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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
CX of HD: severe
- (+) fever, severe prostration, GIT bleeding and explosive watery diarrhea.
- inflammation of both the small intestine and the colon
- ~mild or moderate:
- -relieve chronic constipation (stool softeners and rectal irrigation).
- ~moderate to severe:-TWO-STEP SURGICAL PROCEDURE
- 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.
- 1. NB:
- -failure to thrive
- -refusal to suck
- -abdominal distention
- -bile-stained vomitus
- 2. CHILDREN:
- -failure to gain weight and delayed groeth
- -abdominal distention
- -constipation alternating diarrhea
- -ribbon-like and foul-smelling stool
- 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.
- -bowel fxn
- -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.
- -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