Rib fusion

Introduction Thoracic insufficiency syndrome (TIS) means that the thorax does not support normal breathing and lung growth. TIS is a collective term for a class of syndromes. Most of these diseases are congenital diseases, and there are multiple system malformations. The most common are congenital scoliosis and congenital lateral kyphosis. Patients with congenital spinal deformity usually have ribs and ribs. Absence of thoracic developmental malformations. TIS can be divided into four types: type I is rib absent and scoliosis, type II is rib fusion and scoliosis, type IIIa is a small chest, and type IIIb is a narrow thorax.

Cause

TIS is a combination of a type of syndrome and does not specifically refer to a disease. Most of these diseases are congenital diseases, and there are multiple system malformations. The most common are congenital scoliosis and congenital lateral kyphosis. Patients with congenital spinal deformity usually have ribs and ribs. Absence of thoracic developmental malformations. It is generally believed that most congenital scoliosis is non-hereditary and is caused by environmental factors during embryonic development. Secondly, the rapid progression of idiopathic scoliosis in infants and young children can also lead to TIS, the cause of which is unknown. In addition, patients with early-onset scoliosis, Jeunes syndrome, and Jarcho-Levin syndrome may also have TIS. Jeunes syndrome is also a disease that causes TIS. Jeune syndrome is asphyxiating thoracic dysplasia, also known as thoracic-pelvic-toe (toe) bone dysplasia, which is an autosomal recessive disorder. This disease causes dyspnea and repeated respiratory infections due to thoracic dysplasia, and severe cases die within a few weeks after birth.

an examination

TIS is usually associated with lung and thoracic developmental disorders. Thoracic dysplasia syndrome is a type of thoracic weakness that supports the growth of the normal respiratory system or lungs. Often occurs in children with severe deformities of the ribs and chest wall with scoliosis.

The development of the lungs in children depends on the growth of the thorax, and the thoracic cage must be symmetrical by the ribs and the spine to provide a normal environment. The growth and development of the two are interdependent. First, the thorax is not enough to support the normal breathing of the lungs. When there are ribs or ribs absent, the breathing movement is limited. When the deformity progresses, the chest volume is further reduced, the thoracic dysfunction is aggravated, and the atelectasis and lungs are easily caused. Infection, etc. Secondly, the thoracic cage is insufficient to support the normal growth of the lungs. The thoracic deformity causes the growth of the thorax in all directions to be restricted, resulting in a significant limitation of the normal growth of the lung tissue. Therefore, any disease affecting lung development or thoracic development will cause the same pathological process. The reduction of alveolar capillary epithelial cells leads to the reduction of alveolar, the reduction of lung exchange function, and the limited respiratory movement, which can easily cause lung function. Not complete. There is a natural history of patients with congenital scoliosis, and 36-year-old died of cardiac arrest caused by severely restrictive lung disease.

TIS is divided into the following four types: type I is rib absent and scoliosis, type II is rib fusion and scoliosis, type IIIa is a small chest, and type IIIb is a narrow thorax. This classification has certain reference significance for VEPTR surgery. The mechanisms by which each type causes a decrease in lung function are also different. Type I is because the lungs are compressed in the thorax and the volume of thoracic activity is lost. Type II is because the concave side of the rib shortens the thorax and limits the respiratory movement of the concave side lung. Type IIIa limits the respiratory movement of the lungs due to the small chest profile caused by the reduction of the longitudinal diameter of the bilateral thorax. Type IIIb is a narrow thorax formed by the reduction of the transverse diameter of the bilateral thorax, which limits external breathing.

Differential diagnosis

The thoracic deformity has two sides or one side, and may be integral or limited to a certain part.

First, bilateral anomalies

1. Barrel chest. It is more common in emphysema caused by airway asthma, chronic bronchitis, etc., which can be seen in elderly and short-skinned people. The anteroposterior diameter and left and right diameter of the thorax are increased, especially the anteroposterior diameter is significantly increased, sometimes equal to the left and right diameters, so that the chest is barrel-shaped, the anterior inferior oblique angle of the rib arch is raised, the intercostal space is widened, and the respiratory movement is significant. Decreased, palpation fainting weakened or disappeared, percussion leaves were unvoiced, heart sounds narrowed or not easy to shed, lower lung and liver dullness decreased, auscultation was far away, breath sounds weakened, exhalation prolonged, concurrent infection, lungs There may be wet rales, the X-ray shows thoracic expansion, the intercostal space is widened, the ribs are not working, the activity is weakened, and the diaphragm is lowered and flattened. When emphysema is complicated, the lung capacity is reduced in one second, and the residual volume is increased in obstructive ventilatory dysfunction.

2. Flat chest. Found in chronic wasting diseases such as tuberculosis, can also be seen in patients with elongated body shape, flattened chest, small anteroposterior diameter and often short and half diameter; prominent clavicle, obvious upper and lower fossa, rib sag, small intercostal space, intercostal muscle atrophy, abdomen The upper corner is acute, and the flat chest is often accompanied by weakness signs, visceral drooping, poor thoracic expansion, and prone to tuberculosis. It used to be called tuberculosis.

