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Congenital Syphilis - Pediatrics - MSD Manual Professional Edition PDF
Congenital Syphilis - Pediatrics - MSD Manual Professional Edition PDF
Professional Version
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Congenital Syphilis
By Brenda L. Tesini , MD, University of Rochester School of Medicine and Dentistry
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.
Late congenital syphilis typically manifests after 2 yr 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.
Manifestations of Late
Congenital Syphilis
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Diagnosis
Early congenital syphilis: Clinical evaluation; darkfield microscopy of lesions, placenta, or umbilical cord;
serologic testing of mother and neonate; possibly CSF analysis
Late congenital syphilis: Clinical evaluation, serologic testing of mother and child
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.
Wimberger Sign
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 Congenital Syphilis : 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.
Follow up
All seropositive infants and those whose mothers were seropositive should have VDRL or RPR titers every 2 to 3 mo
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 mo. Passively acquired treponemal antibodies may be
present for longer, perhaps 15 mo. 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 mo of age or titers increase, the infant should be reevaluated (including
lumbar puncture for CSF analysis, and CBC with platelet count, long-bone x-rays, and other tests as clinically indicated).
Treatment
Parenteral penicillin
Pregnant women
Pregnant women in the early stages of syphilis receive benzathine penicillin G (2.4 million units IM in a single dose). For
later stages of syphilis or neurosyphilis, the appropriate regimen for nonpregnant patients should be followed (see
Syphilis : Late 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.
After adequate treatment, RPR and VDRL test results decrease 4-fold by 6 to 12 mo in most patients and revert to
negative by 2 yr in nearly all patients. Erythromycin therapy is inadequate for both the mother and fetus and is not
recommended. Tetracycline is contraindicated.
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 assured.
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 assured, some clinicians defer
penicillin and do nontreponemal serologic testing monthly for 3 mo and then at 6 mo; antibiotics are given if titers rise
or are positive at 6 mo.
Prevention
Pregnant women should be routinely tested for syphilis in the 1st trimester and retested if they acquire other sexually
transmitted diseases during pregnancy. 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 wk 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.
Key Points
Manifestations of syphilis are classified as early congenital (birth through age 2 yr) and late congenital
(after age 2 yr).
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.
More Information
Centers for Disease Control and Prevention (CDC) guidelines for congenital syphilis (2015)
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