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Understanding Congenital Syphilis

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 Infants & Young Children
Vol. 31, No. 4, pp. 287–296
Copyright C 2018 Wolters Kluwer Health, Inc. All rights reserved.

Understanding Congenital
Syphilis
Sallie Porter, DNP, PhD, APN; Rubab Qureshi, MD, PhD;
Downloaded from http://journals.lww.com/iycjournal by VNq14+bl+ueiX7tRBlTmYXoG5U20PZ9McV/1q+BVOKnFTm8uFFwII3FloJS/b7XMuwZP7+RZ3O1kW5M6fl1Jmyjm7e1rNUCjjH3m2vOE4IZH3qpvGsp1w/a/5PC/z9VA on 08/31/2018

Irina Benenson, DNP, FNP-C CEN

The incidence of infants with congenital syphilis (CS) has been accelerating in the United States
and remains an issue of global concern. Infants with CS often experience poor birth, health,
and developmental outcomes. These poor outcomes (e.g., prematurity, bone changes, neurode-
velopmental impairment) may be exacerbated by social vulnerabilities (e.g., housing instability,
incarceration) experienced by their mothers and families. As such, infants with CS may benefit
from neurodevelopmental assessments offered early in life, comprehensive in scope, and repeated
over time; developmental intervention, as well as family support services that acknowledge the
co-occurring health, developmental, and social challenges they may face. Key words: congenital
infection, congenital syphilis, early intervention, infants, syphilis

A N INFANT with congenital syphilis (CS)

is at increased risk for neurodevelop-
mental impairment. This increased risk is mag-
nified by co-occurring family and social chal-
lenges that together may lead to poor health
and developmental outcomes for the infant.
Infants and young children with CS or with a
history of treated CS may be enrolled in early
intervention programs, making it important
for professionals to understand the science
behind and treatment of the condition as well
as the implications for developmental moni-
toring, family support, and advocacy.
Author Affiliations: Division of Advanced Nursing
Practice (Drs Porter and Benenson) and Division of
Nursing Science (Dr Qureshi), Rutgers School of
Nursing, Newark, New Jersey; and New Jersey
Leadership Education, Neurodevelopmental and
Related Disabilities Program, Robert Wood Johnson
Medical School Boggs Center (Dr Porter), Rutgers
Center for Gender, Sexuality, Law, and Policy
(Dr Qureshi), and Robert Wood Johnson University
Hospital (Dr Benenson), New Brunswick, New Jersey.
The authors thank Margaret P. Disston for her editing
assistance.
The authors report no conflicts of interest.
Correspondence: Sallie Porter, DNP, PhD, APN, Rut-
gers School of Nursing, Division of Advanced Nursing
Practice, 65 Bergen St, Newark, NJ 07101 (portersa@
sn.rutgers.edu).
DOI: 10.1097/IYC.0000000000000125
Globally, almost 1 million pregnant women
are infected with syphilis annually (World
Health Organization, 2017). By one estimate,
annually, CS led to143,000 perinatal deaths
and stillbirths, 62,000 neonatal deaths, 44,000
preterm births or low–birth-weight births,
and 102,000 infected infants worldwide
(Wijesooriya et al., 2016). With 64% of the
adverse outcomes listed, Africa was dispro-
portionately affected with the highest bur-
den (Wijesooriya et al., 2016). More recently,
the rates of congenital infection have been
on the decline (a decrease of 39% between
2008 and 2012) with the greatest decline in
Southeast Asia, particularly India (Wijesooriya
et al., 2016). However, the data did not in-
clude the United States (Wijesooriya et al.,
2016). In 2015, Cuba was verified free
of mother to fetus/infant transmission of
syphilis, thus serving as a potential model
for other countries to accomplish the same
(World Health Organization, 2015).
