Neonatal Gastrointestinal Duplication
Introduction
Introduction to neonatal digestive tract duplication Duplication of digestivetract (newborn) refers to the attachment to the side of the digestive tract, which has the same characteristics as a part of the wall of the digestive tract. It is a congenital malformation of a cystic or tubular cavity structure that can occur in the digestive tract. The site can occur from the base of the tongue to the anus, but the most common ileum is derived from the endoderm. There have been various names in the literature, such as intestinal cysts, intestinal cysts, large diverticulum of the gastrointestinal tract, atypical Meckel's diverticulum and recurrent ileum, most of which occur during neonatal and pediatric periods. basic knowledge The proportion of the disease: the disease is rare, the incidence rate is about 0.0001% - 0.0003% Susceptible people: children Mode of infection: non-infectious Complications: intestinal obstruction hemoptysis anemia peritonitis anal atresia umbilical bulging spina bifida
Cause
Neonatal digestive tract repeat disease etiology
(1) Causes of the disease
It is a malformation of embryonic development, but there are many factors that cause malformation, which is considered to be multi-sourced.
1. Abnormal embryonic intestinal lumen process: the embryonic period begins in the fifth week of the embryo, vacuolization, cavityization process, if a cystic cavity is not connected with the intestinal tube during the lumen, the intestinal cyst type can be formed. Digestive tract repeats can form from the small intestine to the colon.
2. The diverticulum-like outer bag is developed: many temporary diverticulum often appear in the midgut of the embryonic period, forming an outer bag, which will disappear afterwards. If it is not degraded or isolated, it becomes a tubular or isolated sac that communicates with the intestine. Intestinal repeat deformity.
3. The theory of spinal cord division: in the third week of embryonic ectoderm and endoderm, the spinal cord grows to the head end, and the adhesion between the ectodermal layer and the endoderm occurs locally. The spinal cord grows to the adhesion and splits into two branches, from the adhesion two. Lateral bypass, and then continue to grow to the head end, the ectoderm forms a neural tube, the notochord is surrounded by mesoderm to form the spine, and the adhesion of the ectoderm to the endoderm is formed by the intestinal wall to form a diverticulum, which also hinders the formation of the vertebral body. Vertebral body rupture occurs, so 1/3 of the disease is associated with spinal deformity. If the diverticular neck is closed, a cystic intestinal repeat deformity is formed.
4. Terminal axillary malformation: a small number of cases have long tubular repeat deformities parallel to the whole colon and rectum, and often accompanied by repeated deformities of the genitourinary organs, such as double uterus, double vagina, double bladder, double urethra, double external genitalia, etc. .
5. Poor separation of laryngotracheal sulcus and foregut: the laryngotracheal sulcus appears in the fourth week of the embryo, and gradually closes into a tube and esophagus. If part of the embryo is not closed, the anterior intestinal tract is formed. This sputum may partially degenerate during the embryonic period. Repeated malformation of the esophagus.
(two) pathogenesis
Repeated deformity can occur in any part of the digestive tract, with the most common ileum, accounting for about 50%, followed by the jejunum, cecum, esophagus, stomach, duodenum, colon, and about 21.5% in the chest.
Repeated deformities can be divided into 4 types:
1. Extra-intestinal cyst type: more common, spherical or elliptical, located in the mesentery of the intestine, mostly closely adhered to the adjacent intestinal wall, with a common wall layer, more than the intestinal lumen, only a few holes, cyst The surface is smooth, covered with serosa, and there is colorless or yellowish mucus in the cyst cavity, which makes the whole cyst have tension and elasticity.
2. Enterocyst cyst type: The cyst is located in the submucosa or muscle layer of the intestinal wall, which can cause the intestinal lumen to pass through the obstacle.
3. Tube type or double intestine type: There is a tubular intestine in the mesentery parallel to the normal intestine. It is a double-tube type intestine with different lengths ranging from several centimeters to 50cm. Generally, the distal end of the lumen is more digested. The channels are connected, sometimes only one end is connected, similar to the diverticulum, the small intestine is open at the distal end, and the colon is mostly open at the proximal end.
