Gastrointestinal: Hirschsprung disease: practice question

A Pathology question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

Try it

Pathology · Gastrointestinal: Hirschsprung disease

Sample item

A newborn has not passed meconium in 48 hours and has abdominal distension. Rectal examination reveals a tight, empty rectum followed by explosive passage of stool and gas. Which tissue finding on rectal suction biopsy confirms the diagnosis?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

B. Absence of ganglion cells in the submucosal plexus with hypertrophic nerve trunks

Hirschsprung disease results from failure of neural crest cells to migrate into the distal bowel, often with RET mutations. The aganglionic segment, starting at the rectum, cannot relax, so proximal bowel dilates. Biopsy shows absent ganglion cells in Meissner and Auerbach plexuses with hypertrophic nerve bundles, and staining for acetylcholinesterase is increased.

Option by option

  • A. Pneumatosis intestinalis with transmural coagulative necrosis

    Pneumatosis with necrosis describes necrotising enterocolitis in premature infants.

  • B. Absence of ganglion cells in the submucosal plexus with hypertrophic nerve trunks (correct)

    Correct. Aganglionosis with hypertrophic nerves is the diagnostic histology.

  • C. Hyperplasia of submucosal ganglia with giant ganglia

    Giant ganglia describe intestinal neuronal dysplasia, a different and rarer motility disorder.

  • D. Inspissated meconium plugs obstructing the ileum

    Inspissated meconium describes meconium ileus, a feature of cystic fibrosis.

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.

More Pathology questions

Questions about this Pathology question

What is the answer to this Pathology question?

The best answer is B, Absence of ganglion cells in the submucosal plexus with hypertrophic nerve trunks. Hirschsprung disease results from failure of neural crest cells to migrate into the distal bowel, often with RET mutations.

Why is "Pneumatosis intestinalis with transmural coagulative necrosis" not the answer?

Pneumatosis with necrosis describes necrotising enterocolitis in premature infants.

Why is "Hyperplasia of submucosal ganglia with giant ganglia" not the answer?

Giant ganglia describe intestinal neuronal dysplasia, a different and rarer motility disorder.

Why is "Inspissated meconium plugs obstructing the ileum" not the answer?

Inspissated meconium describes meconium ileus, a feature of cystic fibrosis.

What concept does this question test?

It tests Gastrointestinal: Hirschsprung disease in Pathology. The aganglionic segment, starting at the rectum, cannot relax, so proximal bowel dilates. Biopsy shows absent ganglion cells in Meissner and Auerbach plexuses with hypertrophic nerve bundles, and staining for acetylcholinesterase is increased.

How hard is this question?

We tag it as an application question: it asks you to use a fact on a patient or a finding. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Gastrointestinal: Hirschsprung disease?

Learn each disease as a bundle: cause, key histology, markers, and the clinical hallmark. Then use the flashcards and the other Pathology questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.