Hernia umbilical cord, or umbilical hernia(omphalocele) is a developmental defect in which, by the time the child is born, part of the abdominal organs is located outside the peritoneum - in the umbilical cord membranes, consisting of amnion, Wharton's jelly and primary primitive peritoneum (Fig. 149). Occurs in 1 in 5000-6000 newborns.
Rice. 149. Hernia of the umbilical cord (diagram).
The origin of the hernia is associated with a violation of the development of embryogenesis in the first weeks of intrauterine life. At this time, the abdominal cavity cannot accommodate the rapidly increasing intestinal loops. Located extraperitoneally, in the umbilical cord membranes, they go through a temporary stage of “physiological embryonic hernia”, and then, having completed the process of rotation, return to an increasing abdominal cavity. If, as a result of a violation of the process of intestinal rotation, underdevelopment of the abdominal cavity or a violation of the closure of the anterior abdominal wall Some organs remain in the umbilical cord membranes, and the child is born with a hernia of the umbilical cord.
Depending on the time of stopping the development of the anterior abdominal wall, two main types of umbilical hernias are distinguished - embryonic and fetal. In embryonic hernias, the liver does not have a Glissonian capsule and fuses with the membranes of the umbilical cord, which plays an important role during surgical intervention.
By clinical signs umbilical cord hernias are divided into in the following way:
- by hernia size: small - up to 5 cm, medium - up to 10 cm, large - more than 10 cm;
- according to the condition of the hernial membranes: uncomplicated (with unchanged membranes), complicated (rupture of membranes, purulent melting, intestinal fistulas).
Approximately 65% of children with umbilical cord hernias have associated developmental defects (heart, gastrointestinal tract, genitourinary system).
Clinical picture. When examining the child, it is discovered that part of the abdominal organs is located in the umbilical cord membranes. The hernial protrusion is located in the projection of the navel, above the defect of the anterior abdominal wall. The umbilical cord extends from the upper pole of the hernial protrusion. If developmental arrest occurs early, then a significant part of the liver and most of the intestines are located outside the abdominal cavity. In cases of later developmental delay, only part of the intestinal loops remains extraperitoneal. In practice, the contents of an embryonic hernia can be all organs except the rectum. With a defect in the diaphragm, ectopia of the heart is observed.
In the first hours after birth, the puovine membranes that form the hernial sac are shiny, transparent, and whitish. However, by the end of the first day they dry out, become cloudy, then become infected and covered with fibrinous deposits. If measures are not taken to prevent and treat infected membranes, peritonitis and sepsis may develop. When the membranes become thinner and rupture, eventration occurs.
Rice. 150. Treatment of umbilical cord hernia (diagram). A - radical surgery with layer-by-layer suturing of the abdominal wall tissues; b - Gross operation (suturing the skin over the unopened hernia membranes); c - alloplastic method; d - conservative treatment.
Treatment . Children with umbilical cord hernias begin to be treated immediately after diagnosis. Two treatment methods are used: surgical and conservative (Fig. 150).
Absolute contraindications To operative method treatments are congenital heart disease, severe prematurity and severe birth traumatic brain injury. For these children, conservative treatment is used, which consists of daily treatment of the umbilical cord membranes with 2% tincture of iodine and alcohol. After the coagulation crusts have separated and granulations have appeared, they switch to ointment dressings (Vishnevsky ointment, Shostakovsky balm). Antibiotics, physiotherapy (ultraviolet irradiation, electrophoresis with antibiotics), restorative and stimulating therapy are prescribed. The hernial sac is slowly covered with epithelium and, shrinking, becomes smaller. Complete epithelization is observed after 2-3 months.
A relative contraindication to surgery is large hernias with a clear discrepancy between them and the volume of the abdominal cavity, since the simultaneous reduction of internal organs into the underdeveloped abdominal cavity leads to a sharp increase in intra-abdominal pressure, limited mobility of the diaphragm and the development of a sharp respiratory failure, which often causes death.
However, in these children, if they were born full-term and without severe accompanying defects development and diseases, a two-stage Gross operation or closing the defect with alloplastic material can be successfully used. According to the method proposed by Gross, only the excess part of the umbilical cord is excised. The membranes are treated with 5% tincture of iodine, the skin is widely mobilized to the sides. The selected edge of the muscular aponeurotic defect is sutured to the membranes hernial sac perhaps closer to the upper pole. The skin is sutured over the hernial sac using interrupted silk sutures. To reduce tension, skin incisions are made in a checkerboard pattern. Muscular aponeurotic plasty is performed in the second stage in children older than one year.
When using alloplastic material, the hernial sac is covered with Dacron, Teflon, suturing it along the edge of the muscular aponeurotic defect. In the next few days postoperative period the capacity of the hernial sac is reduced with the help of collecting sutures, which makes it possible to gradually immerse the organs in the abdominal cavity and perform delayed plastic surgery of the anterior abdominal wall on the 7-10th day after the birth of the child.
Newborns with small and medium-sized hernias with a well-formed abdominal cavity are subject to radical surgical intervention.
Radical surgery is reduced to excision of the umbilical membranes, reduction of the viscera and plastic surgery of the anterior abdominal wall. The operation is performed under endotracheal anesthesia. The use of muscle relaxants is undesirable, since it is not possible to timely diagnose the increase in intra-abdominal pressure that occurs with large hernias.
