Congenital intestinal malrotation

　　1. Etiology

　　The early embryonic intestinal tract is a straight tube with a common mesentery. During the 6th to 10th week of embryonic development, the rapid development of the midgut cannot be accommodated in the slower-developing abdominal cavity, and is pushed into the yolk sac by the rapidly expanding liver, forming a physiological umbilical hernia as most of the midgut protrudes through the umbilical ring. By the 10th to 11th week of embryonic development, the abdominal cavity develops faster and increases in volume, and the midgut returns to the abdominal cavity. With the superior mesenteric artery as the axis, it gradually rotates 270° in a counterclockwise direction, making the duodenojejunal flexure move from right to left, from behind the superior mesenteric artery to the left side, forming the duodenal suspensory ligament. It makes the ileocecal junction turn from left to right in front of the superior mesenteric artery to the upper right abdomen. Afterward, it gradually descends to the right iliac fossa. After the normal rotation is completed, the transverse colon is located in front of the superior mesenteric artery, the ascending and descending colon are attached to the posterior abdominal wall by the colonic mesentery, and the small intestinal mesentery extends from the upper left abdomen diagonally to the lower right abdomen, attaching to the posterior abdominal wall.

　　2. In recent years, some people have cited the illustrative descriptions of Snyder and Chaffin's original work, dividing the midgut into two parts: the duodenojejunal loop and the ileocecal loop. The former rotates from the original position on the right side above the superior mesenteric artery, initially rotating 90° counterclockwise to the lower right, then rotating 90° to the posterior of the artery, and finally rotating 90° to the left side of the artery, forming the duodenal suspensory ligament. The rotation of the latter is also similar, with the loop originally located below the artery, rotating 270° counterclockwise through three 90° rotations to place the transverse colon in front of the artery, moving the cecum to the upper right abdomen, and then gradually descending to the lower right abdomen. With the rotation of the intestine, in the last stage, the mesentery of the midgut fuses with the posterior abdominal wall, and the root of the mesentery runs diagonally from the Treitz ligament to the cecum in the lower right abdomen. In this way, the duodenum, cecum, and ascending colon are all fixed. The mesentery of the small intestine has a relatively wide base attached to the posterior peritoneum. Under this normal anatomical condition, the midgut is not prone to torsion.

　　II. Pathogenesis

　　If the normal rotation process of the intestinal tract is obstructed or abnormal at any stage, it will result in abnormal positioning of the intestinal tract and can lead to various types of intestinal obstruction. The types of malrotation of the intestine during embryogenesis include:

　　1. Non-rotated midgut The midgut does not rotate in any degree counterclockwise when it retreats into the abdominal cavity, with both the small intestine and colon hanging from a common mesentery. The root of the mesentery is arranged in a sagittal plane in front of the spine, often accompanied by umbilical hernia and abdominal cleft deformities.

　　2. Incomplete rotation of the intestine loop stops rotating after 90°, with the small intestine hanging in the right side of the abdominal cavity, the cecum and proximal colon located in the left side of the abdominal cavity, and the vermiform appendix located in the lower left abdomen, which is a common rotational abnormality. The lower part of the duodenum is located on the right side of the mesenteric root, without the duodenojejunal flexure, and the ascending colon is in front of or on the left side of the spine. The duodenum, small intestine, and colon hang from a common free mesentery.

　　3. Malrotation of the intestine type I The intestine loop stops rotating after 180°, with the lower part of the duodenum located behind the mesenteric root, the cecum and ascending colon located in the midline of the abdomen, and there are lobular peritoneal adhesions or bands, crossing in front of the second part of the duodenum and attaching to the posterior wall of the right abdomen. When the proximal colon development stops, the cecum is located on the right side of the spine in front of the duodenum, compressing the duodenum or forming a high-lying cecum.

　　4. Malrotation of the intestine type II, this type is characterized by the reverse rotation of the duodenum, with a complex abnormal rotation process, resulting in various malformations. Initially, the proximal part of the midgut rotates 90° counterclockwise as normal and then no longer rotates in the original direction, abnormally rotating 90° to 180° clockwise from right to left, causing the duodenojejunal flexure to remain in front of the superior mesenteric artery instead of the normal posterior position. At the same time, the ileocecal loop of the distal midgut migrates to the right side and is located in the upper right abdomen in front of the duodenum, forming a high-lying cecum. If the ileocecal loop continues to advance to the lower right abdomen, it will wrap the small intestine and mesenteric vessels in the proximal colonic mesentery, forming a colonic mesenteric hernia. The ascending colon mesentery constitutes the hernial sac, and it may lead to small bowel obstruction due to compression of the hernial orifice or mutual compression of the small intestine within the sac in the future.

