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Physiology · GI physiology: enteric nervous system
Sample itemA newborn has not passed meconium for 48 hours and has abdominal distension. Anorectal manometry shows absence of the normal fall in internal sphincter pressure when the rectum is distended with a balloon. What is the underlying defect?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Absence of enteric ganglion cells in the distal bowel, so the inhibitory reflex cannot be relayed
In Hirschsprung disease, neural crest cells fail to migrate to the distal bowel, leaving an aganglionic segment starting at the rectum. Without the intrinsic neurons that relay the rectoanal inhibitory reflex, the internal sphincter does not relax and the segment remains contracted, causing functional obstruction. Diagnosis is confirmed by rectal biopsy showing absent ganglion cells.
Option by option
A. Absence of enteric ganglion cells in the distal bowel, so the inhibitory reflex cannot be relayed (correct)
Absent ganglion cells remove the intrinsic pathway of the reflex.
B. Excessive inhibitory neuron activity causing tonic relaxation of the sphincter
Excess inhibition would cause incontinence, not obstruction.
C. Failure of the gastrocolic reflex from lack of gastrin
The gastrocolic reflex is not the reflex tested, and it does not depend on gastrin alone.
D. Spinal cord transection above the sacral segments
The rectoanal inhibitory reflex is intrinsic and persists after spinal transection.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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