umbilical process
Introduction
Introduction The umbilical process 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 associated with chromosomal abnormalities, sick children may be accompanied by malformations of other organs while suffering from umbilical bulging. 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, necrosis and infection. In a few cases, the capsule is gradually fibrillated to form a scar, which protects the viscera and prevents early death. The mortality rate of this disease is related to the treatment time. If necessary, surgery should be performed as soon as possible. Umbilical disease is the umbilical bulge of the newborn. "Certificate of Correction and Prescription": "Because of the newborn bath, the umbilical cord is not tight, and the water is immersed in the inside. After the postpartum period, the umbilical light flutters like a blow, and the sputum turns slightly, and the room is horrified." Umbilical dysplasia, or excessive crying, severe cough, etc., resulting in increased abdominal pressure, resulting in compression of the intestine to the umbilicus. Most can heal themselves. However, if the age is more than two years old, it should be treated with umbilical hernia. Consider surgical resection and repair.
Cause
Cause
(1) Causes of the disease
Umbilical bulging is caused by the pause in the process of embryonic body cavity closure during embryonic development. The early embryonic intestine is differentiated from the yolk sac, and the middle part of the original intestine and the yolk sac are connected by the yolk tube. Due to the rapid growth of the dorsal axis during embryonic development, when the dorsal axis grows, the abdominal wall around the open umbilical cord cavity forms a wrinkle similar to a bag-like shape, which is contracted from the periphery to the center. It can be divided into 4 areas:
1 head, the body layer will form the chest wall, upper abdominal wall and diaphragm.
2 cercariae, the body layer and the allantoic sac will form the lower abdominal wall and bladder.
3 Two lateral tendons form the abdominal wall on both sides.
These four ridges merge toward the center, and the apex forms an umbilical ring. During this process, the yolk tube gradually becomes smaller, but is still connected between the yolk sac and the midgut, and plays a traction effect on the midgut. Since the intestinal growth rate is faster than the abdominal wall, the intestinal tract and other internal organs are temporarily pulled into the umbilical cord during the 6th to 10th week of the embryo, becoming a physiological temporary umbilical hernia. After 10 weeks of embryos, the volume of the abdominal cavity rapidly expanded, and the skin and muscles of the abdominal wall rapidly grew from the dorsal side to the ventral side, and the midgut and abdominal organs returned to the abdominal cavity. At the 12th week of the embryo, the midgut completes the 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 embryos, obstacles occur in a certain part of the development process, and the development of one of the four sputum is restricted, the internal pressure is increased, the traction of the umbilical cord and the anterior abdominal wall are adjacent to the umbilical cord. Defects, and due to the degree of inhibition of development in the four sputum, there will be corresponding visceral bulging deformities, such as cephalic development defects: umbilical bulging, sputum, sternal defect and ectopic heart; developmental defects of lateral ridge: Umbilical bulging, abdominal fissure; cercaria development defects: umbilical bulging, bladder valgus, small intestine bladder fissure, anorectal atresia.
(two) 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 greater than 5 cm. Therefore, the midgut that migrated to the outside of the body cavity 10 weeks ago cannot be reintroduced into the smaller abdominal cavity, and the extra-abdominal growth is retained throughout the fetal period. The defect of the abdominal wall above the umbilical cord is often more than that of the lower part. Therefore, the liver, spleen and pancreas can protrude to the outside of the body, especially the liver. Because of its large volume and position, it is more likely to bulge, which is a sign of giant umbilical bulging. The viscera of the umbilical bulge is surrounded by a capsule, which is composed of an amnion and an intima fusion corresponding to the peritoneum of the parietal layer. There is a layer of jelly-like connective tissue (warthon jelly) between the two. Slightly white and transparent, 1 to 2 mm thick, uneven thickness. A residual strain of the umbilical cord is visible at or near the lower half 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 incorporated into 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 is intestinal fistula in the capsule, and the internal organs such as liver and spleen are not prominent in the body.
3. Associated with deformity
In 1986, Moore reported that in 490 cases of abdominal wall malformation, umbilical bulge accounted for 287 cases, 54% of which were associated with malformation, which was related to heredity. China Medical University reported that 17 of 56 cases of umbilical bulge were associated with other malformations, accounting for 30.4%, and 7 cases with more than 2 malformations, accounting for 12.5%.
The umbilical expansion can be combined with the presence of patent ductus arteriosus, Mcckel diverticulum, uterine urethral closure, colonic absence, bladder valgus, intestinal duplication, diaphragmatic dysplasia and defects, incomplete chest and abdomen malformation, and abdominal wall development pause Related diseases, while intestinal malrotation is the most common concurrent malformation.
