Intestinal Malrotation

What is malrotation?

The term “malrotation” means your child’s intestines are not in the normal position. When babies are developing, their intestines begin as a short straight tube. During the first few months of pregnancy, the intestines start to grow longer. As they grow, they first move outside the abdominal cavity and then they return inside the abdomen. When they reach the correct position, they attach to the back of the abdomen in a specific way. This holds them in place. In babies or older children with malrotation, the intestines don’t return and attach in the normal way. About 1 in 500 babies born in the United States have malrotation.

When intestinal malrotation is present several areas of the intestine may be abnormally located. The cecum (the first part of the large intestine) and appendix (attached to the cecum) are normally located in the lower right side of the abdomen. This is why the pain of appendicitis is located in this region. With malrotation, the cecum and appendix can be in the upper right side of the abdomen or even toward the middle of the abdomen. The first part of the small intestine is also abnormally located in the cases of malrotation. More specifically, an area called the “Ligament of Treitz” is in an abnormal location. Bands of tissue called Ladd's bands form between the cecum and the abdominal wall and can create a blockage in the duodenum (the beginning of the small intestine). A volvulus is a serious complication that can occur as a result of intestinal malrotation. When malrotation is complicated by a volvulus, the intestine becomes twisted, causing an intestinal blockage and cutting off blood flow to the intestine. A volvulus can cause severe intestinal damage if not repaired quickly.

Some children with other conditions may be more likely to have malrotation. These include some types of congenital heart defects, heterotaxia (where other organs are not in the normal place), and other problems, such as being born with more than one spleen or none at all. Most children with malrotation have symptoms before they are age one. Some children don’t have symptoms until they are older, and some never have symptoms. Some people live with malrotation their whole life and never know they have it.

Symptoms develop if the intestine becomes blocked (such as by Ladd’s bands or volvulus) or if blood flow to the intestines is cut off (volvulus). The most common symptom is green or yellow vomit (also called bilious because it contains bile, a liquid made in the liver to help digest fats). Green vomiting represents a possible serious problem and your doctor should be made aware that it has occurred. 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. When infants experience this cramping they may: pull up their legs and cry, a swollen abdomen that's tender to the touch, stop crying suddenly and behave normally for 15 to 30 minutes and repeat this behavior when the next cramp happens. Infants also may be irritable, lethargic, or have irregular stools diarrhea and/or bloody stools (or sometimes no stools at all).

Children with malrotation who have no symptoms may never have their condition diagnosed. It may be found by accident when they have an X-ray or surgery for some other reason, maybe not until they are adults. If your child has symptoms, your child’s doctor will ask for a detailed history of your child’s illness. The doctor will do a thorough exam. To confirm a diagnosis of intestinal malrotation your child may have a variety of diagnostic imaging studies done:
  1. Abdominal x-ray: This test can reveal any intestinal obstruction but is not very specific in terms of diagnosis. 
  2. Upper GI series: X-rays are taken of the upper digestive tract from the mouth to the small intestine. During the upper GI series, your child swallows a fluid that can be seen with x-rays. This liquid coats the insides of the upper digestive organs and allows the radiologist to assess the location of the specific areas of the intestine that are affected by malrotation. 
  3. Contrast enema: Contrast fluid is inserted via the anus into the rectum. It coats the inside of the lower digestive organs so they show up on an x-ray. The position of the colon and specifically the cecum is assessed.

How is malrotation and intestinal volvulous treated?

Malrotation of the intestines is not usually evident until the intestine becomes twisted (volvulus) or obstructed by Ladd's bands and symptoms are present. A volvulus is considered a life-threatening problem, because the intestine can die when it is twisted and does not have adequate blood supply.

Your child may be started on IV (intravenous) fluids to treat dehydration and antibiotics to prevent infection. A tube called a nasogastric (or NG) tube will be guided from the nose, down the throat and esophagus, to the stomach to prevent gas and fluid buildup in the stomach. As soon as possible, surgery is performed to untwist the intestine. If it is not damaged, the intestinal circulation may be restored after it is untwisted.

The surgery is done under general anesthesia. The surgeon makes a small cut (incision) above the belly button and brings the intestines out through this opening. If the intestine is healthy, an operation called the Ladd's procedure is performed to repair the malrotation. During the Ladd’s procedure the intestine is placed in a way that will minimize the change of future twist and since the appendix is not in its normal location it is removed.

If your child has intestinal malrotation with a volvulus, the intestine is carefully untwisted. During the surgery, the surgeon checks the health of the intestines. The surgeon looks for a pink color in the tissue, which shows that blood flow is good. If the tissue looks healthy, the surgeon folds the small intestine into the right side of the belly (abdomen) and the large intestine (colon) into the left side of the belly. The intestines are placed so they are not likely to twist in the future.

