2011 PREP SA on CD-ROM

Question: 50
A 27 weeks gestation preterm male infant who weighs 900 g is delivered at a community hospital by
emergent cesarean section due to abruptio placentae. After intubation in the delivery room, he is taken
to the nursery for stabilization, including umbilical venous line placement, prior to transfer to a tertiary
center.
Of the following, the MOST appropriate initial solution for parenteral administration should include

A. 5% dextrose
B. 5% dextrose and 0.2% sodium chloride
C. 10% dextrose
D. 10% dextrose and 0.2% sodium chloride
E. 0.9% sodium chloride

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Critique: 50

Preferred Response: C

After the resuscitation and stabilization of a very low-birthweight (VLBW) infant (birthweight =1,500 g),
the initial assessment should include the measurement of blood glucose and the initiation of parenteral
fluids with 10% dextrose solution. The VLBW infant requires a constant glucose infusion to prevent
hypoglycemia due to reduced endogenous glycogen and fat stores and a limited ability to perform
gluconeogenesis. The goal should be to maintain the blood glucose concentration greater than 50
mg/dL (2.8 mmol/L) in the first 24 hours after birth. This value is consistent with the normal fetal glucose
concentration in the second half of gestation, which is not less than 54 mg/dL (3.0 mmol/L).
Administration of 10% dextrose solution without electrolytes should be initiated at a rate of 80 mL/kg
per day in the VLBW infant, providing a glucose infusion rate of 5.5 mg/kg per minute. This supports the
glucose requirement for VLBW infants, which generally is 4 to 6 mg/kg per minute. For the preterm
infant, the blood glucose values must be monitored closely to avoid either hypoglycemia or
hyperglycemia. Hyperglycemia is defined as a blood glucose concentration greater than 120 mg/dL (6.7
mmol/L). Some preterm infants manifest hyperglycemia due to the high infusion rates needed to support
insensible fluid losses or endogenous glucose production in response to stress-reactive hormones such
as epinephrine.
The initial parenteral fluids for VLBW infants do not need to contain electrolytes. With the exception
of calcium, electrolytes rarely are needed until after the first 24 hours after birth. Administering
parenteral fluids with 5% dextrose puts the VLBW infant at risk for hypoglycemia because this provides
a glucose infusion rate of only 2.8 mg/kg per minute when run at 80 mL/kg per day.
References:
American Academy of Pediatrics. Sugar and safe care. In: Karlsen KA, ed. The S.T.A.B.L.E. Program:
Post Resuscitation/Pre-Transport Stabilization of Sick Infants: Guidelines for Neonatal Healthcare
Providers. 5th ed. Elk Grove Village, Ill: American Academy of Pediatrics; 2006:5-42
Angert R, Adam HM. In brief: care of the very low-birthweight infant. Pediatr Rev. 2009;30:32-35. DOI:
10.1542/10.1542/pir.30-1-32. Available at:
http://pedsinreview.aappublications.org/cgi/content/full/30/1/32
Hay WW Jr. Early postnatal nutritional requirements of the very preterm infant based on a presentation
at the NICHD-AAP workshop on research in neonatology. J Perinatol. 2006;26(suppl 2):s13-s18. DOI:
10.1038/sj.jp.7211426. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/16801962

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Question: 66
An infant is delivered at 35 weeks gestation by cesarean section because of worsening maternal
pregnancy-induced hypertension. The infant is initially vigorous but develops worsening respiratory
distress with an increasing oxygen requirement and an escalation of her work of breathing over the first
4 hours after birth. You place an umbilical venous line and obtain a chest radiograph (Item Q66).
Because she is requiring 60% hood oxygen to maintain saturations of 93%, you elect to intubate her
endotracheally.
Of the following, the treatment that is MOST likely to decrease her respiratory distress is

A. chest tube placement

B. increased oxygen delivery (to 100%)
C. inhaled nitric oxide
D. prostaglandin therapy
E. surfactant

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Question: 66

(Courtesy of B Carter)

