Congenital Syphilis
Congenital Syphilis
Congenital Syphilis
ABSTRACT
Background: Syphilis is caused by the spirochete bacterium Treponema pallidum. Syphilis left untreated, or inade-
quately treated during pregnancy, can result in congenital syphilis (CS). Congenital syphilis can lead to severe sequelae
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yphilis is a sexually transmitted bacterial Ethnic disparities, low socioeconomic status,
infection. If detected early, syphilis is consid- unsafe sexual practices, inadequate treatment during
ered both treatable and curable, with little pregnancy, and partial or no prenatal care due to
risk for comorbidities. Despite proactive efforts by limited access to medical care in certain North
the World Health Organization (WHO) (2007- American regions are positively associated with an
2012) to curb the spread of syphilis, cases of primary increased risk for syphilis infection during preg-
and secondary syphilis continue to trend upward.1-3 nancy and subsequent CS.2,5,6 Furthermore, the
More specifically, rates of primary and secondary stigma and discrimination associated with sexually
syphilis infections rose from 0.9 to 1.9 cases per transmitted infections often deter at-risk females
100,000 females (2012-2016), with rates of syphilis from seeking appropriate prenatal care.2,5,6 Inconsis-
highest among females living within the Western and tent maternal syphilis screening during pregnancy
Southern regions of North American.4 Similarly, contributes to missed diagnostic and curative oppor-
rates of congenital syphilis (CS) infection rose from tunities, fetal infection, and resultant mortality and
8.4 to 15.7 cases per 100,000 live births from 2012 morbidity risks.2,5,6
to 2016, an 86.9% increase.4 Congenital syphilis impacts both perinatal and
neonatal care. Congenital syphilis can be acquired
transplacentally, as early as the 14th week of fetal
Author Affiliation: East Carolina University, Greensboro, North development, or by direct skin-to-skin contact with
Carolina. a vaginal syphilitic lesion during delivery.7 Syphilis
Work occurred at East Carolina University. left untreated during pregnancy can lead to severe
The authors declare no conflicts of interest. fetal neurological, developmental, and musculoskel-
Correspondence: Christine R. Rowe, MSN, RN, CCRN, East Carolina etal impairments, as well as fetal demise.8,9 Morbid-
University, 2314 Meadow Gate Dr, Greensboro, NC 27455 (rowech16@
students.ecu.edu; rnchrissy25@yahoo.com). ity and mortality risks during the perinatal period
Copyright © 2018 by The National Association of Neonatal Nurses are estimated at 33.6% and 6.5%, respectively.10
DOI: 10.1097/ANC.0000000000000534 Maternal syphilis infection may be without obvious
Copyright © 2018 National Association of Neonatal Nurses. Unauthorized reproduction of this article is prohibited.
Congenital Syphilis 439
clinical manifestations and hidden behind a shroud Ethnic disparities are well reported. The incidence
of shame; therefore, properly timed prenatal screen- of syphilis among African Americans is 43.1 cases
ing is crucial.11 per 100,000 live births compared with American
Increased awareness of CS is essential for the Indians with 31.6 cases per 100,000 live births.3
obtainment of optimal neonatal outcomes. The pur- Hispanics account for 20.5 cases per 100,000 live
pose for this article is to present pertinent epidemio- births, while Asian and Pacific Islanders account for
logical trends, as well as the pathophysiology, diag- 9.2 cases per 100,000 live births, and whites with
nosis, and management of CS, as it relates to 5.3 cases per 100,000 live births.4 Thoughtful con-
collaborative nursing and medical care of the sideration of ethnicity as a risk factor for CS infec-
affected family unit. tion is important.
