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Intestinal Malrotation - Causes, Symptoms, Complications, Diagnosis, and Treatment

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Intestinal malrotation is a congenital condition in which the developing baby’s intestines get rotated incorrectly, causing twisting. Let us learn more.

Medically reviewed by

Dr. Veerabhadrudu Kuncham

Published At April 3, 2023
Reviewed AtApril 3, 2023

What Is Intestinal Malrotation?

Intestinal malrotation is an abnormal condition that can happen early during a pregnancy when the intestines of the baby do not form into a coil in the abdomen. Malrotation means that the intestines (or bowel) are twisted, which can lead to obstruction (blockage). Some children with malrotation never develop any problems, and the condition is not diagnosed. But most of them have symptoms and get diagnosed by age one. Although surgery is required to correct malrotation, most children will go on to grow and develop normally after treatment.

Intestinal Malrotation in Adults:

Intestinal malrotation is rarely present in adulthood. Therefore, it is difficult to diagnose but can have severe consequences if not detected early. Often, these patients suffer from various gastrointestinal disorders, from abdominal pain to constipation and food intolerance.

How Does Intestinal Malrotation Happen?

The intestines form the longest part of the digestive system. When they are stretched to their full length, they measure more than 20 feet long by adulthood, but since they get coiled up, it is easier for them to fit into the relatively small space of the abdominal cavity. When an embryo develops in the womb, the intestines start forming as a small, straight tube between the stomach and the rectum. As this tube evolves into separate organs, the intestines move into the umbilical cord, through which they supply nutrients to the developing fetus. During the end of the first trimester of pregnancy, the intestines shift from the umbilical cord into the abdomen. Malrotation occurs if they do not turn appropriately after moving into the abdomen. The exact cause of why this happens is unknown. Some kids with intestinal malrotation are born with a few other associated conditions that may include:

  • Other abnormalities of the digestive system.

  • Heart problems.

  • Defects of other organs, including the liver or spleen.

What Are the Complications of Intestinal Malrotation?

Malrotation can lead to the following complications:

  • In a condition known as volvulus, the intestines twist on themselves, blocking the blood flow to the tissues and causing the death of the tissues. Symptoms of volvulus, such as abdominal pain and cramps, often lead to the diagnosis of intestinal malrotation.

  • Bands of tissue known as Ladd's bands may form that obstruct the first part of the small intestine (the duodenum).

Obstruction of the intestine caused due to volvulus or Ladd's bands is a potentially life-threatening condition. The bowel can stop functioning, and intestinal tissue can die as a result of a lack of blood supply, if the obstruction is not detected and treated. Volvulus, in particular, is a medical emergency that jeopardizes the entire small intestine.

What Are the Signs and Symptoms of Intestinal Malrotation?

An intestinal blockage can prevent the proper movement of food. So one of the earliest signs of both intestinal malrotation and volvulus is abdominal pain and cramping, which occur when the bowel is unable to push food past the obstruction.

A baby with abdominal cramps might:

  • Pull the legs up and cry.

  • Suddenly stop crying.

  • Behave normally for a short duration, like 15 to 30 minutes.

  • Repeat this behavior on the onset of another cramp.

  • Infants also may be irritated, lethargic, or have difficulty pooping.

  • Another malrotation symptom is vomiting, which can help the doctor determine the location of the obstruction. When the vomiting happens soon after the baby starts to cry often indicates the blockage is in the small intestine and delayed vomiting usually means the blockage is in the large intestine. The vomit may be yellow or green, which means it contains bile or may resemble feces.

Other symptoms of malrotation and volvulus may include the following:

  • Swollen abdomen that is usually tender to the touch.

  • Diarrhea with bloody poop (or sometimes no poop at all).

  • A fussy baby or crying in pain that nothing seems to help.

  • Rapid heartbeat and breathing.

  • Little or sometimes no pee at all because of fluid loss.

  • Fever.

How Is Intestinal Malrotation Diagnosed?

Suppose the healthcare provider suspects volvulus or another intestinal blockage. In that case, they will perform a physical examination of the child and then may order an X-ray, computed tomography (CT) scan, or abdominal ultrasound.

The healthcare provider may use barium or another liquid contrast agent to see the X-ray or scan more clearly. The contrast can reveal if the intestine has a malformation and can usually locate where the blockage is. Adults and older children typically drink barium in a liquid form. For infants, barium may need to be given through a tube inserted from the nasal opening into the stomach. Sometimes, they are given a barium enema, where the liquid barium is inserted through the rectum.

How Is Intestinal Malrotation Treated?

Children having symptoms of malrotation need surgery. It is treated like an emergency since the development of volvulus could be fatal. Surgery is performed to move the intestines, clear any blockages, and reduce the chances of the intestines twisting sharply.

