• Wednesday April 8,2020

gastroschisis

Gastroschisis is a malformation of the child's abdominal wall, which already develops in the womb. Due to unexplained causes, the abdominal wall splits and internal organs penetrate outside. Therapy of the disease must be done immediately after birth.

What is gastroschisis?

By ultrasound examination (sonography), the gastroschisis can be detected with great certainty (90%) already from the 16th week of pregnancy, sometimes even earlier.
© Gorodenkoff - stock.adobe.com

Gastroschisis is an abdominal wall defect that develops before birth (prenatal). The term comes from the Greek of gastro = stomach, stomach and s-chismà = split and is therefore called abdominal cleft.

In gastroschisis, even in early pregnancy, a gap of about 2-3 cm forms in the abdominal wall of the fetus, usually to the right of the navel, through which internal organs penetrate to the outside. In most cases, the intestine is pushed through the abdominal opening and lies in the amniotic fluid. But other organs, such as the liver or stomach, can also fall through the cleft from the abdomen.

Because the intestine then floats freely in the amniotic fluid and experiences no limitations through the abdominal wall, it does not develop normally. The intestinal loops dilate and the organ becomes bigger than it should be. In addition, it can lead to torsion of the intestine and consequently to circulatory disorders, which in the worst case tissue dies. Gastroschisis is rare, but has increased in recent years.

causes

There is no clear explanation for the origin of the gastroschisis. There are different theories. An explanatory approach assumes that the right umbilical vein, a vessel that is present only at the beginning of pregnancy and regresses later, is responsible for the disease. The task of this vein is the nutrition of the abdominal wall. If it forms too early, the abdominal wall is underserved, tissue dies and the defect develops.

Since about the same time the growth of the intestine begins, this expresses itself through the opening of the body. This thesis would also explain why gastroschisis usually occurs right next to the navel. Another theory suggests that the occlusion of a right-sided artery leads to a type of tissue infarction and causes cleavage.

A third hypothesis assumes that a disorder causes vascular malformations and thus does not close the abdominal wall. Lastly, there is still the opinion that the gastroschisis can be caused by a rupture of the membrane around the umbilical cord.

Symptoms, complaints & signs

The gastroschisis manifests itself through an abdominal cleft, which is detected immediately during and after the birth of the child. Essentially, parts of the intestine exit from this abdominal wall. This can be both parts of the large and small intestines. Before birth, the gastroschisis can be detected by ultrasound.

While the intestine is still protected from birth, it leads to severe and sometimes fatal complications. The bowel loops located outside the abdomen are unprotected and therefore very susceptible to infection. The infections easily develop into peritonitis, which often takes a very serious course. Furthermore, a threatening bowel inflammation can develop, which leads to the death of parts of the intestine.

The outer intestinal loops also store fluid and therefore appear swollen. Through the formation of fibrin also intestinal parts can stick together. Finally, an intestinal obstruction can occur in which large parts of the intestine die off. Without treatment, gastroschisis is fatal in the newborn due to the complications mentioned.

However, the disease is easy to treat surgically. The healing process can take several weeks or months. As a rule, however, the gastroschisis heals completely. In some cases, however, there are accompanying malformations such as narrowing or atresia in the intestine, which must be treated.

Diagnosis & History

By ultrasound examination (sonography), the gastroschisis can be detected with great certainty (90%) already from the 16th week of pregnancy, sometimes even earlier. The size of the defect, how much of the intestine or which other organs have escaped from the abdomen, can be easily recognized by sonography.

If gastroschisis is suspected due to the ultrasound findings, an amniocentesis examination is often carried out for further clarification. An increased AFP value (AFP is a protein) in the amniotic fluid may be another indication of the condition, but is not considered as evidence. Due to the defect in the abdominal wall, the internal organs, especially parts of the intestine, fall into the amniotic sac and swim freely in the amniotic fluid.

Due to lack of limitation, the intestinal loops grow strongly and are swollen (edematous). If the intestine is twisted, it can lead to circulatory disorders and thus to the death of intestinal tissue. In addition, the amniotic fluid is burdened by the excretions of the fetus.

In specialized clinics, in rare cases, if the concentration of waste is too high, the amniotic fluid is replaced. The gastroschisis must be closely monitored during pregnancy. It always keeps both the maturity of the child in mind and the extent of organ damage.

complications

Gastroschisis can in the worst case lead to death of the child if the illness is not treated directly after birth. In most cases, however, a very early diagnosis is possible, so that the treatment can be started immediately after birth. Thus, consequential damage and further complications can be avoided.

If there is a twisting of the intestine, the intestinal tissue can die off, as it is no longer properly supplied with blood. Likewise, other organs can be damaged. The damage depends strongly on the severity of the gastroschisis. As a rule, regular check-ups are necessary to permanently monitor the damage.

Treatment usually involves surgery. In this procedure, the intestine is turned back, so there are no complications and not to the death of the tissue. Similarly, other damage to organs may need to be investigated and treated. In most cases, the child will experience a positive course of illness if treatment is given immediately after birth. Further complications do not occur.

