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Hirschsprungs DX

HIRSCHSPRUNGS DX

PropertyDescription
Definition massive enlargement of the colon, resulting from obstructio caused by an AGANGLIONIC segment of the bowel
patho the absence of ganglion cells in a portion of the colon prevents peristalsis in that segment of bowel;
resulting in accumulation of stool leads to dilation of the proximal bowel and abdominal distention
failure of the internal sphincter to relax preventing the evacuation of stool in response to rectal distention
clinical s/s should be suspected in any infant who fails to pass meconium in the 1st 24hrs, who requires rectal stimulation to induce bowel movements
other symptoms in INFANTS refusal to suck, abdominal distention, bile stained emesis, untreated the condition progresses to complete obstruction, resp distress and shock
symptoms in OLDER CHILDREN failure to gain wt, delayed growth, anemia, hx of abd distention, severe constipation alternating with diarrhea vomiting, stool have ribbon like appearance.
ALERT!! the occurence of FEVER AND DIARRHEA in children with Hirschsprung's are ominous signs that suggest the presence of a coexisting ENTEROCOLITIS. the infamation of the bowel alon with the distention increases the chance of bowel perforation, septic shock,
diagnostics rectal biopsy, upper GI series
medical tx= mild dietary modification, stool softeners, isotonic irrigation; goal is to PREVENT impaction untill the child is potty-trained
surgical correction 1st=temp colostomy to decompress and rest the bowel 2nd excision of the aganglionic bowel and reanastomosis of normal bowel by pulling the ganglionc segment down thru the rectum
timing of surgical repair colostomy- done as soon as the dx is made and the child is stable, removal of aganglionic segment and the reanastomosis of the bowel- done when child is approx 10kg or 1yr
post op monitor for infection,pain, hydration-iv fluids, abd girth, NG , ostomy, skin integrity, parent teaching
discharge planning parents need to be tought; care of ostomy appliance , prevention of skin breakdown, management of mutrition and hydration, and signs of complication
Created by: meinmethoo on 2004-06-07



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