neonatal congenital diaphragmatic hernia
Introduction
Introduction to congenital sputum in newborns Congenital diarrhea (CDH) is a disease caused by dysplasia of the diaphragm in the embryonic period to the unilateral or bilateral diaphragmatic muscles, and some abdominal organs enter the thoracic cavity through the defect, resulting in abnormal anatomical relationship., hiatal hernia and congenital sternal posterior iliac crest. basic knowledge The proportion of illness: 0.035% Susceptible people: children Mode of infection: non-infectious Complications: neonatal respiratory distress syndrome metabolic acidosis gastroesophageal reflux disease intestinal obstruction shock anemia
Cause
Neonatal congenital delirium
(1) Causes of the disease
Part of the diaphragmatic defect in the development of the embryo is the basis of the disease. The attachment site around the diaphragm is divided into three parts, namely the sternum, the rib and the spine. There are three sites for the sputum:
1. chest and abdomen hole (Bochdalek hole): there is a small triangular gap between the posterior edge of the bilateral rib and the outer edge of the lumbar rib arch, called the chest and abdomen hole (Bochdalek hole), where the posterior lateral iliac crest, ie the chest and abdomen Rifted sputum or Bochdalek , 85% to 90% of congenital sputum is thoracic and abdominal hernia, of which 80% on the left side, 15% on the right side, less than 5% is bilateral, the incidence rate is 1:10000~1 3,000, males are slightly more than females, 28% to 31% are accompanied by malformations, and cardiovascular system abnormalities are more common. The main symptoms are respiratory distress, and many symptoms appear in the neonatal period. In recent years, the concept of treatment has been significantly improved. The effect has been improved.
2. Posterior sternal or Morgagni: a small triangular gap between the lateral edge of the sternum and the medial edge of the bilateral ribs, called the Morgagni hole, normal connective tissue filling, this hole occurs nicknamed the posterior sternum or Morgagni, in clinical It is relatively rare.
3. Esophageal hiatus hernia: The esophageal hiatus is fusiform, with a tough connective tissue connection between the periphery and the esophageal wall. The anterior and posterior walls are tightly connected and the two sides are weak. If there is a defect, the esophageal hiatus is called the disease. In particular, there is no definitive statistical data for children. In the past, it has been considered that Europe is more common, but it is rare in North America. In recent years, due to the improvement of detection technology at home and abroad, especially with pediatric professional X-ray physicians, the disease has increased year by year. Not rare in China
(two) pathogenesis
Generally, during the 10th week of pregnancy, when the intestine returns to the abdominal cavity through the umbilical cord basal, due to the presence of the thoracic and abdomen, the intestine can enter the chest through the thoracic and abdomen, and even the Dalian stomach, spleen, colon, left lobe and the like are brought into the chest. Internal lung dysplasia is closely related to sputum. The severity of lung dysplasia is related to the time and extent of visceral sputum formation. The clinical manifestations are related to the surface area of the affected alveolar and pulmonary vascular bed and other malformations. In the autopsy of dying stillborns, 95% of the children had other defects, and many deaths were associated with these malformations. About 25% of the sputum was associated with poor intestinal rotation, and 10% to 20% of cases had sacs. Different degrees of lung dysplasia due to visceral embedding, bronchial growth arrest, the number is reduced, the total amount of alveolar is reduced, the total number of pulmonary branches is also reduced, and the pulmonary arteriolar muscle layer is thickened, the resistance is increased, resulting in neonatal pulmonary hypertension, pulmonary hypertension Causes a right-to-left shunt of the foramen ovale and the patent ductus arteriosus, hypoxemia and hypercapnia, which in turn promotes pulmonary vasospasm Circulatory referred newborns with persistent pulmonary hypertension (of PPHN) clinically.
Lung dysplasia causes changes in pulmonary ventilation function and pulmonary hemodynamics. For example, neonates with sputum begin to breathe after birth, swallow air into the gastrointestinal tract in the chest, aggravate compression of the lungs, and increase pulmonary vascular resistance. Closed arterial catheter and foramen ovale produce shunt from right to left. All of this includes acidosis, which stimulates pulmonary tracheal spasm, and leads to increased lung tracheal resistance and pulmonary hypertension. Figure 1 shows the pathophysiological changes in children with sputum.