At the border of the skin and umbilical cord membranes, a 0.25% solution of novocaine is injected and carefully, without opening the abdominal cavity, a bordering incision is made around the hernial protrusion. The contents of the hernial sac are inserted into the abdominal cavity. The membranes are gradually excised, starting from the upper pole, and at the same time plastic surgery of the anterior abdominal wall begins. The peritoneum is sutured together with the aponeurosis, and sometimes with the edge of the muscles, using interrupted sutures. A second row of sutures is placed on the skin. If there is tension when suturing the aponeurosis, two rows of sutures (U-shaped and interrupted silk) are applied to the skin. If the hernia membranes are tightly sealed to the liver, they are left, treated with tincture of iodine and immersed together with the liver into the abdominal cavity. This is necessary due to the fact that separation of the membranes from the liver, deprived of the Glissonian capsule, leads to damage to the organ and persistent bleeding.
After Gross's operation and conservative treatment, a ventral hernia is formed (Fig. 151). For prevention severe forms for ventral hernias, after the child is discharged from the hospital, wearing a bandage, massage, and gymnastics are necessary.
Rice. 151. Ventral hernia.
Ventral hernia is eliminated surgically in children older than one year. Preliminarily carried out functional test, allowing you to find out how much the increase in intra-abdominal pressure is compensated. Blood gases are examined before and after hernia repair. The simplest tests are to determine heart rate and breathing. If after reduction of the hernia they remain within normal limits, elimination of the hernia is possible. If increased heart rate and shortness of breath are observed, surgery is postponed until the abdominal cavity has reached sufficient volume and surgery is possible.
The most common are two methods of plastic surgery of the anterior abdominal wall for ventral hernias.
One of them involves muscular aponeurotic plastic surgery of the defect: flaps are cut out from the outer layers of the aponeurosis, which are sutured along the midline. During surgery using the Shilovtsev method, the deep-epidermalized skin flap covering the ventral hernia is displaced under the skin.
Inguinal hernias, which often accompany ventral hernias, are eliminated surgically 3-6 months after surgery for a ventral hernia.
Prognosis for umbilical cord hernias always serious. Mortality at surgical treatment umbilical cord hernia rate remains high and ranges from 30% for small hernias to 80% for large and complicated hernias. Children successfully operated on during the neonatal period subsequently grow and develop normally.
Isakov Yu. F. Pediatric surgery, 1983
An intestinal hernia is one of the most common pathological external or internal protrusions in the abdominal cavity. It can be congenital or acquired, and can take a person by surprise at any age. Ignoring a hernia is fraught with negative consequences, even death, so it is important to know the causes of its occurrence, promptly recognize the symptoms, carry out diagnosis and appropriate treatment.
Definition
An intestinal hernia is a pathological protrusion of the intestinal organs.
In the WHO classification of diseases, intestinal hernia is not listed separately, since the intestines can be included as component to such entities abdominal hernias, How:
- Umbilical(with underdevelopment of the umbilical ring).
- Epigastric or a hernia of the so-called white (midline) line of the abdomen at the junction of muscle groups.
- Inguinal(protrusion of intestines and peritoneum into the inguinal canal).
- Femoral(exit of the intestine beyond the boundaries of the anterior abdominal wall through the femoral canal).
- Postoperative(ventral), formed at the site of scarring of connective tissues that could not withstand muscle pressure. Due to the location of the previous surgical incision, postoperative protrusions are possible, as a result of which hernias of the colon and small intestine, duodenum, stomach.
The picture illustrates potential sites of intestinal hernia formations.
Causes
The etymology of intestinal hernia is due to genetic predisposition or congenital pathologies, anomalies of intrauterine development, for example, a physiological hernia of the fetal intestine with insufficient weak development of the umbilical ring.
The causes of intestinal hernia can also be acquired. Protrusions of this nature are caused by the following risk factors:
- excessive physical activity;
- lifting weights;
- trauma-damage to the anterior abdominal wall;
- overexertion with debilitating prolonged and frequent cough;
- straining with constipation;
- flatulence;
- chronic perivisceritis (adhesions in an ulcer between duodenum, stomach and neighboring organs);
- obesity (overweight);
- exhaustion, weakening of the body associated with old age;
- in women during pregnancy.
Video
The surgeon talks about the causes of hernias.
Symptoms
The main symptoms of an intestinal hernia are:
- round or oval protrusion;
- swelling in the corresponding areas of the abdominal cavity, formed during tension, straining. With a reducible hernia of this kind, the formation is hidden after reduction with the help of hands at the moment of muscle relaxation or when taking a lying position. With an unreducible hernia, such a protrusion does not disappear;
- aching or pressing pain in the area of the hernia protrusion, especially manifested when coughing or physical activity;
- dyspepsia (digestive disorders such as bloating, constipation, diarrhea);
- belching, nausea;
- urinary disturbance;
- sharp and intense pain syndrome caused by poor circulation due to strangulated hernia;
- acute intestinal obstruction caused by the accumulation of feces in the intestinal lumen due to hernial strangulation.
Internal hernias occur extremely rarely - when the abdominal organs penetrate into peculiar internal pockets, such as the omental bursa or the area of the cecum. Symptoms of internal pathology can be pain of a different nature and a fairly wide range (cramping and colicky, convulsive, dull, intolerable), as well as a feeling of fullness and distension, belching, nausea, vomiting, constipation (constipation). In case of strangulation of internal hernias, the symptoms are similar intestinal obstruction.
Note!
A strangulated hernia is life-threatening and requires immediate elimination, that is, surgical intervention! If you suspect a hernial strangulation, you should immediately call a doctor. Ignoring and delaying in such cases is fraught with acute necrosis (death) of compressed intestinal loops, peritonitis (inflammation of the peritoneum), acute intestinal obstruction, severe intoxication, renal-liver failure and death.
The figure shows a strangulated intestinal hernia: part of the intestine (loop) can become pinched, resulting in necrosis.
