Congenital malformations of the esophagus. Pediatric surgery: lecture notes (M. V. Drozdova)

Anatomy and physiology of the esophagus.

The esophagus is a muscular tube about 25 cm long (from the pharynx to the cardia). cervical- 5 cm, thoracic region - 15 cm, cardiac region 3-4 cm.

Anatomy: the esophagus is a hollow cylindrical tube that connects the pharynx with the stomach and is located at the level of C6-Th11.

1.Cervical department.

In adults, it extends from the level of the cricoid cartilage (C6) to the jugular notch of the manubrium of the sternum (Th2). Length about 5-8 cm.

2. Thoracic

From the jugular notch of the manubrium of the sternum to the esophageal opening of the diaphragm (Th10). Its length is 15-18 cm. From a practical point of view, the following topography is appropriate in the thoracic esophagus:

The upper part is up to the aortic arch.

The middle part corresponding to the aortic arch and tracheal bifurcation;

The lower part is from the bifurcation of the trachea to the esophageal opening of the diaphragm.

3. Abdominal department.

Length 2.5 - 3 cm. The transition of the esophagus to the stomach, as a rule, corresponds to Th11.

Physiological narrowing of the esophagus:

1. Upper - at the point of transition of the lower part of the pharynx into the esophagus (C6-C7).

2. Average - at the intersection with the left bronchus (Th4-Th5).

3. Lower - at the passage of the esophagus through the opening of the diaphragm (Th10). This is where the lower esophageal sphincter is located, which prevents acidic gastric contents from being thrown into the esophagus.

Malformations of the esophagus.

Congenital atresia of the esophagus and esophagotracheal fistulas.

Occurrence: occurs 1 case per 7-8 thousand newborns. The most common is complete atresia of the esophagus in combination with a tracheobronchial fistula: the proximal end of the esophagus is atrezed, and the distal end is connected to the trachea. Less common is complete atresia of the esophagus without tracheobronchial fistula.

Clinic: the disease manifests itself immediately after birth. When a newborn swallows saliva, colostrum, liquid, respiratory failure, cyanosis immediately occurs. With complete atresia without an esophageal-tracheal fistula, belching and vomiting occur at the first feeding.

Diagnostics:

· Clinical manifestations;

Probing of the esophagus;

Contrast study of the esophagus with gastrografin;

Plain radiograph of the chest and abdominal cavity: signs of areas of atelectasis, signs of pneumonia (aspiration), lack of gas in the intestine. Gas in the intestines can be in the event that there is a connection of the lower segment of the esophagus with the trachea (fistula).

· If there are no signs of atelectasis, pneumonia - a one-stage operation of closing the esophagotracheal fistula and anastomosing the upper and lower segments of the esophagus.

If the disease is complicated by aspiration pneumonia, atelectasis in the lungs, then the following treatment is carried out: at the beginning, a gastrostomy is applied, intensive care until the condition improves and then close the fistula and make an anastomosis between the upper and lower segments of the esophagus.

In multiple malformations, in severely debilitated newborns, the proximal end of the esophagus is brought out to the neck to avoid accumulation of saliva in it, and a gastrostomy is placed for feeding. After a few months, an anastomosis is performed. If it is impossible to compare the upper and lower segments, plastic surgery of the esophagus is performed.

Congenital stenosis of the esophagus.

As a rule, the stenosis is located at the level of the aortic narrowing.

Clinic: hiatal hernia, esophagitis, achalasia. With a significant narrowing of the esophagus, a suprastenotic expansion of the esophagus occurs. Symptoms usually do not appear until the introduction into diet baby solid food.

Diagnostics:

· Clinical manifestations;

Fibroesophagogastroscopy;

Contrast study of the esophagus;

Treatment: in most cases, expansion of the esophagus by dilatation or bougienage is sufficient. Surgical treatment is carried out in case of unsuccessful conservative.

congenital membrane diaphragm esophagus.

The diaphragm is made up of connective tissue covered with keratinized epithelium. This diaphragm often has holes through which food can enter. It is localized almost always in the upper esophagus, much less often in the middle section.

Clinic: the main clinical manifestation is dysphagia, which occurs when solid food is introduced into the child's diet. With significant holes in the membrane, food can enter the stomach. Such patients usually chew everything thoroughly, which prevents food from getting stuck in the esophagus. The membrane often becomes inflamed under the influence of food debris

Diagnostics:

· Clinical manifestations

Contrast study of the esophagus

Treatment: gradual expansion of the esophagus with probes of various diameters. When the diaphragm completely covers the lumen, it must be removed under endoscopic control.

Congenital short esophagus.

It is believed that during intrauterine development, the development of the esophagus is slower, and part of the stomach, penetrating through the diaphragm, forms the lower esophagus. Congenital short esophagus occurs in Marfan's syndrome, there are family cases of the disease.

Clinic: clinical manifestations are similar to those of a sliding hiatal hernia - pain in the chest after eating, heartburn, may be vomiting.

Diagnostics:

· Clinical manifestations

It is often possible to differentiate a congenital short esophagus from a sliding hiatal hernia only during surgery

Fibroesophagogastroscopy

Treatment: with symptoms - surgical, as a rule, in the absence of adhesions of the esophagus and aorta, it is possible to restore the normal position of the esophagus and stomach by stretching it.

Congenital esophageal cysts.

Cysts are located intramurally, paraesophageally. Such cysts are lined with bronchial, esophageal epithelium.

Clinic: in children, cysts can cause dysphagia, cough, respiratory failure, cyanosis. In adults, cysts are usually less than 4 cm, if more than 4 cm then clinical symptoms the same as in leiomyomas. Cysts can be complicated by mediastinitis when infected, bleeding and malignancy.

