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

Various diseases of the esophagus occur in children of all age groups. Most often, the need for urgent surgical intervention arises in connection with congenital malformations and damage to the esophagus.

Somewhat less often, indications for emergency care are due to bleeding from the dilated veins of the esophagus with portal hypertension.

Malformations of the esophagus

Malformations of the esophagus are among the diseases that often cause the death of children in the first days of life or the occurrence of serious complications in them that disrupt further development. Among the numerous congenital defects of the esophagus for emergency surgery, those types that are incompatible with the life of a child without urgent surgical correction are of interest: congenital obstruction (atresia) and esophageal-tracheal fistulas.

Obstruction of the esophagus

Congenital obstruction of the esophagus is due to its atresia. This complex malformation is formed in the early stages of intrauterine life of the fetus and, according to our data, occurs relatively often (1 in every 3500 children is born with a malformation of the esophagus).

Perhaps the formation of 6 main types of congenital obstruction of the esophagus (Fig. 7).

With atresia, in most cases, the upper end of the esophagus ends blindly, and the lower part communicates with the trachea, forming a tracheoesophageal fistula (90-95%). It is clear that the amniotic fluid and the fluid that the baby swallows after birth cannot enter the stomach and accumulate with saliva in the upper blind sac of the esophagus, then regurgitate and aspirate. The presence of a fistula in the upper segment of the esophagus accelerates aspiration - the liquid after swallowing partially or completely enters the trachea. The child quickly develops aspiration pneumonia, which is aggravated by the throwing of the contents of the stomach into the trachea through the esophageal-tracheal fistula of the lower segment. A few days after birth, the baby dies of aspiration pneumonia. Such children can be saved only by urgent surgical correction of the defect.

A favorable outcome of the treatment of congenital obstruction of the esophagus depends on many reasons, but above all on the timely detection of atresia. Early initiation of special treatment reduces the possibility of aspiration. A diagnosis made in the first hours of a child's life (before the first feeding!), will prevent or significantly alleviate the course of aspiration pneumonia. However, pediatricians of maternity hospitals do not know enough about the symptoms of congenital obstruction of the esophagus. For example, among 343 children operated on by us from 1961 to 1981, only 89 were referred on the 1st day after birth, while the rest were admitted within 1 to 23 days.

The general condition of the patient and the degree of lung damage are directly dependent on the time of admission: in the group of patients admitted on the 1st day, only 29 patients were clinically diagnosed with pneumonia; in 126 children admitted on the 2nd day, the condition was regarded as severe, and bilateral pneumonia was diagnosed; almost all newborns who were referred over the age of 2 days (128) had a more severe process in the lungs (1/3 of them, in addition to pneumonia, had atelectasis of varying degrees on one or both sides).

clinical picture. The first earliest and most consistent sign that suggests esophageal atresia in a newborn is a large amount of frothy discharge from the mouth and nose. Unfortunately, this sign, which is noted in the documentation of maternity hospitals for all children, is not always given due importance and correctly assessed. Suspicion of esophageal atresia should increase if, after the usual suction of mucus, the latter continues to accumulate rapidly in large quantities. Mucus at times has a yellow color, which depends on the throwing of bile into the trachea through the fistula of the distal segment of the esophagus. In all children with obstruction of the esophagus, by the end of the 1st day after birth, quite distinct respiratory disorders (arrhythmia, shortness of breath) and cyanosis can be detected. Auscultation in the lungs is determined by an abundant amount of moist rales of various sizes. Abdominal distention indicates an existing fistula between the distal esophagus and the airways.

For early detection of a defect, we consider it necessary for all newborns and especially premature babies to perform probing of the esophagus immediately after birth. This will not only help identify atresia, but also allow you to suspect other anomalies of the digestive tract. It should be noted that for a newborn who does not have malformations, sounding will be beneficial, since the evacuation of the contents of the stomach prevents regurgitation and aspiration.

If the first indirect signs of atresia were detected in the maternity hospital, then the diagnosis should also be confirmed or rejected by probing the esophagus.

For probing, use the usual thin rubber catheter (No. 8-10), which is inserted through the mouth or nose into the esophagus. With atresia, a freely pushed catheter is delayed at the level of the top of the bag of the proximal segment of the esophagus (10-12 cm from the edge of the gums). If the esophagus is not changed, then the catheter easily passes for a greater distance. At the same time, it must be remembered that in some cases the catheter can develop, and then a false impression is created about the passage.

bridges of the esophagus. To clarify the diagnosis, the catheter is carried to a depth of more than 24 cm, and then its end (if there is atresia) will certainly be found in the child's mouth.

At the first feeding obstruction of a gullet comes to light quite definitely. All drunk liquid (1-2 sips) immediately pours back. Feeding is accompanied by a sharp violation of breathing; the newborn turns blue, breathing becomes superficial, arrhythmic, it stops. An attack of coughing can last from 2 to 10 minutes, and difficulty and arrhythmia of breathing can last even longer. These phenomena occur during each feeding. Gradually increasing cyanosis. When listening to the lungs, an abundant amount of different-sized moist rales is revealed, more on the right. The general condition of the child progressively worsens.

With exhaustive completeness and reliability, the diagnosis is established on the basis of an X-ray examination of the esophagus using a contrast agent, which is carried out only in a surgical hospital. The data obtained are a necessary part of the preoperative examination and serve as a guideline for choosing the method of surgical intervention. X-ray examination of children with suspected esophageal atresia begins with a plain chest x-ray (it is very important to carefully assess the condition of the lungs!). Then, a rubber catheter is passed into the upper segment of the esophagus and the mucus is sucked off, after which 1 ml of 30% water-soluble contrast agent is poured into the esophagus through the same catheter using a syringe. The introduction of a large amount can lead to an undesirable complication - overflow of the blind upper esophagus and aspiration with filling of the bronchial tree with a contrast agent . Radiographs are made with the child in the vertical position in two projections. The contrast agent after X-ray examination is carefully sucked off. The use of barium sulfate to study the esophagus in newborns with any form of atresia is contraindicated, since its entry into the lungs, which is possible in this case, causes atelectatic pneumonia.

In the general serious condition of the child (late admission, prematurity III-IV degree, etc.), you can not undertake examinations with a contrast agent, but limit yourself to the introduction of a thin rubber catheter into the esophagus (under the control of an X-ray screen), which will allow you to fairly accurately determine the presence and level of at -resions. It should be remembered that with a rough introduction of a low-elastic thick catheter, the pliable film of the blind oral segment of the esophagus can be displaced, and then a false impression is created about the low location of the obstruction.

A characteristic radiological symptom of esophageal atresia when examined with a contrast agent is a moderately enlarged and blindly ending upper segment of the esophagus (Fig. 8) -. The level of atresia is more accurately determined on lateral radiographs. On the-

The presence of air in the gastrointestinal tract indicates an anastomosis between the lower segment of the esophagus and the respiratory tract.

