RNC-NIC Congenital Anomalies 4 — Questions and Answers
Question 1: A neonate is born with VACTERL association. Which component of this association poses the MOST immediate threat to survival?
- Cardiac defects (Correct answer)
- Vertebral anomalies
- Limb abnormalities
- Anal atresia
Correct answer: Cardiac defects
Cardiac defects in VACTERL association (occurring in ~75% of cases) pose the most immediate life-threatening risk and require urgent evaluation.
Question 2: When caring for a neonate with an imperforate anus, which finding indicates a LOW lesion (below the levator ani) with the BEST prognosis for continence?
- Anal dimple present with meconium visible at the perineum within 24 hours (Correct answer)
- Flat perineum with no anal dimple and meconium in urine
- Abdominal distension without meconium passage after 24 hours
- Meconium seen in the vaginal introitus
Correct answer: Anal dimple present with meconium visible at the perineum within 24 hours
A visible anal dimple and perineal meconium within 24 hours indicate a low lesion, which has better sphincter preservation and continence outcomes.
Question 3: A neonate with a myelomeningocele at the L3-L4 level. Which functional deficit should the nurse anticipate?
- Flaccid paralysis of the lower extremities with bladder and bowel dysfunction (Correct answer)
- Normal lower extremity function with isolated bladder dysfunction
- Spastic upper extremity paralysis only
- Complete sensory loss without motor deficit
Correct answer: Flaccid paralysis of the lower extremities with bladder and bowel dysfunction
L3-L4 lesions affect motor and sensory function below that level, causing lower extremity paralysis and neurogenic bladder and bowel.
Question 4: A nurse is preparing to care for a neonate immediately post-op repair of myelomeningocele. Which position is CONTRAINDICATED?
- Supine with the operative site directly against the bed surface (Correct answer)
- Prone with the head turned to the side
- Side-lying with a roll to support the back
- Prone with the chest elevated on a small foam roll
Correct answer: Supine with the operative site directly against the bed surface
Supine positioning places direct pressure on the surgical repair site and is contraindicated; prone or side-lying positions protect the wound.
Question 5: Which PRENATAL finding on ultrasonography is MOST suggestive of an open neural tube defect?
- Lemon sign (frontal bossing) and banana sign (curved cerebellum) (Correct answer)
- Polyhydramnios with a normal intracranial structure
- Hyperechogenic bowel with dilated cerebral ventricles
- Echogenic cardiac focus with absent nasal bone
Correct answer: Lemon sign (frontal bossing) and banana sign (curved cerebellum)
The lemon sign (scalloped frontal bones) and banana sign (effaced cerebellum pulled into the foramen magnum) are hallmark ultrasound findings of open spina bifida.
Question 6: A neonate is born with bilateral renal agenesis (Potter sequence). Which physical finding results directly from severe oligohydramnios?
- Pulmonary hypoplasia causing respiratory failure at birth (Correct answer)
- Cleft palate from restricted oral movement
- Congenital cataracts from reduced amniotic fluid bathing
- Microcephaly from external cranial compression
Correct answer: Pulmonary hypoplasia causing respiratory failure at birth
Oligohydramnios from absent fetal urine production restricts lung development, causing lethal pulmonary hypoplasia as the primary cause of death.
Question 7: A neonate with ambiguous genitalia has a 46,XX karyotype and elevated 17-hydroxyprogesterone. Which condition does this MOST likely represent?
- Congenital adrenal hyperplasia (21-hydroxylase deficiency) (Correct answer)
- Complete androgen insensitivity syndrome
- Turner syndrome with virilization
- Ovotesticular disorder of sex development
Correct answer: Congenital adrenal hyperplasia (21-hydroxylase deficiency)
Elevated 17-OHP in a 46,XX infant confirms 21-hydroxylase deficiency CAH, causing androgen excess and virilization of female external genitalia.
RNC-NIC Congenital Anomalies 4
A neonate is born with VACTERL association.
Which component of this association poses the MOST immediate threat to survival?