saber syndrome
Introduction
Introduction to Saber Syndrome The scimitar syndrome (scimitarsyndrome) is the pulmonary hypoplasia syndrome, also known as the scimitar syndrome, first proposed by Neil et al. (1960). This sign is a congenital vascular malformation, a type of pulmonary venous malformation (subcardiac type), characterized by the opening of the right pulmonary vein in the inferior vena cava. It is a group of symptoms caused by abnormal pulmonary venous return. Its main features are: 1 right lung hypoplasia; 2 due to deformed veins, X-ray examination found a knife-shaped shadow along the right heart; 3 heart moved to the right Bit, approximate to the right heart. basic knowledge The proportion of illness: 0.0002%-0.0003% Susceptible people: infants and young children Mode of infection: non-infectious Complications: cough, aversion to cold, hemoptysis
Cause
The cause of saber syndrome
Cause:
The cause of the intrinsic remains unclear, but most scholars believe that it is associated with abnormal development of the embryonic lung.
Pathogenesis
During the formation of lung buds, pulmonary vascular displacement and vascular drainage malformation may be caused by the residual connection between the embryonic venous plexus, the large venous system and the umbilical yolk vein. The right heart shift is related to the reduction of lung volume. Secondary changes.
Prevention
Saber syndrome prevention
Usually need to prevent colds and avoid cold. Develop a reasonable diet and keep a light diet. Eat more fresh vegetables and aquatic products. Such as green vegetables, radishes, kelp, seaweed and so on. Should eat less meals. Fasting fat meat and animal offal, do not eat spicy onions, peppers, coffee, etc. Eat less refined sugar, honey, fruit candy, cakes, etc. Strengthen nutrition and use foods that are easy to digest, contain enough calories, protein and vitamins. Such as porridge, milk, soft rice, soy milk, eggs, lean meat, fresh vegetables and fruits rich in vitamins A, B, C. These foods can enhance the body's resistance. Be careful not to drink carbonated drinks, coffee, strong tea, etc.
Complication
Saber syndrome complications Complications, cough, aversion, fever, hemoptysis
Concurrent right lung infection. The clinical manifestations of right lung infection caused by different bacterial infections are quite different. Common symptoms include fever, cough, cough, purulent sputum or blood stasis, with or without chest pain; severe cases have difficulty breathing and respiratory rate is accelerated. Right lung percussion dullness, increased vocal fibrillation, bronchial breath sounds, wet rales, etc. Blood routines suggest a white blood cell count and a general increase in neutrophils. The culture of sputum or pleural effusion is more common for pathogenic diagnosis.
Symptom
Saber Syndrome Symptoms Common Symptoms Breathing Difficulties Chronic Cough Recurrent Right Lung Infections Hem Bloodshots Breathing Sounds Low Dizziness Systolic Vocal Hearts
Symptom
Different degrees of breathing difficulties, increased activity, recurrent right lung infection, cough, hemoptysis, fever and other symptoms, can also have chronic cough, asthma, dizziness, cyanosis, some life-time asymptomatic, age of onset, more Found between the ages of 10 and 20, the incidence rate of men and women is 1:2, and some cases have developmental abnormalities.
2. Signs
The heart is dulled to the right, like the right heart. The heart auscultis is audible and systolic murmur in the second intercostal space on the left sternal border. The right lung has low breath sounds. The heart and lungs can also have no abnormal signs. For example, the heart murmur is extensive. Other congenital heart disease possible.
Examine
Saber syndrome examination
When combined with pulmonary infection, white blood cells can be elevated.
X-ray inspection
It can be seen that there is a curved knife-like shadow along the right heart, and there is an image of right lung hypoplasia when the heart is displaced to the right.
2. Bronchography and bronchoscopy
There may be right lung hypoplasia and abnormal bronchial branches, stenosis and obstruction of terminal bronchioles.
3. Cardiac catheterization
It can be seen that the right atrium has more blood oxygen content than the peripheral vein, and sometimes the catheter is directly inserted into the pulmonary vein.
4. Angiography
It can be seen that the right pulmonary artery is thinned, and the artery of the systemic circulation can enter the right lung.
Diagnosis
Diagnosis and identification of saber syndrome
According to the clinical manifestations and auxiliary examination can make a diagnosis, clinical classification, type I: right lung hypoplasia, and the systemic circulation to the right lung, type II: only right lung hypoplasia, no systemic circulation to the right lung Type III: no complicated deformity of the above two types.
Clinical should be differentiated from lung infections and pneumonia.