Diagnostics
Intestinal hernia formations are usually not difficult to diagnose. The doctor determines the symptoms and examines the formations, using the following non-instrumental examination methods:
- tapping (percussion);
- palpation (palpation);
- listening (auscultation).
To differentiate an intestinal hernia from other diseases, a symptom of a cough impulse is used: in the process of coughing, a movement of the formation is observed under the hand placed on the protrusion. On initial stage disease, when you strain or cough, you feel the release of the contents of the hernia. If a strangulation has already occurred, the symptom of a shock when coughing will be negative. If an intestinal loop forms in the “hernial sac,” a tympanic (drum-like) sound will be observed during percussion, and rumbling during auscultation.
To get a complete picture of the condition of the intestines, specialists resort to instrumental methods: contrast radiography Gastrointestinal tract (gastrointestinal tract) and ultrasound. When diagnosing internal hernias, which do not appear externally and are often discovered only during intra-abdominal operations, ultrasound, X-rays with barium contrast, and irrigoscopy (examination of the colon by introducing a special radiopaque agent and taking pictures) are also used. The main symptoms will be displacement of the small intestine and improper placement of intestinal loops.
Treatment of intestinal hernia formations in adults
The main method of getting rid of intestinal hernial protrusions in adults is surgery under general anesthesia, during which the “hernial sac” is dissected and the intestines are returned (reduced) to their original place in the abdominal cavity. After this, the “hernial orifice” (the openings through which the hernia protruded) undergo plastic surgery.
The likelihood of relapse depends precisely on the closure of the hernia gate. Today there are the following operational methods for this process:
- with tension, that is, using the patient’s own tissues;
- without tension, using a graft - a special synthetic mesh.
The second method is more effective, since the likelihood of relapse when used is minimal. Modern transplants are safe and made of high-quality materials that are not subsequently rejected by the patient’s body, so today preference is given to tension-free grafting, especially in cases of illness in the elderly, recurrent hernia, postoperative ventral hernia. The operation is carried out by implanting a mesh to strengthen the defects of the abdominal wall not only with its own tissues, but also with an allotransport agent, prolene mesh, which provides a more stable closure of the “hernial orifice”.
If strangulation has occurred and intestinal loops that have died due to circulatory disorders are found, the operation is complicated by intestinal resection - removal of the affected area and further stitching of the remaining living parts of the intestine, forming a passage (anastomosis) to restore patency in the intestinal lumen.
For internal hernias, doctors also gain access to organs through laparotomy - cutting the abdominal wall.
Another method of treating intestinal hernias with the least invasive intervention is endoscopy, which consists of introducing an endoscopic apparatus and necessary tools for carrying out certain manipulations from the inside through incisions-holes in the abdominal cavity with a diameter of approximately 1 cm. This method, in comparison with surgery with open access to the insides, has a number of advantages:
- an enlarged image from the endoscope is displayed on the screen, which ensures precise work by surgeons;
- shorter overall length of patient stay in hospital;
- minimal scarring and postoperative pain;
- reducing the possibility of complications;
- fastest recovery body.
Video
A practicing surgeon talks about the types of intestinal hernias, the danger of strangulation, as well as the tactics of surgical treatment.
In cases where surgical intervention contraindicated (if malignant formations, acute infections or heart disease, pregnancy, too early or, on the contrary, old age), conservative therapeutic methods, among which the most effective is considered to be wearing an anti-hernia bandage in the form of special swimming trunks or a belt. The essence of this method is to fix a pressure pad (a padded raised bandage) at the site of the protrusion, which prevents the subsequent development of a hernia.
Treatment of hernia in pregnant women
Most often, women expecting a child, especially over the age of 35, are susceptible to the following hernias:
- inguinal (provoked, for example, by frequent constipation);
- umbilical (due to weakening of the ligamentous apparatus and divergence of the umbilical ring);
- incisional (appearing at the site of a postoperative scar during a second pregnancy).
Risk factors such as:
- polyhydramnios;
- fruit size;
- bearing several fruits;
- poor development of the abdominal muscles;
- chronic cough;
- lifting weights;
Closed bandage for pregnant women with umbilical hernia
The most dangerous periods for the manifestation of pathology are the second and third trimesters, which is associated with fetal growth, enlargement of the uterus and increased intrauterine pressure, which the muscles cannot cope with. Painful attacks can be caused by fetal movement. The protrusion can pulsate and increase with coughing and pushing. Reduction of the hernia in the later stages is not possible.
The operation is contraindicated due to the harmful effects of anesthesia and medications on the development of the fetus, especially since, as a rule, after delivery, hernias in women disappear on their own. The main method of treating intestinal hernia during pregnancy is wearing a support bandage, special compression garments that weaken muscle tension. Expectant mothers are also prohibited from lifting heavy objects and physical exercise. During the birth process, when pushing, the doctor usually holds the hernia with his hand to avoid strangulation.
The bandage should be used by pregnant women only after consulting a specialist, because proper fixation requires certain skills. Otherwise, incorrectly wearing the bandage can cause pathologies in the development of the fetus or its incorrect intrauterine placement.
Intestinal hernias are not an indication for delivery by cesarean section. It can be prescribed to patients in cases of inguinal hernia large in size, when there are complications, other associated anomalies and pathologies, for example, due to improper placement of the fetus.
Omphalocele in the fetus
A hernia of the anterior abdominal wall at the site of the umbilical ring can occur in the fetus while still in the mother’s womb and is called an omphalocele. Boys are more often affected by this defect.
Among the factors in the formation of this hernial pathology in the fetus are:
- use of medications during pregnancy;
- smoking expectant mother;
- woman's age over 35 years;
- the presence of other intrauterine developmental defects.
