Intestinal Malrotation

What Is Intestinal Malrotation?

An intestinal malrotation is an abnormality that can happen early in pregnancy when a baby's intestines don't form into a coil in the abdomen. Malrotation means that the intestines (or bowel) are twisting, which can cause obstruction (blockage).

Some kids with malrotation never have problems and the condition isn't diagnosed. But most develop symptoms and are diagnosed by 1 year of age. Although surgery is needed to repair malrotation, most kids will go on to grow and develop normally after treatment.

How Does Intestinal Malrotation Happen?

The 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're folded up, they fit into the relatively small space inside the abdomen.

When a fetus develops 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 into the umbilical cord, which supplies nutrients to the developing embryo.

Near the end of the first trimester of pregnancy, the intestines move from the umbilical cord into the abdomen. If they don't properly turn after moving into the abdomen, malrotation occurs. It happens in 1 out of every 500 births in the United States and the exact cause is unknown.

Some children with intestinal malrotation are born with other associated conditions, including:

• other defects of the digestive system
• heart defects
• abnormalities of other organs, including the spleen or liver

What Problems Can Intestinal Malrotation Cause?

Malrotation can lead to these complications:

• In a condition called volvulus (VAHL-vyuh-lus) the bowel twists on itself, cutting off the blood flow to the tissue and causing the tissue to die. Symptoms of volvulus, including pain and cramping, are often what lead to the diagnosis of malrotation.
• Bands of tissue called Ladd's bands may form, obstructing the first part of the small intestine (the duodenum).
• Obstruction caused by volvulus or Ladd's bands is a potentially life-threatening problem. The bowel can stop working and intestinal tissue can die from lack of blood supply if an obstruction isn't recognized and treated. Volvulus, especially, is a medical emergency, with the entire small intestine in jeopardy.

What Are the Signs & Symptoms of Intestinal Malrotation?

An intestinal blockage can prevent the proper passage of food. So one of the earliest signs of malrotation and volvulus is abdominal pain and cramping, which happen when the bowel can't push food past the blockage.

A baby with cramping might:

• pull up the legs and cry
• stop crying suddenly
• behave normally for 15 to 30 minutes
• repeat this behavior when the next cramp happens

Infants also may be fussy, lethargic, or have trouble pooping.

Vomiting is another symptom of malrotation, and it can help the doctor determine where the obstruction is. Vomiting that happens soon after the baby starts to cry often means the blockage is in the small intestine; delayed vomiting usually means it's in the large intestine. The vomit may contain bile (which is yellow or green) or may resemble feces.

Other symptoms of malrotation and volvulus can include:

• a swollen abdomen that's tender to the touch
• diarrhea and/or bloody poop (or sometimes no poop at all)
• fussiness or crying in pain, with nothing seeming to help
• rapid heart rate and breathing
• little or no pee because of fluid loss
• fever

How Is an Intestinal Blockage Diagnosed?

If volvulus or another intestinal blockage is suspected, the doctor will examine your child and then may order X-rays, a computed tomography (CT) scan, or an abdominal ultrasound.

The doctor may use barium or another liquid contrast agent to see the X-ray or scan more clearly. The contrast can show if the bowel has a malformation and can usually find where the blockage is.

Adults and older kids usually drink barium in a liquid form. Infants may need to be given barium through a tube inserted from the nose into the stomach, or sometimes are given a barium enema, in which the liquid barium is inserted through the rectum.

How Is Intestinal Malrotation Treated?

Treating significant malrotation almost always requires surgery. The timing and urgency will depend on the child's condition. If there is already a volvulus, surgery must be done right away to prevent damage to the bowel.

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. The child may also be given intravenous (IV) fluids to help prevent dehydration and antibiotics to prevent infection.

During the surgery, which is called a Ladd procedure, the intestine is straightened out, the Ladd's bands are divided, the small intestine is folded into 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 when there is malrotation (normally, the appendix is found on the right), it is removed. Otherwise, should the child ever develop appendicitis, it could complicate diagnosis and treatment. 

If it appears that blood may still not be flowing properly to the intestines, the doctor may do a second surgery within 48 hours of the first. If the bowel still looks unhealthy at this time, the damaged portion might be removed.

If the child is seriously ill at the time of surgery, an ileostomy or colostomy usually will be done. 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 (poop) 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, the ileostomy or colostomy is often a temporary condition that can later be reversed with another operation.

Most of these surgeries are successful, although some kids 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 happen later.

Children who had a large portion of the small intestine removed can have too little bowel to maintain adequate nutrition (a condition known as short bowel syndrome). They might need intravenous (IV) nutrition for a time after surgery (or even permanently if too little intestine remains) and may require a special diet afterward.

Most kids in whom the volvulus and malrotation are found and treated early, before permanent injury to the bowel happens, do well and develop normally.

If you suspect any kind of intestinal obstruction because your child has bilious (yellow or green) vomiting, a swollen abdomen, or bloody stools, call your doctor immediately and take your child to the emergency room right away.