congenital hiatal hernia
Introduction
Introduction to congenital chest and abdomen Congenital pleural effusion (congenitalpleuroperitonealforamenhernia) is an abnormality of the lumbar rib triangle in the embryonic diaphragm, a local defect or weakness, a type of sputum formed by the abdominal organ through the defect or weak area into the chest cavity, also known as congenital Congenital posterolateral diaphragmatichernia or Bochdalek hiatus hernia is the most common congenital diaphragmatic hernia. The clinical manifestations involve three systems of respiration, circulation and digestion. The mortality rate is high and surgery is the only effective treatment. At the 8th week of embryonic development, the transverse part of the heart is formed and meets the dorsal side of the anterior mesentery, developing into the central iliac crest of the diaphragm. At the 9th week of the embryo, the posterior wall of the thoracic and peritoneum merged with the muscle fibers of the lateral body wall to complete the development of the diaphragm. If the original diaphragm and the chest and abdomen diaphragm cannot merge with each other, a defect is formed on the diaphragm, and the original intestine can enter the chest cavity through the defect from the dorsal side of the umbilical cord to form a fistula. Because the right ankle is formed earlier than the left ankle, the incidence of left ankle is higher than the right. If the sputum occurs on the left side, the stomach, small intestine, colon, spleen, etc. can break into the chest. If sputum occurs on the right side, some or all of the liver can break into the chest. The abdominal organs that break into the chest compress the lungs of the fetus, affecting the development of the lungs. In severe cases, the mediastinum is displaced to the opposite side, and the contralateral lung is compressed to make it underdeveloped. basic knowledge The proportion of illness: 0.0002% - 0.0005% Susceptible people: young children Mode of infection: non-infectious Complications: toxic shock syndrome pneumonia
Cause
Congenital causes of thoracic and abdominal hernia
Cause:
At the 8th week of embryonic development, the transverse part of the heart is formed and meets the dorsal side of the anterior mesentery, developing into the central iliac crest of the diaphragm. At the 9th week of the embryo, the posterior wall of the thoracic and peritoneum merged with the muscle fibers of the lateral body wall to complete the development of the diaphragm. If the original diaphragm and the chest and abdomen diaphragm cannot merge with each other, a defect is formed on the diaphragm, and the original intestine can enter the chest cavity through the defect from the dorsal side of the umbilical cord to form a fistula. Because the right ankle is formed earlier than the left ankle, the incidence of left ankle is higher than the right. If the sputum occurs on the left side, the stomach, small intestine, colon, spleen, etc. can break into the chest. If sputum occurs on the right side, some or all of the liver can break into the chest. The abdominal organs that break into the chest compress the lungs of the fetus, affecting the development of the lungs. In severe cases, the mediastinum is displaced to the opposite side, and the contralateral lung is compressed to make it underdeveloped.
Pathogenesis:
Due to the embryonic stomach, intestinal tube and other large abdominal organs enter the thoracic cavity, the lungs are under pressure, development is blocked (the number of lung bronchial branches and the number of alveoli are greatly reduced), the newborn's respiratory function is poor after birth, manifested as difficulty breathing, breathing after birth As the swallowed air enters the gastrointestinal tract, it further aggravates the compression of the lungs, hinders gas exchange, reduces arterial oxygen partial pressure, increases carbon dioxide partial pressure, respiratory acidosis, hypoxia, acidosis can cause lungs Vasospasm leads to increased pulmonary artery resistance, and the flow of blood from the right to the left through the arterial catheter and the foramen ovale increases, further aggravating hypoxemia and acidemia, and forming a vicious circle.
During the fetal period, 85% of the blood discharged from the right ventricle does not pass through the lungs, but flows directly into the aorta through the foramen ovale or arterial catheter to the placenta for gas exchange. The shunt of the fetal circulation is due to the filling of the embryonic lung with fluid and pulmonary arterioles. Due to the low blood oxygenation and contraction, the pulmonary vascular resistance is increased. After the newborn is born, the lungs are filled with air, the fluid in the alveoli is absorbed, and the pulmonary arterioles are expanded due to the increased oxygen partial pressure, which causes the pulmonary vascular resistance to decrease, and the umbilical cord is broken. After the circulation, the blood pressure is increased, and the circulation is converted into the adult type. Three kinds of pulmonary vascular dysplasia after birth can cause pulmonary vascular resistance and pulmonary right to left shunt. One: the pulmonary blood vessels are extensive and small, making the pulmonary blood vessels The total blood volume is reduced; the second is: the branch of the pulmonary artery is reduced, so that the number of blood vessels in the unit lung tissue is reduced; and the third is: the smooth muscle layer of the pulmonary arteriole is thickened.
