Congenital short esophagus

Introduction to congenital short esophagus Short esophagus means that the length of the esophagus is shorter than normal. Due to the short esophagus, a portion of the esophagus that replaces the shortage of the corpuscle is located above the diaphragm to become the thoracic stomach. The cause is congenital and secondary, congenital short esophagus is a relatively rare deformity. basic knowledge The proportion of illness: 0.0003% Susceptible people: infants and young children Mode of infection: non-infectious Complications: Bronchopneumonia

Congenital short-term esophagus

(1) Causes of the disease

The congenital short esophagus is a disorder in the development of the embryo, forming a short esophagus. The mechanism is still unclear. After the tracheal separation in the 5th to 6th week of the embryo, the tubular esophagus has formed. With the development of the thoracic cavity, the esophagus is also rapidly pulled. Long, the stomach moves down, and when it moves to the armpit, it is fixed in the lower abdomen cavity. If there is an obstacle in this process, the stomach body fails to reach the armpit, the cardia and part of the stomach are Fixed in the chest, the length of the esophagus is short, forming a congenital short esophagus and chest and stomach malformations.

(two) pathogenesis

Pathological anatomy, congenital short esophagus combined with the thoracic stomach without serosal membrane, the blood vessels from the thoracic aorta, and never in the normal position of the abdominal cavity, so unlike the esophageal hiatus hernia, the esophageal lumen is often reduced in the short esophagus change.

Congenital short tube 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.

Congenital short-term esophageal complications Complications bronchopneumonia

Easy complicated with bronchial pneumonia.

Congenital short-term esophageal symptoms Common symptoms Gastroesophageal reflux symptoms, atelectasis, sputum, cough, repeated upper respiratory tract infection, weight loss, dysphagia, dyspnea, upper gastrointestinal bleeding, esophageal stenosis

The natural progression of the disease varies, and can be roughly divided into two categories, that is, some have no clinical manifestations, and the patient has no discomfort. It is only found by chance, and the other part is mostly accompanied by symptoms of varying degrees, such as Flow, vomiting, cough, gastrointestinal bleeding and respiratory infections, this disease is more common in infants and young children, vomiting is sometimes normal, and the function of the cardia is not yet perfect, but if it continues for more than 4 months, it is still vomiting, and it is regular Most of them are pathological. The vomiting of this disease is characterized by aggravation of the lying position, reduction or disappearance of the standing position, continuous vomiting, insufficient fluid and calorie intake, and weight loss and dysplasia in the child. In the case of secondary reflux esophagitis Esophageal intima erosion, ulcers, vomit can carry blood, there are black stools or tar-like stools, children crying during feeding may be caused by swallowing pain caused by reflux esophagitis, due to infant sleep In the supine position, the stomach content cannot be evacuated by gravity. In addition, the pressure on the upper esophageal sphincter is reduced when sleeping, so gastroesophageal reflux often occurs, and because the child does not have the ability to adjust the position independently, it is easy to inhale the gas. Causing cough, fever, bronchial pneumonia, segmental atelectasis in the tube, in the congenital short esophagus of the chest and stomach, the chest and stomach swollen after a full meal pressure the lungs, respiratory tract and heart, so it can show cough, difficulty breathing, Shortness of breath and pulse, and short esophagus with esophageal stricture have difficulty swallowing.

Congenital short esophagus examination

1. X-ray chest examination

Ordinary posterior chest radiographs have a cystic semicircular cavity and liquid level shadows protruding from the mediastinum to the right lung field or the left lung field, the edges are clear, and the lateral chest radiograph is located in the posterior mediastinum.

2. Upper gastrointestinal angiography

It can be seen that there is a stomach bubble on the diaphragm. If the tincture is filled satisfactorily, the gastric mucosa in the stomach cavity is not difficult to identify. The esophagus and stomach junction is above the diaphragm, usually at the T7 or T8 level, the position is fixed, sometimes there is a narrow, and the esophage length is short. The level of the diaphragm is not reached, the upper part of the esophagus is slightly dilated, the thoracic stomach passes through the stenosis of the diaphragm, and there is no positional movement in the supine position and the standing position.

3. Esophagoscopy

The esophageal mucosa and the gastric mucosa can be clearly observed. The esophageal mucosal mark is a jagged scaly columnar epithelial junction. Under normal circumstances, the boundary between the two is clear. The esophageal mucosa is smooth and pale red, and the mucosal folds of the stomach are coarse. Dark red color, easy to identify, but when the esophagus has inflammation, congestion, edema, redness, erosion, ulcers, bleeding is not easy to distinguish, at this time can be stained with 2% Lugol solution, the gastric mucosa appears blue, if you can pass this ring, Further down into the inferior part of the stomach, a mild stenosis ring appears as a sacral hole. The ring is characterized by narrowing when inhaling, widening during exhalation, and entering the underarm cavity through the ring. The upper stomach and the infraorbital stomach change the size of the stomach cavity when inhaling and exhaling. The upper stomach is inhaled and the exhalation becomes smaller; the lower abdomen is inhaled and the exhalation becomes larger. This can be used to identify the position of the gastric sac, clearly determine the depth of the gastric vestibule and the sacral hole, calculate the length of the esophagus, the degree of stenosis and the nature of the stenosis, is caused by inflammation or edema or scars must be identified, generally congenital The length of the narrow area is about 2cm short Fibrosis induced esophagitis stenosis are long and diffuse.