If these patients do not get an effective treatment after birth, it will definitely die. Of course, a small number of children are not obvious in the neonatal period. Because of the small defect of the thoracic and abdominal cavity, the lung development is not damaged, so the condition appears. Also light.
Prevention
Neonatal congenital spasm prevention
Should be well-preserved during pregnancy, prevention and treatment of various infectious diseases during pregnancy, strengthen nutrition and monitoring during pregnancy, such as the discovery of polyhydramnios and detection of lecithin and sphingomyelin below normal, ultrasound imaging can be seen in the fetal thoracic cavity, such as abdominal organs Prenatal diagnosis can be made. If there are other malformations such as chromosomes or heart, if necessary, the pregnancy can be terminated or treated as soon as possible.
Complication
Neonatal congenital diaphragmatic complications Complications neonatal respiratory distress syndrome metabolic acidosis gastroesophageal reflux disease intestinal obstruction shock anemia
Concurrent respiratory distress, metabolic acidosis, hypoxemia, hypercapnia, gastroesophageal reflux, intestinal obstruction, and even shock, etc., can also be associated with scarring of the lower esophagus, anemia, nutritional deficiencies, growth and development disorders, repeated Respiratory infections, etc.
Symptom
Neonatal congenital diaphragmatic symptoms Common symptoms Chest tightness, cyanosis, shortness of breath, dyspnea, abdominal distension, blood in the stool, stop the exhaust, black stool, abdominal depression, scaphoid peritonitis
1. Thoracic and abdominal hernia: The main clinical manifestations in the neonatal period are acute symptoms of respiratory, circulatory and digestive systems, but with respiratory symptoms as prominent.
(1) Symptoms: Difficulty breathing, urgency, cyanosis and other symptoms may appear after birth or within hours after birth, the severity depends on the size of the diaphragmatic defect, the number of abdominal organs entering the chest and lung dysplasia, Difficulty breathing and cyanosis can be paroxysmal and variability, that is, aggravation during crying or eating, sudden aggravation and progressive deterioration, when breathing crying, vigorously breathing, the affected chest produces a great negative pressure, the abdominal cavity Organs are incorporated into the chest, causing severe breathing difficulties. If not treated or treated improperly, they can die immediately. The abdominal organs enter the chest not only to oppress the lungs, but also to distort the pulmonary artery, thicken the arterial wall, reduce the cross-sectional area of the vascular bed, etc. The result is persistent pulmonary hypertension, in addition to cyanosis, shortness of breath, acidemia, hypoxemia, hypothermia, hypocalcemia, hypomagnesemia and other symptoms, vomiting symptoms are relatively rare in the clinic, only chest and abdomen Vomiting occurs when the hiatal hernia is associated with intestinal malrotation or intestinal obstruction caused by intubation of the abdominal cavity.
(2) Signs: The thoracic respiratory movement of the affected side is weakened, full, the intercostal space is widened, and the heart is displaced to the healthy side. Sometimes it is misdiagnosed as the right heart. The chest is percussed with dullness or drum sound, which is often turbid drum sound, which is Involvement of the nature of the chest organ or the degree of intestinal inflation, auscultation of the side of the breath sounds weakened or disappeared, and often can hear the bowel sounds, which is important for the diagnosis of congenital diaphragmatic hernia, the position of the neonatal diaphragm is low, often Up to the 8th to 9th level of the thoracic vertebrae, the diaphragm and the chest and abdomen wall are weak, it is easy to transmit the bowel sounds to the chest, so it should be repeatedly examined and analyzed, there is a diagnostic significance, the abdomen is a boat-shaped abdomen, because the abdominal organs break into the chest Become emptiness, if there is less sputum, then the sag is not obvious.
2. Esophageal hiatus hernia: Pediatric esophageal hiatus hernia is more common in infants and young children, clinical manifestations are diverse, and can not describe the medical history, there is no typical clinical symptoms, if the parents do not observe carefully, often delay diagnosis and treatment, common clinical manifestations are as follows Several kinds:
(1) Vomiting: The most common symptoms of full-term newborns, infants and elderly children, accounting for 80% to 95%, can occur in the first week after birth, vomiting in various forms, often in supine position or at night Heavy, sometimes slightly milky, severe jet vomiting, vomit is initially stomach content, accompanied by bile in severe cases, often due to reflux esophagitis of the lower esophagus, vomiting coffee-like liquid or hematemesis, but the amount is not More, if you can often maintain a semi-sitting position or feeding, a thick diet, vomiting is significantly improved, after 8 to 9 months of illness, the number of vomiting is reduced, may be a better condition, may also be the lower esophageal fibrosis to form scarring stenosis.
