Nursing management of patients with congenital disorders
Time: 15:00
What is the priority pre-operative nursing intervention for a newborn with a myelomeningocele?
[a] Placing the infant in a supine position to facilitate bonding.
[b] Applying a dry sterile dressing to the sac to prevent infection.
[c] Positioning the infant prone and covering the sac with a sterile, moist, non-adherent dressing.
[d] Initiating oral feedings to assess for swallowing difficulties.
A nurse is feeding an infant with an unrepaired cleft palate. Which technique is most appropriate?
[a] Use a standard nipple with a fast flow rate.
[b] Position the infant flat on their back to prevent aspiration.
[c] Use a specialized nipple, hold the infant upright, and burp frequently.
[d] Feed the infant quickly to ensure adequate caloric intake.
An infant with Tetralogy of Fallot (TOF) becomes cyanotic and tachypneic after crying. The nurse's first action should be to:
[a] Administer oxygen via a face mask.
[b] Place the infant in a knee-chest position.
[c] Start an IV line for fluid administration.
[d] Notify the physician immediately.
A 4-week-old infant is diagnosed with pyloric stenosis. The nurse would expect the parent to report which classic symptom?
[a] Frequent, diarrheal stools.
[b] Bile-stained (green) vomitus.
[c] Projectile, non-bilious vomiting after feedings.
[d] A sausage-shaped mass in the upper right quadrant.
When assessing a newborn, the nurse suspects Developmental Dysplasia of the Hip (DDH). Which finding would support this suspicion?
[a] Symmetrical gluteal folds.
[b] Equal leg lengths.
[c] Asymmetry of the gluteal and thigh skin folds.
[d] Full abduction of both hips.
A newborn is suspected of having Hirschsprung's disease. The nurse should assess for which cardinal sign of this disorder in the neonatal period?
[a] A scaphoid abdomen.
[b] Failure to pass meconium within the first 24-48 hours.
[c] Frequent, watery stools.
[d] An olive-shaped mass in the epigastrium.
A nurse is caring for an infant with a newly placed ventriculoperitoneal (VP) shunt for hydrocephalus. The nurse should position the infant:
[a] In a prone position to protect the incision.
[b] In a Trendelenburg position to decrease intracranial pressure.
[c] On the non-operative side with the head of the bed flat or slightly elevated as ordered.
[d] In a semi-Fowler's position on the operative side.
The nurse is providing discharge teaching to parents of an infant in a Pavlik harness for DDH. Which statement indicates the parents understand the instructions?
[a] "We should adjust the straps daily to make sure they are tight."
[b] "We can remove the harness for several hours each day for bath time."
[c] "We need to check the skin under the straps for any redness or breakdown."
[d] "We should apply lotion or powder under the straps to prevent rubbing."
What are the "3 C's" that are classic signs of a tracheoesophageal fistula (TEF) in a newborn?
[a] Crying, Cuddling, and Comforting.
[b] Constipation, Cramping, and Colic.
[c] Coughing, Choking, and Cyanosis with feedings.
[d] Cool, Clammy, and Congested skin.
A nurse is caring for a child with Down Syndrome (Trisomy 21). The nurse knows that these children are at an increased risk for which of the following?
[a] Pyloric stenosis and muscular hypertrophy.
[b] Congenital heart defects and hypotonia.
[c] Neural tube defects and hydrocephalus.
[d] Renal failure and hypertension.
Post-operative nursing care for an infant following a cleft lip repair (cheiloplasty) should include:
[a] Placing the infant in a prone position to promote drainage.
[b] Using elbow restraints to protect the suture line.
[c] Offering a pacifier for comfort.
[d] Cleaning the suture line with full-strength hydrogen peroxide.
A key assessment finding in an infant with coarctation of the aorta is:
[a] A loud, harsh murmur at the left sternal border.
[b] Generalized cyanosis that does not improve with oxygen.
[c] Strong, bounding pulses in all four extremities.
[d] Bounding pulses in the arms with weak or absent pulses in the legs.
