What does a child with a diaphragmatic hernia look like? Congenital diaphragmatic hernia

Diaphragmatic hernias in children are observed relatively often (1 in 1700). The mortality rate for this disease is 1-3% of the total mortality rate of newborns, and during the first year of life - 12% mortality rate among children dying due to developmental defects.

Origin of diaphragmatic hernia in children

The formation of a thinned zone or a through defect in the diaphragm dome occurs in early stages development in the embryo or fetus. Deviations in the formation of the muscular layer of the diaphragm arise due to disruption of trophic processes associated with the peculiarities of metabolism in the body of the mother and fetus. Subsequently, forces acquire pathological significance intra-abdominal pressure fetus, promoting the movement of internal organs through the underdeveloped diaphragm. At the same time, the air-intestinal pockets remain unobliterated, which turn into preformed hernial sacs, similar to the vaginal process of the peritoneum with inguinal hernia. Acquired diaphragmatic hernias in children occur more often due to closed injury pelvis, abdomen and chest or due to an infectious-toxic process (poliomyelitis, tuberculosis).

Symptoms of diaphragmatic hernia in children

The clinical picture is due to changes that occur when organs move abdominal cavity. These include disorders of the respiratory function, disruption of the passage of food through the digestive tract and its trauma, abnormalities in cardiac activity and general disorders. The combination of these symptoms depends on age and type of hernia. How younger age child, the more pronounced the signs of a hernia appear. With diaphragmatic hernias in children, shortness of breath during exertion (running, walking, crying), abdominal pain, weakness, increased fatigue. Developmental delays in children are associated with oxygen starvation and recurrent pneumonia, which often cause the death of these patients. Newborns and infants experience bouts of cyanosis, vomiting, and sometimes coughing and hiccups. The borders of the heart are sharply shifted in the direction opposite to the hernia, usually to the right. Among hernias of the diaphragm itself great danger For the patient they represent false hernias, in which strangulation is possible. Hernias hiatus manifested by persistent vomiting. As a result of erosive-ulcerative esophagitis and gastritis, patients experience bloody vomiting, tarry stools, and develop (hemorrhagic syndrome). Developmental delays in children with esophageal hernias are the result of malnutrition. Hernias anterior section diaphragms can be asymptomatic or with abdominal pain, shortness of breath, and stridor breathing. More striking symptoms are observed in patients with phrenopericardial hernia. 30% of all children with diaphragmatic hernia have chest deformity; 25% of children are asymptomatic.

Physical examination of children reveals pathological abnormalities(presence of areas of tympanitis or dullness of percussion sound, disappearance and weakening of respiratory sounds, appearance of audible intestinal peristalsis, rumbling, splashing) in areas of the chest corresponding to the localization a certain type hernias. With a diaphragmatic hernia, changes are noted in the corresponding half of the chest, with an esophageal hernia - in the interscapular region, with an anterior hernia - at the level of the sternum and parasternally. In these areas, deviations are detected during x-ray examination, which allows us to establish a final and accurate topical diagnosis. A diaphragmatic hernia is characterized by a number of symptoms: the appearance in the lung field of air bubbles of a cellular structure or bubbles with the presence of levels and areas of darkening; inconsistency of data noted during repeated studies (“symptom of variability”); high position of the diaphragm, violation of the continuity or correctness of its contour, violation of the mobility of the diaphragm; displacement of the borders of the heart. Clarification of the diagnosis in most cases requires the use of a contrast study of the gastrointestinal tract (in newborns and infants - lipoidol, in older children - barium suspension). Sometimes pneumoperitoneum is necessary. Differential diagnosis When examining patients, it is carried out between partial and complete (relaxation) thinning of the dome of the diaphragm. When the diaphragm relaxes, the presence of a highly located boundary line is noted, which does not shift and represents a regular arcuate curve; at deep breath no rocking movements are observed, which indicates the absence of functioning muscle layers in the thoraco-abdominal barrier. Establishing a preoperative diagnosis of complete thinning of the dome of the diaphragm (relaxation) is important; in order to avoid relapse, it is advisable to use alloplastic material. Hiatal hernias in children are differentiated from undescended stomach (thoracic stomach, short esophagus). The differential diagnosis of a hernia and undescended stomach is of practical importance, because in case of a hernia, surgery is indicated, and in case of undescended stomach, conservative treatment is indicated.