In 2016, the rate of CS was 16 per 100,000
live births in the United States accelerating
from a rate of 11.6 cases per 100,000 live
births (Bowen, Su, Torrone, Kidd, & Wein-
stock, 2015). The overall national rate of CS
increased by 38% from 2012 to 2014 (Bowen
et al., 2015) and then by another 28% among
newborns from 2015 to 2016 (Centers for

287

Copyright © 2018 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
 288 INFANTS & YOUNG CHILDREN/OCTOBER–DECEMBER 2018
Disease Control and Prevention [CDC],
2017b). There were 461 infants with CS in
2014, 492 in 2015, and 628 in 2016. Prior to
2012–2014, syphilis rates had been trending
downward in the United States for 6 years.
Less condom use, less awareness among
health care professionals and patients, and
less availability of clinics devoted to treat-
ment for sexually transmitted diseases are con-
tributing to the rising rates of syphilis (Karla-
mangla, 2017). While late or limited prenatal
care is a contributing factor in the increase
in CS, the failure of health care profession-
als to adhere to recommended screening and
treatment guidelines is also a problem (CDC,
2017a).
All racial/ethnic groups showed a rise in
CS rates in 2012–2014 and then again in
2016 (Bowen et al., 2015; CDC, 2017a). Like
many conditions that manifest in infancy and
young childhood, health disparities exist. In-
fants with Black mothers are the “majority of
CS cases” (Bowen et al., 2015, p. 1241). Per
the CDC, Black, non-Hispanic mothers had the
highest rates of infants with CS—38.2 cases
per 100,000 live births in 2014 (Bowen et al.,
2015). The CS rate among Black, non-Hispanic
mothers rose to 43.1 per 100,000 live births
in 2016 (CDC, 2017a).
American Indian/Alaska Native mothers
have the next highest rate at 12.8 cases of
infants with CS per 100,000 live births in
2014, rising to 31.6 per 100,000 live births in
2016 (Bowen et al., 2015; CDC, 2017a). The
rate for Hispanic mothers is 7.0 cases of infant
with CS per 100,000 births in 2014 rising
to 20.5 cases of CS per 100,000 live births
in 2016 (Bowen et al., 2015; CDC, 2017a).
Asian mothers had infants with CS at a rate
of 9.2 per 100,000 live births in 2016 (CDC,
2017a). White, non-Hispanic mothers have
the lowest rate: 3.7 cases of infants with CS
per 100,000 births in 2014 but also showed
an increased rate in 2016 of 5.3% of CS per
100,000 live births (Bowen et al., 2015; CDC,
2017a). The doubling and even near tripling
of CS rates among American Indian/Alaska
Native infants and Hispanic infants are
alarming.
Copyright © 2018 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
There are geographic disparities in the
United States as well, with the highest rates of
CS in 2014 found in the South region (15.5),
followed by the West region at 12.8 (Bowen
et al., 2015). CS rates have “more than dou-
bled” in the West region (Bowen et al., 2015,
p. 1242) and rose again in 2016 to a rate of
25.6 (CDC, 2017a). In the South region, the
rate for 2016 increased to 17.8 (CDC, 2017a).
Women living in an area with a high preva-
lence of syphilis may be more likely to be ex-
posed to the disease. Early intervention pro-
fessionals may find it useful to research the
prevalence of syphilis in their locale and dis-
cuss the issue with their program’s consult-
ing pediatrician or pediatric advanced prac-
tice nurse.
PATHOPHYSIOLOGY
Congenital syphilis is caused by the organ-
ism Treponema pallidum and occurs when
an infected pregnant woman passes the in-
fection to her fetus in the womb or to the
neonate during birth. T. pallidum is a gram-
negative bacteria spirochete with a helical
shape and corkscrew motility (Porth & Matfin,
2009). The congenital effects of CS are irre-
versible, but with adequate treatment, further
damage can be prevented. Unfortunately, CS
is underdiagnosed often related to a lack of
prenatal care and missed screening opportu-
nities and sometimes inadequately treated or
the mother is reinfected (Heston & Arnold,
2018; Pessoa & Galvao, 2011). In CS, mater-
nal to fetal/infant transmission occurs when
the T. pallidum bacteria crosses the placenta,
or the birthing infant is directly exposed to a
syphilitic chancre. A chancre is an open sore
that leaks T. pallidum bacteria.