(2) hematemesis, blood in the stool: in addition to vomiting coffee-like substances in sick children with vomiting, hematemesis, tar-like and black stools, most stool tests, occult blood is often positive, prolonged hematemesis and blood in the stool because of Due to reflux esophagitis, insufficient nutrient intake, sick children appear anemia, hemoglobin is often between 80 ~ 100g / L, body length, weight is often lower than the same age, resulting in poor growth and development.
(3) symptoms of respiratory tract infection such as cough, asthma, etc.: Because gastroesophageal reflux occurs mostly at night, it often causes aspiration, repeated symptoms of respiratory infection, 30% to 75% of infants and childhood esophageal hiatus hernia is repeated respiratory tract Infection is the main complaint, and although the anti-inflammatory treatment, respiratory infections can be improved, but can not be cured, because some children usually do not notice a small amount of stomach contents, often inhaled trachea, forming repeated respiratory infections, some allergies A sick child with a small amount of stomach contents was mistakenly sucked into the trachea, resulting in an allergic asthma-like episode.
(4) Dysphagia: The reflux esophagitis of the sliding esophageal hiatus hernia gradually worsens, the inflammation has invaded the muscular layer, and the lower end of the esophagus is fibrotic. As a result, not only the esophageal shortening is caused, but also the gastric fundus of the cardia enters the chest cavity, and it also appears. Esophageal stricture, often dysphagia, early fasting and anti-inflammatory treatment can be improved, in the late stage can not eat or vomit white mucus.
(5) paraesophageal sputum: sometimes the esophagus and stomach junction is still in the normal position of the abdominal cavity, there is a stomach part of the stomach into the chest cavity or twisting to the right side of the sputum, poor gastric exhaust caused by retention gastritis, ulcers, bleeding, Torsion for too long, embedding, obstruction, post-sternal pain, chest tightness, shortness of breath.
(6) Physical examination: poor development and nutritional status of the sick child, anemia appearance, general examination without positive signs, only when the giant esophageal hiatus hernia occurs or gastric torsion, the upper abdomen may have symptoms of peritoneal inflammation, lung breath sounds weakened.
3. Congenital sternal posterior iliac crest: There is no specific clinical symptom of parasternal paralysis, usually accompanied by crying, supine position, increased abdominal pressure, paroxysmal dyspnea, shortness of breath, cyanosis, etc. Position, quiet, when the abdominal pressure is reduced, the above symptoms disappear or reduce. If the digestive tract into the chest cavity is embedded, vomiting, abdominal distension, stop exhaustion and other signs of intestinal obstruction, sometimes gastrointestinal bleeding, causing anemia Signs, the colon into the chest often has upper abdominal discomfort.
Examine
Neonatal congenital diaphragmatic examination
Blood gas analysis should be done, PaCO2 increased, up to 8 ~ 19kPa (60 ~ 142mmHg); PaO2 decreased significantly, up to 5 ~ 10.4kPa (38 ~ 78mmHg); blood pH can reach 6.85 ~ 7.11, to determine the emergence of respiratory Acidosis, or metabolic or mixed acidosis.
1. X-ray examination: It is an important means of diagnosing this disease. The chest radiograph shows a round or elliptical shadow on the top of the palpitations. The lateral position is like an inflated or liquid-faced shadow behind the sternum of the precordial area. Enema examination not only confirms the diagnosis, but also identifies the type of organ that breaks into the chest.
For the esophageal hiatus X-ray examination is the main method of diagnosis, it can fully understand the shape of the stomach, location, esophageal hiatus size and gastric peristalsis changes.
X-ray images of thoracoabdominal hernia have the following characteristics:
(1) The image of the transverse edge of the diaphragm is interrupted, unclear or disappears.
(2) The chest cavity contains a liquid-vapor surface or a gas-filled intestinal tube honeycomb image, and this image has a continuous chest and abdomen.
