The commonest congenital gut motility disorder is:

AIIMS Raipur NO- 2019 (shift 3rd)

Child Health Nursing (Pediatrics)

Easy

Appeared in: AIIMS Raipur NO- 2019 (shift 3rd)

Explanation

Hirschsprung disease is a congenital disorder, meaning it is present at birth.
It is specifically a motility disorder caused by the absence of ganglion nerve cells (aganglionosis) in the distal bowel wall.
This lack of innervation prevents peristalsis and leads to a functional obstruction, making it the classic example of a congenital gut motility disorder.
It is the most common cause of lower intestinal obstruction in neonates, occurring in about 1 in 5,000 live births.

Option B: Constipation is a symptom, not a specific disease entity. It describes the difficult or infrequent passage of stool.
Option C: A choledochal cyst is a congenital anomaly of the biliary tree (bile ducts), not the intestine. It affects bile drainage, not gut motility.
Option D: Intussusception is a mechanical obstruction caused by the telescoping of one bowel segment into another. It is typically an acquired condition in infancy, not a primary congenital motility disorder.

Clinical Relevance

Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Congenital gastrointestinal motility disorders as background academic context rather than a clinical decision trigger.
A key nursing responsibility is to assess newborns for the passage of meconium within the first 24-48 hours. Failure to do so is a red flag for Hirschsprung disease.
Nurses must monitor for signs of Hirschsprung-Associated Enterocolitis (HAEC), such as fever, explosive diarrhea, and abdominal distension, as it is a life-threatening emergency requiring immediate intervention.
What if? If an older child presents with chronic constipation and failure to thrive, but had a normal passage of meconium at birth, functional constipation is more likely. However, Hirschsprung disease can have a delayed presentation (especially short-segment disease) and should still be considered if constipation is severe and refractory to treatment.

How to Approach the Question

First, analyze the question to identify the key concepts: 'commonest', 'congenital' (present at birth), and 'gut motility disorder' (problem with intestinal movement).
Evaluate each option against these three criteria.
Eliminate 'Constipation' as it is a symptom, not a specific congenital disorder.
Eliminate 'Choledochal cyst' because it affects the bile ducts, not gut motility.
Eliminate 'Intussusception' as it's a mechanical obstruction and typically acquired, not a primary congenital motility issue.
Identify 'Hirschsprung disease' as the only option that is a primary congenital disorder characterized by failed gut motility.

Concept Tested & Keywords

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E6 Pathology- ROBBINS & COTRAN PATHOLOGIC BASIS OF DISEASE 10TH Ed p. 757-759

E6 Nelson Textbook of Pediatrics(2024) — Volume 2 pp. 160-162, 76-78