Complications

The main complication of diaphragmatic hernia in children is its strangulation. In newborns, it has a well-known peculiarity: flatulence of intestinal loops located in the chest cavity causes a sharp displacement of the heart and atelectasis. lung tissue. The cause of death of children in such cases is asphyxia. Circulatory disorders in the gastrointestinal tract or obstruction of its patency are usually not observed. It would be more correct to call such infringement asphyxial. In older children in the clinical picture strangulated hernia symptoms of gastrointestinal obstruction and respiratory failure are combined.

Treatment of diaphragmatic hernia in children

Tactics for diaphragmatic hernia should be active: all patients are subject to surgery, with the exception of children with limited protrusion of the right dome of the diaphragm, which is usually asymptomatic and does not threaten the child with complications. Emergency surgery performed when the diaphragm ruptures or in newborns when a hernia is strangulated. Planned surgeries in children, especially younger ones, it is more advisable to perform in specialized institutions where there is experience in using surgical methods treatment, modern pain management and caring for children after surgery, which often determines the final success of the operation.

Anesthesia- intratracheal anesthesia with nitrous oxide or ether with the use of short-term relaxants (ditylin, listenone).

Online access- transabdominal. With limited protrusions of the central zone of the dome, with right-sided localization of the hernia, as well as with esophageal hernias, transthoracic access can be used.

Operative and technical techniques depend on the type of diaphragmatic hernia in children. They should be simple and non-traumatic. The absence of adhesions allows the organs to be easily brought down into the abdominal cavity. For false hernias, air is introduced into the pleural cavity through a thick catheter, which promotes the reduction of intestinal loops. For small defects of the diaphragm, traumatic and anterior hernias, simple suturing of the hernial orifice with one or two rows of interrupted sutures is sufficient, without refreshing the hernial orifice. Use thick (No. 3-4) suture material (nylon or silk) to avoid muscle eruption. If there is a thinned area of the diaphragm large area it is strengthened either by plicating the hernial sac, suturing it with prefabricated sutures, tamponade of a weak area with a dense organ (liver, spleen), or by using alloplastic material (polyvinyl alcohol, nylon fabric or mesh). If there is a significant defect in the diaphragm, a number of techniques are used to help reduce the defect (resection of the ribs, moving the diaphragm 1-2 ribs higher). However, even then it is advisable to use alloplastic material, provided that it is isolated from the free pleural cavity with a peritoneal flap (to avoid prolonged pleurisy).

For a hiatal hernia in children, the operation consists of bringing the stomach and other displaced organs into the abdomen, excising the hernial sac or dissecting it in two circular fixation zones - in the area of the cardia of the stomach and along the line of the esophageal opening of the diaphragm. The main stage of the intervention is the movement of the esophagus from its bed near the spine to the anterior outer section of the esophageal ring, where the most favorable conditions are created for circular coverage of the esophagus by the muscle and prevention of relapse. During surgery for an esophageal hernia, you should not injure vagus nerves to avoid gastric atony and persistent postoperative vomiting. In some cases, the child’s abdominal cavity is underdeveloped and the reduced organs do not fit into it. Then stitching abdominal wall divided into two stages: first, only the skin is sutured, after a week or later, the abdominal wall is sutured in layers.

Results of treatment of diaphragmatic hernia in children

A study of the results of operations for diaphragmatic hernias in children shows that the surgeon’s active tactics are correct: children after surgery develop normally, catching up and even ahead of their peers.

The article was prepared and edited by: surgeon

Pathologies of the development of the peritoneal muscles in utero are often diagnosed as diaphragmatic hernia in newborns. With this disease, the child is subject to urgent surgery, as organ displacement occurs digestive tract into the chest cavity, causing underdevelopment respiratory system and hearts.

Usually, a diaphragmatic hernia in newborns is detected immediately after birth, as well as during pregnancy at 22-24 weeks - the pathology can be seen using ultrasound.

Immediately after childbirth, you can suspect a diaphragmatic hernia based on the following symptoms:

general cyanosis (blueness) skin);
weak cry (due to insufficient expansion of the lungs);
uneven breathing with noises (reminiscent of intestinal rumbling);
pulsation decreases, attacks of apnea and asphyxia are possible;
the newborn's belly is sunken and the chest is convex
vomiting blood.

You can see some symptoms of diaphragmatic hernia in newborns in the photo.

On a note! If a baby is born with a bluish skin color, it is recommended to take a chest x-ray and assess the condition of the respiratory system organs and identify their possible underdevelopment.

The disease is divided into degrees. Diagnosis of diaphragm pathology can be based on type and severity. The first group includes a true hernia (this is a protrusion with a hernial sac formed connective tissues) and false (this is the movement of the peritoneal organs into the chest cavity without a hernial sac, which provokes chest tension).