Omphalocele is diagnosed using prenatal ultrasound, Doppler color mapping, showing that the umbilical cord is directly attached to the “hernial sac”. This pathology of fetal development is dangerous because it often appears in combination with other chromosomal abnormalities and defects, and may be part of a serious disease or syndrome. For this reason, termination of pregnancy is often indicated for omphalocele. In cases of continued pregnancy, special ultrasound monitoring of fetal development should be carried out. The choice of delivery method for omphalocele is determined by the size of the hernia. The child's subsequent surgery is usually performed within the first 24 hours of his life. Possible postoperative complications(sepsis, intestinal obstruction, hernia of the anterior abdominal wall).
Diagnosis of an isolated (without accompanying defects and anomalies) omphalocele is not an indication for termination of pregnancy.
Treatment of intestinal hernia in a child
Intestinal hernias in children (inguinal or umbilical) are most often congenital or appear shortly after birth. Children's umbilical hernias are determined by straining, screaming, and restless behavior of the child. They are easy to reduce and rarely become pinched. For this reason, it is worth holding off on surgery in newborns in cases where the hernia is easily reducible and does not cause any discomfort to the child in terms of pain, digestion and urination. In addition, this pathology can resolve itself within up to 5 years with the reduction and closure of the umbilical ring.
Treatment methods for umbilical hernia pathology in children under 5 years of age are conservative: massage, special physiotherapy. At an older age, if the hernia does not disappear, surgical intervention is indicated. It should not be postponed, since over time the pathology will develop further, and pinching and inflammation may occur, which will reduce the effect of the operation. The possibilities of hernia repair and laparoscopy in case of prolonged delay will be reduced to nothing.
Note!
Be careful when using adhesive bandages for the umbilical ring on newborns, since their skin is very vulnerable and can become infected.
Also, mainly among boys, an indirect inguinal hernia is common, often accompanied by testicular retention in inguinal canal or abdominal cavity. The protrusion appears in the groin area when straining or the child cries; it is easily pushed back into the abdominal cavity in a supine position. In case of strangulation of an inguinal hernia, the child becomes restless due to severe pain, the muscles are tense, and the protrusion ceases to be reduced. Although the pain may decrease after a couple of hours, the child will remain lethargic and will also experience symptoms of intestinal obstruction. In such a situation, surgery is urgently needed. During the first 10 hours from the moment of infringement, it is possible to use conservative measures (administration of antispasmodics, warm bath, etc.).
Additional Information!
To prevent the occurrence of intestinal hernia, especially in cases of hereditary predisposition to the disease, you should regularly perform physical exercise, strengthening the abdominal muscles, especially the oblique abdominal muscles. And also avoid situations that can significantly increase intra-abdominal pressure and weaken the abdominal wall, excessive physical overload, control your weight and digestion processes.
IN at different ages treatment of intestinal hernial pathologies, which are an integral part of various abdominal hernias, has its own characteristics. If you notice their symptoms, you should immediately consult a doctor for diagnosis and treatment, since in case of strangulation, an intestinal hernia can turn into a time bomb.
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During the development of the embryo, between the 6th and 10th weeks, the intestine increases in size, lengthens, and intestinal loops, which do not fit in the abdominal cavity, are pushed out of it through the umbilical ring at the site of attachment of the umbilical cord to the anterior abdominal wall. Located extraperitoneally, in the umbilical membranes, they go through a temporary stage of “physiological intestinal hernia”, and then, after completing the process of rotation, they return to the expanding abdominal cavity. If, as a result of a violation of the process of intestinal rotation, underdevelopment of the abdominal cavity or a violation of the closure of the abdominal wall, some organs remain in the umbilical cord membranes, the child is born with a hernia of the umbilical cord, or OMPHALOCELE. By the 11th week of pregnancy, normally, the intestinal loops return back into the abdominal cavity and the hernial protrusion disappears.
By the time you come for your 1st trimester screening at 11-13 weeks, in most cases this process is already complete, but it is worth remembering that until 12-13 weeks the fetal intestines can normally extend beyond the border of the abdominal cavity, and this would still be considered a physiological intestinal hernia. Protrusion of intestinal loops into the umbilical cord, which occurs during normal development, is usually accompanied by an increase in the diameter of its base by less than 7 mm.
In the case when the released intestinal contents are determined separately from the area where the umbilical cord enters the abdominal cavity and is not covered with a membrane, then such an echographic picture will be diagnostic sign already another anomaly in the development of the anterior abdominal wall - gastroschisis, even in the first trimester of pregnancy.
When calculating the risk of chromosomal abnormalities (CA) in software FMF at 11-13 weeks, although the detection of an omphalocele is indicated, it does not affect the result.
So. Omphalocele is a defect of the anterior abdominal wall in the area of the umbilical ring with the formation of a hernial sac with intraperitoneal contents, covered with an amnioperitoneal membrane. 
At the birth of a child, the omphalocele can be small, with only a small part of the intestinal loop, but it can also be large, up to 10 cm or more, including, in addition to the intestines, the liver and other organs. The incidence of small omphaloceles (up to 5 cm) is 1:5000 live births, large ones (10 cm or more) - 1:10000 live births.
Ultrasound diagnosis of omphalocele is based on the detection of a round or oval-shaped formation adjacent to the anterior abdominal wall with clear, even contours, filled with heterogeneous contents, to which the umbilical cord is directly attached.
Omphalocele can be isolated when there are no other changes or developmental abnormalities in the fetus.