Acquired syphilis is classified by stage:
primary, secondary, latent, and tertiary. Each
stage of acquired syphilis has different signs
and symptoms. CS infection occurs frequently
during maternal primary syphilis infection
or maternal secondary syphilis infection
when the spirochetes are most numerous
(Kumar, Abbas, Fausto, & Aster, 2014). The
rate of transmission to infants is 60%–100% in

mothers infected with primary and secondary
syphilis (Jackson, Long, Kimberlin, & Brady,
2015). In 25%–40% of untreated cases of
maternal syphilis, spontaneous abortion, in-
trauterine, or perinatal death occurs (Jackson
et al., 2015; Kumar et al., 2014).
Although placental transmission of T. pal-
lidum can occur at any time during gesta-
tion, the risk of transmission increases during
the second and third trimesters of pregnancy
(Heston & Arnold, 2018; Mandell, Douglas,
& Bennett, 2015). The transmission is great-
est in cases of untreated maternal infection of
recent duration with an increased likelihood
of infection in primary or secondary maternal
syphilis (Mandell et al., 2015). At the onset of
CS, T. pallidum is released directly into the
fetal bloodstream, leading to the widespread
dissemination of the microorganism to almost
all organs.
The clinical manifestations result from
the body’s inflammatory response to T. pal-
lidum (Cohen, Powderly & Opal, 2016). The
pathogenesis of the endarteritis is unknown
(Kumar et al., 2014). There is an intense in-
flammatory response with infiltrates rich in
T cells, plasma cells and macrophages, and
production of Treponeme specific antibodies
(Kumar et al., 2014). Despite the activation
of these defense mechanisms, the infection
persists mostly because of antigenic diversity
(Kumar et al., 2014). The bones, liver, pan-
creas, intestine, kidney, and spleen are the
most frequently affected (Cohen et al., 2016).
IDENTIFICATION AND TREATMENT
OF PREGNANT WOMEN WITH SYPHILIS
Most poor pregnancy outcomes caused by
in utero syphilis infection can be avoided with
early screening and treatment. Despite profes-
sional association guidelines and legal man-
dates, not all cases of syphilis exposure in
utero are identified during the early prenatal
period although overall screening for syphilis
in pregnant women is nearly universal, screen-
ing in early pregnancy is less so (Koumans et
al., 2012; Neblett Fanfair, Tao, Owusu-Edusei,
Gift, & Bernstein, 2017; Ross, Tao, Patton,
& Hoover, 2015). In addition, not all preg-
Copyright © 2018 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Understanding Congenital Syphilis 289
nant women receive prenatal care and some
women may only first present for natal care at
the time of delivery. Per the Kids Count data
center (2018), 6% of births to women receive
late or no prenatal care.
Approximately 20% of pregnant women are
not tested for syphilis prior to hospital admis-
sion for delivery and some pregnant women
acquire the syphilis infection after testing
in early pregnancy was completed (Bowen
et al., 2015; Koumans et al., 2012). In addi-
tion, not all pregnant women with syphilis
receive adequate treatment or any treatment,
at all. Therefore, the lack of a positive result is
essentially meaningless without confirmation
of adequate testing.
Ideally, all pregnant women are tested for
syphilis early in their pregnancy. The U.S.
Preventative Services Task Force (USPSTF)
recently circulated a draft recommendation
statement reaffirming support of universal
screening for syphilis infection in all pregnant
women (USPSTF, 2018). The USPSTF recom-
mendation is in congruence with guidance
from the CDC, the American Academy of Pe-
diatrics, and the American College of Obstet-
rics and Gynecology (AAP Committee on Fe-
tus and Newborn and ACOG Committee on
Obstetric Practice, 2017; Lin, Eder, & Bean,
2018); however, per Koumans et al. (2012),
only 80% of pregnant women are screened
prior to hospital admission for delivery. Forty-
six states mandate screening for syphilis in
pregnant women, but most require only one
test and almost all specify that the test be
done at the first visit or early in the current
pregnancy (Hollier, Hill, Sheffield, & Wendel,
2003). Third-trimester screening for syphilis
is less mandated with only nine states requir-
ing it in all pregnant women and another
three states requiring it for high-risk pregnant
women only. This “lesser” mandate may leave
already “at risk for syphilis” pregnant women
and their unborn children at additional risk.
