Gastroschisis

Gastroschisis is a defect in the development of the anterior abdominal wall, in which the organs of the abdominal cavity are equiped through the defect of the anterior abdominal wall, usually located to the right of the normally formed cord.

[1], [2], [3], [4], [5], [6]

Epidemiology

Gastroschisis occurs approximately with the same frequency in boys and girls with a slight predominance in the first, with a frequency of 3-4 per 10 000 live births. More than 70% of children are born prematurely and have prenatal hypotrophy.

[7], [8], [9]

Causes of the gastroschisis

Until now, no single factor has been identified that would unequivocally cause the development of gastroschisis. According to the hypothesis, premature involution of the right umbilical vein can lead to ischemia and (as a result) mesodermal to ectodermal defects, and disruption of the formation of the distal segment of the omphalosenterial artery - to the ischemia of the Laraumbilic region on the right and, accordingly, to the occurrence of a paraumbilical defect. The ischemic process in the structures supplied with the upper mesenteric artery may be the reason not only for the development of the defect of the anterior abdominal wall, but also for the impoverishment of the blood supply by this vessel, as a result, the intestinal wall can be resorbed with the formation of atresia, which explains the combination of gastroschisis with the developmental defects of the intestinal tube. Extremely rare in gastroschisis combined abnormalities of other organs and systems and chromosomal abnormalities.

[10], [11], [12], [13]

Symptoms of the gastroschisis

Ultrasound of pregnant women allows early detection of developmental defects - already at the 12-15th week of pregnancy. Determine the loops of the intestine, located outside the abdominal cavity. In the early diagnosis of gastroschisis, a woman in the future should be carefully and often surveyed: in the second trimester of pregnancy, ultrasound is carried out once a month, in the third trimester - weekly.

Manifestations of gastroschisis are obvious, and after the initial examination of the newborn, the diagnosis does not require any additional methods of investigation. Eventually, the loops of the small and large intestines, the stomach, less often the bottom of the bladder, the girls - the appendages and the uterus, the eggs in some cases - the testicles, if at the time of birth they do not descend into the scrotum. The liver is always in the abdominal cavity, formed incorrectly. The equatorial organs have a characteristic appearance: the stomach and intestine are dilated, atonic, the walls of the intestinal tube are infiltrated, the small and large intestines are located on a common mesentery having a narrow root - approximately 2-6 cm in diameter). All the sustained organs are covered with a layer.

Intestine with gastroschisis is somewhat shorter, its length is reduced in comparison with the norm by 10-25%. Amniotic fluid, being a chemical "compressor" for the serous membranes of the eutentered organs, causes their damage - the so-called chemical peritonitis. The color of the fibrin coating of theventional organs depends on the characteristics of the intrauterine environment: from dark red to yellowish-greenish. It must be remembered that this fibrin clot, as a rule, hides absolutely viable organs. The protocol of surgical examination of a newborn with gastroschisis includes ECHO-cardiography, neurosonography. To clarify the question of the mechanical patency of the intestinal tube in children with gastroschisis before surgery, it is necessary to carry out a high washing of the evolved colon - the presence of meconium in the large intestine testifies to the intestinal permeability.

[14], [15], [16]

Forms

Recently, the following working classification of gastroschisis has been adopted, which makes it possible to choose the optimal way of conducting pregnancy and childbirth, as well as surgical correction of the defect.

• A simple form of gastroschisis.
• Complicated form of gastroschisis - with or without visceral-abdominal disproportion.

Patients with a complicated form, as a rule, need stage surgical treatment.

[17], [18]

Treatment of the gastroschisis

Prehospital stage

To prevent the child's cooling, the exposed organs immediately after exposure are covered with a dry sterile cotton-gauze dressing or they are obstructed by organs in a sterile plastic bag and covered with a cotton-gauze dressing. Maintenance of body temperature is ensured by placing a newborn in a Kuvez with a temperature of 37 ° C and a humidity close to 100%. Immediately, you should put a permanent nasogastric or orogastric tube to prevent aspiration of gastric contents and for decompression of the stomach. The probe must remain open for the entire transport time. Intubation of the trachea should be performed only on individual indications.

Transportation of a patient with gastroschisis is performed by a resuscitator in a specialized reanimobile equipped with a kouvez, respiratory equipment and apparatus for monitoring the functions of vital organs. The transfer of a child to a surgical hospital should be carried out as soon as possible after birth.

Hospital Stage

Preoperative preparation

In a specialized hospital, the main tasks of preoperative preparation are the maintenance of the functions of vital organs, the replenishment of circulating blood, the reduction of hemoconcentration, the correction of hemorheological disorders, the prevention of child cooling, the reduction of the degree of visceral-abdominal disproportion by decompression of the gastrointestinal tract (gastric tube, high colon washing).

