Gastroschism

Gastroschisis is a developmental defect, characterized by the formation of a defect of all layers of the anterior abdominal wall at the stage of intrauterine development with the release( exit) of contents of the abdominal cavity( stomach, intestine) into the external environment. A decade ago, gastroschisis was an absolute indication for abortion. At the present stage, the provision of highly specialized and highly skilled care for newborns, provides survival with a favorable prognosis for the further harmonious development of the child. This is due to the development of prenatal diagnostic capabilities, which allow you to correct the management of pregnancy and plan the tactics of delivery. New technologies, the development of specialized surgical, resuscitative care for newborns allow us to restore the vice and reduce the consequences of gastroschisis to a minimum.

Fetal gastroschisis - what is it?

Fetal gastroschisis is detected in prenatal ultrasound screening, which is performed during the period from 10 to 14 gestational week of pregnancy. With a higher probability to diagnose gastroschisis can be on the second diagnostic screening. This is due to the fact that in the early stages of the fetus, a physiological hernial protrusion is revealed, which, when the intrauterine development proceeds normally, passes without a trace.

The vice of gastroschisis in utero is revealed as the finding of intestinal loops outside the abdominal cavity. Observation and management of a pregnant woman in the second trimester does not differ from the conduct of a physiologically occurring pregnancy. In the third trimester, fetal monitoring is more thorough, as gastroschisis in children correlates with the development of fetal distress, intrauterine growth retardation and prematurity.

Premature delivery is indicated only for emergency indications from the pregnant or fetus. But, if possible, it is better to prolong the pregnancy until the fetus lungs mature. Even with the appearance of dilatation( expansion) of the intestinal loops, the tactician is relatively expectant about the delivery, since:

- not always the expansion of the intestinal loops in the fetus indicates its defeat;

- the reverse situation, that if there is a lesion of the intestinal wall, dilatation may not be present;

- at the time when the expansion of the intestine is detected, there may be an immature lung tissue that repeatedly worsens the prognosis for the disease and life of the newborn;

- at any time, a bowel resection may be required.

When intestinal dilatation is more than 16-18 mm, the incidence of atresia( intestinal laceration) and peritonitis caused by the release of meconium( original feces) into the abdominal cavity increases. The combination of gastroschisis with these pathologies increases many times the incidence and mortality of newborns.

A common opinion about the nature of delivery, in the presence of gastroschisis in the fetus, did not work out. Some authors say that conducting a cesarean section does not improve the prognosis of the disease, and births naturally occurring normally do not carry danger, explaining this by the fact that the body organs are covered with a film of fibrin, which protects the organs from damage.

Gastroschisis: causes of

The causes of gastroschisis development are not fully determined. This congenital anomaly of the abdominal wall is not associated with chromosomal pathologies or any precise teratogens( factors provoking fetal malformations).

Gastroschisis in children refers to fetopathies, that is, it is a congenital malformation of development, which arose from the 12th week of intrauterine life and manifested itself in the formation of an embryonic fissure. Among the causative factors of the pathology of intrauterine development are:

- somatic diseases of the future mother( heart diseases, hypo- and hypertensive states, anemia, chronic diseases of the respiratory system, kidney pathology, antiphospholipid syndrome, connective tissue pathology, endocrinopathies);

- complications of current pregnancy( threat of early miscarriage, severe toxicosis and / or gestosis, placental insufficiency);

- physical effects( high or low temperature, radiation);

- chemical pathogens( drugs, narcotic substances, alcohol, household and industrial chemicals, nicotine);

- biological factors( various infectious agents, their toxins);

- young mother's age( up to 25 years).

Gastroschisis in children occurs in boys more often. According to one of the theories of the pathogenesis of gastroschisis, under the influence of some pathogenic factor involution( reverse development) of the umbilical vein on the right occurs, a disruption of the formation of the segments of the umbilical artery, which leads to insufficient blood supply to the embryonic tissues that form the wall of the abdominal cavity. As a result, a slit fault appears on the right. Disturbance of the blood supply of the mesenteric artery may cause resorption( dissolution) of the intestinal wall, which causes the onset of atresia of the intestine( infection of the lumen).This causes a frequent combination of gastroschisis and malformations of the intestinal tube.

Gastroschisis: Symptoms and Signs

Gastroschisis manifests itself as an event( outward exit) of the intestine, stomach, rarely bladder, the boys have testicles, if they are not lowered into the scrotum, the girls have the uterus and appendages. Together with the loops of the intestine, the root of the mesentery emerges.

Thick and small intestine have one mesentery, intestines and stomach are dilated, atonic. The intestinal wall is edematous, infiltrated. All theventured organs are covered with a film of fibrin, its color depends on the nature of the amniotic waters. Under the influence of an aggressive environment of amniotic waters, chemical peritonitis develops( inflammation of the peritoneum).Intestine with gastroschisis shortened.

It should be remembered that under the layer of fibrin, viable organs are almost always determined. Before the operation, a high intestinal rinse is mandatory, if after that meconium begins to depart, there is no intestinal obstruction.

The vice of gastroschisis is not accompanied by an exit from the abdominal cavity of the liver.

The defect of the anterior abdominal wall in dimensions does not exceed 4 centimeters, always located to the right of the correctly formed cord.

Isolate a simple form of gastroschisis, while the size of the defect corresponds to the volume of the eutentered organs. The complicated form of gastroschisis is characterized by a large volume of the event in comparison with the size of the defect. In this case, there may be an infringement of organs with the rapid development of their necrosis.

