congenital esophageal stricture
Introduction
Introduction to congenital esophageal stricture Congenitalesophagostenosis (CES) refers to the abnormal shape of the esophageal wall structure that is already present after birth. It is very rare in the clinic, more than the onset of childhood, often requires surgery. Clinical attention should be paid to the difference between secondary esophageal stricture. basic knowledge The proportion of illness: 0.003% Susceptible people: young children Mode of infection: non-infectious Complications: aspiration pneumonia, acute respiratory distress syndrome in children
Cause
Causes of congenital esophageal stricture
(1) Causes of the disease
The disease is caused by excessive proliferation of the components of the trachea, the base of the esophagus or the esophageal sac in the development of the esophageal embryo, and occurs mostly in the position below the tracheal bifurcation.
(two) pathogenesis
In embryonic development, in the vacuole stage of esophageal formation, excess mucous membranes are absorbed, vacuoles disappear, but some or complete esophageal mucosal annular septum remains, and others believe that scaly epithelium replaces villi during esophageal development. Columnar epithelial cells are caused by excessive growth.
According to the pathological features of congenital esophageal stricture, Nihoul-Fekete (1987) divided it into 3 types:
1. Membrane sputum or sputum formation: also known as membrane stenosis, is the most rare type, it is considered to be a confusing form of esophageal atresia, often occurs in the middle or lower part of the esophagus, membrane or sacral surface The squamous epithelium is covered, and the typical onset of symptoms is just when eating is heavily weighted into solid food.
2. Fibromuscular hypertrophy: also known as idiopathic muscular hypertrophy or fibromuscular stenosis, is the most common type of congenital esophageal stricture, histological features are submucosal smooth muscle fibers and fibrous connective tissue hyperplasia and normal scale Epithelial coverage, similar to pyloric hypertrophic stenosis, but no clear embryonic and pathogenic factors to explain the lesion.
3. Remnant trachea in the esophageal wall, bronchial tissue: also known as tracheal primordial stenosis, its trachea and bronchial residuals may be part of the anterior genital pleats of the embryo. In 1936, Frey and Duschel first reported a 19-year-old female. After dying from the achalasia, it was discovered that Holder et al. (1964) paid special attention to 1058 cases of esophageal end stenosis in infants with esophageal atresia. In 1973, Spitz confirmed that the disease was based on congenital lesions. In Germany, nearly 50 cases of congenital esophageal stenosis caused by residual esophageal bronchial tissue were reported.
Congenital esophageal stricture occurs in the upper or lower part of the esophagus. It is reported that about 50% occur in the third half of the esophagus, and 25% occurs in the lower third of the esophagus. Fibromuscular hypertrophy is more common in the thoracic esophagus. Or the lower part of the esophagus, while the tracheal wall remains trachea, bronchial tissue type is narrow, it is found in the lower part of the esophagus.
Prevention
Congenital esophageal stricture prevention
Pregnant women with excessive amniotic fluid should be alert to the possibility of congenital malformation, amniocentesis and amniotic fluid alpha-fetoprotein, acetylcholinesterase increased at the same time to help prenatal diagnosis.
Complication
Congenital complications of esophageal stricture Complications, aspiration pneumonia, acute respiratory distress syndrome
Patients with esophageal malformation and tracheal fistula, more often within a few days after birth, cough, runny nose and aspiration pneumonia, esophageal aponeurosis, the symptoms are also earlier, the younger the baby, the symptoms of aspiration pneumonia The more obvious, the symptoms of respiratory distress often occur.
Symptom
Congenital esophageal stenosis symptoms Common symptoms galactorrhea growth slow cough esophageal obstruction clock swing sign wheezing esophageal stenosis migraine
Congenital esophageal stenosis often has symptoms such as slow cough, galactorrhea, and esophageal obstruction with age. Children are often accompanied by slow growth, stunting, and younger age, the incidence of inhaled pneumonia. The higher the height.
Examine
Congenital esophageal stricture examination
Feasible 24h esophageal pH monitoring, if necessary, take the esophageal mucosal living tissue for pathological examination to aid diagnosis.
