Intestinal malrotation and volvulus

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

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  • 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 1 out of every 500 live births in the United States.

Of those children who have malrotation and develop symptoms, most symptoms will occur in the first year of life.

  • Twenty-five to 40 percent of cases are diagnosed in the first week of life.
  • Fifty to 60 percent are diagnosed by the first month of life.
  • Seventy-five to 90 percent are diagnosed by one year of age.
  • The remaining cases (10 to 25 percent) are diagnosed after one year of age.

Some people who have malrotation go through their entire life without having any symptoms and are never diagnosed. Others may not have symptoms until childhood, 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.
  • Barium swallow / upper GI test - a procedure performed to examine the intestine for abnormalities. A fluid called barium (a metallic, chemical, chalky, liquid 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.
  • Barium enema - a procedure performed to examine the intestine for abnormalities. A fluid called barium (a metallic, chemical, chalky, liquid used to coat the inside of organs so that they will show up on an x-ray) 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 life-threatening 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.

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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. A colostomy may be done so that the digestive process can continue. With a colostomy, the two remaining healthy ends of intestine are brought through openings in the abdomen. Stool will pass through the opening (called a stoma) and then into a collection bag. The colostomy may be temporary or permanent, depending on the amount of intestine that needed to be removed.

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 catheters.