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Critique: 66

Preferred Response: E

The infant described in the vignette has respiratory distress syndrome (RDS), which is treated best by
the administration of surfactant into the lungs via an endotracheal tube. RDS is caused by a deficiency
of pulmonary surfactant and is seen most commonly in the preterm infant. Surfactant is produced by the
type II pneumocytes in the lung, with production beginning around 23 weeks gestation and increasing
as the lungs evolve from the saccular to alveolar phase, during which time the type II pneumocytes
proliferate and mature. The presence of surfactant in the lungs improves alveolar stability by decreasing
surface tension, leading to increased pulmonary compliance. Surfactant deficiency is seen in 60% of
infants born before 28 weeks gestation and affects fewer than 5% of infants born after 34
weeks gestation.
The clinical symptoms of RDS include tachypnea, grunting, flaring, retractions, central cyanosis, and
apnea. Initially, the work of breathing is increased because of the need to distend the alveoli to prevent
the development of atelectasis. If atelectasis occurs, the functional residual capacity of the lung
decreases, leading to lung injury, protein exudation, and edema. The characteristic chest radiograph of
an infant who has RDS demonstrates a ground-glass appearance, with air bronchograms and diffuse
atelectasis (Item C66).
Treatment strategies for RDS are preventive, supportive, and interventional. Antenatal steroids have
been shown to decrease RDS, intraventricular hemorrhage, and death when administered to women at
high risk of preterm delivery prior to 34 weeks gestation. Infants manifesting mild RDS may receive
supplemental hood oxygen or continuous positive airway pressure. Intubation and delivery of
exogenous surfactant may be used prophylactically in infants younger than 28 weeks gestation or as
rescue therapy in older neonates manifesting the clinical and radiologic findings of RDS. Multiple studies
have demonstrated decreases in death and chronic lung disease with surfactant administration.
Pneumothorax requiring chest tube placement is a complication of surfactant deficiency due to
alveolar mismatch during ventilation, but it is not apparent on the chest radiograph for this infant. The
hypoxia found in the infant who has RDS is improved with increased oxygen delivery, but such delivery
does not improve the underlying cause of the respiratory distress or the ability to ventilate. It is essential
to consider additional diagnoses in a late preterm infant experiencing respiratory distress, including
pneumonia, persistent pulmonary hypertension, and cardiac disease. The apparent improvement in
saturation with oxygen therapy reported for this infant makes cyanotic heart disease less likely. If the
infant does not improve after surfactant administration, echocardiography should be undertaken to rule
out noncyanotic heart disease that requires prostaglandin therapy or coexisting pulmonary hypertension
that requires inhaled nitric oxide.
References:
Jobe AH. Pharmacology review: why surfactant works for respiratory distress syndrome. NeoReviews.
2006;7:e95-e106. Available at: http://neoreviews.aappublications.org/cgi/content/full/7/2/e95
Rodriquez RJ, Martin RJ, Fanaroff AA. Respiratory distress syndrome and its management. In: Martin
RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martins Neonatal-Perinatal Medicine: Diseases of the
Fetus and Infant. 8th ed. Philadelphia, Pa: Mosby Elsevier; 2006:1097-1107
Warren JB, Anderson JM. Core concepts: respiratory distress syndrome. NeoReviews.
2009;10:e351-e361. Available at: http://neoreviews.aappublications.org/cgi/content/full/10/7/e351

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Critique: 66

(Courtesy of B Carter)
Underinflation, a "ground glass" appearance, and air bronchograms (arrows)
are characteristic of respiratory distress syndrome.

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Question: 135
You are called to the newborn nursery to evaluate an infant who has a heart murmur. He was born at
term and has normal weight, length, and head circumference. On physical examination, you note
multiple unusual features: epicanthal folds with ocular hypertelorism; a broad, low nasal bridge; a short,
upturned nose (Item Q135); a grade III/VI harsh holosystolic murmur at the left sternal border; and
hypoplastic finger- and toenails.
Of the following, this infants unusual features are MOST likely related to prenatal exposure to

A. cocaine
B. methamphetamine
C. phenytoin
D. valproic acid
E. warfarin

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Question: 135

(Courtesy of M Rimsza)
Upturned nose, as described for the infant in the vignette.

