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Ward Class is an online resource for student nurses. Created with the aim of helping student nurses cope with the demands of nursing school and clinicals, it offers free downloads, notes, sample NCPs, sample drug studies, study aids, news and updates, and practical tips to its users.

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Gastrointestinal Disorders: Hirschsprung's Disease


  • also called Aganglionic Megacolon
  • absence of ganglionic innervation to the muscle of a section of the large intestines, often the the lower portion of the sigmoid colon just above the anus
  • causes an absence of peristaltic waves to further the passage of stool through the affected part of the large intestines-->results in chronic constipation or small, ribbon-like stools
  • a familial disease diagnosed during infancy; occurs more often in males than in females
  • caused by an abnormal gene on chromosome 10; incidence is 1 in 5000 live births
  • associated with Down Syndrome
Assessment Findings
  • failure or delay in passing meconium within the first or second day of life
  • anorexia, nausea, and vomiting (vomitus may contain bile)
  • constipation and abdominal distension
  • ribbonlike stools (Note: often emphasized in most reference books)
  • diarrhea as only liquid can pass through impaction
  • lack of weight gain
  • problems absorbing nutrients-->leads to weight loss or growth failure
  • infections in the colon especially in newborns or very young children--e.g. enterocolitis due to fecal stagnation
  • anemia in older children--> due to blood lost in the stool
  • diagnostic test: rectal biopsy shows lack of innervation by the presence of aganglionic cells
Medical Management
  1. stool softeners--> usually given daily
  2. isotonic enemas-->prescribed to achieve daily bowel movements; normal saline (.9% NaCl) is instilled into the bowel
  3. dietary regimen-->low-residue diet with vitamin supplements
Surgical Management
  • surgery is the only proven and effective treatment for Hirschsprung's disease-->involves a pull-through surgery; the section of the colon that has no ganglia cells is removed and then remaining healthy end of the colon is connected to the rectum
  • for palliative surgery (for relief of symptoms only), a loop or double-barrel colostomy is performed
Nursing Management

1. administer enemas as ordered
  • mineral oil or isotonic saline (.9% NaCl) is used
  • never use tap water as it is hypotonic-->causes water intoxication leading to cardiac congestion or cerebral edema and resulting deaths
  • use volume appropriate to child's weight: 150-200 ml for infants and 250-500 ml for children
2. do not treat loose stools
3. administer TPN as ordered-->usually prescribed to offer another source of nutrition
4. provide a low-residue diet-->omits irritants from the intestinal tract
5. instruct caregivers on colostomy care and low-residue diet to prevent complications
  • discuss omission of milk, fried and highly-seasoned food from diet
Prognosis

Overall prognosis is very good. Most infants achieve good bowel control after surgery.

Prevention

Hirschsprung's disease is a congenital abnormality and is not preventable.

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