Malrotation is an abnormality in which an infant's intestine hasn't formed in the right way in the abdomen. Malrotation isn't often evident unless the baby experiences a twisting of the intestine known as a volvulus. Though malrotation can lead to complications, it's treatable when caught early.


What is malrotation?

Malrotation is a rare prenatal abnormality in which a baby’s intestine doesn’t form or rotate in the right way in their abdomen. It occurs early in pregnancy (around the 10th week) and develops when a baby’s intestine fails to coil into the proper position. This means that parts of the intestines are in the wrong location.

Malrotation can be asymptomatic (have no symptoms), and isn’t often evident unless a baby experiences an abnormal twisting of the intestine known as a volvulus. A volvulus causes an obstruction or blockage 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 is an emergency and must be treated as soon as possible. A volvulus can be treated successfully with surgery, and your baby can grow and develop normally.


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How common is malrotation?

Malrotation occurs in around one out of every 500 births in the United States and usually presents within the first year of a baby’s life, though it may also be asymptomatic. Only around one out of every 6000 infants will develop symptomatic malrotation, with 30% to 60% of cases 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. Malrotation may also be associated with other birth or intestinal defects.

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.

Babies with cramps and pain due to malrotation frequently follow a typical pattern: they may draw their legs up and cry, settle for about 10 to 15 minutes and then begin to cry again. If your baby follows this pattern or experiences any of the following symptoms call your pediatrician right away.

Symptoms of malrotation may include:

  • Frequent vomiting, often green or yellow-green in color.
  • A swollen, firm abdomen.
  • Pale color.
  • Poor appetite.
  • Little or no urine (due to fluid loss).
  • Infrequent bowel movements.
  • Blood in the stools.
  • Fever.
  • Lethargy (showing little energy).

Diagnosis and Tests

How is malrotation diagnosed?

After performing a thorough physical exam of your baby, your pediatrician will order tests that evaluate the position of the intestine, and show whether it’s 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 more clearly 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 (computerized axial tomography) scan 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, a CT scan looks for a blockage in one of the intestines. To help do this, a harmless dye is 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.

  • Your baby will be started on IV (intravenous) fluids to prevent dehydration. Your baby will receive antibiotics to prevent infection, and sedation so your baby sleeps through the procedure.
  • First, the surgeon untwists the baby’s intestine and checks it for damage. If the intestine is healthy, it’s 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.
  • Your surgeon performs another operation within 24 to 48 hours to check the health of your baby’s 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 don’t have long-term problems if the volvulus was repaired promptly and there was no intestinal damage. Your medical team will help you understand the specific prognosis for your baby.

Medically Reviewed

Last reviewed on 04/19/2021.

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