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    Hirchsprung Disease: A.K.A. Congenital Aganglionic

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    desh_dacanay
    Hirschsprung disease is a congenital disorder caused by the absence of ganglion cells in parts of the intestine, resulting in inadequate motility. It occurs more often in males and has genetic and familial links in some cases. Clinical manifestations include constipation, abdominal distention, and failure to pass meconium in newborns. Diagnosis involves tests like barium enema and rectal biopsy to detect the absence of ganglion cells. Treatment is a pull-through surgery to remove the diseased intestine and reconnect the healthy parts.

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    Hirchsprung Disease: A.K.A. Congenital Aganglionic

    Uploaded by

    desh_dacanay
    260 views29 pages
    Hirschsprung disease is a congenital disorder caused by the absence of ganglion cells in parts of the intestine, resulting in inadequate motility. It occurs more often in males and has genetic and familial links in some cases. Clinical manifestations include constipation, abdominal distention, and failure to pass meconium in newborns. Diagnosis involves tests like barium enema and rectal biopsy to detect the absence of ganglion cells. Treatment is a pull-through surgery to remove the diseased intestine and reconnect the healthy parts.

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    Hirchsprung Disease

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    Hirschsprung disease is a congenital disorder caused by the absence of ganglion cells in parts of the intestine, resulting in inadequate motility. It occurs more often in males and has genetic and familial links in some cases. Clinical manifestations include constipation, abdominal distention, and failure to pass meconium in newborns. Diagnosis involves tests like barium enema and rectal biopsy to detect the absence of ganglion cells. Treatment is a pull-through surgery to remove the diseased intestine and reconnect the healthy parts.

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    260 views29 pages

    Hirchsprung Disease: A.K.A. Congenital Aganglionic

    Uploaded by

    desh_dacanay
    Hirschsprung disease is a congenital disorder caused by the absence of ganglion cells in parts of the intestine, resulting in inadequate motility. It occurs more often in males and has genetic and familial links in some cases. Clinical manifestations include constipation, abdominal distention, and failure to pass meconium in newborns. Diagnosis involves tests like barium enema and rectal biopsy to detect the absence of ganglion cells. Treatment is a pull-through surgery to remove the diseased intestine and reconnect the healthy parts.

    Copyright:

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    Download as pptx, pdf, or txt
    of 29

    HIRCHSPRUNG DISEASE

    A.k.a. Congenital Aganglionic


    Megacolon
    HIRCHSPRUNG DISEASE
    • Congenital anomaly that results in mechanical obstruction
    from inadequate motility of part of the intestine.
    • absence of ganglion cells
    -Myenteric plexus of Auerbach
    -Submucosal plexus of Meissner
    • These ganglion cells were formerly known as intramural
    ganglia of the parasympathetic nervous system
    -classified as elements of an independent enteric
    nervous system (ENS)
    HIRCHSPRUNG DISEASE
    • Four times more common in males than in
    females
    -follows a familial(family unit) pattern in a
    small number of cases
    • 80% (estimate) of the cases are due to
    autosomal dominant genetic mutations with
    incomplete penetrance
    -associated with down syndrome
    HIRCHSPRUNG DISEASE
    CLINICAL MANIFESTATIONS
    CLINICAL MANIFESTATIONS
    • Newborn
    -abdominal distention
    -vomiting
    -constipation
    -failure to pass the meconium within last
    24-48 hours of life
    CLINICAL MANIFESTATIONS
    • Neonates
    -signs of acute abdominal obstruction
    -relieved by rectal stimulation or
    enema
    -vomiting
    -delayed meconium passage
    CLINICAL MANIFESTATIONS
    • Older children
    -often have chronic constipation with
    passage of ribbon like, foul smelling stool
    and abdominal distention
    -have evidence of:
    -previous GI dysfunction
    -Failure to thrive
    -Chronic constipation
    DIAGNOSTIC EVALUATION
    DIAGNOSTIC EVALUATION
    • Suspected Diagnosis
    -(neonate)
    -clinical signs of intestinal obstruction
    -failure to pass meconium
    -(infants and older children)
    -medical history
    -constipation
    DIAGNOSTIC EVALUATION
    Barium enema often demonstrates the transition zone
    -between the dilated proximal (colon) megacolon
    and narrow distak segment may not develop until
    the age of two months or later
    Rectal biopsy
    -surgically- to obtain a full-thickness biopsy specimen
    -suction biopsy- for histologic evidence of the basic
    ganglion cells
     
    DIAGNOSTIC EVALUATION
    Anorectal manometry
    -a catheter with a balloon attached is inserted
    into the rectum
    -the test records the reflex pressure response
    to the internal anal sphincter to distention of
    balloon
    -normal response: relaxation of the internal
    sphincter
    PATHOPHYSIOLOGY
    PATHOPHYSIOLOGY
    NURSING INTERVENTION
    NURSING INTERVENTIONS
    Help the parents adjust to the congenital
    disorder
    -fostering an infant- parent bonding
    -prepare the parents for medical surgical
    intervention
    -assisting them in caring for the colostomy
    after discharge
    NURSING INTERVENTIONS
    • Post operative care
    -monitor bowel sounds and passafe of stool
    -will indicate when can oral feeding can be
    initiated
    • Home care
    -provide instructions about colostomy care
    -skin care, emptying and changing the ostomy
    surfaces, and monitoring for problems.
    TREATMENT
    TREATMENT
    Pull-through Surgery
    Hirschsprung's disease is treated with surgery. The
    surgery is called a pull-through operation. There are
    three common ways to do a pull-through, and they
    are called the Swenson, the Soave, and the
    Duhamel procedures. Each is done a little
    differently, but all involve taking out the part of the
    intestine that doesn't work and connecting the
    healthy part that's left to the anus. After pull-
    through surgery, the child has a working intestine
    TREATMENT
    Before surgery: The diseased section is
    the part of the intestine that doesn't work.
    TREATMENT
    Step 1: The doctor removes the diseased
    section.
    TREATMENT
    Step 2: The healthy section is attached to
    the rectum or anus.
    TREATMENT
    Colostomy and Ileostomy
      Often, the pull-through can be done right after
    the diagnosis. However, children who have
    been very sick may first need surgery called
    an ostomy. This surgery helps the child get
    healthy before having the pull-through. Some
    doctors do an ostomy in every child before
    doing the pull-through.
    TREATMENT
    Colostomy and Ileostomy
    In an ostomy, the doctor takes out the diseased
    part of the intestine. Then the doctor cuts a small
    hole in the baby's abdomen. The hole is called
    a stoma. The doctor connects the top part of the
    intestine to the stoma. Stool leaves the body
    through the stoma while the bottom part of the
    intestine heals. Stool goes into a bag attached to the
    skin around the stoma. You will need to empty this
    bag several times a day.
    TREATMENT
    Step 1: The doctor takes out most of the
    diseased part of the intestine.
    TREATMENT
    Step 2: The doctor attaches the healthy
    part of the intestine to the stoma (a hole in
    the abdomen).
    TREATMENT
    If the doctor removes the entire large
    intestine and connects the small intestine to
    the stoma, the surgery is called an ileostomy.
    If the doctor leaves part of the large intestine
    and connects that to the stoma, the surgery is
    called a colostomy.
    TREATMENT
    Later, the doctor will do the pull-through. The
    doctor disconnects the intestine from the
    stoma and attaches it just above the anus. The
    stoma isn't needed any more, so the doctor
    either sews it up during surgery or waits about
    6 weeks to make sure that the pull-through
    worked.
    • END :3

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