Due to congenital diaphragmatic pulmonary arteriogenesis, pulmonary wall muscle hypertrophy, very sensitive to hypoxia and hypercapnia, more prone to vasospasm, which is the main cause of pulmonary hypertension and right to left shunt in children. In addition to pulmonary vasospasm, lung dysplasia, pulmonary vascular stenosis, and a small number of pulmonary branches directly lead to a decrease in total blood volume in the pulmonary blood vessels, which is also an important cause of increased pulmonary vascular resistance and right-to-left shunting of blood circulation.
If the ankle ring (diaphragmatic defect) is too large, the incarceration is rare, and the small one is prone to incarceration. When coughing, crying or forced defecation, the intra-abdominal pressure can be increased, causing more gastrointestinal tubes to enter the chest cavity. Due to the compression of the ankle ring, the intestinal tube is evacuated, and severe cases are strangulated. As the intestinal wall edema and the intestinal lumen expand, the compression of the lungs and heart is aggravated, and breathing and circulation gradually become obstacles if the intubated intestine occurs. Blood circulation disorder, strangulated necrosis, can lead to severe chest and abdominal infection, toxic shock.
Prevention
Congenital prevention of chest and abdomen
There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Congenital complications of thoracic and abdominal hernia Complications toxic shock syndrome pneumonia
Invasive abdominal organ incarceration
Symptoms of gastrointestinal symptoms or acute intestinal obstruction may occur. If the intubation of the intestine is strangulated and necrotic, chest, abdominal infection, or toxic shock may occur.
2. Respiratory infection or pneumonia
Some sick children often suffer from respiratory infections or pneumonia due to lung pressure, ventilatory disorders, manifested as cough, asthma or fever, occasionally breathing difficulties, fever can aggravate acidemia and hypoxemia.
Symptom
Congenital chest and abdomen hiatal hernia symptoms Common symptoms Difficulty breathing, respiratory acidosis, shortness of breath, respiratory sounds, weakened bowel, asthma, abdominal discomfort, abdominal infection, post-sternal pain
The clinical manifestations of congenital chest and abdomen hiatal hernia in infants, infants and children are very different, especially in the neonatal period, the disease progresses rapidly, the risk is high, and the mortality rate is high.
Neonatal period
The clinical manifestations of congenital chest and abdomen hiatal hernia in the neonatal period mainly involve three systems of breathing, circulation and digestion, and mainly respiratory and circulatory disorders, followed by gastrointestinal symptoms.
(1) Respiratory and circulatory disorders:
1 difficulty breathing, cyanosis: in the fetal period, 85% of the blood discharged from the right ventricle directly through the foramen ovale, the arterial catheter flows into the aorta to the placenta for gas exchange, although the fetal lung is compressed by the contents of the chest cavity, and is atrophied. Does not threaten the life of the fetus, but after birth, the exchange of gas, the oxygenation of the blood is completely dependent on the lungs of the child, because the affected lung is atrophied, stunted, and the mediastinum shifts to the healthy side to oppress the healthy lung. There are also developmental disorders in the healthy lungs, and insufficient ventilation after birth, resulting in hypoxia and carbon dioxide retention in the body, and reflexive respiratory frequency increases to compensate for lack of ventilation, manifested as difficulty breathing, urgency, cyanosis, etc. .
Difficulty breathing, urgency, cyanosis and other symptoms can occur within a few hours after birth or within a few hours after birth, the severity and size of the diaphragmatic muscle, the number and volume of abdominal organs into the chest, lung compression and dysplasia and health The status of lateral lung development disorder is related to dyspnea and the degree and onset of snoring may be a paroxysmal change. If the heart and lung are affected, the child may have no obvious symptoms when he is quiet, or appear or aggravate when crying or feeding; When the person is severe, there is difficulty in breathing, shortness of breath, periorbital fistula, and the symptoms of the affected side or semi-sitting position are relieved or alleviated; in severe cases, there is significant difficulty in breathing after the birth, and the body is cramped, showing progressive aggravation or sudden deterioration. Breathing, sudden cardiac arrest or death may occur at any time. Some children may breathe vigorously after severe crying, which may cause a significant increase in the negative pressure in the affected side, and even further inject the abdominal internal organs into the chest cavity, causing the affected lung. Under pressure, the mediastinum shifts to the healthy side, causing severe breathing difficulties. If the treatment is improper or the treatment is not timely, the sick child can die quickly.