Watch a video on how diaphragmatic hernia in newborns is treated.

Diaphragmatic hernias of two types are determined by the degree of severity: by the volume of organs that have moved into the chest and by the presence associated complications(pathologies of the development of the heart, lungs, problems with work gastrointestinal tract).

Sometimes during diagnostics a sliding and axial hernia esophageal opening. For such pathologies, treatment is determined individually.

On a note! If ultrasound does not clearly identify a diaphragmatic hernia in the fetus during pregnancy or the pathology does not worsen, treatment is postponed until the neonatal period, and the pregnant woman is placed on special control for observation. When congenital pathology obvious and disturbing further development baby, offer intrauterine treatment(surgery).

By individual indications An urgent termination of pregnancy may be prescribed.

Children say! - Why do you want to laugh so much?
- Dina heals my stomach by rail…

Causes of development of diaphragmatic hernia in infants

The disease may be asymptomatic in the first few weeks. And with the progression of some symptoms, it is imperative to find out the reasons for the underdevelopment that has occurred.

Usually the development of such pathology is associated with past infections during pregnancy, hereditary factors, lack of vitamins with unbalanced diet, chronic diseases the mother, as well as her bad habits.

Diaphragmatic hernia in newborns: treatment in stages

Treatment of diaphragmatic hernia in newborns is always surgical. It is noted that even after intervention, the child’s chances of recovery are 50%. The operation should be performed within the first 24 hours after the baby is born. During this period, the baby's intestines have not yet had time to fill with gases, so it will be easier to place him in the abdominal cavity. This treatment is considered jewelry, since all parts of the body are small and it is important not to damage them. In progress surgery artificial tissues are sewn in, which, having taken root, play the role of muscles that hold internal organs in the abdominal cavity. After the procedure, the newborn must be connected artificial ventilation lungs, and if necessary, a surfactant is administered. It helps the lungs complete their development.

On a note! As a result of late surgery, difficult reduction is possible digestive organs, sometimes they return to the chest, rupturing the artificial diaphragm. As a result, cardiac activity is disrupted and the child dies.

Prenatal treatment of the disease is possible. A puncture is made in the abdominal cavity, a special balloon is inserted into the baby's trachea and tissue is sewn in to prevent organs from penetrating from the peritoneum into the sternum. The further course of pregnancy is then monitored, and after birth the balloon is removed and the baby is placed in an incubator under ventilation.

Not excluded after intrauterine surgery. Treatment during pregnancy is resorted to only when there is a risk fatal outcome very large for a child after birth.

To maintain the vital capacity of the fetus before birth, it is possible to use medications that support normal development pregnancy, or strict bed rest is prescribed.

Complications of diaphragmatic hernia

In the postoperative period, the baby needs to pay a lot of attention, monitor the hernial protrusion (recurrence is possible), and also analyze general state baby. With positive dynamics, the child has a chance of full recovery and recovery. After prolonged mechanical ventilation, it is important to carefully reduce the need child's body in it and let the baby try to breathe on his own.

Children say! My daughter (3.5 years old) says today:
“To make boys like you, you need to do this,” and bares his shoulder.

As a consequence of the disease and its untimely treatment A rupture of the diaphragm may occur, and the death of the infant cannot be ruled out. For preventive purposes, it is recommended to monitor vital signs after surgery and monitor blood composition.

After undergoing surgery on the diaphragm in the neonatal period, severe colds and infectious diseases at the baby's. All signs of acute respiratory infections should be stopped immediately, because after compression of the respiratory system, pneumonia or bronchial asthma may develop.

In approximately 85% of cases of CDH there is a left-sided hernia, in 13% the hernia is located on the right, in 2% it is bilateral. Right-sided defects are associated with greater mortality (45–80%) due to the presence of the liver in the chest cavity.

High pulmonary resistance and reduced lung surface area for gas exchange lead to the clinical picture of PLH and possible inadequate oxygen delivery to organs and tissues. Hypoxemia and metabolic acidosis contribute to further vasospasm of the pulmonary vessels, forming a vicious circle.

Symptoms and signs of congenital diaphragmatic hernia in newborns

Usually the EDC clinic begins in the first minutes or hours after birth respiratory distress, scaphoid (flattened) belly, barrel-shaped rib cage, cyanosis. Sometimes on auscultation you can hear intestinal peristalsis in the chest.

