Duplication of the esophagus
Introduction
Introduction to esophageal duplication Esophageal duplication (esophageal duplication) refers to a structure in which the side attached to the esophageal wall has the same tissue shape as a part of the digestive tract, and has a spherical or tubular cavity structure. Often considered to be a foregut cyst, accounting for approximately 30% of the mass in the mediastinum of the baby, usually located in the right posterior mediastinum. Some cases have deformities of the cervical vertebrae or thoracic vertebrae. The attachments of the masses are connected with the dura mater, which is called the chorizotomy syndrome. At this time, the repeated deformity is called the neural tube primitive cyst. Because the clinical symptoms of the child are complicated, the condition changes greatly. It is easy to delay diagnosis and treatment and cause adverse consequences. basic knowledge The proportion of illness: 0.001%-0.008% Susceptible people: no special people Mode of infection: non-infectious Complications: esophageal cyst anemia
Cause
Causes of esophageal duplication
Congenital factors (60%):
During embryonic development, when the upper digestive tract evolves from the solid phase to the vacuole phase, its vacuolation process is disordered, failing to fuse with the normal digestive tract, and finally forming a single or polycystic sac in the chest, and The esophagus is closely connected, that is, it becomes a repeated deformity of the esophagus. If a single vacuole remains, a round cyst-like repeat deformity is formed. If several vacuoles are strung together along the longitudinal axis of the esophagus, a lumen-like repeat deformity is formed.
Pathological morphology
Smith et al. performed microscopic studies on the separation of trachea and esophagus, noting that a small number of cells from the esophageal or tracheal embryonic basis were translocated into mesodermal tissue and became esophageal or extramuscular membranes outside these tissues, if A small number of these cells are left behind and continue to grow. Some of the deformities may be formed by the stimulation of endoderm cells, and the location of the repeat deformity depends on the distance from which the cells are separated from the original embryonic base, and the epithelium is displaced. The form is different, and the type of deformity is different.
In pathological anatomy, there are generally 2 esophageal lumens in the deformity. The inner membrane of the deformed tube is mostly ectopic gastric mucosa. Sometimes a small amount of pancreatic tissue is visible. The gastric mucosa secretes gastric acid, often causes peptic ulcer, hemorrhage, and the middle layer of the cystic wall is smooth muscle. The outer layer is a serosa layer, and the cyst can also be co-walled with the esophagus, which is not easy to separate and has no serosa.
Pathological typing
According to the morphology and pathological features of the cyst, the clinical classification is as follows:
(1) Single cyst type: clinically common, different forms, but the overall structure is a large capsule.
(2) multiple cystic type: there are multiple isolated cysts on the leading edge or both sides of the mediastinal spine, and some may also occur in the anterior mediastinum.
(3) luminal type: the lower part of the esophageal cyst located in the chest is a thin tube that passes through the diaphragm into the abdominal cavity and communicates with the intestine.
(4) chest, abdomen multi-segment repeat type: in addition to the esophageal cyst in the chest, the abdominal intestine can also occur multiple segments of repeated deformity.
(5) Diverticulum type: very rare.
Prevention
Esophageal repeated deformity 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
Esophageal duplication malformation complications Complications Esophageal cyst anemia
Anemia and respiratory infections are common complications of repeated esophageal malformations. Long-term chronic blood loss of luminal esophageal cysts is the main cause of anemia, while tracheal compression is narrow, secretions cannot be discharged in time, and acidic cysts are on the tracheal wall. Corrosion is an important factor leading to respiratory infections.
Symptom
Esophageal repeated deformity symptoms Common symptoms Acoustic dysphagia, sputum, tracheal displacement, dyspnea, tracheal compression, pulmonary infection, hemoptysis, shortness of breath, suffocation
It depends mainly on the size of the cyst, the presence or absence of internal bleeding and infection, and the symptoms of different degrees of compression or corrosion of adjacent organs (trachea, esophagus, lung). The onset can be acute and slow, and the following symptoms are often seen:
Symptom
(1) Respiratory obstruction: stenosis of the trachea can cause shortness of breath, wheezing, bruising around the mouth, such as infection in the lungs, the hypoxia is more obvious, severe cases can suddenly suffocate, when the cyst is huge, it can cause mediastinal shift The heart is stressed, cardiopulmonary dysfunction can occur at the same time, such as timely diagnosis and treatment, emergency cyst puncture decompression, the condition can be relieved immediately, in order to win the timing of surgery, otherwise, it is easy to cause death.
