congenital anomaly of intestinal rotation
Introduction
Introduction to congenital intestinal rotation abnormalities Intestinal rotation abnormality refers to the incomplete or abnormal rotational movement of the superior mesenteric artery as the axis during the development of the midgut during the embryonic period, which causes the intestinal position to mutate and the mesenteric adhesion is incomplete, thereby causing intestinal obstruction or volvulus, which is about 6 One in 1,000 newborn babies. Most of them develop symptoms during the neonatal period, and a few develop symptoms in infants or children. The incidence of men is twice as high as that of women. basic knowledge Sickness ratio: 0.0001% Susceptible people: no special people Mode of infection: non-infectious Complications: Hirschsprung's disease Mesenteric cyst
Cause
Causes of congenital intestinal rotation abnormalities
If the normal rotation of the intestine occurs, obstacles or abnormalities occur at any stage, abnormalities in the position of the intestinal anatomy can occur, and various types of intestinal obstruction can occur, resulting in various complicated pathological conditions.
The pathological mechanism of abnormal intestinal position is:
1 embryonic bowel rotation disorder or abnormal rotation, including the umbilical ring is too large, the midgut does not rotate, the rotation is incomplete, the reverse rotation;
2 intestinal dysplasia;
3 The mesentery is not attached, showing the dorsal total mesentery;
4 due to intestinal dysplasia or mesenteric insufficiency, the proximal colon or small intestine fistula continues to rotate to form intestinal umbilical bulge and abdominal fissure deformity.
The types of intestinal rotation abnormalities in the embryonic period are:
(1) The midgut unrotated midgut does not rotate when it is retracted into the abdominal cavity, and remains in the original position. The small intestine and the colon are hung on the common mesentery. The mesenteric root is arranged in the sagittal plane in front of the spine, often accompanied by
(2) Intestinal rotation incomplete intestinal fistula is rotated 90° and then stopped. The small intestine is suspended on the right side of the abdominal cavity. The cecum and proximal colon are located on the left side of the abdominal cavity. The appendix is located in the left lower abdomen. It is a common abnormal rotation and the lower part of the duodenum is not The superior mesenteric artery intersects, and is located on the right side of the mesenteric root. There is no duodenal jejunum. The terminal ileum enters the cecum from the right to the left. The ascending colon is in front of the spine or on the left side. The duodenum, small intestine and colon are overhanged. On the common free mesentery, the development of the colon itself makes the transverse colon transverse, the hepatic curvature of the proximal colon extends to the right at an acute angle, and the duodenum and the proximal colon are coiled.
(3) Abnormal intestinal rotation. The type I intestinal fistula is rotated 180° and then stopped. The lower part of the duodenum is behind the mesenteric root. The cecum and ascending colon are located in the midline of the abdomen. There is a flaky peritoneal adhesion or cord, which spans the twelve fingers. The front of the second part of the intestine is attached to the posterior wall of the right abdomen. When the proximal colon develops, the cecum is pressed against the duodenum on the right side of the spine in front of the duodenum.
(4) Intestinal rotation abnormality type II such as reverse rotation or mixed rotation.
1. After the midgut is rotated 90° in the counterclockwise direction, it is rotated 90° to 180° in the clockwise direction, so that the descending part of the duodenum is located in front of the superior mesenteric artery.
2. The proximal end of the colon moves to the right, and all or part of it is in front of the duodenum and mesentery.
3. When the proximal colon and its mesangium are displaced to the right, the small intestine and mesenteric vessels are wrapped in the mesenteric membrane to form the mesenteric hernia, and the ascending mesenteric membrane constitutes the sac wall, and the small intestine of the capsule may be obstructed.
4. After the midgut rotates 180° clockwise, the transverse colon runs behind the peritoneum, the small intestine and ascending colon are normal, the transverse colon crosses behind, and the lower part of the duodenum is in front, as the midgut continues to rotate clockwise 180 °, the intestine torsion is formed with the mesenteric root as the axis, the cecum is displaced to the left, and the duodenum is located to the right.