4. Thoracic digestive tract repeat deformity: The cyst is more near the esophagus or tracheobronchial, and there may be a cord with the vertebral body, accompanied by vertebral body fissure or spinal deformity. The tubular type can pass through the diaphragm into the mesentery and close to the digestive tract.
The wall of the re-digestive tract has muscle layer and mucous membrane, the nature of which is the same as that of the adjacent digestive tract, but not necessarily the same as the same level. About 20% is the gastrointestinal mucosa of the fauna, such as gastric mucosa or pancreatic tissue. Can cause peptic ulcer, bleeding and perforation, more closely attached to a part of the digestive tract, with a common source of blood.
Prevention
Neonatal digestive tract repeat prevention
Do a good job in genetic counseling, do a good job in pregnancy care, actively prevent and cure various infectious diseases, preventive measures refer to other birth defects diseases, in order to reduce and reverse the incidence of neonatal birth defects, prevention should be from pre-pregnancy to prenatal: premarital medical examination It plays an active role in preventing birth defects. The size of the action depends on the examination items and contents, including serological tests (such as hepatitis B virus, treponema pallidum, HIV), reproductive system tests (such as screening for cervical inflammation), and general physical examination ( Such as blood pressure, electrocardiogram) and ask about the family history of the disease, personal medical history, etc., do a good job in genetic disease counseling.
Complication
Neonatal digestive tract repeat complications Complications, intestinal obstruction, hemoptysis, peritonitis, anal atresia, umbilical bulging, spina bifida
Can be complicated by intestinal obstruction, ulcer, can be complicated by intestinal hemorrhage, hematemesis or hemoptysis, can be anemia, can be complicated by intestinal necrosis and peritonitis, can coexist with intestinal or anal atresia, poor intestinal rotation, Meckel, anal atresia and umbilical bulging Etc., but also have a double uterus, double vagina, double bladder, double urethra, and even double external reproductive organs, etc., often accompanied by spina bifida, cerebrospinal meningeal and other deformities.
Symptom
Symptoms of neonatal digestive tract recurrence common symptoms intussusception, bloating, dysphagia, low intestinal obstruction, blood stasis, cystic hemoptysis, repeated vomiting, stomach, repeated deformity
Due to the location, size, type and presence or absence of gastric mucosa, the clinical manifestations are different. Generally, it can be asymptomatic for life. Symptoms occur only when complications occur.
1. Digestive tract obstruction: ileum, ileocecal area, the incidence of repeated malformation in the cavity is high, so the symptoms of small intestinal obstruction are the most common, cystic or tubular deformity can oppress the intestinal lumen, which is the main cause of obstruction, cystic due to The mucous membrane can secrete a large amount of fluid, the pressure in the cavity increases, the cyst swells, causing obstruction, and the tubular such as the proximal end communicates with the intestinal lumen, and the distal side is a blind end. Due to the accumulation and enlargement of the intestinal contents, partial intestinal obstruction may also occur. It is characterized by repeated vomiting and bloating. Repeated deformities at the ileocecal end are sometimes accessible in the right lower abdomen. Large cysts in the mesentery can be reversed, and intestinal torsion can be caused to cause strangulated intestinal obstruction. Can cause intussusception, the cystic deformity of the stomach and duodenum is often small, but can cause pyloric or duodenal obstruction in the neonatal period, the left upper abdomen can touch cystic mass, with vomiting, hematemesis or Blood in the stool.
2. Gastrointestinal hemorrhage: Tubular repeat deformity is open in the normal intestine. If there is a fascinating gastric mucosa, it may be followed by peptic ulcer, bleeding or even perforation, repeated hematemesis and blood in the stool, resulting in anemia.