Treatment: removal of the cyst during fibrogastroscopy.

Vascular anomalies.

Congenital anomalies of the aorta and large vessels can compress the esophagus and cause dysphagia. For example, an abnormal right subclavian artery. As a rule, dysphagia manifests itself in the first 5 years of life. Sometimes there is a double arch of the aorta that surrounds the trachea and esophagus and when eating, cyanosis and cough occur, and later dysphagia joins

Abnormalities in the development of the esophagus are numerous and varied. Here are just some of the most common or clinically important malformations.

1. Esophageal agenesis - complete absence of the esophagus, is extremely rare and is combined with other severe developmental disorders.

2. Esophageal atresia – characteristic feature is the formation of congenital anastomoses (fistulas) between the esophagus and the respiratory tract. The development of atresias and tracheoesophageal fistulas is based on a violation of the formation of the laryngotracheal septum in the process of dividing the foregut into the esophagus and trachea. Often, esophageal atresia is combined with other malformations, in particular with congenital heart defects, gastrointestinal tract, urogenital apparatus, skeleton, CNS, with facial clefts. The population frequency is 0.3: 1000. Depending on the presence or absence of tracheoesophageal fistulas and their localization, several forms are distinguished:

A) Atresia of the esophagus without tracheoesophageal fistulas - the proximal and distal ends end blindly or the entire esophagus is replaced by a cord devoid of lumen (7-9%).

B) Esophageal atresia with tracheoesophageal fistula between the proximal esophagus and trachea (0.5%).

C) Esophageal atresia with tracheoesophageal fistula between the distal segment of the esophagus and the trachea (85-95%).

D) Atresia of the esophagus with tracheoesophageal fistulas between both ends of the esophagus and the trachea (1%).

3. Hypoplasia of the esophagus (syn.: microesophagus) - manifested by shortening of the esophagus. Can lead to hernial protrusion of the stomach into the chest cavity.

4. Macroesophagus (syn.: megaesophagus) - an increase in the length and diameter of the esophagus due to its hypertrophy.

5. Doubling the esophagus(syn.: diaesophagia) - tubular forms are extremely rare, diverticula and cysts are found somewhat more often. The latter are usually located in posterior mediastinum, often at the level of the upper third of the esophagus.

STOMACH

The stomach is the most expanded and the most complex in structure. digestive tract. At the time of birth, the stomach has the shape of a bag. Then the walls of the stomach collapse, and it becomes cylindrical. IN infancy the entrance to the stomach is wide, so young children often spit up. The fundus of the stomach is not expressed, and its pyloric part has a relatively great length than an adult.

Physiological capacity the stomach of a newborn does not exceed 7 ml, during the first day it doubles, and by the end of the 1st month it is 80 ml. The physiological capacity of the stomach of an adult is 1000-2000 ml. Average length adult stomach 25-30 cm, its diameter is about 12-14 cm.

mucous membrane forms numerous folds. The surface of the mucous membrane in a newborn is only 40-50 cm 2, in postnatal life it increases to 750 cm 2. The mucous membrane is covered with elevations with a diameter of 1 to 6 mm, called gastric fields. They have numerous dimples 0.2 mm in diameter, into which the gastric glands open. The number of gastric pits is up to 5 million. The number of glands in an adult reaches 35-40 million. They have a length of 0.3-1.5 mm, a diameter of 30-50 microns, there are about 100 of them per 1 mm 2 of the surface of the mucous membrane. These glands secrete up to 1.5 liters per day gastric juice containing 0.5% hydrochloric acid. However, up to 2.5 years, the glands do not produce hydrochloric acid.

There are three types of stomach glands: own glands of the stomach (fundic), cardiac and pyloric.

Own glands of the stomach the most numerous, their secretory surface reaches 4 m 2 . They include five types of cells: chief (secrete pepsinogen), parietal or parietal (produce hydrochloric acid), mucous and cervical (secrete mucus), endocrine (produce biologically active substances- gastrin, serotonin, histamine, somatostatin, etc., these substances are tissue hormones that affect local and general processes of regulation of functions in the body).

cardiac glands(glands of the body of the stomach) mainly consist of mucous and chief cells.

Pyloric glands contain predominantly mucous cells that produce mucus. It should be noted that mucus provides not only mechanical protection of the mucous membrane, but also contains antipepsin, which protects the stomach wall from self-digestion.

Muscular layer of the stomach formed by circular and longitudinal fibers. The pyloric sphincter is well expressed. The development of muscles continues up to 15-20 years. Longitudinal muscles are formed mainly along the curvatures of the stomach, they regulate the length of the organ. The tone of the muscles of the stomach depends on the intake of food. When the organ is filled, peristalsis waves begin in the middle of its body and after 20 seconds. reach the gatekeeper.

Shape, size and position of the stomach healthy person exceptionally varied. They are determined by its filling, the degree of muscle contraction, depend on respiratory movements, body position, condition of the abdominal wall, intestinal filling. In a living person, 3 forms of the stomach are radiologically distinguished: in the form of a hook, a bull's horn, and an elongated shape. There is a connection between the forms of the stomach, age, gender and body type. IN childhood often found stomach in the form of a bull's horn. In dolichomorphic people, especially women, the stomach is usually elongated, with a brachymorphic type, a stomach in the form of a bull's horn is observed. Bottom line stomach when it is filled is at the level of III - IV lumbar vertebrae. With the prolapse of the stomach, gastroptosis, it can reach the entrance to the small pelvis. In old age, there is a decrease in the tone of the longitudinal muscles, as a result of which the stomach is stretched.

