Intestinal (Bowel) Malrotation and Volvulus

What Is Intestinal Malrotation and Volvulus?

Intestinal malrotation, also called twisted intestines, occurs when the intestine does not twist correctly. Instead, the way in which it twists blocks the intestine. Sometimes, abnormal tissue referred to as Ladd's bands attaches the cecum to the duodenum (beginning of the small intestine) and may create a blockage in the duodenum.

Our intestines are formed while we are fetuses in the womb, during the tenth week of gestation. As the intestines develop, they normally move from the base of the umbilical cord back into the abdominal cavity. As the intestine returns to the abdomen, it makes two rotations and settles into its normal position.

What Causes Intestinal Malrotation?

When rotation is incomplete and the intestine does not become fixed into that position, this creates intestinal malrotation.

The malrotated intestine is prone to twisting in on its own blood supply, blocking the flow. This is called intestinal volvulus. When intestinal volvulus involves the entire small bowel, it is referred to as mid-gut volvulus.

Who Is at Risk for Intestinal Malrotation?

In the United States, about one of every 500 children is born with intestinal malrotation. Although intestinal malrotation can occur in older children (or even adults), up to 90% of patients are diagnosed by age one – many within the first week after birth. A small minority of people who have intestinal malrotation never experience symptoms, and sometimes live their whole lives without being diagnosed. While many boys with intestinal malrotation develop symptoms earlier, malrotation occurs equally in boys and girls.

Many children with intestinal malrotation also have another congenital (present at birth) problems. These may involve the abdominal wall or the digestive system, the heart, or the liver or spleen.

Volvulus and Intestinal Malrotation Symptoms

Intestinal malrotation is usually not evident until the intestine becomes obstructed by Ladd's bands or twisted. When the intestine is obstructed by Ladd's bands or when the blood supply is twisted, symptoms may include:

  • Vomiting bile (greenish-yellow digestive fluid)
  • Drawing up the legs
  • Pain in the abdomen (belly)
  • Abdominal distention (swelling)
  • Rapid heart rate
  • Rapid breathing
  • Bloody stools
  • Malnutrition
  • Slowed growth

Intestinal Malrotation and Volvulus Diagnosis

To confirm a diagnosis of intestinal malrotation, patients have various blood tests and diagnostic imaging studies done. These tests include:

  • Abdominal X-ray – Reveals any intestinal obstruction.
  • Barium swallow upper GI test – Examines the small intestine for abnormalities and to check the position of the jejunum. A chalky fluid called barium is swallowed or placed into the stomach through a small nasogastric tube. The barium coats the inside of the stomach and intestine so that they will show up on X-rays.
  • Barium enema – Examines the large intestine, and uses the same radiographic contrast agent as mentioned above. Barium is given into the rectum as an enema. X-rays can show that the large intestine is not in normal position.
  • Abdominal ultrasound – Produces moving images of internal organs using invisible electromagnetic energy. Ultrasounds can help doctors evaluate the function of the intestine and monitor the blood flow.

Volvulus and Intestinal Malrotation Treatment

Once intestinal volvulus and/or intestinal malrotation is diagnosed, children begin receiving fluids and antibiotics intravenously. The fluids keep them from becoming dehydrated, and the antibiotics prevent infections. A nasogastric tube is placed from the nose into the stomach to prevent gas buildup in the stomach.

As soon as possible, twisted bowel surgery is performed to untwist the intestine. If it is not damaged too badly, the intestine's circulation may be restored after it is untwisted. If the intestine is healthy, an operation called the Ladd's procedure is performed to repair the malrotation.

If the surgeons are not sure the intestine will receive an adequate blood supply even after untwisting, they may need to perform another operation. This is usually performed within 24 to 48 hours of the first operation. If they find a section of intestine that is damaged so badly it can not be saved, that portion is removed. To allow the intestine to heal, the surgeons sometimes have to create a small bowel stoma, an opening through the abdominal wall to the skin that diverts the body's waste products into a collection bag. The stoma may be removed after several weeks to restore normal intestinal function.

The long-term outcome is generally very good when intestinal malrotation is surgically corrected before intestinal damage occurs. Older children also tend to do well. However, when a large portion of intestine has to be removed because of intestinal injury, the remaining intestine has trouble absorbing nutrients and fluids. The child's regular diet may need to be supplemented or replaced with total parenteral nutrition (TPN). TPN is a high-calorie solution that is given intravenously to bypass the intestine. TPN is very effective, but if it is given over a long period of time, children are at risk for developing chronic liver disease. In a case like this, a child may be considered for an intestinal transplant to protect his or her liver.

Learn more about other Intestine Transplant Disease States.