be waited before surgery. However, if they cause clinical complaints, the patient is prepared for surgery after the baby is born and the surgery is performed.

 

Images: CAM's Lung Tomography and Surgical Image

Pulmonary Sequestration

It is characterized by the lack of a connection between a lobe or segment and the normal tracheobronchial system. Arterial circulation is mostly provided by the systemic artery originating from the aorta and sometimes passing through the diaphragm and reaching the sequestered tissue from the abdominal aorta. Venous return is usually to the pulmonary veins and rarely to the systemic veins. It constitutes 7% of congenital anomalies of the lung. It is seen 3 times more in males.

The diagnosis of the disease is made by history and physical examination and confirmed by imaging methods. Prenatal ultrasonographic findings in the history include solid, well-defined echogenic mass, mediastinal shift, polyhydramnios and hydropic changes.

If there is no prenatal diagnosis, pulmonary sequestration should be considered in children with complaints of recurrent pneumonia (lung infection) in the same region of the lung after birth. Diagnosis is made by Computed Tomography. Systemic arterial circulation can be detected by color doppler USG and MRI. There are two types of pathology.

 

 

Images: Chest X-ray and Tomography images of Pulmonary Sequestration

1. Extralobar Sequestration

Sequestered tissue is separate from the normal lung and covered with its own visceral pleura. It constitutes 25% of sequestrations and 90% of them are seen as triangular lesions adjacent to the diaphragm in the left hemithorax. Diaphragmatic hernia, diaphragmatic paralysis, achalasia, pectus excavatum and congenital cardiac malformations are seen in 50% of the cases.

2. Intralobar Sequestration

Sequestered tissue is located within the normal lung parenchyma. It is seen in both sexes. It is common in the left lung. They usually do not show symptoms until adolescence. Chronic infection may develop. In this case, the sequestered segment is seen as cystic and filled with pus.

The treatment of Pulmonary Sequestration is surgical removal of the affected lung part.

Can Lung Surgery Diseases Be Performed Thoroscopically (Closed)?

Yes, it can be done. With the development of technology, closed surgeries can be performed even on 8-12 month old babies.

How Are Lung Surgery Diseases Performed Thoroscopically?

In closed surgery methods, a large incision is not made between the ribs as in open surgery. It does not have to be opened by placing a retractor. 5-10 mm diameter instruments are placed in 2 or 3 holes in the thoracic wall (chest wall) and surgeries are performed through these holes. Closed surgeries provide a wider field of view compared to open surgeries.

Does Closed Surgery (Thoracic) Have Advantages?

Yes, there are, the main ones are; less pain, shorter recovery period and more aesthetics, and there are many advantages. The thoracoscopic method provides a wider field of view compared to open surgeries. The patient may need to stay in intensive care for one day and is discharged in 2-3 days.

It should not be forgotten that thoracoscopic (closed) surgeries require serious experience, knowledge and skill along with advanced technology. Otherwise, it should not be forgotten that serious complications may occur.

*** The information provided here and the content of the website are designed to inform visitors, especially families. No information should be considered as advice by visitors and should not lead to any decision or action. The patient should definitely be examined by a pediatric surgeon on the subject, and a decision should be made by consulting him/her and consulting his/her personal knowledge.