Abnormalities in the development of the stomach

Abnormalities in the development of the stomach are numerous and varied. Here are just some of the most common or clinically important malformations.

1. Agenesia of the stomach - the absence of a stomach, an extremely rare defect, combined with severe anomalies in the development of other organs.

2. Atresia of the stomach - usually localized in the pyloric region. In most cases, with atresia, the exit from the stomach is closed by a diaphragm localized in the antrum or pylorus. Most of the membranes are perforated and represent a fold of the mucous membrane without involvement of the muscular one.

3. Hypoplasia of the stomach (syn.: congenital microgastria) - the small size of the stomach. Macroscopically, the stomach has a tubular shape, its segments are not differentiated.

4. Pyloric stenosis congenital hypertrophic stomach (syn.: hypertrophic pyloric stenosis) - narrowing of the lumen of the pyloric canal due to an anomaly in the development of the stomach in the form of hypertrophy, hyperplasia and impaired innervation of the pyloric muscles, manifested by a violation of the patency of its opening in the first 12-14 days of a child's life. The population frequency is from 0.5:1000 to 3:1000.

5. doubling of the stomach (syn.: double stomach) - the presence of an isolated or communicating with the stomach or duodenum hollow formation, more often located on the greater curvature or on the posterior surface of the stomach. It accounts for about 3% of all cases of duplications of the gastrointestinal tract. The presence of an additional organ located parallel to the main one is casuistry. A case of "mirror" doubling of the stomach is described, the accessory stomach was located along the lesser curvature, having a common muscular wall with the main stomach, the lesser omentum was absent.

SMALL INTESTINE

This is the longest part of the digestive tract, divided into the duodenum, jejunum and ileum. The last two are characterized by the presence of a mesentery in them and, therefore, the sign is allocated to the mesenteric part of the small intestine, located intraperitoneally. The duodenum is devoid of a mesentery and, with the exception of the initial section, lies extraperitoneally. The structure of the small intestine to the greatest extent corresponds to the general plan for the design of hollow organs.

Duodenum

It has a length of 17-21 cm in a living person. Its initial and final parts lie at level I lumbar vertebra. The shape of the intestine is most often annular, the bends are weakly expressed and form after 6 months. The position of the intestine depends on the filling of the stomach. With an empty stomach, it is located transversely, with a full stomach, it turns, approaching the sagittal plane. There are U-shaped (15% of cases), V-shaped, horseshoe-shaped (60% of cases), folded, ring-shaped (25% of cases) forms.

LEAN AND ILE INTESTINAL

They make up about 4/5 of the entire length of the digestive tract. There is no clear anatomical boundary between them. In newborns and children, the relative length of the small intestine is greater than in adults. The length of the small intestine in a newborn is about 3 m and is related to body length as 5.4:1. In the first year of life, rapid growth of the small intestine continues, and its ratio to body length reaches 5.7:1. Intensive development of the small intestine lasts up to 3 years, after which its growth slows down. A new growth-organ acceleration occurs between 10 and 15 years of age.

In adults, the length of the small intestine varies from 3 to 11 m, according to different authors. The values ​​obtained from measurements on cadavers are very different from in vivo observations. It has been shown that the length of the small intestine of a living person is only 261 cm. Individual differences in the length of the small intestine depend on many factors. It is believed that the length of the intestine is determined by food regimen. People who eat a predominantly plant-based diet have longer intestines than people whose diet is dominated by animal products. Most researchers note that in men the length of the small intestine is greater than in women. Sex differences are absent in newborns and appear in childhood. The diameter of the mesenteric part of the small intestine in the initial section is approximately 45 mm and gradually decreases to 30 mm.

Individual differences in bowel arrangement also appear early. In newborns, the small intestine occupies a more limited space, since the upper half of the abdominal cavity is occupied by the liver, and protruding into its lower section pelvic organs. Mesentery of the small intestine in newborns and children early age short, and loops of intestines are located relatively high. With the lengthening of the mesentery and the lowering of the pelvic viscera, the small intestine moves into the hypogastrium.

The intestinal mucosa has adaptations that increase its absorption surface. These include circular folds, villi, and microvilli. The number of circular folds in the entire intestine is 500-1200. They have different height- up to 8 mm. Although they are called circular, they do not form closed rings, but extend to 2/3 or more of the intestinal circumference. The longest folds reach a length of 5 cm. In the duodenum and upper jejunum they are higher, and in the ileum they are lower and shorter. Total number villi are estimated at 4 million. There are 22-40 villi per 1 mm 2 of the jejunum, and 18-31 in the ileum. The surface area of ​​the small intestine in adults, taking into account the mucosal folds, is 10,000 cm 2, taking into account the villi - 100,000 cm 2, taking into account the microvilli - 2,000,000 cm 2, or 200 m 2. Such a large suction surface ensures a high degree of utilization of nutrients.

The muscular coat is characterized by a more powerful development of the circular layer compared to the longitudinal one. Strictly speaking, neither layer is a regular circular or longitudinal layer. In both, the muscle bundles have a spiral direction, but in the circular direction they form a very steep spiral (the length of one stroke is about 1 cm), and in the outer one it is very gentle (the stroke length is from 20 to 50 cm). Due to the spiral course of the muscle bundles, continuity of the muscles is created throughout the small intestine. The motor function of the small intestine consists of several types of movements. Peristaltic movements help to move the contents. Waves of peristalsis can be traced for 12 cm, after which they fade. Mixing of the contents and its contact with the mucosa is provided by rhythmic segmentation and pendulum movements. The latter are expressed in periodic shortening and lengthening of the segment of the intestine from 15-20 to several tens of centimeters. During segmentation, the intestine is laced into small sections, while the folds play the role of filtering and delaying devices. Such movements are repeated 20-30 times per minute.

