Congenital syphilis is a multisystem infection caused by Treponema pallidum and transmitted to the fetus via the placenta. Early signs are characteristic skin lesions, lymphadenopathy, hepatosplenomegaly, failure to thrive, blood-stained nasal discharge, perioral fissures, meningitis, choroiditis, hydrocephalus, seizures, intellectual disability, osteochondritis, and pseudoparalysis (Parrot atrophy of newborn). Later signs are gummatous ulcers, periosteal lesions, paresis, tabes, optic atrophy, interstitial keratitis, sensorineural deafness, and dental deformities. Diagnosis is clinical, confirmed by microscopy or serology. Treatment is penicillin.
( See also Syphilis in adults and Overview of Neonatal Infections.)
Overall risk of transplacental infection of the fetus is about 60 to 80%, and likelihood is increased during the 2nd half of the pregnancy. Untreated primary or secondary syphilis in the mother usually is transmitted, but latent or tertiary syphilis is transmitted in only about 20% of cases. Untreated syphilis in pregnancy is also associated with a significant risk of stillbirth and neonatal death. The rate of congenital syphilis in the US has been rising dramatically in recent years with an over 500% increase in cases since 2010. Over 2000 cases were reported in 2020, including at least 149 stillbirths and infant deaths (1). Congenital syphilis has also become more geographically widespread in the US and disproportionately affects infants in certain racial and ethnic minority groups, particularly American Indian or Alaskan Native groups, which likely reflects differential access to health care (1) rather than intrinsic predisposition.
In infected neonates, manifestations of syphilis are classified as early congenital (ie, birth through age 2 years) and late congenital (ie, after age 2 years).
General reference
1. Centers for Disease Control and Prevention (CDC): Sexually Transmitted Disease Surveillance 2020: Preliminary 2021 Data: Syphilis.
Symptoms and Signs of Congenital Syphilis
Many patients are asymptomatic, and the infection may remain clinically silent throughout their life.
Early congenital syphilis
Late congenital syphilis typically manifests after 2 years of life and causes gummatous ulcers that tend to involve the nose, septum, and hard palate and periosteal lesions that result in saber shins and bossing of the frontal and parietal bones. Neurosyphilis is usually asymptomatic, but juvenile paresis and tabes may develop. Optic atrophy, sometimes leading to blindness, may occur. Interstitial keratitis, the most common eye lesion, frequently recurs, often resulting in corneal scarring. Sensorineural deafness, which is often progressive, may appear at any age. Hutchinson incisors, mulberry molars, perioral fissures (rhagades), and maldevelopment of the maxilla resulting in “bulldog” facies are characteristic, if infrequent, sequelae.
Osteoperiostitis of the tibia leads to characteristic saber shins in this patient with congenital syphilis.
Image courtesy of Robert Sumpter via the Public Health Image Library of the Centers for Disease Control and Prevention.
Interstitial keratitis manifests with varying degrees of corneal opacification and neovascularization. The neovascularization appears as the faint, pink areas in the peripheral cornea from the 11 o'clock to the 1 o'clock position.
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This photo shows triangular shaped deformity of right upper and left lower incisors caused by congenital syphilis.
Image courtesy of Robert E. Sumpter via the Public Health Image Library of the Centers for Disease Control and Prevention.
Osteoperiostitis of the tibia leads to characteristic saber shins in this patient with congenital syphilis.
Image courtesy of Robert Sumpter via the Public Health Image Library of the Centers for Disease Control and Prevention.
Interstitial keratitis manifests with varying degrees of corneal opacification and neovascularization. The neovascularization appears as the faint, pink areas in the peripheral cornea from the 11 o'clock to the 1 o'clock position.
© Springer Science+Business Media
This photo shows triangular shaped deformity of right upper and left lower incisors caused by congenital syphilis.
Image courtesy of Robert E. Sumpter via the Public Health Image Library of the Centers for Disease Control and Prevention.
Diagnosis of Congenital Syphilis
Early congenital syphilis: Clinical evaluation; darkfield microscopy of lesions, placenta, or umbilical cord; serologic testing of mother and neonate; possibly cerebrospinal fluid (CSF) analysis
Late congenital syphilis: Clinical evaluation, serologic testing of mother and child
Early congenital syphilis
Diagnosis of early congenital syphilis is usually suspected based on maternal serologic testing, which is routinely done early in pregnancy, and often repeated in the 3rd trimester and at delivery. Neonates of mothers with serologic evidence of syphilis should have a thorough examination, darkfield microscopy or immunofluorescent staining of any skin or mucosal lesions, and a quantitative nontreponemal serum test (eg, rapid plasma reagin [RPR], Venereal Disease Research Laboratory [VDRL]); cord blood is not used for serum testing because results are less sensitive and specific. The placenta or umbilical cord should be analyzed using darkfield microscopy or fluorescent antibody staining if available.
Infants and young children with clinical signs of illness or suggestive serologic test results also should have a lumbar puncture with CSF analysis for cell count, VDRL, and protein; complete blood count (CBC) with platelet count; liver tests; long-bone x-rays; and other tests as clinically indicated (ophthalmologic evaluation, chest x-rays, neuroimaging, and auditory brain stem response).
Syphilis can cause many different abnormalities on long-bone x-rays, including
Periosteal reactions
Diffuse or localized osteitis
Metaphysitis
The osteitis is sometimes described as "diffuse moth-eaten changes of the shaft." Metaphysitis commonly appears as lucent or dense bands that can alternate to give a sandwich or celery stalk appearance. The Wimberger sign is symmetric erosions of the upper tibia, but there can also be erosions in the metaphysis of other long bones. Excessive callus formation at the ends of long bones has been described. Many affected infants have more than one of these findings.
