Neonatal Intestinal Malrotation - Causes, Symptoms, and Management

Introduction:

Malrotation is a rare abnormality that usually occurs around the 10th week of pregnancy when there is a malformation or malrotation of the infant’s intestine. The baby can be asymptomatic unless the baby encounters an abnormal or unusual twisting of the intestine called a volvulus resulting in an obstruction in the intestine. This interferes with normal digestion. The twisted portion of the intestine may be cut off from the regular blood supply leading to the death of that intestinal segment.

Who Are at Risk of Developing Intestinal Malrotation?

This abnormal condition is common in both boys and girls but boys are affected more and display symptoms by one month of age. Babies with other health conditions such as diaphragmatic hernia (a birth defect in which there is a hole in the diaphragm that separates the chest from the abdomen), omphalocele (a birth defect in which there is a hole in the belly button that causes the abdominal organs and the intestines to stick out) and duodenal atresia (an abnormality in which the first part of the small intestine called the duodenum has developed improperly).

Is Intestinal Malrotation Common?

Intestinal malrotation is incident in one out of every 500 newborns in the United States. It can be asymptomatic in a few cases, but in symptomatic cases, the babies display symptoms by one year of age. Symptomatic malrotation presents in one out of 6000 newborns, and 30 to 60 % of the babies are diagnosed during the first week after delivery.

What Are the Causes of Intestinal Malrotation?

The longest part of the digestive system is the intestines, which when unfolded, would measure about 20 feet or longer in adults. This long portion is folded and accommodated within the small portion inside the abdomen. During the development of the fetus in the mother’s womb, the intestine begins to form a small, straight tube that extends between the stomach and the rectum.

With the formation of various separate organs from this tube, the intestines accommodate the umbilical cord, which supplies the necessary nutrients to the fetus. Near the end of the first trimester of pregnancy, the intestines move away from the umbilical cord into the abdomen. Malrotation can occur if the intestines do not turn properly inside the abdomen. The cause of this malrotation is unknown and can also happen due to other conditions, such as heart defects or abnormalities in the spleen or the liver.

What Are the Signs and Symptoms of Intestinal Malrotation?

An obstruction in the food passage can affect the passage of food. An early sign of this is abdominal cramping and pain as a result of the blockage, which causes the food to be pushed against the blockage. The following symptoms may be shown by a baby with intestinal malrotation:

• Crying and pulling the legs up.
• Stops crying.
• Remains normal for 15 to 30 minutes.
• Repeats the above pattern of behavior during the next episode of cramp.
• Fussy, cranky, and lethargic (less energetic).
• Vomiting.
• The vomitus may be yellow or green as it contains bile or look like feces.
• Vomiting helps to identify the part of the intestine which is blocked. If the crying in babies is followed by vomiting, then the obstruction is in the small intestine. Delayed vomiting indicates obstruction in the large intestine.
• Difficulty in pooping. The poop may be bloody, or there may be no poop at all.
• Diarrhea.
• Bloated and tender stomach.
• Rapid heart rate and breathing.
• Fever.
• Little or no urine as there is fluid loss.
• Loss of appetite.
• Pale color and irregular bowel movements.
• Bleeding from the rectum or the bottom.

How Is Intestinal Malrotation Diagnosed?

If a doctor suspects intestinal obstruction after examining the baby, they may suggest X-rays, computed tomography (CT) scan, or abdominal ultrasound to study the intestines. Various diagnostic techniques are as follows:

• Contrast agents such as barium may be used by doctors to study the X-ray or the scan since the contrast used shows the malformation in the intestine and helps to predict the site of obstruction. Older children and adults may consume barium in a liquid form, but infants are given barium through a tube inserted in the nose that extends to the stomach, or liquid barium is administered through the rectum called a barium enema.
• Blood tests may be advised to check the level of electrolytes.
• A stool test may be taken to detect the presence of blood in stools.
• Flexible sigmoidoscopy is a procedure done to study the lower gastrointestinal tract (GIT), rectum, and colon to check for volvulus (obstruction due to twisting of the intestine).

How Is Intestinal Malrotation Treated?

The treatment of intestinal malrotation requires surgery. When the baby has volvulus and shows the above signs and symptoms, emergency treatment is required, and delayed treatment could be life-threatening. General anesthesia will be administered to the baby so that they sleep through the procedure. A procedure called the Ladd procedure is performed to correct intestinal malrotation. The procedure is done as follows:

• A nasogastric tube is inserted through the nose that reaches the stomach, and the contents of the stomach and upper intestines are emptied. This also prevents gas and fluid from collecting in the abdomen.
• Intravenous (IV) fluids and antibiotics may be administered to the baby to prevent dehydration and infection.
• In the Ladd procedure, the intestine is straightened, the Ladd’s bands (tissue formation between the cecum and the intestinal wall) are separated, the small intestine is folded to the right of the abdomen, and the colon (the longest part of the large intestine), is positioned on the left side.
• The appendix (a small, finger-like pouch attached to the large intestine) is usually located on the right of the abdomen, but in malrotation, it is situated on the left. The appendix is removed during the procedure as appendicitis (inflammation of the appendix) in the future can cause complications in the individual.
• If there is an inappropriate blood flow even after the surgery, a second surgery will be performed within 48 hours after the first surgery. Even if the second surgery is unsuccessful, the damaged portion of the intestine will be removed.
• In seriously ill children, ileostomy (for the small intestine) or colostomy (for the large intestine) will be performed. In this procedure, the diseased or unhealthy part of the intestine is completely removed. The healthy portion of the intestine is introduced to the anterior abdominal wall through an opening (stoma) made on the skin of the abdomen. The poop exits through this opening and gets collected in a bag that is secured with adhesive on the baby’s belly.
• Based on how much intestine is removed, ileostomy or colostomy is a temporary condition in young kids.
• Children in whom a large portion of the intestine is removed may have poor nutrition due to the small size of the intestines. This condition is called short bowel syndrome. These kids will require nutrition that may be given intravenously (foods and drugs administered through the vein) for a short period after surgery. If minimum intestine remains, a special diet may be required later or may require a long-term supply of nutrition through the intravenous route called total parenteral nutrition (TPN).

What Are the Risks Associated With the Surgery for Intestinal Malrotation?

Every surgical procedure can accompany a few risks. The baby might require a blood transfusion during the procedure. Care must be taken by surgeons to not damage the liver or surrounding organs. Rarely, wounds can get infected. Few children may develop obstructions in the future due to the formation of scar tissue. Removal of large portions of the intestine may require a prolonged supply of nutrients through the intravenous route.

Conclusion:

A volvulus or obstruction due to the twisting of the intestines is a major issue that has to be treated right away. Green or yellow vomitus in babies should not be ignored. Most children lead a normal life after the surgery. Babies may continue to have a breathing tube for some time after the surgery. Medications to relieve pain will be given, and milk feeds will be started slowly after a few days. If any signs or symptoms mentioned above reoccur, or if the operation site becomes red, swollen, or infected with any noticeable discharge, the doctor should be consulted immediately.