I. Overview
Congenital myxomatous defects of the gastric wall refer to defects of the myxomatous layer of the gastric wall due to embryonic developmental disorders. It is less common and is often seen only after the occurrence of gastric perforation, mostly in the first 3-5 days after birth, mainly in early neonatal period, and mostly in premature babies.
Etiology
There are several theories about the etiology of congenital gastric wall muscular layer defect as follows.
1.Embryonic developmental abnormalities
During embryonic development, the circular muscle of the gastric wall is the first to occur, starting at the lower end of the esophagus and gradually progressing to the fundus and the greater curvature of the stomach, with the oblique muscle appearing in the ninth week of embryonic development and finally forming the longitudinal muscle. If there is a developmental disorder at a certain stage it will lead to a defect in the muscle layer of the gastric wall.
2. Localized ischemia of the gastric wall
In perinatal respiratory disorders, hypothermia and hypoxemia, local ischemia due to asphyxia before and after birth, impaired blood flow to the gastric wall, damage to the mucosal muscle layer, or excessive increase in gastric acid, compensatory redistribution of blood can occur in the infant, resulting in increased blood supply to important organs such as the brain and heart, and significantly reduced blood supply to the stomach and intestines, resulting in ischemic necrosis of the gastrointestinal tract.
3. Increased intragastric pressure
The submucosal tissues of newborns are fragile and the elastic fibers are underdeveloped, making them highly susceptible to gastric dilatation.
If the passage of gastric contents of the child is delayed, swallowing air during nursing, sucking and crying can cause the intragastric pressure to rise and gastric dilatation to occur, finally leading to rupture at the defect of the muscular layer of the gastric wall.
III. Clinical manifestations
Congenital gastric wall muscular layer defect is not easily diagnosed before the occurrence of perforation, and the child is generally in good condition after birth, without obvious prodromal symptoms and with a history of normal fetal defecation. The onset of the disease often occurs 3 to 5 days after birth. There are also individual cases as early as the second day or as late as the eighth day.
1.Initial symptoms
Rapid onset, sudden onset of acute abdominal symptoms, refusal of milk, vomiting, yellow-green or coffee-colored vomit, low cry, depression, progressive abdominal distension, respiratory distress and cyanosis.
2.Late stage symptoms
Peritonitis, fever, intestinal paralysis, dehydration, electrolyte disturbance, shock may appear.
IV. Examination
1.Peripheral blood examination
The peripheral blood count increases significantly when the infection is present.
2.Biochemical examination
Blood sodium, potassium, chloride, calcium and blood pH should be checked: in the presence of peritonitis, peritoneal puncture fluid leukocytes are often >1000/mm3, mainly granulocytes, and ascites pH 7.35~7.24 (normal 7.45).
3.X-ray examination
X-ray examination is the preferred method of examination for this disease, which is important for early detection of gastric perforation and early surgical treatment.
X-ray examination can detect pneumoperitoneum and liquid pneumoperitoneum.
The following signs can be seen in different positions: saddle sign, disappearance of gastric vesicle sign, pencil line sign, soccer sign, sickle ligament sign. Congenital muscular defect of the gastric wall is often associated with gastric perforation and may have characteristic radiographic manifestations.
(1) lying abdominal plain film: distention, gas, and “soccer ball sign” can be seen.
(2) Standing abdominal plain film: free gas under the diaphragm, elevation of diaphragm, plane of gas and liquid across the whole abdomen, disappearance of gastric vesicles and other signs.
(3) Horizontal lateral view shows a large amount of free gas under the abdominal wall and the outline of the anterior margin of the intestinal canal, and a long liquid-gas plane is seen. The above characteristic X-ray manifestations are very important for early diagnosis, timely surgery and prognosis of this disease.
4. Routinely do ultrasound
V. Treatment
1.Diagnosis is clear.
2, immediate preoperative preparations, such as fasting, cleaning the intestinal tract, if necessary, feasible gastric tube exhaust decompression.
3.Give relevant rehydration fluids as prescribed by the doctor to correct water and electrolyte disorders and shock, and anti-infective effects.
4.Respiratory management (do not use positive pressure oxygen administration), laparotomy decompression.
5.Surgical repair of perforation.
6.Continuous postoperative gastrointestinal decompression for 72 hours and nutritional support.
VI. Prognosis
This disease is congenital gastrointestinal dysplasia, no effective preventive measures, early detection and surgical treatment is the key.