3. Funnel chest. The disease is congenital dysplasia and has a genetic predisposition such as marfans syndrome. The funnel chest is a funnel-shaped thoracic deformity in the lower part of the sternum and the xiphoid. The lower thorax of the patient is flat, and the sternal angle collapses to the rear. The xiphoid is most obvious. The distance between the lower end of the sternum and the spine is shortened. The ribs on both sides of the sternum are curved, and may be associated with posterior vertebral protrusion or other deformities. The thoracic motion is often limited, and the heart is displaced by the pressure, and most of them are called to the left. Clinical manifestations, thoracic deformity in infants and young children can be inconspicuous, more asymptomatic, with the increase of age, chest and chest deformities gradually worsen, dyspnea, palpitations in the anterior region of the heart and chronic bronchitis, symptoms of pneumonia, lungs can be heard and wet rales Due to heart shift, audible heart sound splitting and systolic murmurs can be heard. X-ray examination showed a shift in the heart and the degree of depression after the lower end of the sternum. Electrocardiogram has changes in heart shift and transition, and pulmonary function tests are restrictive ventilatory dysfunction. If combined with chronic bronchitis, recurrent episodes can cause obstructive ventilatory dysfunction.

4. Chicken breasts. Because of rickets, repeated respiratory infections in childhood can also cause chicken breasts. The chest of the chicken chest is protruding forward, especially the lower part. The anteroposterior diameter of the thorax is enlarged, the transverse diameter is reduced, and the lateral or bilateral rib cartilage is often depressed. The joint between the rib and the costal cartilage is thickened and thickened, and is beaded; the anterior cranial valgus is valgus, and the respiratory muscles contract. The inner side of the chest wall is pulled inwardly, and a deep groove is formed from the sternum xiphoid along the iliac muscle attachment site inwardly. It is called the rib groove. The disease is more common in children and adolescents, also known as rickets. X-ray examination showed flattened thoracic sides, anterior sternum and rib cartilage anterior. Pulmonary function tests are restrictive ventilatory dysfunction.

5. Spinal deformity. There are many causes, due to congenital dysplasia accompanied by vertebral deformity, or secondary to tuberculosis, rickets, osteomalacia, trauma, neuromuscular lesions and severe pleural thickening, thoracoplasty, etc., but most of the causes are unknown. The disease is thoracic deformity caused by the lateral and backward curvature of the thoracic vertebrae. In severe cases, the rib space on the convex side of the spine is significantly widened, and the thoracic bulge is enlarged; the intercostal space of the concave side of the thorax is narrowed, and the thoracic cavity is subsided, resulting in pulmonary compression and lung development. Poor or causing localized atelectasis; uncompensated lungs can produce compensatory emphysema. Patients are prone to chronic bronchitis and develop to right heart hypertrophy and pulmonary heart disease. The main clinical manifestations are progressive dyspnea, shallow breathing, increased heart rate, and cyanosis. Lower respiratory tract infections and pneumonia are the main causes of respiratory failure and heart failure. There are usually no obvious symptoms in childhood. X-ray examination can be used for angular spinal deformity and chest, diaphragm muscle activity and lung conditions. Pulmonary function was restricted ventilatory dysfunction, accompanied by chronic bronchitis, varying degrees of obstructive ventilatory dysfunction.

Second, unilateral anomalies

1. One side increases. A large amount of effusion or empyema is formed in one side of the chest, and the tumor in the thoracic cavity can cause the affected side to bulge. Impaired lung function in the affected side, the intrathoracic tumor can make the affected side bulge. Impaired lung function in the affected side, compensatory emphysema in the opposite side of the chronic case. The trachea moved to the health, the perspiration of the pneumothorax was observed, and the percussion of the pleural effusion was voiced or real, and the auscultation of the breath sounds weakened or disappeared. X-ray examination, chest puncture and sputum or liquid examination can confirm the nature of the disease, and if necessary, thoracoscopic examination.

2. One side reduction. One side of the thoracic collapse can be caused by extensive pleural thickening due to empyema or pleurisy, adhesion and contraction, atelectasis, pulmonary fibrosis, chronic fibrotic tuberculosis, chronic lung suppuration, etc., clinical manifestations vary with primary disease Differently, the trachea moves to the affected side, the thoracic contour of the affected side becomes smaller, and the common scoliosis of the spine is common.

Third, local anomalies

1. Local uplift. Found in heart enlargement, pericardial effusion, aortic aneurysm, intrathoracic or chest wall tumors. Patients with circulatory diseases may have abnormal changes in cardiac auscultation and changes in ECG. Chest dysplasia mainly includes rib fractures of tuberculous inflammatory tumors.

2. Local depression. Pleural adhesion can cause partial collapse of the thorax, and the depression of the upper and lower fossa of the clavicle may be caused by fibrosis of the pneumonia tuberculosis, atelectasis, etc.; the rib cage of the rickets is subsided along the diaphragm.

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