Diagnosis of congenital diaphragmatic hernia in newborns

Prenatal diagnosis

Prenatally, CDH is diagnosed in 2/3 of cases. The main symptom is the presence of gastrointestinal organs in the chest cavity. The right-sided defect is less detectable because liver tissue is difficult to distinguish from lung tissue. In addition to fetal ultrasound, diagnostics include fetal MRI and genetic testing.

Laboratory diagnostics

Arterial blood gases.
Radiography.
It is advisable to perform an ultrasound in the first 24 hours of life to exclude other birth defects, first of all - hearts. Subsequently, ultrasound monitoring is necessary to determine the severity pulmonary hypertension and right-left bypass and determining the patient’s stability to make a decision about surgery.
Pulse oximetry.

Differential diagnosis

Differential diagnosis should include bronchogenic cysts, cystadenomatosis of the lung, bronchogenic sequestration, congenital lobar emphysema, pulmonary agenesis and eventration of the diaphragm

Treatment of congenital diaphragmatic hernia in newborns

Hemodynamic support

Maintain the required level of blood pressure (to begin with, you should maintain normal indicators for gestational age) with the help of adequate volume load and inotropic drugs (dopamine, dobutamin, adrenaline). Recommended initial volume infusion therapy(first 24 hours of life) - 40 ml/kg. If the pressure is pulmonary artery exceeds the systemic one and there is a right-to-left shunt through oval window, you should consider prescribing prostaglandin E1.

Sedation and analgesia

Adequate analgesia and sedation are mandatory. Routine administration of muscle relaxants to children with CDH is not recommended.

Nitric oxide inhalation

The use of iNO allows achieving selective vasodilation of pulmonary vessels. A randomized, double-blind, multicenter study of the effectiveness of nitric oxide in diaphragmatic hernia found that iNO did not reduce mortality or the incidence of ECMO. 43% of children in the control group and 48% in the iNO group died, 54% of children in the control group and 80% in the iNO group were transferred to ECMO (p = 0.043). A meta-analysis of the effectiveness of nitric oxide administration to full-term and near-term infants with DN, which also included a subgroup with CDH, showed that nitric oxide administration to these children may slightly worsen the prognosis. A consensus group of European experts recommends prescribing iNO in cases of obvious intracardiac right-to-left shunting, oxygenation index >20 and/or difference in post- and preductal saturation >10%. Many centers test the effectiveness of iNO with withdrawal if there is no response. A recent study of 1713 children with CDH in 33 hospitals showed that increasing the frequency of iNO use did not reduce the mortality of patients with CDH.

Extracorporeal membrane oxygenation

Unfortunately, the benefit of ECMO in this group of patients is questionable, and probably this procedure will be useful only for a limited number of patients with severe pulmonary hypoplasia in whom it is impossible to achieve adequate gas exchange or change the fetal circulation pattern.

Experimental treatment

In uncontrolled studies of the treatment of PLH in CDH, sildenafil was used in cases of iNO failure.

Surfactant. A retrospective analysis showed that surfactant therapy does not improve survival or reduce the incidence of ECMO and CLD in children with CDH. It is possible that surfactant treatment worsens the prognosis of CDH. Individual centers decide on the administration of surfactant individually based on gestational age and radiological findings.

Surgery

Currently, the following approach to surgical treatment has been formed:

no need for early intervention;
the operation is carried out after stabilization of hemodynamics and gas exchange.

Indicators of a patient's readiness for surgery are considered:

MAP is normal for gestational age;
preductal saturation 80-95% at FiO 2<50%;
lactate<3 ммоль/л;
diuresis >2 ml/kg/h;
pressure on the pulmonary artery is less than 50% of the systemic one;
dose reduction or discontinuation of inotropic drugs.

It should be noted that conclusions about the preference for later intervention are based on a large number of observational studies. The meta-analysis did not show a benefit for delayed intervention (possibly due to the small number of RCTs performed to date).

Antenatal surgery. There are two types of interventions - plastic surgery of the diaphragm and ligation or occlusion of the fetal trachea. Data from two RCTs of occlusion are conflicting. At present, antenatal intervention cannot be recommended routinely and further research is needed.

Outcomes and prognosis for congenital diaphragmatic hernia in newborns

Some children who have had CDH (usually the most severe cases) develop CLD.

Although some pediatric surgical centers describe a 40% survival rate for children with CDH, only 30% of children diagnosed prenatally survive to 1 year of age.

A newborn is more likely to survive if he is born in a facility that can provide the full range of neonatal intensive care, as well as in clinics that have the greatest operational experience. If possible, gestation should be extended to full term pregnancy. The method of delivery for patients with CDH is not of fundamental importance and should be dictated by the obstetric situation.