Which condition occurs due to the congenital absence of parasympathetic ganglionic nerve cells in the colon?

DSSSB - 28 August 2019 (Shift-1)

Child Health Nursing (Pediatrics)

Easy

Appeared in: DSSSB - 28 August 2019 (Shift-1)

Explanation

Hirschsprung's disease, also known as congenital aganglionic megacolon, is defined by the absence of ganglion cells in the distal intestine.
This absence is a result of the failure of neural crest cells to migrate to the wall of the colon during embryonic development.
The lack of these nerve cells prevents peristalsis (coordinated muscle contractions) in the affected segment, leading to a functional bowel obstruction.
This condition typically presents in newborns with failure to pass meconium, abdominal distension, and constipation.

Option A: Volvulus is a mechanical obstruction caused by the twisting of the intestine on itself, which cuts off blood supply. It is not caused by an absence of nerve cells.
Option C: Intestinal obstruction is a general term for a blockage in the intestines. While Hirschsprung's disease causes an intestinal obstruction, it is not the specific diagnosis. The question asks for the specific condition defined by the absence of ganglion cells.
Option D: Meconium ileus is an obstruction of the small intestine (ileum) caused by abnormally thick and sticky meconium. It is most commonly seen in newborns with cystic fibrosis and is not related to the absence of ganglion cells in the colon.

Clinical Relevance

Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Pathophysiology of congenital gastrointestinal disorders as background academic context rather than a clinical decision trigger.
A key nursing assessment for newborns is monitoring for the passage of the first meconium stool. Failure to pass meconium within 24-48 hours is a red flag for Hirschsprung's disease and requires immediate reporting.
Post-operative nursing care for a child after surgical correction (pull-through procedure) involves monitoring for signs of infection, managing pain, providing ostomy care if applicable, and educating parents on long-term bowel management.
What if? If an older child presents with severe, chronic constipation that is resistant to standard treatments, and a rectal exam reveals an empty rectum, the nurse should suspect Hirschsprung's disease, as it can sometimes have a delayed presentation.

How to Approach the Question

First, identify the key phrase in the question: 'congenital absence of parasympathetic ganglionic nerve cells in the colon'. This is a very specific pathophysiological description.
Next, analyze the options provided. Your goal is to match the specific description in the question stem to one of the disease names in the options.
Recall or look up the definitions of each condition. Volvulus is twisting, meconium ileus is thick meconium (related to cystic fibrosis), and intestinal obstruction is a general term.
Recognize that 'Hirschsprung's disease' is the specific medical term for congenital aganglionic megacolon, which perfectly matches the description in the question.
Eliminate the other options because they describe different mechanisms of bowel obstruction.

Concept Tested & Keywords

Concept Tested: Pathophysiology of congenital gastrointestinal disorders
Stem keywords: congenital absence, parasympathetic ganglionic nerve cells, colon
Lead-in keywords: Which condition
Negative lead-in flag: false

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