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INTUSSUSCEPTION

Intussusception (in-tuh-suh-SEP-shun) is a serious condition in which part of the


intestine slides into an adjacent part of the intestine. This "telescoping" often blocks food
or fluid from passing through. Intussusception also cuts off the blood supply to the part of
the intestine that's affected, which can lead to a tear in the bowel (perforation), infection
and death of bowel tissue.
Intussusception is the most common cause of intestinal obstruction in children
younger than 3. The cause of most cases of intussusception in children is unknown.
Though rare in adults, most cases of adult intussusception are the result of an underlying
medical condition, such as a tumor.
In children, the intestines can usually be pushed back into position with an X-ray
procedure. In adults, surgery is often required to correct the problem.

Symptoms

Children
The first sign of intussusception in an otherwise healthy infant may be sudden,
loud crying caused by abdominal pain. Infants who have abdominal pain may pull their
knees to their chests when they cry.
The pain of intussusception comes and goes, usually every 15 to 20 minutes at
first. These painful episodes last longer and happen more often as time passes.
Other frequent signs and symptoms of intussusception include:
 Stool mixed with blood and mucus (sometimes referred to as "currant jelly" stool
because of its appearance)
 Vomiting
 A lump in the abdomen
 Lethargy
 Diarrhea
 Fever
Not everyone has all of the symptoms. Some infants have no obvious pain, and some
children don't pass blood or have a lump in the abdomen. Some older children have pain
but no other symptoms.

Adults
Because intussusception is rare in adults and symptoms of the disorder often
overlap with the symptoms of other disorders, it's more challenging to identify. The most
common symptom is abdominal pain that comes and goes. Nausea and vomiting may
also occur. People sometimes have symptoms for weeks before seeking medical
attention.

When to see a doctor


Intussusception requires emergency medical care. If you or your child develops
the signs or symptoms listed above, seek medical help right away.
In infants, remember that signs of abdominal pain may include recurrent bouts of
pulling the knees to the chest and crying.

Causes
Your intestine is shaped like a long tube. In intussusception, one part of your
intestine — usually the small intestine — slides inside an adjacent part. This is sometimes
called telescoping because it's similar to the way a collapsible telescope folds together.
In some cases, the telescoping is caused by an abnormal growth in the intestine, such as
a polyp or a tumor (called a lead point). The normal wave-like contractions of the intestine
grab this lead point and pull it and the lining of the intestine into the bowel ahead of it. In
most cases, however, no cause can be identified for intussusception.

Children
In the vast majority of cases of intussusception in children, the cause is unknown.
Because intussusception seems to occur more often in the fall and winter and because
many children with the problem also have flu-like symptoms, some suspect a virus may
play a role in the condition. Sometimes, a lead point can be identified as the cause of the
condition — most frequently the lead point is a Meckel's diverticulum (a pouch in the lining
of the small intestine).

Adults
In adults, intussusception is usually the result of a medical condition or procedure,
including:
 A polyp or tumor
 Scar-like tissue in the intestine (adhesions)
 Weight-loss surgery (gastric bypass) or other surgery on the intestinal tract
 Inflammation due to diseases such as Crohn's disease
Risk factors
Risk factors for intussusception include:
 Age. Children — especially young children — are much more likely to develop
intussusception than adults are. It's the most common cause of bowel obstruction
in children between the ages of 6 months and 3 years.
 Sex. Intussusception more often affects boys.
 Abnormal intestinal formation at birth. Intestinal malrotation is a condition in
which the intestine doesn't develop or rotate correctly, and it increases the risk
for intussusception.
 A prior history of intussusception. Once you've had intussusception, you're at
increased risk of developing it again.
 A family history. Siblings of someone who's had an intussusception are at a
much higher risk of the disorder.

Complications
Intussusception can cut off the blood supply to the affected portion of the intestine.
If left untreated, lack of blood causes tissue of the intestinal wall to die. Tissue death can
lead to a tear (perforation) in the intestinal wall, which can cause an infection of the lining
of the abdominal cavity (peritonitis).
Peritonitis is a life-threatening condition that requires immediate medical attention.
Signs and symptoms of peritonitis include:
 Abdominal pain
 Abdominal swelling
 Fever
Peritonitis may cause your child to go into shock. Signs and symptoms of shock
include:
 Cool, clammy skin that may be pale or gray
 A weak and rapid pulse
 Abnormal breathing that may be either slow and shallow or very rapid
 Anxiety or agitation
 Profound listlessness
A child who is in shock may be conscious or unconscious. If you suspect your child is
in shock, seek emergency medical care right away.

