About Intestinal Malrotation
An
intestinal
obstruction
is a blockage of the digestive tract that prevents the proper
passage of food. Some intestinal obstructions are present at birth,
while others are caused by such problems as hernias, abnormal scar
tissue growth after an abdominal operation, and inflammatory bowel
disease (IBD).
Malrotation
is twisting of the intestines (or bowel) caused by abnormal
development while a fetus is in utero, and can cause obstruction.
Malrotation occurs in 1 out of every 500 births in the United
States.
Some children with intestinal malrotation are born with other
associated conditions, including:
- other defects of the
digestive system
- heart defects
- abnormalities of other organs, including the spleen or
liver
Some kids with malrotation never experience complications
and are never diagnosed. But most develop symptoms during infancy,
and the majority are diagnosed by 1 year of age. Although surgery
is required to repair malrotation, most kids will go on to grow and
develop normally after treatment.
What Is Malrotation?
The small and large 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. Malrotation occurs when the intestines
don't position themselves normally during fetal development and
aren't attached inside properly as a result. The exact reason
this occurs is unknown.
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
embryo.
Around the 10th week of pregnancy, the intestines move from the
umbilical cord into the abdomen. When they don't properly
turn after moving into the abdomen, malrotation occurs.
Malrotation in itself may not cause any problems. However, it
can lead to other complications:
- Bands of tissue called
Ladd's bands
may form, obstructing the first part of the small intestine (the
duodenum).
- In a condition called
volvulus
, the bowel twists on itself, cutting off the blood flow to the
tissue and causing the tissue to die. The symptoms associated
with volvulus, including pain and cramping, are often what lead
to the diagnosis of malrotation.
- Obstruction caused by volvulus or Ladd's bands is a
potentially life-threatening problem. The bowel can stop
functioning and intestinal tissue can die from lack of blood
supply if an obstruction isn't recognized and treated.
Volvulus, especially, is an emergency situation, with the entire
small intestine in jeopardy.
Signs and Symptoms
One of the earliest signs of malrotation and volvulus is
abdominal pain and cramping caused by the inability of the bowel to
push food past the obstruction. When infants experience this
cramping they may:
- pull up their 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 irritable, lethargic, or have irregular
stools.
Vomiting is another symptom of malrotation, and it can help the
doctor determine where the obstruction is located. Vomiting that
happens soon after the baby starts to cry often means the
obstruction is in the small intestine; delayed vomiting usually
means the blockage is in the large intestine. The vomit
may contain bile (which is yellow or green in color) or may
resemble feces.
Additional symptoms of malrotation and volvulus may include:
- a swollen abdomen that's tender to the touch
- diarrhea and/or bloody stools (or sometimes no stools at
all)
- irritability or crying in pain, with nothing seeming to
help
- rapid heart rate and breathing
- little or no urine because of fluid loss
- fever
Diagnosis
If volvulus or another intestinal blockage is suspected, the
doctor will examine your child and then may order X-rays, a
computed tomography (CT) scan, or an ultrasound of the abdominal
area.
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 determine where a blockage is located. Adults and older
children 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.
Treatment
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 performed right away
in order 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. Your 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 (it is normally 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 perform a second surgery
within 48 hours of the first. If the bowel still looks unhealthy at
this time, the damaged portion may be removed.
If the child is seriously ill at the time of surgery, an
ileostomy
or
colostomy
will usually be performed. 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 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.
The majority of these surgeries are successful, although some
children 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 occur
later.
Children who require removal of a large portion of the small
intestine can have too little bowel to maintain adequate nutrition
(a condition known as short bowel syndrome). They may be dependent
on intravenous 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 identified
early, before permanent injury to the bowel has occurred, 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.
Reviewed by:
Philip Wolfson, MD
Date reviewed: April 2007
Note: All information is for educational purposes only. For specific medical advice,
diagnoses, and treatment, consult your doctor.
© 1995-2009 The Nemours Foundation/KidsHealth. All rights reserved.