　　5. Inverse rotation of the intestine: when the midgut rotates abnormally in a clockwise direction by 180° in type II, the duodenojejunal flexure is in front of the superior mesenteric artery. At the same time, the proximal colon rotates in a clockwise direction to the posterior side of the superior mesenteric artery and the mesentery of the small intestine, and the cecum continues to move downward to the right, causing the middle part of the transverse colon to remain behind the artery. Subsequently, transverse colon obstruction occurs. If the proximal part of the midgut continues to rotate an additional 180° around the superior mesenteric artery in a clockwise direction, the duodenojejunal flexure will move to the left posterior side of the superior mesenteric artery. At this time, the cecum moves and remains in the left abdominal cavity.

　　6. The total mesentery of the ascending colon mesentery is not attached to the posterior abdominal wall, which is a combined abnormality of midgut malrotation. It can also be an isolated symptom of normal intestinal rotation, or it can coexist with intestinal malrotation. At this time, the lower part of the duodenum is located behind the superior mesenteric artery, and the duodenal flexure is located on the left side of the abdomen. The mesentery root forms a thin pedicle when it is the total mesentery, extending fan-shaped from below the pancreas. The ascending colon is close to the right abdominal wall but without adhesion. If the mesentery of the ascending colon is partially adherent to the posterior abdominal wall, the cecum and adjacent ascending colon are free.

　　Combined malformations: accounting for 30% to 62%, half of which are duodenal atresia, others include jejunal atresia, congenital megacolon, mesenteric cysts, etc.

　　7. Abnormal rotation of the stomach and duodenum: due to the reverse rotation of the umbilical ileal loop with the reverse movement of the stomach or abnormal rotation, this type is often accompanied by congenital heart malformations such as tetralogy of Fallot, trilocular heart, or arterial pseudo-artery.

　　Due to the complex process of intestinal rotation abnormality, it causes various pathological manifestations and complications. The following is described according to the frequency of occurrence:

　　When the ileocecal flexure is obstructed and the cecum remains in the upper right abdomen, below the stomach, or in the upper left abdomen, a membranous connective tissue called the mesentery ligament (ladd band) is emitted from the cecum and ascending colon, crossing in front of the second or third part of the duodenum and attaching to the right posterior abdominal wall, causing compression of the duodenum. Sometimes, the cecum itself is fixed in front of the duodenum. Clinically, it is manifested as complete or incomplete duodenal obstruction. The mesentery ligament exists in all patients with intestinal malrotation.

　　More than half of intestinal volvulus can occur with small bowel or midgut volvulus. Due to the lack of attachment of the mesentery of the small intestine, cecum, and ascending colon, they are only connected to the posterior abdominal wall by a narrow mesenteric pedicle at the root of the superior mesenteric artery, and the entire midgut hangs from the thin pedicle of the mesenteric root, making it easy for the intestine to twist around the mesenteric pedicle in a clockwise direction (i.e., in the opposite direction of normal rotation), causing acute high-grade intestinal obstruction. The degree of volvulus varies from 360° to 720°, and multiple tight twists can cause infarction of mesenteric vessels and intestinal necrosis.

　　3, Membranous adhesion at the proximal jejunum: When the rotation of the duodenojejunal flexure is obstructed, causing the duodenum to remain in front of or to the right of the superior mesenteric artery, the proximal jejunum can be seen to be wrapped and adhered by membranous tissue, causing the jejunum to twist and obstruct.

　　Malrotation is one of the more common causes of neonatal intestinal obstruction. In digestive tract malformations, this condition is only slightly less common than hypertrophic pyloric stenosis. This point is very different from China and Western countries, where hypertrophic pyloric stenosis is more than 10 times more common than intestinal malrotation. However, not all patients with intestinal malrotation show symptoms in the neonatal period. Such cases may also be encountered in infants and children, and a few may develop symptoms in adulthood. Intestinal malrotation often leads to the following clinical manifestations: (1) Intestinal torsion; (2) Acute or chronic duodenal obstruction; (3) Intra-abdominal hernia, with an onset age that is not specified, most common in neonates, accounting for about 80% of cases. Some cases may appear symptoms in childhood or adulthood, and a few cases may have no clinical symptoms, and are occasionally discovered during X-ray examination or other surgical procedures.

