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What Is Intestinal Malrotation?

Medically Reviewed by Dany Paul Baby, MD on April 27, 2022

Intestinal malrotation is a rare congenital condition that happens when there is a disruption in normal fetal development. The baby’s intestines rotate in the wrong way, causing the intestines to settle in the wrong part of the abdomen, twisted or blocked.

Malrotation can cause intestinal volvulus, a condition in which the bowel twists itself, obstructing blood flow to the tissue, which, when prolonged, causes intestinal failure. Volvulus can be fatal and needs immediate treatment. It usually requires surgery. Symptoms of volvulus are often what lead to the diagnosis of malrotation.

Intestinal Malrotation in Children

Malrotation will present itself within the first year of your child’s life. It occurs in both boys and girls but will, however, discernibly manifest in boys within the first month of life more often than in girls. 

This condition can be asymptomatic unless your baby experiences a volvulus. Not everyone born with malrotation will develop volvulus. 

This condition may also be associated with other birth and intestinal defects like diaphragmatic hernia and omphalocele.

Intestinal Malrotations in Adults

It is rare for malrotations to present in adulthood. They are difficult to diagnose but can have serious consequences if not recognized early. Often, patients suffer from a variety of gastrointestinal disorders, from abdominal pain to food intolerance and constipation. This condition can also present acutely due to chronic volvulus, which may cause intestinal ischemia and gangrene.

What Are the Symptoms of Intestinal Malrotations?

Patients can present with chronic abdominal pain and cramping due to the bowel’s inability to push food past the blockage. However, each person presents symptoms differently and might vary in each child. Consult a pediatrician right away for a diagnosis if your child shows symptoms such as:

  • Vomiting green fluid
  • Drawing up their legs
  • Swollen belly
  • Diarrhea
  • Constipation
  • Failure to thrive
  • Fast heart rate
  • Fast breathing
  • Bloody stools
  • Stomach pain

Adults may also experience similar symptoms to those present in children, like diarrhea, constipation, fast heart rate, and bloody stools. Other symptoms of malrotations include:

  • A swollen abdomen that's tender to the touch
  • Pale color
  • Poor appetite
  • Little or no urine because of fluid loss
  • Rectal bleeding
  • Inability to have a bowel movement or pass gas

How Are Intestinal Malrotations Diagnosed?

After performing a thorough physical exam, your doctor will have various blood tests and diagnostic imaging studies done to evaluate the severity of malrotation and to determine whether your child’s intestines are twisted or blocked. These tests may include.

Abdominal x-ray. This goes a long way in revealing any intestinal obstruction.

Upper GI test. Upper gastrointestinal tract examination, also called an upper GI, is a procedure used to examine the structures of the upper digestive tract-the esophagus, stomach, and duodenum. A liquid that contains barium is swallowed or moved down a nasogastric tube that goes down from the nose into the stomach. This liquid coats the lining of the tract so that the images produced by the x-ray machine show any form of malrotation, duodenal obstruction, volvulus, or any other problem.

Barium enema. This procedure is an x-ray exam performed on the large intestines to examine any form of abnormalities. Barium is injected into your rectum through a small tube as an enema to highlight the colon. X-rays can show whether the large intestine is in a normal position or not.

CT scan:  These scans not only show the intestinal malpositioning seen in barium studies but also depict other findings not evident in conventional exams.

Abdominal ultrasound. This is a diagnostic medical procedure that uses high-frequency sound waves to produce visual images of organs, tissue, and blood flow in the body. An ultrasound exam will help your doctor to evaluate the function of the intestines and monitor the blood flow.

How Is Intestinal Malrotation Treated?

Children with symptoms of malrotation require surgery. It is treated as an emergency because the development of volvulus could be life-threatening. Surgery is conducted to move the bowels, clear any blockages, and lower the chances of bowels twisting sharply. 

Before surgery, your pediatrician will insert a tube through your child’s nose into their stomach. The tube empties the contents of their stomach and upper intestines to prevent the build-up of fluids and gas in the abdomen.

Your child’s doctor will place an intravenous line into a vein to provide your child with fluids to prevent dehydration. Your child will receive antibiotics to prevent infection and sedation so they can sleep throughout the procedure.

During this surgery, which is called the Ladd procedure, the surgeon untwists the baby’s intestines and checks them for damage. The surgeon will divide adhesive bands between the duodenum (upper part of the small intestine) and colon, to alleviate the bowel obstruction. If the bowels look healthy and pink, that means that the blood flow is good, and the surgeon returns the intestines carefully so they are not likely to twist in the future.

The surgeon will remove your child’s appendix, as the new position of the intestines might make it hard to treat and diagnose problems such as appendicitis.

In some children, there is a large section of the intestines that is damaged and needs to be removed. In these cases, the end of the healthy intestine is brought out through an opening on the skin of the abdomen called a stoma. This procedure is called a colostomy or ileostomy.

Malrotation Prognosis

Most children affected by intestinal malrotation experience normal growth and development with no long-term issues once the condition is treated. The majority of children who experienced a volvulus don't have long-term problems if the volvulus was completely repaired and there was no intestinal damage. Children who underwent a colostomy, though, might need to be fed by way of IV supplementation to provide adequate fluids and nutrients.

Show Sources

SOURCES:

American Journal of Roentgenology: “Intestinal Malrotations in Adolescents and Adults.”

Cleveland Clinic; “Malrotations.”

Cureus Journal of Medical Science: “Malrotation of the Gut in Adults.”

European Journal of Pediatric Surgery: “Laparoscopic Treatment of Intestinal Malrotations in Children.

Johns Hopkins Medicine: “Barium Enema.”, “Malrotation.”

Journal of The Society of Laparoscopic & Robotic Surgeons: Laparoscopic Treatment of Intestinal Malrotations in Adults.”

Nemours Kids Health: “Intestinal Maltoration.”

Seattle Children’s: “Malrotation”

Stanford Children’s Health: “Intestinal Malrotation and Volvulus in Children.”

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