The new location of the intestines is not intended to be the same as the normal placement. Therefore your child’s appendix (a small organ attached to the colon) would not be in the usual place. If your child ever developed appendicitis, it might be difficult to diagnose. For this reason your surgeon will remove your child’s appendix during surgery for malrotation.

If it’s not clear that the intestine has good blood flow, your child may need another surgery within 24 to 48 hours to recheck the intestine. If a segment of intestine does not look healthy, the surgeon may remove that segment. Part of the intestine may be damaged or the tissue may die if it isn’t getting good blood flow. If a segment of your child’s intestine is damaged, the surgeon will remove that segment. The goal is to keep as much healthy intestine as possible. Surgeons cut on each side of the damaged segment and take it out. If the rest of the intestine looks healthy, they sew the two ends of the intestine together (primary anastomosis). If it does not look healthy, an ostomy may be needed, to allow the intestine to heal. With an ostomy, the healthy end of intestine is brought through an opening in the abdomen. Stool will pass through the opening (called a stoma) and then into a collection bag. The ostomy may be temporary or permanent (uncommon), depending on the amount of damage.

Even if your child has had no symptoms of malrotation, talk with your child’s surgeon about whether your child should have surgery to correct it. Nearly all children need surgery to prevent serious problems later. Your child’s surgeon can explain the risks and benefits and help you decide what’s best for your child.

What will happen in the hospital?

After obtaining imagining studies in the pediatric emergency department and confirming the diagnosis of malrotation your child will be admitted to the Pediatric Surgery service. You will meet your surgeon to discuss the planning of your surgery and consent to perform the operation will be obtained. Often a nasogastric tube (NG) will be placed while in the emergency department to empty the child’s stomach and most children will also need an IV and IV (intravenous) fluids to treat dehydration and to give them nutrients; this will be part of your child’s care before, during and after surgery. Your child will also be given IV antibiotics to prevent infection. After surgery your child is usually admitted to the Pediatric Surgery Unit 7-2 for further post-operative care but may need to stay in the Pediatric Intensive Care Unit for closer monitoring if it is felt necessary.

When will my child be discharged?

The length of hospital stay for children with malrotation is usually about a one to two weeks. This depends on factors like how quickly the intestine recovers from surgery and how soon the child can start eating again.

What will be my child's recovery?

After surgery, your child will be in the recovery room for about an hour and then go to a room in the hospital. Some children who are quite ill stay in our intensive care unit (ICU) or neonatal ICU (NICU), where they get the high level of care they need. When they’re ready they will transfer to the Pediatric Surgery Unit.

After surgery, your child will get all their fluids or nutrition through an IV (intravenous) line. They need this because their intestine goes to sleep, in a sense, after surgery. It takes a few days for the intestine to work again. After your child starts passing gas, the surgeons will know the intestine is working again and feedings may be slowly started at the discretion of your surgeon. Immediately after surgery your child will be given IV pain medications to make them comfortable.

Most often over the counter pain relievers such as Children’s Tylenol or Children’s Advil are adequate for pain control after the first several days. The steri-strips that are placed across the cut should remain in place for at least ten days after the surgery. Your child may shower but should not tub bathe or swim for two weeks until the incisions have healed or until your surgeon allows these activities. Your child should avoid vigorous activity for two weeks. When your child goes home, the surgeon will schedule a follow-up visit two weeks after the surgery to make sure the incision is healing and that all else is well.

What should I be looking out for after the operation?

In the first two weeks after surgery it is important to monitor for signs and symptoms of infection such as redness and swelling around the surgical site or fever. As with all operative procedures the most common complication is potential for infection of the surgical site. It is also important to monitor your child for stomach pain and swelling, vomiting, or inability to pass stool.

The majority of children with malrotation who experienced a volvulus do not have long-term problems if the volvulus was repaired promptly and there was no intestinal damage. If the child has a volvulus, the outcome depends on how damaged the intestine is and how much is removed. Children with intestinal injury who had the damaged part removed may have some long-term problems that can affect the digestive process. Although most children experience normal growth and development once the condition is treated and corrected. Despite the surgery to prevent the twisting of the intestines, it may rarely occur again. Symptoms that would suggest recurrence of the twisting are failure of passage of stool, abdominal distension, and bilious (green) vomiting. Intestinal obstruction from postoperative scarring could also cause these symptoms. If these symptoms occur seek immediate medical assistance and contact your surgeon.