The visible upper blind sac and the absence of gas in the gastrointestinal tract suggest atresia without a fistula between the distal esophagus and the trachea. However, this radiographic finding does not always completely rule out a fistula between the distal esophagus and the airways. The narrow lumen of the fistula is clogged with a mucous plug, which serves as an obstacle to the passage of air into the stomach.

The presence of a fistula between the upper segment of the esophagus and the trachea can be detected radiographically by throwing a contrast agent through the fistula into the respiratory tract. This examination does not always help to detect a fistula, which in such cases is found only during surgery.

When analyzing clinical and radiological data and making a final diagnosis, one should be aware of possible combined malformations, which, according to our data, occur in 44.7% of patients, and some of them (26%) require emergency surgical correction or are incompatible with life ( 5%).

Differential diagnosis have to be carried out with asphyxic conditions of the newborn caused by birth trauma and aspiration pneumonia, as well as an isolated tracheoesophageal fistula and "asphyxic infringement" of a diaphragmatic hernia. In such cases, esophageal atresia is excluded by probing.

Treatment. Published data and analysis of our observations show that the success of surgical intervention depends on the early diagnosis of the defect, and hence the timely start of preoperative preparation, the rational choice of the surgical method, and the correct postoperative treatment.

Many combined defects significantly affect the prognosis. It is extremely difficult to treat premature babies, the number of which among those referred to our clinic reached 38%, and in the last 5 years - 45.4%. In newborns of this group, rapidly developing pneumonia, reduced child resistance and a peculiar reaction to surgical intervention are observed, which often creates insurmountable difficulties in treatment.

It should be noted that in the last 5 years, among the 44 premature children operated on by us, 23 recovered (8 of them without concomitant malformations).

Preoperative preparation. Preparation for the operation begins from the moment the diagnosis is established in the maternity hospital. The trachea is intubated, humidified 40% oxygen is continuously given, antibiotics and vitamin K are administered. The mucus secreted in large quantities is carefully sucked off through a soft rubber catheter inserted into the nasopharynx at least every 10-15 minutes. Feeding by mouth is absolutely contraindicated.

Transportation of the patient to the surgical department is carried out according to the rules provided for newborns, with the obligatory continuous supply of oxygen and periodic suction of mucus from the nasopharynx. From remote areas and regions, newborns are delivered by plane (the child usually transfers the flight satisfactorily).

Further preparation for the operation is continued in the surgical department, striving mainly to eliminate the phenomena of pneumonia. The duration of preparation depends on the age and general condition of the child, as well as on the nature of pathological changes in the lungs.

Children admitted in the first 12 hours after birth do not require long preoperative preparation (1 1/2-2 hours are enough). At this time, the newborn is placed in a heated incubator, humidified oxygen is constantly given, mucus is sucked out of the mouth and nasopharynx every 10-15 minutes. Administer antibiotics, vitamin K.

Children admitted later after birth with symptoms of aspiration pneumonia are prepared for surgery 6-24 hours. The child is placed in an elevated position in a heated incubator with a constant supply of humidified oxygen. Every 10-15 minutes, mucus is sucked out of the mouth and nasopharynx (an individual nursing post is required!). With long-term preparation, every 6-8 hours, mucus is sucked from the trachea and bronchi. Enter antibiotics, prescribe an aerosol with alkaline solutions and antibiotics. For late admission, parenteral nutrition is indicated (for liquid calculation, see Chapter 1). Preoperative preparation is stopped with a noticeable improvement in the general condition of the child and a decrease in the clinical manifestations of pneumonia.

If during the first six hours preoperative preparation does not have a noticeable success, then the presence of a fistulous tract between the upper segment and the trachea, in which mucus inevitably enters the respiratory tract, should be suspected. Continuation of preoperative preparation in such cases is useless, it is necessary to proceed to surgical intervention.

Surgery in case of esophageal atresia, it is carried out under endotracheal anesthesia and protective blood transfusion. The operation of choice should be considered the creation of a direct anastomosis. However, the latter is possible only in cases where the diastasis between the segments of the esophagus does not exceed 1.5 cm (with a high location of the upper segment, at the level of Th 1, -Th 2, anastomosis can only be created using a special stapler). The imposition of an anastomosis with a high tension of the tissues of the esophageal segments is not justified due to technical difficulties and the possible eruption of sutures in the postoperative period.

In newborns weighing less than 1500 g, multi-stage "atypical" operations are performed: delayed anastomosis with preliminary ligation of the esophageal-tracheal fistula, etc. (Bablyak D. E., 1975, etc.).

On the basis of clinical and radiological data, it is almost impossible to establish the true distance between the segments. Finally, this issue is resolved only during surgery. If during thoracotomy a significant diastasis (more than 1.5 cm) between the segments or a thin lower segment (up to 0.5 cm) is detected, then the first part of the two-stage operation is performed - the esophageal-tracheal fistula of the lower segment is eliminated and the upper end of the esophagus is brought to the neck. These measures prevent the development of aspiration pneumonia, thereby saving the life of the patient. The created lower esophagostomy serves to feed the baby until

Stage II of the operation - the formation of an artificial esophagus from the colon.

The operation of creating an anastomosis of the esophagus. It is most rational to use the extrapleural operative approach, which, according to our observations (156 newborns), greatly facilitates the course of surgical intervention and the postoperative period.

Technique of extrapleural access. The child lies on the left side. The right hand is fixed in a raised and retracted anterior position. A diaper rolled up in the form of a roller is placed under the chest. The incision is made from the middle axillary line to the paravertebral along the IV rib. Bleeding vessels are carefully ligated. Carefully dissect the muscles in the fourth intercostal space. The pleura is slowly exfoliated (first with a finger, then with a wet small tipper) along the incision up and down by 3-4 ribs. With a special screw retractor of small sizes, the edges of the wound of the chest wall are bred, after which the lung covered with pleura is retracted anteriorly and downward. The mediastinal pleura is exfoliated above the esophagus up to the dome. If the anatomical relationship allows you to create a direct anastomosis, then proceed to the mobilization of the segments of the esophagus.

Technique for mobilization of segments of the esophagus. Find the lower segment of the esophagus. The reference point is the typical location of the vagus nerve. The latter is removed inside, the esophagus is relatively easily isolated from the surrounding tissues and taken on a holder (rubber strip). The lower segment of the esophagus is mobilized over a short distance (2-2.5 cm), since its significant exposure can lead to impaired blood supply. Directly at the point of communication with the trachea, the esophagus is tied up with thin ligatures and crossed between them. The stumps are treated with an alcoholic solution of iodine. The ends of the thread on the short stump of the esophagus (near the trachea) are cut off. Tracheal fistula closure is usually not required. Only a wide lumen (more than 7 mm) is an indication for applying one row of continuous suture to the stump. The second thread at the free lower end of the esophagus is temporarily used as a "holder". The upper segment of the esophagus is found by the catheter inserted into it before the start of the operation through the nose. On top of the blind sac, a suture-“holder” is applied, for which it is pulled up, exfoliating the mediastinal pleura, and carefully isolated upwards with a wet tupfer (do not touch with tweezers!). The oral segment has a good blood supply, 1 which allows it to be mobilized as high as possible. Tight adhesions to the posterior wall of the trachea are carefully dissected with scissors. If there is a fistula between the upper segment and the trachea, the latter is crossed, and the holes formed in the trachea and esophagus are sutured with a single-row continuous marginal suture with atraumatic needles. The mobilized segments of the esophagus are pulled towards each other by the “handle” threads (Fig. 9). If their ends freely go behind each other, then they begin to create an anastomosis.

