Intestinal Malrotation Symptoms & Causes

What causes intestinal malrotation?

As a fetus grows in its mother's uterus, its organ systems are developing and maturing. The digestive tract starts as a straight tube from the stomach to the rectum; for a while, a part of the intestine protrudes from the abdomen into the umbilical card. But once the fetus reaches 10 weeks, the intestine separates from the umbilical cord, returns to the abdomen and makes two turns, so that it is no longer a straight tube.

Malrotation occurs when the intestine does not make these turns as it should.

What complications may result from malrotation?

Intestinal malrotation causes the end of the small intestine, called the cecum, to develop abnormally, which leads to abnormal positioning.

Normally, the cecum is located in the lower right side of the abdomen. With malrotation, the cecum and appendix (which is attached) remain in the upper right side of the abdomen. Bands of tissue form between the cecum and the intestinal wall and can create a blockage in the beginning of the small intestine.

Intestinal Malrotation
Intestinal malrotation with volvulus
(click to enlarge)

A volvulus is a problem that can occur after the baby is born as a result of malrotation: The intestine becomes twisted, causing an intestinal blockage or cutting off blood flow to the intestine.

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 baby's first year of life.

  • 25 to 40 percent of babies are diagnosed in the first week of life.

  • 50 to 60 percent of babies are diagnosed by the first month of life.

  • 75 to 90 percent of babies are diagnosed by age 1.

  • 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 30 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?

Yellow or green vomit is an early sign of malrotation. If your child has this kind of discolored vomit, she should be evaluated immediately by a doctor. If your child's intestine becomes twisted or obstructed, her symptoms may include:

  • vomiting bile
  • drawing up the legs
  • abdominal pain
  • abdominal swelling or distention
  • rapid heart rate
  • rapid breathing
  • bloody stools

Will my child have life-long problems, even after treatment?

The majority of children with malrotation who experienced a volvulus do not have long-term problems if the volvulus is repaired promptly and they have 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.