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Critique: 135

Preferred Response: C

There is good evidence that the prenatal use of some antiepileptic drugs (AEDs) for the treatment of
seizure disorders is associated with a two- to threefold increased risk for congenital malformations in
exposed infants compared with the general population. Even so, it is generally accepted that the
benefits of treatment outweigh the risks for congenital anomalies. One of the challenges facing
researchers in this area is to design sufficiently large studies to draw meaningful conclusions because
epilepsy occurs in 6 in 1,000 pregnant women, and congenital malformations are present in 3 in 100 live
births in the general population.
The pattern of unusual features described for children who have been prenatally exposed to AEDs
commonly is called fetal anticonvulsant syndrome. This term refers to the overlap of features seen in
affected individuals, but it is important to recognize that each anticonvulsant may be associated with its
own characteristic abnormalities. For example, prenatal phenytoin exposure is associated with a broad,
low nasal bridge; epicanthal folds; wide-spaced eyes (hypertelorism); cardiovascular abnormalities; and
distal digital hypoplasia (fetal hydantoin syndrome), as described for the infant in the vignette. Prenatal
exposure to valproic acid is associated with an increased risk for neural tube defects as well as cardiac,
limb, and renal anomalies, and a characteristic facies (fetal valproate syndrome). Phenobarbital and
carbamazepine also are associated with increased risks for malformations and dysmorphic features
following prenatal exposure. Of note, polydrug therapy to control seizures in the pregnant woman is
associated with an increased risk for anomalies over monotherapy. In addition to malformations,
prenatal AED exposures are associated with developmental delays and cognitive impairment.
Prenatal cocaine exposure is not associated with a well-defined syndrome, and facial features are
typically normal. Cocaine is associated with placental abruption and fetal vascular disruption.
Prenatal exposure to methamphetamine is not known to be associated with unusual facial features or
birth defects. Long-term outcome information is not available for exposed individuals, but there is
concern for potential neurodevelopmental problems.
Approximately one third of individuals exposed to warfarin between 6 and 9 weeks gestation have
facial anomalies and bone stippling referred to as fetal warfarin syndrome. Facial anomalies include
nasal hypoplasia and a depressed nasal bridge that may contribute to upper airway obstruction.
Exposure to warfarin during the second and third trimesters is associated with central nervous system
abnormalities, most likely due to hemorrhage.
References:
Cocaine, Methamphetamine. REPROTOX. 2009
Cocaine, Methamphetamine. TERIS. 2009
Dean JCS, Hailey H, Moore SJ, Lloyd DJ, Turnpenny PD, Little J. Long term health and
neurodevelopment in children exposed to antiepileptic drugs before birth. J Med Genet.
2002;39:251-259. DOI: 10.1136/jmg.39.4.251. Available at:
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1735079/
Dolk H, McElhatton P. Assessing epidemiological evidence for the teratogenic effects of anticonvulsant
medications. J Med Genet. 2002;39:243-244. DOI: 10.1136/jmg.39.4.243. Available at:
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1735083/
Gallagher RC, Kingham K, Hoyme HE. Fetal anticonvulsant syndrome. In: Cassidy SB, Allanson JE,
eds. Management of Genetic Syndromes. 2nd ed. Hoboken, NJ: John Wiley & Sons; 2005:239-250
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Holmes LB. The teratogenicity of anticonvulsant drugs: a progress report. J Med Genet.
2002;39:245-247. DOI: 10.1136/jmg.39.4.245. Available at:
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1735092/
Jones KL. Fetal warfarin syndrome. In: Smiths Recognizable Patterns of Human Malformation. 6th ed.
Philadelphia, Pa: Elsevier Saunders; 2006:656-657

