Omphalocele

Omphalocele (hernia of the umbilical cord, umbilical hernia, embryonic hernia) is the protrusion of the abdominal organs through the defect of the midline at the base of the navel.

Ophalocele is an abnormality of development, in which, as a result of early organogenesis, the organs of the abdominal cavity develop to some extent outside the trunk of the embryo, which entails not only the abnormal development of these organs, but also the defects in the formation of both the abdominal cavity and the thorax. The hernial protrusion is covered with a hernial sac, which is located outside the amnion, from the inside - from the peritoneum, with the mesenchyme (Varganov's jelly) between them.

In omphaloceles, the protrusion of the organs is covered with a thin membrane and can be small (only a few loops of the intestine) or may contain most of the organs of the abdominal cavity (intestine, stomach, liver). Immediate danger is the drying out of internal organs, hypothermia and dehydration due to evaporation of water, as well as infection of the peritoneum. In newborns with omphaloceles, there is a very high incidence of other congenital malformations, including intestinal atresia; chromosomal abnormalities, such as Down syndrome; and anomalies of the heart and kidneys, which should be identified and examined before surgical correction.

ICD-10 code

Q79.2. Exofalosis.

Epidemiology

The incidence of omphalocele defect is 1-2 per 10 000 live births, there are no special differences in gender. Most of the children with omphalocele are born full-blooded.

[1], [2], [3], [4], [5], [6], [7], [8]

What causes an omphalocele?

Representations about the ethology and pathogenesis of omphalocele are still contradictory. It is believed that in the genesis of the embryonic hernia of the umbilical cord, the main role is played by two factors - the violation of the rotation of the intestine in the first period of the turn and the underdevelopment of the anterior abdominal wall. Disruption of the bowel rotation is manifested in the form of the preservation of a temporary "physiological" umbilical hernia, which is formed in a 5-week embryo because of inconsistency in the rates of growth of the intestine and abdominal cavity and which itself disappears by the 11th week of development.

According to another theory, the omphalocele is "the persistence of the trunk stem in the area normally occupied by the somatopleurotic". This idea of the violation of lateral mesodermal replacement of the peritoneum, amnion and mesoderm of the stem explains the diversity of anomalies noted in omphaloceles - from cloacal exstrophy to the Cantrell pentada.

How is the omphalocele manifested?

In children with omphalocele, multiple combined vices of other vital organs and systems are often encountered, as well as chromosomal abnormalities. Most often these are congenital heart defects, malformations of the kidneys, orthopedic defects, etc. Omphalocele of small size is often combined with the uncontaminated yolk duct.

Perhaps a combination of omphalocele with Down's disease, trisomy on chromosomes 13 and 18.

Omphaloceles is a component of the Beckwith-Wiedemann syndrome, also called OMG syndrome (omphalocele-macroglossia-gigantism). For this syndrome, in addition to omphalocele, the presence of a large tongue, sometimes causing difficulty in breathing, and gigantism, most often realized in the gigantism of the parenchymal organs (hepatosplenomegaly, pancreatic hyperplasia), are characteristic, which may manifest as hyperinsulinism and hypoglycemia, especially dangerous in the period of the newborn. Less often they reveal the partial gigantism of the skeleton.

Omphaloceles sometimes is a component of such severe anomalies as Cantrell pentada and cloacal exstrophy, whose treatment presents enormous difficulties and still has disappointing results in most clinics. It is the severity of the combined lesions and their curability that determine the severity of the ballroom with omphaloceles and the prognosis, and in tanatogenesis or disability: the leading role often belongs not to omphalocele, but to co-developmental defects or genetic syndromes. All of the above dictates the need for early detection of omphalocele in the antenatal period for a timely decision on the issue of maintaining or aborting a pregnancy.

Classification

According to the working classification of omphalocele, depending on the size of the defect of the anterior abdominal wall (hernial portal) and the volume of the contents of the hernial sac, small, medium and large omphalocele are isolated. The contents of small and medium hernias are talcous intestinal loops (in small - one or more). A large omphalocele always contains not only intestinal loops, but also the liver.

In the form of a hernial protrusion, hemispherical, spherical and mushroom-shaped hernias are distinguished.

[9], [10], [11], [12], [13]

How to recognize an omphalocele?