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Diagnosis is confirmed by microscopic visualization of spirochetes in samples from the neonate or the placenta. Diagnosis based on neonatal serologic testing is complicated by the transplacental transfer of maternal IgG antibodies, which can cause a positive test in the absence of infection. However, a neonatal nontreponemal antibody titer > 4 times the maternal titer would not generally result from passive transfer, and diagnosis is considered confirmed or highly probable. Maternal disease acquired late in pregnancy may be transmitted before development of antibodies. Thus, in neonates with lower titers but typical clinical manifestations, syphilis is also considered highly probable. In neonates with no signs of illness and low or negative serologic titers, syphilis is considered possible; subsequent approach depends on various maternal and neonatal factors (see Follow up).
The utility of fluorescent assays for antitreponemal IgM, which is not transferred across the placenta, is controversial, but such assays have been used to detect neonatal infection. Any positive nontreponemal test should be confirmed with a specific treponemal test to exclude false-positive results, but confirmative testing should not delay treatment in a symptomatic infant or an infant at high risk of infection.
Late congenital syphilis
Diagnosis of late congenital syphilis is by clinical history, distinctive physical signs, and positive serologic tests ( see also Diagnostic tests for syphilis). The Hutchinson triad of interstitial keratitis, Hutchinson incisors, and 8th cranial nerve deafness is diagnostic. Sometimes the standard nontreponemal serologic tests for syphilis are negative, but the fluorescent treponemal antibody absorption test (FTA-ABS) is positive. The diagnosis should be considered in cases of unexplained deafness, progressive intellectual deterioration, or keratitis.
Follow up
All seropositive infants and those whose mothers were seropositive should have VDRL or RPR titers every 2 to 3 months until the test is nonreactive or the titer has decreased 4-fold. In uninfected and successfully treated infants, nontreponemal antibody titers are usually nonreactive by 6 months. Passively acquired treponemal antibodies may be present for longer, perhaps 15 months. It is important to remember to use the same specific nontreponemal test to monitor titers in mothers, neonates, infants, and young children over time.
If VDRL or RPR remain reactive past 6 to 12 months of age or titers increase, the infant should be reevaluated (including lumbar puncture for CSF analysis, and complete blood count with platelet count, long-bone x-rays, and other tests as clinically indicated).
Treatment of Congenital Syphilis
Parenteral penicillin
Pregnant women
see Late latent or tertiary syphilis). Occasionally, a severe Jarisch-Herxheimer reaction occurs after such therapy, leading to spontaneous abortion. Patients allergic to penicillin may be desensitized and then treated with penicillin.
Early congenital syphilis
In confirmed or highly probable cases, the 2021 Centers for Disease Control and Prevention (CDC) STI treatment guidelines see Table: Recommended Dosages of Selected Parenteral Antibiotics for Neonates). If ≥ 1 day of therapy is missed, the entire course must be repeated. This regimen is also recommended for infants with possible syphilis if the mother fits any of the following criteria:
Untreated
Treatment status unknown
Treated ≤ 4 weeks before delivery
Inadequately treated (a nonpenicillin regimen)
Maternal evidence of relapse or reinfection (≥ 4-fold increase in maternal titer)
In infants with possible syphilis whose mothers were not adequately treated but who are clinically well and have a completely negative full evaluation, a single dose of benzathine penicillin 50,000 units/kg IM is an alternative treatment choice in selected circumstances but only if follow-up is ensured.
Infants with possible syphilis whose mothers were adequately treated and who are clinically well also can be given a single dose of benzathine penicillin 50,000 units/kg IM. Alternatively, if close follow-up is ensured, some clinicians defer penicillin and do nontreponemal serologic testing monthly for 3 months and then at 6 months; antibiotics are given if titers rise or are positive at 6 months.
Older infants and children with newly diagnosed congenital syphilis
Many patients do not revert to seronegativity but do have a 4-fold decrease in titer of reagin (eg, VDRL) antibody. Patients should be reevaluated at regular intervals to ensure the appropriate serologic response to therapy has occurred and that there is no indication of relapse.
Prevention of Congenital Syphilis
Pregnant women should be routinely tested for syphilis in the 1st trimester and, if they live in a community with high rates of syphilis or have any risk factors for syphilis, retested in the 3rd trimester and at delivery. In the US, most states require 1st trimester screening, and many require the later testing as well (1). In 99% of cases, adequate treatment during pregnancy cures both mother and fetus. However, in some cases, syphilis treatment late in pregnancy eliminates the infection but not some signs of syphilis that appear at birth. Treatment of the mother < 4 weeks before delivery may not eradicate fetal infection.
When congenital syphilis is diagnosed, other family members should be examined for physical and serologic evidence of infection. Retreatment of the mother in subsequent pregnancies is necessary only if serologic titers suggest relapse or reinfection. Women who remain seropositive after adequate treatment may have been reinfected and should be reevaluated. A mother without lesions who is seronegative but who has had venereal exposure to a person known to have syphilis should be treated, because there is a 25 to 50% chance that she acquired syphilis.
Prevention reference
1. Centers for Disease Control and Prevention (CDC): Sexually Transmitted Diseases: State Statutory and Regulatory Language Regarding Prenatal Syphilis Screenings in the United States.
Key Points
Manifestations of syphilis are classified as early congenital (birth through age 2 years) and late congenital (after age 2 years).
Risk of transmission of maternal primary or secondary syphilis is 60 to 80%; risk of transmission of latent or tertiary syphilis is about 20%.
Diagnose clinically and by serologic testing of mother and child; darkfield examination of skin lesions and sometimes of placenta and umbilical cord samples may help diagnose early congenital syphilis.
Treat with parenteral penicillin.
More Information
The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.
Centers for Disease Control and Prevention (CDC): Sexually Transmitted Infections Treatment Guidelines (2021)
