Which assessment finding should the nurse expect in an infant with Hirschsprung disease?
A.
Constipation with passage of foul-smelling, ribbon-like stools
B.
Diarrhea
C.
Foul-smelling, fatty stools
D.
“Currant jelly” stools
The Correct answer and Explanation is:
Correct Answer:
A. Constipation with passage of foul-smelling, ribbon-like stools
Explanation:
Hirschsprung disease, also known as congenital aganglionic megacolon, is a condition that affects the large intestine (colon) and causes problems with passing stool. This condition is present at birth (congenital) and is due to missing nerve cells in the muscles of the baby’s colon. The absence of these nerve cells (ganglia) in segments of the colon prevents the muscles in the bowel from contracting properly, leading to a blockage of the bowel and an inability to pass stool normally.
Assessment Findings:
The most characteristic assessment finding in an infant with Hirschsprung disease is constipation with passage of foul-smelling, ribbon-like stools. This occurs because the area of the colon affected by the absence of ganglion cells is unable to relax, resulting in a buildup of stool proximal to the affected segment. As the stool accumulates and is eventually forced through the narrow segment, it becomes thin, resembling a ribbon, and often has a foul odor due to prolonged stasis in the colon.
Explanation of the Options:
- Option A: Constipation with passage of foul-smelling, ribbon-like stools is the hallmark of Hirschsprung disease. The narrowed segment of the colon prevents normal stool passage, leading to constipation. As the stool is forced through, it takes on a ribbon-like appearance and may have a strong odor due to prolonged retention in the colon.
- Option B: Diarrhea is not typically associated with Hirschsprung disease. While some children might develop enterocolitis (inflammation of the intestine) as a complication of the disease, which can lead to diarrhea, the primary symptom of Hirschsprung disease is usually constipation rather than diarrhea.
- Option C: Foul-smelling, fatty stools, also known as steatorrhea, are more indicative of malabsorption syndromes like cystic fibrosis or celiac disease, where fat is not properly absorbed by the intestine. This is not a typical finding in Hirschsprung disease.
- Option D: “Currant jelly” stools refer to stools that are dark red and resemble jelly, often containing blood and mucus. This finding is typically associated with intussusception, a different gastrointestinal condition where one part of the intestine telescopes into another, causing obstruction and ischemia.
Clinical Implications:
Early diagnosis of Hirschsprung disease is crucial because it can lead to severe complications like enterocolitis, which can be life-threatening. If an infant presents with signs such as a failure to pass meconium within the first 48 hours after birth, chronic constipation, abdominal distension, or ribbon-like stools, Hirschsprung disease should be suspected. Diagnosis is typically confirmed with a rectal biopsy showing the absence of ganglion cells. The treatment for Hirschsprung disease usually involves surgical removal of the affected segment of the colon, followed by reconnecting the healthy parts of the intestine to allow normal bowel function.