Page 976 - Saunders Comprehensive Review For NCLEX-RN

f. Change the dressing as prescribed, and
record the type and amount of
drainage.
g. Perform wound irrigations if
prescribed.
h. Maintain nasogastric tube suction and
patency of the tube if present.
i. Administer antibiotics and analgesics as
prescribed.
X. Hirschsprung’s Disease (Fig. 33-4)
A. Description
1. Hirschsprung’s disease is a congenital anomaly also
known as congenital aganglionosis or aganglionic
megacolon.
2. The disease occurs as the result of an absence of
ganglion cells in the rectum and other areas of the
affected intestine.
3. Mechanical obstruction results because of inadequate
motility in an intestinal segment.
4. The disease may be a familial congenital defect or may
be associated with other anomalies, such as Down’s
syndrome and genitourinary abnormalities.
5. A rectal biopsy specimen shows histological evidence
of the absence of ganglionic cells.

6. The most serious complication is enterocolitis;

signs include fever, severe prostration,
gastrointestinal bleeding, and explosive watery
diarrhea.
7. Treatment for mild or moderate disease is based on
relieving the chronic constipation with stool softeners
and rectal irrigations; however, many children require
surgery.
8. Treatment for moderate to severe disease involves a 2-
step surgical procedure.
a. Initially, in the neonatal period, a
temporary colostomy is created to
relieve obstruction and allow the
normally innervated, dilated bowel to
return to its normal size.
b. When the bowel returns to its normal
size, a complete surgical repair is
performed via a pull-through
procedure to excise portions of the
bowel; at this time, the colostomy is
closed.

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