Malformations of the esophagus: causes, symptoms, diagnosis, treatment

The developmental defects of the esophagus include its dysgenesis, relating to its shape, size and topographical relationship to surrounding tissues. The frequency of these vices is on the average 1:10 000, the sex ratio is 1: 1. Anomalies of the esophagus can concern only one esophagus, but can also be combined with anomalies of the development of the trachea - a fact that can be explained if one considers that both the esophagus and the trachea develop from the same embryonic rudiments. Some anomalies of the esophagus are incompatible with life (the death of a newborn occurs several days after birth), others are compatible, but require certain interventions.

Congenital malformations of the esophagus include constrictions, complete obstruction, agenesis (absence of the esophagus), hypogenesis, affecting various parts of the esophagus, esophageal and tracheal fistula. According to the well-known researcher of developmental malformations of P.Ya.Kossovsky, and English authors led by the famous esophagologist R.Shimka, combinations of complete obstruction of the esophagus with esophageal-tracheal fistula are most often encountered. Less commonly observed combination of narrowing of the esophagus with esophageal-tracheal fistula or the same combination, but without narrowing of the esophagus.

In addition to the developmental defects of the esophagus itself, doomed to newborns or to rapid starvation, or traumatic surgical interventions, congenital dysphagia can be caused by malformations of adjacent organs with normal esophagus (abnormal departure of the right carotid and subclavian arteries from the arch of the aorta and from its descending part to the left, which predetermines the pressure of these abnormally located large vessels on the esophagus at the intersection site - dysphagia lussoria). ISKozlova et al. (1987) distinguish the following types of esophagus atresia:

1. atresia without an esophagus-tracheal fistula, in which the proximal and distal ends terminate blindly or the entire esophagus is replaced by a fibrous strain; this form is 7.7-9.3% of the number of all anomalies of the esophagus;
2. atresia with esophageal-tracheal fistula between the proximal segment of the esophagus and the trachea, of 0.5%;
3. atresia with esophageal-tracheal fistula between the distal segment of the esophagus and the trachea (85-95%);
4. atresia of the esophagus with an esophageal-tracheal fistula between both ends of the esophagus and the trachea (1%).

Often atresia of the esophagus is combined with other malformations, in particular with congenital heart defects, gastrointestinal tract, genitourinary system, skeleton, CNS, with facial crevices. In 5% of cases, abnormalities of the esophagus occur in chromosomal diseases, for example, in Edwards syndromes (characterized by congenital anomalies in children, paresis and paralysis in various peripheral nerves, deafness, multiple dysgenesis of internal organs, including the organs of the chest) and Down (characterized by congenital dementia and characteristic signs of physical deformities - low growth, epicanth, small short nose, enlarged pleated tongue, face of "clown" and many others, 1 case for 600-900 newborns), in 7% observation is a component of nonchromosomal etiology.

Atresia of the esophagus. With congenital obstruction of the esophagus, the upper (pharyngeal) end of it ends blindly at the level of the sternum or a little lower; the continuation of it is a muscle-fibrous cord of greater or lesser length, passing into the blind end of the lower (cardiac) segment of the esophagus. Communication with the trachea (esophageal-tracheal fistula) is often 1-2 cm higher than its bifurcation. Fistulous openings open either into the pharyngeal or into the cardiac blind section of the esophagus, and sometimes both. Congenital obstruction of the esophagus is detected from the first feeding of the newborn and is especially bright if it is combined with the esophageal-tracheal fistula. In this case, this developmental flaw is manifested not only by the obstruction of the esophagus, which is characterized by persistent salivation, regurgitation of all swallowed food and saliva, but also severe disorders caused by the ingress of fluid into the trachea and bronchi. These disorders are synchronized with each gulp and manifest from the first minutes of the child's life by coughing, choking, cyanosis; they occur with a fistula in the proximal part of the esophagus, when the fluid from the blind end enters the trachea. However, even with esophageal tracheal fistula in the region of the cardial segment of the esophagus, respiratory distress develops soon, because gastric juice enters the respiratory tract. In this case, persistent cyanosis occurs, and free hydrochloric acid is found in the sputum. In the presence of these abnormalities and without emergency surgery, children die early either from pneumonia or from exhaustion. To save a child is possible only by plastic surgery, gastrostomy can be used as a temporary measure.