However:
- In approximately 30% of cases, a combination of omphalocele with chromosomal abnormalities is observed. According to the results of E.V. Yudina, the frequency of CA with omphalocele in the fetus in the group of patients over 35 years old was 54.5%, and in patients under 35 years old - 28%. All chromosomal abnormalities were detected only in cases of combined omphaloceles; their frequency in this group was 46.4%;
- Some children are subsequently diagnosed with Beckwith-Wiedemann syndrome;
- More than half of children with omphalocele have defects in other organs and systems. Most often these are defects of the cardiovascular, genitourinary system, spine, diaphragmatic hernia, skeletal dysplasia.
- Remember that up to 12-13 weeks this may be normal and wait for the results of the screening examination.
- If, according to the screening results, the individual risk of CA is low, then repeat the ultrasound in 2-3 weeks. By this time, the physiological intestinal hernia should disappear.
- If the risk of CA is high, prenatal medical and genetic counseling is in any case indicated and fetal karyotyping is recommended. Or you can choose to terminate the pregnancy.
- If an omphalocele is detected again after 2-3 weeks, even with a low individual risk of CA in the first trimester, prenatal medical and genetic counseling is indicated, fetal karyotyping is recommended, as well as a thorough assessment of the ultrasound anatomy of the fetus at 18-20 weeks to exclude combined malformations.
The method of choice for delivery is often a planned cesarean section in the interests of the fetus, although the opinions of specialists on this issue are ambiguous.
After birth, with very small hernias (up to 1.5 cm), one can hope for independent closure of the umbilical ring in a few weeks, but if, upon reaching the age of three, the hernia has not closed on its own, a decision is made about surgery. Larger omphaloceles are usually operated on in the first day of life. The large size of the hernial sac requires a two-stage operation with gradual reduction of the organs into the abdominal cavity.
Kaiser et al followed the development of children operated on for omphalocele for 1-28 years and concluded that the presence isolated omphalocele is not an indication for termination of pregnancy, since the subsequent development and social adaptation of children proceeds without complications.
A few words about Beckwith-Wiedemann syndrome
This is a genetically determined disease that is characterized by a combination of macrosomia (rapid, hypertrophic growth), omphalocele, macroglossia (a large tongue that does not fit in the mouth), a predisposition to embryonic tumor formations and neonatal hypoglycemia (low blood sugar levels in a newborn). In addition, abnormal development of the ears and hemihyperplasia, when some parts of the body are larger on one side than the other, are common.
Such children are born quite large (about 4 kg) and in the first months of life they gain significant weight and height. At birth, attention is drawn to a large tongue protruding from the mouth and a defect of the anterior abdominal wall, mainly an omphalocele.
With timely correction of hypoglycemia, accelerated growth rates gradually decrease already in childhood, intellectual development is usually not affected, and adults usually do not experience medical problems associated with this disease. But early diagnosis Beckwith-Wiedemann syndrome is important, since such children have an increased risk of developing various tumors, primarily Wilms tumor (nephroblastoma) and hepatoblastoma. After 10 years of age, the risk of tumor formation decreases to the general population level.
It is possible to suspect Beckwith-Wiedemann syndrome during ultrasound, but only in the third trimester with a combination of macroglossia, omphalocele and macrosomia with a normal karyotype. In the journal Prenatal Diagnostics for 2003, a description of a case of prenatal ultrasound diagnosis of Beckwith-Wiedemann syndrome without omphalocele in a fetus in the third trimester of pregnancy was presented. The presence of this syndrome was suspected when ultrasound examination at 30-31 weeks of pregnancy based on macroglossia, microrhinia, visceromegaly and macrosomia. The diagnosis was confirmed after the birth of the child.
Umbilical hernia(fetal hernia, umbilical cord hernia, umbilical hernia) is a protrusion of abdominal organs through a midline defect at the base of the umbilicus.
Umbilical cord hernia- a developmental pathology in which, due to an early violation of organogenesis, the abdominal organs to a certain extent develop outside the body of the embryo. This is followed by deviations not only in the development of organs, but also in the formation of the abdominal cavity and chest. The hernial protrusion covers the hernial sac, which consists of the amnion on the outside and the peritoneum on the inside. And between them is mesenchyme (Jew's jelly).
In the case of an umbilical hernia, the protrusion of organs is covered with a thin membrane and can either be small (just a few loops of intestine) or contain most of the abdominal organs (liver, stomach, intestines). Main danger diseases - hypothermia, drying out of internal organs, dehydration due to water evaporation, infection of the peritoneum. Newborns with an umbilical hernia have a very high incidence of other congenital malformations. These include intestinal atresia, chromosomal abnormalities (particularly Down syndrome), and abnormalities of the heart and kidneys, which are important to identify and evaluate before surgical correction. The defect occurs in 1-2 patients out of 10,000 live newborns, without any significant differences by gender. Most babies with an umbilical hernia are born full term. Based on the working classification, umbilical hernia is divided into small, medium and large sizes. This depends on the hernial orifice (the size of the defect in the anterior abdominal wall) and the volume of the contents of the hernial sac. Also, the contents of small and medium hernias can be intestinal loops (in a small hernia - one or even several). The contents of a large umbilical hernia always consist not only of intestinal loops, but also of the liver.
According to the shape of the hernial protrusion, mushroom-shaped, spherical and hemispherical hernias are distinguished.
What provokes / Causes of Umbilical Hernia in Children:
Knowledge of pathogenesis and ethology umbilical hernia Quite controversial at the moment. It is believed that in the development of embryonic hernia of the umbilical cord, the main role belongs to two factors - underdevelopment of the anterior abdominal wall and impaired intestinal rotation in the first period of rotation. Impaired intestinal rotation manifests itself in the fact that temporary “physiological” umbilical hernia. It is formed in a 5-week embryo due to the fact that the growth rates of the intestine and abdominal cavity do not correspond. It disappears on its own by the 11th week of development.
