Hirschsprung’s disease is
So the correct answer is likely related to the absence of ganglion cells in the myenteric plexus. The options might include other possibilities like a stricture or tumor, but the key is the lack of ganglion cells. For example, if the options are A) congenital absence of ganglion cells in the myenteric plexus, B) excessive ganglion cells, etc., then A is correct.
Now, why each wrong option is incorrect. Let's say option B is about a stricture. Hirschsprung's is not a stricture but a functional issue. Option C might be a tumor, but it's a congenital absence. Option D could be something else like a motility issue due to another cause. The clinical pearl is that Hirschsprung's is diagnosed via rectal biopsy showing no ganglion cells and increased acetylcholine receptors. The correct answer here would be A.
**Core Concept**
Hirschsprung's disease is a **congenital aganglionic megacolon** caused by the **absence of ganglion cells in the myenteric (Auerbach’s) and submucosal (Meissner’s) plexuses** of the distal colon. This leads to uncoordinated peristalsis and functional obstruction.
**Why the Correct Answer is Right**
The absence of ganglion cells in the bowel wall prevents normal peristalsis, causing proximal bowel dilation and chronic constipation. The aganglionic segment is typically the rectosigmoid junction, but it can extend proximally. Diagnosis is confirmed by **rectal biopsy** showing **no ganglion cells** and **hyperplasia of submucosal ganglion cells** (acetylcholine receptor clusters).
**Why Each Wrong Option is Incorrect**
**Option A:** *Congenital stricture of the rectum* – Incorrect. Hirschsprung’s is a functional, not structural, obstruction.
**Option B:** *Neuroendocrine tumor of the colon* – Incorrect. Tumors do not cause aganglionosis.
**Option C:** *Acquired motility disorder* – Incorrect. Hirschsprung’s is congenital, not acquired.
**Clinical Pearl / High-Yield Fact**
Remember: **"Hirschsprung’s = no ganglion cells + transition zone + ‘microcolon’ on enema."** The classic triad of symptoms in neonates is **constipation, abdominal distension, and vomiting**. Pull-through surgery (e.g., Soave procedure) is the treatment.
**Correct Answer: A. Congenital absence of ganglion cells in the myenteric plexus**