What is Hirschsprung's disease?
Hirschsprung's disease occurs when some of the nerve cells that
are normally present in the intestine do not form properly while a
baby is developing during pregnancy.
As food is digested, muscles move food forward through the
intestines in a movement called peristalsis. When we eat, nerve
cells that are present in the wall of the intestines receive
signals from the brain telling the intestinal muscles to move food
forward.
In children with Hirschsprung's disease, a lack of nerve cells in
part of the intestine interrupts the signal from the brain and
prevents peristalsis in that segment of the intestine. Because
stool cannot move forward normally, the intestine can become
partially or completely obstructed (blocked), and begins to expand
to a larger than normal size.
The problems a child will experience with Hirschsprung's disease
depend on how much of the intestine has normal nerve cells
present. Seventy percent of babies with Hirschsprung's disease are
missing nerve cells in only the last one to two feet of the large
intestine.
Hirschsprung's disease causes 25 percent of intestinal
obstructions that occur in newborns.
What causes Hirschsprung's disease?
Between the 5th and the 12th weeks of pregnancy, while the fetus
is growing and developing, nerve cells form in the digestive
tract, beginning in the mouth and finishing in the anus. For
unknown reasons, the nerve cells do not grow past a certain point
in the intestine in babies with Hirschsprung's disease.
Scientists are not sure why the intestinal nerve cells do not form
completely. Nothing has been shown to cause this problem,
including medications a mother takes while pregnant or what a
mother eats during pregnancy.
How often does Hirschsprung's disease
occur?
Hirschsprung's disease occurs in 1 out of every 5,000 live births.
Who is at risk for Hirschsprung's
disease? Hirschsprung's disease occurs five times more
frequently in males than in females. Children with Down syndrome
have a higher risk of having Hirschsprung's disease.
There is possibly a genetic, or inherited, cause for
Hirschsprung's disease. If a family has a child with
Hirschsprung's disease, there is a 3 to 12 percent chance that
another baby they have will also have the disease. Also, there is
an increased chance that a couple will have a child with
Hirschsprung's disease if one of the parents has the disease. The
chances are higher if the mother is the parent with Hirschsprung's
disease.
Why is Hirschsprung's disease a concern?
Because a segment of the intestine lacks normal nerve cells,
digested food and stool cannot move forward through that portion
of the digestive tract. The intestine becomes blocked with stool,
and the baby will be constipated, or unable to have normal bowel
movements.
The obstruction (blockage) causes pressure on the inside of the
intestine, causing part of the intestinal wall to wear thin.
Eventually, a bacterial infection can develop in the digestive
tract, causing serious problems.
What are the symptoms of Hirschsprung's
disease?
Eighty percent of children with Hirschsprung's disease show
symptoms in the first 6 weeks of life. Children who only have a
short segment of intestine that lacks normal nerve cells may not
show symptoms for several months or years. The following are the
most common symptoms of Hirschsprung's disease. However, each
individual may experience symptoms differently. Symptoms may
include:
not having a bowel movement in
the first 48 hours of life
gradual bloating of the
abdomen
gradual onset of vomiting
fever
Children who do not have
early symptoms may also present the following:
constipation that becomes
worse with time
loss of appetite
delayed growth
passing small, watery stools
Symptoms of Hirschsprung's
disease may resemble other conditions or medical problems. Please
consult your child's physician for a diagnosis.
How is Hirschsprung's disease diagnosed?
A physician will examine your child and obtain a medical history.
Other tests may be done to evaluate whether your child has
Hirschsprung's disease. These tests may include:
abdominal x-ray - a diagnostic
test which may show a lack of stool in the large intestine or
near the anus and dilated segments of the large and small
intestine.
barium enema - a procedure
performed to examine the large intestine for abnormalities. A
fluid called barium (a metallic, chemical, chalky, liquid used
to coat the inside of organs so that they will show up on an
x-ray) is given into the rectum as an enema. An x-ray of the
abdomen shows strictures (narrowed areas), obstructions
(blockages), and dilated intestine above the obstruction.
anorectal manometry - a test
that measures nerve reflexes which are missing in Hirschsprung's
disease.
biopsy of the rectum or large
intestine - a test that takes a sample of the cells in the
rectum or large intestine and then looks for nerve cells under a
microscope.
Treatment for Hirschsprung's disease:
Specific treatment for Hirschsprung's disease will be determined
by your child's physician based on the following:
the extent of the
problem
your child's age, overall
health, and medical history
your child's tolerance for
specific medications, procedures, or therapies
expectations for the course of
the disorder
the opinion of the physicians
involved in the child's care
your opinion and preference
An operation is usually
necessary to deal with intestinal obstruction caused by
Hirschsprung's disease. The surgeon removes the portion of the
rectum and intestine that lacks normal nerve cells. A colostomy is
done so stool can leave the body. With a colostomy, the upper end
of the intestine is brought through an opening in the abdomen
known as a stoma. Stool will pass through the opening and then
into a collection bag. The colostomy may be temporary or
permanent, depending on the amount of intestine that is needed to
be removed. After a healing period, many children can have the
intestine surgically reconnected above to the anal opening and
have the colostomy closed.
Will my child have
problems in the future?
Problems in the future often depend on the amount of intestine
that lacked nerve cells, and how much intestine was removed.
Children who are able to have their colostomy closed may have
temporary or intermittent problems, including the following:
Stools may be frequent
and loose at first. Cleaning the anal area carefully to remove
stool, and applying diaper rash creams or lotions may prevent
skin irritation.
Children may have problems
sensing the need to have a bowel movement. Since the urge to
have a bowel movement is greater after eating, setting aside 10
minutes after meals to spend on the toilet may help.
Some children have problems
with bowel movements because the anal opening is tight, and this
can be helped by a special technique called rectal dilation.
Your child's physician can teach you this technique if it is
appropriate for your child.
Children who had a large section
of intestine removed may have long-term problems. When a large
portion of the intestine is removed, the digestive process can be
affected. Nutrients and fluids are absorbed from food in the small
intestine. Removing a large segment of the intestine can prevent a
child from getting adequate nutrients and fluids. Children can
have problems with improper digestion, slow growth, and infection.
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