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Question: 151
You are examining a baby girl who was brought to the clinic by her foster mother for her 2-week health
supervision visit. You do not have access to her prenatal or newborn records, but the foster parent tells
you that the infants mother is a polydrug abuser who was incarcerated shortly before delivery. No
information is available on the babys father. On physical examination, the babys weight, length, and
head circumference are less than the 5th percentiles. She is irritable and tremulous, and she has short
palpebral fissures, midface hypoplasia, a narrow upper vermilion border, and hirsutism (Item Q151).
Of the following, this babys features are MOST likely due to prenatal exposure to

A. alcohol
B. barbiturates
C. cocaine
D. methamphetamine
E. phencyclidine (PCP)

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Question: 151

(Courtesy of M Rimsza)
Narrow vermilion border and hirsutism of the forehead, as described for the
infant in the vignette.

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Critique: 151

Preferred Response: A

The infant described in the vignette has physical features that are consistent with fetal alcohol syndrome
(FAS); that is, evidence for poor somatic growth, deficient brain growth (microcephaly), and
characteristic facial dysmorphisms (short palpebral fissures and narrow upper vermilion). In early
infancy, hirsutism and tremulousness are common, but these conditions typically are self-limiting and
may no longer be evident by 6 months of age.
The offspring of women who have epilepsy and take barbiturates such as phenobarbital during
pregnancy may have a two- to threefold risk for birth defects that include cardiovascular defects, oral
clefts, and genital malformations. This risk may be greater when phenobarbital is taken with another
anticonvulsant, such as phenytoin. Although the unusual features associated with prenatal exposure to
antiepileptic drugs vary with the drug ingested, they do overlap to some degree, and the term fetal
anticonvulsant syndrome sometimes is used to describe them.
Prenatal cocaine exposure has received a great deal of attention in the medical literature, but there
may have been a bias toward reporting unfavorable outcomes. A number of early claims regarding
resultant anomalies have dissipated. Maternal use of cocaine during pregnancy is associated with an
increased risk for placental abruption and vascular disruption. The extent to which prenatal cocaine
exposure influences cognitive function remains unclear. One of the confounding variables in studying
pregnancy outcomes associated with cocaine is that the drug frequently is used concomitantly with other
substances of abuse.
There are not sufficient data on the effects of prenatal exposures to methamphetamine and
phencyclidine at this time to draw conclusions about their impact on physical and neurological
development. Some early evidence suggests that both drugs may cause neurodevelopmental
abnormalities.
As with all potential teratogens, it is important to inquire about the amount of the substance to which
the mother has been exposed, the manner in which it was delivered, the timing of exposure(s) during
the pregnancy, and associated adverse outcomes for the mother. Unfortunately, these questions are not
asked regularly. This is especially problematic when the exposed baby is raised outside of the biological
home and no one is available to answer such questions.
References:
Ackerman JP, Riggins T, Black MM. A review of the effects of prenatal cocaine exposure among
school-aged children. Pediatrics. 2010;125:554-565. DOI: 10.1542/peds.2009-0637. Available at:
http://pediatrics.aappublications.org/cgi/content/full/125/3/554?nfstatus=200&nfstatusdescription=SUCC
ESS%3A+Login+worked
Gallagher RC, Kingham K, Hoyme HE. Fetal anticonveulsant syndrome. In: Cassidy SB, Allanson JE,
eds. Management of Genetic Syndromes. 2nd ed. Hoboken, NJ: John Wiley & Sons; 2005:239-250
Hoyme HE, May PA, Kalberg WO, et al. A practical approach to diagnosis of fetal alcohol spectrum
disorders: clarification of the 1996 Institute of Medicine criteria. Pediatrics. 2005;115:39-47. DOI:
10.1542/peds.2004-0259. Available at: http://pediatrics.aappublications.org/cgi/content/full/115/1/39
Phenobarbital, Cocaine, Methamphetamine, Phencyclidine. REPROTOX. 2009.
Phenobarbital, Cocaine, Methamphetamine, Phencyclidine. TERIS. 2009.