Anastomosis technique. Creating an anastomosis is the most difficult part of the operation. It should be noted that difficulties arise not only in connection with diastasis between the segments of the esophagus, but also depend on the width of the lumen of the distal segment. The narrower its lumen, the more difficult the suturing, the greater the likelihood of their eruption and the occurrence of narrowing of the anastomosis site in the postoperative period. According to our data, most often the distal segment has a lumen width of up to 0.4-0.6 cm.

Due to the variety of anatomical options for esophageal atresia, various methods of creating an anastomosis are used. Atraumatic needles are used for suturing.

Anastomosis by connecting segments of the esophagus in an end-to-end fashion. The first row of separate silk sutures is applied through all layers of the lower end of the esophagus and the mucous membrane of the upper segment (Fig. 10, a, b, c). The second row of sutures is passed through the muscle layer of both segments of the esophagus (Fig. 10, d. e). The greatest difficulty of this method lies in the imposition of the first row of sutures on extremely thin and delicate tissues, which cut through with the slightest tension. Anastomosis is used for small diastasis between the segments of the esophagus and the wide lower segment.

To connect the ends of the esophagus, you can use special sutures such as wrapping. Four pairs of such threads, superimposed symmetrically on both segments of the esophagus, initially serve as "holders" for which the ends of the esophagus are pulled up. After the convergence of their edges, the corresponding threads are tied. When tying the sutures, the edges of the esophagus are screwed inward. The anastomosis is reinforced with a second row of separate silk sutures. Such sutures make it possible to impose an anastomosis with some tension, without fear of cutting through the delicate tissues of the organ with threads (Fig. 11, a).

The technique of oblique anastomosis of the esophagus significantly reduces the possibility of stricture formation at the suturing site (Fig. 11b). We have developed and used in the clinic since 1956 the method of anastomosis, which lengthens the segments of the esophagus. On the lateral surfaces of the central segment of the esophagus, at a distance of approximately 0.7 cm from its top, the 1st and 2nd sutures are applied, capturing only the muscle layer of the esophagus 2-3 times with each thread (“twisted” suture, Fig. 12, a ). The free ends of these threads temporarily serve as "holders", sipping on which it is possible to completely avoid trauma to the esophagus with instruments. The 3rd seam is applied along the front surface 0.5 cm above the side ones, and the 4th one is also on the front surface, but slightly lower than the previous one. An arcuate incision is made between the 3rd and 4th sutures, forming a flap with a diameter of 0.5-0.7 cm from all layers of the esophageal wall. The 4th suture remains on the formed flap, and when the latter is turned down, it is on the posterior surface of the esophagus (Fig. 12b). The 5th and 6th sutures (Fig. 12, c) are placed on the lower segment of the esophagus on both sides, departing from the top of the mobilized end by 0.5-0.7 cm. These sutures temporarily serve as "holders". Then the lower segment is dissected between the 5th and 6th sutures along the posterior surface longitudinally from the apex downwards. The length of the incision should be about 1-1.5 cm. The 7th suture is placed at the upper edge of the anterior surface of the lower segment of the esophagus. The last, 8th, suture is placed on the posterior surface at the end of the longitudinal incision. The dissected part of the lower segment is flattened, the valve is rounded off (Fig. 12, d, e).

The catheter, which is inserted through the nose into the upper segment of the esophagus before the start of the operation, is further advanced through the hole formed into the lower segment and the stomach. Above the catheter, the corresponding threads - "holders" are connected, first the posterior, and then the lateral and anterior ones. Thus, the first row of anastomosis is created (Fig. 12, f). The second row of anastomosis sutures is formed with separate silk threads, which should be applied with particular care on the posterior surface of the esophagus. After completion of the second row of sutures, the anastomosis can be considered complete.

In those cases when during the operation it turns out that the segments of the esophagus are in contact without tension or go behind each other, we use the simplest anastomosis - “end to side”. After mobilization, ligation and cutting off, from the trachea, the lower segment of the esophagus is sutured through the muscle layers of the posterior surface for up to 0.8 cm with a continuous suture to the bottom of the expanded upper segment, forming the first row of sutures on the posterior lip of the future anastomosis (Fig. 13, a). The lumen of both segments of the esophagus is opened (0.5-0.7 cm), retreating 1-2 mm from the suture line, and a second row of sutures is placed on the posterior lip of the anastomosis (Fig. 13, b). Then, the catheter previously inserted into the upper segment is removed from the esophagus and a thin polyethylene tube is passed through the nose into the stomach for subsequent feeding of the child (the tube is carried out in all cases of anastomosis!). The anterior lip of the anastomosis is formed over the tube with a two-row suture (Fig. 13, c, d), the second row closes the stump of the lower segment.

In our clinic, the mechanical suture of the esophageal segments was successfully used with a special stapling device NIIKHAI. The use of a mechanical suture significantly reduces the time of the operation and simplifies the technique of creating an anastomosis. Contraindications are a significant diastasis between the segments, a sharp underdevelopment of the distal segment of the esophagus and prematurity III-IV degree.

Technique of imposing an anastomosis with the help of a stapler. After the isolation and mobilization of the segments of the esophagus, ligation and intersection of the esophageal-tracheal fistula, one is convinced of the possibility of creating an anastomosis. Then, a blanket suture is applied to the edge of the lower segment of the esophagus without tightening it.

Before applying the anastomosis, the surgeon carefully prepares and checks the device (Fig. 14, a). To do this, the thrust head 1 is unscrewed from the rod 2, its pointed end is removed inside the body of the device 3 and fixed in this position with a lock 4. After checking the charging of the device with tantalum clips, the movable handle is moved to its original position and the safety lock 5 is closed. In this position, the assistant introduces the device carefully through

the child's mouth is the upper segment of the esophagus (Fig. 14, b). Having opened the latch, move the rod forward, piercing the wall of the blind end of the esophagus with it. Then the surgeon, turning the rod by the button, screws on the thrust head and plunges it into the lower segment. A previously imposed “twisting” suture is tied over the stop head, the thread is cut off (Fig. 14, c). Pulling the rod into the body, bring the lower and upper segments into contact (Fig. 14, d), fixing this position with a retainer with a gap of 0.7 or 1.2 mm (depending on the thickness of the esophageal walls to be sutured). Having opened the fuse, the assistant, by smoothly pressing the movable handle, stitches the esophagus with staples. In this case, sections of the lower and upper segments are cut out and a lumen of the created anastomosis is formed. Further, returning the handle to its original position and opening the safety lock by turning the lock, the rod is released and the thrust head is advanced into the lower segment by 0.5-1 cm, and then the end of the apparatus body is carefully advanced past the anastomosis line (Fig. 14, e, f) . Only after that, having closely closed the thrust head with the body, the apparatus is carefully removed from the esophagus. The apparatus is removed strictly along the longitudinal axis of the esophagus under visual control.