Esophageal barium meal angiography
According to the imaging characteristics of barium meal angiography, esophageal stricture can be divided into two types.
(1) Long-segment type: stenosis occurs in the lower part of the esophagus, about several centimeters long, the stenosis edge is not smooth, the esophagus is dilated above the stenosis, the sputum is slow, and the peristalsis is seen. The clinical symptoms of this type appear earlier, and the reflux Sexual esophagitis is similar, and X-ray is difficult to identify.
(2) short segment type: often occurs in the esophagus, at the junction of the lower segment, the stenosis segment is about several millimeters to 1 cm long, the edge is smooth, the mucosa is regular, the esophagus is slightly dilated above the stenosis segment, the expectorant is descending, and the distal esophagus is narrow. The shape is normal, and sometimes foreign matter or food blocks are likely to remain on the stenosis, and the narrow segment cannot expand.
This disease is often associated with aspiration pneumonia, routine chest X-ray examination.
2. Esophagoscopy
Pediatric microscopy provides a primary objective basis for the nature of the stenosis.
3. Esophageal pressure measurement.
Diagnosis
Diagnosis and diagnosis of congenital esophageal stricture
diagnosis
Different from congenital esophageal stenosis, vomiting occurs in infants with sputum sputum 2 to 3 times / day or once every 2 to 3 days, the amount is rot and odor, and the esophageal dilation can be seen by X-ray examination. It causes difficulty in the passage of tincture, and the typical end of the esophagus is a typical beak-like stenosis. Esophageal angiography of the esophageal stricture shows that the stenosis can still pass through the stenosis, so there is contrast agent retention between the stenosis and the stomach, congenital shortness. Esophageal and congenital diaphragmatic esophageal hiatus development defects, is conducive to the formation of congenital esophageal hiatus hernia, its pathological features are transversely wide and wide esophageal hiatus, the muscle fibers around the hiatus are thin and weak, the stomach into the chest cavity to varying degrees, The vagus nerve is particularly loose, and the vagus nerve is particularly loose. 80% of congenital esophageal hiatus hernia develops symptoms 1 week after birth, 15% occurs within 1 month. Typical symptoms are vomiting, vomit often has blood, and many children have more No difficulty in swallowing, so I still want to eat after vomiting, but if crying when swallowing, there may be ulcers causing pain. The diagnosis depends on X-ray examination. After seeing, the stomach can be seen in the chest after swallowing. Continuous esophageal pH measurement showed that the esophageal pH was significantly acidic. Such children sometimes had congenital pyloric hypertrophy, migraine and migrating migrating syndrome (migrine and periodic syndrome), tracheal malformation or mental retardation. Congenital esophageal stenosis, it should also be noted that congenital esophageal stricture often coexists with congenital esophageal hiatus hernia, the vast majority of stenosis occurs in the lower and middle esophagus.
Therefore, all infants and young children have vomiting after birth, especially after eating, and progressive exacerbation should take into account the disease, esophagography can make a preliminary diagnosis.
Children repeatedly have food reflux or cough after meal, and older children have postprandial wheezing, etc., should be highly suspected of this disease, X-ray esophageal barium meal and esophagoscopy showed esophageal stricture or membranous fistula, etc., can be clearly diagnosed .
Differential diagnosis
X-ray examination is the main basis for the diagnosis of this disease, and its image needs to be identified with the following diseases:
1. Cardiac achalasia: the stenosis is located in the cardia, intermittently open, the tincture can be sprayed into the stomach, congenital esophageal stricture is persistent stenosis, no open jet signs, but the expectorant can continue to pass, usually narrow The above esophageal dilatation is not as obvious as the achalasia.
2. Acquired reflux esophagitis: the esophagus in the stenosis is not smooth, uneven, mucosal destruction or sputum, sometimes visible hiatus hernia. In the follow-up observation, the degree of stenosis can be aggravated, lengthened, and clinical symptoms are aggravated.
