Gastroschisis
The content of the article:
- Causes and risk factors
- Forms of the disease
- Symptoms
- Diagnostics
- Treatment
- Possible complications and consequences
- Forecast
Gastroschisis is an international term denoting a congenital malformation with prolapse of intestinal loops (less often of other organs) from the abdominal cavity through a through defect of the anterior abdominal wall, located, as a rule, to the right of a normally formed umbilical cord, at the border of the umbilical cord and abdominal skin. The average size is from 1.5 to 4 cm. Localization of the defect on the left occurs in isolated cases.
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Despite the trend towards an increase in the incidence of pathology in recent years, gastroschisis is quite rare: the frequency is on average 1 case per 3000-4000 live births.
Pathology is more often recorded in boys (approximately 1.6 times more often than in girls). The mortality rate for this malformation is high: in some regions it exceeds 45%. The best survival rates were achieved in the countries of North America and Western Europe: the probability of death is no more than 17% (in some countries - 4%).
As a rule, gastroschisis is an isolated defect: it is not combined with other anomalies in the development of organs and systems. Babies with gastroschisis are usually born earlier than the expected date of birth (average gestational age 37-38 weeks), functionally immature.
Synonym: intrauterine eventration of internal organs.
Causes and risk factors
Until now, there are no reliable data on the reasons for the formation of gastroschisis. According to some authors, the aggression of the mother's immune system towards the fetus plays a role. There is a high probability of inheriting gene mutations that provoke the formation of a defect.
Risk factors for developing gastroschisis:
- the young age of the mother (40% of cases occur in mothers under 20);
- smoking during pregnancy (the risk of defect formation increases by 50%);
- eating a large amount of nitrosamines during pregnancy;
- taking certain medications in the first trimester (aspirin, ibuprofen, pseudoephedrine, phenylpropanolamine);
- insufficient amount of alpha-carotene, some amino acids in the diet of a pregnant woman;
- exposure to the embryo in the early stages of development of ionizing radiation;
- reception by the mother during pregnancy of illegal drugs, drugs, alcohol.
Taking some drugs by a woman is a risk factor for the development of gastroschisis in the fetus.
The defect is formed at the 5-8th week of intrauterine development due to the violation of local blood circulation, which leads to necrotization and lysis of embryonic structures, from which the anterior abdominal wall is subsequently formed. According to a number of authors, the conditions for the formation of gastroschisis appear already in the first 3 weeks of pregnancy.
Forms of the disease
Gastroschisis comes in the following forms:
- total;
- subtotal;
- local.
The total form is characterized by the following features:
- abdominal wall defect - more than 3 cm;
- loss to the outside of all parts of the gastrointestinal tract;
- a significant decrease in the volume of the abdominal cavity;
- extreme viscero-abdominal imbalance.
With subtotal form:
- defect of the anterior abdominal wall - 1.5–3 cm;
- the small and large part of the large intestine is eventrated;
- viscero-abdominal imbalance is pronounced.
The local form is rare, in isolated cases. Her signs:
- small defect of the abdominal wall (less than 1.5 cm);
- prolapse of only a section of the small or large intestine;
- lack of expression of viscero-abdominal imbalance.
Symptoms
The clinical manifestation of gastroschisis is the eventration (prolapse) of the abdominal organs through a through defect in the anterior abdominal wall.
More often the stomach, loops of the small intestine and large intestine fall out, less often - the bladder, uterus with appendages in girls, testes in boys (if at the time of birth they have not yet descended into the scrotum).
The intestine has a characteristic appearance: its loops are atonic, edematous, dilated (up to several centimeters), peristalsis is depressed, the pulsation of the mesenteric vessels is poorly expressed. Sometimes the loops are soldered into a single conglomerate, their surface is covered with a "shell" of fibrin and collagen deposits.
The color of the eventrated organs varies from greenish-gray to purple-cyanotic. Often, with gastroschisis, a child has signs of intrauterine chemical peritonitis.
The interposition of the abdominal organs with this malformation is always violated: there is a lack of differentiation of the intestine into thin and thick, its shortening, defective reversal of loops.
In addition to organ prolapse, gastroschisis is characterized by a decrease in the volume of the abdominal cavity in relation to the increased volume of eventrated internal organs, which is referred to as viscero-abdominal imbalance.
Diagnostics
The main way to diagnose gastroschisis is ultrasound screening during pregnancy. With reliable confirmation of the defect, the operation should be performed urgently in the first hours after the birth of the child (preoperative preparation lasts from 3 to 12 hours, on average - about 6 hours).
Fetal gastroschisis on ultrasound
Treatment
Treatment of gastroschisis is carried out by surgery. Preference is given to radical one-stage abdominal wall plasty with local tissues with the placement of eventrated organs into the abdominal cavity.
At present, biological and synthetic materials are widely used for plastics of the anterior abdominal wall. From biological the most widespread are the dura mater and amniotic membrane, from synthetic ones - lavsan and Teflon nets, silastic bags and plates, silasticdacron prostheses, collagen-vicryl tissues.
Surgical treatment of gastroschisis
Possible complications and consequences
The complication of gastroschisis, which most often leads to death, is hypothermia of the newborn, which is due to the large area of heat transfer from the eventrated organs. Hypothermia in this case entails a shift in acid-base balance, severe metabolic disorders, acute renal failure, cerebral hemorrhage.
Also, the consequences of immersion of eventrated organs with pronounced viscero-abdominal imbalance pose a high danger. Due to the small volume of the abdominal cavity after the placement of the changed organs, intra-abdominal pressure rises sharply, which leads to compression of the inferior vena cava, cardiovascular and respiratory disorders, acute renal failure, impaired blood supply to the intestinal wall and causes complications and deaths.
Major postoperative complications (early and late):
- surgical infection with the development of sepsis;
- ulcerative necrotizing enterocolitis;
- toxic hepatitis and liver failure;
- adhesive intestinal obstruction.
Forecast
Gastroschisis is a correctable developmental defect that, with timely diagnosis and qualified treatment, allows the child to survive and socially adapt in the future.
Olesya Smolnyakova Therapy, clinical pharmacology and pharmacotherapy About the author
Education: higher, 2004 (GOU VPO "Kursk State Medical University"), specialty "General Medicine", qualification "Doctor". 2008-2012 - Postgraduate student of the Department of Clinical Pharmacology, KSMU, Candidate of Medical Sciences (2013, specialty "Pharmacology, Clinical Pharmacology"). 2014-2015 - professional retraining, specialty "Management in education", FSBEI HPE "KSU".
The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!