　　1, Neonatal intestinal malrotation: Within 24 hours after birth, there is normal meconium excretion, and the initial feeding goes well. Generally, a large amount of bilious vomiting suddenly occurs between the 3rd and 5th day. Due to varying degrees of duodenal compression, it can manifest as partial or complete obstruction. In cases of partial obstruction, intermittent vomiting occurs, containing bile, and the abdomen is not distended, with no positive signs. In cases of complete obstruction, vomiting is continuous and frequent, accompanied by dehydration, weight loss, and constipation. If intestinal torsion occurs, the symptoms are more severe, with vomiting coffee-like fluid or blood, and hematochezia, indicating that intestinal strangulation, necrosis, or perforation has occurred. Peritonitis, fever, dehydration, and toxic shock symptoms may occur when peritonitis, fever, dehydration, and toxic shock symptoms occur.

　　About 20% of cases of neonatal intestinal malrotation may be accompanied by jaundice, the cause of which is unclear. It may be due to obstructive jaundice caused by the expanded stomach and duodenum compressing the common bile duct; or due to compression of the portal vein and mesenteric vein, resulting in reduced blood flow. This leads to compensatory increase in hepatic artery blood flow, causing unprocessed indirect bilirubin to return to the circulation. At the same time, due to the reduction in portal vein blood flow, the liver cells become hypoxic, and the formation of the enzyme system is affected.

　　2, Malrotation of the intestine in infants and children: Due to different pathological malformation variations, it manifests as various degrees of intestinal obstruction symptoms, most of which are intermittent vomiting containing bile, which often resolves spontaneously. Repeated attacks may occur, and a few children may have no symptoms after birth, but may develop severe abdominal pain, frequent vomiting, constipation, and symptoms of complete intestinal obstruction due to sudden intestinal torsion. In addition, due to recurrent episodes of partial intestinal obstruction, the intestine may become ischemic, affecting the absorption and transport of nutrients by the intestinal mucosa. Reports indicate that 70% of infants and children with this condition have varying degrees of malnutrition. Therefore, it is recommended that children with growth and development disorders and gastrointestinal symptoms must undergo gastrointestinal X-ray examination to rule out malrotation of the intestine.

　　All newborns with the symptoms of high intestinal obstruction who have had normal meconium excretion should be considered for the diagnosis of intestinal malrotation. Newborn intestinal malrotation has similar clinical symptoms to duodenal atresia or stenosis, annular pancreas, and there is a possibility of coexistence, making it difficult to differentiate. Therefore, if there is clear evidence of duodenal obstruction, it is not advisable to perform excessive examinations, and surgery should be explored as soon as possible.

　　Measure 1: Avoid fever and colds in the early stages of pregnancy. Women who have had a high fever in the early stages of pregnancy may have adverse effects on the development of brain tissue in their children, even if there are no obvious external deformities. This can manifest as low intelligence, poor learning and response abilities, and this low intelligence is irreversible. Of course, fetal deformities caused by high fever are also related to the sensitivity of pregnant women to high fever and other factors.

　　Measure 2: Avoid close contact with cats and dogs. Few people know that infected cats are also a major source of infectious diseases that can cause fetal deformities, and cat feces are the main route of transmission for this malignant infectious disease.

　　Measure 3: Avoid women who wear heavy makeup daily. Surveys show that the incidence of fetal deformities in women who wear heavy makeup daily is 1.25 times higher than that of those who do not. The main adverse effects on the malformed development of the fetus are toxic substances such as arsenic, lead, and mercury contained in cosmetics, which affect the normal development of the fetus. Secondly, some components of cosmetics can produce carcinogenic aromatic amines after being exposed to ultraviolet light from the sun.

　　Measure 4: Avoid stress during pregnancy. The human mood is controlled by the central nervous system and the endocrine system, and one of the endocrine hormones, adrenal cortical hormone, is closely related to mood changes. When pregnant women are stressed, adrenal cortical hormones may hinder the integration of certain tissues of the embryo, and if this occurs during the first three months of pregnancy, it can cause fetal cleft lip or cleft palate and other deformities.

　　Measure 5: Avoid drinking alcohol. Alcohol can pass through the placenta to the developing embryo and cause severe harm to the fetus. This can result in a small head, extremely small ears and nose, and a thick upper lip, among other things.

