What is intestinal malrotation?

Intestinal malrotation is a birth defect involving a malformation of the intestinal tract. Intestinal malrotation is an abnormality that occurs while a fetus is forming in its mother's uterus. Illustration demonstrating a volvulus

Click Image to Enlarge As a fetus is growing in its mother's uterus before birth, different organ systems are developing and maturing.

The digestive tract starts off as a straight tube from the stomach to the rectum. Initially, it is located in the fetus' abdomen, but, for a while, part of the intestine moves into the umbilical cord. At about the 10th week of pregnancy, the intestine leaves the umbilical cord and goes back into the abdomen. After returning to the abdomen, the intestine makes two turns, and is no longer a straight tube.

Malrotation occurs when the intestine does not make these turns as it should. In addition, intestinal malrotation causes the cecum (the end of the small intestine) to develop abnormally. The cecum is normally located in the lower right side of the abdomen. With malrotation, the cecum and the appendix (which is attached to the cecum) stay in the upper right side of the abdomen. Bands of tissue called Ladd's bands form between the cecum and the intestinal wall and can create a blockage in the duodenum (the beginning of the small intestine). A volvulus is a problem that can occur after birth as a result of intestinal malrotation. The intestine becomes twisted, causing an intestinal blockage. This twisting can also cut off the blood flow to the intestine, and the intestine can be damaged.

How often does malrotation and volvulus occur?

Intestinal malrotation occurs in one out of every 500 live births in the United States. The majority of children with malrotation develop symptoms within the first year of life. Intestinal malrotation is most often recognized in infancy, as most infants develop symptoms of acute bowel obstruction within the first week of life. Malrotation is rarely seen in older children, and when it does occur, symptoms may be absent or intermittent. Some people who have malrotation go through their entire life without having any symptoms and are never diagnosed. Others may not have symptoms until adolescence, or adulthood.

Which children are at risk for having malrotation?

Malrotation occurs equally in boys and girls. However, more boys become symptomatic by the first month of life than girls. Up to 70 percent of children with intestinal malrotation also have another congenital malformation, including the following:

digestive system abnormalities cardiac abnormalities abnormalities of the spleen abnormalities of the liver

Why is intestinal malrotation a concern?

A child with malrotation is likely to experience a twisting of the intestine known as a volvulus. This will cause an obstruction, preventing food from being digested normally. The blood supply to the twisted part of the intestine can also be cut off, which can lead to the death of that segment of the intestine. Ladd's bands, formed between the cecum and the intestinal wall, can also create a blockage in the duodenum, preventing food from being digested. A child can become dehydrated quickly when intestinal blockage occurs.

What are the symptoms of malrotation and volvulus?

The following are the most common symptoms of malrotation and volvulus. However, each individual may experience symptoms differently. When the intestine becomes twisted, or obstructed by Ladd's bands, the symptoms may include:

vomiting bile (green digestive fluid) drawing up the legs abdominal pain abdominal distention (the abdomen becomes swollen) rapid heart rate

rapid breathing bloody stools

The symptoms of malrotation and volvulus may resemble other conditions or medical problems. Consult your child's physician for diagnosis.

How is malrotation and volvulus diagnosed or evaluated?

In addition to a physical examination and medical history, diagnostic procedures for malrotation and volvulus may include various imaging studies (tests that show pictures of the inside of the body). These are performed to evaluate the position of the intestine, and whether it is twisted or blocked. These tests may include:

abdominal x-ray - a diagnostic test which may show intestinal obstructions. upper GI test - a procedure performed to examine the intestine for abnormalities. A fluid used to coat the inside of organs so that they will show up on an x-ray is swallowed. An x-ray of the abdomen may show an abnormal location for the small intestine, obstructions (blockages), and other problems. contrast enema - a procedure performed to examine the intestine for abnormalities. A fluid is given into the rectum as an enema. An x-ray of the abdomen may show that the large intestine is not in the normal location.