Having completed the creation of the anastomosis, a thin polyethylene tube is passed through the child's nose into the stomach - drainage for subsequent nutrition. The chest is sutured tightly in layers, leaving a thin polyethylene drainage in the mediastinum for 1-2 days.

Double esophagostomy according to G. A. Bairov is the first stage of a two-stage operation and consists of the elimination of esophageal-tracheal fistulas, the removal of the oral segment of the esophagus to the neck and the creation of a fistula from its distal segment to feed the child in the postoperative period. The milk coming through the lower esophagostomy hole does not flow out after feeding, since during this operation the cardiac sphincter remains preserved.

Technique of lower esophagostomy. After making sure that the creation of a direct anastomosis is impossible, proceed to the mobilization of the segments of the esophagus. First, the upper segment is isolated for as long as possible. In the presence of a tracheal fistula, the latter is crossed, and the resulting hole in the esophagus and trachea is sutured with a continuous marginal suture. Then the lower segment is mobilized, tied at the trachea and crossed between two ligatures. Several separate silk sutures are applied to a short stump near the trachea. The vagus nerve is removed inside, and the esophagus is carefully isolated to the diaphragm (Fig. 15, a). In a blunt way (opening the introduced Billroth clamp), the esophageal opening is expanded, the stomach is pulled up and the peritoneum is dissected around the cardial section (take care of the vagus nerve. После этого ребенка поворачивают на спину и про--изводят верхнюю лапаротомию правым парамедианным разрезом. Мобилизованный дистальный сегмент проводят в брюшную полость через расширенное пищеводное отверстие (рис. 15, б, в). В надчревной области слева от средней линии производят поперечный разрез (1 см) через все слои брюшной стенки.!}

A mobilized esophagus is passed into the hole formed (Fig. 15, d) in such a way that it rises above the skin by at least 1 cm. Its wall is fixed from the inside with several sutures to the peritoneum, and sutured to the skin from the outside (Fig. 15, e). A thin tube is inserted into the stomach through the withdrawn esophagus, which is fixed with a silk thread tied around the protruding part of the esophagus. The wounds of the abdominal wall and chest are sewn up tightly (Fig. 15, f). The operation ends with the removal of the upper segment of the esophagus.

Upper esophagostomy technique. Under the shoulder blades, the child is placed with a thin roller (rolled diaper). The head is turned to the right. A skin incision up to I -1.5 cm long is carried out above the clavicle on the left at the internal crane of the sternocleidomastoid muscle (Fig. 16, a). The superficial fascia is dissected and the tissues are bluntly stratified up to the esophagus (the latter is detected by the catheter inserted into it before the operation). The curved end of the dissector bypasses the esophagus, mobilizes it in the distal direction, and the blind end is brought out into the wound (Fig. 16b). Several (4-5) separate sutures (atraumatic needle) fix its wall along the circumference in the depth of the wound to the muscles of the neck. Then the lumen of the esophagus is opened, cutting off its apex, and the edges are sutured to the skin through all layers (Fig. 16, c).

The second stage of the operation - the formation of the esophagus from the colon - is carried out at the age of 1 1/2-2 years.

postoperative treatment. The success of the operation largely depends on the correct conduct of the postoperative period. For more thorough care and careful observation in the first days after surgery, the child needs an individual nursing post and constant medical supervision. The child is placed in a heated incubator, giving the body an elevated position, constantly giving humidified oxygen. Continue the introduction of antibiotics, vitamins K, C, group B, UHF field on the chest. 24 hours after the operation, a control radiograph of the chest cavity is performed. The detection of atelectasis on the side of the operation is an indication for sanitation of the tracheobronchial tree.

In the first hours after surgery, the child may progressively develop respiratory failure, which requires urgent tracheal intubation and assisted breathing (after suction of mucus from the trachea). After a few hours, the child's condition usually improves and the tube can be removed from the trachea. Re-intubation after anastomosis should be done with great care and only by a clinician experienced in neonatal re-intubation. The erroneous introduction of the tube instead of the trachea into the esophagus threatens to rupture the sutures of the anastomosis.

Oxygenobarotherapy gives a positive effect. In the postoperative period after the simultaneous restoration of the continuity of the esophagus, children receive parenteral nutrition for 1-2 days. The correct calculation of the amount of fluid required for parenteral administration to a child is very important (see Chapter 1).

Some surgeons for feeding the child after the imposition of the anastomosis form a fistula of the stomach. We do not resort to this additional operation, since we insert a thin (0.2 cm) polyethylene drainage tube into the stomach during the main intervention, through which we start feeding the child (every 3 hours, very slowly, with breast milk, alternating with a glucose solution) . A single amount of liquid on the first day of feeding should not exceed 5-7 ml. In the following days, breast milk and glucose are gradually added (5-10 ml each), bringing the daily volume of fluid to the age norm by the end of the week after the operation. Feeding through a probe is carried out with the child in an upright position, which prevents fluid regurgitation through a functionally defective cardiac sphincter in a newborn. With an uncomplicated course, the probe is removed on the 8-9th day.

The patency of the esophagus and the state of the anastomosis is controlled after 9-10 days by X-ray examination with iodolipol. The absence of signs of anastomotic failure allows you to start feeding through the mouth from a horn or a spoon. At the first feeding, the child is given 10-20 ml of a 5% glucose solution, and then - breast milk. half the dose of the amount that the newborn received when feeding through a tube. In the following days, for each feeding, the amount of milk is increased daily by 10-15 ml, bringing it gradually to normal according to age and body weight. Fluid deficiency is compensated parenterally by fractional daily administration of 10% glucose solution, plasma, blood or albumin. This avoids a noticeable drop in body weight in the newborn.

On the 10-14th day, the child is applied to the chest for 5 minutes first and a control weighing is performed. Feeding the child from the horn. During this period, the required amount of food is calculated based on the body weight of the child: it should fluctuate between 1/5 and 1/6 of the body weight of the newborn.

Gradually increase the time of attachment to the breast and at the beginning of the 4th week they switch to 7 times feeding.

Postoperative management of a child who underwent the first part of a two-stage esophageal plasty has some differences due to the possibility of feeding through the esophagostomy opening. The small size of the stomach of a newborn, a violation of its motor function due to trauma during the creation of a fistula, requires fractional feeding with a gradual increase in the amount of fluid administered. Below we give an approximate scheme of feeding a child after the first stage of a two-stage operation: 1st day - 3-5 ml x 10; 2nd day - 7 ml x 10; 3-4th day-15-20ml x 10; 5th day -25-30ml x 10; 6th day - 30-40 ml x 10. The rest of the required amount of liquid is administered parenterally. From the 7th day after the operation, the volume of the stomach increases so much that up to 40-50 ml can be slowly injected through the esophagostomy hole (3/4 of the total amount of liquid is replenished with breast milk and 1/4-10% glucose solution). From the 10th day they switch to 7 meals a day with normal dosages.

