omphalocele
Introduction
Introduction to umbilical bulging Amur bulging (acromphalus) is a common type of congenital abdominal wall malformation. It is due to congenital abdominal wall hypoplasia, forming a defect in the abdominal wall around the umbilical cord, leading to neonatal malformation of the abdominal visceral prolapse. Because the disease is related to chromosomal abnormalities, the sick child In the case of umbilical bulging, it may be accompanied by malformations of other organs. Improper mortality is high. Most of the sick children need surgery immediately after birth, otherwise it is difficult for the sick child to survive due to local skin ulceration and necrosis. In a few cases, the capsule is gradually fibrillated to form a scar, which protects the viscera and prevents early death. If the mortality rate of this disease is related to the treatment time, surgery should be performed as soon as possible. basic knowledge The proportion of illness: 0.001% Susceptible people: seen in newborns Mode of infection: non-infectious Complications: retroperitoneal infection and abscess
Cause
Umbilical bulging cause
Congenital factors ( 89% )
Umbilical bulging is caused by the pause of embryonic body cavity during embryonic development. The early embryo of the embryo is differentiated from the yolk sac. The middle part of the original intestine and the yolk sac are connected by the yolk tube, due to the back axis during embryonic development. The growth is faster. When the dorsal axis grows, the abdominal wall around the open umbilical cavity forms a wrinkle like a purse-like shape toward the center, and is contracted from the outer periphery to the center. It can be divided into four regions: one scorpion, and the body layer will form the chest wall. Upper abdominal wall and diaphragm; 2 scorpion scorpion, its body layer and allantoic sac will form the lower abdomen wall and bladder; 3 two lateral iliacs form bilateral abdominal wall, these four sacs merge toward the center, the apex forms the umbilical ring, in the process The yolk tube gradually becomes thinner, but is still connected between the yolk sac and the midgut, and plays a traction effect on the midgut. Because the intestinal growth rate is faster than the abdominal wall, the intestinal tract and other internal organs are temporarily suspended in the 6th to 10th week of the embryo. It is pulled into the umbilical cord and becomes a physiological temporary umbilical hernia. After 10 weeks of embryos, the volume of the abdominal cavity rapidly expands. The skin and muscles of the abdominal wall grow rapidly from the dorsal to the ventral side, and the midgut and abdominal organs return to the abdominal cavity. At 12 weeks, the midgut completed normal Rotation, while the abdominal wall merges in the center to form the umbilical ring. If the abdominal wall is affected by certain factors during the development of the above embryo, obstacles occur in a certain part of the development process, and the development of one of the four sputum is restricted, and the internal pressure is increased. The traction of the umbilical cord and the defect of the anterior abdominal wall near the umbilical cord, and due to the different degree of inhibition of the development of the four sputum, will produce corresponding visceral bulging deformities, such as cephalic development defects: umbilical bulging, sputum, sternal defect And ectopic heart; lateral defects of developmental defects: umbilical bulging, abdominal fissure; squat development defects: umbilical bulging, bladder valgus, small intestine bladder split, anorectal atresia.
Pathogenesis
According to the size of the defect, the umbilical bulge can be divided into two types, small and giant.
1. Giant umbilical bulge (embryonic umbilical bulge): The developmental pause of the body wall occurs before the 10th week of the embryo, and the diameter of the defect is more than 5cm. Therefore, the midgut that migrated to the outside of the body cavity before 10 weeks cannot be returned to the volume. The small abdominal cavity grows outside the abdominal cavity during the whole fetal period. The abdominal wall defect above the umbilical cord is often more than the lower part. Therefore, the liver, spleen and pancreas can protrude to the outside of the body, especially the liver. Due to the large volume, the position is easier. The bulging is a sign of the giant umbilical bulge. The viscera of the umbilical bulge is covered by a capsular membrane. The capsular membrane is composed of an amnion and an intima fusion corresponding to the peritoneum of the parietal layer. There is a layer of jelly between the two. The connective tissue (warthon jelly), the capsule is slightly white and transparent, 1 to 2 mm thick, and the thickness is uneven. The residual strain of the umbilical cord can be seen in the lower half or near the lower edge of the giant umbilical bulge.
