Syphilis, Congenital
1996 Case Definition
1996 Case Definition
Subtype(s)
- Syphilis
- Syphilis, primary
- Syphilis, secondary
- Syphilis, latent
- Syphilis, early latent
- Syphilis, late latent
- Syphilis, latent unknown duration
- Neurosyphilis
- Syphilis, late, with clinical manifestations other than neurosyphilis
- Syphilitic stillbirth
Clinical Description
A condition caused by infection in utero with Treponema pallidum. A wide spectrum of severity exists, and only severe cases are clinically apparent at birth. An infant or child (aged less than 2 years) may have signs such as hepatosplenomegaly, rash, condyloma lata, snuffles, jaundice (nonviral hepatitis), pseudoparalysis, anemia, or edema (nephrotic syndrome and/or malnutrition). An older child may have stigmata (e.g., interstitial keratitis, nerve deafness, anterior bowing of shins, frontal bossing, mulberry molars, Hutchinson teeth, saddle nose, rhagades, or Clutton joints).
Laboratory Criteria For Diagnosis
Demonstration of T. pallidum by darkfield microscopy, fluorescent antibody, or other specific stains in specimens from lesions, placenta, umbilical cord, or autopsy material.
Subtype(s) Case Definition
Background
Syphilis is a complex sexually transmitted disease that has a highly variable clinical course. Classification by a clinician with expertise in syphilis may take precedence over the following case definitions developed for surveillance purposes.
Comments
The 1996 case definition appearing on this page was re-published in the 2009 CSTE position statement 09-ID-62 (available at URL). Thus, the 1996 and 2010 versions of the case definition are identical.
Clinical Description
A stage of infection with Treponema pallidum characterized by one or more chancres (ulcers); chancres might differ considerably in clinical appearance.
Laboratory Criteria For Diagnosis
Demonstration of T. pallidum in clinical specimens by darkfield microscopy, direct fluorescent antibody (DFA-TP), or equivalent methods.
Case Classification
Probable
A clinically compatible case with one or more ulcers (chancres) consistent with primary syphilis and a reactive serologic test (nontreponemal: Venereal Disease Research Laboratory [VDRL] or rapid plasma reagin [RPR]; treponemal: fluorescent treponemal antibody absorbed [FTA-ABS] or microhemagglutination assay for antibody to T. pallidum [MHA-TP])
Confirmed
A clinically compatible case that is laboratory confirmed
Clinical Description
A stage of infection caused by T. pallidum and characterized by localized or diffuse mucocutaneous lesions, often with generalized lymphadenopathy. The primary chancre may still be present.
Laboratory Criteria For Diagnosis
Demonstration of T. pallidum in clinical specimens by darkfield microscopy, DFA-TP, or equivalent methods
Case Classification
Probable
A clinically compatible case with a nontreponemal (VDRL or RPR) titer greater than or equal to 4
Confirmed
A clinically compatible case that is laboratory confirmed
Clinical Description
A stage of infection caused by T. pallidum in which organisms persist in the body of the infected person without causing symptoms or signs. Latent syphilis is subdivided into early, late, and unknown categories based on the duration of infection.
Case Classification
Probable
No clinical signs or symptoms of syphilis and the presence of one of the following:
- No past diagnosis of syphilis, a reactive nontreponemal test (i.e., VDRL or RPR), and a reactive treponemal test (i.e., FTA-ABS or MHA-TP)
- A past history of syphilis therapy and a current nontreponemal test titer demonstrating fourfold or greater increase from the last nontreponemal test titer
Clinical Description
A subcategory of latent syphilis. When initial infection has occurred within the previous 12 months, latent syphilis is classified as early latent.
Case Classification
Probable
Latent syphilis (see Syphilis, latent) in a person who has evidence of having acquired the infection within the previous 12 months based on one or more of the following criteria:
- Documented seroconversion or fourfold or greater increase in titer of a nontreponemal test during the previous 12 months
- A history of symptoms consistent with primary or secondary syphilis during the previous 12 months
- A history of sexual exposure to a partner who had confirmed or probable primary or secondary syphilis or probable early latent syphilis (documented independently as duration less than 1 year)
- Reactive nontreponemal and treponemal tests from a person whose only possible exposure occurred within the preceding 12 months
Clinical Description
A subcategory of latent syphilis. When initial infection has occurred greater than 1 year previously, latent syphilis is classified as late latent.
