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Intestinal malrotation 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.
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.
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.
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:
To confirm a diagnosis of intestinal malrotation, patients have various blood tests and diagnostic imaging studies done. These tests include:
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, 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.
Children's Hospital's main campus is located in the Lawrenceville neighborhood. Our main hospital address is:
Children’s Hospital of Pittsburgh of UPMC
One Children’s Hospital Way
4401 Penn Ave.
Pittsburgh, PA 15224
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