Treatment for malrotation and volvulus:

Specific treatment for malrotation and volvulus will be determined by your child's physician based on the following:

the extent of the problem your child's age, overall health, and medical history the opinion of the surgeon and other physicians involved in your child's care expectations for the course of the problem your opinion and preference

Malrotation of the intestines is not usually evident until the intestine becomes twisted (volvulus) or obstructed by Ladd's bands and symptoms are present. A volvulus is considered a lifethreatening problem, because the intestine can die when it is twisted and does not have adequate blood supply. Children may be started on IV (intravenous) fluids to prevent dehydration and antibiotics to prevent infection. A tube called a nasogastric (or NG) tube may be guided from the nose, through the throat and esophagus, to the stomach to prevent gas buildup in the stomach. A volvulus is usually surgically repaired as soon as possible. The intestine is untwisted and checked for damage. Ideally, the circulation to the intestine will be restored after it is unwound,

and it will turn pink. If the intestine is healthy, it is replaced in the abdomen. Since the appendix is located in a different area than usual, it would be difficult to diagnose appendicitis in the future; therefore, an appendectomy (surgical removal of the appendix) is also usually performed. If the blood supply to the intestine is in question, the intestine may be untwisted and placed back into the abdomen. Another operation will be done in 24 to 48 hours to check the health of the intestine. If it appears the intestine has been damaged, the injured section may be removed. If the injured section of intestine is large, a significant amount of intestine may be removed. In this case, the parts of the intestine that remain after the damaged section is removed cannot be attached to each other surgically. An ostomy may be done so that the digestive process can continue. With an ostomy, the two remaining healthy ends of intestine are brought through openings in the abdomen. Stool will pass through the opening and then into a collection bag. The stoma may be temporary or permanent, depending on the amount of intestine that needed to be removed. Illustration of bowel resection and colostomy

Click Image to Enlarge

Will my child have problems in the future?

The majority of children with malrotation who experienced a volvulus do not have long-term problems if the volvulus was repaired promptly and there was no intestinal damage. Children with intestinal injury who had the damaged part removed may have long-term problems. When a large portion of the intestine is removed, the digestive process can be affected. Nutrients and fluids are absorbed from food in the small intestine. Removing a large segment of the intestine can prevent a child from getting adequate nutrients and fluids. In this case, nutrition may need to be supplemented with long-term, high-calorie IV (intravenous) solutions given through special IV http://www.lpch.org/DiseaseHealthInfo/HealthLibrary/digest/malrotat.html\

Pathophysiology

Embryology of GI tract
A good understanding of the embryologic development of the GI tract is central to the understanding of malrotation and volvulus. At 4 weeks gestation, the GI system is a short, straight tube centrally located in the abdomen. During the fifth week, the first stage of rotation begins and lasts until the tenth week. As this tube grows and lengthens, it must leave the domains of the fetal abdominal cavity and thus herniates into the area of the umbilical cord, carrying its mesentery with its blood supply (ie, the SMA) as a central axis; it is in line with the omphalomesenteric duct, which is located at the apex of the herniation. The prearterial segment includes the developing duodenum, the jejunum, and much of the ileum, whereas the postarterial segment becomes the terminal ileum, the cecum, the ascending colon, and part of the transverse colon. While in this position (partially outside the fetal abdomen), the developing midgut begins to rotate. The DJ loop is first pushed inferiorly and to the right, possibly by the developing liver. Rotation is in a counterclockwise direction and continues in this fashion. This completes the first 90 of rotation. The cecocolic loop follows, moving to the left of the artery. The DJ loop then completes a second 90 of rotation, coming to rest inferior to the SMA. The second stage of rotation occurs at 10-12 weeks gestation and involves the return of the intestine to the fetal abdominal cavity. Upon the return of the prearterial segment, the DJ loop completes another 90 of rotation for a total of 270, coming to rest to the left of the SMA. The cecocolic loop also completes another 90 of rotation, coming to rest superior and anterior to the SMA. The third and final phase of rotation occurs after 12 weeks gestation. The cecum completes its final descent to the right lower quadrant, accomplishing another 90 of rotation for a total of 270. Once this descent is completed, the intestine becomes fixed to the posterior abdominal wall. The DJ junction is fixed to the left of the aorta, anterior to the left renal vein at the ligament of Treitz, and the cecum forms attachments to the right iliac fossa. These 2 points of attachment leave the mesentery and its blood supply broad-based and fixed. At this point, the GI tract has returned to the peritoneal cavity and the abdominal wall of the fetus has completely formed and closed at the level of the umbilicus. Arrest of development at any stage not only changes the anatomic placement of organs but, perhaps most important, narrows the mesenteric base and impairs fixation, leaving the bowel at high risk for volvulus.

