Examination of infants affected with Hirschsprung disease reveals:

 Abdominal distention. Infants with aganglionic megacolon show tympanitic

abdominal distention and symptoms of intestinal obstruction.
 Chronic constipation. Older infants and children with Hirschsprung disease
usually present with chronic constipation.
 Palpable intestinal loops.  Upon abdominal examination, these children may
demonstrate marked abdominal distention with palpable dilated loops of
colon.
 Absence/delayed passage of meconium. During the newborn period, infants
affected with Hirschsprung disease may present with failure of passage of
meconium.
 Vomiting. Repeated vomiting is present due to intestinal obstruction.
 Malnourishement. Poor nutrition results from the early satiety, abdominal
discomfort, and distention associated with chronic constipation.
Assessment and Diagnostic Findings
The diagnosis of aganglionic megacolon is made through the following data:

 Laboratory studies. CBC count, order this test if enterocolitis is suspected;

elevation of WBC count or a bandemia should raise concern
for enterocolitis.
 Plain abdominal radiography. Perform this test with any signs or symptoms
of abdominal obstruction.
 Unprepared single-contrast barium enema. If perforation and enterocolitis are
not suspected, an unprepared single-contrast barium enema may help
establish the diagnosis by identifying a transition zone between a
narrowed aganglionic segment and a dilated and normally innervated
segment; the study may also reveal a nondistensible rectum, which is a
classic sign of Hirschsprung disease.
 Rectal biopsy. Diagnosis is confirmed through rectal biopsy.
 Rectal manometry. In older children who present with chronic constipation
and an atypical history for either Hirschsprung disease or functional
constipation, anorectal manometry can be helpful in making or excluding
the diagnosis.
Medical Management
Treatment involves:

 Initial therapy. If a child with Hirschsprung disease has symptoms and

signs of a high-grade intestinal obstruction, initial therapy should include
intravenous hydration, withholding of enteral intake, and intestinal and
gastric decompression.
 Decompression. Decompression can be accomplished through placement
of a nasogastric tube and either digital rectal examination or normal
saline rectal irrigations 3-4 times daily.
 Diet. A special diet is not required; however, preoperatively and in the early
postoperative period, infants on a nonconstipated regimen, such as breast
milk, are more easily managed.