The most common malformations of the esophagus: esophageal atresia, esophageal-tracheal fistula, congenital stenosis of the esophagus, congenital short esophagus, chalazia cardia.

Esophageal atresia occurs with a frequency of 1 in 3000-4000 newborns. The defect is formed in the period from the 4th to the 12th week of intrauterine development of the fetus, when the esophagus and trachea are separated from a common rudiment - the cranial foregut. There are 5 main types of esophageal atresia (Fig. 6). The most common (85-90% of cases) is type III atresia, in which upper end the esophagus ends blindly, and the lower one is connected to the trachea by a fistulous tract.

Rice. 6. Types of congenital atresia of the esophagus.

The diagnosis of atresia is clarified with the help of a fairly simple examination: a thin urethral catheter with a rounded end is inserted into the esophagus through the mouth. Normally, the catheter passes freely into the stomach. With atresia, the catheter usually stops at a distance of 10-12 cm from the edge of the gums, which corresponds to the II-III thoracic vertebra. The mucus is sucked off with a syringe, and then 10 cm3 of air is injected through the catheter. With atresia, it noisily exits back through the mouth and nose (Elephant's test). The effectiveness of this simple technique has led many pediatric surgeons to strongly recommend esophageal catheterization to all newborns without exception immediately after delivery.

Having established the diagnosis of esophageal atresia, feeding of the child is excluded, a thin catheter is inserted through the nose into the upper blind horse of the esophagus for periodic suction of mucus, and the child is transported to the surgical department in a half-sitting position.

Further, to clarify the diagnosis and identify pneumonia, x-ray examination. A radiopaque catheter is inserted into the proximal esophagus; in case of atresia, it coils in a blind sac. The introduction of water-soluble contrast agents through the catheter to detect esophageal-respiratory fistula is not currently used because of the risk of developing severe aspiration pneumonia. To localize the fistula and determine its size, fibrotracheobronchoscopy is performed under anesthesia.

When examining newborns with esophageal atresia, it should be taken into account that other malformations occur in 20-50% of such patients. This significantly worsens the prognosis.

The diagnosis of esophageal atresia is an indication for urgent operation. Children admitted to the surgical clinic in the first 10-12 hours after birth, as a rule, do not require long-term preoperative preparation. At a later admission, intensive preoperative preparation is carried out, including the treatment of pneumonia, elimination respiratory failure, correction of violations of water-electrolyte and protein metabolism, acid-base state, parenteral nutrition. Sometimes for good nutrition pre-impose gastrostomy.

With a more or less pronounced improvement in the condition, an operation is started, which consists in right-sided thoracotomy, mobilization of the ends of the esophagus, closure (ligation and intersection) of the esophageal-tracheal fistula and the imposition of esophagoesophagoanastomosis according to one of the proposed methods, which is most often formed using precision single-row atraumatic sutures, using synthetic absorbable suture material.

It is not always possible to form the esophageal anastomosis at once. With a large diastasis of the ends of the esophagus (more than 1.5 cm), the operation is divided into two stages: the first stage is thoracotomy, closure of the esophageal-tracheal fistula, esophagostomy on the neck with the removal of the entire blind sac of the esophagus, gastrostomy; the second stage (usually at the age of over a year) is plastic surgery of the esophagus, most often with a segment of the large intestine.

In the second type of atresia (large diastasis of the ends of the esophagus), which is very rare (1 - 1.5% of cases), sometimes the method of gradual lengthening of the blind ends of the esophagus with the help of spherical magnets inserted there is also sometimes used (this, of course, requires the imposition of a gastrostomy). However, in most cases, this technique cannot be applied due to the large diastasis of the ends of the esophagus (usually 6-7 cm).

Esophageal-tracheal fistula. This defect is rare, forming at the same stage of embryogenesis as esophageal atresia, when the esophagus and trachea are still connected to each other. The fistula is located, as a rule, in the upper thoracic esophagus.

The clinical picture depends on the width of the fistula. Characterized by coughing fits and cyanosis during feeding, especially when lying on the left side. In another variant of the course of the disease, recurrent pneumonia predominates.

Diagnosis is established by radiopaque examination: horizontal position injected into the esophagus through a catheter contrast agent. The fistulous course can be poorly seen with a simple X-ray examination, in such cases X-ray cinematography (video X-ray) is used. The diagnosis is clarified by tracheobronchoscopy with stained fistula contrasting methylene blue saline injected through a catheter into the esophagus.

Treatment of the defect is only operative - closure of the fistula through a right-sided thoracotomy access.

Stenosis. Congenital stenosis of the esophagus is also a rare malformation. There are several variants of congenital stenosis. The most common form is a circular narrowing of the lumen, usually at the border of the middle and lower thirds of the esophagus, while the degree of narrowing can be different.

Very rare is the membranous form of stenosis, formed by a circular or eccentrically located fold of the normal mucosa. Also rarely observed is stenosis caused by the growth in the esophagus of the ectopic gastric mucosa (Barrett's esophagus).

The clinical picture is variable and depends primarily on the degree of stenosis. The first signs of dysphagia may appear during infancy or later as the child gets older. Most often, dysphagia and regurgitation first appear with the introduction of complementary foods. Symptoms are aggravated by the ingestion of thick and solid foods, while the fluid passes freely, but episodes of complete dysphagia may also occur.
Diagnosis is established by radiopaque examination of the esophagus and fibroesophagoscopy.