　　Measure 6: Avoid eating moldy food. Experts point out that if pregnant women consume food contaminated with mycotoxins (moldy food), mycotoxins can pass through the placenta and harm the fetus, causing chromosomal breakage in fetal cells.

　　Personal experience of cleft lip surgery: I finally got rid of my cleft lip! I was born with a cleft lip deformity, and now I have finally had the cleft lip surgery and started my different life.

　　1. X-ray examination

　　(1) Abdominal erect flat film: Newborns may show enlargement of the stomach and duodenum or present with double bubble sign, reduced gas content in the small intestine, and obvious expansion of the intestinal lumen with stepped liquid levels suggesting the possibility of intestinal loop obstruction or intestinal necrosis.

　　(2) Barium enema examination: Traditional barium enema examination shows abnormal positioning of the cecum or colon, which is the X-ray characteristic of this disease.

　　① Abnormal rotation of the intestine Type I and II: The cecum is located below the liver, in front of the duodenum, below the stomach, or in the left hypochondriac region. Sometimes the cecum loops around the upper right abdomen and then turns back to stay in the left hypochondriac region.

　　② Non-rotation of the intestine: The entire or most of the colon is located in the left abdominal cavity and overlaps each other. The cecum is located in the lower left abdomen, and the ileum enters the cecum from right to left. When the appendix is imaged, it hangs down from the right side of the cecum.

　　③ Reverse rotation of the intestine: When barium passes through the middle of the transverse colon, it shows a sawtooth or zipper-like extrinsic narrowing. The ends of the narrowed colon may maintain normal shape or there may be dilation of the hepatic flexure and ascending colon. Sometimes the entire colon is particularly long, or the right half of the colon is free.

　　④ Normal cecum position does not exclude malrotation of the intestine: Patients with malrotation of the intestine may show a normally positioned cecum. This is because the rotational movement of the duodenojejunal flexure is受阻, while the ileocecal loop still rotates normally, and the cecum is in a normal position. In some cases, the cecum and ascending colon are free, and the gravitational effect of barium injection into the cecum during barium enema causes the cecum to enter the right iliac fossa. When this situation occurs, one should not make a hasty diagnosis and should conduct further examinations.

　　(3) Upper gastrointestinal contrast examination: Malrotation of the small intestine always involves abnormal positioning of the duodenum, duodenojejunal flexure, and jejunum, and upper gastrointestinal contrast examination is more diagnostically valuable. Neonates should use iodine oil contrast. Torres (1993) believes that the sensitivity rate of upper gastrointestinal contrast examination for diagnosing malrotation is 95%, and the diagnostic accuracy is 86%. Upper gastrointestinal contrast examination in neonates can also reveal concomitant anomalies in the duodenum. During the examination, it is important to observe the passage of contrast medium or barium through the duodenum until it reaches the entire jejunum to avoid missed diagnosis. The contrast examination may show the following manifestations.

　　① Abnormal duodenum: A. The second or third part of the duodenum shows an extrinsic narrowing, with slow passage or retention of barium, and dilation of the stomach and the first part of the duodenum; B. The horizontal line of the third part of the duodenum and the fourth part are absent.

　　② The duodenojejunal flexure is displaced to the front or right side of the spine: directly below the third part of the duodenum is the beginning of the jejunum, so the duodenojejunal flexure disappears, and the beginning segment of the jejunum is twisted in a corkscrew shape, indicating small bowel torsion.

　　③ All of the small intestine is located on the right side of the abdominal cavity.

　　2. Abdominal ultrasound examination is of great diagnostic value for locating the superior mesenteric artery and vein. During the embryonic period, the midgut rotates normally around the superior mesenteric artery (SMA) as the axis. The position of the artery remains constant during intestinal rotation, while the superior mesenteric vein (SMV) rotates with the midgut. The SMV is scanned at the level below the head of the pancreas, showing a normal B-ultrasound image. SMA is visible behind the body of the pancreas, presenting as a circular echo on the anterior side of the abdominal aorta, while the SMV is oval-shaped and located in front of the SMA on the right side and in front of the inferior vena cava. When there is malrotation of the small intestine, the ultrasound examination shows that the SMV is not in front of the inferior vena cava but is displaced to the anterior side of the abdominal aorta, in the direct or left anterior position of the SMA.