In addition, umbilical bulging can occur in many chromosomal syndromes, such as 13-15, 16-18, and 21-trisomy chromosome syndromes. 40% of children with umbilical swelling can be combined with other congenital malformations such as cleft lip, multi-finger, congenital heart disease. If the umbilical bulge is accompanied by a giant tongue, and the body length and body weight exceed the normal level, it is called umbilical bulging - giant tongue - giant syndrome, sometimes accompanied by hypoglycemia and visceral hypertrophy (Beckwith-Wiedemann syndrome).
The Contell Five-Link is another type of umbilical bulge that is caused by a pause in head lice development. The performance of the abdominal umbilical bulge with distal sternal fissure, anterior midline iliac muscle defect, pericardium and abdominal cavity, intracardiac dysplasia (such as ventricular septal defect, Fallot's quadruple, etc.) and the heart forward displacement. The heart beats through the transparent capsule, and sometimes the intestinal fistula can penetrate the pericardium through the diaphragm defect.
Examine
an examination
Related inspection
Umbilical examination chest perspective
Giant umbilical bulge
The diameter of the defect ring of the abdominal wall exceeds 5 cm, sometimes up to 10 cm, and the diameter of the bulged portion tends to be large. A tumor such as a taro can be protruded in the center of the abdomen, and the umbilical cord is attached to the top of the capsule. After birth, the capsule can be seen through the transparent membrane. In addition to the small intestine and colon, the contents of the capsule include the liver, spleen, pancreas and even the bladder. After 6-8 hours, due to lack of blood supply to the capsule wall and exposure to the air, the capsule became cloudy and the edema thickened. After 2 to 3 days, it becomes dry, fragile, broken, and even necrotic. The rupture of the wall of the capsule can lead to infection of the abdominal cavity and the release of the internal organs of the capsule. In severe cases, the child can be killed, so it should be treated in time.
About 1% of the children's capsules rupture during prenatal or labor, leading to visceral prolapse. Once the capsule is ruptured in the uterus, the organ that has been removed is soaked in the amniotic fluid for a long time, the intestinal wall is edematous, thickened, the surface is dull, and covered with inflammatory exudate. There are many meconium-colored cellulose on the surface, and the abdominal cavity Secondary infections have a very high mortality rate. If the capsule is ruptured during childbirth, the color of the internal organs and intestines is brighter and there is no yellow cellulose coverage. The emergency treatment can save the child. Although the time to rupture of the capsule is different, the residual capsule can be found. The skin at the base of the capsule can crawl along the surface of the capsule, eventually forming a connective tissue covering the surface of the capsule under the capsule. The skin and capsule junctions are prone to infection and can spread into 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. Things. Most of the contents of the capsule are only the small intestine and sometimes the transverse colon. When giving birth, 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.
The diagnosis can be confirmed based on clinical symptoms and signs.
Diagnosis
Differential diagnosis
Differential diagnosis of umbilical processes
The umbilical process needs to be differentiated from the abdominal fissure. The main point of the difference is that the umbilical bulge has no normal umbilical structure, and the ruptured residual capsule can be found between the intestinal tract or the internal organs. The position and shape of the abdominal fissure, umbilical cord and umbilical cord are normal, but there is a crack in the abdominal wall of the umbilicus, and the intestine tube protrudes from the abdomen. X-ray chest fluoroscopy and other examinations should be performed before surgery to understand whether there is any accompanying malformation, so as to be treated together during the operation. Regular abdominal ultrasound examination during pregnancy can be used to detect umbilical bulge early so that treatment can be taken immediately after delivery.
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. top. After birth, the capsule can be seen through the transparent membrane. In addition to the small intestine and colon, the contents of the capsule include the liver, spleen, pancreas and even the bladder. After 6-8 hours, due to lack of blood supply to the capsule wall and exposure to the air, the capsule became cloudy and the edema thickened.
After 2 to 3 days, it becomes dry, fragile, broken, and even necrotic. The rupture of the wall of the capsule can lead to infection of the abdominal cavity and the release of the internal organs of the capsule. In severe cases, the child can be killed, so it should be treated in time. About 1% of the children's capsules rupture during prenatal or labor, leading to visceral prolapse. Once the capsule is ruptured in the uterus, the organ that has been removed is soaked in the amniotic fluid for a long time, the intestinal wall is edematous, thickened, the surface is dull, and covered with inflammatory exudate. There are many meconium-colored cellulose on the surface, and the abdominal cavity Secondary infections have a very high mortality rate. If the capsule is ruptured during childbirth, the color of the internal organs and intestines is brighter and there is no yellow cellulose coverage. The emergency treatment can save the child. Although the time to rupture of the capsule is different, the residual capsule can be found. The skin at the base of the capsule can crawl along the surface of the capsule, eventually forming a connective tissue covering the surface of the capsule under the capsule. The skin and capsule junctions are prone to infection and can spread into 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. Things. Most of the contents of the capsule are only the small intestine and sometimes the transverse colon. When giving birth, 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. The diagnosis can be confirmed based on clinical symptoms and signs. Breakline
