Malrotation

What is malrotation?

Malrotation is a birth defect that occurs when the intestines do not correctly or completely rotate into their normal final position during development. People born with malrotation may develop symptoms and complications, most often when they are babies but sometimes later in life. Some people with malrotation never develop symptoms or complications.

How common is malrotation?

Experts are not sure how common malrotation is because not all people with malrotation develop signs or symptoms. Some studies suggest that about 1 in 500 people have malrotation.4

Who is more likely to get malrotation?

Malrotation is most commonly diagnosed in babies. Up to 80 percent of babies with malrotation are diagnosed in the first month of life.5,6

What other health problems do people with malrotation have?

Children with malrotation may have other birth defects, including defects of the

• gastrointestinal (GI) tract
• wall of the abdomen
• heart
• biliary tract
• pancreas

What are the complications of malrotation?

Malrotation can cause volvulus, or twisting, of the small intestine. Malrotation can also cause intestinal obstruction due to bands of mesentery—the tissues that hold the intestines in place—pressing against the duodenum. These problems can lead to serious and life-threatening complications such as

• lack of blood flow to the blocked part of the intestine and death of the blood-starved tissues
• a perforation, or hole, in the wall of the intestine
• peritonitis, an infection of the lining of the abdominal cavity
• sepsis, a serious illness that occurs when the body has an overwhelming immune system response to an infection
• shock

What are the symptoms of malrotation?

If you or your child has signs or symptoms of malrotation or its complications, you should seek medical help right away.

Common signs of malrotation in babies younger than age 1 include7

• vomiting, often with bile in the vomit, which makes it green in color
• failure to thrive
• pain or tenderness in the abdomen
• bloating of the abdomen
• bleeding from the rectum or passing bloody stools

In children older than age 1 and adults, common signs and symptoms include7

• pain in the abdomen
• vomiting
• nausea
• diarrhea
• bloating
• constipation
• bleeding from the rectum or passing bloody stools
• failure to thrive in children

If malrotation and its complications cut off blood flow to the intestines and cause shock, symptoms may include

• confusion or unconsciousness
• fast heart rate
• pale skin
• sweating

What causes malrotation?

Experts don’t know what causes malrotation. However, genes or changes in genes—called mutations—may play a role.

How do doctors diagnose malrotation?

Doctors diagnose malrotation based on medical and family history, a physical exam, and imaging tests.

Physical exam

During a physical exam, a doctor will check for signs of pain, tenderness, or bloating or visible swelling of the abdomen and may listen to sounds inside the abdomen using a stethoscope. A doctor may also check for signs of shock, such as a rapid pulse or low blood pressure.

Imaging tests

Doctors may use the following imaging tests to diagnose malrotation

• x-rays, which use a small amount of radiation to create pictures of the inside of the body
• lower GI series, which uses x-rays and a chalky liquid called barium to view the large intestine
• upper GI series, which uses x-rays and barium to view the upper GI tract, including the small intestine
• ultrasound, which uses sound waves to create an image of organs

How do doctors treat malrotation?

Doctors treat malrotation and its complications with surgery. During surgery, the surgeon may

• divide any bands of tissue in the abdomen that are causing complications or might in the future
• change the position of the intestines to lower the risk of future complications
• untwist any volvulus
• remove any parts of the intestines damaged by volvulus
• remove the appendix, since malrotation can make it difficult to diagnose appendicitis if the patient develops this condition in the future

References

[4] Hall NJ. Chapter 3: Congenital problems of the gastrointestinal tract. In: Guandalini S, Dhawan A, Branski D, eds. Textbook of Pediatric Gastroenterology, Hepatology and Nutrition. Basel, Switzerland: Springer International Publishing; 2015;23–34.

[5] Shalaby MS, Kuti K, Walker G. Intestinal malrotation and volvulus in infants and children. BMJ. 2013;347:f6949.

[6] Haak BW, Bodewitz ST, Kuijper CF, de Widt-Levert LM. Intestinal malrotation and volvulus in adult life. International Journal of Surgery Case Reports. 2014;5:259–261.

[7] Nehra D, Goldstein AM. Intestinal malrotation: varied clinical presentation from infancy through adulthood. Surgery. 2011;149(3):386–393.

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