Diagnosis
Your or your child's doctor will start by getting a history of the symptoms of the
problem. He or she may be able to feel a sausage-shaped lump in the abdomen. To
confirm the diagnosis, your doctor may order:
 Ultrasound or other abdominal imaging. An ultrasound, X-ray or computerized
tomography (CT) scan may reveal intestinal obstruction caused by
intussusception. Imaging will typically show a "bull's-eye," representing the
intestine coiled within the intestine. Abdominal imaging also can show if the
intestine has been torn (perforated).
 Air or barium enema. An air or barium enema is basically enhanced imaging of
the colon. During the procedure, the doctor will insert air or liquid barium into the
colon through the rectum.
In addition, an air or barium enema can actually fix intussusception 90 percent of the
time in children, and no further treatment is needed. A barium enema can't be used if the
intestine is torn.

Treatment
Treatment of intussusception typically happens as a medical emergency.
Emergency medical care is required to avoid severe dehydration and shock, as well as
prevent infection that can occur when a portion of intestine dies due to lack of blood.
 Initial care
When your child arrives at the hospital, the doctors will first stabilize his or her medical
condition. This includes:
1. Giving your child fluids through an intravenous (IV) line
2. Helping the intestines decompress by putting a tube through the child's nose and
into the stomach (nasogastric tube)
 Correcting the intussusception
To treat the problem, your doctor may recommend:
 A barium or air enema. This is both a diagnostic procedure and a treatment. If an
enema works, further treatment is usually not necessary. This treatment is highly
effective in children, but rarely used in adults.
Intussusception recurs as often as 10 percent of the time and the treatment will have
to be repeated.
 Surgery. If the intestine is torn, if an enema is unsuccessful in correcting the
problem or if a lead point is the cause, surgery is necessary. The surgeon will free
the portion of the intestine that is trapped, clear the obstruction and, if necessary,
remove any of the intestinal tissue that has died. Surgery is the main treatment for
adults and for people who are acutely ill.
In some cases, intussusception may be temporary and go away without treatment.

Preparing for your appointment


Emergency medical care is required to treat intussusception. You may not have
much time to prepare for an appointment.

What to expect from your doctor

Your child's doctor is likely to ask you a number of questions, including:


 When did your child begin experiencing abdominal pain or other symptoms?
 Does your child's pain appear to be continuous — or is it occurring off and on?
 Does the pain begin and end suddenly?
 Has your child experienced nausea, vomiting or diarrhea?
 Have you noticed any blood in your child's stool?
 Have you noticed any swelling or a lump in your child's abdomen?

What you can do in the meantime


Don't give your child any over-the-counter medications to treat symptoms before
the appointment. Don't give your child anything to eat if you see any of the signs or
symptoms of intussusception. Seek immediate medical attention.
HIRSCHSPRUNG'S DISEASE

Hirschsprung's (HIRSH-sproongz) disease is a condition that affects the large


intestine (colon) and causes problems with passing stool. The condition is present at birth
(congenital) as a result of missing nerve cells in the muscles of the baby's colon.
A newborn who has Hirschsprung's disease usually can't have a bowel movement in
the days after birth. In mild cases, the condition might not be detected until later in
childhood. Uncommonly, Hirschsprung's disease is first diagnosed in adults.
Surgery to bypass or remove the diseased part of the colon is the treatment.

Symptoms
Signs and symptoms of Hirschsprung's disease vary with the severity of the
condition. Usually signs and symptoms appear shortly after birth, but sometimes they're
not apparent until later in life.
Typically, the most obvious sign is a newborn's failure to have a bowel movement
within 48 hours after birth.
Other signs and symptoms in newborns may include:
 Swollen belly
 Vomiting, including vomiting a green or brown substance
 Constipation or gas, which might make a newborn fussy
 Diarrhea
In older children, signs and symptoms can include:
 Swollen belly
 Chronic constipation
 Gas
 Failure to thrive
 Fatigue

Causes
It's not clear what causes Hirschsprung's disease. It sometimes occurs in families
and might, in some cases, be associated with a genetic mutation.
Hirschsprung's disease occurs when nerve cells in the colon don't form completely.
Nerves in the colon control the muscle contractions that move food through the bowels.
Without the contractions, stool stays in the large intestine.

Risk factors
Factors that may increase the risk of Hirschsprung's disease include:
 Having a sibling who has Hirschsprung's disease. Hirschsprung's disease can
be inherited. If you have one child who has the condition, future biological children
could be at risk.
 Being male. Hirschsprung's disease is more common in males.
 Having other inherited conditions. Hirschsprung's disease is associated with
certain inherited conditions, such as Down syndrome and other abnormalities
present at birth, such as congenital heart disease.

Complications
Children who have Hirschsprung's disease are prone to a serious intestinal
infection called enterocolitis.
Enterocolitis can be life-threatening. It's treated in the hospital with colon cleaning
and antibiotics.

