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Question 4#

Which of the following statements regarding Hirschsprung disease is FALSE?

a. Constipation and abdominal distention are classic symptoms
b. Approximately 20% of cases are diagnosed beyond the newborn period
c. The underlying pathology is characterized by an absence of ganglion cells in Auerbach plexus
d. Decompressive ostomy should involve distal, nondilated bowel

Correct Answer is D

Comment:

Hirschsprung disease is characterized by the absence of ganglion cells in Auerbach plexus and hypertrophy of associated nerve trunks. It is thought to result from a defect in the migration of neural crest cells, which migrate from cephalad to caudad. In children who do not respond to nonoperative treatment, a decompressive stoma is required. It is important to ensure that this stoma is placed in ganglion-containing bowel, which must be confirmed by frozen section at the time of stoma creation. The hypertrophied, dilated portion of the intestine in Hirschsprung disease contains normal ganglion cells, and it is in the narrow segment of the colon distal to the dilated portion that ganglion cells are absent.