Hirschsprung Disease

Question 1

Def

Answer 1

disorder of motility of the intestinal track resulting in obstruction

due to a lack of ganglion cells in the intestine, which causes inadequate motility in part of the intestine.

AKA congenital aganglionic megacolon

Question 2

S/S

Answer 2

• failure to pass stool (meconium) within 1st 24 hr of life
• ## abdominal distention

Question 3

Management/ Tx

Answer 3

Management:
- Observe for the following signs and symptoms of enterocolitis after postop: fever, abdominal distention, chronic diarrhea or explosive stools, rectal bleeding, or straining. If any of the above symptoms are noted, immediately notify the physician or nurse practitioner
- bowel rest, if fluids, and antibiotics after post op

Tx:
- enema
- Surgical resection of the aganglionic bowel and reanastomosis of the remaining intestine are necessary to promote proper bowel function.