Hirschsprung's Disease Study Guide

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Sample questions from Hirschsprung's Disease Study Guide

This material has 12 questions with answers and rationales. Here are the first 5.

1. 1.A nurse teaches the parents of a newborn with Hirschsprung disease and Down syndrome. Which explanation best describes the intestinal disorder?

   • A.A congenital narrowing of the pylorus prevents milk from leaving the stomach.
   • B.Missing ganglion cells in the distal bowel prevent normal relaxation and cause functional obstruction.
   • C.An immune reaction to dietary gluten damages the intestinal lining and causes constipation.
   • D.Abnormally thick intestinal secretions obstruct the bowel because pancreatic enzymes are absent.

   Rationale: Hirschsprung disease involves absent enteric ganglion cells in a distal bowel segment, impairing relaxation and propulsion. Down syndrome is an associated condition. Pyloric narrowing describes pyloric stenosis, gluten-related injury describes celiac disease, and thick intestinal contents suggest meconium ileus associated with cystic fibrosis rather than aganglionosis.

2. 2.A 4-year-old has severe constipation dating from infancy, abdominal distention, and poor weight gain. The parent recalls that the child first passed meconium more than 48 hours after birth. Which diagnostic teaching is most accurate?

   • A.A contrast enema alone always confirms the diagnosis by showing a transition zone.
   • B.Anorectal manometry confirms the diagnosis whenever resting anal pressure is elevated.
   • C.Rectal biopsy confirms the diagnosis by demonstrating absent ganglion cells; contrast enema and manometry provide supporting information.
   • D.Improvement after a laxative trial rules out Hirschsprung disease and eliminates the need for further testing.

   Rationale: Rectal biopsy demonstrating absent ganglion cells is the definitive diagnostic test. Contrast enema may show a transition zone but cannot always confirm or exclude disease. Manometry assesses the rectoanal inhibitory reflex; its absence supports suspicion, whereas elevated resting pressure alone is not diagnostic. A temporary response to laxatives does not exclude Hirschsprung disease, especially with delayed meconium passage, chronic distention, and poor growth.

3. 3.An infant awaiting surgery for Hirschsprung disease develops fever, increasing abdominal distention, explosive foul-smelling diarrhea, lethargy, and fewer wet diapers. What is the nurse's priority action?

   • A.Notify the surgical team immediately, assess perfusion, and prepare for prescribed bowel rest, IV fluids, antibiotics, and decompression.
   • B.Document that diarrhea indicates relief of the obstruction and continue routine observation.
   • C.Give a prescribed as-needed oral laxative before reporting the change in condition.
   • D.Encourage large oral feedings to replace stool losses and reassess at the next scheduled visit.

   Rationale: These findings suggest Hirschsprung-associated enterocolitis with possible dehydration and sepsis, requiring urgent assessment and treatment. Diarrhea does not exclude obstruction or indicate recovery. A laxative is not appropriate initial treatment for suspected enterocolitis and could delay emergency care. Large oral feedings are unsafe in this acutely ill infant; bowel rest and IV resuscitation may be needed.

4. 4.A hospitalized infant with Hirschsprung disease is receiving preoperative care for bowel obstruction. Which nursing plan is most appropriate?

   • A.Give an oral stimulant laxative daily because medication can restore ganglion cells before surgery.
   • B.Use tap-water enemas whenever the abdomen appears larger, without waiting for a prescription.
   • C.Continue unrestricted formula feeds despite bilious vomiting to prevent poor weight gain.
   • D.Maintain prescribed bowel rest and decompression, give ordered IV fluids, monitor hydration and electrolytes, and perform saline rectal irrigations only as prescribed.

   Answer and rationale are in the full material.

5. 5.A child is preparing for discharge after a pull-through procedure for Hirschsprung disease. Which parent statement indicates correct understanding of postoperative home care and long-term follow-up?

   • A.The surgery eliminates the possibility of enterocolitis, so fever with diarrhea can be managed at home.
   • B.We will follow the prescribed stool and skin-care plan, seek urgent care for fever with distention or explosive diarrhea, and keep follow-up visits for growth and bowel function.
   • C.If stool leakage occurs, we should restrict fluids and avoid discussing it with the child or care team.
   • D.We should begin daily rectal dilations even though the surgeon has not prescribed them, and stop follow-up once the incision heals.

   Answer and rationale are in the full material.