Malrotation is an abnormality of the bowel that occurs when the intestines are incorrectly or incompletely rotated into their normal position during development. This can lead to intestinal blockage known as volvulus, which occurs when the bowel is twisted and causes the blood supply to the bowel to be cut off. It is usually diagnosed in the first month of life and can be a life-threatening condition that can lead to further complications.1
Although malrotation can be a serious condition, if treated, most children experience normal growth and development with no long-term complications. This article will tell you everything you need to know about malrotation, including the symptoms, diagnosis, and treatment.
Causes of malrotation
During early pregnancy, the bowel is a long, straight tube that leads from the stomach to the rectum. It then moves into the umbilical cord as it develops into the large and small bowel. At around ten weeks of pregnancy, the bowel moves back into the abdomen and coils up into the proper position. Malrotation occurs when the bowel fails to coil up into the correct position.
As of now, there are no known causes of malrotation.
Signs and symptoms of malrotation
Malrotation often has no symptoms, and many children may never be diagnosed if it causes no problems. However, the abnormal position of the bowel can cause a kink or twist, known as volvulus, which can then cause further complications and symptoms. This prevents food from easily passing through the duodenum and past the obstruction to the rest of the bowel for digestion.
Therefore, the first sign of malrotation may be symptoms of volvulus. A baby with cramping as a result of volvulus may:
- Pull up their legs and cry
- Suddenly stop crying for 10-15 minutes
- Behave normally until the cramping starts again
Other symptoms of malrotation include:
- Infrequent bowel movement
- Vomiting - often green in colour
- Little or no urine
- A swollen and tender abdomen
- Bloody stool
- Lack of energy (lethargy)
- Poor appetite
- Fast heart and breathing rate
- Pale skin
Management and treatment for malrotation
As malrotation is often a life-threatening condition, it is treated in an operation under general anaesthetic. The timing and urgency of the operation depend on the child’s condition. For example, if the condition develops into volvulus, it immediately requires emergency treatment as the segments of the bowel tissue can die from lack of blood supply, which can prevent it from functioning properly and can lead to infection. As well as this, a direct effect of volvulus is dehydration due to the lack of fluids being absorbed, and this can quickly become a serious and life-threatening issue for young children. Therefore, paediatric surgery is required to treat malrotation.
Before the procedure:
First, your child will be given intravenous (IV) fluids to prevent dehydration. They will also be given antibiotics to prevent as well as sedation so they sleep through the procedure.
A nasogastric (NG) tube will then be inserted through your child’s nose and down into the stomach which will empty the contents of the stomach and bowel and prevent fluid and gas from building up in the abdomen.
Your child will then undergo a paediatric surgery known as Ladd’s procedure:
The surgeon will untwist your child’s bowel, check for any damaged areas, and untwist any volvulus. If the bowel looks healthy, it is coiled back up into the abdomen in a safe position.
The position of the intestines is changed to lower the risk of future complications.
The surgeon may remove your child’s appendix as malrotation can make it difficult to diagnose and treat appendicitis.
The surgeon will also remove any parts of the intestines that have been damaged or died due to the lack of blood supply. If a large section is removed, then the remaining parts of the intestines may not be able to surgically attach. To correct this, a colostomy is done where the two healthy ends of the intestines are brought through openings in the abdomen. This will allow stool to pass through the opening (known as a stoma) into a collection bag. This may be temporary or permanent, depending on the number of intestines that need to be removed.
Most of these operations are successful and rarely result in recurrent complications. However, possible risks include
- Bleeding or infection during or after the operation
- Adhesions - bands of tissue that block the bowel and cause obstruction. This is also known as recurrent volvulus and should be corrected immediately through another operation
- If a large portion of the bowel has been removed,, then your child may not have enough bowels to maintain adequate nutrition. This is a condition known as small bowel syndrome and can be treated through intravenous (IV) nutrition after surgery or a special diet.
Diagnosis of malrotation
Malrotation is diagnosed based on a physical examination and various imaging tests.
During a physical exam, your doctor will check for signs of malrotation, such as tenderness, pain, bloating or swelling of the abdomen. They may also use a stethoscope to listen to sounds inside the abdomen.
After a physical examination, your doctor will use a variety of imaging tests to find out the position of the intestines and whether it is twisted or blocked.
Possible tests include:
- X-ray: an abdominal X-ray that may show intestinal obstruction
- Barium enema X-ray: a liquid called barium is ingested by the child, which helps the intestines show up more clearly on the X-ray
- CT scan: produces pictures from multiple angles to show possible intestinal obstruction
Most children with malrotation are diagnosed by the age of one, and many are even diagnosed before one month of age if their condition has developed into volvulus. However, children of any age can be diagnosed with malrotation.
How common is malrotation?
Malrotation and volvulus affect around 1 in every 2500 - 3000 babies.
Can malrotation be prevented?
There is no clear cause of malrotation, and it does not occur due to anything that has happened during pregnancy; therefore, there is no way to prevent it.
Who is at risk of malrotation?
Malrotation commonly affects babies within the first year of life. It is most common in children with other birth defects, such as digestive system abnormalities, heart defects, and abnormalities of the liver or spleen.
When should I see a doctor?
If your child is showing symptoms of intestinal volvulus, you should contact your child’s healthcare provider immediately, as it can be life-threatening and lead to serious complications. One of the most serious complications includes shock caused by intestinal volvulus, which cuts off the blood flow to the intestines. The symptoms shown include confusion or unconsciousness, rapid heart rate, sweating, and pale skin. You must seek medical attention if your child is experiencing these symptoms.
In summary, malrotation is a birth defect which occurs when the intestines are incorrectly rotated into their normal position during development. This can lead to intestinal blockage, known as volvulus, which causes a lack of blood flow to the blocked part of the intestines. It is a condition that mostly affects babies within their first year of life and rarely affects adults. The symptoms of intestinal malrotation include vomiting, fever, abdominal pain, infrequent bowel movement, little or no urine, and poor appetite. Your child’s doctor will use both a physical exam and imaging tests such as X-rays, CT scans, and ultrasounds to diagnose your child. Treatment for malrotation involves surgery where the surgeon will straighten out the twisted bowel, check for any damage and then coil it back up into a safe position. The surgeon may also remove any damaged parts of the intestines that have died due to lack of blood supply.
- Bhatia S, Jain S, Singh CB, Bains L, Kaushik R, Gowda NS. Malrotation of the gut in adults: an often forgotten entity. Cureus [Internet]. [cited 2023 Apr 14];10(3):e2313. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5947924/