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Intestinal Malrotation & Volvulus

Malrotation is an anomaly that occurs when we have an incomplete rotation of the intestine during fetal development around the 10th week of gestation mark. In intestinal malrotation, the beginning of the large intestine (cecum) and the appendix are not attached to the right lower abdominal wall, and are located in the upper abdomen.

It is possible the bowel may twist on its own blood supply (mesentery), and in this instance, it is termed volvulus. If the volvulus involves the entire small bowel, it is called a mid-gut volvulus. This can result in loss of most of the intestine, and can result in death.

A volvulus is essentially a twisting of the bowels resulting in a closed-loop bowel obstruction. This can lead to bowel ischaemic, necrosis, and perforation.


Up to 70% of children with intestinal malrotation have another congenital birth defect, including abdominal wall defects, digestive system abnormalities, heart defects, or abnormalities of the liver or spleen.

Normal Embryology
In normal embryology, midgut maturation is divided into 4 stages: herniation (week 4 gestation), rotation (where the bowel rotated counterclockwise 270 degrees around the superior men's enteric artery), retraction, and fixation.

Malrotation occurs in around 1 in 6'000 live births, and there are a number of different types of rotational disorders.

Nonrotation occurs when there is a failure of 270 degree normal intestinal rotation.

Incomplete rotation occurs when there is rotation at or near 180 degrees of rotation.

Reversed rotation occurs when there is errant 90 degree clockwise rotation.

Signs & Symptoms
Symptoms do not always show, and are not always immediate. These may develop at any age.

However where there is an obstruction or volvulus, symptoms can include:

☞ The cardinal symptom: bilious vomiting.
☞ Chromic abdominal pain (in infant, they may draw up their legs)
☞ Abdominal swelling
☞ Tachycardia
☞ Shock
☞ Bloody bowel movements
☞ Malabsorption and malnutrition, leading to growth disturbances.

Diagnosis
Abdominal x-rays can show intestinal obstructions.

Doppler ultrasound to view internal organs and assess blood flow through vessels.

Upper GI test, to examine the small intestine for abnormalities using NGT contrast to show on X-ray

Barium enema, to examine the large intestine using the same radiographic contrast agent.

Treatment
Children are stabilised, ensuring adequate hydration and nutrition, as well as NGT insertion to prevent gas buildup in the stomach.

Surgical repair is undertaken as soon as possible to untwist the bowl and assess for damage. If the blood flow is still in question after untwisting, another operation is usually performed within 1-2 days after to re-evaluate and determine extend of bowel necrosis.

Long-Term Outlook
If intervention is achieved before there is intestinal damage sustained, there are usually no issues long term. However removal of large portions of bowel due to intestinal injury can affect the digestive process, in nutrition absorption, leading to the necessity of supplemental nutrition options to manage.

If total parental nutrition (TPN) is required for this supplementary management, these children are at increased risk for developing chronic liver disease.

And finally, children who have had necrotic bowel removed have significantly higher rates of mortality than those without.

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