congenital esophageal duplication
Introduction
Introduction to congenital esophageal repetition Duplication of the digestive tract can occur anywhere in the pharynx to the anal digestive tract. Repeated esophageal malformation refers to a cystic or localized tubular enlarged hollow structure attached to the side wall of the esophagus. The road repeats deformity by 20%. basic knowledge The proportion of sickness: 0.004% - 0.005% Susceptible people: infants and young children Mode of infection: non-infectious Complications: neonatal asphyxia
Cause
Congenital esophageal recurrence
(1) Causes of the disease
The disease is a malformation caused by abnormal embryonic development. At present, the pathogenesis of esophageal duplication is still poorly understood. The mechanism of esophageal repetitive malformation has been cavitation in the early years, the formation of diverticulum and some twins, from the embryonic development process. The reasons for the malformation have been explained, but the most practical one is the phenomenon observed by Smith in photomicrography in 1957. He found that when the trachea and esophagus are separated for 4 to 5 weeks, a small amount comes from the tracheal or esophageal embryonic base. The cell mass is shifted into the mesodermal tissue that will become the esophageal muscle layer in the future, and then further developed into the outer membrane of the esophagus. If there is a small number of these cell clusters, the cells are gradually developed under the stimulation of the endoderm cells. It formed some deformities.
(two) pathogenesis
Gross pathology
According to their form, they are mainly divided into three types.
(1) Cyst type: Most of these types are spherical, elliptic or polycystic, attached to the side wall of the esophagus, most of which are not connected to the lumen, have longitudinal muscle fibers on the surface, and are filled with covered mucosal epithelium. Transparent or brown mucus, with the increase of secretions, cysts gradually increase, such as the main component of the inner wall is the gastric mucosal epithelium, which can secrete a large amount of gastric acid, which can cause ulceration, bleeding or perforation of the capsule wall. Between the wall muscles, it protrudes into the esophageal lumen, which is called the intramural type, and the other sticks to the wall of the esophagus and protrudes outward into the wall.
(2) Tubular type: On one side of the normal esophagus, there is an abnormal tubular cavity structure parallel to the esophagus, and one or both ends of the tube are connected or completely disconnected from the esophagus, and this type is rare.
(3) Diverticulum type: the esophagus protrudes outward from the esophagus and communicates with the normal esophagus. The outer part is the esophageal adventitia and muscle layer connected to the normal esophageal wall. The inner wall of the cavity is lined with mucous membranes, most of which are adjacent digestive tract mucosa. However, it may be partially or completely the same as the distant gastrointestinal mucosa, which is a variation of repeated malformations.
2. Microscopic pathology
The structure of the repetitive esophageal wall is similar to that of the normal digestive tract. There are two layers of smooth muscle tissue on the outside, the outer layer is arranged longitudinally, the inner layer is arranged in a ring, and then the inferior submucosa and mucosal muscle layer are inward, containing esophageal or bronchial glands, and the inner wall More covered with intact gastric mucosa, ciliated epithelium, squamous epithelium or pseudo-stratified columnar epithelium.
Prevention
Congenital esophageal repetitive prevention
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 esophageal recurrence complications Complications, neonatal asphyxia
Often cause respiratory infections, wheezing, difficulty breathing, severe cases can suffocate and die.
Symptom
Congenital esophageal repetitive symptoms Common symptoms Difficulty breathing Esophageal recurrent chest pain Dysphagia Difficulties Weight loss Anorexia hemoptysis
Repeated esophageal malformations mainly include hypopharyngeal difficulty, respiratory symptoms and chest pain. The severity of symptoms depends on the size of the lesion, the location, the type of mucosal epithelium and the presence or absence of infection. The repetitive malformation of the esophageal mucosa is the endometrium of the digestive tract and can be continuously secreted. Liquid, the volume of the lesion gradually increased to compress the surrounding tissue, such as compression of the esophagus obstruction symptoms, such as difficulty swallowing, reflux after eating, vomiting, followed by fear of eating, anorexia and weight loss, if the trachea, bronchi, lungs are compressed Tissue cough, wheezing, difficulty breathing and recurrent respiratory infections are roughly divided into two types. One is the digestive tract type, which is mainly digestive tract symptoms. It is more common in adults. There is no obvious symptom in the early stage of digestive tract until adulthood. After accidental discovery, or a slight gastrointestinal symptoms, after examination, it is found that this type of disease progresses slowly or no progress, few people are life-threatening, and the second is respiratory tract infection, hemoptysis, dyspnea as the main manifestation of respiratory tract, pediatric More common, the condition is generally heavier, this is because the deformity of children is larger than that of adults, and the trachea and lung tissue are compressed. Heavier, but the development of children's trachea is immature, the lumen is small, the tracheal ring cartilage is soft, the tracheal wall is thin and tender, the support force is weak, the anti-squeezing ability is poor, often cause respiratory infection, wheezing, difficulty breathing, severe suffocation Death, due to repeated oppression and erosion of the esophagus, can produce peptic ulcer, chest pain, perforation, hemoptysis, emergency measures should be taken.
Examine
Congenital esophageal repetitive examination
1. X-ray chest X-ray: see the posterior mediastinal mass shadow, such as tubular or wall-type can push the esophagus, trachea or bronchi, to make it shift.
2. Esophageal angiography: It can be seen that the curved edge of the lumen is smooth and curved, which is very similar to the esophageal leiomyoma, circular or elliptical, moving up and down with the swallowing movement (inside the wall).
3. Esophagoscopy: see mucosal normal, there are different degrees of pressure on the esophageal wall, mucosal biopsy is not helpful for diagnosis, because most of the tissues taken are normal esophageal mucosa, it is not recommended, and it will affect the extramucosal swelling after biopsy. Removal of objects.
4. CT and ultrasonography: It is helpful for the diagnosis and differential diagnosis of this disease. It is necessary for bronchoscopy in children with hemoptysis. The degree and location of tracheal or bronchial invasion can be observed, which is helpful for diagnosis. It also has guiding significance.
Diagnosis
Congenital esophageal repeated diagnosis
Infants and young children have clinical symptoms, especially hemoptysis should be thought of this disease, mostly caused by gastric mucosal ulcers in the cyst into the trachea or bronchus, such as found with genitourinary system, spinal or spinal deformity, combined with X-ray Esophagoscopy and CT examination support the diagnosis of this disease, but the confirmed diagnosis must be based on surgical findings and histological examination.
