An intestinal malrotation is an abnormality that can happen early in pregnancy
when a baby's intestines don't form into a coil in the abdomen. Malrotation
means that the intestines (or bowel) are twisting, which can cause obstruction (blockage).
Some kids with malrotation never have problems
and the condition isn't diagnosed. But most develop symptoms and are diagnosed by
1 year of age. Although surgery is needed to repair malrotation, most kids will go
on to grow and develop normally after treatment.
How Does Intestinal Malrotation Happen?
The intestines are the longest part of the digestive
system. If stretched out to their full length, they
would measure more than 20 feet long by adulthood, but because they're folded up,
they fit into the relatively small space inside the abdomen.
When a fetus develops in the womb, the intestines start out as a small, straight
tube between the stomach and the rectum. As this tube develops into separate organs,
the intestines move into the umbilical cord, which supplies nutrients to the developing
Near the end of the first trimester of pregnancy, the intestines move from the
umbilical cord into the abdomen. If they don't properly turn after moving into the
abdomen, malrotation occurs. It happens in 1 out of every 500 births in the United
States and the exact cause is unknown.
Some children with intestinal malrotation are born with
other associated conditions, including:
other defects of the digestive system
abnormalities of other organs, including the spleen or liver
What Problems Can It Cause?
Malrotation can lead to these complications:
In a condition called volvulus, the bowel twists on itself, cutting
off the blood flow to the tissue and causing the tissue to die. Symptoms of volvulus,
including pain and cramping, are often what lead to the diagnosis of malrotation.
Bands of tissue called Ladd's bands may form, obstructing
the first part of the small intestine (the duodenum).
Obstruction caused by volvulus or Ladd's bands is a potentially life-threatening
problem. The bowel can stop working and intestinal tissue can die from lack of blood
supply if an obstruction isn't recognized and treated. Volvulus, especially, is a
medical emergency, with the entire small intestine in jeopardy.
What Are the Signs of Intestinal Malrotation?
An intestinal blockage can prevent the proper passage of food. So one of the earliest
signs of malrotation and volvulus is abdominal pain and cramping, which happen when
the bowel can't push food past the blockage.
A baby with cramping might:
pull up the legs and cry
stop crying suddenly
behave normally for 15 to 30 minutes
repeat this behavior when the next cramp happens
Infants also may be fussy, lethargic, or have trouble pooping.
Vomiting is another symptom of malrotation, and it can help the doctor determine
where the obstruction is. Vomiting that happens soon after the baby starts to cry
often means the blockage is in the small intestine; delayed vomiting usually means
it's in the large intestine. The vomit may contain bile (which is yellow or green)
or may resemble feces.
Other symptoms of malrotation and volvulus can include:
a swollen abdomen that's tender to the touch
diarrhea and/or bloody poop (or sometimes no poop at all)
fussiness or crying in pain, with nothing seeming to help
The doctor may use barium or another liquid contrast agent to
see the X-ray or scan more clearly. The contrast can show if the bowel has a malformation
and can usually find where the blockage is.
Adults and older kids usually drink barium in a liquid form. Infants may need to
be given barium through a tube inserted from the nose into the stomach, or sometimes
are given a barium enema, in which the liquid barium is inserted through the rectum.
How Is Intestinal Malrotation Treated?
Treating significant malrotation almost always requires surgery. The timing and
urgency will depend on the child's condition. If there is already a volvulus, surgery
must be done right away to prevent damage to the bowel.
Any child with bowel obstruction will need to be hospitalized. A tube called a
nasogastric(NG) tube is usually inserted through
the nose and down into the stomach to remove the contents of the stomach and upper
intestines. This keeps fluid and gas from building up in the abdomen. The child may
also be given intravenous (IV) fluids to help prevent dehydration and antibiotics
to prevent infection.
During the surgery, which is called a Ladd procedure, the intestine
is straightened out, the Ladd's bands are divided, the small intestine is folded into
the right side of the abdomen, and the colon is placed on the left side.
Because the appendix is usually found on the left side of the abdomen when there
is malrotation (normally, the appendix is found on the right), it is removed. Otherwise,
should the child ever develop appendicitis,
it could complicate diagnosis and treatment.
If it appears that blood may still not be flowing properly to the intestines, the
doctor may do a second surgery within 48 hours of the first. If the bowel still looks
unhealthy at this time, the damaged portion might be removed.
If the child is seriously ill at the time of surgery, an ileostomy
or colostomy usually will be done. In this procedure, the diseased
bowel is completely removed, and the end of the normal, healthy intestine is brought
out through an opening on the skin of the abdomen (called a stoma).
Fecal matter (poop) passes through this opening and into a bag that is taped or attached
with adhesive to the child's belly.
In young children, depending on how much bowel was removed, the ileostomy or colostomy
is often a temporary condition that can later be reversed with another operation.
Most of these surgeries are successful, although some kids have recurring problems
after surgery. Recurrent volvulus is rare, but a second bowel obstruction due to adhesions
(scar tissue build-up after any type of abdominal surgery) could happen later.
Children who had a large portion of the small intestine removed can have too little
bowel to maintain adequate nutrition (a condition known as short bowel syndrome).
They might need intravenous (IV) nutrition for a time after surgery (or even permanently
if too little intestine remains) and may require a special diet afterward.
Most kids in whom the volvulus and malrotation are found and treated early, before
permanent injury to the bowel happens, do well and develop normally.
If you suspect any kind of intestinal obstruction because your child has bilious
(yellow or green) vomiting, a swollen abdomen, or bloody stools, call your doctor
immediately, and take your child to the emergency
room right away.