Treatment depends on the form of stenosis. For short strictures, bougienage may help. In membranous stenosis, the method of choice is excision or cruciform incision of the membrane through the endoscope. In other cases, resort to surgical treatment— segmental resection of the area of ​​stenosis with restoration of the patency of the esophagus by end-to-end anastomosis. A variant of the operation is a longitudinal dissection of the esophageal wall through the stricture area with wound suturing in the transverse direction.

Short esophagus. The origin of the congenital short esophagus (brachiesophagus, or chest stomach) is associated with a delay in intrauterine lowering of the stomach from the chest cavity into the abdominal cavity, which occurs in the period from the 8th to the 16th week of intrauterine development of the fetus. Anatomically, the congenital short esophagus differs from the acquired one by the absence of a hernial sac. At the same time, in an externally correctly formed esophagus, a part of the mucous membrane (usually in the lower third) can be represented by a cylindrical epithelium, i.e. epithelium of the gastric mucosa.

When the stomach is not omitted, the His angle, as a rule, exceeds 90 °, the obturator function of the lower esophageal sphincter is disturbed, which creates conditions for the free flow of aggressive gastric contents into the esophagus. As a result, severe reflux esophagitis and peptic stricture of the esophagus develop.

The clinical picture depends on the stage of the disease. For early period characteristic frequent vomiting gastric contents, often mixed with blood (with the development of erosive and ulcerative esophagitis). Over time, as peptic stricture develops, dysphagia and regurgitation begin to predominate. The diagnosis is established by means of radiopaque research, at the same time the data received at research in horizontal position is most indicative.

TO indirect signs shortening of the esophagus include the absence or reduction in the size of the gas bubble of the stomach, its displacement up and to the right, the wedge-shaped deformation of the upper medial section of the gas bubble, the high location of the epiphrenic ampulla. The diagnosis is confirmed by fibroesophagogastroscopy.

Quite often recommended conservative treatment brachiesophagus for several months in the hope of spontaneous prolapse of the stomach (elevated position, especially during feeding, fractional feeding of thick food, restorative therapy). However, the development of such severe complications, as ulcerative esophagitis and peptic stricture, dictates the need for surgical treatment. The method of choice is antireflux surgery (fundoplication), in some cases, resection of the esophagus may be necessary.

Chalazia (insufficiency, gaping of the cardia). The essence of the defect lies in the violation of the development nerve elements the walls of the esophagus, namely the underdevelopment of the sympathetic germ. As a result, the lower esophageal sphincter loses its normal tone. Although at the same time the cardia is in its usual place (under the diaphragm), but the reflex of closing it after the end of the act of swallowing is disturbed. This also creates conditions for the free reflux of aggressive gastric contents into the esophagus with all the ensuing consequences (severe reflux esophagitis, peptic stricture of the esophagus).

Clinically, the disease is characterized by persistent vomiting that occurs shortly after feeding the child, more often in the supine position, as well as when the child screams and cries. If in children of the first days of life, slight regurgitation is physiological, then in the future this symptom requires more attention.

Diagnosis is established by radiopaque examination of the esophagus and stomach in a horizontal and Trendelenburg position. At the same time, free flow of the contrast agent from the stomach into the esophagus is noted, and the absence of distal narrowing of the esophagus is also characteristic. The diagnosis is confirmed by fibroesophagogastroscopy.

Treatment begins with conservative measures - feeding in an upright position (preferably with thicker food), giving an elevated position after feeding, during sleep. Often, all symptoms disappear within a few months as the nerve elements of the esophagus mature and the tone of the cardia is restored. In cases where there is no improvement in the second half of life, it is necessary to tend to surgical treatment. The method of choice is an organ-preserving antireflux operation such as fundoplication.

A.F. Chernousov, P.M. Bogopolsky, F.S. Kurbanov

Embryology. The esophagus and trachea develop from the primary gut of the embryo. Their separation begins in the area of ​​the carina and continues in the cranial direction. With violations of this complex process, defects such as esophageal atresia and tracheal atresia can occur. Since the trachea normally has cartilage, then with anomalies in the development of the esophagus, as a rule, it also has cartilage remnants in its wall.

The muscular wall of the esophagus consists of internal circular and external longitudinal muscles. The esophagus does not have a serous membrane. Its upper third, represented by striated muscles, is under voluntary control, while the two distal thirds, consisting of smooth muscles, are controlled autonomously. The mucous membrane of the esophagus is supplied with its own glands, which, in the presence of acute obstruction, produce a large number of mucus.

From a surgical point of view, the cervical, thoracic and abdominal parts can be distinguished in the esophagus. The cervical region is intimately connected with the larynx. The thinnest wall of the esophagus is immediately below and posterior to the cricopharyngeal muscles. This part of the esophagus, where the risk of perforation is especially high, and which receives blood supply from the thyroid-cervical vessels.

The thoracic part of the esophagus curves around the lateral side of the aortic arch and, with an anomaly called the vascular ring, is significantly compressed. In this section, the esophagus is intimately in contact with the aorta and pericardium, which, respectively, can also be affected by foreign bodies or chemical burns esophagus.

The abdominal part of the esophagus provides the mechanism of action of the lower esophageal sphincter and is supplied with blood from the diaphragmatic branches and gastric vessels. Rupture of the mucous membrane of this department as a result violent vomiting and the development of varicose veins in portal hypertension are potential sources of profuse blood loss from the lower third of the esophagus.