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Question: 167
You are examining male and female twin siblings who were delivered by cesarean section several hours
ago to a 24-year-old gravida 1 woman. Both infants are normally grown and vigorous. The female twin
has torticollis, and the male has varus positioning of his feet that can be corrected passively.
Of the following, the MOST plausible explanation for the twins musculoskeletal findings is a history of
fetal

A. akinesia
B. deformation
C. disruption
D. dysplasia
E. malformation

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Critique: 167

Preferred Response: B

When evaluating the newborn who has dysmorphisms, it is critical not only to identify the abnormalities
that are present but to recognize the mechanisms by which they have occurred. Understanding
mechanisms allows appropriate management, discussion of natural history, and provision of information
on recurrence risks.
The four primary mechanisms of unusual embryonic/fetal formation are malformation, deformation,
disruption, and dysplasia. Malformations, such as cardiac defects, are due to intrinsic problems in the
developing tissue. Deformations, such as pugilistic facies, are caused by extrinsic forces acting upon an
otherwise normally developing structure. Disruptions, such as amniotic bands, are due to the destruction
of previously normally developed tissue. Dysplasias, such as hemangiomas, occur when organization of
cells into tissue is abnormal.
The twin newborns described in the vignette each have deformations related to in utero constraint.
Deformations are more common in the context of multiple births and first pregnancies or in any situation
where the fetus is crowded (eg, uterine leiomyoma, abnormal uterine structure, large fetus). In twin
pregnancies, the more inferiorly placed twin is at increased risk for torticollis related to head and neck
position. Unusual foot position also is associated with in utero crowding. Both of these conditions
resolve gradually after birth because the constraining forces no longer exist. Of note, not all torticollis
and unusual foot positioning are due to in utero constraint; both can be caused by structural defects that
do not self-correct.
Fetal akinesia, the most extreme form of decreased fetal movement, has myriad causes, including
central nervous system, spinal cord, peripheral nerve, neuromuscular, and muscular abnormalities.
Affected infants may have a variety of mechanisms of abnormal formation, depending on the primary
cause of absent in utero movement.
References:
Graham JM Jr. Clinical approach to deformation problems. In: Smiths Recognizable Patterns of Human
Deformation. 3rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:3-24
Jones KL. Morphogenesis and dysmorphogenesis. In: Smiths Recognizable Patterns of Human
Malformation. 6th ed. Philadelphia, Pa: Elsevier Saunders; 2006:783-795
Jones KL. Pena-Shokeir phenotype. In: Smiths Recognizable Patterns of Human Malformation. 6th ed.
Philadelphia, Pa: Elsevier Saunders; 2006:188-189

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Question: 210
A woman who has chronic hypertension presents for a prenatal pediatric visit at 32 weeks gestation.
She is coming in early because her obstetrician has said that she is likely to deliver prematurely. She
has a previous history of an intrauterine demise at 34 weeks gestation that was attributed to "a small
placenta." During your discussion, she asks how her baby can be monitored prenatally.
Of the following, the MOST appropriate statement regarding evaluation of her fetus is that

A. amniotic fluid volume assessment can be used to predict perinatal outcome

B. biophysical profile testing can be used to assess fetal growth
C. contraction stress testing can be used to look for evidence of uteroplacental insufficiency
D. home uterine activity monitoring can be used to decrease preterm delivery and neonatal
complications
E. nonstress testing can be used to document flow through the umbilical vessels