As for the other theory, umbilical hernia- this is “persistence of the trunk stalk in the area that is normally occupied by somatopleura.” This opinion about the disruption of lateral mesodermal replacement of the peritoneum, amnion and stalk mesoderm explains many anomalies that are noted with an umbilical hernia (from Cantrell’s pentad to cloacal exstrophy).
Symptoms of Umbilical Hernia in Children:
Children suffering from an umbilical hernia often have multiple defects of other vital signs. important systems and organs. Chromosome abnormalities are also possible. The most frequently detected congenital orthopedic defects, heart defects, kidney malformations, etc. A small umbilical hernia is often accompanied by a patent vitelline duct.
The main symptoms of an umbilical hernia
A combination of umbilical hernia and trisomy on chromosomes 13 and 18, Down's disease is possible. An umbilical hernia is a component of Beckwith-Wiedemann syndrome, also called OMG syndrome (omphalocele-macroglossia-gigantism). This disease, in addition to umbilical hernia, is characterized by (this is reflected in the name) big tongue, often causing difficulty breathing, as well as gigantism, manifested in most cases in gigantism of parenchymal organs (pancreatic hyperplasia, hepatosplenomegaly). These symptoms may include hypoglycemia, which is very dangerous for newborns, and hyperinsulinism. More rarely, partial skeletal gigantism is detected.
In some cases, an umbilical hernia is a component of such severe anomalies as cloacal exstrophy and Cantrell's pentad. Treatment of these diseases is carried out with great difficulty and at the present stage has disappointing results in most clinics. It is the curability and severity of combined pathologies that determine the severity of a patient’s condition with an umbilical hernia and the prognosis for cure. At the same time, in the patient’s disability or thanatogenesis, the main role is often given not to the umbilical hernia itself, but to genetic syndromes and combined developmental defects. The above facts prove the need for the most early detection umbilical hernia already in the antenatal period for a timely solution to the question: to maintain or terminate the pregnancy.
How to recognize an umbilical hernia?
Visualization of an umbilical hernia is possible during ultrasound as early as the 14th week of pregnancy. A mother's alpha-fetoprotein (AFP) test is very informative. In congenital malformations, its content is increased. If the AFP level increases, the fetus must be carefully examined to determine the presence of concomitant congenital malformations. If an umbilical hernia is detected along with genetic abnormalities or incurable developmental defects, future parents may be advised to terminate the pregnancy.
The birth of a child with a small or medium umbilical hernia can occur naturally if there are no other indications for a cesarean section. In the case of large GPCs, the method of childbirth is selected individually in each individual case. Caesarean section should only be performed if there is a risk of rupture thin shells hernias
Diagnosis of umbilical hernia in children:
Prenatal diagnosis of umbilical hernia
After the birth of a child, diagnosing an umbilical hernia, in most cases, does not seem difficult. However, in the case of a small umbilical hernia in maternity hospital During processing of the umbilical cord, mistakes may be made that will have severe consequences. Most often, with this type of anomaly, there are one or two loops of intestine in the hernial membranes, which means that the volume of the formation is small, and such an umbilical hernia may look like a thickened umbilical cord. If the doctor or midwife does not recognize a small umbilical hernia and places a crushing clamp or wall ligature on the border between the shadows of the umbilical cord and the skin, and the remainder of the umbilical cord is cut off, damage to the intestine may occur.
Therefore, in ambiguous cases (with umbilical cord vascular dysplasia, thick umbilical cord), it is important to remember the possible disease and apply a ligature no less than 10-15 cm from the skin edge. Such a newborn needs immediate transfer for examination to a surgical hospital.
X-ray examination for umbilical hernia
An x-ray examination in a lateral projection can confirm or exclude the diagnosis of small umbilical hernia. In case of an umbilical hernia outside the anterior abdominal wall, intestinal loops (gas bubbles) are diagnosed in the umbilical cord membranes. And if there is no communication between the umbilical cord membranes and the abdominal cavity, the integrity of the anterior abdominal wall will not be compromised on the x-ray.
Since with an umbilical hernia there are often associated concomitant malformations, in addition to radiography of the abdominal cavity in vertical position and chest organs, the mandatory protocol for examining the patient should include ultrasonography of the brain, retroperitoneum and abdominal cavity and ultrasound of the large vessels of the heart.
Treatment of umbilical hernia in children:
When providing first aid to a child for an umbilical hernia in the maternity hospital, the main attention should be paid to protecting the hernial sac from adverse external factors and maintaining body temperature. Patients with an umbilical hernia need emergency care.
The choice of treatment methods for an umbilical hernia depends on its size, the patient’s condition, and the capabilities of the hospital where treatment is carried out. It can be surgical or conservative and carried out in one or several stages.
Conservative treatment of umbilical hernia
IN last years Conservative treatment, as resuscitation support improves and resuscitation develops, is used extremely rarely. This happens when, for some reason, surgery needs to be postponed. This tactic can be used for huge hernias of the umbilical cord or when they are combined with multiple severe developmental anomalies. Most often, tanning solutions are used for these purposes, for example, merbromine, 5% potassium permanganate solution, povidone-iodine. Behind cord remnant the hernial sac is fixed in a vertical position over the patient. Several times a day, the hernia shell is treated with one of the above solutions to achieve the formation of a dense crust. A scar will gradually form under it, forming a large ventral hernia. But this method has many serious disadvantages: rupture and infection of the membranes, pronounced adhesions, a long period healing, etc. Therefore, this method can only be used in extraordinary cases.