For pregnant women who are at higher
risk for syphilis due to personal or geo-
graphic factors, repeat testing for syphilis is
recommended early in the third trimester at
approximately 28 weeks’ gestation and then
again, after delivery to detect new recent
 290 INFANTS & YOUNG CHILDREN/OCTOBER–DECEMBER 2018
infections (Jackson et al., 2015). Pregnant
women with no or inadequate health insur-
ance, substance use issues, who are transient,
who live in rural areas, have been or are
incarcerated, have multiple or concurrent
partners, have a history of previous preg-
nancy loss, and/or have a history of syphilis
infection are at higher risk for delivering an
infant with CS (Bowen et al., 2015; Heston
& Arnold, 2018; USPSTF, 2018). With the
alarming rise in opiate abuse throughout the
United States, more pregnant women may be
at higher risk for delivery of an infant with
CS than in previous decades as women who
are/have been incarcerated and those who
exchange sex for drugs are considered at
increased risk. Biswas et al. (2018) reported
risk behavior characteristics of mothers of
infants with CS and mothers of infants with
syphilis exposure in utero that included “sex
while intoxicated or high (29%),” “metham-
phetamine use (21%),” and “incarceration in
the last 12 months (13%)” (p. 10). To prevent
CS, pregnant women with syphilis are treated
with benzathine penicillin G. Treatment of
sexual partners should be ensured, as well
(McCancre, Huether, Brashers, & Rote, 2010).
CHARACTERISTICS OF CS INFECTION
Congenital syphilis is divided into early CS
or late CS (Kwak & Lamprecht, 2015). The
severity of the illness is variable and can range
from a normal-appearing infant with minimal
abnormalities on laboratory tests to a life-
threatening involvement of multiple organs
(Cohen et al., 2016). The signs and symptoms
of CS infection vary with the length of time
the infection has been present and untreated
in the infant and young child. Early CS is iden-
tified in young children under 2 years of age.
Earliest identification or better yet, prevention
of CS, though optimal treatment of syphilis
exposure in utero, is key for improved infant
outcomes. Prevention for the purposes of this
article does not refer to the absolute preven-
tion of syphilis in a woman, but rather the
prevention of the progression of fetal syphilis
exposure in utero to CS in the infant. Labo-
Copyright © 2018 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
ratory verification is required to confirm CS
(CDC, 2015a).
Early congenital syphilis infection
There is a continuum of severity in CS
with approximately 60% of infants show-
ing no symptoms at birth and other in-
fants more severely affected (CDC, 2015b).
Most newborn infants show no indications
of CS by clinical examination (Su et al.,
2016). Gomez et al. (2013) reported signs
and symptoms of syphilis in 15% of infants
with untreated mothers. The most common
signs and symptoms of CS include skin le-
sions, jaundice, and enlarged liver and spleen
(Bowen et al., 2015). The rash may involve
the palms and soles and will often progress
from flat to bumpy to peeling (Butterfield,
2014; Tsimis & Sheffield, 2017). Jaundice is
a yellowness or sallowness of the skin and
sclera. Per Kingston et al. (2015), 40%–60%
of infants with CS will have one of the
following signs or symptoms: hemorrhagic
rhinitis, hepatosplenomegaly, lymphaden-
opathy, rash, and skeletal abnormalities.
Other findings include bone deformities
noted via X-ray and cerebrospinal fluid (CSF)
abnormalities identified via spinal tap results
(Bowen et al., 2015). Congenital syphilis can
end in stillbirth or neonatal death (Bowen
et al., 2015). Liveborn infants born with CS
are more often born low-birth-weight or pre-
mature than noninfected newborns (Gust,
Levine, St Louis, Braxton, & Berman, 2002).