FIGURE 1
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440 Rowe et al
FIGURE 2
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Congenital Syphilis 441
FIGURE 3
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442 Rowe et al
swelling and/or inflammation of the elbows and infection. Nontreponemal tests include the venereal
knees.7,24 Early diagnosis is important to prevent pro- disease research laboratory (VDRL) and the rapid
gression to LCS and severe, long-term sequelae.25,26 plasma reagin (RPR), which measure the levels of
IgG and IgM antibodies.27 These antibodies can be
Lifelong Implications of Congenital Syphilis detected as soon as 6 days after bacterial invasion.27
Infants affected by CS are at risk for severe, lifelong The RPR is predominantly used for serum testing
sequelae associated with the disease. If unidentified and the VDRL for cerebrospinal fluid testing.25 A
and/or left untreated, the disease can progress to positive screen should be followed by a treponemal
organ damage, including heart failure; brain damage test, which is more specific.23,27
and infections, which can result in seizures and The treponemal serology tests are used for confir-
paralysis; and deformities of the arms and legs mation of the presence of the bacteria and detect
resulting in immobility.7,8 Other detrimental conse- antibodies against T. pallidum antigens.23,27 Trepo-
quences may include growth restrictions, hearing nemal tests include fluorescent treponemal anti-
loss, blindness, and death.7,8 These medical implica- body-absorbed test, microhemagglutination assay
tions exacerbate the emotional and economic bur- for T. pallidum, enzyme-linked immunosorbent
dens faced by families due to the need for frequent assays, and Treponemal IgM in serum.27,29 Trepone-
follow-ups aimed at limiting these sequelae; this mal tests can detect both IgM and IgG antibodies
highlights the importance of the nurse’s role in early that are specific to T. pallidum.27 These antibodies
identification of the disease.8 remain positive for life; therefore, positive results
need to be verified with nontreponemal testing to
DIAGNOSIS OF CONGENITAL SYPHILIS determine past versus active infection.
Although used less commonly, polymerase chain
The easy transfer of immunoglobulin (Ig) G antibod- reaction techniques used to amplify and detect the T.
ies across the placenta to the fetus makes the diagno- pallidum DNA in tissue and body fluid samples are
sis of CS challenging in the fetal and early neonatal more reliable due to rapid turnaround times, valid-
period, as it can complicate the interpretation of ity, sensitive detection of T. pallidum, and diagnostic
serologic tests in the neonate.1,11 Prenatal testing for accuracy.27,30 These characteristics of polymerase
CS can also be complicated by the inability to suc- chain reaction enable rapid diagnosis and implemen-
cessfully culture the T. pallidum bacteria.2 Direct tation of treatment.30 Dark-field microscopy or
visualization of the spirochete and serologic testing immunofluorescent staining can be performed to
continue to be the gold standard for diagnosing the directly visualize the bacteria in fluid samples from
infection due to these perplexities, with serologic lesions and infected tissues.27
testing the more common due to cost-effectiveness, The presence of T. pallidum in amniotic fluid or
ease of use, and reliability.2,27 Imaging studies and fetal blood can confirm the diagnosis in utero. Pre-
percutaneous umbilical cord blood sampling can natal ultrasounds may also reveal features that are
also aid in the diagnosis.28 suggestive of CS including hepatomegaly, spleno-
megaly, placentomegaly, and fetal growth restric-
Prenatal Diagnosis tion.28 Prenatal screening allows for prompt treat-
Prenatal screening results in decreased fetal mortal- ment and reduction in the sequelae of CS, highlighting
ity and is the rationale for serologic testing in the the importance of serologic screening at different
early trimesters.23 Positive maternal serologic testing stages of pregnancy.23
during any stage of pregnancy is concerning for
ECS, mandating neonatal testing.23 The Centers for Postnatal Diagnosis
Disease Control and Prevention (CDC) recommends The diverse clinical features of CS can make the diag-
routine testing at the first prenatal visit for all preg- nosis daunting. Assessment should begin with a thor-
nant women, and in the second trimester and at ough physical examination for skin lesions, jaundice,
delivery for high-risk women and those living in mucous membrane fissures or patches, and thick or
high prevalence areas.11 Laboratory serologic testing bloody nasal discharge.19,23 Next, detailed palpation
falls into 2 categories: nontreponemal and trepone- should occur to assesses for organomegaly.19
mal. Direct detection in the form of DNA assessment All infants suspected of having CS should be tested
and visualization of the bacteria are confirmatory with the same nontreponemal tests that were per-
testing methods as well.9 formed on the mother, and the results should be ana-
Nontreponemal tests are nonspecific and may lyzed for the difference in titers.4,23 A positive serum
produce similar results in the presence of other viral IgM in the infant, detected through RPR, is reflective
