Intestinal malrotation is a birth defect in which the intestines do not move into their normal location in the abdomen as the fetus is developing. Complications are potentially life-threatening.
The cause of this defect is not known.
Some people have no symptoms, whereas others have vomiting, diarrhea, and abdominal pain and swelling.
The diagnosis is based on different kinds of x-rays.
Intestinal malrotation with a twisted loop of intestine (volvulus) is an emergency that requires surgery.
Malrotation itself can cause blockage of the intestine because of the way bands of supporting tissue are stretched across the intestine.
Because the small intestine is not anchored as it should be, it may also suddenly twist (volvulus), cutting off the blood supply to the intestines, which is an emergency. Volvulus occurs most often in infants (and sometimes is how the underlying malrotation is discovered), but it can occur at any age.
Infants born with intestinal malrotation often have other birth defects. Why the intestines do not move into the correct location in the abdomen is not known. Although malrotation is usually found in infancy or early childhood, it can also be found during adulthood. In some people, malrotation is found incidentally as part of an evaluation for another problem.
(See also Overview of Digestive Tract Birth Defects.)
Symptoms of Intestinal Malrotation
Symptoms can develop during infancy, young childhood, or adulthood.
Some people do not have any symptoms.
People who do have symptoms can suddenly develop vomiting, diarrhea, and abdominal pain and swelling due to blockage of the intestines.
If the blood supply to the middle of the intestine is completely cut off because of a volvulus, sudden, severe pain and vomiting develop.
Bile, a substance formed in the liver, may be vomited, making the vomit appear green. Infants who vomit bile need to be seen by a doctor immediately.
Diagnosis of Intestinal Malrotation
X-rays of the abdomen
Barium x-rays
Sometimes ultrasound or computed tomography (CT) scan
If an infant is vomiting bile, doctors take x-rays of the abdomen to look for the blockage.
X-rays also are taken after barium is placed in the infant's rectum (called a barium enema) or swallowed in a liquid (called an upper gastrointestinal or upper GI series). Barium looks white on x-rays and outlines the digestive tract, showing the contours and lining of the esophagus, stomach, small intestine, and large intestine. Barium x-rays can help doctors see whether the lower intestines are in an abnormal position.
Doctors may also do an ultrasound or CT scan of the abdomen to diagnose malrotation or volvulus.
Treatment of Intestinal Malrotation
Emergency surgery for volvulus
Sometimes surgery for malrotation symptoms but no volvulus
Intestinal malrotation with a volvulus is an emergency that requires immediate surgery. Infants are given fluids by vein (intravenously), and emergency surgery is begun within hours. If not treated rapidly, the defect can result in loss of intestinal tissue or can be fatal.
Doctors may surgically repair the intestine in people who have symptoms but who do not have volvulus.
When malrotation is found incidentally in a person who has no symptoms, doctors may opt to surgically repair it, but this is controversial.