(3) The affected side collapsed and the mediastinum shifted to the healthy side.
2. B-ultrasound: It can be found that there are dilated bowel and frequent peristalsis in the thoracic cavity, accompanied by swimming without shadow of echo and gas echo, and the mucosa of the effusion sometimes shows mucosal folds.
3. MRI examination: It is also helpful for the diagnosis of this disease. The MRI coronal plane can clearly see the edge of the ankle ring and the image of the intestine in the thoracic cavity, while the cross-section ankle ring is triangular, with a honeycomb shadow of the intestine. This is similar to CT examination.
4. Endoscopy: It is very important for the pathological changes of esophageal hiatus and the severity of gastroesophageal reflux. It can directly observe the appearance of esophageal mucosa, congestion, edema, erosion, hemorrhage, stenosis, etc., and also observe the retention of esophagus; The degree of relaxation, the amount of gastric mucosa into the esophageal lumen; the distance between the esophageal mucosa and the gastric mucosa to the esophageal hiatus, which is not only conducive to diagnosis, but also provides an objective indicator of further treatment and efficacy judgment of the disease.
5.99mTc radionuclide scanning: The type of esophageal hiatus hernia can be determined according to the characteristics of the scanned image.
6. Esophageal pH 24h dynamic monitoring: using the pH microelectrode portable recorder to monitor the pH value of the lower end of the esophagus, record and mark the start and end time of eating, sleeping, body position and vomiting, and then analyze the monitoring results by computer and software. Simultaneous monitoring of the lower pH of the esophagus, gastric antrum and gastric fundus can be used to determine whether there is duodenum, and gastric reflux is alkaline reflux, which is very important for the choice of surgery and prognosis. .
7. Esophageal pressure measurement: The physiological pressure gauge is used to measure the lower end of the esophagus and the pressure in the stomach. The length of the high pressure region at the lower end of the esophagus, the pressure and the pressure in the stomach, and the pressure difference between the two can be observed.
Diagnosis
Diagnosis and diagnosis of neonatal congenital diaphragmatic hernia
diagnosis
1. Prenatal diagnosis: maternal amniotic fluid in the fetus, amniotic fluid detection can be found that lecithin and sphingomyelin are lower than normal, ultrasound imaging can be seen in the fetal thoracic cavity with abdominal organs, amniocentesis, see contrast agent in the fetal thoracic cavity Within the fetus, intrauterine diagnosis can be made. The prenatal diagnosis time is related to the prognosis. The earlier the time, the worse the prognosis. The prognosis is better when the diagnosis time is greater than 25 weeks.
2. Diagnosis of congenital diaphragmatic hernia: After birth, the newborn has obvious hypoxia, difficulty in breathing, the chest of the affected side and the bowel sounds, and the heart is shifted to the healthy side. The congenital diaphragmaticity should be considered first.
3. Auxiliary examination and diagnosis: X-ray examination often has an unclear contour on one side, and an irregular translucent area or liquid surface caused by inflation of the intestine or stomach bubble in the chest cavity. The parasternal fistula is common in the right anterior palpebral horn area. Downward bulge, densely shadowed edges, gas in the middle, abnormity on the right posterior lateral side, liver elevation or substantial shadow can be misdiagnosed as a tumor, for a more definitive diagnosis, can be placed into the stomach tube or oral contrast agent If the stomach tube or contrast agent is seen on the affected side of the chest, the diagnosis is established, the neonatal banned sputum examination, and the diagnosis may be performed by CT.
Differential diagnosis
In the differential diagnosis, it is necessary to distinguish from sputum swelling, congenital pulmonary vesicle disease, congenital heart disease, pleural effusion and lung inflammation.
In particular, it is congenital sputum swelling, which is caused by the lack of muscle layer and collagen fiber layer of sputum. The sputum becomes a translucent thin membrane composed of peritoneum, and clinical manifestations can also cause dyspnea, sometimes in X. It is difficult to distinguish the sputum from the squat on the flat chest and abdomen. However, when observing the respiratory movement and watching the sputum movement on the X-ray screen, the squat movement can often be abnormally squatting; that is, the sputum drops during normal inhalation, and the sputum is swollen during normal inhalation. The lateral diaphragm muscles rise instead, and the diaphragmatic muscles of the affected side decrease when exhaled.
