Intestinal Malrotation Foundation
Spreading Awareness and Offering Resources to Families Dealing with Intestinal Malrotation

Information

Intestinal

Malrotation
and Volvulus

 

What is Intestinal Malrotation?

Intestinal malrotation (IM) is a condition that affects the positioning of a person's intestines within their abdomen.

It usually affects a person's small and large intestine. Sometimes only the small intestine or only the large intestine is affected¹. Most people don't know they have intestinal malrotation unless they begin to experience complications.

What causes Intestinal Malrotation?

Intestinal malrotation is a congenital birth defect, which means it occurs while a fetus is still developing.

In utero, the digestive system begins as a straight tube. At 5 weeks gestation, the tube starts to develop into the esophagus, stomach, small intestine and large intestine. During this time, the part of the digestive tract that will become the intestine, moves out of the abdomen and into the umbilical cord.

For most babies, as the intestine forms, it re-enters the abdomen, makes two separate turns, and lands in the typical position by 12 weeks gestation. If an error occurs at any time during the normal rotation process, it can cause the intestine to be positioned incorrectly in the abdomen - called intestinal malrotation. 

Although intestinal malrotation is present at birth, symptoms or complications can begin at any age.

What is Volvulus?

Volvulus is a type of intestinal obstruction, and is a dangerous complication of intestinal malrotation. The unique position of the digestive system in intestinal malrotation makes it possible for the intestine to twist around itself, this twist is called a volvulus. A volvulus can restrict blood flow and lead to intestinal death quickly. It can also cause extreme dehydration and electrolyte imbalances, perforation of the intestine and sepsis. Volvulus is a surgical emergency. If not diagnosed or treated in a timely manner, volvulus can be fatal.

What are the Symptoms of Intestinal Malrotation with Volvulus?

Symptoms can vary greatly from person to person depending on age and what part of the intestine is affected by volvulus. Some people experience several symptoms, and some experience only one or two symptoms. If you or your child are experiencing symptoms of volvulus, please go to the emergency department as quickly as possible. Volvulus is a surgical emergency, and every minute matters.

Vomiting

  • Green or Yellow Vomit - This is called bile or bilious vomit. It is considered the most common sign of volvulus, and is reported in 80% of pediatric patients with volvulus². Vomiting bile should be considered intestinal malrotation and volvulus until proven otherwise.
  • "Coffee Ground" Vomit
  • Vomiting Undigested Food
  • Forceful or Projectile Vomiting
  • Vomiting Large Quantities - May seem to vomit entire feed
  • Normal Vomit

Changes in Bowel Movements

  • Absence or decrease in bowel movements
  • Painful bowel movements
  • Diarrhea
  • Dark brown, black, or bloody stools
  • Inability to pass gas

Other Symptoms

  • Abdominal Distention
  • Abdominal Pain - Could be constant or intermittent, and range from mild to severe.
  • Refusal to eat, lack of appetite
  • Lethargy, fatigue
  • Excessive or inconsolable crying (infants and young children)
  • Dehydration, lack of urine - Because fluid and nutrients are absorbed in the intestine, dehydration and electrolyte imbalances may occur very rapidly in a person whose intestine is blocked by volvulus
  • Signs of infection - fever, elevated white blood cells

What are the Symptoms of Intestinal Malrotation (without Volvulus)?

Symptoms of intestinal malrotation (without volvulus) usually fall into three categories.
1) Vague abdominal symptoms. These symptoms may be constipation, diarrhea, abdominal pain, lack of appetite, bowel incontinence, delayed gastric emptying, failure to thrive or reflux.
2) Extreme abdominal symptoms such as the ones listed above for volvulus. These symptoms may occur rarely or in frequent episodes. In some cases of intestinal malrotation, it may be possible to experience intermittent volvulus.
3) No signs or symptoms (asymptomatic). Many people with intestinal malrotation are asymptomatic. Some may continue to be asymptomatic their entire lives while others may begin experiencing symptoms at a later point.

What are the other complications of Intestinal Malrotation?

In addition to volvulus, intestinal malrotation can lead to obstruction by Ladd's bands. In many cases of intestinal malrotation, fibrous bands called Ladd's bands run from the large intestine to the abdominal wall. Ladd's bands have the potential to block the duodenum (the first part of the small intestine). This can lead to symptoms similar to volvulus, but does not typically compromise the intestine's blood supply the way volvulus does. Obstruction by Ladd's bands can occur suddenly, or can come and go intermittently.

Short bowel syndrome is another potential complication of intestinal malrotation. Short bowel syndrome can occur as a result of volvulus. During volvulus, the blood supply to the intestine is cut off. Without blood, the intestines can die in just a few short hours. Any part of the intestine that has died must be removed. If less than 50% of the bowel remains after surgery, the person suffers from short bowel syndrome.

How common is Intestinal Malrotation?

The exact incidence of intestinal malrotation is unknown. Birth defect surveillance data indicates that symptomatic intestinal malrotation affects between 2 and 3 out of every 10,000 babies in the U.S., or about 1 in 4000 babies each year³. However, about 40%⁴ of those affected by intestinal malrotation are diagnosed after age 1, making the rate of symptomatic intestinal malrotation more like 1 in 2300.