Surgical treatment of umbilical hernia
Surgical treatment can be radical, involving layer-by-layer suturing of the abdominal wall following the immersion of organs into the abdominal cavity, or staged. The second option involves the gradual formation of the anterior abdominal wall. In this case, at intermediate stages, auto- or alloplastic materials are used.
Radical intervention is the operation of choice, which is performed when the ratio between the volume of the hernial formation and the capacity of the abdominal cavity (viscero-abdominal disproportion) is moderate. In this case, layer-by-layer suturing of the anterior abdominal wall does not cause a significant increase in intra-abdominal pressure. It becomes clear that radical surgery is performed for small and medium-sized umbilical hernias, and rarely for large umbilical hernias.
If a small umbilical hernia is combined with a vitelline duct, then radical intervention is supplemented by resection of the vitelline duct. It is important not to forget that the components of almost any umbilical hernia (a rare exception may be small hernias) are malrotation, a common mesentery of the large and small intestine. In the case of immersion of organs into the abdominal cavity, the small intestine should be in the right flank and center of the abdominal cavity, and the thick intestine should be moved to the left flank. After the end of the intraperitoneal stage of the operation, layer-by-layer suturing of the wound of the anterior abdominal wall is performed and a “cosmetic” navel is formed.
The greatest number of difficulties arise in the treatment of large umbilical hernias with a significant degree of viscero-abdominal disproportion. In this case, radical surgery is impossible due to possible sharp increase intra-abdominal pressure. Therefore, it is necessary to use different types of staged surgical treatment.
In 1948, Robert Gross from Boston described methods of staged surgical treatment of umbilical cord hernias big size. The first stage consists of removing the hernia membranes and immersing the organs (as far as possible) into the abdominal cavity. This is followed by wide separation of skin flaps of the abdominal wall (up to the lumbar region) and suturing of the skin with the formation of a ventral hernia. At the second stage, the ventral hernia is eliminated (this occurs at the age of 1-2 years). Nowadays, this technique is practically not used due to a large number of disadvantages, which include: large sizes of the ventral hernia, pronounced adhesions, lack of conditions for increasing the volume of the abdominal cavity - since almost all organs are located in the skin hernial sac. A rapid leap in the treatment of large umbilical hernias was made in 1967. that year when Schuster described a method of temporarily applying a plastic covering to reduce the size of a fascial defect.
Then, in 1969, Allen and Wrenn proposed the use of a single-layer silastic covering sutured to the edges of the fascial defect. Subsequently, with the help of manual compression, the volume of the hernia formation is gradually reduced, and this allows for delayed primary closure of the abdominal wall.
When, after the first stage of surgery, intestinal motility is restored, it empties and decreases in volume, the second will follow (most often after 3-14 days), which involves removal of the sac and radical plastic surgery of the anterior abdominal wall or the formation of a small ventral hernia. This method is still the main one in the treatment of this pathology.
Surgical technique for staged treatment of large umbilical hernias
The operation begins with an incision in the skin around the hernial formation. After making sure that it is impossible to immerse all the organs, a silicone bag with a silastic coating should be sutured to the muscular-aponeurotic edge of the defect of the anterior abdominal wall. They cover part of the contents of the hernia, which was placed in the abdominal cavity. The bag is tied over the organs and secured in a vertical position over the patient. As the organs from the sac spontaneously descend into the abdominal cavity, it is bandaged lower and lower (relative to the abdominal wall). This reduces its volume and allows some degree of compression. The second stage consists of removing the sac after 7-14 days and radical layer-by-layer suturing of the anterior abdominal wall and the formation of a small ventral hernia. In this case, the last stage of surgical intervention (liquidation of the ventral hernia and layer-by-layer suturing of the abdominal wall) is performed no earlier than 6 months.
Methods for the use of allotransplantation of synthetic or biological origin have been developed and are very successfully used, which are sutured into the fascial defect of the anterior abdominal wall as a patch in case of a pronounced degree of viscero-abdominal disproportion.
Postoperative management
At the beginning of the postoperative period, anesthesia, artificial ventilation, antibacterial therapy. Decisive moment treatment - throughout the entire period of healing of the abdominal wall and restoration of intestinal functions, exclusively parenteral nutrition. In children with concomitant severe anomalies during the postoperative period, it is necessary to determine a method for timely correction of these abnormalities. This process requires the participation of doctors of these specialties. Great attention should be paid to patients with Besquit-Wiedemann syndrome and those prone to severe hypoglycemia. Careful control of blood sugar levels makes it possible to prevent a serious condition and prevent the development of encephalopathy in patients.
Prognosis of umbilical hernia
All patients with an umbilical hernia who do not have fatal malformations of other organs and systems remain alive. But in the case of a combination of umbilical hernia with various pathologies timely diagnosis and cooperation with doctors of related specialties make it possible not only to cure children with severe congenital defects of the kidneys, heart, musculoskeletal system, central nervous system, but also to give them an acceptable quality of life. This can only be done in a multidisciplinary children's hospital, in which all specialists and services have extensive experience in caring for newborns with such a complex pathology. Dispensary monitoring of the patient’s condition should be carried out for several years until the completion of the rehabilitation course.
Prevention of umbilical hernia in children:
Specific prevention of the development of umbilical hernia has not been developed.
Which doctors should you contact if you have an umbilical hernia in children:
Pediatrician
Cardiologist
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During the development of the embryo, between the 6th and 10th weeks, the intestine increases in size, lengthens, and intestinal loops, which do not fit in the abdominal cavity, are pushed out of it through the umbilical ring at the site of attachment of the umbilical cord to the anterior abdominal wall. Located extraperitoneally, in the umbilical membranes, they go through a temporary stage of “physiological intestinal hernia”, and then, after completing the process of rotation, they return to the expanding abdominal cavity. If, as a result of a violation of the process of intestinal rotation, underdevelopment of the abdominal cavity or a violation of the closure of the abdominal wall, some organs remain in the umbilical cord membranes, the child is born with a hernia of the umbilical cord, or OMPHALOCELE. By the 11th week of pregnancy, normally, the intestinal loops return back into the abdominal cavity and the hernial protrusion disappears.
