Hirschsprung's disease

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    • -congenital anomaly.
    • -also known as congenital aganglionosis or aganglionic megacolon. 
    • -ABSENCE of GANGLIONIC CELLS in the rectum and other areas of the intestine. 
    • -(+)mechanical obstruction bec of inadequate motility of the intestinal segment. 
    • -may be familial or assoc with down syndrome and genitourinary abn. 
    • -RECTAL BIOPSY: (-)ganglion cells

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  2. CX of HD: severe
    • (+) fever, severe prostration, GIT bleeding and explosive watery diarrhea.
    • inflammation of both the small intestine and the colon
  3. TX:
    • ~mild or moderate:
    • -relieve chronic constipation (stool softeners and rectal irrigation).

    • ~moderate to severe:
    • a. neonatal period: 
    • -(+)temporary colostomy is created (relieve obstruction and allow the normally innervated. dilated bowel to return to its size.
    • b. bowel return to normal size:
    • -complete surgical repair is performed via PULL-THROUGH procedure to excise portions of the bowel; colostomy is closed.
  4. ASSESS:
    • 1. NB:
    • -failure to thrive
    • -refusal to suck
    • -abdominal distention
    • -bile-stained vomitus

    • 2. CHILDREN:
    • -failure to gain weight and delayed groeth
    • -abdominal distention
    • -vomiting
    • -constipation alternating diarrhea
    • -ribbon-like and foul-smelling stool
  5. INT:
    • Medical management:
    • -low-fiber, high-caloric diet, high-protein diet
    • -extreme nutrition: parenteral nutrition
    • -stool softeners
    • -daily rectal irrigation (normal saline) to promote elimination and prevent obstruction.
    • PREOP:
    • -bowel fxn
    • -NPO
    • -monitor: hydration, f&e and resp distress assoc with abdominal distention. 
    • -IV fluids
    • -antibiotics or colonic irrigation with an antibiotic soln to clear the bacteria in the bowel.
    • -avoid taking temp rectally
    • -measure abdominal girth daily and weigh pt daily.

    • POSTOP:
    • -VS (avoid taking temp rectally)
    • -measure abd girth daily and PRN
    • -assess surgical site for: redness, swelling and drainage
    • -STOMA: if present for bleeding or skin breakdown (N: moist and red)
    • -anal area: assess for (+) stool, redness or discharge.
    • -NPO until bowel sounds return or flatus is passed (48-72 hours)
    • -maintain NGT until peristalsis returns
    • -IV fluid until the child tolerates oral intake (diet: clear liquids to regular)
    • -assess: dehydration, fluid overload and pain
    • -monitor I&O
    • -weigh daily
    • -parents: colostomy care and skin care
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Hirschsprung's disease
review about HD
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