Diagnosis of congenital short esophagus

diagnosis

According to clinical manifestations, common chest radiographs and upper gastrointestinal angiography can confirm the diagnosis.

Differential diagnosis

1. Hiatal hernia

Congenital short esophagus is a rare disease, because it is very similar to the clinical manifestations of hiatal hernia, and the treatment methods of the two are different in principle, so the differential diagnosis between the two is very important, in practice. At work, congenital short esophagus and esophageal hiatus are difficult to identify, and sometimes must be identified by surgery.

Patients with esophageal hiatal hernia have seizure chest tightness, abdominal pain, swallowing discomfort after eating, chest radiograph showing posterior mediastinal mass, esophageal angiography showing normal esophageal length, esophageal movement through the esophageal hiatus to the underside of the diaphragm, then up Swirling into the left thoracic cavity; the position of the stomach is normal, only part of the stomach into the chest cavity, such as the stomach intrusion part is larger, the esophagus can still have distortion, and most esophageal hiatus hernia is reversible, the stomach The cardia can slide up and down as the position or intra-abdominal pressure changes.

If the esophageal hiatal hernia is fixed on the diaphragm by the secondary fibrotic changes, it is especially difficult to distinguish from the congenital short esophagus, allowing the patient to eat the expectorant under fluoroscopy and pay close attention to the lower end of the esophagus. Its relationship with the diaphragm helps the differential diagnosis of both.

2. Esophageal ampulla (ampulla of esophagus)

Due to the contraction of the diaphragm, a cystic dilatation may occur at the lower end of the esophagus, called the esophageal ampulla or ampulla of the ampulla. The esophageal ampulla is a temporary physiological dilatation, sometimes clinically similar to a partial thoracic stomach. Identification with congenital short esophagus.

In general, the esophageal ampulla can be affected by breathing or swallowing. When the gas is closed or the swallowing action is completely stopped, the esophageal ampulla disappears partially or completely. The esophageal ampulla of the esophagus is characterized by an expansion of the 4.5cm esophageal lumen into an ellipse. The shape and smooth edge can be contracted and reduced due to peristalsis of the esophagus. When the esophageal ampulla is reduced, the barium meal is characterized by slender, longitudinal, smooth mucosal folds, which are different from the reticulated folds at the bottom of the stomach.

3. Acquired short esophagus (acquired brachyesophagus)

Acquired short esophagus is a secondary shortening of the esophagus, allowing the upper or the whole body of the corpus to temporarily or permanently enter the thoracic cavity through the enlarged esophageal hiatus. This disease can be secondary to reflux esophagitis and esophageal ulcer. Such diseases, or due to surgery such as esophagectomy, or esophageal hiatectomy, etc. caused by esophageal fibrosis, in addition, adults can cause vagus nerve reflex when stimulated by abdominal organs, resulting in esophageal shortening, that is, functional esophageal shortness If the diaphragmatic hiatus is loose at this time, part of the corpus can be lifted up to the chest.

Acquired short-term esophageal clinical manifestations may have similar symptoms of ulcer disease and gastroesophageal reflux, shortness of breath, dysphagia or upper gastrointestinal bleeding, esophageal X-ray barium meal examination showed that the esophagogastric junction is located above the level of the diaphragmatic esophageal hiatus, esophagus The length is shortened and the diaphragm level is not reached down.

4. Congenital cardia dysfunction or cardia relaxation (congenital cardiochalasia)

Congenital cardia dysfunction refers to the infants and young children have a significant and large amount of reflux after eating, and only reflux, the position of the esophagus and stomach of this child is normal, only the stomach contents reflux, the cause is mainly Due to neuromuscular dysplasia in the gastroesophageal junction of infants and young children, causing dysfunction, defects in the development of local anatomical structures, such as loose structure around the esophageal hiatus, excessively large holes, etc. can aggravate the degree of dysfunction of the cardia.

The clinical manifestation of this disease is that the child is vomiting when feeding because of improper position, excessive food intake or excessive gas intrusion. If the child is lifted up for a while after feeding, the symptoms of vomiting can be alleviated or avoided, and the vomiting symptoms are light. It can be expressed as a small amount of natural spillage of milk into the milk, occasionally with bloodshot, and more neglected; in severe cases, it can be expressed as a spray once to spit out all the ingested milk, and each feeding can occur, and Progressive aggravation, vomit can also be mistakenly attracted to the lung infection, often vomiting can make children malnutrition and water and electrolyte balance disorders.

Most children with congenital cardia dysfunction can gradually improve. If the child grows longer and cannot heal, it may be secondary to reflux esophagitis or acquired short esophagus.

5. Posterior mediastinal tumor

The most common posterior mediastinal tumors in children are neurogenic tumors, such as schwannomas and ganglionomas. Malignant people have occupying signs, often accompanied by anemia, weight loss, fever, etc., and other sites of metastases or osteolysis occur soon. Sexual destruction and pathological fractures can be used to detect characteristic tumor cells, which can be distinguished from congenital short esophagus.

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