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Any blockage of the digestive tract that prevents the proper passage of food is known as an intestinal obstruction. Some causes of intestinal obstruction include a congenital (present at birth) malformation of the digestive tract, hernias, abnormal scar tissue growth after an abdominal operation, and inflammatory bowel disease. These blockages are also called mechanical obstructions because they physically block a portion of the intestine or another part of the digestive tract.

Malrotation is a type of mechanical obstruction caused by abnormal development of the intestines while a fetus is in the mother’s womb. It occurs in one out of every 500 births in the United States and accounts for approximately 5% of all intestinal obstructions. Some children with malrotation have other congenital malformations including:

defects of the digestive system
heart defects
abnormalities of other organs, including the spleen or liver
Some people who have malrotation never experience complications and are never diagnosed. But most children with this condition develop symptoms during infancy, often during the first month of life, and the majority are diagnosed by the time they reach 1 year of age. Although surgery is required to repair malrotation, most children experience normal growth and development once the condition and any problems associated with it are treated and corrected.

What Is Malrotation?
The small and large intestines are the longest part of the digestive system. If stretched out to their full length, they would measure more than 20 feet long by adulthood, but because they are coiled up, they fit into the relatively small space of the abdominal cavity. Malrotation occurs when the intestines don’t “coil” properly during fetal development. The exact cause is unknown.

When a fetus is developing in the womb, the intestines start out as a small, straight tube between the stomach and the rectum. As this tube develops into separate organs, the intestines move for a time into the umbilical cord, which supplies nutrients to the developing embryo.

Around the tenth week of pregnancy, the intestine moves from the umbilical cord into the abdomen. It fits in by making two turns that allow it to lie in a specific position within the abdomen. When the intestine does not make these turns properly, malrotation has occurred.

Malrotation in itself may not cause any problems. However, it may be accompanied by additional complications:

Bands of tissue called Ladd bands may form, obstructing the first part of the small intestine (the duodenum).
After birth, volvulus may occur. This is when the intestine twists on itself, causing a lack of blood flow to the tissue and leading to tissue death. Malrotation is often diagnosed when volvulus occurs, frequently during the first weeks of life.
Obstruction caused by volvulus or Ladd bands are both life-threatening problems. The intestines can stop functioning and intestinal tissue can die from lack of blood supply if an obstruction isn’t recognized and treated.

Signs and Symptoms
One of the earliest signs of malrotation and volvulus is abdominal pain and cramping caused by the inability of the bowel to push food past the obstruction. Infants cannot tell you when their stomachs hurt, but you may notice the following pattern of behavior:

pulling up the legs and crying
stopping crying suddenly
behaving normally for 15 to 30 minutes
repeating this behavior when the next cramp happens
Infants may also be irritable, lethargic, or have irregular stools.

Vomiting is another symptom of malrotation, and it can help your child’s doctor determine where the obstruction is located. Vomiting that happens soon after your baby starts to cry often means the obstruction is in the small intestine; if it’s delayed, it’s usually in the large intestine. The vomit may be bilious (this means it contains bile, which is yellow or green in color) or may resemble your child’s feces.

Additional symptoms of malrotation and volvulus may include:

a swollen abdomen that’s tender to the touch
diarrhea and/or bloody stools (or sometimes no stools at all)
irritability or crying in pain, with nothing seeming to help
rapid heart rate and breathing
little or no urine because of fluid loss
fever
Diagnosis and Treatment
If your child’s doctor suspects volvulus or another intestinal blockage, he or she will order X-rays, a computed tomography (CT) scan, or an ultrasound of the abdominal area.

Your doctor may use barium to see the X-ray or scan more clearly. Barium provides a contrast that can show if the intestine has a malformation and can usually determine where a blockage is located. Adults and older children usually drink barium in a liquid form. Infants may need to be given barium through a tube inserted from their nose into the stomach, or sometimes are given a barium enema, in which the liquid barium is inserted through the rectum.

The specific treatment of malrotation will depend on your child’s age and other health problems. Because malrotation is usually only recognized after a blockage occurs because of volvulus or Ladd bands, your child’s doctor will order corrective treatment immediately.

Any child with bowel obstruction will need to be hospitalized. A tube called a nasogastric (NG) tube is usually inserted through the nose and down into the stomach to remove the contents of the stomach and upper intestines. This keeps fluid and gas from building up in the abdomen. Your child may also be given intravenous (IV) fluids to help prevent dehydration and antibiotics to prevent infection.

Surgery to correct bowel obstruction from malrotation is always necessary and is often performed as an emergency procedure to prevent irreversible, life-threatening injury to the bowel. During surgery, which is called a Ladd procedure, the intestine is straightened out, the Ladd bands are divided, the small intestine is coiled on the right side of the abdomen, and the colon is placed on the left side. Because the appendix is usually found on the left side of the abdomen in cases of malrotation (it is normally found on the right), it is removed. Its altered position would make symptoms of appendicitis difficult to determine in the future.

If the doctor suspects that blood may still not be flowing properly to the intestines (because they don’t look pink and healthy after being untwisted), he or she may perform a second surgery within 48 hours of the first. If the intestine still looks unhealthy at this time, the damaged portion may be removed.

If the baby is seriously ill at the time of surgery, an ileostomy or colostomy will usually be performed. In this procedure, the diseased bowel is completely removed, and the end of the normal, healthy intestine is brought out through an opening on the skin of the abdomen (called a stoma). Fecal matter passes through this opening and into a bag that is taped or attached with adhesive to the child’s belly. In young children, depending on how much bowel was removed, ileostomy or colostomy is often a temporary condition that can later be reversed with another operation.

The doctor will monitor your child’s progress after surgery to make sure she’s developing normally. The majority of these surgeries are successful, although some children have recurring problems after surgery. Recurrent volvulus is rare, but a second bowel obstruction due to adhesions (scar tissue build-up after any type of abdominal surgery) could occur later.

Children who require removal of a large portion of the small intestine can have too little bowel to maintain adequate nutrition (a condition known as short bowel syndrome). They may be dependent on intravenous nutrition for a time after surgery and may require a special diet afterward. Most children in whom the volvulus and malrotation are identified early, before permanent injury to the bowel has occurred, do well and develop normally.

When to Call the Doctor
If you suspect any kind of intestinal obstruction because your child has bilious vomiting, a swollen abdomen, or bloody stools, take her to the emergency room immediately.

Reviewed by: Philip Wolfson, MD
Date reviewed: January 2004
Originally reviewed by: Peter Mattei, MD