The diagnosis of atresia of the esophagus is established on the basis of the above signs of aphagia, with the help of sounding and radiography of the esophagus with contrasting iodideol.

Stenosis of the esophagus with its partial patency is mostly related to stenoses, compatible with life. Most often, narrowing is localized in the lower third of the esophagus and is probably due to a violation of its embryonic development. Clinically, the stenosis of the esophagus is characterized by a violation of swallowing, which manifests itself immediately upon feeding on semi-liquid and especially dense food. With X-ray examination, the smoothly narrowing shadow of the contrasting substance with a spindle-like expansion over the stenosis is visualized. With fibrogastroscopy, stenosis of the esophagus with an ampoule expansion above it is determined. The mucosa of the esophagus is inflamed, in the stenosis region it is smooth, without cicatricial changes. Stenosis of the esophagus causes food blockages by dense food products.

Treatment of esophageal stenosis consists in the expansion of stenosis with the help of bougie. Food blockages are removed during esophagoscopy.

Congenital disorders of the size and position of the esophagus. These disorders include congenital shortening and expansion of the esophagus, its lateral displacements, as well as secondary diaphragmatic hernia, caused by the divergence of the diaphragm fibers in the cardiac part of the esophagus, with retraction into the chest cavity of the cardiac part of the stomach.

Congenital shortening of the esophagus is characterized by its underdevelopment in length, as a result of which the adjoining part of the stomach leaves through the esophageal opening of the diaphragm into the thoracic cavity. Symptoms of this anomaly consist of recurring nausea, vomiting, regurgitation of food with an admixture of blood, the appearance of blood in the feces. These phenomena quickly lead the newborn to mass loss and dehydration.

Diagnosis is established with the help of fibro-esophagoscopy and radiography. Differentiate this anomaly from the ulcer of the esophagus, especially in infants.

Congenital esophagus enlargement is an anomaly that occurs extremely rarely. Clinically, it manifests itself as stagnant phenomena and slow passage of food through the esophagus.

Treatment in either case - non-operative (appropriate diet, maintaining the vertical posture after feeding the baby). In rare cases, with pronounced functional impairment - plastic surgery.

Deviations of the esophagus occur with abnormalities of the development of the chest and the appearance of voluminous pathological formations in the mediastinum, which can move the esophagus relative to its normal position. Deviations of the esophagus are divided into congenital and acquired. Congenital diseases correspond to abnormalities of the development of the thoracic skeleton, acquired, which occurs much more often, are caused either by a traction mechanism arising from some scar process that seizes the esophagus wall or external pressure caused by diseases such as goitre, mediastinal and lung tumors, lymphogranulomatosis, aneurysm of the aorta, pressure of the spine, etc.

Deviations of the esophagus are divided into total, subtotal and partial. Total and subtotal deviations of the esophagus - a rare phenomenon that occurs with significant cicatricial changes in the mediastinum and, as a rule, they are accompanied by deviation of the heart. The diagnosis is established on the basis of an x-ray examination, in which the displacement of the heart is established.

Partial deviations are observed quite often and are accompanied by deviations of the trachea. Usually deviations occur in the transverse direction at the level of the clavicle. Radiologic examination reveals the approach of the esophagus to the sternoclavicular junction, the intersection of the esophagus with the trachea, the angular and arched distortions of the esophagus in this region, the combined displacements of the esophagus, heart and large vessels. Most often the deviation of the esophagus occurs to the right.

Clinically, these shifts themselves are not manifested in any way, at the same time, the clinical picture of the pathological process that causes deviation can have its own significant pathological effect on the general state of the organism, including the function of the esophagus.

[1], [2], [3], [4]