Rotational abnormalities
The common rotational abnormalities may be broadly grouped according to the stage at which they occur, as follows:

Arrest in the first phase leaves the bowel herniated into the umbilical cord region, covered by a membrane, creating the so-called omphalocele; this is often accompanied by other congenital abnormalities (eg, cardiac and urogenital defects) Arrest in the second phase may result in nonrotation, incomplete rotation, hyperrotation, or reversed rotation; gastroschisis and diaphragmatic hernias seem to potentially also occur during this period. mesocolic paraduodenal hernias also occur during the late second phase and early third phase Arrest in the third phase results in a mobile cecum, an unattached duodenum, or an unattached small bowel mesentery, which allows cecal volvulus and internal hernias

It is important to note that intestinal malrotation may occur as an isolated event or in association with other types of congenital anomalies as listed above. Nonrotation is the most common abnormality and results from failure of normal counterclockwise rotation around the SMA. Rotation may be completely absent or arrested at 90. The small intestine remains entirely to the right of the artery, with the cecum at or near the midline and the colon in the left abdomen. This variant of malrotation is usually considered benign, given the fact that it may not cause any symptoms unless a volvulus develops. In nonrotation, the proximal jejunum and colon pass very closely to the SMA, leaving a narrow pedicle as the base of the mesentery. This is sometimes called the bell-clapper or the omega configuration and leaves the midgut highly susceptible to volvulus and infarction. In addition, the peritoneal attachments of the cecum pass anterior and lateral to the duodenum, which may lead to some degree of extrinsic duodenal obstruction (a common cause of intermittent bilious vomiting). Incomplete rotation is the next most common rotational abnormality, involving arrest of rotation at or near 180. It is the most important form of malrotation. In this condition, midgut rotation is incomplete, the DJ loop remains to the right of the SMA, and the ileocecal loop comes to lie in the right upper quadrant, anterior to the SMA and closely related to the duodenum. With incomplete rotation, the entire midgut is attached by a very narrow pedicle, consisting mostly of the SMA and the SMV. Twisting or volvulus of this pedicle may occur, leading to ischemia and mechanical obstruction of the intestine, which may lead to intestinal necrosis (see the image below). The ischemia begins with venous congestion, which progresses to arterial occlusion, and, finally, bowel necrosis.

Malrotation and midgut volvulus with intestinal ischemia. Note narrow pedicle at base of mesentery. No resection was required since ischemic necrosis had not yet developed.

As previously described, abnormal cecal peritoneal bands that cross over the duodenum in the right upper quadrant may be found in cases of malrotation. These abnormal cecal attachments are called Ladd bands. In both nonrotation and incomplete rotation, cecal bands may cause obstruction and ischemia before birth, leading to congenital duodenal atresia or stenosis. Therefore, the patency of the duodenum should be checked routinely during corrective surgery. In addition, intermittent antenatal volvulus may cause varying degrees of ischemia, leading to atresia or duplication of parts of the jejunum and ileum. In many cases, the abnormal Ladd bands cause only partial and intermittent obstruction, which can manifest as intermittent episodes of bilious vomiting and/or feeding intolerance. Mixed rotational abnormalities are more uncommon and varied. Hyperrotation may narrow the SMA pedicle or lead to a confusing clinical picture in appendicitis. Failure of descent of the cecum may be inconsequential or may lead to cecal volvulus. Reversed rotation results in a 90 clockwise rotation, leaving the transverse colon behind the SMA and the duodenum anterior to the artery. In this case, the transverse colon may herniate into a mesocolic pocket or become obstructed by the SMA (also described as an internal hernia). Mesocolic paraduodenal hernias occur with failure of fixation to the posterior abdominal wall. Spaces remain between the mesentery and the wall, allowing possible sequestration and strangulation of bowel segments. This occurs more often on the left and is associated with nonrotation. Right-side hernia is associated with congenital short bowel.