In the first days and weeks after the intervention, careful care of fistulas on the neck and anterior abdominal wall is necessary. The latter especially needs frequent dressing changes, skin treatment with antiseptic pastes, UV radiation. A thin rubber drain inserted into the stomach is not removed 10-1 2 days until the complete formation of the lower esophageal fistula. Then the probe is inserted only at the time of feeding.

The sutures are removed on the 10-12th day after the operation. Children are discharged from the hospital (with an uncomplicated course) after a stable weight gain is established (week 5-6). If a newborn has had a double esophagostomy, the child's mother should be taught proper fistula care and feeding techniques.

Further monitoring of the child is carried out on an outpatient basis by a surgeon together with a pediatrician. Preventive examinations should be carried out every month before the child is sent to the second stage of the operation - the creation of an artificial esophagus.

Surgical complications in in the postoperative period, they occur mainly in newborns who underwent simultaneous plastic surgery of the esophagus.

The most severe complication should be considered anastomotic suture failure, which, according to published data, occurs in 10-20% of operated patients. The resulting mediastinitis and pleurisy usually end in death, despite the creation of a gastrostomy, drainage of the mediastinum and pleural cavity. With early recognition of the divergence of the anastomotic sutures, an urgent rethoracotomy and the creation of a double esophagostomy are necessary.

In some cases, after the creation of a direct anastomosis, re-nalization of the tracheoesophageal fistula occurs, which is manifested by sharp bouts of coughing with each attempt to feed through the mouth. The complication is recognized when examining the esophagus with a contrast agent (iodolipol flows into the trachea), and the final diagnosis is established by subanesthetic tracheobronchoscopy. Treatment is carried out by

creation of a gastrostomy and drainage of the mediastinum (with an increase in the phenomena of mediastinitis).

After the subsidence of inflammatory changes and the final formation of the fistulous tract, a second operation is indicated. Sometimes recanalization is combined with stenosis in the area of ​​the anastomosis.

After the simultaneous creation of an anastomosis of the esophagus, all children are subject to dispensary observation for at least 2 years, since late complications may occur during this period.

In some children, after 1 1/2-2 months after the operation, a rough cough appears, which is associated with the formation of adhesions and scars in the area of ​​the anastomosis and trachea. Carrying out physiotherapeutic measures helps to get rid of this late complication, which is usually eliminated at the age of 5-6 months.

In the period up to 1-1 1/2 years after surgery, an isolated narrowing of the esophagus in the area of ​​the anastomosis may occur. The treatment of this complication usually does not cause difficulties: several sessions of bougienage (under the control of esophagoscopy) lead to recovery.

In rare cases, conservative measures are unsuccessful, which is an indication for repeated thoracotomy and plastic surgery of the narrowed esophagus.

long-term results, traced by us for 5-40 years in children after the creation of the anastomosis, show that they do not lag behind their peers in development. The use of a mechanical suture of the esophagus does not adversely affect the development of the organ.

Examination of 35 children who completed stage II of a two-stage operation (retrosternal plastic surgery of the esophagus with the colon) revealed that the created esophagus functions well, there is no regurgitation due to preserved cardiac sphincter.

RCHD (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical Protocols of the Ministry of Health of the Republic of Kazakhstan - 2014

Esophageal atresia without fistula (Q39.0) Esophageal atresia with tracheoesophageal fistula (Q39.1) Other congenital malformations of esophagus (Q39.8)

Congenital diseases, Pediatrics, Neonatal surgery

general information

Short description

Approved by the Expert Commission

For Health Development

Ministry of Health of the Republic of Kazakhstan

Esophageal atresia- this is an incomplete formation of the esophagus, often combined with a tracheoesophageal fistula. The diagnosis is made when it is impossible to pass a nasogastric tube into the stomach. Treatment is operative. Frequency - 1 in 3000-5000 newborns.

I. INTRODUCTION

Protocol name: Malformations of the esophagus in newborns

Protocol code:

ICD-10 code(s):

Q 39 Congenital anomalies (malformations) of the esophagus

Q 39.0 Congenital atresia of esophagus without fistula

Q 39.1 Congenital atresia of esophagus with tracheoesophageal fistula

Q39.8 Other congenital malformations of esophagus

Abbreviations used in the protocol:

Er-erythrocytes;

FiO2 - concentration of supplied oxygen

Hb-hemoglobin

Ht- hemotocrit

L-leukocytes

NIPPV - nasal intermittent positive pressure ventilation

ALT-alanine aminotransferase

BPD - bronchopulmonary dysplasia

IVH - intraventricular (non-traumatic) hemorrhage

DIC - disseminated intravascular coagulation

GIT - gastrointestinal tract

IVL - artificial lung ventilation

IT - intensive care

ELISA

CCT - contrast CT

CT - computed tomography

KShchS - acid-base state

MV - mechanical ventilation

MRI - magnetic nuclear tomography

KLA - complete blood count

OAM - general urinalysis

PDA - open ductus arteriosus

BCC - volume of circulating blood

PCR - polymerase chain reaction

RDS − Respiratory Distress Syndrome

ROP - retinopathy of prematurity

See H2O - centimeters of water column

CPAP - continuous positive airway pressure

CRP - C-reactive protein

SUV - Air Leak Syndrome

TBI is a severe bacterial infection

TTN - transient tachypnea of ​​the newborn

RR - respiratory rate

HR - heart rate

EchoCG - echocardiography

Protocol development date: 2014

Protocol Users: pediatrician, neonatal surgeon of the hospital /

Classification

Clinical classification

About 100 variants of this defect are known, but there are three most common:

Esophageal atresia and fistula between the distal esophagus and trachea (86-90%),

Isolated esophageal atresia without fistula (4-8%),

Tracheoesophageal fistula, "type H" (4%).

In 50-70% of cases of esophageal atresia, combined malformations occur:

Congenital heart defects (20-37%),

Malformations of the gastrointestinal tract (20-21%),

Defects of the genitourinary system (10%),

Defects of the musculoskeletal system (30%),

Defects of the craniofacial region (4%).

In 5-7% of cases, esophageal atresia is accompanied by chromosomal abnormalities (trisomy 18, 13 and 21). A peculiar combination of developmental anomalies in esophageal atresia is designated as "VATER" in the initial Latin letters of the following malformations (5-10%):

Spinal defects (V),

Anus defects (A),

Tracheoesophageal fistula (T),

Esophageal atresia (E),

Radius defects (R).

30-40% of children with esophageal atresia are not full-term or have intrauterine growth retardation.

