In the course of many years, there was no consensus in the literature on the innate short-term esophagus as an independent developmental anomaly. The greatest contribution to the etiology, pathogenesis, clinico-morphological data, diagnosis and treatment of congenital short esophagus was made by B. Barret (1959). This disease is described in the literature under different names: brachiozophagus, chest stomach, non-admission of stomach, partial chest stomach.
The appearance of aroused short esophagus is explained by the violation of the formation of the digestive tract in the early embryonic period, when the esophagus was lined with a cylindrical epithelium. The replacement of this epithelium, beginning with the middle third of the esophagus, is disturbed, as a result of which the lower third of it is lined with gastric epithelium. Thus there is a dystopia of the gastric mucosa in the lower part of the esophagus (with normal cardia location).
Confirmation of this assumption is the fact that the supra-diaphragmatic part of the so-called undefended stomach, with the exception of the mucous membrane, is formed as an esophagus and devoid of serous cover. In the same part, the transverse and longitudinal muscle layers, characteristic of the esophagus, are differentiated, and the blood supply is carried out, as usual, from the aorta.
Clinical symptoms
The congenital short esophagus is accompanied, as a rule, by gastroesophageal reflux, which is complicated by esophagitis and the onset of peptic esophageal stenosis.
The congenital short esophagus is revealed in children of the first three years of life, because they have a stable reflux syndrome. In the anamnesis, such patients often experience frequent vomiting soon after birth. Often there are indications of frequent respiratory diseases: bronchitis, pneumonia (in 30% of cases). In the future, clinical manifestations depend on the formation of secondary stenosis. If the stenosis is not formed, the main symptom is vomiting with gastric contents. Vomiting is provoked by crying, by the horizontal position of the body, and by a slight physical strain that causes an increase in intra-abdominal pressure. Blood vomit is found in the vomit; less frequent vomiting coffee. With secondary narrowing of the esophagus due to peptic ulcerative anopharyngitis, the symptoms of a violation of the oesophageal obstruction predominate: dysphagia, vomiting of the food.
Children older than eight years complain of pain behind the sternum, the emergence of which is due todisagia and ulcerative esophagitis. Often, children try to drink a pishchuk. Due to the constant bleeding from the dystopic mucous membrane, as evidenced by the admixture of blood in the vomit masses and a positive reaction to the latent blood in the feces, anemia develops. Anemic syndrome is observed in 1/3 of patients. Children with congenital short esophagus lag behind in physical development.
For diagnostics of a congenital short esophagus, an X-ray examination is used, with the help of which, first of all, gastric reflux is found. In Trendelenburg position, the patient of esophageal esophageal reflux is determined to throw the contrast substance of the ovary into the esophagus. The distal part of the esophagus, as a rule, is widened downwards in the form of a funnel or bell. X-ray images in the distal esophagus show longitudinal folding, characteristic of the mucous membrane of the stomach. The passage of the esophagus during examination of the patient in standing position is intact. With secondary stenosis, the patency of the contrast medium is hampered, and the constriction of the esophagus is determined above the constriction. A radiological picture with deformation in the region of constriction and excessive storage of the lining requires differentiation from the hernia of the esophageal opening of the diaphragm and the diverticula of the esophagus.
It is widely used for the diagnosis of esophagofibroscopy. The most important endoscopic sign is the upward shift of the mucosal lining of the esophagus in the gastric mucosa, i.е. above the projection of the diaphragmatic slit. In the absence of stenosis in the distal esophagus, catarrhal or ulcerative fibrinous esophagitis occurs. With the development of stenosis, as a rule, is located on the border of the mucous membrane of the stomach and esophagus.
In connection with the fact that in patients with congenital short esophagus always has a place of gastroesophageal reflux, a certain diagnostic value has an intraepithelial pH-metry.
Inhabitants and infants carry out differential diagnosis with pyloric stenosis, pilorospasm, herniation of the esophagus of the diaphragm, chalasia, congenital and acquired stenoses of the distal esophagus.
Treatment
Treatment is often surgical, aimed at eliminating gastroesophageal reflux. The most reliable antireflux surgery is the operation of NN Kanshin in the modification of YF Isakova et al., Based on the valve gastroplication. In cases where the congenital short esophagus is complicated by peptic stenosis , antireflux surgery is complemented by the imposition of gastrostomy. In the future, it is stalled by bougie.
From the point of view of clinicians considering the congenital short esophagus as a non-disruption of the stomach, it is necessary to conduct long-term conservative therapy in the calculation of the gradual lowering of the stomach (as the child develops).
Conservative treatment provides for a constant elevated position of the upper half of the trunk, frequent feeding of the child in small portions of food of thick consistency. Conduct also general restorative and symptomatic treatment.
Growth!