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Critique: 210

Preferred Response: C

The mother described in the vignette has chronic hypertension is at risk for uteroplacental insufficiency,
and may require a contraction stress test (CST) for assessment of fetal well-being. Medical conditions
associated with uterine ischemia such as chronic hypertension are associated with uteroplacental
insufficiency and intrauterine growth restriction. It has been estimated that more than 50% of
unexplained stillbirths near term may be related to restricted growth and poor placental function.
Mothers at risk should be followed closely with antenatal fetal surveillance that includes measurement of
fetal growth as well as monitoring of placental function.
Growth of the at-risk fetus is monitored by serial ultrasonography that is used to measure biparietal
diameter, head circumference, abdominal circumference, and femur length for estimation of fetal weight
and correlation to existing standards. Further assessment of fetal well-being is performed using the
nonstress test (NST). During the NST, the fetal heart rate is monitored to observe accelerations that
occur with fetal movement. The heart rate accelerates unless the fetus is acidotic or neurologically
depressed. A reactive NST includes two or more fetal heart rate accelerations within a 20-minute period.
Results of this study should be interpreted cautiously at less than 28 weeks gestation because up to
50% of the NSTs may not be reactive. The NST does not assess flow through the umbilical vessels.
If the NST is nonreactive, further evaluation is indicated. One study that may be performed is the
CST, which examines the response of the fetal heart rate to uterine contractions and can suggest
evidence of uteroplacental insufficiency. Nipple stimulation or intravenous oxytocin is used to initiate
uterine contractions if less than three spontaneous contractions occur in a 10-minute period. The test is
considered negative if there are no late or significant variable decelerations with at least three moderate
uterine contractions and positive if there are late decelerations with at least 50% of the contractions. A
positive CST suggests that the worsened oxygenation during a contraction negatively affects an already
compromised fetus and supports consideration of early delivery.
Biophysical profile testing is an additional tool that may be used to assess fetal well-being but does
not assess fetal growth. It is a score composed of the results of the NST, fetal breathing movements,
fetal body movements, fetal reflex movements, and amniotic fluid volume. Doppler assessment of
umbilical artery flow also may be used to assess placental function in uteroplacental insufficiency.
These two fetal assessment tools frequently are used in combination with the NST. Amniotic fluid
volume assessment can reflect the uterine environment, but it is not predictive of outcome.
Unfortunately, home uterine activity monitoring has not been found to decrease preterm delivery in
at-risk mothers.
References:
ACOG practice bulletin. Antepartum fetal surveillance. Number 9, October 1999. Clinical management
guidelines for obstetrician-gynecologists. Int J Gynecol Obstet. 2000;68:175-185. Abstract available at:
http://www.ncbi.nlm.nih.gov/pubmed/10717828
Devoe LD. Antenatal fetal assessment: contraction stress test, nonstress test, vibroacoustic stimulation,
amniotic fluid volume, biophysical profile, and modified biophysical profile-an overview. Semin Perinatol.
2008;32:247-252. DOI:10.1053/j.semperi.2008.04.005. Abstract available at:
http://www.ncbi.nlm.nih.gov/pubmed/18652922
Miller J, Turan S, Baschat AA. Fetal growth restriction. Semin Perinatol. 2008;32:274-280. DOI:
10.1053/j.semperi.2008.04.010. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/18652928
Turan S, Miller J, Baschat AA. Integrated testing and management in fetal growth restriction. Semin
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Perinatol. 2008;32:194-200. DOI: 10.1053/j.semperi.2008.02.008. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/18482621

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Question: 226
During the health supervision visit of a 4-month-old infant, the mother expresses concern that her
daughter does not respond to her voice. The mother is 20 years old and worked in a child care center
during her pregnancy. The infant was delivered at 38 weeks gestation, weighing 2,800 g. Otoacoustic
emission testing (OAE) was passed bilaterally on postnatal day 2. The infant has had no medical
problems since birth. After searching the Internet, the mother would like to know more about hearing
screening technologies used in infants and whether auditory brainstem response (ABR) testing would
be helpful.
Of the following, the MOST accurate information to provide this mother about hearing screening in the
newborn and infant is that

A. ABR testing does not detect sensory hearing loss

B. ABR testing does not reflect the status of the peripheral auditory system
C. otoacoustic emission (OAE) testing does not detect neural dysfunction
D. OAE testing does not miss isolated frequency region losses
E. OAE testing does not result in failed screening tests due to transient middle ear conditions