4.7.1 Esophageal atresia(AP) – a malformation in which its upper segment ends blindly, and the lower one in most cases flows into the trachea, forming an anastomosis. Other forms of atresia are also known.

Pathogenesis. On the 19th day of gestation, the foregut of the human embryo is represented by a single-layer tube located between the pharynx and stomach. After a few days, the ventral part of this intestine begins to thicken and forms a gutter lined with stratified ciliated columnar epithelium, which subsequently becomes a mucous membrane. respiratory tract. The separation of the dorsal part of the foregut (esophagus) from the ventral (trachea) occurs first in the area of ​​the carina and then spreads in the head direction. On the 26th day of gestation, these two structures become completely separated to the level of the larynx.

Interruption for one reason or another of the process of separation of the esophagus and trachea leads to the formation of tracheoesophageal fistula (TPS). Grunewald and some other authors believe that the cause of AP is the initially (before AP) formed TPS. According to these researchers, the trachea grows so rapidly in the caudal direction that if the esophagus is fixed with a fistula to the trachea, then its dorsal wall is pulled forward and down to “keep up” with the trachea and remain connected to it, which leads to the formation of AP.

The etiology of AP is not fully understood. And although there is no obvious evidence of a hereditary nature, however, there are a number of reports of familial cases of AP. For example, several cases of AP have been described in identical twins, as well as in mother and child, and in father and child. Moreover, a case is described when in one family there were 5 male members with this pathology. From the anamnesis of pregnancy, polyhydramnios and the threat of miscarriage in the first trimester are typical.

Often AP is combined with other malformations - congenital malformations of the heart, gastrointestinal tract, genitourinary system, etc. The population frequency is 0.3:1000. The combination of male and female 1:1.

Anatomy and classification. AP and TPS can occur as independent isolated defects, but their combination is most often noted. There are numerous, constantly evolving and expanding classifications of these defects.

Ø AP with distal TJ (88%)- the most common form of vice. The upper segment of the esophagus in this variant ends blindly, usually at the level of Th 3, although it can be much higher (C 7), or vice versa, low (Th 5). Its muscular wall is thickened, and the diameter is much larger than the diameter of the lower segment, which starts from the trachea, usually from its membranous part. lower section. Sometimes the fistula departs from the bifurcation of the trachea, in this case the trachea has, as it were, a “trifurcation”. The connection of the fistula with the bronchus is rare. There is usually diastasis between the segments of the esophagus, the magnitude of which determines the choice of treatment method.

Ø Isolated AP (without RTP - 5%) almost always accompanied by a large diastasis between the segments. In the absence of a lower TJ, the distal segment is very short, sometimes protruding only 1–2 cm above the diaphragm.

Ø Isolated TPS (without AP - 3%), the diameter of which, as a rule, is 2-4 mm, usually has an oblique direction from the anterior wall of the esophagus upward diagonally to the membranous part of the trachea. TPS can be at any level, but more often departs from the lower cervical or upper thoracic part of the trachea.

Ø In AP with proximal TPS (1%) the fistula usually begins 1-4 cm above the bottom of the upper segment and goes diagonally upwards, flows into the membranous part of the trachea. As a rule, there is no distal TJ in this variant of the defect; therefore, the lower segment is short, and the diastasis is correspondingly large.

Ø In AP with proximal and distal (two) TJ (3%) the upper segment with a fistula, as well as the lower one, do not differ anatomically from each of these forms described above when they occur separately. Usually diastasis with this variant of the defect is small.

Clinic. Diagnostics.“It seems that the child is very hungry and greedily grabs with his mouth everything that is given to him from a spoon, but when he is about to swallow, the food seems to encounter an obstacle and does not go inside, but returns through the mouth and nose, while the child falls into similar condition seizure". This first description by Gibson (1696) of the behavior of a child with esophageal atresia is of value to this day.

The first symptom of AP is copious excretion saliva that the child cannot swallow. If the newborn begins to feed, then he chokes, coughs, often with cyanosis and regurgitation. After aspiration of the contents of the nasopharynx, it soon reappears. Very quickly, wheezing begins to be heard in the lungs, shortness of breath increases. Diagnosis is confirmed by esophageal catheterization thin urethral catheter with a rounded end. The catheter is inserted through the nose; having passed to a depth of 6-8 cm, the catheter rests against the blind end of the esophagus or, wrapping itself, exits through the child's nose. Produce suction of mucus. Air introduced into the blind end of the esophagus is emitted with noise from the nasopharynx (positive symptom of Elefant). Considering the great importance early diagnosis esophageal atresia - before the onset of aspiration pneumonia, esophageal probing is advisable in all infants with the syndrome respiratory disorders immediately after birth. The clinical picture may have some features depending on the form of atresia. In the most common form of atresia with a distal tracheoesophageal fistula, abdominal distension is detected, especially in epigastric region. The severity of the aspiration syndrome depends on the diameter of the tracheoesophageal anastomosis.

The final diagnosis is made after x-ray study. The catheter is inserted into the esophagus until it stops, after which a plain radiograph of the chest and abdominal organs is performed. With atresia, a radiopaque catheter is clearly visible in the blind segment of the esophagus. Detection of air in the stomach and intestines indicates the presence of a fistula between the trachea and the abdominal segment of the esophagus.

In children with fistulous forms of atresia, the length of the diastasis between the ends of the esophagus can be judged to some extent by the lateral radiograph. The use of radiopaque solutions for diagnosis, especially barium suspension, is highly undesirable because of the risk of aspiration pneumonia. An abnormal variant of the defect can be specified bronchoscopically directly on the operating table before the operation. Some surgeons believe that bronchoscopy is indicated for all newborns with AP to detect proximal TPS and tracheomalacia. Sagittal computed tomography (CT) can help determine diastasis between segments.