Diagnostics

II. METHODS, APPROACHES AND PROCEDURES FOR DIAGNOSIS AND TREATMENT

The list of basic and additional diagnostic measures:

UAC (6 parameters)

Determination of blood gases

General urine analysis

Coagulogram

Determination of total protein

Determination of urea and residual nitrogen

Definition of ALT, AST

Determination of glucose

Determination of bilirubin

Determination of potassium and sodium

Definition of diastase

Determination of blood group and Rh factor

Plain chest x-ray

Ultrasound of the abdominal cavity and retroperitoneal space

Echocardiodoplerography

Neurosonography

X-ray examination with contrasting of the esophagus.

Diagnostic criteria

Complaints and anamnesis:

Prenatal diagnosis: polyhydramnios, lack of visualization of the stomach, visualization of the dilated proximal segment of the esophagus.

Postnatal diagnosis: large amount of frothy discharge from the mouth and nose.

Suspicion should be increased if, after the usual suction of mucus, the latter continues to accumulate rapidly in large quantities.

Inability to pass a nasogastric tube into the stomach.

Physical examination: in all children with obstruction of the esophagus, by the end of the first day after birth, quite distinct respiratory disorders (arrhythmia, shortness of breath) and cyanosis can be detected. Auscultatory: in the lungs there is an abundant amount of moist rales of various sizes. Abdominal distension indicates an existing fistula between the distal esophagus and the airways.

Laboratory research:

Blood test for electrolytes - metabolic acidosis,

KLA - leukocytosis.

Instrumental Research :

Catheterization of the esophagus through the nose with a thin radiopaque catheter with a rounded end. With atresia of the esophagus, the catheter, having passed to a depth of about 6 centimeters, sticks into the blind end of the esophagus or, wrapping itself, exits through the child's nose.

. "Elephant's test" (English elephant - elephant) - the air introduced through the probe into the blind end of the esophagus comes out of the nose with noise.

. Plain radiography of the chest and abdominal organs:

A) non-contrast - with atresia, the radiopaque catheter is clearly visible in the blind segment of the esophagus. The presence of air in the stomach and intestines indicates a fistula between the trachea and the abdominal end of the esophagus. With fistulous forms - the absence of gas in the gastrointestinal tract;

B) contrast (contrasting the inserted probe with water-soluble contrast agents) - with atresia, the blind end of the esophagus is clearly visible, in the presence of fistulas, the contrast flows into the trachea.

Indications for expert advice:

Genetics - the presence of a congenital malformation,

Cardiologist - exclusion of concomitant pathology from the cardiovascular system,

Neurologist - the presence of asphyxia (according to indications).

Differential Diagnosis

Differential Diagnosis:

Asphyxic conditions of the newborn, caused by birth trauma and aspiration pneumonia.

Isolated tracheoesophageal fistula.

. "Asphyxic infringement" of diaphragmatic hernia.

Differential diagnosis (X-ray picture)

Treatment

Treatment Goals

Elimination of the tracheo-esophageal anastomosis with the formation of an anastomosis of the esophagus.

Treatment tactics -

Non-drug treatment:

Mode - intensive care unit,

Total parenteral nutrition before correction of congenital malformations,

A set of measures aimed at preventing aspiration of saliva by placing a catheter and its continuous aspiration,

Prevention of reflux of gastric contents through the fistula by creating an elevated position of the child.

Medical treatment

Antibiotic therapy

Preparations of "starting" therapy:

Cephalosporins 2-3rd generation,

aminoglycosides,

aminopenicillins,

macrolides;

"Reserve" drugs:

cephalosporins 3-4th generation,

Aminoglycosides of the 2nd-3rd generation,

carbapenems,

Rifampicin.

Each neonatal unit should develop its own protocols for the use of antibiotics, based on the analysis of the spectrum of pathogens that cause early sepsis.

Painkillers:

Before surgery infusion administration of sedative, analgesic or muscle relaxant drugs is indicated:

Diazepam or medozalam 0.1 mg/kg/hour

Promedol 0.1-0.2 mg/kg/hour

Fentanyl 1-3 mcg/kg/hr (to prevent sympathetic pulmonary vasoconstriction in response to severe external stimuli (such as tracheal suctioning).

Atracurium besilate 0.5 mg/kg/hour

Pipecuronium bromide 0.025 mg/kg/hour

Postoperative pain relief:

Intravenous continuous infusion of fentanyl 5-10mcg/kg/hour in combination with acetaminophen (paracetamol) rectally or intravenously in a single dose of 10-15 mg/kg 2-3 times a day or metamizole sodium 5-10 mg/kg 2-3 times intravenously;

The dosage of analgesics and the duration of administration are selected individually depending on the severity of the pain syndrome.

When the child is desynchronized with the ventilator, sedatives:

Midozalam (Relanium) up to 0.17 mg/kg/hour (with continuous infusion). Should be avoided in neonates up to 35 weeks' gestation.

Phenobarbital (10-15 mg/kg/day)

Muscle relaxants in the tension of the anastomosis:

Listenon, arduan;

Infusion therapy and parenteral feeding:

Most neonates should be started on intravenous fluids at 70-80 ml/kg per day.

In newborns, the volume of infusion and electrolytes should be calculated individually, allowing for 2.4-4% weight loss per day (15% in total) in the first 5 days

Sodium intake should be limited in the first few days of postnatal life and initiated after the onset of diuresis, with close monitoring of fluid balance and electrolyte levels.

If there is hypotension or poor perfusion 10-20 ml/kg 0.9% NaCl should be administered once or twice.

CALCULATION OF THE REQUIRED VOLUME OF ELECTROLYTES

It is advisable to start the introduction of sodium and potassium no earlier than the third day of life, calcium from the first day of life.

SODIUM DOSE CALCULATION

− Sodium requirement is 2 mmol/kg/day

− Hyponatremia<130 ммоль/л, опасно < 125 ммоль/л

− Hypernatremia > 150 mmol/l, dangerous > 155 mmol/l

− 1 mmol (mEq) of sodium is contained in 0.58 ml of 10% NaCl

− 1 mmol (mEq) of sodium is contained in 6.7 ml of 0.9% NaCl

1 ml of 0.9% (physiological) sodium chloride solution contains 0.15 mmol Na

CORRECTION OF HYPONATREMIA (Na< 125 ммоль/л)

Volume of 10% NaCl (ml) = (135 -Na patient) × mbody × 0.175

CALCULATION OF THE DOSE OF POTASSIUM

− Potassium requirement is 2-3 mmol/kg/day

− Hypokalemia< 3,5 ммоль/л, опасно < 3,0 ммоль/л

- Hyperkalemia > 6.0 mmol/l (in the absence of hemolysis), dangerous > 6.5 mmol/l (or if there are pathological changes on the ECG)

− 1 mmol (mEq) of potassium is contained in 1 ml of 7.5% KCl

− 1 mmol (mEq) of potassium is contained in 1.8 ml of 4% KCl

V(ml 4% KCl) = requirement for K+(mmol)×mbody×2

CALCULATION OF THE DOSE OF CALCIUM

- The need for Ca ++ in newborns is 1-2 mmol / kg / day

− Hypocalcemia<0,75 -0,87 ммоль/л (доношенные -ионизированный Са++), < 0,62 -0,75 ммоль/л (недоношенные -ионизированный Са++)

− Hypercalcemia>1.25 mmol/l (ionized Ca++)

− 1 ml of 10% calcium chloride contains 0.9 mmolCa++

− 1 ml of 10% calcium gluconate contains 0.3 mmolCa++

CALCULATION OF THE DOSE OF MAGNESIUM:

− The need for magnesium is 0.5 mmol/kg/day

− Hypomagnesemia< 0,7 ммоль/л, опасно <0,5 ммоль/л

− Hypermagnesemia > 1.15 mmol/l, dangerous > 1.5 mmol/l

− 1 ml of 25% magnesium sulfate contains 2 mmol of magnesium

The volume of infusion therapy can be very variable, but rarely exceeds 100-150 ml/kg/day.