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Critique: 226

Preferred Response: C

Hearing loss affects 2 to 4 per 1,000 infants, and delays in identification of such loss beyond 6 months
of age decrease the ability to intervene in language and speech development. Selective screening of
high-risk infants only identifies 50% of those who have hearing loss. Universal hearing screening for all
infants younger than 1 month of age was endorsed by the Joint Committee on Infant Hearing in 2007,
and more than 38 states have legislation mandating these programs. The two commonly used
noninvasive methods for newborn and infant hearing screening are otoacoustic emission (OAE) testing
and auditory brainstem response (ABR) testing.
OAE testing effectively assesses the function of the peripheral nervous system, but it is unable to
screen for neural dysfunction associated with the auditory nerve and brainstem. A probe is placed in the
ear canal, a stimulus provided, and the echoes generated by the cochlea are measured using a
microphone. OAE testing can measure sensory hearing loss. This hearing screen can result in a referral
due to ambient noise, vernix in the ear canal, and structural abnormalities of the outer ear in an infant
who has normal hearing.
ABR testing uses surface electrodes to measure neural activity in the cochlea, auditory nerve, and
brainstem in response to a stimulus delivered using an earphone, allowing it to detect both sensory
hearing loss and neural dysfunction. The result is delivered as pass or fail, with further testing needed to
localize the degree and nature of the hearing loss. Unlike OAE testing, it is unaffected by ambient noise.
Both testing methods miss isolated frequency region losses.
Any infant for whom the caregiver expresses concern about hearing loss should have repeat
screening, even if the initial testing was passed, regardless of the screening test used. The mother of
the infant in this vignette is young and worked in a child care center, two risk factors for congenital
cytomegalovirus (CMV) infection in her infant. CMV is believed to be a leading cause of sensorineural
hearing loss. Of note, CMV infection occurs in 0.5% to 1% of births, with 22% of affected infants
developing sensorineural hearing loss independent of symptomatology at birth.
References:
Foulon I, Naessens A, Foulon W, Casteels A, Gordts F. A 10-year prospective study of sensorineural
hearing loss in children with congenital cytomegalovirus infection. J Pediatr. 2008;153:84-88. DOI:
10.1016/j.jpeds.2007.12.049. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/1857154
Joint Committee on Infant Hearing. Year 2007 position statement: principles and guidelines for early
hearing detection and intervention programs. Pediatrics. 2007;120: 898-921. DOI:
10.1542/peds.2007-2333. Available at: http://pediatrics.aappublications.org/cgi/content/full/120/4/898
Wrightson AS. Universal newborn hearing screening. Am Fam Physician. 2007;75:1349-1352. Abstract
available at: http://www.ncbi.nlm.nih.gov/pubmed/17508530

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Question: 242
You are called to attend the cesarean section delivery of a 42 weeks gestation infant because of failure
to progress complicated by severe oligohydramnios. Results of maternal screens are unremarkable,
including a negative group B streptococcal test. Rupture of the membranes at the time of delivery
reveals scant fluid that is meconium stained. Upon assessment on the warmer, the infant appears to be
vigorous and has good respiratory effort, a heart rate of greater than 100 beats/min, central cyanosis,
and meconium staining of peeling "post dates" skin. You begin blow-by oxygen, which improves his
color, but he develops tachypnea and grunting 5 minutes after birth. He is transferred to the special care
nursery, where he is intubated endotracheally and an umbilical venous line is placed. Pre- and
postductal saturations are 97% while receiving 60% oxygen on the ventilator. His chest radiograph is
seen in (Item Q242).
Of the following, the infants clinical presentation is MOST consistent with

A. group B streptococcal pneumonia

B. meconium aspiration syndrome
C. persistent pulmonary hypertension
D. retained fetal lung liquid syndrome
E. transposition of the great vessels

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Question: 242

(Courtesy of S Izatt)
Radiographic findings, as exhibited by the infant in the vignette.