AP can be diagnosed antenatal if polyhydramnios is detected on ultrasound and the fetal stomach is not visible. Sometimes an enlarged proximal esophagus is determined, which all the more confirms AP.

Associated anomalies. Approximately half of children with AP and TPS have concomitant anomalies, often severe and adversely affecting the results of treatment. Combined pathology is much more likely than AP itself to be the cause of death. Associated anomalies are more common in premature infants with AP. In 1973, the association VATER was described (see Chapter 1), which includes AP. If the combination of defects is denoted by the abbreviation VACTER, then the VATER-combination is also supplemented with cardiac (cardiac) defects, which are most often combined with AP and determine, in most cases, an unfavorable outcome. Also often in AP there are anomalies of the limbs (limb) and then the term acquires the most modern sound - VACTERL. In children with VACTERL association, as a rule, there is a high proximal segment of the esophagus, complicated course, higher mortality than in newborns with AP without this combination. If a child has one of the anomalies included in this association, one should suspect and look for others.

Treatment. Only early surgical intervention can save the life of a child with esophageal atresia. Already in maternity hospital preoperative preparation should be started, including aspiration of the contents of the oropharynx and nasopharynx every 15-20 minutes, the complete exclusion of oral feeding. Transportation must be carried out by a specialized team in the maximum short term. The total duration of preoperative preparation is determined by the severity of homeostasis and hemodynamic disturbances, respiratory failure, and the degree of dehydration. At obvious signs aspiration, respiratory failure, and even more so with pneumonia or atelectasis, it is necessary to resort to direct laryngoscopy with tracheal catheterization and aspiration as soon as possible. If the latter is ineffective under anesthesia, bronchoscopy or tracheal intubation is performed with careful aspiration of the contents. The patient is placed in an incubator, providing a continuous supply of oxygen, aspiration of the contents of the oropharynx, and warming. Prescribe infusion, antibacterial, symptomatic therapy. The choice of the method of surgical intervention is determined by the form of atresia and the patient's condition. In the most common form of atresia with a distal tracheoesophageal fistula in patients with low operational risk (term, without concomitant malformations of vital important organs and symptoms of intracranial birth injury) it is advisable to start with a thoracotomy, division of the tracheoesophageal fistula. If the diastasis between the ends of the esophagus does not exceed 1.5-2 cm, a direct anastomosis is applied. With a large diastasis of the segments of the esophagus, a cervical esophagostomy and a gastrostomy are applied according to Kader. In non-fistulous forms, due to significant diastasis, gastrostomy and esophagostomy are performed. In patients with a high operational risk, surgery often begins with the imposition of a double gastrostomy (the first for feeding through a tube inserted into the duodenum, the second for gastric decompression and aspiration reduction). The second stage of the operation is performed after the improvement of the condition after 2-4 days. In the postoperative period, the started intensive therapy is continued. The child is fed through a probe inserted intraoperatively through the anastomosis or into the gastrostomy after the passage through the intestine is restored. On the 6-7th day, the consistency of the anastomosis is examined. In the absence of complications, the child begins to feed through the mouth. 2-3 weeks after the operation, a control fibroesophagogastroscopy is performed with an assessment of the degree of patency of the anastomosis zone, the state of the cardia, and symptoms of esophagitis. The narrowing of the anastomosis, which occurs in 30-40% of cases, requires bougienage (bougie No. 22-24). The duration of bougienage is controlled by esophagoscopy. In the postoperative period during the first year of life, constant dispensary observation. Possible phenomena of dysphagia, complicated by obstruction in the area of ​​the anastomosis, which requires urgent esophagoscopy. In this regard, children in the first year of life are recommended to be given a homogenized food mass. Insufficiency of the cardia and gastroesophageal reflux, often complicating postoperative period, are clinically manifested by nocturnal regurgitation, repeated pneumonia, regurgitation and require timely diagnosis. In connection with operating injury recurrent nerve in children in the next 6-12 months, hoarseness is possible. In children with esophago- and gastrostomy aged 2-3 months to 3 years, the second stage of the operation is performed - esophageal plasty with a colonic graft.

4.7.2 Isolated congenital tracheoesophageal fistula (TPE) refers to severe malformations: its frequency is 3% among all anomalies of the esophagus. The first observation was described by Lamb in 1873. The occurrence of a malformation is associated with a violation of the separation of the esophagus and trachea in the early stages of embryonic development (4-5 weeks).

There are three types of tracheo-esophageal fistulas depending on the diameter of the anastomosis (G. A. Bairov, Z. A. Trofimova, 1986):

with a narrow and long fistulous passage,

with a short and wide fistula (most common),

· with the lack of separation between the esophagus and trachea for a long time.

In the literature there are descriptions of cases when the patient had two or even three fistulas at the same time. In some classifications TPS without AP is usually referred to as N-type TPS, and esophageal patency is not impaired.

Clinic The severity of symptoms depends on the diameter of the fistula and the angle of entry into the trachea. A wide fistula is characterized by a triad of symptoms:

1) coughing fits when taking liquids and food;

2) expansion of the stomach after coughing;

3) damage to the lungs (bronchitis, bronchiectasis, etc.).

With narrow fistulous passages, the disease proceeds either asymptomatically or under the mask chronic pneumonia and respiratory diseases (V. I. Geraskin, 1971; Schlosser et al., 1969).

