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(See also Sexually Transmitted Diseases (STDs): Syphilis.)
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's 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.
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 usually is not. In neonates, manifestations of syphilis are classified as early congenital (ie, birth through age 2 yr) and late congenital (ie, after age 2 yr).
Symptoms and Signs
Many patients are asymptomatic, and the infection may remain clinically silent throughout their life.
Early congenital syphilis commonly manifests during the first 3 mo of life. Manifestations include characteristic vesiculobullous eruptions or a macular, copper-colored rash on the palms and soles and papular lesions around the nose and mouth and in the diaper area, as well as petechial lesions. Generalized lymphadenopathy and hepatosplenomegaly often occur. The infant may fail to thrive and have a characteristic mucopurulent or blood-stained nasal discharge causing snuffles. A few infants develop meningitis, choroiditis, hydrocephalus, or seizures, and others may be intellectually disabled. Within the first 8 mo of life, osteochondritis (chondroepiphysitis), especially of the long bones and ribs, may cause pseudoparalysis of the limbs with characteristic radiologic changes in the bones.
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's incisors, mulberry molars, perioral fissures (rhagades), and maldevelopment of the maxilla resulting in “bulldog” facies are characteristic, if infrequent, sequelae.
Diagnosis
Early congenital syphilis:
Diagnosis is usually suspected based on maternal serologic testing, which is routinely done early in pregnancy, and often in the 3rd trimester and at delivery. Neonates of mothers with positive tests should have a thorough examination, darkfield microscopy 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. Infants 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; CBC; liver function tests; and long-bone x-rays.
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 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 (if maternal infection is diagnosed) negative serologic titers, syphilis is considered possible; subsequent approach depends on various maternal and neonatal factors (see Infections in Neonates: 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 is by clinical history, distinctive physical signs, and positive serologic tests (see also Sexually Transmitted Diseases (STDs): Diagnostic tests for syphilis). Hutchinson's triad of interstitial keratitis, Hutchinson's incisors, and 8th cranial nerve deafness is diagnostic. Sometimes the standard serologic tests for syphilis are negative, but the fluorescent treponemal antibody absorption test (FTA-ABS) is usually 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 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.
If VDRL or RPR remain reactive past 6 to 12 mo 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
Pregnant women:
Pregnant women in the early stages of syphilis receive one dose of benzathine penicillin G (1.2 million units IM in each buttock for a total of 2.4 million units). For later stages of syphilis or neurosyphilis, the appropriate regimen for nonpregnant patients should be followed (see Sexually Transmitted Diseases (STDs): 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. RPR and VDRL tests become negative by 3 mo after adequate treatment in most patients and by 6 mo in nearly all patients. Erythromycin therapy is inadequate for both the mother and fetus and is not recommended. Tetracycline is contraindicated.
Early congenital syphilis:
In confirmed or highly probable cases, 2006 Centers for Disease Control and Prevention (CDC) guidelines recommend aqueous penicillin G 50,000 units/kg IV q 12 h for the first 7 days of life and q 8 h thereafter for a total of 10 days or procaine penicillin G 50,000 units/kg IM once/day for 10 days. This regimen also is used for infants with possible syphilis if the mother fits any of the following criteria:
In infants with possible syphilis and a negative 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 can also 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.
Older infants and children with newly diagnosed congenital syphilis:
CSF should be examined before treatment starts. The CDC recommends that any child with late congenital syphilis be treated with aqueous crystalline penicillin G 50,000 units/kg IV q 4 to 6 h for 10 days. Many patients do not revert to seronegativity but do have a 4-fold decrease in titer of reagin (eg, VDRL) antibody. Interstitial keratitis is usually treated with corticosteroid and atropine drops in consultation with an ophthalmologist. Patients with nerve deafness may benefit from penicillin plus a corticosteroid such as prednisone 0.5 mg/kg po once/day for 1 wk, followed by 0.3 mg/kg once/day for 4 wk, after which the dose is gradually reduced over 2 to 3 mo. (Corticosteroids have not been critically evaluated in these conditions.) Contacts should be traced, and patients should be kept under long-term surveillance.
Prevention
Pregnant women should be routinely tested for syphilis 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, treatment late in pregnancy eliminates the infection but not some signs of syphilis that appear at birth.
When congenital syphilis is diagnosed, other family members should be examined regularly for physical and serologic evidence of infection. Retreatment of the mother in subsequent pregnancies is necessary only if serologic titers remain positive. Women who remain seropositive after adequate treatment may have been reinfected and should be retreated. A mother without lesions who is seronegative but who has had venereal exposure to a known person with syphilis should be treated, because there is a 25 to 50% chance that she acquired syphilis.
Last full review/revision October 2009 by Mary T. Caserta, MD
Content last modified February 2012
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