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Thread: Hirschsprung's Disease Pictures - Pediatric cases

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    Default Hirschsprung's Disease Pictures - Pediatric cases

    Definition
    Hirschsprung's (HIRSH-sproongz) disease is a condition that affects the large intestine (colon) and causes problems with passing stool. Hirschsprung's disease is present when a baby is born (congenital) and results from missing nerve cells in the muscles of part or all of the baby's colon.

    A newborn who has Hirschsprung's disease is usually unable to have a bowel movement in the first days after birth. In mild cases, the condition might not be detected until later in childhood.

    Hirschsprung's disease is treated with surgery to bypass or remove the diseased part of the colon.

    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 of Hirschsprung's disease is a newborn's failure to have a bowel movement within 48 hours after birth.

    Causes
    It's not clear what causes Hirschsprung's disease. It sometimes occurs in families and may in some cases be associated with a genetic mutation.

    Hirschsprung's disease occurs when nerve cells in the colon don't form completely. Nerve cells are critical to the functioning of the colon. They control the regular muscle contractions that keep food moving through the bowels.
    Hirschsprung's Disease Pictures Pediatric cases attachment.php?s=e7debc9ee65f0c76ccb5a3a62e8719ac&attachmentid=314&d=1435791649

    As a baby develops before birth, bundles of nerve cells (ganglia) normally begin to form between the muscle layers along the length of the colon. This process begins at the top of the colon and ends at the bottom (rectum). In children who have Hirschsprung's disease, the nerve-growing process fails to finish. Most commonly, ganglia fail to form (aganglia) in the last segment of the colon the rectum and the sigmoid colon. Sometimes aganglia affects the entire colon and even part of the small intestine.

    Complications
    Children who have Hirschsprung's disease are prone to a serious infection of the intestines called enterocolitis.

    Enterocolitis is caused by stool backing up behind the immobile section of colon. The stagnant mass of stool provides a fertile environment for bacteria to grow. As the stool mass expands, it presses on the blood vessels in the walls of the colon. Decreased blood flow can lead to a breakdown of the lining of the colon (mucosa), making it susceptible to infection.

    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 also might 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 inherited heart problems and Down syndrome. It may also be associated with multiple endocrine neoplasia, type IIB a syndrome that causes noncancerous tumors in the mucous membranes and adrenal glands (located above the kidneys) and cancer of the thyroid gland (located at the base of the neck). Roughly a third of children who have Hirschsprung's disease have other abnormalities.
    • Enterocolitis can be a life-threatening complication. It's treated in the hospital with colon cleaning and antibiotics.


    Preparing for your appointment
    Hirschsprung's disease is often diagnosed in the hospital shortly after birth. Signs of the disease can also show up later. If your child has signs or symptoms that worry you, particularly constipation and a swollen abdomen, consult the doctor. You may 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, and what to expect from the doctor.
    Hirschsprung's Disease Pictures Pediatric cases attachment.php?s=e7debc9ee65f0c76ccb5a3a62e8719ac&attachmentid=315&d=1435791679

    Tests and diagnosis
    Your child's doctor may 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. During the manometry test, the doctor inflates a balloon inside the rectum. The surrounding muscle should relax as a result. If it doesn't, Hirschsprung's disease may be the cause.
    • Removing a sample of colon tissue for testing. Removing a sample of tissue for testing (biopsy) is the surest way to identify Hirschsprung's disease. A biopsy sample can be collected using a suction device and is done on an outpatient basis, meaning it doesn't require a hospital stay.


    References:
    Hirschsprung's disease Tests and diagnosis - Mayo Clinic











    Last edited by Medical Photos; 09-01-2015 at 07:34 PM.

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