Symptoms may be vague and include poor
feeding, a runny nose, and rash that are com-
mon in other conditions as well, thus mak-
ing CS challenging to diagnose (Benza &
Stankovic, 2015). Thick nasal discharge often
referred to as snuffles may make breathing
and feeding difficult for the infant and is of-
ten the first noted symptom (Kwak & Lam-
precht, 2015). For infants with CS not initially
presenting with any symptoms, most will be-
gin exhibiting symptoms at approximately
1–2 months of age (Benza & Stankovic, 2015;
Kwak & Lamprecht, 2015; Meissner, 2016;
Nissanka-Jayasuriya, Odell, & Phillips, 2016;
Table 1).

Table 1. Congenital Syphilis in Infants
Under 2 Years of Age: Most Noted Physical
Assessment Findings
Skin lesions (especially palms, soles, mouth,
anus)
Jaundice
Enlarged liver and spleen
“Snuffles”/rhinitis/runny nose
Large or swollen lymph nodes
Long bone changes
Edema/swelling
Decreased movement of limb
Note. From Bowen et al. (2015); Kingston et al. (2015);
and Meissner (2016).
If testing of the mother reveals syphilis,
physical evaluation of the newborn must
includes examination for edema/swelling
from nonimmune hydrops, jaundice, hep-
atosplenomegaly, rhinitis, skin rash, osteitis,
and lack of limb motion likely due to pain
(Meissner, 2016). Nonimmune hydrops oc-
cur when syphilis-related severe anemia af-
fects the body’s ability to manage fluid (Med-
line Plus, 2017). Hepatosplenomegaly is an
enlargement of the liver and spleen. Osteitis
is inflammation of the bone.
The infection may manifest as infantile
syphilis, occurring in the first 2 years of life, or
later as tardive syphilis (Kumar et al., 2014).
The most common symptoms include copious
nasal discharge and congestion. A desquamat-
ing, bullous, rash on the hands and feet or
around the mouth or anus leads to slough-
ing skin. These skin lesions and nasal dis-
charge are highly contagious (Jackson et al.,
2015). Skeletal abnormalities, osteochondritis
and periostitis, may occur.
The prevention of mother-to-infant trans-
mission of syphilis is the ideal with the effi-
cacy of benzathine penicillin G approximately
98% effective for preventing CS (Alexan-
der, Sheffield, Sanchez, Mayfield, & Wendel,
1999). Newborns with CS are treated with
penicillin G. The CDC offers detailed guid-
ance of the treatment and follow-up of infants
with CS (CDC, 2015b). It is essential to use
Copyright © 2018 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Understanding Congenital Syphilis 291
the most up-to-date testing and treatment au-
thoritative guidelines. Hospitals are advised
not to discharge newborns to home without
first ensuring that the mother has had a nega-
tive syphilis test during the current pregnancy
(Meissner, 2016). Noting that for women who
become infected in very late pregnancy, they
may exhibit initial negative syphilis screening
results for both mother and infant (Bembry,
Anderson, & Nelson, 2017).
Late congenital infection syphilis
infection
In late CS, which is initially identified two
or more years after birth, children may ex-
hibit dental defects in the permanent teeth
(i.e., Hutchinson teeth—peg shaped, notched
central incisors), interstitial keratitis that may
lead to glaucoma or corneal scarring, and high-
frequency hearing lost due to damage to the
eighth cranial nerve (Jackson et al., 2015; Pes-
soa & Galvao, 2011). Keratitis is inflammation
of the cornea. Untreated CS may result in in-
tellectual disability and seizures (Butterfield,
2014; CDC, 2017a). Treatment of late CS is
essential, but it will not reverse any problems
already noted. Skeletal lesions cause destruc-
tion of the vomer, collapse the bridge of the
nose and lead to the characteristic saddle nose
deformity (Kumar et al., 2014). Anterior bow-
ing of the tibia results from periostitis and ex-
cessive new bone growth. The eighth nerve
deafness can occur between 10 and 40 years
of age, whereas interstitial keratitis can man-
ifest between 5 and 20 years of age (Jackson
et al., 2015). Hepatosplenomegaly, with gum-
mas, diffuse fibrosis, vascular changes, and
lymphoplasmacytic infiltrates, is also com-
mon (Kumar et al., 2014).