Many sources state that some degree of intestinal malrotation may affect as many as 1 in 500 people, this is based on a very small study from 1934⁵ and does not indicate what percent of participants experienced symptoms or complications. 

How do I know if I have Intestinal Malrotation?

If you suspect that you or your child have intestinal malrotation (without volvulus) you should ask your doctor or gastroenterologist to schedule diagnostic testing. Common diagnostic tests that might show intestinal malrotation (without volvulus) are upper GI series, lower GI series, CT Scan, and ultrasound.

Upper GI Series (Barium Swallow) - 95% accurate⁶
Lower GI Series (Barium Enema) - 80% accurate⁷
Computed Tomography (CT) scans - 97-99% accurate⁸
Ultrasound - The SMV (superior mesenteric vein) and SMA (superior mesenteric artery) may be positioned incorrectly in some cases of intestinal malrotation, which may be evident on ultrasound. However, many cases of intestinal malrotation have correct positioning of the SMV and SMA⁹ . Therefore, ultrasound may be an easy and noninvasive way to detect some cases of intestinal malrotation, but should never be used to rule intestinal malrotation out.

When intestinal malrotation (without volvulus) is suspected, doctors usually begin by ordering an upper GI series. The upper GI series has a high accuracy rate, with a lower dose of radiation than a CT scan. If the upper GI series is negative, but you feel intestinal malrotation is a possibility, you should push your doctor for more diagnostic testing. It may take a combination of tests to successfully diagnose or rule out intestinal malrotation.

If you or your child is experiencing signs of volvulus, diagnostic testing should be performed in the emergency department immediately. For some patients, volvulus may be seen on plain abdominal x-ray or ultrasound. If plain abdominal x-ray and ultrasound are inconclusive, upper GI series, CT scan, or occasionally lower GI may be used for volvulus.

What is the Treatment for Intestinal Malrotation?

The most common treatment for intestinal malrotation is a surgical procedure called a Ladd’s procedure. A Ladd's procedure is usually performed immediately on those diagnosed with intestinal malrotation and volvulus. If a person is diagnosed with intestinal malrotation without volvulus, surgery may be scheduled to avoid the possibility of a volvulus in the future.

A Ladd's procedure does not place the intestines in the correct anatomical position. The goal of the Ladd’s procedure is to untwist a volvulus (if present) and place the small and large intestine in a position that makes them less likely to twist in the future.

The Ladd’s Procedure for Intestinal Malrotation without Volvulus usually involves:
Cutting Ladd’s bands that run across the duodenum
Removing the appendix*
Placing the small intestines on the patient’s right side, and the large intestines on the patient’s left side.

The Ladd’s Procedure for Intestinal Malrotation and Volvulus usually involves:
Manually untwisting the volvulus
Removing the damaged part of the intestine (if volvulus caused tissue death), and reconnecting the healthy part**
Cutting Ladd’s bands that run across the duodenum
Removing the appendix*
Placing the small intestines on the patient’s right side, and the large intestines on the patient’s left side.

*Most surgeons prefer to remove the appendix during a Ladd's procedure. As the site of the removed appendix heals, it can create adhesions that help the intestines stay in their new position. Removing the appendix also helps avoid a future case of appendicitis, which would be difficult to diagnose.

**If the surgeon is not able to reconnect the intestine, an illeostomy or colostomy may be created. If possible, a second surgery will be performed at a later date to reconnect the intestine.


¹ SamuelM, Boddy SA, Nicholls E, Capps S. Large bowel volvulus in childhood. Aust N Z J Surg2000;70:258–262.
² SeashoreJH, Touloukian RJ. Midgut volvulus: an ever-present threat. Arch Pediatr Adolesc Med1994;148:43–46.
³ Forrester, M. B. and Merz, R. D. (2003), Epidemiology of intestinal malrotation, Hawaii, 1986–99. Paediatric and Perinatal Epidemiology, 17: 195–200.
⁴ Aboagye, J., Goldstein, S. D., Salazar, J. H., Papandria, D., Okoye, M. T., Al-Omar, K., ... Abdullah, F. (2014). Age at presentation of common pediatric surgical conditions: Reexamining dogma. Journal of Pediatric Surgery49(6), 995-999.
⁵ Kantor JL. Anomalies of the colon: their Roentgen diagnosis and clinical significance. Radiology. 1934;6:651–662.
⁶ Sizemore, A.W., Rabbani, K.Z., Ladd, A. et al. Pediatr Radiol (2008) 38: 518.
⁷ Strouse PJ. Disorders of intestinal rotation and fixation (“malrotation”). Pediatr Radiol 2004;34:837–51.
⁸ CT appearance of the duodenum and mesenteric vessels in children with normal and abnormal bowel rotation. Taylor GA Pediatr Radiol. 2011 Nov; 41(11):1378-83.
⁹ Zhang, W., Sun, H., & Luo, F. (2017). The efficiency of sonography in diagnosing volvulus in neonates with suspected intestinal malrotation. Medicine96(42), e8287.