Case Classification
Probable
Latent syphilis (see Syphilis, latent) in a patient who has no evidence of having acquired the disease within the preceding 12 months (see Syphilis, early latent) and whose age and titer do not meet the criteria specified for latent syphilis of unknown duration.
Clinical Description
A subcategory of latent syphilis. When the date of initial infection cannot be established as having occurred within the previous year and the patient's age and titer meet criteria described below, latent syphilis is classified as latent syphilis of unknown duration.
Case Classification
Probable
Latent syphilis (see Syphilis, latent) that does not meet the criteria for early latent syphilis, and the patient is aged 13-35 years and has a nontreponemal titer greater than or equal to 32
Clinical Description
Evidence of central nervous system infection with T. pallidum
Laboratory Criteria For Diagnosis
A reactive serologic test for syphilis and reactive VDRL in cerebrospinal fluid (CSF)
Case Classification
Probable
Syphilis of any stage, a negative VDRL in CSF, and both the following:
- Elevated CSF protein or leukocyte count in the absence of other known causes of these abnormalities
- Clinical symptoms or signs consistent with neurosyphilis without other known causes for these clinical abnormalities
Confirmed
Syphilis of any stage that meets the laboratory criteria for neurosyphilis
Clinical Description
Clinical manifestations of late syphilis other than neurosyphilis may include inflammatory lesions of the cardiovascular system, skin, and bone. Rarely, other structures (e.g., the upper and lower respiratory tracts, mouth, eye, abdominal organs, reproductive organs, lymph nodes, and skeletal muscle) may be involved. Late syphilis usually becomes clinically manifest only after a period of 15-30 years of untreated infection.
Laboratory Criteria For Diagnosis
Demonstration of T. pallidum in late lesions by fluorescent antibody or special stains (although organisms are rarely visualized in late lesions)
Case Classification
Probable
Characteristic abnormalities or lesions of the cardiovascular system, skin, bone, or other structures with a reactive treponemal test, in the absence of other known causes of these abnormalities, and without CSF abnormalities and clinical symptoms or signs consistent with neurosyphilis
Confirmed
A clinically compatible case that is laboratory confirmed
Comments
Analysis of CSF for evidence of neurosyphilis is necessary in the evaluation of late syphilis with clinical manifestations.
Clinical Description
A fetal death that occurs after a 20-week gestation or in which the fetus weighs greater than 500 g and the mother had untreated or inadequately treated* syphilis at delivery
Comments
For reporting purposes, syphilitic stillbirths should be reported as cases of congenital syphilis.
*Inadequate treatment consists of any non-penicillin therapy or penicillin given less than 30 days before delivery.
Case Classification
Probable
A condition affecting an infant whose mother had untreated or inadequately treated* syphilis at delivery, regardless of signs in the infant, or an infant or child who has a reactive treponemal test for syphilis and any one of the following:
- Any evidence of congenital syphilis on physical examination
- Any evidence of congenital syphilis on radiographs of long bones
- A reactive cerebrospinal fluid (CSF) venereal disease research laboratory (VDRL)
- An elevated CSF cell count or protein (without other cause)
- A reactive fluorescent treponemal antibody absorbed--19S-IgM antibody test or IgM enzyme-linked immunosorbent assay
Confirmed
A case that is laboratory confirmed.
Comments
Congenital and acquired syphilis may be difficult to distinguish when a child is seropositive after infancy. Signs of congenital syphilis may not be obvious, and stigmata may not yet have developed. Abnormal values for CSF VDRL, cell count, and protein, as well as IgM antibodies, may be found in either congenital or acquired syphilis. Findings on radiographs of long bones may help because radiographic changes in the metaphysis and epiphysis are considered classic signs of congenitally acquired syphilis. The decision may ultimately be based on maternal history and clinical judgment. In a young child, the possibility of sexual abuse should be considered as a cause of acquired rather than congenital syphilis, depending on the clinical picture. For reporting purposes, congenital syphilis includes cases of congenitally acquired syphilis among infants and children as well as syphilitic stillbirths.
*Inadequate treatment consists of any non-penicillin therapy or penicillin given less than 30 days before delivery.