　　This method was first reported by Gaines (1987). Ahmad (1993) performed ultrasound examinations on 19 patients with intestinal malrotation before surgery, all showing displacement of the superior mesenteric vein. 18 cases showed displacement to the left anterior side of the SMA, and 1 case to the anterior side of the artery. All 19 cases were confirmed by surgery, but only 9 were diagnosed with intestinal malrotation in the routine preoperative X-ray examination. Ahmad also used 20 cases of other gastrointestinal diseases as controls, all showing normal SMV positions.

　　3. CT and MRI diagnosis CT and MRI have the same significance as ultrasound in the localization of superior mesenteric vessels. Nichols and Li (1983) performed pancreatic CT scans on 3 adult patients misdiagnosed with chronic pancreatitis and found that during the transverse scan at the level of the left renal vein entering the inferior vena cava, the superior mesenteric vein (SMV) was clearly rotated to the left anterior side of the superior mesenteric artery (SMA), which is called the superior mesenteric vein rotation sign. These 3 patients were then re-examined with X-ray and confirmed to have intestinal malrotation. Subsequently, Shatzkes et al. (1990) obtained similar conclusions from CT and MRI examinations of other adult cases.

　　The efficacy of honey:Skin care and beauty, antibacterial and anti-inflammatory, promoting tissue regeneration, promoting digestion, enhancing immunity, promoting longevity, improving sleep, protecting the liver, anti-fatigue, promoting the growth and development of children, protecting the cardiovascular system, moistening the lungs and relieving cough, etc. The bamboo charcoal cake skin is made from eggs, all-purpose flour, bamboo charcoal powder, fine sugar, honey, and bamboo charcoal cake skin. The bamboo charcoal cake skin is made from eggs, all-purpose flour, bamboo charcoal powder, fine sugar, honey, etc.

　　I. Treatment

　　Patients without symptoms should not undergo surgery and should be observed. Obstruction symptoms or acute abdominal pain attacks are indications for surgery, and early surgical treatment should be performed after necessary preoperative preparations. The presence of intestinal bleeding or signs of peritonitis suggests volvulus and must be treated as an emergency.

　　1. Preoperative preparation

　　(1) For newborns with acute high-level intestinal obstruction accompanied by dehydration, rapid fluid replacement and administration of an appropriate amount of plasma are necessary to correct acid-base imbalance. Symptoms such as hematochezia, abdominal distension, and peritoneal irritation suggest volvulus, and immediate surgery should be prepared actively within 1 to 2 hours.

　　(2) For patients with chronic incomplete obstruction accompanied by malnutrition and anemia, the preoperative preparation should last 3 to 5 days, with daily gastric lavage, intravenous infusion of plasma or amino acids. Anemia patients should be given whole blood to correct anemia and malnutrition, and surgery should be performed as soon as possible after correcting these conditions.

　　(3) The surgeon must be familiar with the various pathological and complication characteristics of this malformation before performing surgery.

　　2. The principle of surgery is to relieve obstruction and restore the patency of the intestines.

　　The operation can be performed through a transverse rectus muscle incision, a right upper abdominal midline incision, or a transverse upper abdominal incision, fully exposing the intestinal tract. The surgeon must have a thorough understanding of such anomalies in order to understand the abnormal conditions observed during the operation and to provide proper treatment. Otherwise, he may be at a loss and give incorrect treatment, resulting in persistent symptoms. When judging the condition of the intestinal tract, attention should be paid to the relationship between the lower part of the duodenum and the root of the mesentery, understand the local anatomical position of the proximal colon, and often the entire intestinal tract needs to be moved outside the abdominal cavity. After the twisted intestinal tract is realigned in the clockwise direction, the type of intestinal malrotation can be identified.

　　(1) General mesentery surgery: If the position of the intestinal tract is normal but there is a general mesentery, the cecum and ascending colon are fixed to the right lateral posterior peritoneum of the abdominal cavity, and the serosal layer of the posterior peritoneum of the colon on the posterior side is sutured in an interrupted manner with fine silk thread, and the suture lines need to be closely arranged to prevent abnormal movement of the right half of the colon. The mesentery of the ascending colon starts from the ileocecal valve and斜向 the duodenojejunal flexure, and the lateral and posterior peritoneal mattress sutures are made to fix the mesentery of the colon.