The severity of a newborn's condition is usually due to the degree of prematurity or delay in intrauterine development.

Gastroschisis is rarely accompanied by a combination of anomalies in the development of other organs and systems, in very rare cases it can be: water scarcity or polyhydramnios, heart defects, urinary system, hydrocephalus, prune( abdominal wall abnormalities, bladder abnormalities, kidneys, ureters, Bilateral cryptorchidism).

Gastroschisis: Diagnosis

The diagnosis of gastroschisis is rarely erroneous. This developmental defect is diagnosed in utero. There is a certain stage in the diagnosis of fetal malformations:

is a general obstetric ultrasound. It is carried out by specialists of women's centers. At this stage, the "norm" or "deviation from the norm" is determined.

is a prenatal specialized ultrasound. It is carried out in medical genetic counseling centers, specialized ultrasound units of large maternity hospitals. At this stage, confirm the presence and nature of the pathology of fetal development, resolve all issues related to this.

- prenatal expert ultrasound. The study is conducted with the aim of establishing the final diagnosis - gastroschisis and determining the plan for further management.

After the instrumental examination, a consultation of physicians( perinatologists, obstetrician-gynecologists, pediatric surgeons, neonatologists, geneticists) is held, where the issue of continuation of this pregnancy, the tactics of delivery and the further management of a newborn is being decided collegially.

An important aspect in the formulation of the clinical diagnosis is the differential diagnosis of gastroschisis and omphalocele. Ophalocele - a serious abdominal wall defect, localized in the region of the umbilical ring, through the defect of which the organs of the abdominal cavity prolapse, while the hernial sac is the cord of the umbilical cord.

The vice of gastroschisis is characterized by the fact that the defect of the abdominal wall is located paraumbilikalno( next to the umbilical ring), the umbilical ring itself is not enlarged and the umbilical cord has the usual structure. Also, unlike omphalocele, with gastroschisis, the liver never leaves the defect and there is no hernial sac.

The protocol of examination for gastroschisis includes neurosonography and ECHO-graphy of the heart, to exclude the combined defects of intrauterine development.

Gastroschisis: treatment and prevention

The treatment of gastroschisis occurs in several stages:

- Pre-hospital stage. Immediately after birth, the child is transferred to the heated reanimation table. The equatorial organs are placed in a sterile package, the cotton-gauze dressing is superimposed on top. After a complex of primary measures, including resuscitation, if necessary, the child in the transport kuveze is transferred to the intensive care unit, where the most comfortable conditions are created to prevent intestinal drying out. The child is placed in a kuvez with humidity parameters close to 100% and an ambient temperature of 37 ° C.With gastroschisis, decompression of the stomach is necessarily performed by introducing an oro- or nasogastric tube, which must be constantly open.

- Preparation before operation. In the absence of shock and other acute conditions, preparation for surgery takes up to 4 hours. Vascular access is provided for prolonged parenteral feeding( bypassing the gastrointestinal tract), infusion therapy. Connection to the therapy of antibacterial drugs of a wide spectrum of action with gastroschisis is mandatory.

At this stage, the correction of acid-base state, infusion therapy, reduction of hemoconcentration( blood thickening).Continued prevention of hypothermia, decompression of the stomach, intestines.

- Surgical treatment. Three main techniques are used for the surgical treatment of gastroschisis.

The primary radical surgery is performed in the presence of a small volume of eutenteric organs. It consists in the fact that the correct direction of the organs and the sewing of the defect are made. Bianchi direction. The organs are corrected without widening the hole, by traction over the umbilical cord.

Delayed radical plastic surgery is performed when the volume of the large-sized organisms is large. It is carried out in two stages. The equatorial organs are placed in a silicone bag and suspended, gradually tied at the bottom of the bag and under the influence of negative intra-abdominal pressure the organs slowly enter the abdominal cavity. And then the suturing of the defect is carried out.

In the complicated course of gastroschisis with atresia, the application of a double entero-or colostomy is first performed, followed by their closure.

Complications arising in the postoperative period:

- obstruction of thrombus of mesenteric vessels, necrosis of the intestinal wall;

- adhesive intestinal obstruction;

- attachment of bacterial flora, development of necrotic enterocolitis, sepsis.

Given that the exact cause of the development of gastroschisis has not been established, then there is no specific prevention of this pathology. The general principles for the prevention of malformations include:

- pregnancy planning with the participation of medical genetic counseling, especially if the family already has cases of the birth of children with developmental abnormalities;

- the use of complex vitamin-mineral preparations, high-grade food, a mode of work and rest;

- prevention of the threat of early miscarriage, treatment of severe toxicosis;

- prevention of infectious diseases, especially in the early stages;

- excluding the use of alcohol, nicotine, narcotic drugs;

- refusal of treatment with drugs that have a teratogenic effect;

- elimination of exposure to pathogenic physical and chemical agents;

- elimination of stressful situations.

Gastroschisis in children is a serious developmental disorder diagnosed in utero and having characteristic clinical manifestations. At the present stage, positive results have been achieved in correcting this anomaly, which allows surviving children who were previously unpromising patients. An important point in the provision of highly specialized care for newborns with gastroschisis is the choice of the site of delivery. This should be a regional perinatal center, which includes resuscitation units, an intensive care unit for newborns and, most importantly, a department of pediatric surgery where surgical intervention will be performed directly. Timely and successful operation, provided that there are no combined severe defects and a smooth course of the postoperative period, allows to completely reverse the vice and enable the kids to develop harmoniously.