2. Small umbilical bulge (fetal umbilical bulge): The body layer forming the abdominal wall appears to have a developmental pause after 10 weeks, and the defect of the abdominal wall is less than 5 cm. At this time, the body cavity has a considerable volume, and part of the midgut can also be included in the abdominal cavity. The residual strain of the umbilical cord is in the center of the capsule. This capsule is the enlarged umbilical cord base, also known as the umbilical cord hernia. There are intestinal fistulas in the capsule, while the internal organs such as liver and spleen are not prominent in the body.
3. Associated with malformations: In 1986, Moore reported that 287 cases of umbilical bulge in 490 cases of abdominal wall malformation, 54% have associated malformations, related to heredity, China Medical University reported that 56 cases of 56 cases of umbilical bulge Other malformations accounted for 30.4%, and 7 cases with more than 2 malformations, accounting for 12.5%.
Umbilical expansion can be combined with the presence of patent ductus yolk, Mcckel diverticulum, uterine urethral closure, colonic absence, bladder eversion, intestinal duplication, diaphragmatic dysplasia and defect, incomplete chest and abdomen malformation and abdominal wall development pause Related diseases, and intestinal malrotation is the most common concurrent malformation. In addition, umbilical bulging can occur many chromosomal syndromes, such as 13-15, 16-18 and 21-trisomy chromosome syndrome, 40% umbilical bulge Children can be combined with other congenital malformations such as cleft lip, multi-finger, congenital heart disease, etc., such as umbilical bulge with a giant tongue, while the body length, body weight exceeds normal levels, it is called umbilical bulging - giant tongue - giant syndrome, Sometimes accompanied by hypoglycemia and visceral hypertrophy (Beckwith-Wiedemann syndrome).
Contell's five-link syndrome is another type of umbilical bulge. It is caused by the development of cephalic sputum. It shows abdominal umbilical bulge with distal sternal fissure, anterior midline diaphragmatic defect, pericardium and abdominal cavity, and intracardiac dysplasia ( Such as ventricular septal defect, Fallot's quadruple sign, etc.) and the heart shifts forward, the heart beats through the transparent capsule, and sometimes the intestinal fistula can enter the pericardium through the diaphragm defect.
Prevention
Umbilical bulge prevention
For the healthy birth of a healthy baby, pregnant women who eat food must reduce the amount of food, and regular inspections are also necessary. For example, regular abdominal ultrasound examination during pregnancy can be used to detect umbilical bulge early so that treatment can be taken immediately after delivery.
Foods that are prone to fetal malformations during pregnancy are as follows:
1, pig liver
Finland and the United States have advised pregnant women about the need to eat less pig liver. Because in modern feeds that quickly fertilize livestock, too much fertilizer is added, which has a high vitamin A content, causing it to accumulate in the liver of animals. Pregnant women overeating pig liver, a large amount of vitamin A will easily enter the body, which is very harmful to fetal development, and even teratogenic. Vitamin A excess can cause damage to almost every part of the body, including the eyes, bones, blood, skin, central nervous system, liver, reproduction and urinary system.
2, tuna
The US environmental protection group found that seven kinds of seafood, including tuna, have a serious excess of mercury, and pregnant women often have a teratogenic condition. Some doctors in Hong Kong pointed out that the absorption of excessive mercury in the mother's body will affect the development of the brain's nerves, and the learning ability will be defective in the future, as well as the sequelae of mental retardation.
3, too much acidic food
The study found that pregnant women eat too much acidic foods such as meat, fish, chocolate, sugar, etc., their body fluids will change, forming an "acidification", which further promotes the level of catecholamines in the blood, causing negative emotions such as irritability of pregnant women. . This kind of bad negative emotion can increase the secretion of hormones and other toxic substances in the mother, which may cause fetal cleft palate, cleft lip and other organ malformations.
4. Contaminated food
Pregnancy Mommy lacks inorganic salts or trace elements can cause fetal malformations, and pregnant women often eat contaminated foods that can also cause fetal malformations. Foods contaminated with DDT, BHC and other organochlorine pesticides and organic mercury pesticides such as Xi Lisheng, which are highly concentrated pesticides, enter the body, and the poisons will accumulate in the pregnant women through the blood circulation into the placenta, causing fetal poisoning, causing miscarriage, Teratogenic, stillbirth, etc. Therefore, contaminated food must not be eaten.