3. Repeated malformation of the digestive tract located in the thoracic cavity: can cause symptoms of organ compression, esophageal compression can cause difficulty swallowing, respiratory tract compression can cause cough, asthma, bruising and other symptoms, respiratory distress can occur in the neonatal period, such as Respiratory secretions can be recurrent, respiratory tract infections can occur, cystic malformations such as gastric mucosa can cause ulcers, ulceration to the esophagus or trachea, hemoptysis or hematemesis.
Examine
Examination of neonatal digestive tract duplication
Increased white blood cells and neutrophils in the presence of infection; decreased hemoglobin concentration and decreased red blood cell count in the presence of anemia. There may be water and electrolyte disorders, blood sodium, potassium, chlorine, calcium, magnesium and blood pH, creatinine and other tests should be done.
The incidence of this disease is not high, clinical symptoms are different, preoperative diagnosis is difficult, less than 25% of patients diagnosed before surgery.
1. X-ray examination: plain abdomen flat, can understand the presence or absence of intestinal obstruction and peritonitis, chronic cases can be applied with barium enema, chest radiograph can be found in the thoracic repeat deformity, showing a clear circular shadow, or visible Cardiopulmonary and mediastinal shift to the contralateral side, for diagnostic reference, chest X-ray film can also understand the presence of spina bifida, hemivertebra and scoliosis, etc., to help diagnose.
2. B-ultrasound: The nature of the abdominal mass can be identified, cystic or substantial, cystic mass helps the diagnosis of intestinal duplication.
3. Nuclide examination: The use of radionuclide 99mTc abdominal scan is helpful for the diagnosis of digestive tract malformation of gastric mucosa, but it is not easy to distinguish with Meckel's diverticulum.
4. CT examination: It can show that the chest or intestine mass is cystic or substantial, which indirectly contributes to the identification of repeated malformations of the digestive tract.
Diagnosis
Diagnosis and diagnosis of neonatal digestive tract duplication
diagnosis
In the neonatal period, if coughing, asthma, bruising, dysphagia, vomiting, etc. occur repeatedly, the possibility of the disease should be considered. In addition, the symptoms of incomplete obstruction of the digestive tract in the neonatal period and the cystic mass in the abdomen should be considered. In the diagnosis of the disease, about half of the cases can be diagnosed before surgery, acute intestinal obstruction or acute bleeding cases, it is difficult to make a correct diagnosis before surgery.
Ultrasound examination can detect cystic masses. Radionuclide scanning can show gastric mucosal tissue under fluorescence. X-ray abdominal plain film can mainly diagnose obstruction, and can also find tumor shadow. The chest radiograph has oval streak in the posterior mediastinum. Smooth shadows on the edge of the shape, especially those with thoracic deformity, are more helpful in diagnosis. The sacral mass can be seen in the barium meal. The larger extraintestinal cyst can cause the impression on the attached intestinal wall or push around. The intestinal fistula, a small number of tubular deformities connected to the intestinal lumen, can be seen in the sputum into the deformed lumen.
The final diagnosis must be based on postoperative examination.
Differential diagnosis
1. Mesenteric cyst: Generally, it is difficult to identify before operation. The mesenteric cyst is located in the mesentery. The wall of the capsule is very thin. There is no muscular layer. It contains yellow-white lymph fluid. It is separated from the intestinal wall and has a certain distance. The intestinal wall is stripped, but the repeated deformity is not easy to be removed from the intestinal wall, but the nearby common intestinal tube should be removed together, but the repeated deformity is occasionally not adhered to the intestinal tube, and the cyst is lined with the intestinal wall. Epithelium, accompanied by spinal deformity, Rickham called this cyst mesenteric cyst with intestinal wall epithelium, should be identified during surgery.
2. Meckel's diverticulum: this disease can cause intestinal obstruction and gastrointestinal bleeding before surgery, but it is difficult to distinguish with intestinal duplication before operation. The operation can be repeated according to the side of the mesenteric mesenteric margin according to the Meckel's diverticulum. The malformation is located on the lateral edge of the mesentery for identification.