Maintain desired blood pressure with adequate volumetric loading.

− dopamine 5-15 mcg/kg/min,

- dobutamine 5-20 mcg / kg / min,

- adrenaline 0.05-0.5 mcg / kg / min.

Other treatments are not.

Surgical intervention

Preparation for the operation can last for one or several days - the more severe the pneumonia, the more prolonged the preoperative preparation.

Absolute readings for emergency surgery for esophageal atresia:

Wide lower tracheoesophageal fistula

The combination of atresia of the esophagus with duodenal obstruction.

Relative readings

Isolated esophageal atresia without fistula.

In case of isolated esophageal atresia without fistula:

Placement of a gastrostomy

The main stage of the operation - esophagoesophagoanastomosis - is performed on a delayed basis at the age of 3 months - 2 years with diastasis between the segments of the esophagus less than 2 cm.

In case of esophageal atresia with tracheoesophageal fistula:

Elimination of tracheo-esophageal fistula,

With diastasis between the segments of the esophagus less than 2 cm - separation of the esophageal-tracheal fistula and the imposition of a direct anastomosis between the ends of the esophagus.

With a larger diastasis of the ends of the esophagus, surgical treatment is carried out in two stages:

Esophagostomy and gastrostomy.

At the age of 2-3 months to 3 years - plastic surgery of the esophagus with an intestinal transplant.

In all cases, with esophageal atresia with a tracheoesophageal fistula, an anastomosis of the esophagus is created - primary or delayed. The primary anastomosis is imposed when, after cutting off the tracheoesophageal fistula from the trachea and immobilizing the segments, they can be brought together without tension.

With a significant diastasis between the segments of the esophagus (usually more than 2.0 cm), a decision is made to impose a delayed anastomosis. Stage 1: elimination of the tracheoesophageal fistula, suturing of the trachea, suturing of the lower segment and fixing it to the chest wall, gastrostomy, drainage of the retropleural space. After the operation, a constant passive outflow of saliva and frequent (every 30 minutes, by the end of the 2nd month every 10-15 minutes) active aspiration from the upper segment are established. After 6-8 weeks, an x-ray examination is performed. With a reduction in diastasis, they proceed to the 2nd stage - rethoracotomy and the imposition of a delayed anastomosis.

With isolated atresia of the esophagus, the following stages of treatment are carried out:

Lower esophagostomy from abdominal access (or gastrostomy);

Retrosternal plastic surgery of the esophagus at the age of 4-6 months with simultaneous esophagocolonostomy on the neck;

Closure of the lower esophagostomy (or gastrostomy).

Preventive actions:

Long-term mechanical ventilation (6-8 days);

Maintaining a state of deep medical sleep and muscle relaxation;

The probe does not change until the anastomosis heals (10-12 days);

Retropleural drainage is removed on the 6th day;

By the 8th day, the child is extubated and enteral feeding is started;

X-ray examination is carried out on the 14-15th day.

Further management, rehabilitation: on the 21st day, calibration bougienage (up to No. 22) is performed under screen control and general anesthesia. Esophagography after 3 months

All children operated on for congenital atresia of the esophagus need dispensary observation.

Indicators of treatment efficacy and safety of diagnostic and treatment methods described in the protocol:

Free patency of the esophagus for food;

No respiratory problems.

Drugs (active substances) used in the treatment

Hospitalization

Indications for hospitalization:

Emergency hospitalization of a newborn to establish a diagnosis.

Information

Sources and literature

1. Minutes of the meetings of the Expert Commission on Health Development of the Ministry of Health of the Republic of Kazakhstan, 2014
   1. 1) Isakov Yu.F., Dronov A.F. Pediatric surgery: national guidelines. - Moscow. - GEOTAR-Media. - 2009. - 1168 pages. 2) Bairov G.A., Urgent surgery for children. Guide for doctors. - St. Petersburg: Peter, 1997.- 462 p. 3) Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus. /Reiss a T. Schaible b L. van den Houta I. Capolupo c K. AllegaertdA. van Heijst e M. Gorett Silva f A. Greenough g D. Tibboel a for the CDH EURO consortium., 2010 4) Ashcraft K.W., Holder T.M., Pediatric surgery. T. 1-3, St. Petersburg, 1996. 5) G.W.Holcomb, K.E.Georgeson, S.S.Rothenberg Atlas of pediatric laparoscopy and thoracoscopy.-2008.- 315 p. 6) Stepanov E.A., Razumovsky A.Yu., Children's surgery (national leadership), vol. 2: “Thoracic surgery”. M.: Publishing group GEOTAR-Media, 2008, 200-301. 7) Puri P., Hollwarth M.E. Pediatric surgery. Springer, Berlin, Heidelberg, 2006. 8) Swenney B.T., Oldham K.T., Pulmonary Malformation In P. Pury, Hollwarth M.E. (eds), Pediatric Surgery, Springer Surgery Atlas Series. Springer-Verlag, Heidelberg, NewYork, 2006, 107-14/ 9) Prenatal sonography. Ed. M.V. Medvedev. 1st edition. M.: Realnoe Vremya, 2005, 341-68 10) Langston C., New concepts in the pathology of congenital lung malformations. Semin. Pediatric Surg., 2003, 12, 17-37
   
   

   Attached files

   Attention!

   • By self-medicating, you can cause irreparable harm to your health.
   • The information posted on the MedElement website and in the mobile applications "MedElement (MedElement)", "Lekar Pro", "Dariger Pro", "Diseases: Therapist's Handbook" cannot and should not replace an in-person consultation with a doctor. Be sure to contact medical facilities if you have any diseases or symptoms that bother you.
   • The choice of drugs and their dosage should be discussed with a specialist. Only a doctor can prescribe the right medicine and its dosage, taking into account the disease and the condition of the patient's body.
   • The MedElement website and mobile applications "MedElement (MedElement)", "Lekar Pro", "Dariger Pro", "Diseases: Therapist's Handbook" are exclusively information and reference resources. The information posted on this site should not be used to arbitrarily change the doctor's prescriptions.
   • The editors of MedElement are not responsible for any damage to health or material damage resulting from the use of this site.