(2) hemoptysis, blood in the stool: large cysts often adhere to the lungs, acidic cystic fluid corrodes the cyst wall into the lungs, causing lung tissue destruction, vascular bleeding and manifested as hemoptysis, there are two sources of blood in the stool: 1 child does not It will vomit, the blood from the trachea is swallowed into the intestine through the pharynx, and the internal hemorrhage of the 2 luminal esophageal cyst can be directly discharged from the intestine.
(3) dysphagia or vomiting: As the cysts increase the pressure on the esophagus, the child may have progressive dysphagia, even vomiting, and the sputum examination is easy to confirm.
2. Signs
The affected side is full of thoracic, the intercostal space is widened, the main trachea is displaced, the percussion is voiced, and the auscultation may have a reduced breath sound. When the lung is infected, it may smell wet.
3. Clinical manifestations
There are no obvious incentives for breathing difficulties, cyanosis, hemoptysis, blood in the stool and difficulty in swallowing. Physical examination: the affected side is full of thoracic, the intercostal space is widened, the main trachea is displaced, and a large deformity of the neck occurs in the neck. See the bulging mass on the side.
4. Auxiliary inspection
Prompt posterior mediastinal mass, oppression of the esophagus and trachea.
Watersion's study of 50 cases of esophageal repetitive malformation pointed out that there are two important manifestations of this type of malformation in the diagnosis. One is that the esophageal repetitive malformation has more gastric mucosal tissue, and it is prone to ulceration, and even penetrates the wall of the tube into the trachea, causing hemoptysis. Secondly, the repeat deformity is more complicated with a half vertebral deformity.
Examine
Examination of esophageal duplication
The cyst fluid was taken for examination. The normal cyst fluid was light yellow, clear, slightly sticky, and the pH was too acidic. When there was old internal bleeding, it was dark purple or brown.
1. X-ray inspection:
(1) Chest flat: large esophageal repetitive deformity can be shown as a soft tissue mass with uniform density and clear boundary in the posterior mediastinum, which widens or protrudes into the chest cavity, even occupying most of the chest, and the trachea is opposite or The front is displaced and the lumen is sometimes narrowed.
(2) esophageal barium angiography: visible curved indentation and advancement, a small number of cysts can be inflated or have a gas-liquid surface, esophageal repeated deformities can have the following forms:
1 semi-circular or triangular cyst: its long axis is consistent with the esophagus, and the triangular shadow cyst extends into the right middle and middle interlobular fissures, which is caused by the compression of the upper and middle lobe. The semicircle is the most common type, and the edges are sharp. It can occupy half of the chest, compress the surrounding lung tissue, and the mediastinum shifts to the opposite side.
2 neck and superior mediastinal cyst: semi-circular protrusion to the tip of the lung, compression of the trachea to the contralateral displacement, lateral radiograph showing thick tissue shadow of the posterior pharyngeal wall, tracheal lumen narrowing forward.
3 semi-goose-shaped cyst: when the right cyst is large, there is often a dent between the upper part and the lower part, like a gourd shape, which may be caused by vascular compression.
4 inflatable cysts: This type of cyst is rare. Some authors report that 1 case is a 6-day baby boy. There is a huge thin-walled inflatable cyst in the right thoracic cavity. The lung tissue can be seen in the lung tip and rib angle. The cyst protrudes backwards and downwards, and the heart shadow shifts to the left. The preoperative diagnosis is congenital pulmonary cyst. The surgery and pathology confirmed the intrathoracic repeat deformity. It contains milk blocks and green liquid, which communicate with the underarm duodenum.
5 mediastinal soft tissue shadow widened and extended to the underarm to connect with the gastric cyst.
6 tuning fork esophagus: repeated esophageal tube is parallel with the normal esophagus, the lower part of the confluence, can be combined with esophageal hiatus hernia, reflux esophagitis and esophageal stricture, mediastinal without mass shadow.
In some cases, vertebral malformation (vertebral canal fissure, semi-vertebral body more common), lung non-development, esophageal atresia, intra-abdominal repeat deformity, etc. on X-ray films are of great significance for diagnosis.
2. B-ultrasound and CT examination:
It has important reference value for identifying the nature of the tumor (cystic or solid).
3. Fiber esophagoscopy:
It can be seen that the tumor protruding into the esophageal cavity is generally not examined by microscopy, so as to avoid tissue adhesion and affect the surgical treatment.
Diagnosis
Diagnosis and diagnosis of esophageal duplication
Diagnosis is based on clinical performance and examination.
The disease needs to be differentiated from empyema, intrathoracic teratoma, intrapulmonary tumor, bronchial cyst, neurogenic tumor and anterior spinal dural bulging.
