Intestinal malrotation is a birth defect involving a malformation of the intestinal tract; it occurs while a fetus is forming in its mother's uterus.
As a fetus grows in its mother's uterus before birth, different organ systems develop and mature. The digestive tract starts off as a straight tube from the stomach to the rectum. Initially, it is located in the fetus' abdomen, but, for a short time, part of the intestine moves into the umbilical cord.
At about the 10th week of pregnancy, the intestine leaves the umbilical cord and re-enters the abdomen. At that point, the intestine makes two turns and is no longer a straight tube.
Malrotation occurs when the intestine does not make these turns as it should.
In addition, intestinal malrotation causes the cecum (the end of the small intestine) to develop abnormally. The cecum is normally located in the lower-right side of the abdomen. With malrotation, the cecum and the appendix (which is attached to the cecum) stay in the upper-right side of the abdomen. Bands of tissue called Ladd's bands form between the cecum and the intestinal wall and can create a blockage in the duodenum (the beginning of the small intestine).
A volvulus occurs after birth as a result of intestinal malrotation. The intestine becomes twisted, causing an intestinal blockage. This twisting can also cut off the blood flow to the intestine, and the intestine can be damaged.
Intestinal malrotation occurs in one out of every 500 live births in the United States. Malrotation occurs equally in boys and girls. However, more boys become symptomatic by the first month of life than girls.
The majority of children with malrotation develop symptoms within their first year of life. Intestinal malrotation is most often recognized in infancy, as most infants develop symptoms of acute bowel obstruction within the first week of life. Malrotation is rarely seen in older children, and when it does occur, symptoms may be absent or intermittent. Some people who have malrotation go through their entire life without having any symptoms and are never diagnosed. Others may not have symptoms until adolescence or adulthood.
Up to 70% of children with intestinal malrotation also have another congenital malformation, including the one or more of the following:
A child with malrotation is likely to experience a twisting of the intestine known as a volvulus. This will cause an obstruction, preventing food from being digested normally and dehydration to occur. The blood supply to the twisted part of the intestine also may be cut off, which can lead to the death of that segment of the intestine.
Ladd's bands, formed between the cecum and the intestinal wall, also may create a blockage in the duodenum, preventing food from being digested.
The following are the most common symptoms of malrotation and volvulus. However, each individual may experience symptoms differently. When the intestine becomes twisted or obstructed by Ladd's bands, the symptoms may include:
The symptoms of malrotation and volvulus may resemble other conditions or medical problems. Consult your child's physician for a diagnosis.
In addition to a physical examination and medical history, diagnostic procedures for malrotation and volvulus may include various imaging studies (tests that show pictures of the inside of the body). These are performed to evaluate the position of the intestine, and whether it is twisted or blocked. These tests may include:
This is a diagnostic test that may show intestinal obstructions.
This procedure is performed to examine the intestine for abnormalities. A fluid called barium (a metallic, chemical, chalky liquid used to coat the inside of organs so that they appear on an X-ray) is swallowed. An X-ray of the abdomen may show an abnormal location for the small intestine, obstructions (blockages) and other problems.
This procedure is also performed to examine the intestine for abnormalities. The barium fluid is given into the rectum as an enema. An x-ray of the abdomen may show that the large intestine is not in the normal location.
Specific treatment for malrotation and volvulus will be recommended by your child's physician based on the following:
Children may be started on IV (intravenous) fluids to prevent dehydration and antibiotics to prevent infection. A tube called a nasogastric (NG) tube may be guided from the nose through the throat and esophagus to the stomach to prevent gas buildup in the stomach.
A volvulus is usually surgically repaired as soon as possible. The intestine is untwisted and checked for damage. Ideally, the circulation to the intestine will be restored after it is unwound, and it will turn pink.
If the intestine is healthy, it is replaced in the abdomen. Since the appendix is located in a different area than usual, it would be difficult to diagnose appendicitis in the future; therefore, an appendectomy (surgical removal of the appendix) is also usually performed at this time.
If the blood supply to the intestine is in question, the intestine may be untwisted and placed back into the abdomen. Another operation will be done in 24–48 hours to check the health of the intestine. If it appears the intestine has been damaged, the injured section may be removed.
In some cases, the condition of the patient or the appearance of the bowel makes reconnecting the bowel ends risky, and the bowel may need to be brought to the surface of the skin temporarily. This is done by creating a stoma — the end of the bowel is brought through an opening in the abdominal wall, and stool passes through this into a collection bag, which is taped over the opening. The stoma is temporary, and after the patient has fully recovered, the bowel ends can be safely reattached one to another.
The majority of children with malrotation who experienced a volvulus do not have long-term problems if the volvulus is repaired promptly and no intestinal damage occurred.
Children with intestinal injury who had the damaged part removed may have long-term problems. When a large portion of the intestine is removed, the digestive process can be affected. Nutrients and fluids are absorbed from food in the small intestine. Removing a large segment of the intestine can prevent a child from getting adequate nutrients and fluids. In this case, nutrition may need to be supplemented with long-term, high-calorie IV (intravenous) solutions given through special IV catheters.