and bacterial infections, or autoimmune condi- of active syphilis infection because maternal IgM
tions.23,27 Pregnancy can also cause false-positive does not cross the placenta.23 Congenital syphilis is
results. The nontreponemal serology tests are used indicated when the nontreponemal serologic titer in
to both screen and monitor the status of the the infant is fourfold higher than that of the
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Congenital Syphilis 443
mother.11,23 The American Academy of Pediatrics nontreponemal serologic testing every 3 months
and the CDC recommend that all infants born to until the tests are nonreactive or the titers are less
mothers who were inadequately treated during preg- than fourfold.4
nancy should be further evaluated with complete
blood counts and cerebrospinal fluid analysis for IMPLICATIONS OF CONGENITAL
protein, cell count, and quantitative VDRL.23 Other SYPHILIS
diagnostic tests include eye examinations to assess
for structural abnormalities; chest and long bones Congenital syphilis is a preventable disease; timely,
radiography, which may show radiolucency, osteo- adequate treatment of the pregnant woman affected
chondritis, periostitis, bone destruction, and opaci- with syphilis can limit the associated emotional,
ties; and liver function tests.23,25 Once diagnosis is social, economic, and medical burdens.10,31 The emo-
made, prompt and adequate treatment is necessary tional loss of a fetus or child can be traumatizing for
to minimize sequelae. parents.31 When losses are caused by congenital infec-
tions, such as syphilis, the psychological effect of the
MANAGEMENT OF CONGENITAL traumatic event can be escalated by feelings of guilt,
SYPHILIS blame, and in some cases, depression in the mother.31
Therefore, implementation of programs that place
Parenterally administered penicillin G is the only focus on the prevention of CS is warranted.31
known effective antimicrobial to treat maternal The economic burden associated with the treat-
syphilis and prevent maternal transfer to the fetus or ment of CS affects not only families, but society as a
newborn.7,9,11 No other antibiotic efficaciously whole.31 The hospitalization cost for an infant affected
destroys the T. pallidum bacteria; therefore, desensi- by CS is as much as 7 times higher, and the length of
tization has to be instituted and the therapy contin- hospital stay is approximately 8 days longer than for
ued in cases of penicillin allergy.2,9,13,23 The efficacy a healthy infant.32 These cost increase estimates do
of intramuscularly administered benzathine penicil- not include postdischarge medical expenses related to
lin G against syphilis is credited to its slow release late CS.32 In comparison, prenatal screening offers a
into the body tissues.13 Management and treatment long-term cost-benefit for public health entities.31
of CS depend on the stage and treatment of maternal
disease, clinical manifestations, and evaluation of IMPLICATIONS FOR NURSING
the findings in the infant (Table 1).23 PRACTICE
Diagnosis made later in infancy is subject to more
aggressive and more frequent dosing due to the risk Nurses play an integral role in the detection, early
of neurosyphilis.23 The infant diagnosed with CS implementation of treatment, effective management,
should have structured follow-up that includes and elimination of CS.33 The expert knowledge
Copyright © 2018 National Association of Neonatal Nurses. Unauthorized reproduction of this article is prohibited.
444 Rowe et al
possessed by neonatal nurses and neonatal nurse prac- prenatal screening and adequate penicillin treat-
titioners empowers them to be vigilant in gathering ment.19,20,25 Syphilis left untreated during pregnancy
comprehensive pertinent maternal history and per- poses the greatest risk of severe irreversible sequelae
forming careful detailed physical examinations of the and/or fetal, neonatal, and infant death.4 Vigilant
newborn. Ordering the appropriate diagnostic tests; prenatal and at delivery screening, treatment of the
correctly interpreting results; initiating timely treatment infected mother during pregnancy, meticulous
and management strategies; and preparing the infant assessment of the newborn, and prompt initiation of
for structured follow-up while effectively communicat- treatment with benzathine penicillin G when indi-
ing the plan of care to the family are all activities that cated, along with appropriate follow-up postdis-
neonatal nurse practitioners are efficient at.7,8,15,33 charge, are crucial in reducing the incidence of CS
The maternal history will provide pertinent infor- and constraint of negative sequelae.4,11,15,25 Neonatal
mation regarding the need for further diagnostic eval- nurses are experts in newborn care, advocates at the
uation of the neonate. Newborns of mothers with a bedside, and knowledgeable about infectious dis-
reactive nontreponemal or treponemal serologic test eases; as such, they have a critical role in the inter-
or who never received syphilis screening during preg- professional strategic approach to decrease the inci-
nancy should have serologic testing done in the form dence and limit the severe sequelae of CS.
of RPR or VDRL prior to hospital discharge.9,11 Bed-
side registered nurses are influential in ensuring that References
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