Diagnosis
Your child's doctor will perform an exam and ask questions about your child's
bowel movements. He or she might recommend one or more of the following tests to
diagnose or rule out Hirschsprung's disease:
 Abdominal X-ray using a contrast dye. Barium or another contrast dye is placed
into the bowel through a special tube inserted in the rectum. The barium fills and
coats the lining of the bowel, creating a clear silhouette of the colon and rectum.
The X-ray will often show a clear contrast between the narrow section of bowel without
nerves and the normal but often swollen section of bowel behind it.
 Measuring control of the muscles around the rectum. A manometry test is
typically done on older children and adults. The doctor inflates a balloon inside the
rectum. The surrounding muscle should relax as a result. If it doesn't,
Hirschsprung's disease could be the cause.
 Removing a sample of colon tissue for testing (biopsy). This is the surest way
to identify Hirschsprung's disease. A biopsy sample can be collected using a
suction device, then examined under a microscope to determine whether nerve
cells are missing.

Treatment
Surgery
Surgery to bypass the part of the colon that has no nerve cells treats
Hirschsprung's disease. The lining of the diseased part of the colon is stripped away, and
normal colon is pulled through the colon from the inside and attached to the anus. This is
usually done using minimally invasive (laparoscopic) methods, operating through the
anus.
In children who are very ill, surgery might be done in two steps.
First, the abnormal portion of the colon is removed and the top, healthy portion of
the colon is connected to an opening the surgeon creates in the child's abdomen. Stool
then leaves the body through the opening into a bag that attaches to the end of the
intestine that protrudes through the hole in the abdomen (stoma). This allows time for the
lower part of the colon to heal.
Ostomy procedures include:
 Ileostomy. The doctor removes the entire colon and connects the small intestine
to the stoma. Stool leaves the body through the stoma into a bag.
 Colostomy. The doctor leaves part of the colon intact and connects it to the stoma.
Stool leaves the body through the end of the large intestine.
Later, the doctor closes the stoma and connects the healthy portion of the intestine to
the rectum or anus.

Results of surgery
After surgery, most children pass stool normally — although some may have
diarrhea at first.
Toilet training may take longer because children have to learn how to coordinate
the muscles used to pass stool. Long term, it's possible to have continued constipation,
a swollen belly and leaking of stool (soiling).
Children continue to be at risk of developing a bowel infection (enterocolitis) after
surgery, especially in the first year. Be aware of signs and symptoms of enterocolitis, and
call the doctor immediately if any of these occur:
 Bleeding from the rectum
 Diarrhea
 Fever
 Swollen abdomen
 Vomiting

Lifestyle and home remedies


If your child has constipation after surgery for Hirschsprung's disease, discuss with
your doctor whether to try any of the following:
 Serve high-fiber foods. If your child eats solid foods, include high-fiber foods.
Offer whole grains, fruits and vegetables and limit white bread and other low-fiber
foods. Because a sudden increase in high-fiber foods can worsen constipation at
first, add high-fiber foods to your child's diet slowly.
If your child isn't eating solid foods yet, ask the doctor about formulas that might help
relieve constipation. Some infants might need a feeding tube for a while.
 Increase fluids. Encourage your child to drink more water. If a portion or all of
your child's colon was removed, your child may have trouble absorbing enough
water. Drinking more water can help your child stay hydrated, which may help ease
constipation.
 Encourage physical activity. Daily aerobic activity helps promote regular bowel
movements.
 Laxatives. If your child does not respond to or can't tolerate increased fiber, water
or physical activity, certain laxatives — medications to encourage bowel
movements — might help relieve constipation. Ask the doctor whether you should
give your child laxatives and about the risks and benefits.

Preparing for your appointment


Hirschsprung's disease is often diagnosed in the hospital shortly after birth, or
signs of the disease show up later. If your child has signs or symptoms that worry you,
particularly constipation and a swollen abdomen, talk to your doctor.
You might be referred to a digestive disorders specialist (gastroenterologist) or to
the emergency department if your child's symptoms are severe.
Here's some information to help you get ready for your appointment.
What you can do
When you make the appointment, ask if there's anything your child needs to do in
advance, such as fasting for a specific test. Make a list of:
 Your child's signs or symptoms, including details about bowel movements —
frequency, consistency, color and associated pain
 Your child's key medical information, including other conditions he or she has and
family medical history
 All medications, vitamins or supplements your child is taking and how much water
he or she drinks in a typical day
 Questions to ask your child's doctor
Take a family member or friend along, if possible, to help you remember the
information you're given.
For Hirschsprung's disease, basic questions to ask your doctor include:
 What is likely causing my child's symptoms?
 What are other possible causes?
 What tests does my child need?
 What's the best course of action?
 If you recommend surgery, what should I expect from my child's recovery?
 What are the risks of surgery?
 What's my child's long-term prognosis after surgery?
 Will my child need to follow a special diet?
 Are there any brochures or other printed materials I can have? What websites do
you recommend?
Don't hesitate to ask other questions.

What to expect from the doctor


 Your child's doctor is likely to ask you questions, including:
 When did your child's symptoms begin?
 Have the symptoms worsened?
 How often does your child have a bowel movement?
 Are your child's bowel movements painful?
 Are your child's stools loose? Do they contain blood?
 Has your child been vomiting?
 Does your child tire easily?
 What, if anything, seems to improve your child's symptoms?
 What, if anything, appears to worsen your child's symptoms?

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