Visualization of omphalocele is possible with ultrasound from the 14th week of pregnancy. Very informative test for the maintenance of the mother of alpha-fetoprotein (AFP), its content is increased in congenital malformations. In this case (with an increase in the number of AFP), it is necessary to carefully examine the fetus for the presence of combined congenital malformations. If omphalocele is detected in combination with non-curable developmental anomalies or genetic abnormalities, future abortion can be recommended for future parents.

Birth of children with a small or medium omphalocele can occur naturally, unless there are other indications for conducting cesarean section. At large COD, the method of delivery is chosen individually in each specific case. Usually, it is advisable to perform a cesarean section in connection with the danger of rupture of the thin membranes of a hernia.

Prenatal diagnosis of ophalocele

Diagnosis of omphalocele after the birth of a child, as a rule, does not cause difficulties. However, with an omphalocel small in umbilical cord processing, mistakes with severe consequences can be made in the maternity hospital. Usually in the hernial membranes with this type of anomaly there is one or two loops of the intestine, i.e. The volume of education is small, and such an omphalocele often looks like a thickened umbilical cord. If the doctor or midwife did not recognize the small omphalocele and the crushing terminal or ligature was placed on the border between the shadows of the umbilical cord and the skin, and the umbilical cord cut off, the intestinal wall may be damaged. Therefore, in doubtful cases (with a thick umbilical cord, umbilical cord dysplasia), it is important to remember about small size ophalocele and apply a ligature at a distance of at least 10-15 cm from the skin margin. Such a newborn needs immediate transfer to a surgical hospital for examination. To confirm or to exclude the diagnosis of an omphalocele of small sizes allows an x-ray examination in the lateral projection. In case of an omphalocel outside the anterior abdominal wall, intestinal loops (gas bubbles) are defined in the umbilical shells, whereas in the absence of communication between the abdominal cavity and the umbilical cord, the integrity of the anterior abdominal wall on the radiograph is not compromised. Considering the fact that with ombhalocel infection are often associated developmental anomalies, the ultrasonography of the brain, abdominal cavity and retroperitoneal space, as well as ultrasound of the heart of large vessels, is an obligatory protocol for examining the patient, in addition to radiographing the chest and abdominal organs in an upright position.

Treatment of omphalocele

At the first aid to the child with omphaloceles in the maternity hospital, the main attention should be paid to maintaining the temperature of his body, protecting the hernial sac from adverse external influences. Patients with omphalocele need emergency help.

The choice of method of treatment of omphalocele depends on the size of the hernia, the condition of the patient and the capacity of the hospital where this treatment takes place. It can be conservative or surgical and can be performed in one or more stages.

Conservative treatment of omphalocele

Conservative treatment in recent years as the development of resuscitation and improvement of resuscitation support is used in extremely limited cases, when for some reason they suggest postponing surgical intervention. Such tactics can be used for huge hernias of the umbilical cord, or their combination with multiple severe developmental malformations. Most often for these purposes, tanning solutions such as povidone-iodine, merbromin, 5% potassium permanganate solution are used. The hernial sac for the umbilical remnant is fixed over the patient in an upright position, the hernial membranes are treated several times a day by one of the listed solutions, achieving the formation of a dense crust, under which the scar gradually forms, forming a large ventral hernia. However, this method has many serious shortcomings (infection of the membranes, their rupture, a long healing period, a pronounced adhesion process, etc.), so it should be used only in extraordinary cases.

[14], [15], [16], [17], [18], [19], [20], [21]

Surgical treatment of omphalocele

Surgical treatment can be radical (layer-by-layer suturing of all layers of the abdominal wall after immersion of organs in the abdominal cavity) or stage. The second option involves the gradual formation of the anterior abdominal wall using the auto- or alloplastic materials in the intermediate stages.

Radical intervention is a choice operation performed when visceral-abdominal disproportion (the ratio between hernia volume and abdominal cavity volume) is moderately expressed, and layer-wise suturing of the anterior abdominal wall does not cause a significant increase in intra-abdominal pressure. Correspondingly, a radical operation is usually performed with an omphalocel of small and medium size, less often with a large omphalocele.

