Congenital pulmonary dysplasia

The lungs are very important respiratory organs in the human body. Congenital pulmonary dysplasia is a relatively serious lung disease. Although the incidence rate is not very high, it will lead to the death of the child if the condition is serious. More than half of the babies die from lung failure. Congenital pulmonary dysplasia is a complex and serious condition that requires correct understanding and active treatment of this disease.

Causes

Congenital pulmonary agenesis and pulmonary hypoplasia are caused by impaired development of the lung bud during embryonic development.

Impaired lung bud development at a certain stage during embryonic development leads to lung agenesis and pulmonary hypoplasia. Most of the time, other developmental defects are also present, the more common of which are incomplete and absent development of the trachea, bronchi and pulmonary arteries, abnormal development of the spine, and herniation of abdominal organs through the thoracoperitoneum into the pleural cavity. 50% to 80% of infants with congenital diaphragmatic hernia die from lung failure, mainly due to congenital lung hypoplasia.

Clinical manifestations

Recurring respiratory infections. Patients with unilateral pulmonary hypoplasia often have mild dyspnea, poor physical strength and endurance. Some patients may cough up blood due to collateral circulation from the systemic circulation. When combined with respiratory tract infection, dyspnea may worsen, cyanosis may occur, and breath sounds may become coarse. Those with growth retardation and malformations of the heart, bones or other organs may have corresponding symptoms. The chest is usually free of deformity and is bilaterally symmetrical or nearly symmetrical. The respiratory movement on the affected side is weak, the breath sounds are weakened or absent, and percussion may be solid or too soft. Patients with lobar agenesis have fewer clinical symptoms and the disease is hidden. Physical examination only reveals decreased breath sounds on the affected side. If accompanied by lung infection, rough breath sounds and rales may occur on the affected side.

examine

1. X-ray examination

Unilateral lung aplasia is manifested in uniformly dense chest cavity on the affected side on chest X-ray, lacking aerated lung tissue, bronchial shadows, and vascular texture. The heart and mediastinal structures are shifted to the affected side, and the diaphragmatic surface of the affected side is unclear. The normal lung on the opposite side shows varying degrees of compensatory emphysema, resulting in a lowered diaphragm and a flattened diaphragmatic surface. Over-expanded lungs may form a mediastinal hernia. If hemivertebra is also seen, it is very helpful for diagnosis. Another part of pulmonary hypoplasia may show inflated lung tissue on chest X-ray, but with sparse lung texture.

2. Bronchography

It can show that the main bronchus on the affected side is absent, the trachea seems to be directly connected to the main bronchus on the other side, or the main bronchus is hypoplastic and deformed, or the number of bronchial branches is sparse. Pulmonary angiography can show that the main trunk of the pulmonary artery on the affected side is hypoplastic or absent, which helps to confirm the diagnosis.

3. Pulmonary artery perfusion scan

The affected side shows decreased or significantly reduced pulmonary blood flow.

diagnosis

If respiratory infections recur for unknown reasons, one should consider whether there is congenital pulmonary hypoplasia. The diagnosis must be made very carefully. X-ray examination is the first choice. If the diagnosis cannot be confirmed, bronchial angiography or pulmonary artery perfusion scan can be performed, which can usually identify the cause.

treat

Pulmonary hypoplasia without obvious clinical symptoms does not require any treatment. Patients with repeated hemoptysis or lung infection, or even developmental delay with residual lung, bronchial or vascular malformations, must undergo lobectomy or pneumonectomy. However, pneumonectomy must be performed with extreme caution and it must be ensured that the function of the healthy lung is completely normal, otherwise it will cause disability or even death. During surgery, special attention should be paid to anatomical variations and surrounding organs should not be damaged. If there is concurrent developmental abnormalities of the heart or great vessels, a thorough preoperative assessment should be performed and, if necessary, correction performed during surgery.

prevention

Keep the respiratory tract open, use antibiotic nebulization inhalation at any time, and assist the patient in expectoration.