The nurse is administering pancreatic enzymes to a child with cystic fibrosis. The nurse should teach the parents to give the medication:
[a] One hour after meals.
[b] Mixed with a full glass of milk.
[c] With meals and snacks.
[d] Only when the child has fatty stools.
Which medication is often used to promote the closure of a patent ductus arteriosus (PDA) in a premature infant?
[a] Prostaglandin E1.
[b] Digoxin.
[c] Indomethacin.
[d] Furosemide.
Initial management of an infant born with clubfoot (talipes equinovarus) typically involves:
[a] Surgical correction within the first week of life.
[b] Observation, as many cases resolve spontaneously.
[c] Application of a series of casts or braces (Ponseti method).
[d] Use of corrective shoes with a special bar.
The parents of a school-age child with a VP shunt ask the nurse what symptoms would indicate a shunt malfunction. The nurse's best response would be:
[a] "A sunken fontanelle and decreased head circumference."
[b] "Increased appetite and a sudden growth spurt."
[c] "Headache, vomiting, and lethargy."
[d] "A high fever and a rash on the abdomen."
Which instruction is essential for a nurse to give parents before their child undergoes surgery for hypospadias repair?
[a] "Ensure your child is potty-trained before the surgery."
[b] "Limit fluid intake for 24 hours before the procedure."
[c] "Your child should not be circumcised, as the foreskin may be needed for the repair."
[d] "Practice using a urinary catheter with your child at home."
When feeding an infant with congestive heart failure (CHF) due to a ventricular septal defect (VSD), the nurse should implement which strategy?
[a] Use a low-calorie formula to reduce the workload on the heart.
[b] Encourage long feeding sessions to maximize intake.
[c] Provide small, frequent feedings with a higher-calorie formula.
[d] Position the infant flat during feedings to reduce tachypnea.
The nurse is caring for a child who has just returned from surgery for a cleft palate repair (palatoplasty). Which item should be kept away from the child?
[a] A soft stuffed animal.
[b] A cup for drinking liquids.
[c] A spoon or straw.
[d] A picture book.
The hallmark sign of a large Ventricular Septal Defect (VSD) on auscultation is a:
[a] Faint, blowing murmur heard best at the apex.
[b] Loud, harsh, holosystolic murmur at the left lower sternal border.
[c] Continuous machinery-like murmur under the left clavicle.
[d] Widely split and fixed S2 heart sound.
Post-operative care for a child with a temporary colostomy after surgery for Hirschsprung's disease includes:
[a] Restricting fluids to decrease ostomy output.
[b] Irrigating the stoma with sterile saline every shift.
[c] Assessing the stoma for color and providing skin care to the peristomal area.
[d] Keeping the ostomy bag on for 3-4 days before changing it.
A newborn is diagnosed with gastroschisis. The priority nursing care involves:
[a] Initiating gavage feedings with sterile water.
[b] Covering the exposed bowel with a sterile, moist dressing and placing the infant in a sterile bag.
[c] Vigorously cleaning the exposed bowel with an antiseptic solution.
[d] Placing the infant in a prone position to reduce pressure on the abdomen.
A common feeding challenge for an infant with Down Syndrome is related to:
[a] A hyperactive gag reflex.
[b] An unusually strong suck.
[c] Muscle hypotonia and a protruding tongue.
[d] A very small oral cavity.
The nurse's role in supporting the family of a child newly diagnosed with a major congenital anomaly is to:
[a] Provide all information in a single, detailed session to be efficient.
[b] Discourage the parents from expressing feelings of grief or guilt.
[c] Facilitate communication, provide emotional support, and refer them to appropriate resources.
[d] Make decisions about the child's care on behalf of the overwhelmed parents.
A child is prescribed digoxin for a congenital heart defect. The nurse should teach the parents to withhold the medication and call the clinic if the infant's heart rate is:
[a] Above 150 beats/minute.
[b] Exactly 100 beats/minute.
[c] Below 90-110 beats/minute (or as specified by the provider).
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