Syphilis, Congenital (rev. 01/2015)

Clinical description

A condition caused by infection in utero with Treponema pallidum. A wide spectrum of 

severity exists, from inapparent infection to severe cases that 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 Treponema pallidum by:

• Darkfield microscopy of lesions, body fluids, or neonatal nasal discharge, or

• Polymerase chain reaction (PCR) or other equivalent direct molecular methods of 

lesions, neonatal nasal discharge, placenta, umbilical cord, or autopsy material, or

• Immunohistochemistry (IHC), or special stains (e.g., silver staining) of specimens 

from lesions, placenta, umbilical cord, or autopsy material.

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 non-treponemal test for syphilis (Venereal Disease Research Laboratory 

[VDRL], rapid plasma reagin [RPR], or equivalent serologic methods) AND any one of 

the following:

• Any evidence of congenital syphilis on physical examination (see Clinical 

description)

• Any evidence of congenital syphilis on radiographs of long bones

• A reactive cerebrospinal fluid (CSF) venereal disease research laboratory test 

(VDRL) test

• In a nontraumatic lumbar puncture, an elevated CSF leukocyte (white blood cell, 

WBC) count or protein (without other cause)

Suggested parameters for abnormal CSF WBC and protein values:

1. During the first 30 days of life, a CSF WBC count of >15 WBC/mm3 or a CSF 

protein >120 mg/dL.

2. After the first 30 days of life, a CSF WBC count of >5 WBC/mm3 or a CSF 

protein >40 mg/dL, regardless of CSF serology
The treating clinician should be consulted to interpret the CSF values for the specific 

patient.

Confirmed: a case that is laboratory confirmed

Syphilitic Stillbirth

Clinical case definition

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.

*Adequate treatment is defined as completion of a penicillin-based regimen, in 

accordance with CDC treatment guidelines, appropriate for stage of infection, initiated 30 

or more days before delivery.

Comment

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, WBC count, and protein 

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. While maternal antibodies can 

complicate interpretation of serologic tests in an infant, reactive tests past 18 months of 

age are considered to reflect the status of the child. 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.