HIRSCHSPRUNGS DX
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| Definition | massive enlargement of the colon, resulting from obstructio caused by an AGANGLIONIC segment of the bowel
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| patho | the absence of ganglion cells in a portion of the colon prevents peristalsis in that segment of bowel;
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| resulting in | accumulation of stool leads to dilation of the proximal bowel and abdominal distention
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| failure of the internal sphincter to relax | preventing the evacuation of stool in response to rectal distention
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| 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
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| other symptoms in INFANTS | refusal to suck, abdominal distention, bile stained emesis, untreated the condition progresses to complete obstruction, resp distress and shock
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| 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.
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| 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,
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| diagnostics | rectal biopsy, upper GI series
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| medical tx= mild | dietary modification, stool softeners, isotonic irrigation; goal is to PREVENT impaction untill the child is potty-trained
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| 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
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| 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
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| post op | monitor for infection,pain, hydration-iv fluids, abd girth, NG , ostomy, skin integrity, parent teaching
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| discharge planning | parents need to be tought; care of ostomy appliance , prevention of skin breakdown, management of mutrition and hydration, and signs of complication
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