TESTING
The screening and testing regime for diag-
nosis and follow-up of CS can be complex as
there are different forms of testing that may
be inconclusive or show false-positive results.
Some tests do not distinguish between older
and more recent infections. Treponemal test
results may remain reactive for life in most
persons with a prior history of syphilis, even
 292 INFANTS & YOUNG CHILDREN/OCTOBER–DECEMBER 2018
with prior adequate treatment. Certain tests
are useful for screening, while others are use-
ful to ascertain a presumptive diagnosis (Jack-
son et al., 2015). There are many reasons for
false-positive serologic tests for syphilis (Mc-
Cancre et al., 2010). Use of the current CDC
guidelines along with input from a pediatric
infectious disease specialist is suggested. Per
Jieun Kwak and pediatric infectious disease
specialist Catherine Lamprecht, evaluation of
the infant who has concerning clinical signs
or whose mother has concerning laboratory
results should include physical examination
and laboratory testing (Kwak & Lamprecht,
2015). The diagnostic workup may also in-
clude liver function tests, long-bone and chest
X-rays, and ophthalmologic examination. Ide-
ally, the prevention of CS is the desired out-
come, but when that is not achieved, careful
testing, treatment, and follow-up will ensure
adequate treatment.
TREATMENT AND FOLLOW-UP
Once CS is diagnosed and treatment ini-
tiated, using guidance from the CDC, there
needs to be close follow-up for approximately
3–6 months or longer if laboratory test results
do not indicate treatment success. The treat-
ment of CS is intravenous aqueous crystalline
penicillin G for 14 days (Jackson et al., 2015).
Consultation with a pediatric infectious dis-
ease specialist is useful in interpretation of
often complex testing requirements, results,
and follow-up (Butterfield, 2014). Beyond hav-
ing a foundational understanding of the sci-
ence behind CS, early intervention profession-
als will consider the following aspects of in-
fant health and development.
Pediatric health care
Per the American Academy of Pediatrics,
infants with CS should have careful physical
examination, parent education, and close
follow-up every 2–3 months over the first
12 months of life and until the titers are
nonreactive (Heston & Arnold, 2018; Jackson
et al., 2015). Health supervision visits, prefer-
ably with the input of a pediatric infectious
disease specialist, are beneficial in interpreta-
tion of often complex testing requirements,
Copyright © 2018 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
results, and follow-up (Butterfield, 2014).
Testing for other sexually transmitted dis-
eases is indicated. Cerebrospinal fluid testing
should be considered and may need to
be repeated every 6 months until the CSF
laboratory results are normal (Jackson et al.,
2015). Neurosyphilis may occur at any stage
of infection in infants with CS (Jackson
et al., 2015). Bone X-rays should be consid-
ered. Screening and treatment of parents and
their partners for syphilis and other sexually
transmitted infections should be ensured
(Heston & Arnold, 2018; Jackson et al., 2015).
Congenital syphilis is a challenging
condition to diagnose. Early intervention
professionals may observe findings that are
consistent with CS and require additional
investigation. In these instances, the early
intervention professionals may need to
approach the family and pediatric primary
care clinician about their concerns.
Breastfeeding and nutrition
T. pallidum is not transferred via breast
milk, but transmission may occur if the
mother has an infectious lesion (chancre)
on her breast (Mandell et al., 2015). Per the
Office of Women’s Health, U.S. Department
of Health and Human Services (2017), if a
woman has syphilis, she can breastfeed if her
infant or the breast pumping equipment does
not touch an open chancre sore. Mothers
should be directed to discuss any breastfeed-
ing concerns with their infant’s pediatric
clinician. Support from a lactation counselor
may also be valuable. Infants with CS tend to
be smaller than their non-CS peers, as such,
nutritional counseling may be warranted as
well (Lago, Viccari, & Fiori, 2013).
