Congenital defects of gastric musculature

　　The occurrence of congenital defects in the muscular layer of the gastric wall is related to abnormal embryonic development, local ischemia of the gastric wall, and increased intra-gastric pressure. The pathological manifestations are mainly the defect in the muscular layer of the gastric wall, the most common site being the greater curvature of the stomach. The specific causes and mechanisms are described as follows.

　　1. Etiology of congenital defects in the muscular layer of the gastric wall:

　　There are several theories about the etiology of congenital defects in the muscular layer of the gastric wall.

　　1. Abnormal embryonic development:During the process of embryonic development, the circular muscle of the gastric wall occurs earliest, starting from the lower end of the esophagus and gradually developing towards the fundus and greater curvature of the stomach. By the 9th week of embryogenesis, the oblique muscle appears, and finally, the longitudinal muscle is formed. If there is a developmental disorder at any stage, it can lead to defects in the muscular layer of the gastric wall.

　　2. Local ischemia of the gastric wall:During perinatal respiratory disorders, hypothermia, and hypoxemia, there can be compensatory redistribution of blood in the newborn's body, increasing blood supply to important organs such as the brain and heart, while significantly reducing blood supply to the stomach and intestines, leading to ischemic necrosis of the gastrointestinal tract.

　　3. Increased intra-gastric pressure:The main factors promoting perforation. The mucosal submucosal tissue of newborns is fragile, and the elastic fibers are underdeveloped, making it easy to have gastric dilation. If the gastric contents of the child pass through delayed, or if air is swallowed during breastfeeding, suckling, and crying, it can cause an increase in intra-gastric pressure, leading to gastric dilation, and finally causing the rupture of the gastric wall defect.

　　2. Pathogenesis of congenital defects in the muscular layer of the gastric wall:

　　The pathological manifestations of congenital defects in the muscular layer of the gastric wall are mainly the defect in the muscular layer of the gastric wall, the most common site being the greater curvature of the stomach. The specific causes and mechanisms are described as follows. The main pathological change is the defect in the muscular layer of the gastric wall, the most common site being the greater curvature, followed by the anterior wall, cardia, fundus, lesser curvature, posterior wall, and pylorus. The size of the defect varies. The defect area only has mucosa, submucosa, and serosa. Any factor that increases intra-gastric pressure after birth can cause a sudden increase in intra-gastric pressure, with the lesion site protruding outward, resembling a diverticulum. If the pressure continues to increase, it will affect blood circulation, leading to necrosis and perforation of the gastric wall with muscular layer defects.

　　The child generally has good condition after birth, with no obvious prodromal symptoms and a normal history of passing meconium. Congenital defects in the muscular layer of the gastric wall can lead to the occurrence of gastric perforation, peritonitis, intestinal paralysis, electrolyte and water imbalance, and shock.

　　Congenital defects in the muscular layer of the gastric wall are difficult to diagnose before perforation occurs. The child generally has good condition after birth, with no obvious prodromal symptoms and a normal history of passing meconium. The disease usually occurs between 3 to 5 days after birth, with some cases as early as the second day or as late as the eighth day.

　　1. Initial symptoms:The onset is acute, with sudden symptoms of acute abdominal pain, refusal to breastfeed, vomiting, with vomit of yellow-green or brown color, low crying sound, listlessness, progressive abdominal distension, respiratory distress, and cyanosis.

　　2. Late symptoms:Abdominal peritonitis may occur, with symptoms such as fever, intestinal paralysis, dehydration, electrolyte disorder, and shock.

　　The prognosis of congenital defects in the gastric wall muscle layer is poor, so regular examination is very important for the prevention of the disease. In addition, good prenatal care should be done for pregnant and postpartum women, and the specific preventive control measures are described as follows.

　　1. Regular physical examination: in order to achieve early detection, early diagnosis, and early treatment.

　　2. Good follow-up, good prenatal care, prevent premature birth and intrauterine hypoxia.

　　3. Prevent perinatal respiratory disorders, prevent the occurrence of hypothermia and hypoxemia.

　　4. Strengthen physical fitness, improve immunity: Pay attention to rest more, participate in physical exercise more, and eat more vitamin-rich foods.

　　The examination of congenital defects in the gastric wall muscle layer includes peripheral blood cells, blood biochemistry, X-ray, and B-ultrasound examination, and the specific examination methods are described as follows.

　　1. Peripheral blood examination

　　The peripheral blood leukocyte count increases significantly in infection.