(5) The total mesenteric ascending mesenteric membrane is not attached to the posterior wall of the abdomen and is a combined abnormality of the rotation of the midgut. It can also be a single abnormality of normal intestinal rotation. At this time, the lower part of the intestine and duodenum is located behind the superior mesenteric artery. The duodenal curvature is located on the left side of the abdomen. When the total mesentery is present, the mesenteric root forms a fine-handed shape. It protrudes from the lower part of the pancreas and is fan-shaped. The ascending colon is close to the right abdominal wall, but there is no adhesion. If the ascending mesenteric part is adhered to the posterior abdominal wall. The cecum is free from the adjacent ascending colon.
Prevention
Congenital intestinal rotation abnormality prevention
The results of follow-up showed that the surgical treatment was good. Although the small mesentery was still free, it was possible to relapse with intestinal torsion. However, clinical experience has proved that there are rare recurrences, but sometimes intermittent abdominal pain, stubborn digestion and absorption disorder, causing anemia, plasma The protein is low, and the nutrient absorption disorder after removal of the necrotic intestine depends on the length and function of the residual intestinal tube. Most of the death cases have other malformations.
Complication
Congenital intestinal rotation abnormal complications Complications congenital megacolon mesenteric cyst
Combined malformation: 30% to 62%, half of the duodenal atresia, other jejunal atresia, congenital megacolon, mesenteric cyst and so on.
About 20% of cases have hyperbilirubinemia, the cause is still unclear, may be due to dilatation of the stomach and duodenum, compression of the common bile duct, may be due to portal pressure and mesenteric vein compression, blood The flow rate is reduced, and the hepatic arterial blood flow is compensatoryly increased, so that the untreated indirect bilirubin is returned to the circulation. At the same time, the hepatocyte hypoxia and the formation of the enzyme system are affected due to the decrease of portal blood flow.
Symptom
Congenital intestinal rotation abnormal symptoms Common symptoms Intestinal atresia dehydration peritoneal irritation duodenal dilation weight loss constipation shock
All newborns have high intestinal obstruction symptoms, vomit contains a large amount of bile, and those who have had normal fetus discharge should consider the diagnosis of abnormal intestinal rotation. It can be confirmed by X-ray examination. Abdominal plain film can show stomach and duodenum. Enlarged, there is a liquid level, and the small intestine has only a small amount of gas filling. The barium enema is the main diagnostic basis. The location of the cecum can be verified. It can be diagnosed in the upper abdomen or the left abdomen, but the cecum is free or the tincture fills the intestine to make the cecum position. Move down, so the position of the cecum is normal, and the abnormal rotation of the intestine cannot be ruled out. When the intestinal rotation is poor, the duodenal atresia or stenosis and the annular pancreas have high intestinal obstruction and the identification is difficult, it is not suitable for excessive examination. Early surgical exploration should be performed.
In the case of incomplete duodenal obstruction, larger infants and children can be swallowed with a small amount of dilute sputum or lipiodol for examination. It can be seen that the contrast agent stays in the duodenum, only a small amount enters the jejunum, and occasionally the twelve fingers The intestinal jejunum is not perpendicular to the normal curved path, as shown by a complex image of the intestine, suggesting that there is a mid-renal twist.
Lead to three clinical problems:
1 intestinal torsion;
2 duodenal obstruction, acute and chronic;
3 intra-abdominal hernia, the age of onset is uncertain, with more neonates, accounting for about 80% of cases, some cases begin to appear in children or adults, and a few cases have no clinical symptoms, in the X-ray examination or other operations Find.
After the baby is born, normal fetuses are discharged. Symptoms usually appear on the 3rd to 5th day. The symptoms are high intestinal obstruction such as vomiting, intermittent vomiting, bile in the vomit, not fullness in the abdomen, no positive signs, completely When obstructed, vomiting continues and frequent, accompanied by dehydration, weight loss and constipation. If the bowel is reversed, the symptoms are more serious. Vomiting coffee liquid, bloody stool, fever and shock, abdominal distension, peritoneal irritation, must be made early. Diagnosis, timely treatment.