2 acidemia, hypoxemia: clinically, in addition to dyspnea, cyanosis, sick children soon appear acidosis and hypoxemia, initially respiratory acidosis, followed by metabolic or mixed acidosis Physiological indicators and blood gas analysis showed that the pH decreased, PaCO2 increased, and PaO2 decreased significantly.
In addition to difficulty breathing, urgency, cyanosis, acidemia, hypoxemia, there are a series of symptoms such as low or no body temperature, low blood calcium, low blood magnesium.
3 pulmonary hypertension: the abdominal organs enter the thoracic cavity not only make the lungs develop disorders, but also make the pulmonary artery twisted, the number of arteries is small, the arterial wall thickening and the vascular bed cross-sectional area is reduced, etc., acidemia, hypoxemia can cause pulmonary artery spasm As a result, pulmonary artery resistance is increased to produce persistent pulmonary hypertension, resulting in a right-to-left shunt, aggravating hypoxemia and acidemia and forming a vicious circle, eventually dying due to hypoxia.
(2) Digestive tract symptoms: Gastrointestinal symptoms are rare in clinical manifestations, such as anorexia, vomiting, etc., if accompanied by congenital intestinal malrotation or intrusive abdominal organ incarceration, gastrointestinal symptoms or acute Symptoms of intestinal obstruction, if the incarcerated intestine is strangulated and necrotic, chest, abdominal infection, or toxic shock may occur.
(3) Signs:
1 chest signs: the thoracic respiratory motility of the affected side is weakened, full or bulging, the intercostal space is wider than the contralateral side; the mediastinum is displaced, when the heart is compressed, the apex beats to the healthy side, and the severe one must be differentiated from the right heart. Due to the nature of the invaginal organs or the degree of inflation of the gastrointestinal tract, the chest percussion is voiced or drum sounds, often turbid drum sounds, auscultation side breathing sounds weakened or disappeared, such as smell and bowel sounds, The diagnosis of congenital chest and abdomen hiatus hernia is of great significance, but the position of the diaphragm of the neonate is lower (up to the level of the 8th to 9th thoracic vertebrae), and the diaphragm and chest and abdomen wall are weak, and the bowel sound is easily transmitted to the chest. Must be carefully checked and analyzed to avoid misdiagnosis.
2 Abdominal signs: related to the amount of abdominal organs into the chest cavity, if a large number of abdominal organs into the chest cavity, the abdominal cavity is empty, the depression is a boat-like abdomen; if less organs are inserted, the depression is not obvious.
2. Infants and children
(1) Respiratory symptoms: Because most infants and elderly children have small chest and abdomen hiatus ankle ring (diaphragmatic defect), the volume and number of abdominal organs into the chest cavity are small, and the degree of lung development is affected to a small extent. Symptoms are later, and the degree is lighter. Sudden breathing occurs, and the possibility of circulatory disturbances is significantly reduced. Most of the sick children are exposed to respiratory infections due to compression of the affected lungs. Only chest X-ray examinations can be found. Suffering from asthma or fever, occasionally breathing difficulties, breathing difficulties occur when the child is crying or strenuous activity, and then improve after being quiet; the lying position is aggravated and the standing position is improved.
In addition, some sick children, especially the congenital thoracic and sacral hernia on the right side, due to the liver blockage of the ankle ring (the diaphragmatic defect, the gastrointestinal tract does not enter the thoracic cavity, the lung compression and development are affected to a lesser extent, usually no Significant clinical signs and symptoms are often found in accidental physical examination or chest X-rays.
(2) Chronic digestive tract symptoms: older children often complain of chronic gastrointestinal symptoms such as chest pain, abdominal pain or upper abdominal discomfort, and many medical treatments have no obvious curative effect. When they are further examined by barium meal, they have found chest and abdomen hiatus. Young children and children, sometimes in dramatic changes in body position, intense crying, full food and intense activities, sudden shortness of breath, difficulty breathing and cyanosis, accompanied by post-sternal pain and abdominal pain, vomiting, sputum content when incarcerated Abdominal cramps, vomit is a coffee-like substance, stop exhausting defecation.