By the time you come for your 1st trimester screening at 11-13 weeks, in most cases this process is already complete, but it is worth remembering that until 12-13 weeks the fetal intestines can normally extend beyond the border of the abdominal cavity, and this would still be considered a physiological intestinal hernia. Protrusion of intestinal loops into the umbilical cord, which occurs during normal development, is usually accompanied by an increase in the diameter of its base by less than 7 mm.
In the case when the released intestinal contents are determined separately from the area where the umbilical cord enters the abdominal cavity and is not covered with a membrane, then such an echographic picture will be a diagnostic sign of another anomaly in the development of the anterior abdominal wall - gastroschisis, even in the first trimester of pregnancy.
When calculating the risk of chromosomal abnormalities (CA) in the FMF software at 11-13 weeks, the detection of omphalocele, although indicated, does not affect the result.
So. Omphalocele is a defect of the anterior abdominal wall in the area of the umbilical ring with the formation of a hernial sac with intraperitoneal contents, covered with an amnioperitoneal membrane.
At the birth of a child, the omphalocele can be small, with only a small part of the intestinal loop, but it can also be large, up to 10 cm or more, including, in addition to the intestines, the liver and other organs. The incidence of small omphaloceles (up to 5 cm) is 1:5000 live births, large ones (10 cm or more) - 1:10000 live births.
Ultrasound diagnosis of omphalocele is based on the detection of a round or oval-shaped formation adjacent to the anterior abdominal wall with clear, even contours, filled with heterogeneous contents, to which the umbilical cord is directly attached.
Omphalocele can be isolated when there are no other changes or developmental abnormalities in the fetus.
However:
- In approximately 30% of cases, a combination of omphalocele with chromosomal abnormalities is observed. According to the results of E.V. Yudina, the frequency of CA with omphalocele in the fetus in the group of patients over 35 years old was 54.5%, and in patients under 35 years old - 28%. All chromosomal abnormalities were detected only in cases of combined omphaloceles; their frequency in this group was 46.4%;
- Some children are subsequently diagnosed with Beckwith-Wiedemann syndrome;
- More than half of children with omphalocele have defects in other organs and systems. Most often these are defects of the cardiovascular, genitourinary system, spine, diaphragmatic hernia, skeletal dysplasia.
- Remember that up to 12-13 weeks this may be normal and wait for the results of the screening examination.
- If, according to the screening results, the individual risk of CA is low, then repeat the ultrasound in 2-3 weeks. By this time, the physiological intestinal hernia should disappear.
- If the risk of CA is high, prenatal medical and genetic counseling is in any case indicated and fetal karyotyping is recommended. Or you can choose to terminate the pregnancy.
- If an omphalocele is detected again after 2-3 weeks, even with a low individual risk of CA in the first trimester, prenatal medical and genetic counseling is indicated, fetal karyotyping is recommended, as well as a thorough assessment of the ultrasound anatomy of the fetus at 18-20 weeks to exclude combined malformations.
The method of choice for delivery is often a planned cesarean section in the interests of the fetus, although the opinions of specialists on this issue are ambiguous.
After birth, with very small hernias (up to 1.5 cm), one can hope for independent closure of the umbilical ring in a few weeks, but if, upon reaching the age of three, the hernia has not closed on its own, a decision is made about surgery. Larger omphaloceles are usually operated on in the first day of life. The large size of the hernial sac requires a two-stage operation with gradual reduction of the organs into the abdominal cavity.
Kaiser et al followed the development of children operated on for omphalocele for 1-28 years and concluded that the presence isolated omphalocele is not an indication for termination of pregnancy, since the subsequent development and social adaptation of children proceeds without complications.
A few words about Beckwith-Wiedemann syndrome
This is a genetically determined disease that is characterized by a combination of macrosomia (rapid, hypertrophic growth), omphalocele, macroglossia (a large tongue that does not fit in the mouth), a predisposition to embryonic tumor formations and neonatal hypoglycemia (low blood sugar levels in a newborn). In addition, abnormal development of the ears and hemihyperplasia, when some parts of the body are larger on one side than the other, are common.
Such children are born quite large (about 4 kg) and in the first months of life they gain significant weight and height. At birth, attention is drawn to a large tongue protruding from the mouth and a defect of the anterior abdominal wall, mainly an omphalocele.
With timely correction of hypoglycemia, accelerated growth rates gradually decrease in childhood, intellectual development is usually not affected, and adults usually do not experience medical problems associated with this disease. But early diagnosis of Beckwith-Wiedemann syndrome is important, since such children have an increased risk of developing various tumors, primarily Wilms tumor (nephroblastoma) and hepatoblastoma. After 10 years of age, the risk of tumor formation decreases to the general population level.
It is possible to suspect Beckwith-Wiedemann syndrome during ultrasound, but only in the third trimester with a combination of macroglossia, omphalocele and macrosomia with a normal karyotype. In the journal Prenatal Diagnostics for 2003, a description of a case of prenatal ultrasound diagnosis of Beckwith-Wiedemann syndrome without omphalocele in a fetus in the third trimester of pregnancy was presented. The presence of this syndrome was suspected during an ultrasound examination at 30-31 weeks of pregnancy on the basis of macroglossia, microrhinia, visceromegaly and macrosomia. The diagnosis was confirmed after the birth of the child.