When should you go to the doctor?

Expectant mothers should in principle participate in offered pregnancy examinations. The medical controls can detect a variety of irregularities or diseases of the unborn child. In the second trimester the gastroschisis can already be diagnosed by a gynecologist. Therefore, it is advisable, in particular, to take advantage of the possibilities of ultrasound examinations from this point on. If an in-patient childbirth is performed, routine examinations of mother and child are carried out directly after childbirth.

Intervention by relatives is therefore in most cases no longer necessary at this time. Often, due to the detected Gastroschisis, a caesarean section, so that takes place in the clinic immediate medical care of the child. If an unscheduled home birth takes place, it is necessary to appoint a doctor for a home visit or to inform the ambulance service at birth. Even if the condition was not noticed during pregnancy, a doctor should be consulted immediately after a home birth.

In general, a pregnant woman should consult a doctor as soon as she has a vague feeling that something is wrong with her adolescent child. Apart from participating in the follow-up examinations, it is important that a consultation of the physician takes place in case of perceived irregularities, a general feeling of illness or other abnormalities.

Treatment & Therapy

The ability to treat the gastroschisis begins after birth. Usually a caesarean section (section) is advised, but in practice there has not been an obvious advantage over normal vaginal delivery. The only possible therapy for the defect is an operative procedure, which should take place shortly after birth, at the latest 18 hours later. The first supply is that a possibly twisted intestine is turned back (de-doped).

The infant is put on its side so that no vessels are pinched off. Furthermore, the child's body is packed in a sterile plastic wrap, so that the external organs do not dry out and the baby stays warm. In order to be able to supply the body with medication and food, a stomach tube and a venous access are placed. During surgery, the organs are examined for tissue damage and, if possible, returned to the abdominal cavity. This operation is called primary occlusion.

But if the organs are too big they will not find enough space in the abdominal cavity of the infant. This would lead to excessive pressure, which in turn affects vessels and organs, causes circulatory disorders and may affect the heart. In this case, a so-called multi-time closure is made.

The organs are housed in a bag over the abdominal wall defect. This bag is gradually reduced in size, which causes the organs to be pushed slowly into the abdominal cavity. Finally, the occlusion of the abdominal cavity occurs. This method prevents the excessive pressure increase.

Outlook & Forecast

Untreated, it comes with a Gastroschisis to an unfavorable disease course. Immediately after birth, adequate medical care must be provided to patients to ensure their survival.

Although a diagnosis is already possible in the womb and is done through an amniotic fluid examination, treatment can take place only after the birth. In surgery, a correction of the malformation is made. As a result, the functioning of the intestine is produced. If the operation takes place without further complications, the patient is subsequently considered cured.

In particular, in the first weeks and months of life multiple check-ups. The intestinal activity and the blood circulation are clarified. If no complaints occur, no further action is needed.

Complications or inflammations in the abdominal area delay the healing process. The infant will continue to be hospitalized in severe cases until health is stable, so no equipment is needed. There may be tissue damage or a nasogastric tube will be misplaced. Only in very rare cases are the damages of the organs to such an extent that further interventions are necessary. More likely is the use of a bag, which is used to position and move the organs to their destination.

prevention

Preventive measures against gastroschisis do not exist. However, early diagnosis is important. This can monitor the child's development and disease severity, ultimately increasing the success of postnatal treatment.

aftercare

A gastroschisis can not be treated by follow-up measures. The person concerned is dependent on a direct and above all on an early treatment in order to avoid further complaints or even the death of the child. The treatment of gastroschisis is therefore usually directly after birth.

The child is operated on and the organs are returned to the correct position. Mostly there are no special complications or other complaints if the gastroschisis is detected directly before birth. The affected parents must be particularly supported.

Above all, the care and support of friends and family is very helpful and necessary. Above all, a psychotic support can be useful. In severe cases or when the child dies of gastroschisis, a professional psychological counseling service can also be consulted.

The child has to rest after the procedure and should not be subjected to any special or strenuous activities. As a rule, regular examinations by a doctor are necessary even after the procedure. If the treatment of gastroschisis is successful, the life expectancy of the person affected is usually not reduced.

You can do that yourself

After the operation, regular check-ups with the pediatrician are indicated. The physician will tell the parents the exact time intervals and also inform them about reasons for extraordinary investigations. As a general rule, children with gastroschisis must be examined weekly for the first few weeks and months in order to ensure proper wound healing and early detection of any complications.

In dealing with the child further hygiene measures are required. So all utensils (eg vials and dishes) and garments should be cooked after use. The surgical wound itself must be treated according to the instructions of the pediatrician. The parents are also recommended a specialist consultation by a specialist in early childhood malformations. Through comprehensive discussions, the disease can be better understood and ultimately the handling of it easier.

If gastroschisis causes problems later in life, such as gastrointestinal dysfunction, a doctor should be consulted. Later, the child must be informed about the malformation, because it will ask anyway because of the visible scars. Under certain circumstances, an accompanying consultation by the doctor or a therapist makes sense.


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