Developmental surveillance,
developmental screening, and
neurodevelopmental assessment
Generally, the American Academy of Pedi-
atrics recommends the first formal develop-
mental screening using a validated tool occur
at the 9-month well child health supervision
visit (Council on Children with Disabilities,
2006). Developmental surveillance is recom-
mended at all well child health supervision

visits (Hagan, Shaw, & Duncan, 2017). Re-
cent research supports earlier, more detailed,
and extended developmental monitoring of
children whose mothers had syphilis during
pregnancy (Verghese et al., 2018).
Both infants with CS and infants exposed
to syphilis in utero whose mothers have a
reactive syphilis serology in pregnancy are
at increased risk for neurodevelopmental
impairment. Verghese et al. (2018) found that
infants with CS (n = 11) had a 27.3% occur-
rence of any neurodevelopmental impairment
and those with syphilis exposure in utero
(n = 7) had 14.3% occurrence of neurodevel-
opmental impairment. Neurodevelopmental
impairment includes cerebral palsy, visual im-
pairment, sensorineural hearing loss, mental
delay, and seizure disorder. Neurodevelop-
mental assessment should be offered as well
as developmental intervention and support
for both infants with CS and infants exposed
to syphilis in utero whose mothers have a
reactive syphilis serology in pregnancy.
Information about developmental out-
comes in infants treated for CS remains
limited. Lago et al. (2013) reported on a
subsample of 120 young children with CS,
14 young children or 8.57% evidenced devel-
opmental delay between 8 and 60 months of
age per the Denver Developmental Screening
Test 2. However, early intervention profes-
sionals need to consider not just an infant’s
“medical” vulnerability, but socioeconomic
concerns, and other social vulnerabilities
when determining overall risk and need for
early childhood developmental services.
Language development assessment
Speech–language delays were reported in
36.4% of infants with CS and 42.9% for infants
with syphilis exposure in utero (Verghese
et al., 2018). Therefore, a speech–language
evaluation is especially important for this
group of children. Ongoing hearing screening
is recommended for children with CS as it
is a known cause of sensorineural hearing
loss. However, the incidence evidence is
limited with more longitudinal audiometric
data needed (Chau, Atashband, Chang,
Westerberg, & Kozak, 2009).
Copyright © 2018 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Understanding Congenital Syphilis 293
Vision development assessment
Ocular inflammatory signs of CS may be
present at birth and include chorioretinitis,
interstitial keratitis, glaucoma, and congenital
cataract (Hariprasad, Moon, & Allen, 2002).
Most frequent is a multifocal chorioretinitis
with inflammation of the posterior uvea and
retina (American Academy of Ophthalmology
[AAO], 2018). The diagnosis is suspected by a
bilateral “salt-and-pepper” appearance of the
fundus that affects peripheral retina (AAO,
2018). The finding of multifocal chorioretini-
tis usually does not progress and the infant
may have normal vision (AAO, 2018). How-
ever, ongoing vision evaluation is important.
Motor development assessment
One of the skeletal abnormalities of CS
is Parrott’s pseudoparalysis. Local periostitis
may manifest as pseudoparalysis of Parrot
is an intensely painful condition character-
ized by decreased movement primarily affect-
ing the upper limbs, but arms or legs may
be involved as well. It affects the metaph-
ysis of long bones and can be mistaken for
birth-related trauma or other neonatal skeletal
anomalies (Patel, Oussedik, Landis, & Strowd,
2018; Pereira, Castro, Venturini, Cesar, Fortes,
& Costa, 2017). Bone findings are very impor-
tant as pseudoparalysis of Parrot may be the
initial finding in infants with unidentified CS.
(Dobson & Sanchez, 2014).
