What is malrotation?
Malrotation is an abnormality in which the intestine does not form in the correct way in the abdomen. It occurs early in the pregnancy (around the 10th week) and develops when the intestine fails to coil into the proper position in the abdomen. Malrotation is often not evident until the baby experiences a twisting of the intestine known as a volvulus. A volvulus is a disorder that causes an obstruction in the intestine, preventing food from being digested normally. The blood supply to the twisted part of the intestine can also be cut off, leading to the death of that segment of the intestine. This situation can become fatal if not treated as soon as possible.
Malrotation occurs in one out of every 500 births in the United States. Among those children who have malrotation and develop symptoms, most symptoms will occur in the first year of life. Nearly 60% of cases are diagnosed during the first week of life. Malrotation occurs equally in boys and girls. However, more boys show symptoms within the first month of life than girls.
Symptoms and Causes
What causes malrotation?
The exact cause of malrotation is unknown.
What are the symptoms of malrotation?
One of the earliest signs of malrotation is abdominal pain and cramping caused by the inability of the bowel to push food past the obstruction. A baby with cramps and pain due to malrotation frequently follows a typical pattern where he or she will begin crying while pulling his or her legs up, stop crying suddenly, act normal for a 10 or 15 minutes, then begin crying suddenly again, starting the pattern all over.
Other symptoms of malrotation may include:
Diagnosis and Tests
How is malrotation diagnosed?
After performing a thorough physical exam, the doctor will order tests that evaluate the position of the intestine, and show whether it is twisted or blocked. These tests may include:
- Abdominal X-ray: an X-ray that may show intestinal obstructions
- Barium enema X-ray: Barium is a liquid that makes the intestine show up better on the X-ray. For this test, barium is inserted into the intestine through the anus and then X-rays are taken.
- CT scan: CAT or CT is an abbreviation for Computerized Axial Tomography. This test uses computers and X-rays to produce many pictures from multiple angles to give doctors an accurate picture of the body. In the case of possible malrotation, the doctor will use a CT scan to look for a blockage in one of the intestines. To assist in doing this, a harmless dye may be injected so that the blockage is more easily seen.
Management and Treatment
How is malrotation treated?
Malrotation is considered an emergency situation and the development of volvulus is considered a life-threatening condition. Surgery is required to fix the problem.
Often, the baby will be started on IV (intravenous) fluids to prevent dehydration. Antibiotics will be given to prevent infection.
A volvulus is surgically repaired as soon as possible. First, the intestine is untwisted and checked for damage. If the intestine is healthy, it is then replaced in the abdomen.
If the blood supply to the intestine is in question, the intestine may be untwisted and placed back into the abdomen. Another operation is performed within 24 to 48 hours to check the health of the intestine. If it appears that the intestine has been damaged, the injured section may be removed.
For cases in which there is a large section of intestine that is damaged, a significant amount of intestine may be removed. When this occurs, the remaining parts of the intestine may not be able to be attached to each other surgically. To correct this, a colostomy may be done to enable the digestive process to continue. With a colostomy, the two remaining healthy ends of intestine are brought through openings in the abdomen. Stool will pass through the opening (called a stoma) and then into a collection bag. The colostomy may be temporary or permanent, depending on the amount of intestine that needed to be removed.
Outlook / Prognosis
What is the prognosis for malrotation?
Although surgery is required to repair malrotation, most children experience normal growth and development once the condition is treated and corrected. 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. Consult your baby's physician regarding the specific prognosis for your baby.