Complication
Umbilical bulging complications Complications, retroperitoneal infection and abscess
Barrier-free blood supply disorders and rupture can lead to abdominal organ detachment and secondary abdominal infection, which can often endanger the life of the child.
Symptom
Umbilical bulging symptoms Common symptoms Edema umbilical peripheral abdominal wall defect Congenital abdominal wall dysplasia Umbilical sore synovial thickening Infants with umbilical mass protruding
1. Giant umbilical bulge: the diameter of the defect ring of the abdominal wall is more than 5cm, sometimes up to 10cm, and the diameter of the bulging part is often large. It can protrude in the center of the abdomen like a taro-like mass, and the umbilical cord is connected to the capsule. At the top, the organs inside the capsule can be seen through the transparent membrane after birth. The contents of the capsule include the liver, spleen, pancreas and even the bladder in addition to the small intestine and colon. After 6-8 hours, the blood supply to the capsule wall is lacking and exposure to the air. Among them, the capsule becomes cloudy and the edema thickens. After 2 to 3 days, it becomes dry, fragile, ruptured, and even necrotic. The rupture of the cyst wall can lead to infection of the abdominal cavity and the detachment of the cystic organs. In severe cases, the child can be invaded. Death, it should be treated in the early stage, about 1% of the children's capsules rupture in prenatal or labor, leading to visceral prolapse, once the capsule is ruptured in the uterus, the organs that are released are soaked in the amniotic fluid for a long time, the intestinal wall Edema, thickening, dull surface, covered with inflammatory exudate, many meconium-colored cellulose on the surface, secondary infection in the abdominal cavity, extremely high mortality, if the capsule is ruptured during childbirth, the color of the internal organs and intestines is brighter. , Without yellow cellulose coverage, emergency treatment, the child can be saved, although the time of capsule rupture is different, but the residual capsule can be found, the skin of the base of the capsule can crawl along the surface of the capsule, and finally in the capsule The connective tissue is formed to cover the surface of the capsule, and the skin and the capsule junction are susceptible to infection and can spread to the abdominal cavity.
2. Small umbilical bulge: the diameter of the abdominal wall defect ring is less than 5cm, and it protrudes in the center of the abdomen like orange or even olive-like mass. Because the diameter of the bulging part is often larger than the diameter of the abdominal wall defect, it can form a central abdomen. The contents of the capsules are mostly only the small intestine, sometimes there is a transverse colon. When the birth is delivered, if the umbilical part is enlarged, it should be ligated above the umbilical cord to prevent the intestinal tube from being ligated in it, causing intestinal necrosis.
Examine
Umbilical bulge examination
Chest perspective is a direct examination of the patient between the X-ray tube and the screen. Can be fully dynamic and direct observation, such as heart beats, diaphragm movements, gastrointestinal motility, joint activities and so on. It can also be used for X-ray angiography in locating observation, gastrointestinal angiography, and indication diagnosis and treatment, such as cardiac catheterization, fracture reduction, and foreign body removal. The most frequently used part of the fluoroscopy is the chest, examining the lungs, pleura, mediastinum, and heart and large vessel lesions. It can also be used for the examination of bones, soft tissues, foreign bodies in body cavities, gases, stones, and contraceptive rings.
Diagnosis
Diagnosis of umbilical bulge
diagnosis
The diagnosis can be confirmed based on clinical symptoms and signs.
Differential diagnosis
The umbilical bulge needs to be differentiated from the abdominal fissure. The main point of the identification is that the umbilical bulge has no normal umbilical structure, and the residual capsule can be found between the intestinal tract or the viscera, and the location of the abdominal fissure, umbilicus and umbilical cord The morphology is normal, only a crack in the abdominal wall of the umbilicus, the intestine tube protrudes from the abdomen, and X-ray chest fluoroscopy and other examinations should be performed before surgery to understand whether there is a malformation associated with the surgery, so as to be treated together during the operation, regular abdominal ultrasound examination during pregnancy. The umbilical bulge can be found early so that treatment can be taken immediately after delivery.
