Aganglionic disease of the intestinal tract which causes inadequate motility is termed as:

ESIC Nursing Officer - 2019 (Shift-2)

Medical & Surgical Nursing

Easy

Appeared in: ESIC Nursing Officer - 2019 (Shift-2)

Explanation

Hirschsprung's disease is defined as a congenital condition where nerve cells, specifically ganglion cells of the myenteric (Auerbach) and submucosal (Meissner) plexuses, are absent from a portion of the intestinal tract.
This absence of ganglion cells (aganglionosis) disrupts normal peristalsis, leading to a functional, non-mechanical obstruction.
The affected segment of the bowel remains in a state of tonic contraction, causing stool to back up and the proximal, normally innervated bowel to become massively dilated (megacolon).
It is the only option listed that is fundamentally an 'aganglionic' disease.

Option A: Ulcerative colitis is an inflammatory bowel disease (IBD), not a congenital aganglionic condition. Its pathophysiology involves chronic inflammation and ulceration of the colonic mucosa.
Option B: Crohn's disease is also an IBD characterized by transmural inflammation that can occur anywhere in the GI tract, often in a discontinuous 'skip lesion' pattern. It is an inflammatory process, not a lack of nerve cells.
Option D: Diverticular disease is a structural condition involving the formation of outpouchings (diverticula) in the colon wall. It is related to diet and age, not a congenital absence of nerve cells.

Visual 1: Diagram: Illustration of Hirschsprung's disease, showing the narrowed, aganglionic distal segment and the dilated, healthy proximal segment (megacolon). This helps visualize the functional obstruction.
Visual 2: Histology: Microscopic image comparing a normal rectal biopsy with one from a patient with Hirschsprung's disease, highlighting the presence of ganglion cells in the normal sample and their absence in the diseased sample.

Clinical Relevance

Nursing practice connection: Knowing Pathophysiology of congenital intestinal motility disorders helps nurses interpret findings accurately and avoid errors in routine assessment, medication administration, and patient teaching.
Early recognition in newborns is critical. A nurse's assessment for failure to pass meconium within 48 hours, abdominal distension, and bilious vomiting is a key patient safety action.
Pre-operative nursing care involves maintaining NPO status, administering IV fluids, performing nasogastric decompression to prevent vomiting and aspiration, and monitoring abdominal girth.
Post-operatively, nurses must monitor for signs of Hirschsprung-Associated Enterocolitis (HAEC), a life-threatening complication. Key signs include fever, explosive and foul-smelling diarrhea, and severe abdominal distension.

How to Approach the Question

First, identify the core concept in the question stem. The key term here is 'aganglionic disease'.
Break down the term: 'a-' means without, and 'ganglionic' refers to ganglion cells (nerve cells). So, the question is asking for a disease caused by the absence of nerve cells in the intestine.
Evaluate each option based on this definition.
Recall or deduce the pathophysiology of each choice: Ulcerative colitis and Crohn's disease are inflammatory. Diverticular disease is structural.
Hirschsprung's disease is specifically defined by the congenital absence of ganglion cells.
Therefore, Hirschsprung's disease is the only option that matches the description of an 'aganglionic disease'.

Concept Tested & Keywords

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