If an omphalocele of small size is combined with the vitelline duct, radical intervention is supplemented with resection of the yolk duct. It should be remembered that the component of almost any omphalocele, with rare exceptions for small hernias, is malrotation, a common mesentery of the small and large intestine, so when the organs are immersed in the abdominal cavity the large intestine should move to the left flank, and the thin one should be in the right flank and center abdominal cavity. After the completion of the intraperitoneal stage, the operations are performed by layer-wise suturing the wound of the anterior abdominal wall with the formation of a "cosmetic" umbilicus.

The greatest difficulties arise in the treatment of large-sized omphalocele with a significant degree of visceral-abdominal disproportion, when radical surgery is impossible due to a sharp increase in intra-abdominal pressure. In such cases it is necessary to apply different types of stage surgical treatment.

In 1964, Robert Gross from Boston described the method of stage surgical treatment of large hernias of the umbilical cord. The first stage consisted in removing the membranes of the hernia, immersing the organs, as far as possible, into the abdominal cavity by wide separation of the skin flaps of the abdominal wall right up to the lumbar region and suturing the skin with the formation of the ventral hernia. The second stage was the elimination of the ventral hernia (at the age of L-2 years). At present, this technique is practically not used, because it has many disadvantages (pronounced adhesion process, large dimensions of the ventral hernia, lack of conditions for increasing the volume of the abdominal cavity, since almost all organs are in the cutaneous hernial sac).

A rapid leap in the treatment of large omphalocele was made in 1967, when Schuster described the method of temporary application of a plastic coating to reduce the size of a fascial defect.

Then in 1969 Allen and Wrenn proposed the use of a single-layer silastic coating that was sutured to the edges of the fascial defect, followed by a gradual decrease in the volume of the hernial formation with manual compression, which allows a delayed primary closure of the barn wall. Once the intestinal motility is restored after the first stage of the intervention, it will empty and decrease in volume, followed by the second - usually in 3-14 days - removal of the sac and radical plastic of the anterior abdominal wall or the formation of a small ventral hernia. This method remains the main one in the treatment of this pathology and at present

The technique of surgery for the treatment of large omphalocele. The operation begins with a cut of the skin around the hernial formation. Convinced of the impossibility of immersing all organs, a silicone bag with a silastic coating is sewn to the muscular aponeurotic edge of the defect of the anterior abdominal wall. This bag covers that part of the contents of the hernia that could be placed in the abdominal cavity. The bag is tied over the organs, fixed over the patient in an upright position. As the organs from the bag spontaneously descend into the abdominal cavity, the bag is bandaged lower and lower (relative to the abdominal wall), reducing its volume, while allowing some degree of compression. The second stage consists in removing the sac after 7-14 days) and radical layer-by-layer suturing of the anterior abdominal wall in the formation of a small ventral hernia. In this case, the last stage of surgical treatment (elimination of the ventral hernia with layer-by-layer closure of the abdominal wall) is performed at the age of 6 months.

There are methods of applying allotransplantation of synthetic or biological origin that are sewn into the fascial defect of the anterior abdominal wall in the form of a patch with a pronounced degree of viscerabdominal disproportion.

Postoperative management

In the early postoperative period, artificial ventilation of the lungs, anesthesia, antibacterial therapy. The decisive component of the treatment is total parenteral nutrition throughout the entire period of healing of the abdominal wall and restoration of the intestinal functions. Children with combined severe anomalies in the postoperative period should resolve the issue of timely correction of these anomalies, which requires participation in the treatment of doctors of these specialties. Particular attention should be paid to patients with Besquit-Wiedemann syndrome, prone to severe hypoglycemia. Thorough control - keeping blood sugar prevents this condition and prevents the development of encephalopathy in such patients.

Prognosis of omphalocele

All patients with omphaloceles who do not have the lethal malformations of other organs and systems survive. However, when omphalocele is combined with various anomalies, their timely diagnosis, as well as cooperation with physicians of other specialties, allow not only curing children with severe congenital heart and kidney defects. Central nervous system, musculoskeletal system, but to provide them with an acceptable quality of life, which is possible only in a multidisciplinary pediatric hospital, all specialists and services of which have extensive experience in nursing newborns with this complex pathology. Clinical follow-up of patients should be carried out until the completion of rehabilitation for several years.