STAFF AND CAREGIVER HEALTH
AND SAFETY
Contact precautions are indicated when
handling all bodily fluids of children par-
ticipating in early childhood development
services. Moist open lesions and secretions
in an untreated infant with CS are infectious
(Bembry et al., 2017; Jackson et al., 2015).
Any person who has had close contact with
an infant with early CS prior to suspected
diagnosis or within the first 24 hr of antibiotic
treatment should receive evaluation for le-
sions 2–3 weeks after contact. Testing in the
presence of syphilis signs and symptoms for
such exposed persons is required as it is a sub-
sequent follow-up in 3 months. Prophylactic
 294 INFANTS & YOUNG CHILDREN/OCTOBER–DECEMBER 2018
treatment should be considered if exposure to
the individual was substantial (Jackson et al.,
2015).
FAMILY SUPPORT AND ADVOCACY
As with all families, early intervention pro-
fessionals will want to consider social deter-
minants of health when providing family sup-
port. Poverty is a well-known risk factor for
poorer health and development. More than
one third of mothers of infants with CS report
homelessness or housing instability (Diorio,
Kroeger, & Ross, 2018). More than one third
of mothers of infants with CS report a his-
tory of or current incarceration of themselves,
their male sex partner, or both. Screening for
parental economic instability, housing insta-
bility, and unemployment should be consid-
ered. Referrals for basic supports should be
offered, including economic assistance, nutri-
tion programs, housing vouchers, and diaper
banks (Porter & Steefel, 2015; Qureshi, Porter,
& Zha, 2017).
Housing instability and parental incarcera-
tion place a young child at additional risk for
poor health and developmental outcomes.
Concrete resources (e.g., homelessness
prevention and prisoner reentry programs)
to ameliorate these issues should be shared
with mothers and other family members.
Addressing housing instability and poor
housing conditions helps ensure continuous
and consistent attendance at early child edu-
cation programs, including Early Head Start,
Head Start, and preschool. Homelessness, fre-
quent moves, and relocation due to housing
conditions negatively affect early education
program attendance. Initiatives to encourage
better health care and developmental care
access and attendance such as transporta-
tion vouchers and incentives should be
considered.
Professionals will want to consider screen-
ing for parental reading literacy and health
literacy. Addressing literacy deficits will help
ensure that parental uptake of developmental
and parenting content is optimal. Behavioral
supports, too, may help parents improve
Copyright © 2018 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
implementation of developmental interven-
tions. All family members should receive
proactive assistance with their ongoing phys-
ical and emotional health concerns including
substance misuse, violence in the home or
community, history of adverse childhood
experiences, and low educational attainment.
In addition, advocacy to attain home nursing
visits and other home visitation services may
prove beneficial to the infant and family.
Subject to local regulations, children with
CS may not be eligible for early interven-
tion services until a “significant” delay is
documented in one or more domains. For
infants with CS, this may be especially in-
equitable considering the co-occurring social
factors that may place them at greater risk
for poor developmental outcomes as well
as the tendency for developmental perfor-
mance to worsen over time (Verghese et al.,
2018). Early intervention professionals will
want to advocate vigorously for expanded el-
igibility for infants who are at both medical
and social risks and their families to partici-
pate in quality early childhood developmental
programs.
CONCLUSION
Infants with CS may experience poor
birth, health, and developmental outcomes.
These poor outcomes (e.g., prematurity, bone
changes, neurodevelopmental impairment)
may be exacerbated by social vulnerabili-
ties (e.g., housing instability, incarceration)
experienced by their mothers and families.
As such, infants with CS may benefit from
early, comprehensive, and extended neurode-
velopmental assessments and intervention as
well as family support services that acknowl-
edge the co-occurring health, developmental,
and social challenges they may face. Infants
with CS will require excellent family-centered
pediatric health care, developmental care, and
early childhood education care to ensure that
they enter kindergarten ready to learn, thus,
potentially mitigating loss of developmen-
tal capital and long-term adverse educational
outcomes.

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