Infants and young children often manifest as duodenal chronic obstruction, the symptoms are intermittent seizures, often relieved, manifested as weight loss, poor nutritional development, acute intestinal obstruction, and urgent treatment.
About 20% of cases have hyperbilirubinemia, the cause is still unclear, may be due to dilatation of the stomach and duodenum, compression of the common bile duct, may be due to portal pressure and mesenteric vein compression, blood The flow rate is reduced, and the hepatic arterial blood flow is compensatoryly increased, so that the untreated indirect bilirubin is returned to the circulation. At the same time, the hepatocyte hypoxia and the formation of the enzyme system are affected due to the decrease of portal blood flow.
Examine
Examination of congenital intestinal rotation abnormalities
X-ray performance:
1 plain film: gastroduodenal inflation, the liquid level is "double bubble sign", small intestinal insufflation; with intestinal torsion, the signs of high intestinal obstruction are particularly obvious, abdominal peritoneal exudation.
2 angiographic examination: gastric dilatation, second and third obstruction of the duodenum, conical or knife-cut at the obstruction end; abnormal position of the duodenum jejunum; near or all of the jejunum in the right upper abdomen, such as the twelve fingers Intestinal and all right abdomen, can be diagnosed poor intestinal rotation, combined with intestinal torsion, the distal part of the duodenum, the upper part of the jejunum spiral twisted "thix-like".
3 enema: colonic hepatic flexion, liter, transverse colon curled in the left abdomen and mid-upper abdomen, cecum free, located in the right upper abdomen, mid-upper abdomen or left abdomen.
Diagnosis
Diagnosis and diagnosis of congenital intestinal rotation abnormality
diagnosis
Can be diagnosed based on clinical symptoms and laboratory tests.
Differential diagnosis
Congenital intestinal atresia and stenosis
Such as anal finger test and warm saline or 1% hydrogen peroxide solution enema still do not discharge normal fetus, can further exclude fecal constipation and congenital megacolon. In the past, the Farber test was used to check the non-keratinized epithelial cells in meconium. The fetal hair is used to diagnose intestinal atresia, and it has diagnostic value for the formation of intestinal atresia within 3 months. However, the middle and late fetuses have no diagnostic significance due to mechanical or vascular-induced intestinal atresia. The abdominal X-ray film is diagnosed. Great value, high intestinal intestine locking position X slice upper flank can be seen 2 to 3 enlarged liquid level, other intestines are not inflated at all, low intestinal obstruction can be seen to enlarge most of the intestines and liquid level, sputum enema can be seen small contracted fetus Type colon, fetal colon characteristics: 1 diameter is about 0.5cm; 2 colonic bag-like folds are not obvious; 3 colon is straight and short, through the barium enema results, except for congenital megacolon and intestinal malrotation.
Congenital megacolon
Delayed delivery of fetuses, intractable constipation, bloating, children with different clinical manifestations due to different length of intestinal tube, the longer the sputum, the sooner the constipation symptoms become more serious, more than 48 hours after birth, no feces are discharged or only a small amount of feces are discharged. In 2 to 3 days, there may be symptoms of low part or even complete intestinal obstruction. Vomiting and bloating do not defecate. If the sputum is not too long, the meconium and gas may be excreted after rectal examination or warm saline enema. Symptoms are relieved. If the segment is not too long, the symptoms of obstruction are not easy to relieve. Sometimes emergency surgery is needed. Constipation and bloating are still relieved after the symptoms of intestinal obstruction are relieved. The anus enema should be used frequently to defecate. In severe cases, the bowel is not defecation, and the abdominal distension is gradually aggravated. .
X-ray findings of the abdominal position of the flat film showed low colonic obstruction, the typical side of the barium enema and anterior and posterior position can be seen in the typical sacral segment and dilated bowel segment, poor sputum function, there are still sputum retention after 24 hours If the elixir is not washed out in time, the vermiculite can be formed. In the case of enteritis, the intestinal wall of the dilated intestine is serrated. In the neonatal period, the dilatation of the intestine is more than half a month after birth, and if it is still undiagnosed. Then perform the following checks.