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Critique: 242

Preferred Response: B

The history of post dates pregnancy with severe oligohydramnios, the clinical findings of meconium
staining at birth and early-onset respiratory distress, and the chest radiograph demonstrating patchy
opacification and hyperinflation (Item C242A) make the presentation of the infant described in the
vignette most consistent with meconium aspiration syndrome (MAS).
The passage of meconium into amniotic fluid prior to delivery occurs in approximately 13% of live
births and is seen more commonly in post dates infants. Normally, the flow of fetal lung fluid is up and
out of the lungs with in utero breathing. Aspiration of amniotic fluid occurs with gasping inspiratory
breaths that are associated with hypoxia or ischemia, as might be seen with uteroplacental insufficiency.
This suggests that meconium aspiration happens before delivery in response to stress.
MAS causes lung injury through several mechanisms, including chemical pneumonitis due to
inflammation, mechanical obstruction of the airways, inactivation of surfactant, and vasoconstriction of
the pulmonary vessels. Persistent pulmonary hypertension (PPHN) often is seen in association with
MAS.
The infant who has MAS may appear meconium-stained at delivery and have a barrel chest due to
air trapping. Respiratory distress develops immediately or shortly after birth, with grunting, flaring, and
retractions as well as cyanosis. Auscultation of the lung fields may demonstrate rales and rhonchi. The
chest radiograph classically demonstrates patchy opacification and hyperinflation, with complete
opacification seen in extreme cases. A pneumothorax or pneumomediastinum may be seen due to the
increased risk of air leak from the ball-valve effect of meconium.
Congenital cyanotic heart disease (eg, transposition of the great vessels) is less likely due to this
infants response to oxygen, although a hyperoxia test to document a Pao2 measurement greater than
150 mm Hg would help to exclude cyanotic congenital heart disease. Although the infant is at risk for
PPHN, the similar pre- and postductal oxygen saturations do not demonstrate shunting at the ductal
level. The chest radiograph does not demonstrate diffuse parenchymal infiltrates or fluid in the fissures
(Item C242B), which would suggest retained fetal lung fluid syndrome (previously known as transient
tachypnea of the newborn). Underlying group B streptococcal pneumonia cannot be excluded
definitively, but the chest radiograph in this condition may demonstrate patchy infiltrates and small
pleural effusions.
References:
Carbine DN, Serwint JR. In brief: meconium aspiration. Pediatr Rev. 2008;29:212-213. DOI:
10.1542/10.1542/pir.29-6-212. Available at:
http://pedsinreview.aappublications.org/cgi/content/full/29/6/212
Dargaville PA, Copnell B for the Australian and New Zealand Neonatal Network. The epidemiology of
meconium aspiration syndrome: incidence, risk factors, therapies, and outcome. Pediatrics.
2006;117:1712-1721. DOI: 10.1542/peds.2005-2215. Available at:
http://pediatrics.aappublications.org/cgi/content/full/117/5/1712
Flidel-Rimon O, Shinwell ES. Respiratory distress in the term and near-term infant. NeoReviews.
2005;6:e289-e297. Available at: http://neoreviews.aappublications.org/cgi/content/full/6/6/e289
Steinhorn RH, Farrow KN. Pulmonary hypertension in the neonate. NeoReviews. 2007;8:e14-e21.
Available at: http://neoreviews.aappublications.org/cgi/content/full/8/1/e14

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Critique: 242

(Courtesy of S Izatt)
Meconium aspiration: Diffuse patchy opacities, small bilateral pleural
effusions, and mild hyperinflation.

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Critique: 242

(Reprinted with permission from Aly H. Respiratory disorders in the newborn:

identification and diagnosis. Pediatr Rev. 2004;25:201-208)
Chest radiograph in retained fetal lung fluid syndrome: There are increased
pulmonary interstitial markings and fluid in the interlobar fissures (arrows).

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