Preoperative preparation depends on the degree of decompensation of the patient's condition, but usually takes 2-3 hours. It is considered effective if the child's body temperature has risen above 36 ° С and laboratory parameters have improved (hematocrit decreased, acidosis was compensated).

Surgery

Gastroschisis is treated only surgically. At present, the methods of surgical treatment of gastroschisis can be divided into three groups.

Primary radical plastic of the anterior abdominal wall:

• traditional;
• Non-narcotic movement of the eutivated organs into the abdominal cavity (Bianchun procedure).

Delayed radical mastic of the anterior abdominal wall:

• siloplasty - plastic of the anterior abdominal wall:
• alloplasty - the use of patches made of synthetic and biological materials.

Staged treatment with concomitant intestinal obstruction - enterocollectomy with closure of the stoma and plastic of the anterior abdominal wall.

The choice of method of treatment depends on the degree of visceral-abdominal disproportion and the presence or absence of a combination of malformations of the intestinal tube.

Primary radical surgery is the most preferred method. It is performed in children who do not have a pronounced visceral-abdominal disproportion. The technique of therapy does not have any specific features and consists in immersing the equilibrated organs in the abdominal cavity, followed by layer-by-layer suturing of the operating wound. The cord remainder is recommended to leave the navel deformed.

In 2002, the English surgeon A. Bianchi proposed a method for the non-vesicular correction of the eutenteric gut, determined the strict indications and proved its advantages.

Indications

The following cases are subject to bezirkoznomu in the direction of the intestinal gut: with an isolated form of gastroschisis without visceral-abdominal disproportion and with a good bowel condition (in the absence of a dense fibrin case).

Benefits

No need for mechanical ventilation. Anesthesia, large volumes of infusion therapy, the passage on the digestive tract (self-chair on the 4th-6th day) is restored more quickly, the number of bed-days is reduced, it will be possible to obtain an excellent cosmetic result. The procedure is performed directly in the intensive care ward (in the conditions of the perinatal center or the resuscitation department of the surgical hospital).

[19], [20], [21], [22], [23]

Technique of operation

Tract for the umbilical remainder and immersion of the intestinal loops into the abdominal cavity without widening the defect of the anterior abdominal wall. On the edges of the defect, separate or intradermal sutures are applied.

In severe visceral abdominal disproportion, methods of using as a temporary receptacle for a part of the intestine that does not fit in the abdominal cavity, a teflon bag with a silastic coating, which is hemmed or fixed to the fascial margin of the defect of the anterior abdominal wall, is used. The bag is removed after 7-9 days. Performing the plastic of the anterior abdominal wall. In addition, for the treatment of gastroschisis with a high degree of visceral-abdominal imbalance, various patches of collagen-vikril tissue, xenopericardial plates treated with a dura mater are used. As these tissues cause: a violent proliferation of the child's own connective tissue, in most cases the abdominal wall defect is closed without the formation of a ventral hernia.

The treatment of children with gastroschisis and combined anomalies of the intestinal tube presents significant difficulties. With atresia of the intestine, a child with gastroschisis shows superposition of the double entero-or colostomy on the level of atresia, followed by its closure (on the 28th-30th day).

In the postoperative period, treatment is carried out in several directions: maintaining the functions of vital organs and systems, restoring the functions of the gastrointestinal tract. The program for postoperative management of patients includes the following activities.

• Resuscitative support (IVL, IT, antibacterial therapy, immunotherapy, complete parenteral nutrition from 4 days postoperative period).
• Decompression of the stomach and intestines.
• Stimulation of peristalsis.
• The beginning of enteral nutrition.
• Fermentotherapy and eubiotics.

Against the background of the therapy, the independent chair usually begins to retire for the child on the 4th-6th day after the operation, and by the 12th-15th day the passage through the gastrointestinal tract is restored completely, which allows to enter enteral nutrition and quickly bring it to physiological volume .

Complications

Complications of the postoperative period can be conditionally divided into three groups:

• thrombosis of mesentery vessels, bowel necrosis due to excessive increase of vitrobrenous pressure:
• adhesive intestinal obstruction against the background of non-restored functions of the gastrointestinal tract:
• secondary infection, necrotizing enterocolitis, sepsis.

[24], [25], [26]

Forecast

Survival of children with gastroschisis in large centers of neonatal surgery, where extensive experience in the treatment of this pathology has accumulated, is approaching 100%. Children do not lag behind their peers in psychomotor development, study at school on a general program or even under a program with in-depth study of subjects, are engaged in sports sections.

Thus, gastroscheis is an absolutely corrective defect, and rational restorative therapy leads in the overwhelming majority of cases to complete recovery and provides a high quality of life.

[27], [28], [29]