Diagnostics tracheoesophageal fistula is difficult, especially with narrow fistulas. The complex of studies includes radiological and instrumental methods. radiography perform in a horizontal position of the child. A water-soluble contrast agent is injected through a probe inserted into the initial section of the esophagus. Its flow into the trachea indicates the presence of a fistula. The information content of this method is low. More diagnostic value has tracheoscopy. The trachea is examined all the way from the glottis to the bifurcation. A direct sign of a fistula is the appearance of air bubbles with mucus.

Treatment only operational. Preoperative preparation consists in sanitation of the tracheobronchial tree, treatment of aspiration pneumonia. For this purpose, sanitation bronchoscopy is performed, UHF, antibacterial, infusion therapy is prescribed. Completely exclude feeding through the mouth. The operation of mobilization, ligation and intersection of the anastomosis is performed using a right-sided cervical access, less often a posterolateral thoracotomy is performed. At timely diagnosis the prognosis is favorable.

Esophageal atresia, the most common congenital malformation digestive system. The clinic appears immediately after the birth of the baby. Foamy, abundant mucus is secreted from the nose and mouth, after suction it appears again, as a result, aspiration pneumonia inevitably develops. If the diagnosis is not made on time and the child is fed, the general condition of the child deteriorates sharply, signs of pneumonia and respiratory failure increase. Children cough, restless. Episodes acute violation the general condition of the child coincide with feeding. Breast milk flows out through the mouth and nose. The condition of children is even more severe if this defect is combined with a lower tracheoesophageal fistula.

Tracheoesophageal fistula, isolated, without atresia of the esophagus is extremely rare, even more rarely it is diagnosed in a timely manner, immediately after birth. The clinical picture depends on the width of the fistula itself and its communication between the trachea and the esophagus. The wider the fistula, the more signs defect, with any width of the fistula, the clinical manifestations are the same, but different in intensity, these are respiratory disorders that occur during feeding: shortness of breath, cyanosis of the skin, choking. And, it is this connection with the act of sucking that should alert the neonatologist about the presence of a tracheoesophageal fistula in a child. With a sufficiently wide fistula, the child's condition is very difficult and sometimes requires transferring him to artificial lung ventilation. Suction from the trachea of ​​gastric contents is the main diagnostic criterion. To clarify the diagnosis, it is enough to transfer the child to food through a tube and his condition improves. This type of nutrition is important not only for diagnosis, but for the treatment of the child. To clarify the diagnosis, tracheobronchoscopy is performed.

congenital intestinal obstruction, This frequent occasion for the hospitalization of a child surgical departments for newborns. The reasons can be divided into three groups: a violation of the formation of the intestine itself, its tube (its absence, stenosis, or the presence of a membrane); anomalies of the rotation of the intestine and its fixation; vices from others internal organs leading to intestinal compression (tumors, anomalies in the development of the pancreas, etc.). It should be pointed out that there is a so-called "benign" intestinal obstruction, this is meconium ileus. Blockage of the intestinal lumen by very thick meconium, the cause is still unknown.

Intestinal obstruction manifests itself acutely, from the first days, and sometimes even hours of a baby's life. She happens low and high , depends on the level of congenital malformation. The high defects are those that are at the level of the 12th duodenum, and the low ones are below it. Such a division is associated not only with a different clinical picture, but with a further prognosis. Low intestinal obstruction is more severe, and the prognosis is more unfavorable. Complications that are dangerous for the life of a baby can develop: perforation of the small intestine, which leads to peritonitis, but it can develop without perforation, the survival rate of children after such complications is not high. With high obstruction, such complications are not observed. For her clinical picture characterized by the accumulation of food in the stomach and 12 intestines, which is excreted by vomiting or regurgitation.

Leading symptom high intestinal obstruction , is vomiting, it is plentiful, sometimes with an admixture of bile, not frequent 1 time in 3-4 hours, appears for the first time a day after birth, and sometimes for the first time hours of life. If obstruction was not suspected after birth, and the child began to feed, then vomiting always associated with feeding, occurs immediately after it, the volume of vomit corresponds to the amount of food. The condition of children deteriorates rapidly, dehydration occurs and aspiration pneumonia develops with the formation of respiratory failure. Meconium (first stool) passes for a long time for 5-6 days, but later it is not present, such discharge is explained by the absence of intestinal motility, the stomach is swollen. With partial high intestinal obstruction, the clinical manifestations are "smoothed", vomiting may appear on the 2-3rd day of life, not plentiful. Dehydration develops more slowly, meconium passes faster, and even partially there is a stool, in small portions, in the future its amount decreases.

Clinical picture low intestinal obstruction , varied and depends on which part of the intestine is affected. It is rarely partial and its manifestations are acute. Most often there is a rotation disorder, it can occur both in utero and after birth. It is based on constriction of intestinal loops, which leads to impaired blood circulation, which, in turn, causes peritonitis. The clinic is the same for almost all forms. The paroxysmal anxiety of the baby for no apparent reason, associated with intestinal motility, comes to the fore. Bloating is noted immediately after birth, it does not disappear even after emptying the stomach. Vomiting with an admixture of intestinal contents and with blood occurs later than with high obstruction, usually by the end of the 2nd beginning of the 3rd day, not plentiful. There is no meconium, sometimes there is only lumps of mucus with greenish tint. Severe intoxication, intestinal loops can be felt, and in severe cases, they can be seen by eye.

Anomalies in the development of the digestive system, if possible, should be diagnosed in utero, so that it would be possible to prepare for the birth of an "unusual" baby into the world, mother and medical staff. Such childbirth should take place in specialized institutions, as the child after birth should receive qualified surgical care. And if it is not possible to diagnose during pregnancy, to maximize the diagnosis for the first time hours of life.