Occurrence: 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;

sounding of the esophagus;

contrast study of the esophagus;

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

Treatment - surgical:

If there are no signs of atelectasis, pneumonia - a one-stage operation for closing the esophageal-tracheal 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: first, a gastrostomy is applied, intensive therapy is carried out until the condition improves, and then the fistula is closed and an anastomosis is made between the upper and lower segments of the esophagus;

With multiple malformations, in severely weakened newborns, the proximal end of the esophagus is brought out to the neck to avoid the accumulation of saliva in it, and a gastrostomy is applied 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.

8.2. Congenital stenoses of the esophagus

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

Clinic: HH, esophagitis, achalasia. With a significant narrowing of the esophagus, a suprastenotic expansion of the esophagus occurs. Symptoms usually do not appear until solid foods are introduced into the child's diet.

Diagnostics:

clinical manifestations;

fibroesophagogastroscopy;

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

8.3. Congenital membrane diaphragm of the esophagus

The diaphragm consists of connective tissue covered with keratinized epithelium. This diaphragm often has holes through which food can enter. The diaphragm 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, as a rule, 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. With a diaphragm that completely blocks the lumen, it must be removed under endoscopic control.

Anatomy and physiology of the esophagus.

The esophagus is a muscular tube about 25 cm long (from the pharynx to the cardia). Cervical region - 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 abdomen: signs 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 therapy is carried out until the condition improves, and then the fistula is closed and anastomosis is made 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 solid foods are introduced into the child's diet.

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 membranous diaphragm of the esophagus.

The diaphragm consists 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 the clinical symptoms are the same as with 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

The malformations of the esophagus include its dysgenesis, concerning its shape, size and topographic relationship to the surrounding tissues. The frequency of these defects is on 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 in the development of the trachea - a fact that is understandable if we take into account 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 a few days after birth), others are compatible, but require certain interventions.

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

In addition to malformations of the esophagus itself, dooming newborns either to a quick starvation or to traumatic surgical interventions, congenital dysphagia can be caused by malformations of organs adjacent to the normal esophagus (abnormal origin of the right carotid and subclavian arteries from the aortic arch and from its descending part on the left, which predetermines the pressure of these abnormally located large vessels on the esophagus at the intersection - dysphagia lussoria). I.S. Kozlova et al. (1987) distinguish the following types of esophageal atresia:

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

Often, esophageal atresia is combined with other malformations, in particular, with congenital heart, gastrointestinal, genitourinary, skeletal, central nervous system, and facial clefts. In 5% of cases, anomalies of the esophagus occur in chromosomal diseases, such as Edwards syndrome (characterized by congenital anomalies in children, manifested by paresis and paralysis in various peripheral nerves, deafness, multiple dysgenesis of internal organs, including chest organs) and Down syndrome (characterized by congenital dementia and characteristic signs of physical deformities - short stature, epicanthus, small short nose, enlarged folded tongue, "clown" face, etc.; 1 case per 600-900 newborns), in 7% of cases it is a component of non-chromosomal etiology.

Esophageal atresia. With congenital obstruction of the esophagus, the upper (pharyngeal) end of it ends blindly at about the level of the notch of the sternum or slightly lower; its continuation is a muscular-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 located 1-2 cm above its bifurcation. Fistulous openings open either into the pharyngeal or into the cardial blind segment of the esophagus, and sometimes into both. Congenital obstruction of the esophagus is detected from the first feeding of the newborn and is especially pronounced if it is combined with an esophageal-tracheal fistula. In this case, this malformation is manifested not only by obstruction of the esophagus, which is characterized by constant salivation, regurgitation of all swallowed food and saliva, but also severe disorders caused by fluid entering the trachea and bronchi. These disorders are synchronized with every sip and are manifested from the first minutes of a child's life by coughing, choking, cyanosis; they occur with a fistula in the proximal esophagus, when fluid from the blind end enters the trachea. However, even with an esophageal-tracheal fistula in the region of the cardiac segment of the esophagus, respiratory disorders soon develop due to the fact that 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 anomalies and without emergency surgical intervention, children die early either from pneumonia or from exhaustion. The only way to save a child is through plastic surgery; gastrostomy can be used as a temporary measure.

The diagnosis of esophageal atresia is established on the basis of the above signs of aphagia, using probing and radiography of the esophagus with contrasting iodolipol.

Stenosis of the esophagus with its partial patency in the majority refers to stenosis compatible with life. Most often, the narrowing is localized in the lower third of the esophagus and is probably due to a violation of its embryonic development. Clinically, esophageal stenosis is characterized by a violation of swallowing, which manifests itself immediately when eating semi-liquid and especially dense food. An x-ray examination visualizes a smoothly narrowing shadow of the contrast agent with its spindle-shaped expansion above the stenosis. With fibrogastroscopy, stenosis of the esophagus with an ampullo-shaped extension above it is determined. The mucous membrane of the esophagus is inflamed, in the area of ​​stenosis it is smooth, without cicatricial changes. Esophageal stenosis is caused by food blockages with dense foods.

The treatment of esophageal stenosis is to widen the stenosis with bougienage. 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 hernias caused by the divergence of the fibers of the diaphragm in its cardial section with retraction into the chest cavity of the cardial part of the stomach.

Congenital shortening of the esophagus is characterized by its underdevelopment in length, as a result of which the adjacent part of the stomach exits through the esophageal opening of the diaphragm into the chest cavity. Symptoms of this anomaly consist of recurrent nausea, vomiting, regurgitation of food mixed with blood, and the appearance of blood in the stool. These phenomena quickly lead the newborn to weight loss and dehydration.

The diagnosis is established by fibroesophagoscopy and radiography. This anomaly should be differentiated from esophageal ulcers, especially in infants.

Congenital enlargement of the esophagus is an anomaly that occurs extremely rarely. Clinically, it is manifested by congestion and slow passage of food through the esophagus.

Treatment in both cases is non-operative (appropriate diet, maintaining an upright posture after feeding the baby). In rare cases, with severe functional disorders - plastic surgery.

Esophageal deviations occur with abnormalities in the development of the chest and the occurrence of volumetric pathological formations in the mediastinum, which can displace the esophagus relative to its normal position. Deviations of the esophagus are divided into congenital and acquired. Congenital correspond to anomalies in the development of the thoracic skeleton, acquired, occurring much more often, are caused either by a traction mechanism resulting from some kind of cicatricial process that has captured the wall of the esophagus, or by external pressure caused by diseases such as goiter, tumors of the mediastinum and lungs, lymphogranulomatosis, aortic aneurysm, spinal pressure, etc.

Esophageal deviations are divided into total, subtotal and partial. Total and subtotal deviations of the esophagus is 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. An x-ray examination reveals the approach of the esophagus to the sternoclavicular joint, the intersection of the esophagus with the trachea, angular and arcuate curvature of the esophagus in this area, combined displacements of the esophagus, heart and large vessels. Most often, the deviation of the esophagus occurs to the right.