Pentade Cantrell: causes, treatment, prognosis

There are many developmental defects that are formed in utero. They can affect any organs and systems. The most dangerous are the heart, lungs, digestive system, kidneys and brain. Some of them are incompatible with life. Others are amenable to surgical treatment. One of the most complex evils of development is the Pentate of Cantrell. This disease is characterized by a number of violations of several organs at once. In some cases, one can find the incomplete pentad of Cantrell. Despite the severity of the defect, sometimes doctors are able to cope with the disease and restore the vital functions of the body.

Pentade Cantrell - what is this pathology?

This pathology refers to congenital heart disease and other organs. Based on the name, Cantrell's pentada includes 5 defects. Among them, the most dangerous is the violation of the cardiovascular system, which leads to changes in hemodynamics. Also this vice implies a terrible cosmetic defect. You can recognize it right away when the baby is born, as the heart of these newborns "bulges out". Cantrell's syndrome does not always consist of 5 defects. It happens that 1 or 2 violations are absent (tetrad, triad). In addition to heart disease, the disease includes another severe symptom - omphalocele. There are also changes in the anterior abdominal and thoracic wall, defect of the diaphragm.

Pentade Cantrell: causes of the disease

It is important to know that the disease is formed in the second trimester of pregnancy. During this period, all vital organs and systems are laid. As you know, at 14-18 weeks of pregnancy, a mesoderm is formed. Under the influence of adverse environmental factors and various infections of the mother during this period, some violations of embryogenesis may occur. One of these deviations may be the pentad of Cantrell. The exact cause of the disease was not established. Nevertheless, this defect is associated with chromosomal abnormalities. Among them, trisomy 18 and 13, Shereshevsky-Turner syndrome, X-linked pathologies. It is known that this defect is rare. The frequency of its appearance is 3-5 newborns per million. Less than 100 cases are described in the world. The disease affects boys more often.

Despite the fact that it was not possible to find out the main cause of the pathology yet, it is necessary to know about the risk factors leading to various violations of intrauterine development. Among them:

1. Exposure to harmful and toxic substances (heavy metal salts, narcotic and chemical compounds).
2. Radioactive irradiation.
3. Physical effects (injuries).
4. Stressful situations.
5. Smoking and alcoholic drinks.

Mechanism of hemodynamic disorders

In connection with the fact that with the pentad of Cantrell can be observed various heart defects, hemodynamic disturbances at the same time differ from each other. Most often, this disease is accompanied by a defect of the interventricular septum. The development of hemodynamic disorders depends on the magnitude of the defect. With significant defects, a large discharge of blood into the small circle occurs, thereby leading to pulmonary hypertension. At the same time, the left ventricle experiences an overload and is compensated hypertrophically. As a result, there is an increase in both the prostate and the LV.

Less common is the defect of the interatrial septum. Hemodynamic disturbances in it also depend on the size of the defect. With a large communication between the atria, the discharge of blood into the right part significantly increases, leading to hypertrophy and dilatation of the PP. Overload is observed in the left atrium.

The main manifestations of Cantrella pentada

As you know, the classic Cantrell syndrome includes 5 defects. Among them are the following violations:

• The omphalocele of the fetus. In another way, it is called the upper-umbilical abdominal wall defect. This disorder is characterized by the fact that the internal organs, normally located in the abdomen, gather into the umbilical hernial sac and come out.
• Breast wall defect. Most often it is characterized by the absence or bifurcation of the xiphoid process. As a result, the heart also moves forward.
• Absence of a part of the pericardium, which should adjoin the diaphragm.
• Anomalies of heart development. In almost all cases, ectopia is observed - an improper arrangement of the organ. With this defect, the heart may be in the abdominal cavity or neck. In addition to ectopia, various vices are also observed. In addition to VSD and ASD, atresia occurs tricuspid valve, pulmonary artery. In rare cases, a complete absence of the heart chamber is possible .
• Diaphragmatic hernia.

In addition to the fact that Cantrell's syndrome may be incomplete, its combination with other anomalies is also observed. Most often, it is the pathology of the CNS (cerebral hernia, hydrocephalus), the gastrointestinal tract (pathology of the gall bladder and spleen), the lack of fingers or limbs.

Clinical picture of the disease

Symptoms of the disease become apparent from the moment the baby is born. First of all, a significant cosmetic defect, in which the heart and organs of the abdominal cavity "bulge" outwards, manifests itself. In addition, in the first hours of life more terrible symptoms also join. Because of congenital heart defects, the newborn receives little oxygen. As a result, he has shortness of breath, cyanosis, retraction of the intercostal spaces and the lower part of the sternum. Most often these newborns need to be connected to the device of artificial ventilation. To suspect this disease, it is necessary to find out where the heart is located in order to confirm one of the main signs - the ectopia of the organ.

What is the diagnosis of the Cantrell pentada?

Diagnosis of Cantrell's syndrome is based primarily on external examination of the newborn. First, the doctor looks at the presence of an omphalocele - a defect of the abdominal and thoracic walls, and then determines where the heart is located. With the confirmation of ectopia, respiratory and hemodynamic disorders, the diagnosis is practically reliable. Auscultation and echocardioscopy are performed to determine the presence of heart defects. Ultrasound of the abdominal cavity is impractical, since the first thing to do is to remove defects.

Cantrella pentada treatment

Despite the fact that the pentad of Cantrell is a combination and a terrible vice, in some cases it can be coped with. This applies to patients whose hemodynamic disorders are compatible with life. The main treatment for a defect is surgical intervention. The operation consists of several stages and is considered one of the most complicated. The incision is made on the median line of the thoracic and abdominal wall. At the same time, surgeons should correct the cardiac defects and direct the digestive organs. Ectopic heart usually remains. In some cases, surgery for small VSD and ASD is delayed at a more appropriate age (up to 3 years).

Prevention of malformation

The main method of prevention is perinatal diagnosis. Usually the diagnosis of the pentad of Cantrell from the fetus can be made already on the 20th to 24th week of development. Most often, this conclusion is the reason for the termination of pregnancy. Primary prevention includes:

• Avoidance of harmful influences.
• Maintaining a healthy lifestyle.
• Avoidance of stressful situations, especially in the first half of pregnancy.

The prognosis for this disease is ambiguous, since it depends on the degree of heart damage. In some cases, doctors manage to save the child's life. Nevertheless, such children need constant care and supervision of specialists.