Volvulus
Volvulus of the midgut may result in several manifestations, depending on the degree of twisting. Venous and lymphatic obstructions occur first because of lower intravascular pressures. Vascular congestion leads to bowel edema and possible oozing of blood, potentially causing GI bleeding. Lymphatic congestion causes the formation of a mesenteric cyst and/or chylous ascites. It is not uncommon to find chylous fluid in the peritoneal cavity and enlarged lymph nodes at the time of corrective surgery for malrotation and volvulus. If volvulus is intermittent, children may have chronic malabsorption from congestion and edema or intermittent bouts of symptoms, usually vomiting and possibly alternating diarrhea and constipation. Arterial compromise is seen when the twisting is significant enough to occlude venous and arterial vessels. This represents an acute and dangerous event. Sequelae include (in

order of less to more severe) ischemia, mucosal necrosis, intramural air formation, bacterial translocation, gram-negative sepsis, full-thickness intestinal wall necrosis, perforation, peritonitis, and death.

Other congenital abnormalities

Malrotation is often associated with other congenital abnormalities, and, in some regions, as many as 62% of cases are associated with these abnormalities. This is important for several reasons. First, some abnormalities (eg, polysplenia, asplenia, and congenital abdominal wall defects) are commonly associated with malrotation, and their presence should automatically lead to an investigation for malrotation. Patients with congenital wall defects, by definition, have some degree of malrotation, which should be evaluated during correction of the defect. Rotational anomalies in conjunction with dextroisomerism or levoisomerism should point to possible polysplenia or an asplenia syndrome. These syndromes have devastating effects on outcomes, especially asplenia. Second, and perhaps most important, mortality related to malrotation and its correction is strongly correlated with the presence or absence of other congenital abnormalities. Serious anomalies may raise the risk of mortality 22 times. Physicians treating patients with malrotation must be aware of the possibility of other associated congenital abnormalities. Developmental abnormalities such as gastroschisis, omphalocele, and congenital diaphragmatic hernia are associated with displacement of the abdominal contents outside the domain of the abdominal cavity. Since such developmental abnormalities occur at the time of fetal intestinal growth and rotation, they typically result in intestinal malrotation. Malrotation has also been described in also association with duodenal atresia, Meckel diverticulum, intussusception, small bowel atresia, prune belly syndrome, gastric volvulus, persistent cloaca, Hirschsprung disease, and extrahepatic biliary anomalies.
How is malrotation treated? A child with volvulus is usually dehydrated and has a rapid heart rate. IV fluids will be needed immediately with antibiotics. A nasogastric tube will be placed through the childs nose into the stomach to decompress or allow the fluids backing up into the stomach to empty. An exploratory laparotomy (surgery) will be performed to take a look at the bowel. The bowel will be detorsed (unwound) and checked carefully (see pictures). The bowel that turns pink (showing returned circulation) after torsion is good bowel. If all bowel turns pink, a Ladds procedure will be performed to put the bowel in place to prevent another volvulus. An appendectomy is usually done since the appendix will not be located in the normal area in the abdomen. This could lead to confusion and delay in diagnosing appendicitis in the future. If there is a question about the bowels viability, the abdomen will be left open and a second-look procedure will be planned within 24 to 48 hours. If there is a section of necrotic (dead) bowel a colostomy may be needed temporarily. The ostomy nurse will consult with you on ostomy care.

The operation done to repair malrotation is called Ladd's procedure. In Ladd's procedure, the abdomen is opened (Diagram A). The small intestines are seen first and appear to hide the colon. The entire intestinal mass is delivered out of the abdomen (Diagram B). The intestinal mass is rotated to reduce the volvulus (Diagram C). The intestines are re-positioned in the abdomen (Diagram D). Diagram E shows the appearance of the intestines at the end of surgery.