　　2. Biochemical examination

　　It is necessary to check blood sodium, potassium, chloride, calcium, and blood pH value: when peritonitis occurs, the leukocyte count in peritoneal fluid is often >1000/mm³, mainly composed of granulocytes, with ascites pH of 7.35-7.24 (normal 7.45).

　　3. X-ray examination

　　X-ray examination is the first choice for the examination of this disease, and it is of great significance for early detection of gastric perforation and early surgical treatment. The examination can find pneumoperitoneum and liquid-pneumoperitoneum. The following signs can be seen in different positions: saddle sign, disappearance of gastric bubble sign, pencil line sign, football sign, and falciform ligament sign. Congenital defects in the gastric wall muscle layer often accompany gastric perforation and can have characteristic X-ray manifestations.

　　(1) Supine abdominal film Visible abdominal distension, increased gas,呈“football sign”.

　　(2) Standing abdominal film Free gas can appear under the diaphragm, diaphragmatic elevation, a transverse liquid-gas level across the entire abdomen, and disappearance of the gastric bubble.

　　(3) Horizontal lateral position There are a large amount of free gas under the abdominal wall and the outline of the intestinal lumen is displayed, and a long liquid-gas level is seen. The above characteristic X-ray manifestations are very important for early diagnosis, timely surgery, and the prognosis of the disease.

　　4. Routine ultrasound examination.

　　Diet for congenital defects in the gastric wall muscle layer

　　Patients with congenital defects in the gastric wall muscle layer, due to symptoms such as diarrhea, abdominal pain, weight loss, anemia, and systemic malnutrition, it is of great importance to improve these symptoms through dietetic care to promote the recovery of the patient.

　　1. Provide sufficient heat energy and protein. Due to the long-term chronic course, the body consumes a lot, and sufficient heat energy should be provided to prevent further weight loss. High-protein, high-calorie, low-fat semi-liquid or soft rice diet can be provided, with more than 100 grams of protein per day, 40 grams of fat per day, and a total heat energy of 10460 megajoules per day (2500 calories per day). Choose foods with low fat content and easy digestion. In severe cases, intravenous hyperalimentation or elemental diet and blended diet can be adopted to ensure energy and positive nitrogen balance.

　　2. Supplement sufficient vitamins. In addition to food supplementation, vitamin preparations can be provided if necessary. Based on clinical symptoms, focus on supplementing the corresponding vitamins, such as vitamin A, multivitamin B, vitamin C, vitamin D, vitamin K, and others.

　　(3) Pay attention to electrolyte balance. The supplementation of electrolytes is extremely important during severe diarrhea, and early intravenous supplementation can be used. Fresh fruit juice, oil-free meat soup, mushroom soup, and others can be provided in the diet. Patients with iron-deficiency anemia can eat iron-rich foods such as animal liver, and iron preparations can be taken orally if necessary.

　　(4) Small and frequent meals. Choose fine, soft, and easily digestible foods to ensure sufficient nutrition without increasing the burden on the intestines. In cooking, try to make the food fine, crushed, soft, and tender, and prefer to use boiling, braising, stewing, steaming methods, and avoid frying, deep-frying, and stir-frying to reduce the fat supply. Attention should be paid to the color, aroma, taste, and shape of the food, and all means should be used to improve the appetite of the patient. It is advisable to have 6 to 7 meals a day.

　　(5) To ensure nutritional supply, substitute therapy can be used for patients with decreased appetite. Oral essential diet, total nutrition preparations, and others can be used to supplement nutrition.

　　After a clear diagnosis of congenital defect of the gastrointestinal muscle layer of the stomach, surgery should be performed as soon as possible. Early diagnosis and treatment are crucial for the prognosis of this disease, and the specific treatment methods are described as follows.

　　I. Treatment of Congenital Defect of the Gastrointestinal Muscle Layer of the Stomach

　　1. Clear diagnosis.

　　2. Prepare for surgery immediately, such as fasting, cleaning the intestines, and if necessary, gastric tube exhaust decompression.

　　3. Administer relevant fluid replacement as prescribed to correct water and electrolyte imbalances and shock, and to fight infection.

　　4. Respiratory management (do not use positive pressure oxygen therapy), abdominal puncture decompression.

　　5. Surgical repair of perforation.

　　6. Continuous gastrointestinal decompression for 72 hours after surgery, and nutritional support.

　　II. Prognosis of Congenital Defect of the Gastrointestinal Muscle Layer of the Stomach

　　Although this disease is rare, the mortality rate is as high as 25% to 33%. Survival is possible if diagnosed and treated within 6 hours. The survival rate is 50% within 12 hours of diagnosis; the survival rate is 25% if diagnosed after more than 12 hours, and most die from shock.