　　(2) Intestinal realignment: By removing all the small intestine from the incision, it can be found that the small intestine is twisted at the root of its mesentery, and the cecum and part of the colon are also twisted along with it, wrapping around the root of the mesentery. The twist is often in the clockwise direction. The twist can reach 360° to 720°. By lifting all the small intestine with the hands, rotating the mesentery of the small intestine in the opposite direction of the twist can achieve complete realignment. At this point, the color of the small intestine improves, and the lumen is aerated, but the duodenal obstruction has not been completely relieved.

　　(3) Cecum release surgery: After the above small intestine is realigned, the cecum can be seen in the upper right abdomen, covering the duodenum, or the peritoneal band connecting the cecum and colon compresses the second and third parts of the duodenum, causing duodenal obstruction. Therefore, it is necessary to perform cecum release surgery, cut the peritoneal band close to the right side of the cecum, and free the cecum and colon to the left, so that the covered duodenum can be exposed (this method is also known as Ladd surgery). Sometimes, there are also hyperplastic fibrous bands near the duodenojejunal junction that compress the duodenum, causing adhesion and twisting, which must also be cut. Only in this way can the duodenal obstruction be completely relieved, and the cecum can be placed as close to the right abdomen as possible, and the abdominal cavity can be closed after the duodenum is completely exposed.

　　In front of the root of the mesentery, the cecum and ascending colon are fixed to the right parietal peritoneum of the abdomen, and the lower part of the duodenum in front of the mesenteric vessels is moved to the right side of the abdomen to prevent compression, relieve the venous stasis of the mesentery caused by the reversal, and restore the patency.

　　(4) The first step in the reversal of intestinal rotation surgery is to clarify the pathological basis of the intestinal reversal anomaly. At this time, the superior mesenteric artery is located in front of the transverse colon, compressing the middle part of the transverse colon, and the duodenojejunal flexure is again located in front of the superior mesenteric artery. During the correction, the twisted intestinal tract should be rotated counterclockwise by 360° to flip the transverse colon behind the artery to the front of the superior mesenteric artery. To prevent the flipped transverse colon from compressing the duodenum, it is necessary to first release all the adhesion bands near the duodenojejunal flexure, straighten the duodenum, and move it to the right abdominal cavity along with the beginning of the jejunum. After the correction, the cecum and ascending colon are fixed to the right lateral posterior peritoneum. There are reports that if it is difficult to flip the intestinal tract, a short-circuit anastomosis between the ileum and transverse colon can be performed to relieve the obstruction of the transverse colon.

　　(5) Mesenteric hernia surgery Colon mesenteric hernia may be accompanied by incomplete rotation of the colon loop, with the cecum and ascending colon remaining in the upper abdomen. Ladd surgery should be performed first, freeing and moving the cecum and ascending colon to the left, exposing the hernia sac orifice behind them. Carefully cut the margin of the hernia sac at a non-vascular site, enlarge the hernia orifice, remove the small intestine inside, straighten it, close the hernia sac orifice, and suture and fix the hernia sac wall to the posterior peritoneum. Since the anterior margin of the hernia sac wall of the colon mesenteric hernia has the course of the colonic artery, it is strictly forbidden to arbitrarily cut the sac wall or resect the hernia sac when restoring the small intestine inside, to avoid vascular injury.

　　According to the follow-up results of most scholars, the surgical efficacy is still good. Although the mesentery of the small intestine is still free, theoretically, there is a possibility of recurrence of intestinal volvulus, but clinical follow-up has shown that recurrence is rare. However, intermittent abdominal pain may persist, with stubborn digestive and absorptive disorders, leading to anemia and low plasma protein levels. The nutritional absorption disorders after resection of necrotic intestinal tract depend on the length and function of the remaining intestinal tract. The majority of death cases are associated with other malformations.

　　II. Prognosis

　　The surgical treatment of simple intestinal malrotation is satisfactory. In cases of combined intestinal volvulus without necrosis, chronic abdominal pain, dysfunctions of digestion and absorption, anemia, and malnutrition may persist after surgery. Short bowel syndrome may occur after extensive resection of the small intestine, and life must be maintained for a long time with parenteral nutrition. Some cases may transition to oral elemental diet and gradually recover, but some cases may require short bowel corrective surgery, which all come at a considerable cost. Premature infants, those with other severe malformations, and extensive intestinal necrosis are the main causes of death, with a mortality rate of 10% to 24%. The key to reducing mortality lies in early diagnosis and correct surgical treatment.