(3) Physical physique: During the examination, the chest of the affected side may be full, the respiratory movement is weakened, and the heart is displaced to the healthy side; the thoracic deformity is barrel-shaped, and the intercostal space is enlarged; the percussion of the affected side is voiced or turbid, and the drum sounds are interphase; The auscultation of the affected side of the lungs is weak, and the snoring sounds can be heard. In the supine position, the abdominal wall depression is a scaphoid.
Examine
Examination of congenital chest and abdomen hiatus hernia
Blood gas analysis
Arterial oxygen partial pressure (PaO2) can be significantly decreased (normally 95 ~ 100mmHg), carbon dioxide (PaCO2) partial pressure increased (normal value 35 ~ 45mmHg), pH decreased (normal 7.35 ~ 7.45), such as PaO2 <60mmHg, carbon dioxide PaCO2>50mmHg, pH <7.3 indicates significant hypoxemia and respiratory acidosis.
2. Serum electrolyte
There may be abnormalities such as blood calcium and blood magnesium reduction.
3. X-ray inspection
(1) X-ray fluoroscopy: Intestinal fistula and its peristaltic image can be seen in the chest cavity.
(2) X-ray film: Digestive tract angiography and X-ray film examination play a decisive role in the diagnosis of this disease. The film should include the chest and abdomen. The X-ray film images are: 1 The bow image of the normal diaphragm of the affected side is not Clear, interrupted or disappeared; 2 affected side lung collapse; 3 mediastinum, heart shift to the healthy side; 4 chest can be seen into the abdominal organs image: A. content is purely stomach, the abdomen can not see the abdominal cavity The image of the stomach cavity, while the chest cavity shows a larger gastric cavity containing the liquid-gas surface; B. When the contents of the chest cavity are small intestine and the small intestine is not inflated, the X-ray film of the chest and abdomen can be seen in the chest of the affected side. Increase the shadow, as the sick child begins to breathe, the swallowed air enters the intestine, and repeatedly checks the intestines in the chest cavity that can show dilated gas. If the small intestine is inflated, its X-ray film can be seen with gas, translucent Intestinal fistula, and its image continues to the abdomen; C. content is a liver, spleen and other substantial organs, in addition to seeing a substantial image in the chest cavity, it can be seen that the gap left by the liver shadow up and up Intestinal tube filling.
(3) Digestive tract angiography: Highly suspected chest and abdomen hiatus hernia, chest X-ray plain film can not be diagnosed, you can choose digestive tract angiography.
Method: First, put the stomach tube into the stomach tube, and then inject a little air or water-soluble contrast agent (such as 12.5% sodium iodide) into the stomach tube. If the gastrointestinal tract image is found in the chest cavity, the diagnosis can be made.
Newborns and infants should choose air or water-soluble contrast agents, and sputum angiography should be banned to prevent suffocation and death by aspiration. Older children can choose sputum angiography.
(4) CT examination: the edge of the ankle ring (diaphragmatic defect) and the image of the abdominal organ in the thoracic cavity can be clearly displayed. The cross section of the ankle ring is triangular, and the inner shadow is mostly honeycomb.
4.B-ultrasound
It can be found that there are dilated intestinal fistulas in the thoracic cavity and their peristaltic echoes, or swimming shadows with liquid-free echoes and gas-like echoes, and mucosal folds can be seen in the intestinal effusion.
In recent years, domestic and foreign scholars have relied on ultrasound examination to carry out prenatal diagnosis research of this disease. If prenatal ultrasound examination can confirm that the abdominal organs are located in the thoracic cavity, the diagnosis can be confirmed. If the amniotic fluid is found, the mediastinum is displaced, the intraperitoneal cavity is found. In the absence of symptoms such as gastric vesicles, further examination should be performed to see if any abdominal organs have broken into the chest.
5. MRI examination
The coronal plane clearly shows the image of the intestine in the thoracic cavity and the edge of the ankle ring. The transverse section of the ankle ring is similar to the CT image, and is triangular in shape with a honeycomb intestine.
Diagnosis
Diagnosis and diagnosis of congenital chest and abdomen hiatus hernia
diagnosis
1. Clinical features
Due to the size of the diaphragmatic defect, the presence or absence of the hernia sac, the amount of abdominal organs into the chest, the degree of lung atrophy and dysplasia and the development of the contralateral lung development disorders, the clinical symptoms appear very different in the morning and evening.