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

The doctor then inserts an intravenous line into the child’s vein to provide fluids to prevent dehydration. Next, antibiotics are given to the child to avoid infection and provide sedation so they can sleep throughout the procedure.

During this surgery, known as the Ladd procedure, the surgeon straightens the baby’s twisted intestines and checks for damage. Then, the surgeon divides adhesive bands between the duodenum (upper part of the small intestine) and colon to alleviate the intestinal obstruction. Finally, the surgeon carefully places the intestines into their place so they are not likely to get twisted again in the future. The surgeon might also remove the appendix if, in the future, the child may develop appendicitis and the new position of the intestines makes it challenging to diagnose and treat. In some children, a large section of the intestines is damaged and therefore needs to be removed. In these cases, the procedure called a colostomy or ileostomy is performed.

Conclusion:

When intestinal malrotation is treated, most children experience normal growth and development with no long-term issues. Most youngsters who encountered a volvulus do not have long-haul issues in the event that the volvulus was totally fixed and there was no gastrointestinal harm. However, children who had a colostomy may need to be supplemented intravenously in order to get enough fluids and nutrients.

Frequently Asked Questions

1.

How Severe Is Intestinal Malrotation?

Intestinal malrotation's severity varies, ranging from asymptomatic cases to life-threatening bowel obstruction. The degree of rotation and presence of associated complications determine its seriousness.

2.

Is Intestinal Malrotation Treatable?

Treatment for intestinal malrotation typically involves surgery to correct the anatomical abnormalities, reducing the risk of complications such as volvulus or bowel ischemia.

3.

At What Age Is Intestinal Malrotation Typically Diagnosed?

Intestinal malrotation is often diagnosed in infancy or childhood when symptoms like abdominal pain, vomiting, and digestive issues emerge, prompting medical evaluation and diagnostic procedures.

4.

What’s the most common form of non-rotation? Is thentestinal malrotation?

The most common type of malrotation is non-rotation, where the intestines do not rotate properly during fetal development, leading to potential complications in later life.

5.

Does Intestinal Malrotation Necessitate Surgery?

Surgery is usually necessary to correct intestinal malrotation, as it can lead to severe complications if left untreated, including bowel obstruction and compromised blood flow to the intestines. Early intervention is crucial.

6.

Can Malrotation Result in Fatality?

Yes, malrotation can potentially be fatal if complications like volvulus occur, cutting off blood supply to the intestines and causing severe intestinal damage or tissue death, a critical medical emergency that requires immediate intervention.

7.

Is Malrotation Hereditary?

Intestinal malrotation can have a genetic component, but it's often sporadic. While some cases may have a hereditary link, many others develop without a clear familial pattern, making its etiology multifactorial and not solely genetic.

8.

Can Malrotation Recur Post-surgery?

While surgery is the primary treatment for intestinal malrotation, it's important to note that malrotation can recur if not managed appropriately, necessitating ongoing medical attention and care to prevent complications.

9.

Is Malrotation a Rare Condition?

Intestinal malrotation is a rare condition, affecting a relatively small percentage of the population, making it an uncommon gastrointestinal disorder that requires specialized medical attention when diagnosed.

10.

How Do Surgeons Reposition Intestines?

Surgeons reposition intestines during a procedure called Ladd's, which involves detangling and reorienting the intestines to correct malrotation and prevent potential complications. This surgical approach aims to restore normal anatomical positioning.

11.

Does Intestinal Malrotation Lead To Constipation?

Intestinal malrotation may lead to symptoms like constipation, although it's not the only possible cause. Diagnosis should consider various factors, including medical history and imaging results.

12.

Is Surgery Required for Adult Intestinal Malrotation?

Surgery for adult intestinal malrotation is less common but may be required if symptoms or complications arise. Timely intervention is vital for optimal outcomes.

13.

Is Intestinal Malrotation Uncommon?

Intestinal malrotation is indeed an uncommon condition, more frequently diagnosed in childhood. Early detection and management are critical for pediatric patients.

14.

Is Pregnancy Possible With Intestinal Malrotation?

Pregnancy is possible with intestinal malrotation, but it may require careful monitoring and management to minimize potential complications. Consultation with a healthcare provider is essential during pregnancy.

15.

Can Intestinal Malrotation Be Detected via Ultrasound?

Intestinal malrotation can sometimes be detected via ultrasound, but a definitive diagnosis often requires additional imaging or tests to assess the anatomical abnormalities comprehensively.
Dr. Veerabhadrudu Kuncham
Dr. Veerabhadrudu Kuncham

Pediatrics

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congenital abnormalities
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