Syphilis, Congenital
1990 Case Definition
1990 Case Definition
Subtype(s)
- Syphilis
- Syphilis, primary
- Syphilis, secondary
- Syphilis, latent
- Syphilis, early latent
- Syphilis, late latent
- Syphilis, latent unknown duration
- 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 (less than 2 years) may have signs such as hepatosplenomegaly, characteristic skin rash, condyloma lata, snuffles, jaundice (non-viral hepatitis), pseudoparalysis, anemia, or edema (nephrotic syndrome and/or malnutrition). An older child may have stigmata such as 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 with 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.
Clinical Description
The characteristic lesion of primary syphilis is the chancre, but atypical primary lesions may occur.
Laboratory Criteria For Diagnosis
Demonstration of Treponema pallidum in clinical specimens by darkfield, fluorescent antibody, or equivalent microscopic methods
Case Classification
Probable
A clinically compatible case with one or more ulcers (chancres) consistent with primary syphilis and a reactive serologic test
Confirmed
A clinically compatible case that is laboratory confirmed
Clinical Description
A stage of infection due to Treponema pallidum, characterized by localized or diffuse mucocutaneous lesions and generalized lymphadenopathy. Constitutional symptoms are common, and clinical manifestations are protean. The primary chancre may still be present.
Laboratory Criteria For Diagnosis
Demonstration of T. pallidum in clinical specimens by darkfield, fluorescent antibody, or equivalent microscopic methods
Case Classification
Probable
A clinically compatible case with a reactive nontreponemal (VDRL, RPR) test titer of greater than or equal to 4
Confirmed
A clinically compatible case that is laboratory confirmed
Clinical Description
A stage of infection due to Treponema 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 syphilis categories based upon the length of elapsed time from initial infection.
Case Classification
Presumptive
No clinical signs or symptoms of syphilis and the presence of one of the following:
- No past diagnosis of syphilis and a reactive nontreponemal test, and a reactive treponemal (fluorescent treponemal antibody-absorbed [FTA-ABS], microhemagglutination assay for antibody to Treponema pallidum [MHA-TP]) test
- 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.
Case Classification
Presumptive
Latent syphilis (see above) of a person who has evidence of having acquired the infection within the previous 12 months based on one or more of the following criteria:
- A nonreactive serologic test for syphilis or a nontreponemal titer that has dropped fourfold within the past 12 months
- A history of symptoms consistent with primary or secondary syphilis without a history of subsequent treatment in the past 12 months
- A history of sexual exposure to a partner with confirmed or presumptive primary or secondary syphilis, or presumptive early latent syphilis, and no history of treatment in the past 12 months
- Reactive nontreponemal and treponemal tests from an individual 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.
Case Classification
Presumptive
Latent syphilis (see above) of a patient who shows no evidence of having acquired the disease within the past 12 months (see Early Latent Syphilis) and whose age and titer do not meet the criteria specified for unknown latent syphilis
Clinical Description
A subcategory of latent syphilis. When the date of initial infection cannot be established as occurring within the previous year, and the patient's age and titer meet criteria described below, latent syphilis is classified as unknown latent.
Case Classification
Presumptive
Latent syphilis (see above) that does not meet the criteria for early latent syphilis, and the patient is 13-35 years of age with a nontreponemal test serologic titer of greater than or equal to 32
Clinical Description
Evidence of central nervous system infection with Treponema pallidum
Laboratory Criteria For Diagnosis
A reactive serologic test for syphilis and reactive VDRL in cerebrospinal fluid (CSF)
Case Classification
Presumptive
Syphilis of any stage, a negative VDRL in CSF, and both of 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
A fetal death that occurs after a 20-week gestation or in which the fetus weighs greater than 500g, 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
Presumptive
The infection of an infant whose mother had untreated or inadequately treated* syphilis at delivery, regardless of signs in the infant; or the infection of 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 long bone x-ray
- A reactive cerebrospinal fluid (CSF) VDRL
- An elevated CSF cell count or protein (without other cause)
- A reactive test for fluorescent treponemal antibody absorbed-19S-IgM antibody
Confirmed
A case (among infants) 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 long bone x-rays may help, since x-ray changes in the metaphysis and epiphysis are considered classic for congenitally acquired disease. The decision may ultimately be based on maternal history and clinical judgment. The possibility of sexual abuse should be considered.
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.