(1) Symptoms: The clinical symptoms of neonates appear after birth, often with large diaphragm defects, no sacs, more abdominal organs in the chest, and the degree of lung atrophy and development of the affected lungs. Heavier, severe symptoms, rapid changes, dangerous conditions, and even death in the short term after birth due to failure to diagnose and treat in time. Early diagnosis and timely treatment are the key to reducing death, such as acute dyspnea in newborns. Short and bruising, feeding, aggravation after crying, should be highly suspected of this disease, infants and children with clinical symptoms and signs are not the same, repeated respiratory infections or repeated cough, shortness of breath, or with body position changes, crying People who have shortness of breath, difficulty breathing and cyanosis, abdominal pain and vomiting after eating and exercising should also consider this disease.
(2) Signs: shortness of breath and laborious, cyanosis; full chest of the affected side, widened intercostal space; weakened respiratory motility; percussive dullness; sound.
2. Imaging examination
In addition to symptoms and signs, the X-ray image of congenital chest and abdomen hiatus hernia has the following characteristics:
(1) X-ray fluoroscopy can be seen in the chest cavity and its peristalsis.
(2) X-ray plain film of the chest, abdomen or thoracic and upper abdomen showed that the image of the arched edge of the diaphragm was interrupted, unclear or disappeared, and the chest cavity contained a liquid-vapor surface or a honeycomb image of the gas-filled intestine, and the image was continuous in the chest and abdomen. The affected lung collapsed and the heart and mediastinum shifted to the healthy side.
(3) Gastrointestinal angiography shows gastrointestinal images in the thoracic cavity.
(4) CT and MRI scans can show the image of the honeycomb intestine in the edge of the ankle ring and its triangular cross section.
(5) B-ultrasound can be found in the chest cavity with dilated intestinal fistula and its peristaltic echo.
Differential diagnosis
Congenital pulmonary cyst
1 The disease is a process in which the lung buds in the early stage of the embryo develop from a solid cord to a hollow tubular shape. The proximal obstruction, the distal bronchus forms a caecum, the secretory mucus in the capsule cannot be discharged, and gradually expands to form a wall with bronchial tissue. Cysts, there are single and multiple pulmonary cysts, those who do not communicate with the bronchus are closed cysts, the cyst is filled with mucus, open to the bronchus is an open cyst, mucus is discharged through the small passage of the trachea, between the bronchi and the cyst Sometimes a one-way "valve" is formed. When inhaling, the air is easier to enter the cyst and expand. When exhaling, the gas in the capsule is difficult to be discharged and becomes a tension cyst, which compresses the normal lung tissue of the affected side and makes the mediastinum and heart. Displacement, the contralateral lung is also stressed, and respiratory function is impaired;
2 The disease is mostly in the green, the incidence of adulthood, rare in the neonatal period;
3 Most of the symptoms in the neonatal period are open cysts. The initial performance is shortness of breath. The breathing is inhalation dyspnea and continues to increase. Hypoxia and cyanosis, irritability, cold sweat in the head, and children with infection More convulsions, fever, but no gastrointestinal symptoms;
4 There are obvious signs of "three concaves" and increased intrathoracic pressure, but the liver or spleen is dulled down, the abdomen is not sunken, and the auscultation of the chest has no bowel sounds;
On the 5X line chest radiograph, the obvious "intrathoracic gas" sign can be seen. The image of the diaphragm of the affected side is intact, the wall of the capsule is thin, the image of the cyst does not continue to the side of the abdomen, and there is no gastrointestinal image in the chest of the digestive tract.
2. Identification of thoracic and abdominal hiatus hernia and sputum swelling
1 has congenital , Such as birth injury and damage to the neck 3,4,5 nerve roots), paralysis of the phrenic nerve;
2 rarely combined with lung developmental disorders;
3 similar to the symptoms of congenital chest and abdomen hiatal hernia, but acquired dystocia or birth injury history, multiple breech presentation, most children with Erb's palsy, clavicular fracture, tibiofibular fracture;
4 chest and abdomen X-ray film features, the diaphragmatic shadow is significantly increased, the dome is arched, the diaphragm is a complete and smooth curved shadow, there is a stomach bubble or intestinal shadow or liver shadow below the sputum, but the local swell and chest Abdominal hernia is difficult to identify, as long as the symptoms are obvious, there are surgical indications, surgery can be diagnosed and treated.
3. Other
Need to be associated with pneumonia caused by pulmonary bleb, pus and pneumothorax identification, as long as the detailed history, careful examination, combined with imaging examination is generally not difficult to identify.
